porphobilinogen has been researched along with Porphyrias in 281 studies
Porphyrias: A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.
| Excerpt | Relevance | Reference |
|---|---|---|
| " The assay is developed for use in the clinical diagnosis of delta-aminolevulinic acid dehydratase-deficient porphyria, a rare enzymatic deficiency of the heme biosynthetic pathway." | 7.76 | Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry. ( Choiniere, JR; Gelb, MH; Scott, CR; Turecek, F, 2010) |
| "Biochemical disorders caused by allylisopropylacetamide in various animal species resemble human acute intermittent porphyria." | 7.67 | The effects of haem arginate and haematin upon the allylisopropylacetamide induced experimental porphyria in rats. ( Lindén, IB; Tenhunen, R; Tokola, O, 1987) |
| "The purpose of the present work is to investigate the ability of desferrioxamine (DF) as an iron chelator to revert or decrease a severe experimental porphyria induced by hexachlorobenzene (HCB) in rats; DF treatment started after 17 weeks of HCB intoxication and was continued until the 27th week." | 7.67 | [Evaluation of deferoxamine capacity to revert a severe porphyria induced by hexachlorobenzene]. ( Aldonatti, CA; Billi, SC; San Martín de Viale, LC; Wainstok de Calmanovici, R, 1987) |
| "The effect of p-amino-benzoic (PAB) acid on the experimental hexachlorobenzene (HCB) induced hepatic porphyria of female Wistar rats was determined under different conditions: Neither a simultaneous HCB-PAB application (prophylactic administration) nor the PAB application after manifestation of the HCB-porphyria (therapeutic administration) influenced significantly the excretin of urinary porphyrins or precursors (porphobilinogen or 5-amino-levulinic acid)." | 7.66 | [Influence of p-amino-benzoic acid on the hexachlorobenzene induced porphyria in the rat (author's transl)]. ( Goerz, G; Krieg, T; Lissner, R; Sick, N; Vizethum, W, 1980) |
| "Two male patients aged 23 and 25 years with intermittent acute, frequently repeated porphyria syndromes presented an almost total deficiency of porphobilinogen-synthase [(PBG-S); synonym: delta-aminolevulinic acid dehydratase] in peripheral erythrocytes." | 7.66 | Porphobilinogen-synthase (delta-aminolevulinic acid dehydratase) deficiency in bone marrow cells of two patients with porphobilinogen-synthase defect acute porphyria. ( Doss, M; Schneider, J; Tiepermann, RV, 1983) |
| "There is compelling, indirect evidence of hepatic heme deficiency due primarily to the respective genetic errors of the three inducible hepatic porphyrias, acute intermittent porphyria, porphyria variegata, and hereditary coproporphyria." | 7.65 | Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias. ( Bossenmaier, I; Cardinal, R; Pierach, CA; Watson, CJ, 1977) |
| "The effects of dietary manipulations on excretion of the porphyrin precursors, delta-aminolevulinic acid (ALA), and porphobilinogen (PBG) were studied in eight patients with acute intermittent porphyria." | 7.65 | Comparative effects of glycerol and dextrose on porphyrin precursor excretion in acute intermittent porphyria. ( Bonkowsky, HL; Collins, AR; Doherty, JM; Hess, RA; Magnussen, CR; Tschudy, DP, 1976) |
| "The excretion of delta-aminolevulinic acid and porphobilinogen in the urine of 31 patients with multiple sclerosis did not differ significantly from that of 51 hospitalized control patients or eight patients with poliomyelitis." | 7.64 | PORPHOBILINOGEN AND DELTA-AMINOLEVULINIC ACID EXCRETION IN MULTIPLE SCLEROSIS. ( MARKLE, V; PAZDER, LH; TAYLOR, JD, 1965) |
| "Porphyria has been associated with an increase in plasma levels of 5-aminolevulinic acid and porphobilinogen." | 5.29 | Altered 5-aminolevulinic acid metabolism leading to pseudoporphyria in hemodialysed patients. ( Del C Batlle, AM; Guolo, M; Melito, V; Parera, V; Stella, AM, 1996) |
| "Griseofulvin(GF) has become the drug of choice as an antifungal agent for patients who suffer from many kinds of fungal infection." | 5.28 | Effect of ursodeoxycholic acid on experimental hepatic porphyria induced by griseofulvin. ( Cho, HM; Choi, SW; Chung, KW; Han, JH; Kim, BS; Lim, KT; Park, DH; Seo, EJ; Sun, HS, 1991) |
| " After treatment with Resochin in an erroneously high dosage (1." | 5.26 | [Accident in treatment of porphyria cutanea tarda by chloroquine (Resochin) (author's transl)]. ( Eichenauer, MG; Goerz, G; Krieg, T, 1976) |
| " The drug was administered at a dosage of 200 mg t." | 5.26 | Danazol administration to females with menses-associated exacerbations of acute intermittent porphyria. ( Frykholm, BC; Herrera, W; Lamon, JM; Tschudy, DP, 1979) |
| "We studied one patient with major motor seizures and acute intermittent porphyria." | 5.26 | Seizure management in acute hepatic porphyria: risks of valproate and clonazepam. ( Bonkowsky, HL; Emery, S; Sinclair, JF; Sinclair, PR, 1980) |
| "We conclude that in some porphyric patients treatment with erythropoietin reduces urinary delta-aminolaevulinic acid, porphobilinogen and porphyrin levels with an increase in well-being and a reduction in the severity of porphyria attacks." | 5.10 | Erythropoietin treatment in the neuropsychiatric porphyrias. ( Chandler, G; Deacon, AC; Macdougall, IC; Marsden, JT; Peters, TJ; Winkler, AS, 2003) |
| "Porphyrias are a group of eight rare inherited metabolic disorders of heme biosynthesis pathway." | 4.86 | Porphyrias at a glance: diagnosis and treatment. ( Brancaleoni, V; Cappellini, MD; Di Pierro, E; Graziadei, G; Tavazzi, D, 2010) |
| "The quantification of delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) in urine are the first-line tests for diagnosis and monitoring of acute hepatic porphyrias (AHP)." | 4.31 | Quantification of Urine and Plasma Porphyrin Precursors Using LC-MS in Acute Hepatic Porphyrias: Improvement in Routine Diagnosis and in the Monitoring of Kidney Failure Patients. ( Dessendier, N; Gouya, L; Junot, C; Lefebvre, T; Manceau, H; Moulouel, B; Nguyen, AL; Poli, A; Puy, H; Schmitt, C; Talbi, N, 2023) |
| " The assay is developed for use in the clinical diagnosis of delta-aminolevulinic acid dehydratase-deficient porphyria, a rare enzymatic deficiency of the heme biosynthetic pathway." | 3.76 | Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry. ( Choiniere, JR; Gelb, MH; Scott, CR; Turecek, F, 2010) |
| " A provisional diagnosis of acute intermittent porphyria was made and was confirmed by the increased levels of urinary delta-aminolevulinic acid (ALA) and porphobilinogen (PBG)." | 3.68 | Acute intermittent porphyria. A perplexing case. ( Arena, A; Russo, P; Scuderi, D, 1992) |
| "Rats were treated with the well-known porphyrogen hexachlorobenzene (HCB) to induce experimental porphyria." | 3.67 | Influence of chloroquine on the porphyrin metabolism. ( Bolsen, K; Goerz, G; Merk, H, 1985) |
| "The purpose of the present work is to investigate the ability of desferrioxamine (DF) as an iron chelator to revert or decrease a severe experimental porphyria induced by hexachlorobenzene (HCB) in rats; DF treatment started after 17 weeks of HCB intoxication and was continued until the 27th week." | 3.67 | [Evaluation of deferoxamine capacity to revert a severe porphyria induced by hexachlorobenzene]. ( Aldonatti, CA; Billi, SC; San Martín de Viale, LC; Wainstok de Calmanovici, R, 1987) |
| "The manifestations of the acute attack of an hepatic porphyria may result from the actions of the excessive amounts of delta-aminolevulinic acid (ALA) or porphobilinogen (PBG) that accumulate during the event." | 3.67 | Sorbent therapy of the porphyrias. III. Comparative efficacy of experimental plasma perfusion with several commercial hemoperfusion cartridges. ( Tishler, PV; Winston, SH, 1984) |
| "An elderly woman was found to have hepatocellular carcinoma and, incidental to this, markedly elevated levels of porphobilinogen in urine and serum." | 3.67 | Pseudo-porphyria in a patient with hepatocellular carcinoma. ( Bossenmaier, IC; Cardinal, RA; Pierach, CA; Weimer, MK, 1984) |
| "Porphyria was induced in adult male Wistar rats starved for 24 hr by SC injection of 400 mg/kg allylisopropylacetamide (AIA)." | 3.67 | Endogenously formed norharman (beta-carboline) in platelet rich plasma obtained from porphyric rats. ( Bruinvels, J; Schouten, MJ, 1986) |
| "Urinary excretion of porphyrin precursors delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) and total porphyrins was measured during intoxication of rats with 2,4-dithiobiuret (DTB), a chemical which produces delayed-onset neuromuscular weakness, in an attempt to ascertain whether or not DTB poisoning in the rat would serve as an animal model of the neurologic symptoms of acute intermittent porphyria." | 3.67 | Excretion of porphyrins and porphyrin precursors during neuromuscular paralysis produced by dithiobiuret. ( Atchison, WD; Peterson, RE, 1984) |
| "Biochemical disorders caused by allylisopropylacetamide in various animal species resemble human acute intermittent porphyria." | 3.67 | The effects of haem arginate and haematin upon the allylisopropylacetamide induced experimental porphyria in rats. ( Lindén, IB; Tenhunen, R; Tokola, O, 1987) |
| "It was investigated whether an increased demand for glycine, as postulated to occur in patients who have suffered from episodic psychoses accompanied by multiple perceptual distortions, could evoke psychotic reactions." | 3.66 | Serine and glycine-induced catalepsy in porphyric rats: an animal model for psychosis? ( Bruinvels, J; Pepplinkhuizen, L; Schouten, MJ; Wilson, JH, 1983) |
| "Acute intermittent porphyria (AIP) is a primary disorder of haem biosynthesis that is chemically characterised by raised urinary porphobilinogen (PBG)." | 3.66 | Family evaluations in acute intermittent porphyria using red cell uroporphyrinogen I synthetase. ( Frykholm, BC; Lamon, JM; Tschudy, DP, 1979) |
| "The diagnosis of acute intermittent porphyria was made in 10 members of a large kindred because of increased excretion of porphobilinogen and delta-aminolevulinic acid in the urine but normal fecal porphyrins." | 3.66 | Normal erythrocyte uroporphyrinogen I synthase in a kindred with acute intermittent porphyria. ( Mustajoki, P, 1981) |
| "The effect of p-amino-benzoic (PAB) acid on the experimental hexachlorobenzene (HCB) induced hepatic porphyria of female Wistar rats was determined under different conditions: Neither a simultaneous HCB-PAB application (prophylactic administration) nor the PAB application after manifestation of the HCB-porphyria (therapeutic administration) influenced significantly the excretin of urinary porphyrins or precursors (porphobilinogen or 5-amino-levulinic acid)." | 3.66 | [Influence of p-amino-benzoic acid on the hexachlorobenzene induced porphyria in the rat (author's transl)]. ( Goerz, G; Krieg, T; Lissner, R; Sick, N; Vizethum, W, 1980) |
| "Fecal protoporphyrin is increased in patients with variegate porphyria, even during clinical remission, suggesting an enzymatic defect in the terminal portion of the heme biosynthetic pathway." | 3.66 | The enzymatic defect in variegate prophyria. Studies with human cultured skin fibroblasts. ( Bloomer, JR; Brenner, DA, 1980) |
| "Two male patients aged 23 and 25 years with intermittent acute, frequently repeated porphyria syndromes presented an almost total deficiency of porphobilinogen-synthase [(PBG-S); synonym: delta-aminolevulinic acid dehydratase] in peripheral erythrocytes." | 3.66 | Porphobilinogen-synthase (delta-aminolevulinic acid dehydratase) deficiency in bone marrow cells of two patients with porphobilinogen-synthase defect acute porphyria. ( Doss, M; Schneider, J; Tiepermann, RV, 1983) |
| " Before the diagnosis of an "inducible" porphyria is made, a positive Hoesch test requires that indoles, indoleacetic acid, methyldopa, end-stage alcoholic malnutrition, and phenazopyridine HCl be excluded, to avoid misinterpretation." | 3.65 | Comparison of the Hoesch and the Watson-Schwartz tests for urinary porphobilinogen. ( Bossenmaier, I; Cardinal, R; Pierach, CA; Watson, CJ, 1977) |
| " These findings cast doubt on the possibility that ALA or porphobilinogen is responsible for the production of the neural manifestations of acute porphyria." | 3.65 | Prophyrin precursors in blood, urine and cerebrospinal fluid in acute porphyria. ( Percy, VA; Shanley, BC, 1977) |
| "The effects of dietary manipulations on excretion of the porphyrin precursors, delta-aminolevulinic acid (ALA), and porphobilinogen (PBG) were studied in eight patients with acute intermittent porphyria." | 3.65 | Comparative effects of glycerol and dextrose on porphyrin precursor excretion in acute intermittent porphyria. ( Bonkowsky, HL; Collins, AR; Doherty, JM; Hess, RA; Magnussen, CR; Tschudy, DP, 1976) |
| "There is compelling, indirect evidence of hepatic heme deficiency due primarily to the respective genetic errors of the three inducible hepatic porphyrias, acute intermittent porphyria, porphyria variegata, and hereditary coproporphyria." | 3.65 | Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias. ( Bossenmaier, I; Cardinal, R; Pierach, CA; Watson, CJ, 1977) |
| "The effect of DL-propranolol on 3',5'-diethoxycarbonyl-1,4-dihydrocollidine-induced experimental porphyria was studied." | 3.65 | The effect of beta-adrenergic blocking agents on experimental porphyria induced by 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) in vivo and in vitro. ( Atsmon, A; Epstein, O; Schoenfeld, N, 1976) |
| "After the simultaneous intravenous administration of unconjugated bilirubin-(3)H and delta-aminolevulinic acid-4-(14)C, the plasma disappearance curves of unconjugated bilirubin-(3)H and the plasma appearance curves of biosynthesized unconjugated bilirubin-(14)C have been defined in seven patients, three of whom had acute intermittent porphyria (AIP)." | 3.65 | Quantitative studies of the delivery of hepatic-synthesized bilirubin to plasma utilizing -aminolevulinic acid-4- 14 C and bilirubin- 3 H in man. ( Berk, PD; Berlin, NI; Bloomer, JR; Howe, RB; Jones, EA; Shrager, R, 1972) |
| "The excretion of delta-aminolevulinic acid and porphobilinogen in the urine of 31 patients with multiple sclerosis did not differ significantly from that of 51 hospitalized control patients or eight patients with poliomyelitis." | 3.64 | PORPHOBILINOGEN AND DELTA-AMINOLEVULINIC ACID EXCRETION IN MULTIPLE SCLEROSIS. ( MARKLE, V; PAZDER, LH; TAYLOR, JD, 1965) |
| "The non-acute porphyrias are porphyria cutanea tarda, erythropoietic protoporphyria, X-linked protoporphyria, and the rare congenital erythropoietic porphyria." | 2.61 | Clinical Guide and Update on Porphyrias. ( Doss, MO; Schuppan, D; Stölzel, U, 2019) |
| "The porphyrias are uncommon disorders of haem biosynthesis and their effective management requires prompt and accurate diagnosis." | 2.41 | ACP Best Practice No 165: front line tests for the investigation of suspected porphyria. ( Deacon, AC; Elder, GH, 2001) |
| "The porphyrias are disorders of haem biosynthesis which present with acute neurovisceral attacks or disorders of sun-exposed skin." | 1.46 | Best practice guidelines on first-line laboratory testing for porphyria. ( Badminton, M; Brazil, N; Degg, T; Marsden, JT; Reed, P; Stewart, MF; Whatley, S; Woolf, J, 2017) |
| "Porphyria has been associated with an increase in plasma levels of 5-aminolevulinic acid and porphobilinogen." | 1.29 | Altered 5-aminolevulinic acid metabolism leading to pseudoporphyria in hemodialysed patients. ( Del C Batlle, AM; Guolo, M; Melito, V; Parera, V; Stella, AM, 1996) |
| " For groups 2 and 6, atracurium was administered at a dosage of 4 mg/kg of body weight, followed by a continuous infusion of 15 mg/kg/h." | 1.29 | Porphyrinogenic effects of atracurium, vecuronium, and pancuronium in a primed rat model. ( Bach, A; Böhrer, H; Bolsen, K; Goerz, G; Kohl, B; Martin, E; Schmidt, H, 1994) |
| "Griseofulvin(GF) has become the drug of choice as an antifungal agent for patients who suffer from many kinds of fungal infection." | 1.28 | Effect of ursodeoxycholic acid on experimental hepatic porphyria induced by griseofulvin. ( Cho, HM; Choi, SW; Chung, KW; Han, JH; Kim, BS; Lim, KT; Park, DH; Seo, EJ; Sun, HS, 1991) |
| " Adjustments in dosage or route of administration were sometimes needed." | 1.28 | A gonadotropin releasing hormone analogue prevents cyclical attacks of porphyria. ( Anderson, KE; Bardin, CW; Kappas, A; Spitz, IM, 1990) |
| "The porphyrias are caused by hereditary defects in the synthesis of heme." | 1.27 | The porphyrias. ( Rosen, T; Sekula, SA; Tschen, JA, 1986) |
| "We studied one patient with major motor seizures and acute intermittent porphyria." | 1.26 | Seizure management in acute hepatic porphyria: risks of valproate and clonazepam. ( Bonkowsky, HL; Emery, S; Sinclair, JF; Sinclair, PR, 1980) |
| "Porphobilinogen oxygenase was isolated from red cells of healthy persons and of patients with disturbances in porphyrin metabolism." | 1.26 | Porphobilinogen oxygenase from human erythrocytes. ( Frydman, B; Frydman, RB; Tomaro, ML, 1979) |
| "Antipyrine T1/2 was especially prolonged in patients with a history of more severe symptoms, but there was no correlation with the degree of elevation in urinary excretion of the porphyrin precursors delta-aminolevulinic acid (ALA) and porphobilinogen (PBG)." | 1.26 | Studies in porphyria. V. Drug oxidation rates in hereditary hepatic porphyria. ( Alvares, AP; Anderson, KE; Kappas, A; Sassa, S, 1976) |
| " After treatment with Resochin in an erroneously high dosage (1." | 1.26 | [Accident in treatment of porphyria cutanea tarda by chloroquine (Resochin) (author's transl)]. ( Eichenauer, MG; Goerz, G; Krieg, T, 1976) |
| " The drug was administered at a dosage of 200 mg t." | 1.26 | Danazol administration to females with menses-associated exacerbations of acute intermittent porphyria. ( Frykholm, BC; Herrera, W; Lamon, JM; Tschudy, DP, 1979) |
| "Intermittent acute porphyria has recently been distinguished biochemically from other genetic hepatic porphyrias by the observation of diminished hepatic uroporphyrinogen I synthetase activity and increased delta-aminolevulinic acid synthetase activity." | 1.25 | Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria. ( Felsher, BF; Marver, HS; Meyer, UA; Redeker, AG; Strand, LJ, 1972) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 243 (86.48) | 18.7374 |
| 1990's | 30 (10.68) | 18.2507 |
| 2000's | 3 (1.07) | 29.6817 |
| 2010's | 4 (1.42) | 24.3611 |
| 2020's | 1 (0.36) | 2.80 |
| Authors | Studies |
|---|---|
| Poli, A | 1 |
| Manceau, H | 1 |
| Nguyen, AL | 1 |
| Moulouel, B | 1 |
| Dessendier, N | 1 |
| Talbi, N | 1 |
| Puy, H | 1 |
| Junot, C | 1 |
| Gouya, L | 1 |
| Schmitt, C | 1 |
| Lefebvre, T | 1 |
| Stölzel, U | 1 |
| Doss, MO | 4 |
| Schuppan, D | 1 |
| Woolf, J | 1 |
| Marsden, JT | 2 |
| Degg, T | 1 |
| Whatley, S | 1 |
| Reed, P | 1 |
| Brazil, N | 1 |
| Stewart, MF | 1 |
| Badminton, M | 1 |
| Choiniere, JR | 1 |
| Scott, CR | 1 |
| Gelb, MH | 1 |
| Turecek, F | 1 |
| Cappellini, MD | 1 |
| Brancaleoni, V | 1 |
| Graziadei, G | 1 |
| Tavazzi, D | 1 |
| Di Pierro, E | 1 |
| WESTALL, RG | 1 |
| BRUGSCH, J | 2 |
| COOKSON, GH | 1 |
| RIMINGTON, C | 5 |
| BROCKMAN, PE | 1 |
| GRAY, CH | 2 |
| WATSON, CJ | 13 |
| GOLDBERG, A | 13 |
| MERCHANTE, A | 1 |
| WAJCHENBERG, BL | 1 |
| SCHWARTZ, S | 1 |
| HAEGER, B | 1 |
| WEISSENBORN, E | 1 |
| BLEIFER, SB | 1 |
| ALPHAS, SJ | 1 |
| ACKNER, B | 1 |
| COOPER, JE | 1 |
| KELLY, M | 1 |
| NICHOLSON, DC | 1 |
| BOSSENMAIER, I | 9 |
| CARDINAL, R | 7 |
| WITH, TK | 5 |
| JAMES, GW | 1 |
| RUDOLPH, SG | 1 |
| ABBOTT, LD | 1 |
| BARIETY, M | 1 |
| GAJDOS, A | 1 |
| GAJDOS-TOROK, M | 1 |
| POULET, J | 1 |
| RUFFINO, J | 1 |
| SMITH, SG | 1 |
| KELENYI, G | 2 |
| ARATO, G | 2 |
| BUDA, V | 2 |
| ORBAN, S | 2 |
| HAEGER-ARONSEN, B | 2 |
| STRUZIK, T | 1 |
| RUNGE, W | 1 |
| TOWNSEND, JD | 1 |
| TADDEINI, L | 1 |
| SCHLENKER, FS | 1 |
| TAYLOR, NA | 1 |
| KIEHN, BP | 1 |
| TAYLOR, JD | 1 |
| PAZDER, LH | 1 |
| MARKLE, V | 1 |
| GRANICK, S | 3 |
| VANDEN SCHRIECK, HG | 1 |
| JANOUSEK, V | 1 |
| OCENASEK, M | 1 |
| Winkler, AS | 1 |
| Peters, TJ | 2 |
| Deacon, AC | 3 |
| Chandler, G | 1 |
| Macdougall, IC | 1 |
| ERIKSEN, L | 2 |
| Kondo, M | 3 |
| Pierach, CA | 7 |
| Bossenmaier, IC | 1 |
| Cardinal, RA | 2 |
| Weimer, MK | 1 |
| Ibraham, NG | 1 |
| Friedland, ML | 1 |
| Levere, RD | 4 |
| Schouten, MJ | 2 |
| Bruinvels, J | 2 |
| Pepplinkhuizen, L | 1 |
| Wilson, JH | 2 |
| Laiwah, AC | 2 |
| Junor, B | 1 |
| MacPhee, GJ | 2 |
| Thompson, GG | 7 |
| McColl, KE | 8 |
| Atchison, WD | 1 |
| Peterson, RE | 1 |
| Ng, SC | 1 |
| Teo, SK | 1 |
| Tishler, PV | 2 |
| Winston, SH | 1 |
| Knosała, P | 1 |
| Petelenz, T | 1 |
| Zuk-Popiołek, I | 1 |
| Savi, M | 1 |
| David, S | 1 |
| Dall'Aglio, P | 1 |
| Lahav, M | 1 |
| Schoenfeld, N | 5 |
| Epstein, O | 2 |
| Greenblat, Y | 1 |
| Atsmon, A | 6 |
| te Velde, K | 1 |
| Noordhoek, KH | 1 |
| Mital, RN | 1 |
| Gautam, A | 1 |
| Bissell, DM | 2 |
| Bonkowsky, HL | 5 |
| Schady, W | 1 |
| Pathak, MA | 1 |
| West, JD | 1 |
| Sinclair, PR | 1 |
| Emery, S | 1 |
| Sinclair, JF | 1 |
| Moore, MR | 15 |
| Doss, M | 13 |
| Tiepermann, RV | 1 |
| Schneider, J | 2 |
| Gordon, BJ | 1 |
| O'Connor, LJ | 1 |
| Stewart, PM | 1 |
| Hensley, WJ | 1 |
| Goerz, G | 6 |
| Sick, N | 1 |
| Vizethum, W | 1 |
| Lissner, R | 1 |
| Krieg, T | 3 |
| Roby, HP | 1 |
| Harrison, GA | 1 |
| Romeo, G | 1 |
| Sassa, S | 5 |
| Kappas, A | 8 |
| Waldenström, J | 1 |
| Wallace, AM | 2 |
| Day, RS | 1 |
| Eales, L | 6 |
| Alleman, MA | 1 |
| van den Berg, JW | 1 |
| Edixhoven-Bosdijk, A | 1 |
| van Gastel-Quist, LM | 1 |
| Loftin, EB | 1 |
| Marchiori, PE | 2 |
| Scaff, M | 2 |
| de Camargo, E | 1 |
| Moraes, F | 1 |
| Bacheschi, LA | 2 |
| de Assis, JL | 2 |
| Adam, C | 1 |
| Hassoun, A | 1 |
| Defresne, C | 1 |
| Guilmot-Bruneau, M | 1 |
| Wery-Follet, B | 1 |
| Saussez-De Temmerman, A | 1 |
| Lai, CW | 1 |
| Wright, YG | 1 |
| Parvata, CP | 1 |
| Westerhof, W | 1 |
| Smit, EM | 1 |
| Mustajoki, P | 3 |
| Bottomley, SS | 2 |
| Kreimer-Birnbaum, M | 2 |
| Ganda, C | 1 |
| Joshi, P | 1 |
| Kramer, S | 4 |
| Brenner, DA | 1 |
| Bloomer, JR | 2 |
| Kostrzewska, E | 1 |
| Gregor, A | 2 |
| Lipska-Koziołowa, H | 1 |
| Traczyk, A | 1 |
| Lockwood, WH | 1 |
| Poulos, V | 1 |
| Bannerman, RM | 1 |
| El-Khatib, M | 1 |
| Franco-Saenz, R | 1 |
| Wider de Xifra, EA | 2 |
| Batlle, AM | 2 |
| Stella, AM | 3 |
| Malamud, S | 1 |
| von Tiepermann, R | 1 |
| Fuchs, T | 2 |
| Seubert, A | 2 |
| Seubert, S | 2 |
| Ippen, H | 3 |
| Buttery, JE | 2 |
| Gross, U | 1 |
| Honcamp, M | 1 |
| Daume, E | 1 |
| Frank, M | 2 |
| Düsterberg, B | 1 |
| Horie, Y | 1 |
| Norimoto, M | 1 |
| Tajima, F | 1 |
| Sasaki, H | 1 |
| Nanba, E | 1 |
| Kawasaki, H | 1 |
| Pinkham, JM | 1 |
| Southall, DP | 1 |
| Gullotti, D | 1 |
| Schillaci, L | 1 |
| Amato, S | 1 |
| Salerno, L | 1 |
| Böhrer, H | 1 |
| Schmidt, H | 2 |
| Bach, A | 1 |
| Martin, E | 1 |
| Kohl, B | 1 |
| Bolsen, K | 3 |
| Mamet, R | 2 |
| Mevasser, R | 2 |
| Badcock, NR | 1 |
| O'Reilly, DA | 1 |
| Zoanetti, GD | 1 |
| Robertson, EF | 1 |
| Parker, CJ | 1 |
| Guolo, M | 1 |
| Melito, V | 1 |
| Parera, V | 1 |
| Del C Batlle, AM | 1 |
| Ellefson, RD | 1 |
| Ford, RE | 1 |
| Pivnik, AV | 1 |
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| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Evidence-based Assessment of Medication Sensitivity in Acute Hepatic Porphyria[NCT03906214] | 60 participants (Actual) | Observational | 2019-04-03 | Completed | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
29 reviews available for porphobilinogen and Porphyrias
| Article | Year |
|---|---|
Clinical Guide and Update on Porphyrias.
Topics: Aminolevulinic Acid; Gastroenterology; Gastrointestinal Diseases; Humans; Nervous System Diseases; P | 2019 |
Porphyrias at a glance: diagnosis and treatment.
Topics: Acute Disease; Aminolevulinic Acid; Chronic Disease; Genetic Testing; Heme; Humans; Porphobilinogen; | 2010 |
Heme metabolism in erythroid and hepatic cells.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Coproporphyrinogen Oxidase; Erythrocytes; Erythro | 1983 |
Laboratory evaluation in porphyria.
Topics: Acute Disease; Aminolevulinic Acid; Animals; Carrier State; Chemistry, Clinical; Diagnosis, Differen | 1982 |
Neurologic manifestations of acute porphyria.
Topics: Acute Disease; Aminolevulinic Acid; Animals; Central Nervous System; Depression; Electrophysiology; | 1982 |
Porphyrias: office procedures and laboratory tests for diagnosis of porphyrin abnormalities.
Topics: Chromatography, High Pressure Liquid; Chromatography, Paper; Clinical Enzyme Tests; Feces; Humans; I | 1982 |
The hepatic porphyrias.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Chemical Phenom | 1980 |
Genetic, metabolic, and biochemical aspects of the porphyrias.
Topics: Aminolevulinic Acid; Animals; Cat Diseases; Cats; Cattle; Cattle Diseases; Erythrocytes; Heme; Human | 1981 |
The porphyrias--a review.
Topics: Acute Disease; Aged; Alcoholism; Aminolevulinic Acid; Feces; Female; Heme; Humans; Iron; Liver Disea | 1981 |
[Methods of determination of porphyrins and their precursors--introduction of analytical methods for porphyrin metabolites].
Topics: Aminolevulinic Acid; Chromatography; Chromatography, Thin Layer; Electrophoresis; Humans; Porphobili | 1995 |
[Porphyrins in urine, blood, and feces and delta-aminolevulinic acid and porphobilinogen].
Topics: Aminolevulinic Acid; Chromatography, High Pressure Liquid; Diagnosis, Differential; Feces; Humans; P | 1995 |
The porphyrias [corrected]: characteristics and laboratory tests.
Topics: Aminolevulinic Acid; Environmental Exposure; Feces; Humans; Porphobilinogen; Porphyrias; Porphyrins | 1996 |
ACP Best Practice No 165: front line tests for the investigation of suspected porphyria.
Topics: Biomarkers; Humans; Porphobilinogen; Porphyrias; Porphyrins; Quality Assurance, Health Care; Specime | 2001 |
Enzymatic formation and cellular regulation of heme synthesis.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Coenzyme A; Coproporphyrinogens; Enzyme | 1977 |
The neurological manifestations of porphyria: a review.
Topics: Acute Disease; Aminolevulinic Acid; Animals; Heme; Humans; Liver; Liver Diseases; Nervous System; Ne | 1977 |
Hepatic porphyrias. Current concepts.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Coproporphyrinogens; Female; Genetic Diseases, Inborn; | 1977 |
[Pathobiochemistry of porphyrias].
Topics: Acute Disease; Aminolevulinic Acid; Catalase; Chronic Disease; Cytochrome P-450 Enzyme System; Heme; | 1977 |
Use of hematin in the acute attack of the "inducible" hepatic prophyrias.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adult; Female; Heme; Hemin; Humans; Male; Middle Aged; | 1978 |
[Chromosome studies in patients with intermittent porphyria].
Topics: Adult; Aminolevulinic Acid; Chromosome Aberrations; Female; Heme; Humans; Hydroxymethylbilane Syntha | 1979 |
[Porphyria--biochemical background, clinical aspects and diagnosis].
Topics: 5-Aminolevulinate Synthetase; Erythropoiesis; Humans; Light; Porphobilinogen; Porphyrias; Porphyrins | 1979 |
[Porphyria variegata (author's transl)].
Topics: Adult; Aminolevulinic Acid; Coproporphyrins; Erythrocytes; Feces; Female; Hand; Humans; Lip; Porphob | 1975 |
[Acute intermittent porphyria simulating encephalitis].
Topics: Acute Disease; Adult; Diagnosis, Differential; Encephalitis; Epilepsy, Tonic-Clonic; Humans; Hydroxy | 1989 |
Haem biosynthesis and porphyrias: 50 years in retrospect.
Topics: Biochemistry; Heme; History, 20th Century; Humans; Models, Biological; Porphobilinogen; Porphyrias; | 1989 |
Pathogenesis of acute porphyria.
Topics: Acute Disease; Aminolevulinic Acid; Animals; Heme; Humans; Nervous System; Porphobilinogen; Porphyri | 1987 |
Significance of the porphyrin precursors delta-aminolaevulinic acid (ALA) and porphobilinogen (PBG) in the acute attack of porphyria.
Topics: Acute Disease; Adenosine Triphosphatases; Allylisopropylacetamide; Animals; Anura; Brain; Electrophy | 1973 |
Intermittent acute porphyria: the enzymatic defect.
Topics: 5-Aminolevulinate Synthetase; Adenosine Triphosphatases; Alanine; Aminolevulinic Acid; Ammonia-Lyase | 1974 |
[Hepatic porphyrias].
Topics: Animals; Heme; Humans; Levulinic Acids; Liver; Porphobilinogen; Porphyrias; Porphyrins | 1972 |
[Oral contraceptives causing porphyria cutanea tarda].
Topics: Contraceptives, Oral; Female; Humans; Levulinic Acids; Liver; Porphobilinogen; Porphyrias; Porphyrin | 1973 |
[Acute intermittent porphyria].
Topics: Acute Disease; Age Factors; Animals; Diagnosis, Differential; Female; Humans; Levulinic Acids; Male; | 1974 |
2 trials available for porphobilinogen and Porphyrias
| Article | Year |
|---|---|
Erythropoietin treatment in the neuropsychiatric porphyrias.
Topics: Adult; Aged; Aminolevulinic Acid; Erythropoietin; Female; Hematologic Tests; Hemoglobins; Humans; Ma | 2003 |
Effects of sodium valproate on haem biosynthesis in man: implications for seizure management in the porphyric patient.
Topics: 5-Aminolevulinate Synthetase; Adolescent; Adult; Drug Administration Schedule; Epilepsy; Female; Hem | 1988 |
250 other studies available for porphobilinogen and Porphyrias
| Article | Year |
|---|---|
Quantification of Urine and Plasma Porphyrin Precursors Using LC-MS in Acute Hepatic Porphyrias: Improvement in Routine Diagnosis and in the Monitoring of Kidney Failure Patients.
Topics: Aminolevulinic Acid; Chromatography, Liquid; Humans; Porphobilinogen; Porphyrias; Porphyrias, Hepati | 2023 |
Best practice guidelines on first-line laboratory testing for porphyria.
Topics: Acute Disease; Algorithms; Chromatography, Liquid; Chronic Disease; Colorimetry; Feces; Fluorometry; | 2017 |
Direct assay of delta-aminolevulinic acid dehydratase in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry.
Topics: Aminolevulinic Acid; Deuterium; Erythrocytes; Heme; Humans; Kinetics; Porphobilinogen; Porphobilinog | 2010 |
Isolation of porphobilinogen from the urine of a patient with acute porphyria.
Topics: Animals; Body Fluids; Humans; Porifera; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias | 1952 |
[Quantitative determination of free uroporphyrin, uroporphyrin zinc complex salt and porphobilinogen in urine porphyria].
Topics: Animals; Humans; Pigments, Biological; Porifera; Porphobilinogen; Porphyrias; Porphyrins; Uroporphyr | 1952 |
Porphobilinogen; chemical constitution.
Topics: Animals; Humans; Pigments, Biological; Porifera; Porphobilinogen; Porphyrias | 1953 |
Crystalline porphobilinogen from urine of rabbits treated with sedormid.
Topics: Animals; Hypnotics and Sedatives; Naphthalenesulfonates; Porphobilinogen; Porphyrias; Porphyrins; Ra | 1953 |
Some studies of nature and clinical significance of porphobilinogen.
Topics: Body Fluids; Humans; Pigments, Biological; Porphobilinogen; Porphyrias; Porphyrins; Urine | 1954 |
Renal clearance of endogenous porphobilinogen in man.
Topics: Humans; Male; Porphobilinogen; Porphyrias; Porphyrins; Urinary Tract Physiological Phenomena; Urine | 1954 |
Conversion of porphobilinogen to porphyrin by liver homogenates of rats with experimental hepatic porphyria.
Topics: Animals; Liver; Porphobilinogen; Porphyrias; Porphyrias, Hepatic; Porphyrins; Rats | 1957 |
Urinary delta-aminolaevulic acid and porphobilinogen in different types of porphyria.
Topics: Amino Acids; Antifibrinolytic Agents; Humans; Porphobilinogen; Porphyrias; Porphyrins; Urinary Tract | 1958 |
[Differentiation of leuko-combinations of uroporphyrin from porphobilinogen by quantitative studies].
Topics: Body Fluids; Humans; Porphobilinogen; Porphyrias; Uroporphyrins | 1958 |
Acute intermittent porphyria; report of a case associated with porphobilinogen in the urine of four of five children.
Topics: Body Fluids; Child; Humans; Medical Records; Porphobilinogen; Porphyria, Acute Intermittent; Porphyr | 1959 |
Excretion of porphobilinogen and 5-aminolaevulinic acid in acute porphyria.
Topics: Amino Acids; Humans; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias; Pyrroles | 1961 |
Acute intermittent porphyria. Urinary porphobilinogen and other Ehrlich reactors in diagnosis.
Topics: Humans; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias | 1961 |
Excretion of porphobilinogen and Delta-aminolaevulinic acid in acute porphyria.
Topics: Amino Acids; Aminolevulinic Acid; Biological Products; Humans; Pigments, Biological; Porphobilinogen | 1961 |
Delta-amino-levulinic acid, porphobilinogen, and porphyrin excretion throughout pregnancy in a patient with acute intermittent porphyria with "passive porphyria" in the infant.
Topics: Amino Acids; Aminolevulinic Acid; Female; Humans; Infant, Newborn, Diseases; Porphobilinogen; Porphy | 1961 |
[Cutaneous porphyria in adults with urinary excretion of porphobilinogen and presence of uroporphyrin in the sternal marrow and pulmonary tissue].
Topics: Administration, Cutaneous; Bone Marrow; Humans; Lung; Porphobilinogen; Porphyrias; Skin Diseases; St | 1960 |
The porphobilinogen in fresh postmortem tissues from a case of acute intermittent porphyria.
Topics: Animals; Autopsy; Humans; Porifera; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias; Porp | 1960 |
Urinary excretion of porphobilinogen in acute porphyria.
Topics: Humans; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias | 1960 |
Porphyria induced in the rabbit by diethyl 1,4-dihydro-2,4,6-trimethylpyridine-3,5-dicarboxylate. I. Excretion of 5-aminolaevulic acid, porphobilinogen, coproporphyrin and protoporphyrin.
Topics: Amino Acids; Animals; Coproporphyrins; Porphobilinogen; Porphyrias; Porphyrins; Protoporphyrins; Pyr | 1961 |
[Problems in the field of porphyrin synthesis disorders in neoplastic diseases in connection with the presence of a porphobilinogen-like substance in urine of a patient with cancer of the colon].
Topics: Colonic Neoplasms; Humans; Porphobilinogen; Porphyrias; Porphyrins | 1962 |
Increased urinary porphobilinogen and uroporphyrin after administration of stilboestrol in a case of latent porphyria.
Topics: Amino Acids; Diethylstilbestrol; Humans; Pigments, Biological; Porphobilinogen; Porphyrias; Porphyri | 1962 |
AN EVALUATION OF A RECENT MODIFICATION OF THE WATSON-SCHWARTZ TEST FOR PORPHOBILINOGEN.
Topics: Clinical Laboratory Techniques; Humans; Indicators and Reagents; Porphobilinogen; Porphyrias; Porphy | 1964 |
PRESENT STATUS OF THE EHRLICH ALDEHYDE REACTION FOR URINARY PORPHOBILINOGEN.
Topics: Acetates; Alcohols; Aldehydes; Chloroform; Chromatography; Humans; Hydrochloric Acid; Indicators and | 1964 |
THE CHROMATOGRAPHIC SEPARATION, DETERMINATION, AND DAILY EXCRETION OF URINARY PORPHOBILINOGEN, AMINO ACETONE, AND DELTA-AMINOLEVULINIC ACID.
Topics: Acetone; Aminolevulinic Acid; Anemia; Anemia, Sickle Cell; Chromatography; Fluids and Secretions; Ho | 1964 |
PORPHOBILINOGEN AND DELTA-AMINOLEVULINIC ACID EXCRETION IN MULTIPLE SCLEROSIS.
Topics: Amino Acids; Aminolevulinic Acid; Demyelinating Diseases; Diagnosis, Differential; Fluids and Secret | 1965 |
Porphobilinogen and delta-amino levulinic acid in acute porphyria.
Topics: Aminolevulinic Acid; Humans; Keto Acids; Ketones; Porphobilinogen; Porphyria, Acute Intermittent; Po | 1955 |
[Excretion of delta-aminolevulinic acid and porphobilinogen in experimental porphyria in rabbits].
Topics: Amino Acids; Aminolevulinic Acid; Animals; Humans; Porphobilinogen; Porphyrias; Rabbits | 1960 |
[On the excretion of 5-aminolevulinic acid and porphobilinogen in latent porphyria].
Topics: Amino Acids; Aminolevulinic Acid; Humans; Porphobilinogen; Porphyrias; Porphyrins; Pyrroles | 1961 |
Acute porphyria. II. Some observations on the conversion products from porphobilinogen.
Topics: Humans; Porphobilinogen; Porphyria, Acute Intermittent; Porphyrias | 1951 |
Acute porphyria. III. The separation of preformed porphyrin and porphobilinogen in the urine in acute porphyria; a coproporphyrin chromogen in normal and pathological urine.
Topics: Coproporphyrins; Humans; Naphthalenesulfonates; Porphobilinogen; Porphyria, Acute Intermittent; Porp | 1951 |
[Porphobilinogen].
Topics: Acute Disease; Humans; Porphobilinogen; Porphyrias | 2004 |
Pseudo-porphyria in a patient with hepatocellular carcinoma.
Topics: Abdominal Neoplasms; Aged; alpha-Fetoproteins; Carcinoma, Hepatocellular; Feces; Female; Humans; Liv | 1984 |
Serine and glycine-induced catalepsy in porphyric rats: an animal model for psychosis?
Topics: Allylisopropylacetamide; Aminolevulinic Acid; Animals; Catalepsy; Disease Models, Animal; Glycine; H | 1983 |
Charcoal haemoperfusion and haemodialysis in acute intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Charcoal; Female; Hemoperfusion; Humans; Porphobilinogen; | 1983 |
Excretion of porphyrins and porphyrin precursors during neuromuscular paralysis produced by dithiobiuret.
Topics: Aminolevulinic Acid; Animals; Disease Models, Animal; Levulinic Acids; Male; Muscle Hypotonia; Neuro | 1984 |
Acute intermittent porphyria in an Indian male adult.
Topics: Acute Disease; Adult; Alcohol Drinking; Humans; Male; Porphobilinogen; Porphyrias; Porphyrins; Recur | 1984 |
Sorbent therapy of the porphyrias. III. Comparative efficacy of experimental plasma perfusion with several commercial hemoperfusion cartridges.
Topics: Aminolevulinic Acid; Charcoal; Hemoperfusion; Humans; Porphobilinogen; Porphyrias | 1984 |
[A case of acute hepatic porphyria].
Topics: Acute Disease; Adult; Aminolevulinic Acid; Coproporphyrins; Humans; Liver Diseases; Male; Porphobili | 1983 |
[Clinical heterogeneity of hereditary coproporphyria: diagnostic usefulness of biochemical studies. Study of a familial case].
Topics: Aminolevulinic Acid; Coproporphyrins; Female; Humans; Middle Aged; Pedigree; Porphobilinogen; Porphy | 1983 |
Effect of prolonged fasting on heme metabolism in the rat.
Topics: 5-Aminolevulinate Synthetase; Animals; Chromatography, Thin Layer; Fasting; Heme; Male; Phenobarbita | 1984 |
[Hereditary coproporphyria in 2 Dutch families].
Topics: Adult; Aged; Aminolevulinic Acid; Coproporphyrins; Feces; Female; Humans; Male; Middle Aged; Pedigre | 1984 |
Porphyria.
Topics: Adult; Humans; Male; Porphobilinogen; Porphyrias | 1984 |
Seizure management in acute hepatic porphyria: risks of valproate and clonazepam.
Topics: 5-Aminolevulinate Synthetase; Adult; Aminolevulinic Acid; Animals; Benzodiazepinones; Chick Embryo; | 1980 |
Pathogenesis and treatment of acute intermittent porphyria: discussion paper.
Topics: Acute Disease; Aminolevulinic Acid; Blood-Brain Barrier; Heme; Humans; Liver Diseases; Porphobilinog | 1983 |
Porphobilinogen-synthase (delta-aminolevulinic acid dehydratase) deficiency in bone marrow cells of two patients with porphobilinogen-synthase defect acute porphyria.
Topics: Acute Disease; Adult; Bone Marrow; Bone Marrow Cells; Erythrocytes; Homozygote; Humans; Male; Porpho | 1983 |
Sorbent therapy of the porphyrias. II. Experimental plasma or hemoperfusion with a commercial charcoal cartridge.
Topics: Adsorption; Aminolevulinic Acid; Charcoal; Evaluation Studies as Topic; Hemin; Hemoperfusion; Humans | 1983 |
Acute intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Female; Humans; Male; Porphobilinogen; Porphyrias; Seizur | 1981 |
An acute attack of variegate porphyria complicated by severe autonomic neuropathy.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Autonomic Nervous System Diseases; Female; Humans; Porpho | 1981 |
[Influence of p-amino-benzoic acid on the hexachlorobenzene induced porphyria in the rat (author's transl)].
Topics: 4-Aminobenzoic Acid; Aminobenzoates; Aminolevulinic Acid; Animals; Chlorobenzenes; Cytochrome P-450 | 1980 |
Anaesthesia for coronary artery bypass in a patient with porphyria variegata.
Topics: Adult; Anesthesia, General; Coronary Artery Bypass; Humans; Male; Porphobilinogen; Porphyrias; Porph | 1982 |
Alterations in haem biosynthesis during the human menstrual cycle: studies in normal subjects and patients with latent and active acute intermittent porphyria.
Topics: 5-Aminolevulinate Synthetase; Adrenal Cortex Hormones; Adult; Aminolevulinic Acid; Erythrocytes; Fem | 1982 |
Acute intermittent porphyria.
Topics: Acute Disease; Aminolevulinic Acid; Humans; Levulinic Acids; Liver Diseases; Porphobilinogen; Porphy | 1982 |
Familial porphyria cutanea tarda: the pattern of porphyrins formed from porphobilinogen by hemolysates.
Topics: Carboxy-Lyases; Chromatography, High Pressure Liquid; Erythrocytes; Female; Humans; Hydroxymethylbil | 1982 |
Hepatic porphyrias simulating typhoidal tularemia.
Topics: Adult; Diagnosis, Differential; Female; Humans; Liver Diseases; Porphobilinogen; Porphyrias; Tularem | 1982 |
Intermittent acute porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Female; Humans; Porphobilinogen; Porphyrias; Porphyrins | 1982 |
[Porphyrias. Considerations apropos of 26 cases].
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Child; Female; Humans; Male; Middle | 1982 |
[Hemin treatment in a case of porphyria variegata].
Topics: Adult; Aminolevulinic Acid; Coproporphyrins; Female; Heme; Hemin; Humans; Liver Diseases; Porphobili | 1982 |
Carbamazepine in seizure management in acute intermittent porphyria.
Topics: 5-Aminolevulinate Synthetase; Carbamazepine; Humans; Porphobilinogen; Porphyrias; Seizures | 1981 |
Anesthetic considerations of porphyria.
Topics: Adult; Anesthetics; Drug-Related Side Effects and Adverse Reactions; Female; Humans; Porphobilinogen | 1981 |
[Variegate porphyria; a family study].
Topics: Adult; Aminolevulinic Acid; Female; Humans; Male; Middle Aged; Pedigree; Porphobilinogen; Porphyrias | 1981 |
Normal erythrocyte uroporphyrinogen I synthase in a kindred with acute intermittent porphyria.
Topics: Aminolevulinic Acid; Ammonia-Lyases; Erythrocytes; Feces; Finland; Humans; Hydroxymethylbilane Synth | 1981 |
The diagnosis of acute intermittent porphyria. Usefulness and limitations of the erythrocyte uroporphyrinogen I synthase assay.
Topics: Acute Disease; Ammonia-Lyases; Erythrocytes; Hydroxymethylbilane Synthase; Porphobilinogen; Porphyri | 1981 |
Acute intermittent porphyria. A case report.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Coproporphyrins; Feces; Female; Humans; Male; Porphobilin | 1981 |
The enzymatic defect in variegate prophyria. Studies with human cultured skin fibroblasts.
Topics: Adult; Biopsy; Coproporphyrins; Feces; Female; Ferrochelatase; Fibroblasts; Heme; Humans; Oxidation- | 1980 |
[Porphyria variegata in Poland. Diagnosis and differentiation].
Topics: Adolescent; Adult; Aminolevulinic Acid; Child; Diagnosis, Differential; Feces; Female; Humans; Male; | 1980 |
A clinical porphyrin unit: four years' experience.
Topics: Aminolevulinic Acid; Australia; Hospital Units; Humans; Porphobilinogen; Porphyrias; Porphyrins; Ref | 1980 |
Afro-Americans and acute intermittent porphyria (AIP).
Topics: Acute Disease; Adolescent; Adult; Africa; Aminolevulinic Acid; Black People; Child; Child, Preschool | 1980 |
Acute intermittent porphyria--another approach to therapy.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adult; Aminolevulinic Acid; Ammonia-Lyases; Female; Fol | 1980 |
Acute hepatic porphyria syndrome with porphobilinogen synthase defect.
Topics: Acute Disease; Adult; Amino Acids; Aminolevulinic Acid; Erythrocytes; Feces; Female; Humans; Lead; L | 1980 |
Anticonvulsant-induced unclassified porphyria.
Topics: Adult; Aminolevulinic Acid; Anticonvulsants; Diagnosis, Differential; Epilepsy; Humans; Male; Phenob | 1980 |
Is the Watson-Schwartz screening method for porphobilinogen reliable?
Topics: Humans; Mass Screening; Porphobilinogen; Porphyrias | 1995 |
Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias.
Topics: Adult; Aminolevulinic Acid; Contraceptives, Oral, Combined; Contraceptives, Oral, Hormonal; Copropor | 1995 |
Clinical usefulness of cimetidine treatment for acute relapse in intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Blood Glucose; Cimetidine; Coproporphyrins; Feces; Humans | 1995 |
Unusual case of suspected acute porphyria.
Topics: Acute Disease; Adolescent; Humans; Hysteria; Male; Porphobilinogen; Porphyrias | 1995 |
[Clinical and laboratory findings concerning at first dietary and afterwards cimetidine treatment of acute intermittent porphyria].
Topics: Acute Disease; Adult; Aminolevulinic Acid; Cimetidine; Coproporphyrins; Female; Humans; Middle Aged; | 1994 |
Porphyrinogenic effects of atracurium, vecuronium, and pancuronium in a primed rat model.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Atracurium; Liver; Male; Pancuronium; Po | 1994 |
[Porphyria and porphyrinuria].
Topics: Aminolevulinic Acid; Diagnosis, Differential; Humans; Porphobilinogen; Porphyrias; Porphyrins; Retro | 1993 |
Childhood porphyrias: implications and treatments.
Topics: Adolescent; Aged; Aminolevulinic Acid; Child; Child, Preschool; Coproporphyrinogen Oxidase; Female; | 1993 |
Altered 5-aminolevulinic acid metabolism leading to pseudoporphyria in hemodialysed patients.
Topics: Acute Disease; Adult; Aged; Aminolevulinic Acid; Case-Control Studies; Female; Hot Temperature; Huma | 1996 |
Porphyric puzzle: comment on the concise communication by Cohen et al.
Topics: Humans; Porphobilinogen; Porphyrias | 1998 |
[The diagnosis of porphyrias].
Topics: Acute Disease; Chronic Disease; Diagnosis, Differential; Humans; Porphobilinogen; Porphyrias; Porphy | 1998 |
Identification of acute porphyria: evaluation of a commercial screening test for urinary porphobilinogen.
Topics: Acute Disease; Evaluation Studies as Topic; Humans; Porphobilinogen; Porphyrias; Reagent Kits, Diagn | 1998 |
The diagnosis of porphyria.
Topics: Aminolevulinic Acid; Female; Genetic Techniques; Genetic Testing; Heme; Humans; Mutation; Porphobili | 1999 |
The effect of beta-adrenergic blocking agents on experimental porphyria induced by 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) in vivo and in vitro.
Topics: 5-Aminolevulinate Synthetase; Adrenergic beta-Antagonists; Aminolevulinic Acid; Animals; Chick Embry | 1976 |
A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria.
Topics: Aminolevulinic Acid; Ammonia-Lyases; Erythrocytes; Hemolysis; Humans; Hydrogen-Ion Concentration; Po | 1978 |
Prevention of acute porphyric attacks by intravenous haematin.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Blood Pressure; Circadian Rhythm; Drug Evaluation; Female | 1978 |
[Urinary excretion of porphyrins and their precursors. Longterm observations in acute intermittent porphyrias (author's transl)].
Topics: Aminolevulinic Acid; Coproporphyrins; Estrogens; Humans; Porphobilinogen; Porphyrias; Porphyrins; Ur | 1979 |
Quantitation of 3-ethyl-5-hydroxy-4,5-dimethyl-delta 3-pyrrolin-2-one in the urine of patients with acute intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Chromatography, Gas; Female; Heme; Humans; Porphobilinoge | 1979 |
[Letter: Comments and conclusion to H. Stutzer: Typical and atypical roentgen pictures in the acute porphyria. Fortschr. Röntgenstr. 123,6 (1975) 588-590].
Topics: Abdomen, Acute; Acute Disease; Aminolevulinic Acid; Diagnosis, Differential; Female; Humans; Middle | 1976 |
Acute intermittent porphyria.
Topics: Acute Disease; Humans; Porphobilinogen; Porphyrias | 1979 |
Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias.
Topics: 5-Aminolevulinate Synthetase; Adult; Female; Heme; Hemin; Humans; Liver; Male; Porphobilinogen; Porp | 1977 |
Danazol administration to females with menses-associated exacerbations of acute intermittent porphyria.
Topics: Adult; Aminolevulinic Acid; Danazol; Female; Hemin; Humans; Menstruation; Porphobilinogen; Porphyria | 1979 |
Urinary total porphyrins by ion exchange analysis: reference values for the normal range and remarks on preformed prophyrins in acute porphyria urine.
Topics: Acute Disease; Adolescent; Adult; Aged; Female; Humans; Ion Exchange; Male; Middle Aged; Porphobilin | 1979 |
Family evaluations in acute intermittent porphyria using red cell uroporphyrinogen I synthetase.
Topics: Ammonia-Lyases; Erythrocytes; Female; Genes, Dominant; Humans; Hydroxymethylbilane Synthase; Male; P | 1979 |
[Effect of hematin in acute porphyric relapse].
Topics: Acute Disease; Adult; Aminolevulinic Acid; Coproporphyrins; Heme; Hemin; Humans; Male; Porphobilinog | 1979 |
Haem biosynthesis in cutaneous hepatic porphyria: comparison with alcoholism and liver disease.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Coproporphyrins; Erythrocytes; Ferrochelatase; He | 1979 |
[Intermittent acute porphyria (author's transl)].
Topics: Acute Disease; Erythrocytes; Hemin; Humans; Hydroxymethylbilane Synthase; Injections, Intravenous; L | 1979 |
Porphobilinogen oxygenase from human erythrocytes.
Topics: Animals; Erythrocytes; Female; Humans; Kinetics; Liver; Male; Mixed Function Oxygenases; Oxidoreduct | 1979 |
[Laboratory diagnosis of anomalies of porphyrin metabolism].
Topics: Aminolevulinic Acid; Humans; Methods; Porphobilinogen; Porphyrias; Porphyrins | 1979 |
Inherited deficiency of delta-aminolevulinic acid dehydratase.
Topics: Adolescent; Female; Genes, Dominant; Genes, Regulator; Humans; Mutation; Pedigree; Porphobilinogen; | 1979 |
A simple screening strategy for diagnosing the porphyrias.
Topics: Adult; Humans; Liver; Male; Middle Aged; Photosensitivity Disorders; Porphobilinogen; Porphyrias; Po | 1979 |
[Hematin treatment of acute intermittent porphyria. A case report (author's transl)].
Topics: Adolescent; Aminolevulinic Acid; Antihypertensive Agents; Electromyography; Female; Heme; Hemin; Hum | 1979 |
[Acute intermittent porphyria--a clinical review].
Topics: Acute Disease; Aminolevulinic Acid; Diagnosis, Differential; Heme; Humans; Porphobilinogen; Porphyri | 1979 |
Determination of 5-aminolaevulinic acid and porphobilinogen by high-performance liquid chromatography.
Topics: Aminolevulinic Acid; Animals; Chromatography, High Pressure Liquid; Erythrocytes; Levulinic Acids; P | 1979 |
[Uroporphyrinogen-I-synthetase in erythrocytes in acute intermittent porphyria].
Topics: Adolescent; Aminolevulinic Acid; Ammonia-Lyases; Bulgaria; Erythrocytes; Female; Humans; Hydroxymeth | 1977 |
Family studies on the activity of uroporphyrinogen I synthase in diagnosis of acute intermittent porphyria.
Topics: Adolescent; Adult; Aged; Aminolevulinic Acid; Ammonia-Lyases; Clinical Enzyme Tests; Female; Humans; | 1978 |
Acute intermittent porphyria - a case report.
Topics: Acute Disease; Adult; Female; Humans; Porphobilinogen; Porphyrias | 1978 |
Relationships between acute hepatic porphyrias due to genetic variability of primary enzyme defects and limiting function of uroporphyrinogen synthase.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Aged; Aminolevulinic Acid; Ammonia-L | 1978 |
The biosynthesis of haem in congenital (erythropoietic) porphyria.
Topics: 5-Aminolevulinate Synthetase; Adult; Child; Child, Preschool; Coproporphyrinogens; Erythrocytes; Ery | 1978 |
Unusual Watson-Schwartz test from methyldopa.
Topics: Color; Humans; Methyldopa; Porphobilinogen; Porphyrias | 1977 |
[Diagnosis of porphyria].
Topics: Humans; Methods; Porphobilinogen; Porphyrias; Porphyrins | 1977 |
Pathogenesis of neural manifestations in acute porphyria.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Aminolevulinic Acid; Animals; Anura; Brain; Heme; Human | 1977 |
Comparison of the Hoesch and the Watson-Schwartz tests for urinary porphobilinogen.
Topics: Chromatography, Thin Layer; Colorimetry; Humans; Methyldopa; Microchemistry; Porphobilinogen; Porphy | 1977 |
Prophyrin precursors in blood, urine and cerebrospinal fluid in acute porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Female; Humans; Middle Aged; Porphobilinogen; Porphyrias; | 1977 |
Catecholamine uptake, accumulation, and release in acute porphyria.
Topics: Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Animals; Blood Platelets; Catecholamines; Ery | 1977 |
[Pathophysiology and diagnosis of acute intermittent porphyria].
Topics: Acute Disease; Aminolevulinic Acid; Erythrocytes; Humans; Hydroxymethylbilane Synthase; Porphobilino | 1977 |
Screening tests in acute porphyria.
Topics: Acute Disease; Humans; Methods; Porphobilinogen; Porphyrias | 1977 |
[Letter: Porphyria cutanea tarda: normalization of porphyrin-excretion following treatment with p-amino-benzoic acid (Potaba) (author's transl)].
Topics: Adult; Aminobenzoates; Aminolevulinic Acid; Female; Humans; Middle Aged; Porphobilinogen; Porphyrias | 1976 |
Hematin treatment of acute porphyria. Early remission of an almost fatal relapse.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Female; Follow-Up Studies; Heme; Hemin; Humans; Infusions | 1976 |
Acute intermittent porphyria with relapsing acute pancreatitis and unconjugated hyperbilirubinemia without overt hemolysis.
Topics: Acute Disease; Adult; Female; Hemolysis; Humans; Hyperbilirubinemia; Pancreatitis; Porphobilinogen; | 1976 |
Porphyrin biosynthesis from ALA and PBG by human erythrocytes in porphyrin disorders. Kinetic studies of the isomer series I and III.
Topics: Aminolevulinic Acid; Coproporphyrins; Erythrocytes; Hot Temperature; Humans; Isomerism; Kinetics; Le | 1976 |
Porphobilinogen excretion in chemical induced porphyria: reversal by induction of porphobilinogen oxygenase.
Topics: Acetamides; Allylisopropylacetamide; Aminolevulinic Acid; Animals; Enzyme Induction; Fasting; Liver; | 1975 |
Exacerbation of hereditary hepatic porphyria by surreptitious ingestion of an unusual provocative agent--a mouthwash preparation.
Topics: 5-Aminolevulinate Synthetase; Enzyme Induction; Epoxy Compounds; Female; Humans; Liver; Middle Aged; | 1975 |
Porphyrin synthesis and mitochondrial respiration in acute intermittent porphyria: studies using cultured human fibroblasts.
Topics: 5-Aminolevulinate Synthetase; Adult; Cells, Cultured; Female; Fibroblasts; Humans; Male; Middle Aged | 1975 |
Effects of hematin in hepatic porphyria. Further studies.
Topics: Adult; Aminolevulinic Acid; Female; Heme; Hemin; Humans; Infusions, Parenteral; Liver; Male; Middle | 1975 |
Letter: Variegate porphyria.
Topics: Abdomen; Adult; Cholestyramine Resin; Coproporphyrins; Feces; Female; Humans; Pain; Plasmapheresis; | 1975 |
Diminished erythroid ferrochelatase activity in protoporphyria.
Topics: 5-Aminolevulinate Synthetase; Adult; Bone Marrow; Bone Marrow Cells; Chromatography; Erythroblasts; | 1975 |
Editorial: Hematin and porphyria.
Topics: 5-Aminolevulinate Synthetase; Adult; Animals; Female; Heme; Hemin; Humans; Kidney Diseases; Male; Ne | 1975 |
Anesthesia and the porphyrias.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Aminolevulinic Acid; Ammonia-Lyases; Anesthesia; Anesth | 1975 |
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 41-1975.
Topics: 3-Oxo-5-alpha-Steroid 4-Dehydrogenase; 5-Aminolevulinate Synthetase; Demyelinating Diseases; Diagnos | 1975 |
[Acute intermittent porphyria. Detection of asymptomatic carriers of the genetic defect].
Topics: Acute Disease; Adolescent; Adult; Aged; Aminolevulinic Acid; Ammonia-Lyases; Child; Child, Preschool | 1975 |
Studies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: prenatal diagnosis of the porphyric trait.
Topics: Abortion, Induced; Adolescent; Adult; Aminolevulinic Acid; Ammonia-Lyases; Amniocentesis; Amniotic F | 1975 |
Letter: A flase-positive test for bile.
Topics: Acute Disease; Bile; False Positive Reactions; Humans; Porphobilinogen; Porphyrias | 1975 |
[The etiopathogenesis and clinical classification of congenital and acquired porphyrias].
Topics: Animals; Heme; Humans; Porphobilinogen; Porphyrias; Porphyrinogens; Porphyrins | 1975 |
Urinary excretion of 17-oxosteroids in hereditary coproporphyria.
Topics: 17-Ketosteroids; Adult; Aminolevulinic Acid; Androsterone; Coproporphyrins; Etiocholanolone; Female; | 1975 |
Thiopental administration in acute intermittent porphyria without adverse effect.
Topics: Acute Disease; Adult; Anesthesia, Intravenous; Female; Humans; Porphobilinogen; Porphyrias; Thiopent | 1976 |
Studies in porphyria. V. Drug oxidation rates in hereditary hepatic porphyria.
Topics: Adult; Aminolevulinic Acid; Antipyrine; Erythrocytes; Female; Half-Life; Humans; Hydroxymethylbilane | 1976 |
[Diagnostic and therapeutic problems in acute intermittent porphyria].
Topics: Acute Disease; Adrenal Cortex Hormones; Adult; Aminolevulinic Acid; Analgesics; Colic; Color; Diagno | 1976 |
Comparative effects of glycerol and dextrose on porphyrin precursor excretion in acute intermittent porphyria.
Topics: Acute Disease; Adolescent; Adult; Aged; Aminolevulinic Acid; Dietary Carbohydrates; Dietary Fats; Fe | 1976 |
[Accident in treatment of porphyria cutanea tarda by chloroquine (Resochin) (author's transl)].
Topics: Aminolevulinic Acid; Chloroquine; Humans; Liver; Male; Medication Errors; Middle Aged; Mitochondria, | 1976 |
[Acute intermittent porphyria in pregnancy: glucose or hematin therapy?].
Topics: Acute Disease; Adult; Aminolevulinic Acid; Drug Therapy, Combination; Female; Glucose Solution, Hype | 1992 |
[What is your diagnosis? Acute intermittent porphyria].
Topics: Adult; Aminolevulinic Acid; Female; Humans; Porphobilinogen; Porphyrias; Porphyrins; Postoperative C | 1992 |
Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases.
Topics: Acute Disease; Age Factors; Cause of Death; Finland; Humans; Life Tables; Porphobilinogen; Porphyria | 1992 |
Alcoholic beverages in acute porphyria.
Topics: Acute Disease; Alcoholic Beverages; Aminolevulinic Acid; Dose-Response Relationship, Drug; Genetic C | 1992 |
Acute intermittent porphyria. A perplexing case.
Topics: Acute Disease; Aminolevulinic Acid; Female; Humans; Middle Aged; Nervous System Diseases; Pain; Porp | 1992 |
Influence of hepatic tumors caused by diethylnitrosamine on hexachlorobenzene-induced porphyria in rats.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Carcinoma, Hepatocellular; Cytochrome P- | 1991 |
Intravenous heme-albumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Analysis of Variance; Cytochrome P-450 Enzyme System; Fem | 1991 |
Effect of ursodeoxycholic acid on experimental hepatic porphyria induced by griseofulvin.
Topics: Alanine Transaminase; Alkaline Phosphatase; Animals; Bilirubin; Chemical and Drug Induced Liver Inju | 1991 |
Manifestation of acute intermittent porphyria in patients with chronic inflammatory bowel disease.
Topics: Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Colitis, Ulcerative; Crohn Disease; Humans; P | 1991 |
Experimental latent and acute porphyria in the non-fasted rat; preventive effect of propranolol.
Topics: Acute Disease; Allylisopropylacetamide; Aminolevulinic Acid; Animals; Coproporphyrins; Dicarbethoxyd | 1991 |
Reliability of the porphobilinogen screening assay.
Topics: Clinical Laboratory Techniques; Humans; Porphobilinogen; Porphyrias; Sensitivity and Specificity | 1990 |
Elevation of hormone-binding globulins in acute intermittent porphyria.
Topics: Adolescent; Adult; Aminolevulinic Acid; Arginine; Carrier Proteins; Heme; Humans; Hydrocortisone; Li | 1990 |
A gonadotropin releasing hormone analogue prevents cyclical attacks of porphyria.
Topics: Adult; Aminolevulinic Acid; Female; Gonadotropin-Releasing Hormone; Humans; Menstrual Cycle; Periodi | 1990 |
Safety of vancomycin in acute porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Erythrocytes; Female; Humans; Hydroxymethylbilane Synthas | 1990 |
[Evaluation of porphyrinogenic effect of lindane in rats].
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Animals; Heme; Hexachlorocyclohexane; Levulinic A | 1986 |
Acute intermittent porphyria in two patients on anticonvulsant therapy and with normal erythrocyte porphobilinogen deaminase activity.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Aminolevulinic Acid; Ammonia-Lyases; Anticonvulsants; Er | 1989 |
Hepatobiliary implications and complications in protoporphyria, a 20-year study.
Topics: Adult; Aminolevulinic Acid; Child, Preschool; Chromatography, Ion Exchange; Chromatography, Thin Lay | 1989 |
Porphyria in a young man.
Topics: Diagnosis, Differential; Emergencies; Humans; Male; Porphobilinogen; Porphyrias | 1989 |
Screening tests for porphobilinogen are insensitive. The problem and its solution.
Topics: Benzaldehydes; Chromatography, Ion Exchange; False Negative Reactions; Humans; Indicators and Reagen | 1989 |
Latent acute hepatic porphyria.
Topics: Acute Disease; Heterozygote; Humans; Liver Diseases; Porphobilinogen; Porphyrias | 1985 |
Liquid-chromatographic assay of urinary porphobilinogen.
Topics: Chromatography, Ion Exchange; Chromatography, Liquid; Female; Humans; Porphobilinogen; Porphyrias; Q | 1989 |
Endogenously formed norharman (beta-carboline) in platelet rich plasma obtained from porphyric rats.
Topics: Alkaloids; Allylisopropylacetamide; Aminolevulinic Acid; Animals; Blood Platelets; Carbolines; Catal | 1986 |
Determination of erythrocyte hydroxymethylbilane synthase activity and its application for study of acute intermittent porphyria.
Topics: Acute Disease; Adult; Aged; Ammonia-Lyases; Clinical Enzyme Tests; Erythrocytes; Female; Humans; Hyd | 1988 |
[Evaluation of deferoxamine capacity to revert a severe porphyria induced by hexachlorobenzene].
Topics: 5-Aminolevulinate Synthetase; Animals; Carboxy-Lyases; Deferoxamine; Hexachlorobenzene; Liver; Porph | 1987 |
Treatment of acute hepatic porphyria with hematin.
Topics: Acute Disease; Adult; Aged; Aminolevulinic Acid; Female; Heme; Hemin; Humans; Liver Diseases; Male; | 1988 |
Detection of non-typical porphyrin isomers in human urines by ion-pair reversed-phase high-performance liquid chromatography.
Topics: Chromatography, High Pressure Liquid; Coproporphyrins; Decarboxylation; Humans; Isomerism; Porphobil | 1988 |
[Acute intermittent porphyria. A problem of differential diagnosis].
Topics: Adult; Cerebral Hemorrhage; Diagnosis, Differential; Humans; Male; Mental Disorders; Porphobilinogen | 1987 |
Polychemotherapy of acute myelogenous leukemia in a patient with acute intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Antineoplastic Combined Chemotherapy Protocols; Chemical | 1987 |
The porphyrias.
Topics: Adolescent; Adult; Aminolevulinic Acid; beta Carotene; Bloodletting; Carotenoids; Child; Child, Pres | 1986 |
Aminolaevulinate dehydratase porphyria in infancy. A clinical and biochemical study.
Topics: Aminolevulinic Acid; Erythrocytes; Female; Humans; Infant, Newborn; Kinetics; Male; Pedigree; Porpho | 1987 |
Antipyrine metabolism in acute hepatic porphyria in relapse and remission.
Topics: 5-Aminolevulinate Synthetase; Adolescent; Adult; Aged; Aminolevulinic Acid; Antipyrine; Cytochrome P | 1987 |
Acute intermittent porphyria: the first case report in Malaysia.
Topics: Acute Disease; Adult; Female; Heme; Humans; Malaysia; Porphobilinogen; Porphyrias | 1986 |
Washington University case conference. Dual porphyria--an underdiagnosed entity?
Topics: Aminolevulinic Acid; Coproporphyrins; Diagnosis, Differential; Feces; Flavoproteins; Humans; Male; M | 1987 |
[Treatment of acute intermittent porphyria with a new protein-bound lyophilized hematin].
Topics: Acute Disease; Adult; Female; Freeze Drying; Heme; Hemin; Humans; Porphobilinogen; Porphyrias; Prote | 1987 |
The effects of haem arginate and haematin upon the allylisopropylacetamide induced experimental porphyria in rats.
Topics: 5-Aminolevulinate Synthetase; Allylisopropylacetamide; Animals; Arginine; Heme; Hemin; Liver; Male; | 1987 |
[Diagnosis of acute porphyria].
Topics: Acute Disease; Humans; Porphobilinogen; Porphyrias; Porphyrins | 1986 |
[Diagnosis of disturbances of porphyrin metabolism--an examination of laboratory resources in the country].
Topics: Clinical Laboratory Techniques; Erythrocytes; Humans; Hydroxymethylbilane Synthase; Porphobilinogen; | 1986 |
[Acute porphyria: 2 clinical cases].
Topics: Acute Disease; Adult; Aminolevulinic Acid; Female; Humans; Liver; Porphobilinogen; Porphyrias; Porph | 1985 |
Influence of chloroquine on the porphyrin metabolism.
Topics: 7-Alkoxycoumarin O-Dealkylase; Aminolevulinic Acid; Aminopyrine N-Demethylase; Animals; Aryl Hydroca | 1985 |
Use of transcutaneous nerve stimulation in the attacks of acute intermittent porphyria.
Topics: Acute Disease; Adult; Aminolevulinic Acid; Electric Stimulation Therapy; Female; Humans; Porphobilin | 1985 |
Hyperinsulinemia in acute intermittent porphyria.
Topics: Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Female; Glucose Tolerance Test; Humans; Hyper | 1985 |
[Porphyrins and their precursors in the urine of patients with suspected porphyria; a 16-year experience].
Topics: Adult; Aminolevulinic Acid; Female; Humans; Levulinic Acids; Male; Middle Aged; Porphobilinogen; Por | 1985 |
"Glucose effect" and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture: relationship with human acute hepatic porphyrias.
Topics: 5-Aminolevulinate Synthetase; Aminolevulinic Acid; Ammonia-Lyases; Animals; Cells, Cultured; Chick E | 1985 |
Sn-protoporphyrin suppresses chemically induced experimental hepatic porphyria. Potential clinical implications.
Topics: 5-Aminolevulinate Synthetase; Allylisopropylacetamide; Animals; Chemical and Drug Induced Liver Inju | 1985 |
Enzymes in the hepatic porphyrias.
Topics: 5-Aminolevulinate Synthetase; Erythrocytes; Humans; Liver; Liver Diseases; Porphobilinogen; Porphyri | 1972 |
Demonstration of tissue porphyrins in hexachlorobenzene model experiment by the fluorescence technique.
Topics: Animals; Blood Proteins; Cytoplasm; Fluorescence; Hexachlorocyclohexane; Kidney Cortex; Levulinic Ac | 1973 |
[Experimental porphyria model with hexachlorocyclohexane].
Topics: Aminolevulinic Acid; Animals; Chlorobenzenes; Diet; Disease Models, Animal; Feces; Female; Hexachlor | 1974 |
Experimental hepatic porphyria induced by polychlorinated biphenyls.
Topics: 5-Aminolevulinate Synthetase; Animals; Body Weight; Chromatography, Thin Layer; Cytochrome P-450 Enz | 1974 |
[Acute intermittent porphyria associated with Biermer's anemia. Value of the glycocoll test].
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adult; Anemia, Pernicious; Feces; Female; Genes, Domina | 1972 |
Enzyme aspects of acute intermittent porphyria.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Ammonia-Lyases; Binding Sites; Carboxy-Lyases; Cyclic A | 1973 |
The reversal of experimental porphyria and the prevention of induction of hepatic mixed-function oxidase by acetate.
Topics: 5-Aminolevulinate Synthetase; Acetates; Aminolevulinic Acid; Animals; Cytochrome c Group; Cytochrome | 1974 |
NADH 2 generation in drug-induced porphyric rabbits.
Topics: Acetamides; Animals; Carbon Isotopes; Liver; Male; NAD; NADP; Oxidation-Reduction; Porphobilinogen; | 1971 |
[Genetic prognosis of a mother with acute intermittent porphyria (hepatic form)].
Topics: Acute Disease; Adolescent; Adult; Aminolevulinic Acid; Female; Genes, Dominant; Genetic Counseling; | 1974 |
The Hoesch test: bedside screening for urinary porphobilinogen in patients with suspected porphyria.
Topics: Diagnosis, Differential; Evaluation Studies as Topic; Humans; Indicators and Reagents; Methods; Micr | 1974 |
A red cell enzyme method for the diagnosis of acute intermittent porphyria.
Topics: Acute Disease; Aminolevulinic Acid; Ammonia-Lyases; Erythrocytes; Freezing; Hemolysis; Hot Temperatu | 1974 |
Lead poisoning with low blood lead levels.
Topics: Adult; Aminolevulinic Acid; Hemoglobins; Humans; Lead; Lead Poisoning; Male; Occupational Diseases; | 1974 |
[Determination of uroporphyrinogen 1 synthetase in porphyria].
Topics: Adult; Ammonia-Lyases; Benzaldehydes; Clinical Enzyme Tests; Dimethylamines; Erythrocytes; Female; H | 1974 |
Chorio-carcinoma associated with porphobilinogenuria. A case report.
Topics: Adult; Choriocarcinoma; Female; Humans; Liver Neoplasms; Neoplasm Metastasis; Porphobilinogen; Porph | 1974 |
Acute intermittent porphyria with inappropriately elevated ADH secretion.
Topics: Acute Disease; Adult; Ataxia; Barbiturates; Consciousness Disorders; Craniocerebral Trauma; Electroe | 1974 |
A microassay for uroporphyrinogen I synthase, one of three abnormal enzyme activities in acute intermittent porphyria, and its application to the study of the genetics of this disease.
Topics: Adolescent; Adult; Age Factors; Aged; Ammonia-Lyases; Child; Child, Preschool; Erythrocytes; Female; | 1974 |
Comparative aspects of porphyria in man and animals.
Topics: Adult; Aminolevulinic Acid; Animals; Cattle; Drug Stability; Female; Genes, Recessive; Heme; Heteroz | 1974 |
Hepatic porphyrins and urinary porphyrins and porphyrin precursors in liver cirrhosis.
Topics: Adult; Aged; Biopsy, Needle; Chromatography, Thin Layer; Chronic Disease; Female; Fluorescence; Huma | 1972 |
Quantitative studies of the delivery of hepatic-synthesized bilirubin to plasma utilizing -aminolevulinic acid-4- 14 C and bilirubin- 3 H in man.
Topics: Adult; Bile Pigments; Bilirubin; Carbon Isotopes; Chromium Isotopes; Feces; Hematocrit; Heme; Humans | 1972 |
The role of iron in the pathogenesis of porphyria cutanea tarda. An in vitro model.
Topics: Ammonia-Lyases; Animals; Chromatography, Paper; Cysteine; Heating; Humans; In Vitro Techniques; Iron | 1972 |
Effect of hexachlorobenzene on haem synthesis.
Topics: 5-Aminolevulinate Synthetase; Amino Acids; Animals; Benzene Derivatives; Cells, Cultured; Chlorine; | 1972 |
Structure and synthesis of heme and references to globin synthesis.
Topics: 5-Aminolevulinate Synthetase; Globins; Heme; Iron; Levulinic Acids; Metabolism, Inborn Errors; Pepti | 1972 |
[Clinically important disorders of heme synthesis].
Topics: 5-Aminolevulinate Synthetase; Anemia; Anemia, Hypochromic; Anemia, Sideroblastic; Erythrocytes; Heme | 1972 |
[Two screening tests for porphyrias (author's transl)].
Topics: Chromatography, Ion Exchange; Humans; Mass Screening; Porphobilinogen; Porphyrias; Porphyrins | 1972 |
Erythropoietic porphyria of the fox squirrel Sciurus niger.
Topics: Animals; Bone Marrow; Bone Marrow Diseases; Disease Models, Animal; Erythrocytes; Erythropoiesis; He | 1973 |
Hereditary hepatic porphyrias.
Topics: 5-Aminolevulinate Synthetase; Ammonia-Lyases; Animals; Cytochrome P-450 Enzyme System; Erythrocytes; | 1973 |
Effect of hematin in acute porphyric relapse.
Topics: 5-Aminolevulinate Synthetase; Adult; Alcohol Drinking; Heme; Humans; Injections, Intravenous; Male; | 1973 |
[Analysis of clinical manifestations in acute intermittent porphyria].
Topics: Acute Disease; Adult; Blood Pressure; Diuresis; Female; Heart Rate; Humans; Levulinic Acids; Male; P | 1973 |
[Disorders of porphyrin metabolism].
Topics: Chronic Disease; Humans; Levulinic Acids; Liver; Metabolism, Inborn Errors; Porphobilinogen; Porphyr | 1973 |
Erythropoietic protoporphyria. The first case in Finland.
Topics: Adult; Carotenoids; Erythrocytes; Erythropoiesis; Feces; Female; Finland; Fluorescence; Humans; Micr | 1973 |
The effect of DL-propranolol on delta-aminolevulinic acid synthetase activity and urinary excretion of porphyrins in allylisopropylacetamide-induced experimental porphyria.
Topics: 5-Aminolevulinate Synthetase; Allylisopropylacetamide; Animals; Chromatography, Ion Exchange; Enzyme | 1973 |
Porphyria variegata. Report of two cases with brief review of literature.
Topics: Child; Feces; Humans; Male; Porphobilinogen; Porphyrias; Porphyrins; Remission, Spontaneous | 1973 |
Plasma porphyrin concentrations in porphyria cutanea tarda.
Topics: Adult; Aged; Erythrocytes; Feces; Female; Humans; Levulinic Acids; Male; Middle Aged; Porphobilinoge | 1973 |
[Atypical courses of porphyria cutanea tarda].
Topics: Diagnosis, Differential; Electromyography; Female; Follow-Up Studies; Humans; Keratosis; Levulinic A | 1973 |
The role of substrates for glycine acyltransferase, and the role of sulfanilamide and acetate in the reversal of chemically-induced porphyria in the rat.
Topics: 5-Aminolevulinate Synthetase; Acetates; Acyltransferases; Animals; Chromatography, Ion Exchange; Dic | 1973 |
Drug interactions in experimental hepatic porphyria. A model for the exacerbation by drugs of human variegate porphyria.
Topics: 5-Aminolevulinate Synthetase; Administration, Oral; Allylisopropylacetamide; Animals; Chickens; Coba | 1973 |
17-Oxosteroid control of porphyrin biosynthesis.
Topics: 17-Ketosteroids; 5-Aminolevulinate Synthetase; Acute Disease; Adult; Animals; Chromatography, Gas; C | 1973 |
Studies on the inheritance of human erythrocyte delta-aminolevulinate dehydratase and uroporphyrinogen synthetase.
Topics: Acute Disease; Adult; Ammonia-Lyases; Child; Child, Preschool; Diseases in Twins; Erythrocytes; Fema | 1973 |
Metabolism of delta-aminolaevulinic acid and porphobilinogen in human erythrocytes in acute intermittent porphyria.
Topics: Acute Disease; Adult; Chromatography, Thin Layer; Erythrocytes; Female; Humans; Levulinic Acids; Mal | 1973 |
[Comments on paper "Zwei Suchteste für Porphyrein". (Doss, M. and Schmidt, A., (1972) this j. 10 (230-231)].
Topics: Acetates; Aminolevulinic Acid; Chloroform; Coloring Agents; Humans; Indicators and Reagents; Methods | 1973 |
17-Oxosteroid conjugates in plasma and urine of patients with acute intermittent porphyria.
Topics: 17-Ketosteroids; 5-Aminolevulinate Synthetase; Adult; Androgens; Androsterone; Animals; Chromatograp | 1974 |
Porphyria cutanea tarda in a 2-year-old girl.
Topics: Alanine Transaminase; Aspartate Aminotransferases; Binding Sites; Blood Sedimentation; Child, Presch | 1974 |
Acute intermittent porphyria in childhood. A neglected diagnosis?
Topics: Abdomen; Acute Disease; Child; Color; Humans; Hypertension; Hyponatremia; Male; Pain; Porphobilinoge | 1974 |
Urinary dipyrrylmethenes in patients with porphyria. A preliminary study of the brown pigments in the urine of a case of acute intermittent porphyria.
Topics: Acute Disease; Adult; Bilirubin; Chromatography, Thin Layer; Humans; Male; Pigments, Biological; Por | 1974 |
Sub-uroporphyrins and their occurrence. A preliminary communication.
Topics: Animals; Bone and Bones; Cattle; Chromatography, Thin Layer; Cricetinae; Egg Shell; Esters; Feathers | 1974 |
Symptomatic porphyria in a case of Felty's syndrome. I. Clinical and routine biochemcial studies.
Topics: 5-Aminolevulinate Synthetase; Agranulocytosis; Feces; Felty Syndrome; Female; Humans; Infections; Le | 1972 |
Symptomatic porphyria in a case of Felty's syndrome. II. Biochemical investigations.
Topics: 5-Aminolevulinate Synthetase; Chromatography, Thin Layer; Cyclohexanes; Electrophoresis; Ethyl Ether | 1972 |
A new possibility for the demonstration of -amino-laevulinic acid in urine on the basis of Mauzerall-Granick method.
Topics: Acute Disease; Aldehydes; Animals; Benzene Derivatives; Charcoal; Humans; Levulinic Acids; Methods; | 1972 |
Treatment of an acute attack of porphyria variegata with propranolol.
Topics: Abdomen, Acute; Acute Disease; Adult; Feces; Female; Follow-Up Studies; Humans; Mental Disorders; Ne | 1972 |
Intermittent acute porphyria--demonstration of a genetic defect in porphobilinogen metabolism.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Adolescent; Adult; Erythrocytes; Feces; Female; Humans; | 1972 |
Evaluation of two methods for measuring delta-aminolaevulinic acid in urine. II. The influence of some Ehrlich-positive' substances on the measurements.
Topics: Adolescent; Adult; Aminosalicylic Acids; Bacteriuria; Child; Evaluation Studies as Topic; Female; Hu | 1972 |
[Therapy of porphyria cutanea tarda through metabolic alkalization with uralyt-U].
Topics: Adult; Aged; Citrates; Female; Humans; Male; Middle Aged; Porphobilinogen; Porphyrias; Porphyrins; P | 1972 |
Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria.
Topics: Acute Disease; Chromatography, DEAE-Cellulose; Chromatography, Gel; Chromatography, Thin Layer; Eryt | 1972 |
Porphyria in childhood following transient neonatal quadriplegia.
Topics: Child; Cholesterol; Chromatography, Paper; Humans; Hyperkinesis; Infant, Newborn; Infant, Newborn, D | 1972 |
A survey of porphyria among psychiatric patients.
Topics: Acute Disease; Australia; Female; Fluorescence; Humans; Male; Mental Disorders; Porphobilinogen; Por | 1972 |
Acute intermittent porphyria: studies of the enzymatic basis of disordered haem biosynthesis.
Topics: 5-Aminolevulinate Synthetase; Acute Disease; Acyltransferases; Animals; Birds; Heme; Humans; In Vitr | 1971 |
Clinical studies of 63 cases of various porphyrias in Barcelona over the 2-year-period 1968-1970.
Topics: Feces; Humans; Porphobilinogen; Porphyrias; Porphyrins; Spain; Sunlight | 1971 |
Acute intermittent porphyria in Poland.
Topics: Acute Disease; Adenosine Monophosphate; Adolescent; Adult; Aged; Amino Acids; Child; Child, Preschoo | 1971 |
Simple and rapid screening for acute porphyria--'porphobilistix' and Hoesch test.
Topics: Acute Disease; Aldehydes; Benzene; Humans; Hydrochloric Acid; Mass Screening; Methods; Porphobilinog | 1971 |
Porphyria. Case presentations. Case 1: S.V.T., white female, 26 years.
Topics: Adult; Amino Acids; Bilirubin; Feces; Female; Humans; Levulinic Acids; Photosensitivity Disorders; P | 1971 |
Porphyria. Case presentations. Case 3: M.A., non-white female, 26 years.
Topics: Adult; Amino Acids; Bone Marrow Diseases; Erythropoiesis; Feces; Female; Humans; Levulinic Acids; Ph | 1971 |
The porphyrin excretion pattern of symptomatic porphyria.
Topics: Chemical Phenomena; Chemistry; Chromatography, Thin Layer; Feces; Female; Humans; Liver Diseases; Ma | 1971 |
The acute porphyric attack. I. The electrolyte disorder of the acute porphyric attack and the possible role of delta-aminolaevulic acid.
Topics: Acute Disease; Adult; Amino Acids; Chlorides; Extracellular Space; Female; Humans; Hypokalemia; Hypo | 1971 |
Report on a survey for porphyrias in hospital population.
Topics: Adolescent; Adult; Feces; Humans; India; Male; Middle Aged; Porphobilinogen; Porphyrias; Porphyrins | 1970 |