ammonium hydroxide has been researched along with Syndrome in 139 studies
azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.
Syndrome: A characteristic symptom complex.
| Excerpt | Relevance | Reference |
|---|---|---|
| "Mitochondrial ornithine transporter deficiency has been called HHH syndrome, because this disorder is characterized by three biochemical abnormalities; hyperornithinemia, hyperammonemia, and homocitrullinuria, and presents with various neurological symptoms; mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia and episodic disturbance of consciousness or coma due to hyperammonemia." | 8.81 | [Molecular genetic studies of mitochondrial ornithine transporter deficiency (HHH syndrome)]. ( Kanazawa, N; Miyamoto, T; Tsujino, S, 2001) |
| "A liquid chromatography tandem mass spectrometric method is described for the analysis of homocitrulline in human urine, a key metabolite in the differential diagnosis of hyperammonemia, hyperornithinemia, homocitrullinuria (HHH) syndrome." | 7.73 | Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry. ( Al-Dirbashi, OY; Al-Hassnan, ZN; Rashed, MS, 2006) |
| "Currently we know not more than 50 patients who show an interesting combination of increased plasma ornithine concentrations, postprandial hyperammonemia, and homocitrullinuria (HHH-syndrome)." | 7.71 | Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia. ( Dehne, MG; Fuchs, M; Hempelmann, G; Mühling, J; Sablotzki, A; Spatz, J; Weiss, S, 2001) |
| "Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome presents with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia." | 7.70 | Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. ( Eto, Y; Kanazawa, N; Kira, J; Ohashi, T; Saito, T; Tsujino, S; Yamada, T, 2000) |
| "Mitochondria of fibroblasts cultured from the skin obtained at biopsy from three patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome, one of the autosomal recessive, heritable urea cycle disorders, were studied with appropriate controls ultrastructurally." | 7.69 | Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome. Ultrastructural changes of mitochondria in cultured dermal fibroblasts of three patients. ( Dewar, RA; Gatfield, DP; Gordon, BA; Haust, MD, 1996) |
| "Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH)-syndrome is a rare autosomal recessive disorder of the urea cycle, probably caused by a defect in ornithine transport across the hepatic inner mitochondrial membrane." | 7.69 | Ciliated cultured dermal fibroblasts in a patient with hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome. ( Haust, MD, 1995) |
| " They were recently diagnosed with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome." | 7.68 | Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. ( Knopman, DS; Shih, VE; Tuchman, M, 1990) |
| "The HHH syndrome (hyperornithinemia associated with hyperammonemia and homocitrullinuria) is characterized by a very rare genetic defect of ornithine transport in mitochondrial membrane." | 7.68 | Abnormal urinary excretion of polyamines in HHH syndrome (hyperornithinemia associated with hyperammonemia and homocitrullinuria). ( Eto, Y; Maekawa, K; Shimizu, H, 1990) |
| "A 16-year-old boy with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome was reported." | 7.68 | [A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs]. ( Goto, I; Kobayashi, T; Shigeto, H; Yamada, T, 1992) |
| "We measured L-ornithine oxidation in cultured skin fibroblasts from seven patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria (HHH) syndrome (McKusick 23897), and compared it with oxidation by ornithine aminotransferase deficient gyrate atrophy (McKusick 25887) cells and lysinuric protein intolerance (McKusick 22270) cells in which there is an ornithine transport abnormality at the plasma membrane." | 7.67 | Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts. ( Botschner, J; Scriver, CR; Simell, O; Smith, DW, 1989) |
| "The primary defect in patients presenting with a history of protein intolerance, mental retardation, and epilepsy of variable degree, with the unique triad of hyperornithinemia, hyperammonemia, and homocitrullinuria (the HHH syndrome) has been postulated to be a defect in translocation of ornithine into the mitochondria." | 7.67 | The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements. ( Gatfield, DP; Gordon, BA; Haust, MD, 1987) |
| "A patient with the hyperornithinemia, hyperammonemia, homocitrullinuria syndrome is described." | 7.67 | Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria). ( Dyken, PR; Hartlage, PL; Hommes, FA; Metoki, K; Roesel, RA, 1986) |
| "Impairment of urea cycle function in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome is presumably caused, in some patients, by deficient transport of ornithine from cytoplasm into mitochondria." | 7.67 | Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. ( Clow, CL; Mackenzie, S; Scriver, CR; Simell, O, 1985) |
| "A protein load with an ensuing 20 hr fast was performed to assess the function of biotin dependent carboxylases in 3 girls with Rett syndrome." | 7.67 | Biotin and Rett syndrome. ( Bachmann, C; Colombo, JP; da Silva, V; Gugler, E; Kilian, W; Rett, A, 1986) |
| "Two patients with neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome were studied at 4 and 2 1/2 yr of age, respectively." | 7.67 | Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement. ( Bachmann, C; Colombo, JP; Dionisi Vici, C; Gambarara, M; Sabetta, G, 1987) |
| "Mitochondrial ornithine transporter deficiency has been called HHH syndrome, because this disorder is characterized by three biochemical abnormalities; hyperornithinemia, hyperammonemia, and homocitrullinuria, and presents with various neurological symptoms; mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia and episodic disturbance of consciousness or coma due to hyperammonemia." | 4.81 | [Molecular genetic studies of mitochondrial ornithine transporter deficiency (HHH syndrome)]. ( Kanazawa, N; Miyamoto, T; Tsujino, S, 2001) |
| "A liquid chromatography tandem mass spectrometric method is described for the analysis of homocitrulline in human urine, a key metabolite in the differential diagnosis of hyperammonemia, hyperornithinemia, homocitrullinuria (HHH) syndrome." | 3.73 | Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry. ( Al-Dirbashi, OY; Al-Hassnan, ZN; Rashed, MS, 2006) |
| "Currently we know not more than 50 patients who show an interesting combination of increased plasma ornithine concentrations, postprandial hyperammonemia, and homocitrullinuria (HHH-syndrome)." | 3.71 | Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia. ( Dehne, MG; Fuchs, M; Hempelmann, G; Mühling, J; Sablotzki, A; Spatz, J; Weiss, S, 2001) |
| "Mitochondrial ornithine transporter deficiency, or HHH syndrome, is a metabolic disorder resulting in various neurologic symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia." | 3.71 | A novel mutation, P126R, in a Japanese patient with HHH syndrome. ( Hayakawa, C; Kanazawa, N; Miyamoto, T; Tsujino, S, 2002) |
| "Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome presents with various neurological symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia." | 3.70 | Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. ( Eto, Y; Kanazawa, N; Kira, J; Ohashi, T; Saito, T; Tsujino, S; Yamada, T, 2000) |
| " We hypothesized that this syndrome of hyperinsulinism and hyperammonemia was caused by excessive activity of glutamate dehydrogenase, which oxidizes glutamate to alpha-ketoglutarate and which is a potential regulator of insulin secretion in pancreatic beta cells and of ureagenesis in the liver." | 3.70 | Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. ( Burlina, AB; Greenberg, CR; Hopwood, NJ; Hsu, BY; Lieu, YK; Perlman, K; Poncz, M; Rich, BH; Stanley, CA; Zammarchi, E, 1998) |
| "Prenatal diagnosis of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria syndrome is described by the analysis of ornithine incorporation in second-trimester cultured amniotic fluid cells." | 3.69 | Prenatal exclusion of the HHH syndrome. ( Gray, RG; Green, A; Hall, S; McKeown, C, 1995) |
| "Mitochondria of fibroblasts cultured from the skin obtained at biopsy from three patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome, one of the autosomal recessive, heritable urea cycle disorders, were studied with appropriate controls ultrastructurally." | 3.69 | Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome. Ultrastructural changes of mitochondria in cultured dermal fibroblasts of three patients. ( Dewar, RA; Gatfield, DP; Gordon, BA; Haust, MD, 1996) |
| "Hyperornithinemia, hyperammonemia and homocitrullinuria (HHH)-syndrome is a rare autosomal recessive disorder of the urea cycle, probably caused by a defect in ornithine transport across the hepatic inner mitochondrial membrane." | 3.69 | Ciliated cultured dermal fibroblasts in a patient with hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome. ( Haust, MD, 1995) |
| " They were recently diagnosed with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome." | 3.68 | Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. ( Knopman, DS; Shih, VE; Tuchman, M, 1990) |
| "A 16-year-old boy with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome was reported." | 3.68 | [A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs]. ( Goto, I; Kobayashi, T; Shigeto, H; Yamada, T, 1992) |
| "The HHH syndrome (hyperornithinemia associated with hyperammonemia and homocitrullinuria) is characterized by a very rare genetic defect of ornithine transport in mitochondrial membrane." | 3.68 | Abnormal urinary excretion of polyamines in HHH syndrome (hyperornithinemia associated with hyperammonemia and homocitrullinuria). ( Eto, Y; Maekawa, K; Shimizu, H, 1990) |
| "The primary defect in patients presenting with a history of protein intolerance, mental retardation, and epilepsy of variable degree, with the unique triad of hyperornithinemia, hyperammonemia, and homocitrullinuria (the HHH syndrome) has been postulated to be a defect in translocation of ornithine into the mitochondria." | 3.67 | The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements. ( Gatfield, DP; Gordon, BA; Haust, MD, 1987) |
| "Gamma-glutamylornithine has been identified in urine from patients with the HHH syndrome (hyperornithinemia, hyperammonemia and homocitrullinuria) and with gyrate atrophy associated with hyperornithinemia." | 3.67 | Gamma-glutamylornithine excretion in patients with hyperornithinemia. ( Blankenship, PR; Coryell, ME; Hommes, FA; Roesel, RA, 1984) |
| "Out of six patients with hyperkaliemic periodic paralysis observed by the authors, two reported the sensation of the odor of ammonium hydroxide at the moment of paralysis." | 3.67 | [The olfactory phenomenon in hyperkalemic periodic paralysis]. ( Aver'ianov, IuN, 1984) |
| "A protein load with an ensuing 20 hr fast was performed to assess the function of biotin dependent carboxylases in 3 girls with Rett syndrome." | 3.67 | Biotin and Rett syndrome. ( Bachmann, C; Colombo, JP; da Silva, V; Gugler, E; Kilian, W; Rett, A, 1986) |
| "We measured L-ornithine oxidation in cultured skin fibroblasts from seven patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria (HHH) syndrome (McKusick 23897), and compared it with oxidation by ornithine aminotransferase deficient gyrate atrophy (McKusick 25887) cells and lysinuric protein intolerance (McKusick 22270) cells in which there is an ornithine transport abnormality at the plasma membrane." | 3.67 | Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts. ( Botschner, J; Scriver, CR; Simell, O; Smith, DW, 1989) |
| "Two patients with neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome were studied at 4 and 2 1/2 yr of age, respectively." | 3.67 | Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement. ( Bachmann, C; Colombo, JP; Dionisi Vici, C; Gambarara, M; Sabetta, G, 1987) |
| "A patient with the hyperornithinemia, hyperammonemia, homocitrullinuria syndrome is described." | 3.67 | Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria). ( Dyken, PR; Hartlage, PL; Hommes, FA; Metoki, K; Roesel, RA, 1986) |
| "To study the potential contribution of glycine toxicity in the transurethral resection syndrome, we evaluated hemodynamic and visual evoked potential responses to glycine infusion (1 g/kg) in 22 dogs anesthetized with halothane (1." | 3.67 | Effects of glycine on hemodynamic responses and visual evoked potentials in the dog. ( Clark, WM; Creel, DJ; Shahangian, S; Wang, JM; Wong, KC, 1985) |
| "Impairment of urea cycle function in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome is presumably caused, in some patients, by deficient transport of ornithine from cytoplasm into mitochondria." | 3.67 | Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. ( Clow, CL; Mackenzie, S; Scriver, CR; Simell, O, 1985) |
| "Urea cycle disorders and other hyperammonemic syndromes should be considered in the differential diagnosis in newborns with a history of severe vomiting, lethargy, and seizures, and in infants with feeding problems, episodic vomiting, and altered consciousness." | 3.65 | Congenital hyperammonemic syndromes. ( Shih, VE, 1976) |
| " Liver and lung alpha-tocopherol concentrations exhibited a dose-response increase to dietary tocopherol and there was a high correlation between lung and liver tissue alpha-tocopherol (r = 0." | 2.68 | Effect of dietary dl-alpha-tocopherol on tissue alpha- and gamma-tocopherol and pulmonary hypertension syndrome (ascites) in broilers. ( Barnes, D; Beers, KW; Bersi, TK; Bottje, WG; Erf, GF; Wang, S, 1997) |
| "The TUR syndrome is a clinical disorder consisting of circulatory, gastrointestinal, and neurologic signs that are infrequently seen in some patients undergoing endoscopic surgery of the prostate gland with the use of nonconductive irrigating fluids." | 2.39 | Temporary blindness in the TUR syndrome. ( Barletta, JP; Fanous, MM; Hamed, LM, 1994) |
| " These deal mainly with biochemical systems that are known to be affected by VPA, or with the possible idiosyncratic production of toxic VPA metabolites, especially delta 4-VPA." | 2.38 | Valproate hepatotoxicity syndrome: hypotheses of pathogenesis. ( Levy, RH; Stephens, JR, 1992) |
| "Although the syndrome is thought to be relatively common, it was only described in the English literature 5 years ago." | 2.37 | The clinical recognition and differential diagnosis of Rett syndrome. ( Naidu, S; Trevathan, E, 1988) |
| "Because Rett syndrome is a relatively homogeneous and common syndrome of idiopathic mental retardation, epidemiologic methods may be more productive in the study of Rett syndrome than in other syndromes of mental retardation that are less clinically homogeneous." | 2.37 | The epidemiology and public health significance of Rett syndrome. ( Adams, MJ; Trevathan, E, 1988) |
| "Hyperammonemia is a rare, often fatal complication after transplantation." | 1.43 | Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience. ( Bain, KB; Byers, DE; Chen, C; Hachem, RR; Iuppa, JA; Patterson, GA; Trulock, EP; Witt, CA; Yusen, RD, 2016) |
| "All patients had convulsions or loss of consciousness resulting from hypoglycemia at less than 1 year of age." | 1.31 | Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism-hyperammonemia syndrome. ( Hayashi, Y; Kato, H; Miki, Y; Ohura, T; Taki, T; Yanagisawa, M, 2000) |
| "The hyperinsulinism was successfully treated with diazoxide." | 1.31 | Functional hyperactivity of hepatic glutamate dehydrogenase as a cause of the hyperinsulinism/hyperammonemia syndrome: effect of treatment. ( de Klerk, JB; Duran, M; Huijmans, JG; Rovers, MJ; Scholte, HR, 2000) |
| "An unusual case of acute cerebral edema as part of the syndrome of portal systemic encephalopathy in an individual with established chronic liver disease is reported." | 1.29 | Acute cerebral edema as part of the syndrome of hepatic encephalopathy in an individual with chronic liver disease: a case report. ( Jabbour, N; Karavias, D; Van Thiel, DH, 1994) |
| "Rett syndrome is a neurological disorder of females characterized by dementia, autism, movement disorders and an abnormality of respiratory control." | 1.28 | Anaesthesia and Rett syndrome: a case report. ( Bachman, C; Maguire, D, 1989) |
| "We have studied an 8-year-old girl with ornithine transcarbamylase deficiency with many of the manifestations of Rett syndrome." | 1.27 | A case of ornithine transcarbamylase deficiency with Rett syndrome manifestations. ( Batshaw, ML; Hyman, SL, 1986) |
| "The syndrome was identified in nine patients; in eight, hyperammonemia occurred after administration of intensive cytoreductive therapy that resulted in profound leukopenia." | 1.27 | Syndrome of idiopathic hyperammonemia after high-dose chemotherapy: review of nine cases. ( Brusilow, SW; Burke, PJ; Karp, JE; Mitchell, RB; Przepiorka, D; Santos, GW; Saral, R; Storb, R; Wagner, JE; Watson, AJ, 1988) |
| "The definition of the boundaries of "autism" as opposed to other related pervasive developmental disorders is widely debated among clinicians and research investigators alike." | 1.27 | Autistic spectrum disorders: clinical presentation in preschool children. ( Allen, DA, 1988) |
| "The children with autism demonstrated complex stereotypies and verbal but not motor regression." | 1.27 | Rett syndrome: qualitative and quantitative differentiation from autism. ( Jankovic, J; Lewis, KR; Percy, AK; Zoghbi, HY, 1988) |
| "Rett syndrome was also observed in two sisters." | 1.27 | The clinical pattern of the Rett syndrome. ( Hanefeld, F, 1985) |
| "Rett syndrome was first described in 1966 by Dr Andreas Rett, who reported in German his findings in 22 patients." | 1.27 | The history and challenge of Rett syndrome. ( Haas, RH, 1988) |
| "Lysine was incorporated into forebrain proteins at a rate of 1." | 1.26 | Chronic inhibition of brain protein synthesis after portacaval shunting. A possible pathogenic mechanism in chronic hepatic encephalopathy in the rat. ( Conn, M; Lockwood, AH; Wasterlain, CG, 1978) |
| "Hyperammonemia with coma, tachypnea, and respiratory alkalosis developed in a 3-year-old boy with prune"-belly syndrome during a urinary tract infection with Proteus mirabilis." | 1.26 | Ammonia encephalopathy secondary to urinary tract infection with Proteus mirabilis. ( DeBeukelaer, MM; Samtoy, B, 1980) |
| "Orotic aciduria was present (max: 693 mg/day) and was related to NH4 levels." | 1.25 | [Chronic hyperammonemia with orotic aciduria: evidence of pyrimidine pathway stimulation (author's transl)]. ( Beaudry, MA; Collu, R; Dallairf, L; Ducharme, JR; Leboeuf, G; Letarte, J; Melancon, SB, 1975) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 93 (66.91) | 18.7374 |
| 1990's | 32 (23.02) | 18.2507 |
| 2000's | 11 (7.91) | 29.6817 |
| 2010's | 2 (1.44) | 24.3611 |
| 2020's | 1 (0.72) | 2.80 |
| Authors | Studies |
|---|---|
| Qutob, HMH | 1 |
| Saad, RA | 1 |
| Bali, H | 1 |
| Osailan, A | 1 |
| Jaber, J | 1 |
| Alzahrani, E | 1 |
| Alyami, J | 1 |
| Elsayed, H | 1 |
| Alserihi, R | 1 |
| Shaikhomar, OA | 1 |
| Divya, KM | 1 |
| Savitha, DP | 1 |
| Krishna, GA | 1 |
| Dhanya, TM | 1 |
| Mohanan, PV | 1 |
| Shah, SF | 1 |
| Jafry, AT | 1 |
| Hussain, G | 1 |
| Kazim, AH | 1 |
| Ali, M | 1 |
| Rivani, E | 1 |
| Endraswari, PD | 1 |
| Widodo, ADW | 1 |
| Khalil, MR | 1 |
| Guldberg, R | 1 |
| Nørgård, BM | 1 |
| Uldbjerg, N | 1 |
| Wehberg, S | 1 |
| Fowobaje, KR | 1 |
| Mashood, LO | 1 |
| Ekholuenetale, M | 1 |
| Ibidoja, OJ | 1 |
| Romagnoli, A | 1 |
| D'Agostino, M | 1 |
| Pavoni, E | 1 |
| Ardiccioni, C | 1 |
| Motta, S | 1 |
| Crippa, P | 1 |
| Biagetti, G | 1 |
| Notarstefano, V | 1 |
| Rexha, J | 1 |
| Perta, N | 1 |
| Barocci, S | 1 |
| Costabile, BK | 1 |
| Colasurdo, G | 1 |
| Caucci, S | 1 |
| Mencarelli, D | 1 |
| Turchetti, C | 1 |
| Farina, M | 1 |
| Pierantoni, L | 1 |
| La Teana, A | 1 |
| Al Hadi, R | 1 |
| Cicconardi, F | 1 |
| Chinappi, M | 1 |
| Trucchi, E | 1 |
| Mancia, F | 1 |
| Menzo, S | 1 |
| Morozzo Della Rocca, B | 1 |
| D'Annessa, I | 1 |
| Di Marino, D | 1 |
| Choya, A | 1 |
| de Rivas, B | 1 |
| Gutiérrez-Ortiz, JI | 1 |
| López-Fonseca, R | 1 |
| Xu, S | 1 |
| Cheng, B | 1 |
| Huang, Z | 1 |
| Liu, T | 1 |
| Li, Y | 1 |
| Jiang, L | 1 |
| Guo, W | 1 |
| Xiong, J | 1 |
| Amirazodi, M | 1 |
| Daryanoosh, F | 1 |
| Mehrabi, A | 1 |
| Gaeini, A | 1 |
| Koushkie Jahromi, M | 1 |
| Salesi, M | 1 |
| Zarifkar, AH | 1 |
| Studeny, P | 1 |
| Netukova, M | 1 |
| Nemcokova, M | 1 |
| Klimesova, YM | 1 |
| Krizova, D | 1 |
| Kang, H | 1 |
| Tao, Y | 1 |
| Zhang, Q | 1 |
| Sha, D | 1 |
| Chen, Y | 1 |
| Yao, J | 1 |
| Gao, Y | 1 |
| Liu, J | 1 |
| Ji, L | 1 |
| Shi, P | 1 |
| Shi, C | 1 |
| Wu, YL | 1 |
| Wright, AI | 1 |
| M El-Metwaly, N | 1 |
| A Katouah, H | 1 |
| El-Desouky, MG | 1 |
| El-Bindary, AA | 1 |
| El-Bindary, MA | 1 |
| Kostakis, ID | 1 |
| Raptis, DA | 1 |
| Davidson, BR | 1 |
| Iype, S | 1 |
| Nasralla, D | 1 |
| Imber, C | 1 |
| Sharma, D | 1 |
| Pissanou, T | 1 |
| Pollok, JM | 1 |
| Hughes, AM | 1 |
| Sanderson, E | 1 |
| Morris, T | 1 |
| Ayorech, Z | 1 |
| Tesli, M | 1 |
| Ask, H | 1 |
| Reichborn-Kjennerud, T | 1 |
| Andreassen, OA | 1 |
| Magnus, P | 1 |
| Helgeland, Ø | 1 |
| Johansson, S | 1 |
| Njølstad, P | 1 |
| Davey Smith, G | 1 |
| Havdahl, A | 1 |
| Howe, LD | 1 |
| Davies, NM | 1 |
| Amrillah, T | 1 |
| Prasetio, A | 1 |
| Supandi, AR | 1 |
| Sidiq, DH | 1 |
| Putra, FS | 1 |
| Nugroho, MA | 1 |
| Salsabilla, Z | 1 |
| Azmi, R | 1 |
| Grammatikopoulos, P | 1 |
| Bouloumis, T | 1 |
| Steinhauer, S | 1 |
| Mironov, VS | 2 |
| Bazhenova, TA | 2 |
| Manakin, YV | 2 |
| Yagubskii, EB | 2 |
| Yakushev, IA | 1 |
| Gilmutdinov, IF | 1 |
| Simonov, SV | 1 |
| Lan, K | 1 |
| Yang, H | 1 |
| Zheng, J | 1 |
| Hu, H | 1 |
| Zhu, T | 1 |
| Zou, X | 1 |
| Hu, B | 1 |
| Liu, H | 1 |
| Olokede, O | 1 |
| Wu, H | 1 |
| Holtzapple, M | 1 |
| Gungor, O | 1 |
| Kose, M | 1 |
| Ghaemi, R | 1 |
| Acker, M | 1 |
| Stosic, A | 1 |
| Jacobs, R | 1 |
| Selvaganapathy, PR | 1 |
| Ludwig, N | 1 |
| Yerneni, SS | 1 |
| Azambuja, JH | 1 |
| Pietrowska, M | 1 |
| Widłak, P | 1 |
| Hinck, CS | 1 |
| Głuszko, A | 1 |
| Szczepański, MJ | 1 |
| Kärmer, T | 1 |
| Kallinger, I | 1 |
| Schulz, D | 1 |
| Bauer, RJ | 1 |
| Spanier, G | 1 |
| Spoerl, S | 1 |
| Meier, JK | 1 |
| Ettl, T | 1 |
| Razzo, BM | 1 |
| Reichert, TE | 1 |
| Hinck, AP | 1 |
| Whiteside, TL | 1 |
| Wei, ZL | 1 |
| Juan, W | 1 |
| Tong, D | 1 |
| Juan, LX | 1 |
| Sa, LY | 1 |
| Jie, HFM | 1 |
| Xiao, G | 1 |
| Xiang, LG | 1 |
| Jie, HM | 1 |
| Xu, C | 1 |
| Yu, DN | 1 |
| Yao, ZX | 1 |
| Bigdeli, F | 1 |
| Gao, XM | 1 |
| Cheng, X | 1 |
| Li, JZ | 1 |
| Zhang, JW | 1 |
| Wang, W | 2 |
| Guan, ZJ | 1 |
| Bu, Y | 1 |
| Liu, KG | 1 |
| Morsali, A | 1 |
| Das, R | 1 |
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| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Rett Syndrome, MECP2 Duplication Disorder, and Rett- Related Disorders Natural History Protocol[NCT02738281] | 1,044 participants (Actual) | Observational | 2015-11-30 | Completed | |||
| Biobanking of Rett Syndrome and Related Disorders Protocol[NCT02705677] | 752 participants (Actual) | Observational | 2017-09-01 | Completed | |||
| Investigation on the Effects of an Osteopathic Manipulative Treatment for Constipation in People With Rett Syndrome[NCT05687214] | 12 participants (Actual) | Interventional | 2022-11-16 | Completed | |||
| Efficacy of Vitamin E in Hyperinsulinism/Hyperammonemia Syndrome[NCT04984798] | Phase 2 | 0 participants (Actual) | Interventional | 2022-11-30 | Withdrawn (stopped due to No study activity took place. The IND was withdrawn with the FDA by the Sponsor Investigator because of insurmountable hurdles in moving proposed research forward) | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
20 reviews available for ammonium hydroxide and Syndrome
| Article | Year |
|---|---|
Impact of dexamethasone and tocilizumab on hematological parameters in COVID-19 patients with chronic disease.
Topics: Acetaminophen; Acetylcarnitine; Acetylcholinesterase; Acids; Acinetobacter baumannii; Acinetobacter | 2022 |
Ammonia metabolism and hyperammonemic disorders.
Topics: Ammonia; Animals; Arginine; Biological Transport; Brain; Cell Membrane; Humans; Hyperammonemia; Hype | 2014 |
Pathophysiology of the syndrome of hyporeninemic hypoaldosteronism.
Topics: Acidosis; Aged; Aldosterone; Ammonia; Angiotensin II; Bicarbonates; Cations, Monovalent; Extracellul | 1980 |
Temporary blindness in the TUR syndrome.
Topics: Ammonia; Blindness; Glycine; Humans; Hyponatremia; Male; Middle Aged; Postoperative Complications; P | 1994 |
Hepatic encephalopathy in acute liver failure: role of the glutamate system.
Topics: Ammonia; Animals; Biological Transport; Brain; Glutamic Acid; Hepatic Encephalopathy; Liver Failure, | 1997 |
[Hyperornithinemia-hyperammonemia-homocitrullinuria (H.H.H.) syndrome].
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Diagnosis, Differential; Humans; Infant, | 1998 |
[Molecular genetic studies of mitochondrial ornithine transporter deficiency (HHH syndrome)].
Topics: Amino Acid Transport Systems, Basic; Ammonia; Animals; Base Sequence; Carrier Proteins; Citrulline; | 2001 |
Experimental liver diseases.
Topics: Alkaloids; Ammonia; Animals; Bilirubin; Budd-Chiari Syndrome; Carbon Tetrachloride Poisoning; Cattle | 1975 |
Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome: presentation as acute liver disease with coagulopathy.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Blood Coagulation Disorders; Child, Preschool; Citrul | 1992 |
Valproate hepatotoxicity syndrome: hypotheses of pathogenesis.
Topics: Ammonia; Animals; Carnitine; Chemical and Drug Induced Liver Injury; Humans; Liver; Liver Diseases; | 1992 |
[Rett's syndrome: study of 15 cases].
Topics: Ammonia; Autistic Disorder; Developmental Disabilities; Diagnosis, Differential; Electroencephalogra | 1988 |
Nonprotein nitrogen-induced ammonia toxicosis and ammoniated feed toxicity syndrome.
Topics: Ammonia; Animal Feed; Animals; Cattle; Cattle Diseases; Molasses; Ruminants; Syndrome; Urea | 1989 |
The epidemiology and public health significance of Rett syndrome.
Topics: Ammonia; Autistic Disorder; Child; Cross-Sectional Studies; Female; Humans; Intellectual Disability; | 1988 |
The therapist's role in the management of girls with Rett syndrome.
Topics: Ammonia; Autistic Disorder; Child; Child Development; Combined Modality Therapy; Female; Humans; Neu | 1988 |
The clinical recognition and differential diagnosis of Rett syndrome.
Topics: Ammonia; Autistic Disorder; Child; Child Development; Diagnosis, Differential; Female; Humans; Neuro | 1988 |
Research in Rett syndrome: past, present, and future.
Topics: Ammonia; Autistic Disorder; Child; Female; Forecasting; Humans; Neurocognitive Disorders; Research; | 1988 |
Genetic aspects of Rett syndrome.
Topics: Ammonia; Autistic Disorder; Child; Diseases in Twins; Female; Genetic Linkage; Humans; Neurocognitiv | 1988 |
Research on Rett syndrome: strategy and preliminary results.
Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Child; Child, Preschool; Female; Humans; Neurocogniti | 1988 |
Inherited hyperammonemic syndromes.
Topics: Acidosis; Amino Acids; Ammonia; Animals; Arginine; Brain; Brain Diseases; Chemical and Drug Induced | 1974 |
[Comprehensive review. Ammonia, losses and performances in large animal housing].
Topics: Adaptation, Biological; Ammonia; Anemia, Hypochromic; Animals; Cattle; Cattle Diseases; Communicable | 1973 |
3 trials available for ammonium hydroxide and Syndrome
| Article | Year |
|---|---|
Impact of dexamethasone and tocilizumab on hematological parameters in COVID-19 patients with chronic disease.
Topics: Acetaminophen; Acetylcarnitine; Acetylcholinesterase; Acids; Acinetobacter baumannii; Acinetobacter | 2022 |
Chronic sodium benzoate therapy in children with inborn errors of urea synthesis: effect on carnitine metabolism and ammonia nitrogen removal.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine | 1996 |
Effect of dietary dl-alpha-tocopherol on tissue alpha- and gamma-tocopherol and pulmonary hypertension syndrome (ascites) in broilers.
Topics: Ammonia; Analysis of Variance; Animals; Ascites; Body Weight; Chickens; Chromatography, High Pressur | 1997 |
117 other studies available for ammonium hydroxide and Syndrome
| Article | Year |
|---|---|
Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience.
Topics: Aged; Ammonia; Arginine; Biomarkers; Carnitine; Combined Modality Therapy; Decontamination; Female; | 2016 |
The rôle of ammonia in clinical syndromes.
Topics: Ammonia; Humans; Syndrome | 1956 |
[ECK'S FISTULA SYNDROME].
Topics: Ammonia; Blood Chemical Analysis; Electroencephalography; Fistula; Hepatic Encephalopathy; Portacava | 1963 |
Organic-aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation; a clinical entity.
Topics: Ammonia; Cataract; Eye Diseases; Humans; Hydrophthalmos; Intellectual Disability; Kidney; Syndrome | 1952 |
Peracute ammonia toxicity: a consideration in the pathogenesis of Phalaris aquatica 'Polioencephalomalacia-like sudden death' poisoning of sheep and cattle.
Topics: Ammonia; Animal Feed; Animal Husbandry; Animals; Australia; Cattle; Cattle Diseases; Death, Sudden; | 2005 |
A case of hyperinsulinism/hyperammonaemia syndrome with reduced carbamoyl-phosphate synthetase-1 activity in liver: a pitfall in enzymatic diagnosis for hyperammonaemia.
Topics: Adolescent; Ammonia; Carbamoyl-Phosphate Synthase (Ammonia); Diagnosis, Differential; DNA, Complemen | 2005 |
Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry.
Topics: Ammonia; Chromatography, Liquid; Citrulline; Creatinine; Humans; Mass Spectrometry; Ornithine; Syndr | 2006 |
The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria.
Topics: Ammonia; Citrulline; Fibroblasts; Humans; Liver; Male; Middle Aged; Mitochondria, Liver; Ornithine; | 1983 |
The pH of mitochondria of fibroblasts from a hyperornithinaemia, hyperammonaemia, homocitrullinuria-syndrome patient.
Topics: Acid-Base Equilibrium; Ammonia; Citrulline; Cytosol; Fibroblasts; Humans; Hydrogen-Ion Concentration | 1984 |
Ammonia encephalopathy secondary to urinary tract infection with Proteus mirabilis.
Topics: Abdominal Muscles; Absorption; Ammonia; Child, Preschool; Coma; Humans; Male; Proteus Infections; Pr | 1980 |
Rett syndrome--clinical studies and pathophysiological consideration.
Topics: Ammonia; Atrophy; Autistic Disorder; Cerebral Cortex; Child; Child Development; Child, Preschool; El | 1984 |
Gamma-glutamylornithine excretion in patients with hyperornithinemia.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Atrophy; Choroid; Chromatography; Citrulline; Dipepti | 1984 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.
Topics: Adolescent; Adult; Ammonia; Apraxias; Ataxia; Autistic Disorder; Child; Child Development; Child, Pr | 1983 |
[The olfactory phenomenon in hyperkalemic periodic paralysis].
Topics: Adult; Ammonia; Humans; Hyperkalemia; Male; Paralysis; Periodicity; Smell; Syndrome | 1984 |
Reconstitution of ornithine transport in liposomes with Lubrol extracts of mitochondria.
Topics: Amino Acid Transport Systems, Basic; Ammonia; Animals; Biological Transport, Active; Carrier Protein | 1984 |
Management of acute portal systemic encephalopathy.
Topics: Ammonia; Hepatic Encephalopathy; Humans; Syndrome | 1982 |
Transurethral prostatic resection syndrome--a new perspective: encephalopathy with associated hyperammonemia.
Topics: Aged; Ammonia; Coma; Glycine; Humans; Male; Methods; Postoperative Complications; Prostate; Syndrome | 1983 |
Transient neonatal hyperammonaemia. A case report.
Topics: Ammonia; Diagnosis, Differential; Exchange Transfusion, Whole Blood; Female; Humans; Infant, Newborn | 1982 |
Prenatal exclusion of the HHH syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Amniotic Fluid; Cells, Cultured; Citrulline; Female; | 1995 |
Acute cerebral edema as part of the syndrome of hepatic encephalopathy in an individual with chronic liver disease: a case report.
Topics: Acute Disease; Adult; Ammonia; Brain Edema; Chronic Disease; Fatal Outcome; Hepatic Encephalopathy; | 1994 |
Unexpected encephalopathy in chronic renal failure: hyperammonemia complicating acute peritonitis.
Topics: Abscess; Ammonia; Brain Diseases; Diagnosis, Differential; Female; Humans; Kidney Failure, Chronic; | 1994 |
Pathophysiology of the renal acidification defect present in the syndrome of familial hypomagnesaemia-hypercalciuria.
Topics: Acidosis, Renal Tubular; Ammonia; Calcium; Child; Child, Preschool; Female; Glomerular Filtration Ra | 1994 |
Ammoniated forage toxicosis in nursing calves: a herd outbreak.
Topics: Ammonia; Animal Feed; Animals; Cattle; Cattle Diseases; Diagnosis, Differential; Disease Outbreaks; | 1994 |
Na+,K(+)-ATPase activities are increased in brain in both congenital and acquired hyperammonemic syndromes.
Topics: Ammonia; Animals; Brain; Male; Mice; Mice, Mutant Strains; Neurotransmitter Agents; Rats; Rats, Spra | 1995 |
Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH)-syndrome. Ultrastructural changes of mitochondria in cultured dermal fibroblasts of three patients.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Child; Citrulline; Female; Fi | 1996 |
Ciliated cultured dermal fibroblasts in a patient with hyperornithinemia, hyperammonemia and homocitrullinuria (HHH) syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Cells, Cultured; Cilia; Citrulline; Endoplas | 1995 |
Ammonia and encephalopathy in the horse.
Topics: Ammonia; Animals; Biomarkers; Hepatic Encephalopathy; Horse Diseases; Horses; Liver; Syndrome | 1997 |
Hyperammonaemia associated with encephalopathy and abdominal pain without evidence of liver disease in four mature horses.
Topics: Abdominal Pain; Ammonia; Animals; Bicarbonates; Bile Acids and Salts; Bilirubin; Biomarkers; Creatin | 1997 |
A syndrome of congenital hyperinsulinism and hyperammonemia.
Topics: Ammonia; Child; Child, Preschool; Humans; Hyperinsulinism; Infant; Syndrome | 1997 |
[The Ohtahara's syndrome: a special form of age-dependent epilepsy].
Topics: Adrenocorticotropic Hormone; Age of Onset; Ammonia; Brain; Electroencephalography; Epilepsy; Female; | 1997 |
Association of interleukin-6 in the cerebrospinal fluid during crisis of calf with ammoniated feed syndrome.
Topics: Acute Disease; Ammonia; Animal Feed; Animals; Animals, Newborn; Cattle; Cattle Diseases; Humans; Int | 1997 |
Idiopathic hyperammonaemia syndrome following allogeneic peripheral blood progenitor cell transplantation (allo-PBPCT).
Topics: Adult; Ammonia; Brain Diseases; Fatal Outcome; Female; Graft vs Host Disease; Hematopoietic Stem Cel | 1997 |
Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
Topics: Ammonia; Child; Child, Preschool; DNA Mutational Analysis; Female; Glutamate Dehydrogenase; Humans; | 1998 |
Successful treatment of hyperammonemia after lung transplantation.
Topics: Ammonia; Combined Modality Therapy; Glutamate-Ammonia Ligase; Humans; Liver; Lung Transplantation; M | 1998 |
Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Amino Acid Substitution; Amino Acid Trans | 1999 |
Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics.
Topics: Adenosine Diphosphate; Ammonia; Base Sequence; Dose-Response Relationship, Drug; Genetic Markers; Ge | 1999 |
Determination of urinary orotic acid and uracil by capillary zone electrophoresis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Borates; Buffers; Citrulline; Dietary Proteins; Elect | 1999 |
Encephalopathy with idiopathic hyperammonaemia and Alzheimer type II astrocytes in equidae.
Topics: Alzheimer Disease; Ammonia; Animals; Astrocytes; Brain Diseases, Metabolic; Fatal Outcome; Female; H | 1999 |
Novel missense mutations in the glutamate dehydrogenase gene in the congenital hyperinsulinism-hyperammonemia syndrome.
Topics: Adenine; Ammonia; Codon; Cytosine; Female; Glutamate Dehydrogenase; Guanine; Heterozygote; Humans; H | 2000 |
Three novel mutations (G27E, insAAC, R179X) in the ORNT1 gene of Japanese patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Ammonia; Asian People; Biological Transpo | 2000 |
Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators.
Topics: Adenosine Diphosphate; Allosteric Site; Amino Acid Sequence; Ammonia; Enzyme Activation; Exons; Fema | 2000 |
Functional hyperactivity of hepatic glutamate dehydrogenase as a cause of the hyperinsulinism/hyperammonemia syndrome: effect of treatment.
Topics: Ammonia; Female; Glutamate Dehydrogenase; Humans; Hyperinsulinism; Hypoglycemia; Infant, Newborn; Li | 2000 |
Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome.
Topics: Adolescent; Adult; Amino Acid Substitution; Ammonia; Blood Glucose; Child; Child, Preschool; Diazoxi | 2001 |
Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia.
Topics: Adult; Amino Acids; Ammonia; Anesthesia; Blood Coagulation Tests; Citrulline; Clinical Chemistry Tes | 2001 |
A novel mutation, P126R, in a Japanese patient with HHH syndrome.
Topics: Adolescent; Ammonia; Base Sequence; Brain Diseases; Carrier Proteins; Cysts; DNA Primers; Exons; Gen | 2002 |
Amelioration of metabolic acidosis with fludrocortisone therapy in hyporeninemic hypoaldosteronism.
Topics: Acidosis, Renal Tubular; Adult; Aged; Aldosterone; Ammonia; Fludrocortisone; Glomerular Filtration R | 1977 |
Neonatal hyperammonemia.
Topics: Ammonia; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Pregnancy; Syndrome; Time Facto | 1979 |
[Ammonia formation from the amino acids in the kidney tissue of rats with an experimental nephrolithiasis syndrome].
Topics: Amino Acids; Ammonia; Animals; Ethylene Glycols; Kidney; Kidney Calculi; Male; Rats; Syndrome; Time | 1979 |
Hereditary hyperammonaemic syndromes--a six year experience.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Female; Humans; Infan | 1979 |
Reye syndrome: a metabolic response to an acute mitochondrial insult?
Topics: Acute Disease; Adolescent; Adult; Ammonia; Brain Edema; Child; Coma; Diagnosis, Differential; Female | 1978 |
Chronic inhibition of brain protein synthesis after portacaval shunting. A possible pathogenic mechanism in chronic hepatic encephalopathy in the rat.
Topics: Ammonia; Animals; Brain; Electroencephalography; Hepatic Encephalopathy; Lysine; Muscles; Nerve Tiss | 1978 |
[Cerebral energy status and the acute hyperammoniaenia syndrome: interference of S-adenosyl-L-methionine and adenosine].
Topics: Acute Disease; Adenosine; Ammonia; Animals; Biotransformation; Brain; Dogs; Energy Metabolism; Glyco | 1978 |
Congenital hyperammonemic syndromes.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Argininosuccinate Synthase; Argininosuccinic Aciduria | 1976 |
Hyperammonemia (ornithinemia) presenting as a unilateral cerebral mass lesion.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Astrocytes; Brain Diseases; Brain Neoplasms; Carbamoy | 1976 |
[Ammonia concentration in venous blood in chronic cor pulmonale syndrome].
Topics: Adult; Aged; Ammonia; Chronic Disease; Female; Humans; Male; Middle Aged; Pulmonary Heart Disease; S | 1975 |
[Chronic hyperammonemia with orotic aciduria: evidence of pyrimidine pathway stimulation (author's transl)].
Topics: Alanine; Amino Acids; Ammonia; Child, Preschool; Dietary Proteins; Erythrocytes; Growth Disorders; H | 1975 |
[A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs].
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electromyography; Humans; Leg | 1992 |
Neonatal form of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria (HHH) syndrome and prenatal diagnosis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Citrulline; Female; Gestational Age; | 1992 |
Possible platelet contribution to pathogenesis of transient neonatal hyperammonaemia syndrome.
Topics: Ammonia; beta-Thromboglobulin; Catheterization; Evaluation Studies as Topic; Female; Humans; Infant, | 1991 |
Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Adult; Ammonia; Citrulline; Female; Fibroblasts; Glutamine; Humans; Leucine; Male; Metabolism, Inbor | 1990 |
Abnormal urinary excretion of polyamines in HHH syndrome (hyperornithinemia associated with hyperammonemia and homocitrullinuria).
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biogenic Polyamines; Citrull | 1990 |
Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Fibroblasts; Heterozygote; Homozygote; Hu | 1989 |
Anaesthesia and Rett syndrome: a case report.
Topics: Adolescent; Ammonia; Anesthesia, General; Autistic Disorder; Dementia; Female; Humans; Movement Diso | 1989 |
Peripheral nerve findings in Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Biopsy; Child; Child, Preschool; Female; Humans; Micr | 1988 |
[Characteristics of the amino acid spectrum of the blood of children with intellectual deficiency].
Topics: Amino Acids; Ammonia; Arginine; Aspartic Acid; Child; Female; Glutamates; Glutamic Acid; Humans; Int | 1986 |
The physiologic basis of the TUR syndrome.
Topics: Ammonia; Animals; Cardiovascular Diseases; Central Nervous System Diseases; Female; Glycine; Hyponat | 1989 |
[Mother was right; a misdiagnosed syndrome].
Topics: Adult; Ammonia; Atrophy; Autistic Disorder; Brain; Diagnosis, Differential; Female; Humans; Metaboli | 1989 |
The history and challenge of Rett syndrome.
Topics: Ammonia; Autistic Disorder; Child; Diagnosis, Differential; Female; History, 20th Century; Humans; N | 1988 |
Therapeutic effects of a ketogenic diet in Rett syndrome.
Topics: Ammonia; Blood Glucose; Child; Child, Preschool; Dietary Fats; Electroencephalography; Energy Intake | 1986 |
Biotin and Rett syndrome.
Topics: Amino Acids; Ammonia; Biotin; Carboxy-Lyases; Female; Humans; Intellectual Disability; Methylmalonyl | 1986 |
A case of ornithine transcarbamylase deficiency with Rett syndrome manifestations.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Female; Humans; Intellectual Disability; Movem | 1986 |
A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Citrulline; Female; H | 1987 |
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child, Preschool; Citrulline; Creatine; Fem | 1987 |
Syndrome of idiopathic hyperammonemia after high-dose chemotherapy: review of nine cases.
Topics: Adolescent; Adult; Ammonia; Antineoplastic Combined Chemotherapy Protocols; Brain Diseases; Female; | 1988 |
Cognitive profile of Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Child; Child Development; Child, Preschool; Cognition | 1988 |
The nutritional aspects of Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Child; Child, Preschool; Dietary Fats; Energy Intake; | 1988 |
The orthopedic management of Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Autistic Disorder; Child; Child, Preschool; Clubfoot; Follow-Up Studies; | 1988 |
Autistic spectrum disorders: clinical presentation in preschool children.
Topics: Ammonia; Autistic Disorder; Child Development; Child, Preschool; Female; Humans; Intelligence; Langu | 1988 |
Rett syndrome: qualitative and quantitative differentiation from autism.
Topics: Adolescent; Ammonia; Autistic Disorder; Child; Child Development; Child, Preschool; Diagnosis, Diffe | 1988 |
Role of the International Rett Syndrome Association.
Topics: Adaptation, Psychological; Ammonia; Autistic Disorder; Child; Female; Humans; International Cooperat | 1988 |
Recruiting parents of children with a fatal disease as co-investigators.
Topics: Adaptation, Psychological; Ammonia; Autistic Disorder; Child; Humans; Neurocognitive Disorders; Pare | 1988 |
Recommendations regarding handling of the necropsy in Rett syndrome.
Topics: Ammonia; Autistic Disorder; Brain; Child; Female; Humans; Information Systems; Informed Consent; Neu | 1988 |
Rett's syndrome.
Topics: Ammonia; Autistic Disorder; Child, Preschool; Female; Hand; Humans; Infant; Intellectual Disability; | 1988 |
Rett syndrome in Sri Lanka.
Topics: Ammonia; Ataxia; Autistic Disorder; Child; Dementia; Female; Functional Laterality; Humans; Seizures | 1988 |
[Anesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome].
Topics: Adult; Ammonia; Anesthesia; Citrulline; Humans; Male; Ornithine; Syndrome | 1988 |
Possible pathogenetic mechanism in hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Ammonia; Citrulline; Growth Disorders; Humans; Intellectual Disability; Liver; Lymphocytes; Mitochon | 1987 |
Renovascular hypertension in a child with Rett's syndrome.
Topics: Ammonia; Atrophy; Brain; Child, Preschool; Female; Humans; Hypertension, Renovascular; Renal Artery | 1987 |
[Hyperornithinemia, hyperammonemia and homocitrullinuria syndrome in a family].
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electroencephalography | 1987 |
The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Citrulline; Dietary Proteins; Fibroblasts; Hum | 1987 |
Ammoniated forage toxicosis in calves.
Topics: Ammonia; Animal Feed; Animals; Animals, Suckling; Cattle; Cattle Diseases; Central Nervous System Di | 1987 |
The role of ammonia toxicity in the post transurethral prostatectomy syndrome.
Topics: Aged; Ammonia; Glycine; Humans; Male; Postoperative Period; Prostatectomy; Sodium; Syndrome; Therape | 1987 |
Rett syndrome revisited: a patient with biotin dependency.
Topics: Amino Acids; Ammonia; Atrophy; Biotin; Brain; Child; Female; Humans; Syndrome | 1986 |
Clinical and laboratory study in 22 patients with inherited hyperammonemic syndromes.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Coma; Female; Humans; Male; Orotic Acid; Pedig | 1986 |
[H-H-H syndrome: a rare disease also relevant in anesthesiology].
Topics: Adolescent; Ammonia; Anesthesia, General; Citrulline; Femoral Fractures; Fracture Fixation, Internal | 1986 |
Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria).
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biological Transport; Child, Preschool; | 1986 |
Effects of glycine on hemodynamic responses and visual evoked potentials in the dog.
Topics: Ammonia; Animals; Arginine; Blood Pressure; Cardiac Output; Dogs; Evoked Potentials, Visual; Glycine | 1985 |
A contribution regarding the Rett syndrome.
Topics: Ammonia; Atrophy; Cerebral Cortex; Child Development; Child, Preschool; Electroencephalography; Fema | 1985 |
The clinical pattern of the Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Ataxia; Child; Child Development; Child, Preschool; Dementia; Female; Fo | 1985 |
Abnormal breathing in the Rett syndrome.
Topics: Adolescent; Adult; Ammonia; Apnea; Child; Child Development; Dementia; Electroencephalography; Femal | 1985 |
Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Child; Citrulline; Cytoplasm; H | 1985 |
Complications of intravenous feeding.
Topics: Ammonia; Humans; Parenteral Nutrition; Syndrome | 1973 |
Raised plasma-ornithine and gyrate atrophy of the choroid and retina.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Aqueous Humor; Atroph | 1973 |
Arginosuccinicaciduria. The hair abnormality.
Topics: Amino Acids; Ammonia; Arginine; Ataxia; Blood Urea Nitrogen; Child, Preschool; Chromatography, Paper | 1974 |
Grass tetany: an hypothesis concerning its relationship with ammonium nutrition of spring grasses.
Topics: Amides; Ammonia; Animal Feed; Animals; Biodegradation, Environmental; Cattle; Cattle Diseases; Femal | 1974 |
A new diagnostic method of the urolithiasis syndrome of cattle.
Topics: Ammonia; Animals; Calcium; Cattle; Cattle Diseases; Chlorides; Magnesium; Phosphorus; Potassium; Sod | 1974 |
Ophthalmological and genetic considerations on a case of Lowe's syndrome.
Topics: Ammonia; Child, Preschool; Corneal Dystrophies, Hereditary; Eye Diseases; Genetic Counseling; Growth | 1972 |
Hyperglycinemia with ketosis due to a defect in isoleucine metabolism: a preliminary report.
Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Diet Therapy; Dietary Proteins | 1972 |
Reye's syndrome. II. Occurrence in the absence of severe fatty infiltration of the liver.
Topics: Adipose Tissue; Alanine Transaminase; Ammonia; Autopsy; Blood Glucose; Brain Diseases; Child, Presch | 1972 |
Reye's syndrome. I. Blood ammonia and consideration of the nonhistologic diagnosis.
Topics: Adipose Tissue; Alanine Transaminase; Ammonia; Biopsy; Blood Glucose; Brain Diseases; Child; Child, | 1972 |
Oculocerebrorenal syndrome. (Lowe syndrome).
Topics: Ammonia; Child, Preschool; Eye Diseases; Growth Disorders; Humans; Intellectual Disability; Intestin | 1972 |
[Case of Lowe's oculo-cerebro-renal syndrome].
Topics: Ammonia; Child, Preschool; Eye Diseases; Growth Disorders; Humans; Intellectual Disability; Intestin | 1973 |
Lowe's syndrome. Absence of amino acid transport defect in cultured fibroblasts.
Topics: Ammonia; Biological Transport; Carbon Radioisotopes; Culture Techniques; Eye Diseases; Fibroblasts; | 1973 |
The neuropathology of a peculiar form of cerebro-renal syndrome in a child.
Topics: Acidosis; Ammonia; Atrophy; Bone Diseases; Brain; Brain Diseases; Cerebellum; Eye Diseases; Growth D | 1973 |
Hyperlysinuria with hyperammonemia. A new metabolic disorder.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Diet Therapy; Growth Disorders; | 1972 |
[Oculo-cerebro-renal syndrome of Lowe. (Description of a case)].
Topics: Ammonia; Child, Preschool; Eye Diseases; Growth Disorders; Humans; Intellectual Disability; Intestin | 1972 |
[Ophthalmo-cerebro-renal syndrome (OCR) in an infant 6 weeks old].
Topics: Ammonia; Eye Diseases; Growth Disorders; Humans; Infant; Intellectual Disability; Intestinal Absorpt | 1972 |
[Lowe's oculo-cerebro-renal syndrome (histologic ocular study of 2 cases)].
Topics: Ammonia; Cataract; Eye Diseases; Glaucoma; Growth Disorders; Humans; Infant; Infant, Newborn; Intell | 1971 |