pituitrin and Porphyrias

Topics: 5-Aminolevulinate Synthetase; Glucose; Heme; Humans; Liver; Porphyrias; Porphyrins; Vasopressins

Topics: Alcoholism; Animals; Diuretics; Female; Humans; Infant, Newborn; Kidney Diseases; Lactation Disorders; Magnesium; Magnesium Deficiency; Porphyrias; Pregnancy; Vasopressins

delta-Aminolevulinic acid dehydratase deficiency porphyria (ALAD porphyria, ADP) with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 69-year-old woman is reported. The patient was admitted to our hospital complaining of slight cough with low-grade fever, and treated with piperacillin sodium, resulting in complete resolution of the symptoms, following a diagnosis of bronchopneumonia. Thereafter, however, she began to complain of vomiting, abdominal pain, facial numbness and paresis of the extremities with gait disturbance, and became comatose with hyponatremia (serum Na concentration 119 mEq/L) in a few days. Laboratory tests revealed an antidiuretic hormone (ADH) level of 13.5 pg/mL, plasma osmolality 218 mOsm/KgH2O, urinary osmolality 429 mOsm/KgH20, urinary Na concentration > 20 mEq/L, and no abnormalities of thyroid, adrenal or renal function. Neither edema nor dehydration was evident. These data indicated the presence of SIADH. No abnormalities suggestive of malignant or infectious diseases such as lung cancer, pneumonia and Guillain-Barré syndrome were evident from laboratory and roentgenographic findings. As the cause of SIADH, therefore, porphyria was suspected. Metabolites and activities of enzymes in the heme biosynthetic pathway were examined, and very low activity of delta-aminolevulinic acid dehydratase (ALA-D) (0.14 mumol PBG/mL RBC/h) was found. The patient was neither an alcoholic nor a heavy smoker, and she had no past history of heavy metal intoxication, photosensitivity or tyrosinemia. On the basis of these data and clinical features, she was diagnosed as having ADP. We consider this to be the first case of ADP reported in Japan.

Topics: Abdominal Pain; Aged; Coma; Female; Humans; Inappropriate ADH Syndrome; Japan; Osmolar Concentration; Paresis; Porphobilinogen Synthase; Porphyrias; Porphyrins; Sodium; Vasopressins; Vomiting

The authors report the case of a patient who presented, during two exacerbations of intermittent acute porphyria, a grave psychiatric syndrome secondary to severe hyponatraemia. The later was due to inappropriate secretion of anti-diuretic hormone confirmed by laboratory tests and the stimation of anti-diuretic activity in the urine. The course was favourable under the effects of symptomatic treatment including sodium supplements and fluid restriction. The anti-diuretic syndrome disappeared on each occasion without sequelae at the time of regression of the exacerbation of porphyria.

Topics: Acute Disease; Hyponatremia; Porphyrias; Recurrence; Vasopressins

Topics: Blood Proteins; Humans; Inappropriate ADH Syndrome; Porphyrias; Radioimmunoassay; Vasopressins

3 cases of inappropriate vasopressin secretion during one case of anaplastic carcinoma of the lung, one case of carcinoma of the prostate with bony metastases and one case of acute intermittent porphyria are presented. The plasma levels of vasopressin, measured by radioimmunoassay were high. Treatment with demeclocycline was attempted in one case. The clearance of free water was positive but the treatment was poorly tolerated by the digestive tract.

Topics: Acute Disease; Aged; Carcinoma; Demeclocycline; Female; Humans; Lung Neoplasms; Male; Paraneoplastic Endocrine Syndromes; Porphyrias; Prostatic Neoplasms; Syndrome; Vasopressins; Water-Electrolyte Imbalance

Topics: Acute Disease; Adult; Ataxia; Barbiturates; Consciousness Disorders; Craniocerebral Trauma; Electroencephalography; Epilepsy; Female; Humans; Hyponatremia; Nystagmus, Pathologic; Porphobilinogen; Porphyrias; Urinary Incontinence; Vasopressins; Water-Electrolyte Balance

Topics: Adolescent; Female; Humans; Hyponatremia; Porphyrias; Pregnancy; Pregnancy Complications; Sodium; Syndrome; Vasopressins

Topics: Acute Disease; Age Factors; Diuresis; Female; Glomerular Filtration Rate; Humans; Hyponatremia; Middle Aged; Natriuresis; Neurologic Manifestations; Porphyrias; Vasopressins; Water-Electrolyte Balance

Topics: Carcinoma, Bronchogenic; Craniocerebral Trauma; Diuresis; Drinking; Extracellular Space; Humans; Hypertonic Solutions; Hyponatremia; Lung Neoplasms; Methods; Nicotine; Osmolar Concentration; Pituitary Function Tests; Porphyrias; Sodium Chloride; Vasopressins

Topics: Acute Disease; Adult; Amino Acids; Chlorides; Extracellular Space; Female; Humans; Hypokalemia; Hyponatremia; Levulinic Acids; Male; Nitrogen; Porphobilinogen; Porphyrias; Porphyrins; Uremia; Vasopressins; Water-Electrolyte Balance

Topics: Amino Acids; Animals; Anura; Biological Transport; Depression, Chemical; Electrophysiology; In Vitro Techniques; Kidney Tubules; Levulinic Acids; Osmotic Pressure; Porphyrias; Skin; Sodium; Vasopressins; Water; Water-Electrolyte Balance

Topics: Adult; Chlorides; Diagnosis, Differential; Epilepsy; Female; Humans; Hypertension; Hyponatremia; Porphyrias; Porphyrins; Pregnancy; Puerperal Disorders; Vasopressins

Topics: Adult; Hormones, Ectopic; Humans; Hyponatremia; Male; Middle Aged; Porphyrias; Vasopressins; Water-Electrolyte Balance

Topics: Acute Disease; Adult; Humans; Hyponatremia; Male; Middle Aged; Porphyrias; Syndrome; Vasopressins; Water Intoxication; Water-Electrolyte Balance

Topics: Humans; Porphyrias; Vasopressins; Water-Electrolyte Balance

Topics: Chlorides; Humans; Porphyrias; Potassium; Sodium; Urea; Vasopressins

Topics: Enzyme Induction; Growth Hormone; Humans; Hydro-Lyases; Hypothalamo-Hypophyseal System; Lead Poisoning; Levulinic Acids; Melanocyte-Stimulating Hormones; Porphyrias; Propionates; Pyrroles; Thyrotropin; Vasopressins; Water-Electrolyte Balance

Topics: Adolescent; Female; Genes, Dominant; Heme; Humans; Male; Pedigree; Porphyrias; Porphyrins; Vasopressins

Topics: Adolescent; Diuresis; Female; Humans; Hyponatremia; Porphyrias; Vasopressins

Topics: Carcinoma, Bronchogenic; Endocrine System Diseases; Hormones, Ectopic; Humans; Hyponatremia; Infections; Lung Neoplasms; Male; Middle Aged; Myxedema; Pituitary Diseases; Porphyrias; Vasopressins

Topics: Adult; Chemical Phenomena; Chemistry; Female; Humans; Hypothalamus; In Vitro Techniques; Ligases; Liver; Muscular Atrophy; Porphyrias; Porphyrins; Vasopressins

Topics: Female; Humans; Hypothalamo-Hypophyseal System; Levulinic Acids; Neurologic Manifestations; Porphyrias; Vasopressins

Topics: Blood; Glycolates; Hemoglobins; Humans; Hyponatremia; Magnesium; Metabolism; Pharmacology; Porphyria, Acute Intermittent; Porphyrias; Thioglycolates; Urine; Vasopressins; Water-Electrolyte Balance

Topics: Humans; Hyponatremia; Porphyria, Acute Intermittent; Porphyrias; Proteinuria; Seizures; Urine; Vasopressins

Topics: Humans; Hyponatremia; Ions; Porphyria, Acute Intermittent; Porphyrias; Sodium; Vasopressins

Topics: Electrolytes; Humans; Porphyria, Acute Intermittent; Porphyrias; Vasopressins; Water; Water-Electrolyte Balance