Interference of Tienam in colorimetric determination of 5-aminolevulinic acid and porphobilinogen in serum and urine.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A Hassoun.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Cardiac arrhythmias and circulatory collapse account for the high mortality reported after severe chloroquine poisoning. We have recently observed a 17-year-old man who ingested an 8 g chloroquine overdose. Cardiac arrest occurred within 1 h. Cardiogenic shock was refractory to epinephrine, dopamine and molar sodium lactate. Amrinone, a bipyridine analog, was then successfully used to improve haemodynamic conditions.
Porphyria cutanea tarda is a disorder of porphyrin metabolism that results from a deficiency of uroporphyrinogen decarboxylase, resulting in a characteristic pattern of porphyrin excretion. Elevated serum iron values are frequently observed among patients with porphyria cutanea tarda. The hypothesis has been advanced that a hemochromatosis allele is implicated in the clinical manifestation of porphyria cutanea tarda. We report the case of a patient suffering from both idiopathic hemochromatosis and porphyria cutanea tarda. The data of the medical literature concerning such an association are discussed.
Activities of delta-aminolevulinic acid (ALA) dehydratase and porphobilinogen (PBG) deaminase, and haem content were determined in EB-virus transformed lymphocytes from two patients with homozygous ALA dehydratase deficiency, and their family members to determine the expression of the specific gene defect in this cell type. ALA dehydratase activity, but not PBG deaminase activity or haem content, was markedly decreased in lymphocyte preparations from both patients with homozygous enzyme deficiency, and moderately decreased in subjects heterozygous for enzyme deficiency. Immunochemical quantitation of erythrocyte ALA dehydratase suggested the presence of a cross-reactive material in a patient with a late-onset of acute hepatic porphyria due to the homozygous enzyme deficiency.
Explore the source record for details and available documents.
A 48-year-old man chronically treated with digitoxin and verapamil for prevention of atrial fibrillation voluntarily ingested 2.2 mg of digitoxin. Serum digitoxin concentrations and the urinary elimination of the drug were followed over a 12-day period. Urinary data indicate that a large percentage (50%) of the drug was eliminated renally despite administration of multiple doses of activated charcoal, cholestyramine and hyperosmotic laxatives. The possible interaction with two other drugs, heparin and verapamil, is also discussed.
A man without a history of porphyric attacks developed a subacute motor neuropathy at the age of 63. At the same time the first signs of a myeloproliferative disorder were found. He had a homozygous deficiency of erythrocyte delta-aminolevulinic acid dehydratase (ALA-D) with autosomal recessive inheritance. Treatment with parenteral glucose and with hematin had a beneficial influence on the plasma ALA levels. The finding of a motor neuropathy with increased plasma levels of ALA but not of porphobilinogen (PBG) supports the potential role of ALA in the pathogenesis of porphyric neuropathy.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Erythropoietic protoporphyria (EPP), a recently described form of porphyria, often remains unrecognized. We report the case of a 7-year-old girl admitted for investigation of photosensitivity since the age of 18 months without any significant objective cutaneous lesions. Clinical features, quantitative determinations of porphyrins in blood, urine and stools, ferrochelatase activity and cutaneous histopathology helped to confirm the diagnosis of EPP. A familial study was also performed. The clinical, laboratory and genetic characteristics of EPP are reviewed.
In 10 adult patients acutely intoxicated with methanol, the base deficit and the total blood CO2 were highly correlated with blood formate level. No correlation was found between the same parameters and blood methanol level. Formate production is the main cause of acidosis in the early stages of methanol poisoning. The association of a latency period before treatment exceeding 10 hours and a blood formate level above 0.5 g/l (11.1 mmol/l) is predictive of severe methanol poisoning possibly leading to permanent sequelae.
Porphyrin metabolism was investigated in a 63-year-old male patient who developed a subacute onset polyneuropathy with predominance of motor signs in the upper limb. The screening for lead, cadmium, mercury, aluminum and thallium was negative. The study of porphyrin metabolism showed remarkable abnormalities, particularly a very high level of plasmatic 5-aminolaevulinic acid contrasting with a normal level of porphobilinogen and a nearly complete loss of activity of aminolaevulinic acid dehydratase with no regenerative response to dithiothreitol or zinc ions. The other causes of aminolaevulinic acid dehydratase deficiency (tyrosinaemia, alcoholism, smoking, cirrhosis, renal insufficiency, diabetes mellitus) were ruled out. The diagnosis of primary aminolaevulinic acid dehydratase deficiency was proposed and confirmed by the familial study, which revealed the existence of several heterozygous members in this family.
The investigation and follow-up of three cases of EPP led us to reevaluate the signs and symptoms of the disease, the usefulness of early skin biopsies and protoporphyrin and ferrochelatase determinations. A review of the literature brought forth the salient features of this inborn error of porphyrin metabolism and stressed the value of treatment with beta-carotene and canthaxantine but also the risk of the so-called "gold dust" retinopathy.
Explore the source record for details and available documents.
Seventy-two cases of diffuse pulmonary contusion, 50 of which concerned multiple injury patients, are retrospectively described. The pleuro-parietal and associated lesions, the clinical, radiological and laboratory findings and the extent of the damage are analyzed. Treatment of shock (50 cases), mechanical ventilation (51 cases), thoracotomy (7 cases) and surgery of associated lesions are studied. Morbidity and mortality (34.7%) are detailed. The nosology of pulmonary contusion is discussed in relation to several factors, including shock, perfusions and associated lesions. The principles of treatment are restated. The most important complication is pulmonary superinfection. Diffuse pulmonary contusion is a serious condition. Associated lesions and their treatment contribute to its outcome.
In patients with renal failure and on chronic hemodialysis, serum aluminum, serum delta-aminolevulinic acid, serum porphobilinogen and erythrocyte zinc protoporphyrin (ZPP) are significantly elevated, whereas erythrocyte delta-aminolevulinic acid dehydratase activity (ALAD, values in percent) is significantly reduced. The last two parameters (ZPP and ALAD) are statistically related to serum aluminum concentration (Al-S), but only the correlation between Al-S and ALAD remains statistically significant after standardization for the degree of renal insufficiency (expressed in terms of urea level). This study does not support the hypothesis that the retention of aluminum is responsible for the increase of ZPP in uremic patients on dialysis. The disturbances of porphyrin metabolism found in patients with renal failure and on chronic dialysis are not similar to those observed in porphyria cutanea tarda.
Explore the source record for details and available documents.