ABSTRACT
OBJECTIVE: To investigate two related children with congenital permanent diabetes. SUBJECTS AND METHODS: Two related male patients of Arab origin with congenital permanent diabetes were studied for immunological and genetic markers, insulin and glucagon secretion, thiamine levels, pancreatic ultrasounds studies and developmental characteristics. RESULTS: Both patients developed normally. The immunological markers were negative, there was no ketosis at diagnosis, and both patients were negative for diabetes susceptibility alleles at the HLA locus. Insulin levels were undetectable, glucagon secretion, thiamine levels and pancreatic ultrasound studies were normal. CONCLUSIONS: Congenital permanent diabetes is an extremely rare entity that differs from type I or type II diabetes. Our patients have a unique disorder with isolated beta cell defect and no additional manifestations. Autosomal recessive inheritance is suggested.
Subject(s)
Diabetes Mellitus/congenital , Child , Child, Preschool , Diabetes Mellitus/diagnostic imaging , HLA-DR Antigens/analysis , Humans , Male , Pedigree , Thiamine/blood , UltrasonographyABSTRACT
A fatal case of poisoning due to ingestion of an apparently innocuous household product is described. A healthy 28-year-old man accidentally drank floor polish (Cristalizador, a Spanish import). On arrival at the emergency room a few hours later he passed large amounts of bloody stool and lost consciousness. A call to the Spanish Poison Center revealed that although not indicated on the label, the polish contained a highly poisonous salt, fluoro-silicate. Blood gas analysis revealed severe metabolic acidosis. Serum calcium was 3.8 mg/dL. The post-resuscitation ECG showed subendocardial ischemia and ST-elevation in the anteroseptal wall and prolonged QT-interval. In the intensive care unit he received large amounts of fluids, dopamine, sodium bicarbonate and calcium. Despite the treatment, his condition continued to deteriorate: VPB's appeared, there was a short run of ventricular tachycardia and then atrial fibrillation developed. Further treatment included lidocaine, verapamil, amiodarone, and electrical cardioversion. Blood pressure remained low and 11 hours after admission he died of myocardial infarction, severe arrhythmia and multi-organ failure.
Subject(s)
Fluoride Poisoning , Household Products/poisoning , Silicic Acid/poisoning , Adult , Electrocardiography , Fatal Outcome , Fluoride Poisoning/blood , Fluoride Poisoning/physiopathology , Fluorides , Humans , Male , ResuscitationABSTRACT
Obidoxime is an oxime used in several countries as an antidote in organophosphate intoxication. Its pharmacokinetics were studied in a 20 year-old female with severe and complicated methamidophos intoxication. Obidoxime elimination half life was 6.9 h, volume of distribution 0.845 L/kg, total body clearance 85.4 mL/min, and renal clearance 69 mL/min (creatinine clearance 54 mL/min). Eighty percent of the dose was excreted in the urine over 5 h. Possible reasons for the different pharmacokinetic values as compared with values previously reported in healthy volunteers are discussed. Obidoxime dose should be adjusted according to renal function. More studies are needed to establish the therapeutic window of obidoxime in patients with organophosphate intoxication.
