Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162275 (ketonuria)
553 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of propionicacidemia are reported. The first patient was a child aged 16 months with psychomotor retardation, severe disturbances of wakefulness, and vomiting; the second case concerned a new-born baby with metabolic acidosis and neurological disorders. In both cases the diagnosis of propionicacidemia was made after the discovery of ketonuria, and raised blood ammonia and glucose levels. Appropriate dietary measures led to great improvement in the first case, and nearly normal psychomotor development in the second case at 7 months of age. Problems related to biotin sensitivity, leucine intolerance, and raised blood ammonia levels are discussed.
Sem Hop
PMID:[Propionicacidemia. A report on two cases (author's transl)]. 624 24

Fifteen children with severe diabetic ketoacidosis (pH less than 7,10) were treated according to two protocols. Protocol I used in 7 patients, consisted in a low dose insulin therapy by intravenous and intramuscular injections. Protocol II consisted in a low dose continuous intravenous insulin therapy, with insulin added to the rehydration solution at constant concentration (22 U/l). Rehydration and potassium supplementation were similar in both methods. The fall of glucose concentration, rise in venous pH, fall of diuresis, glycosuria, ketonuria were similar in the two groups. With protocol I three hypoglycemias (greater than 4 mM) and three hypokalemias (greater than 3 mEq/l) did occur. With protocol II no hypoglycemia and only one moderate hypokalemia was observed. Thus, continuous infusion of insulin added to the rehydration solution, including isotonic bicarbonate, isotonic saline, glucose and electrolytes is as effective as discontinuous insulin infusion, but with a lower incidence of complications. In addition, this method has the advantage of being easy and applicable everywhere.
Sem Hop 1982 Mar 18
PMID:[Treatment of severe diabetic ketoacidosis in children. A comparative study of two methods (author's transl)]. 627 10