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Biomedical subjects

J Jos

Publications and source records attributed to J Jos.

At least 55 records · Page 3Linked to original sources
PubMedDate not supplied

[Anderson's disease. Clinical and morphologic study of 7 cases].

Anderson's disease is a rare autosomic recessive condition involving the transport of fat through the intestinal mucosa, which could be due to a defect in the intestinal form (B48) of apolipoprotein B. Isolated cases and one important series only have been reported. We wish here to complete the description of the disease. Seven children (age 6 months to 13 years at time of diagnosis) were followed for one month to 15 years. They presented with a malabsorption syndrome, malnutrition, fatty diarrhea (steatorrhea 4-18 g/24 h), failure to thrive (height -1 to -5.5 SD for age) and sometimes disappearance of deep tendon reflexes. Biologically they had signs of malabsorption, hypocalciuria, osteoporosis, low serum iron, decreased levels of vitamins A and E, and hypo-alpha- (50-127 mg/100 ml) and beta- (73-175 mg/100 ml) lipoproteinemia due to decreased levels of plasma cholesterol (40-70 mg/100 ml), and phospholipids (34-67 mg/100 ml); apolipoproteins A1 (26-69 mg/100 ml and B (21-44 mg/100 ml) were also low. After a fatty meal, triglycerides and apolipoproteins did not increase and chylomicrons did not appear. Jejunal biopsies showed the characteristic aspect of enterocytes loaded with lipid droplets. On electron microscopy, these fat droplets were seen in the cytoplasm but neither in the endoplasmic reticulum and the Golgi complex nor in the intercellular spaces. They did not appear to be enclosed in membranes and differed from chylomicrons by their size and density. The disease could thus be due to an abnormal apolipoprotein B48, which would prevent its binding to triglycerides and thus the formation of chylomicrons.

Adolescent↗
PubMedDate not supplied

[Cyclic parenteral nutrition in children and glucose metabolism].

The effect of cyclic perfusion on glucose metabolism and insulin secretion was determined in 10 children under prolonged parenteral nutrition according to glycosylated hemoglobin (HbA1c) levels and C-peptide urinary excretion. Lack of glycosuria and increase in HbA1c levels (m: 4.4 +/- 0.7%) indicates a good regulation of glycemia. A ten times increase in urinary C-peptide (m: 47.2 +/- 33.3 nmol/m2-1) as compared with controls shows an hypersecretion of insulin induced by the high infusion delivery of glucose. This hypersecretion of insulin, which probably involves long-term risks, may be reduced if parenteral nutrition does not contain more than the energetic requirements for growth and if about 30% of the energetic intake is given as a lipid perfusion.

Adolescent↗
PubMedDate not supplied

[Acute cerebral edema complicating therapy for diabetic ketoacidosis in children ].

Cerebral edema complicating treatment of diabetic ketoacidosis does not seen to be very rare. This neurologic complication, which was observed in 3 cases, presents similarly in all patients, occurs mainly in children and adolescents and is usually lethal. Its mechanism has not been clearly explained yet. Various etiologic factors have been considered; sodium depletion, rapid infusion of hypotonic solutions and rapid fall of plasma glucose level might have a deleterious effect. On the basis of these data, some preventive therapeutic measures are proposed.

Blood Glucose↗