Permanent exocrine and endocrine pancreatic deficiency following hemolytic uremic syndrome.
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Biomedical subjects
Publications and source records attributed to Ambika Ashraf.
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AIM: To further define the pathogenesis of infantile hypocalcemia, the prevailing vitamin D status, and treatment outcomes. METHODS AND RESULTS: Of the 23 infants admitted with infantile hypocalcemia, 21 had biochemical evidence of hypocalcemia and hyperphosphatemia and the other two had isolated hypocalcemia. The majority of these infants had relatively low serum intact parathyroid hormone responses against the backdrop of hypocalcemia. Thirteen (56.5%) of these infants had low 25-hydroxyvitamin D (25-OHD) levels, of whom 69% were Hispanic and 23% were African American. Infantile serum vitamin D status reflected that of the mother in all the 16 instances in which it was measured. Treatment with calcitriol hastened recovery from hypocalcemia in our series. CONCLUSIONS: Relative hypoparathyroidism is the etiology in the majority of cases of late onset and early infantile hypocalcemia. We identified vitamin D deficiency in a significant percentage of infants with hypocalcemia, especially Hispanics and African Americans. Maternal 25-OHD concentrations should be ascertained if the infant has low 25-OHD levels.
CONTEXT: Considerable experimental evidence inculpates vascular endothelial growth factor (VEGF) as one of the candidate factors providing a mechanistic link between hyperglycemia and diabetic complications. AIM: The aim of the study was to assess the effect of insulin treatment and glycemic control on plasma VEGF levels in children with new-onset diabetes. METHODS: This prospective study assessed the changes in plasma VEGF levels after treatment of diabetes with insulin. We also aimed to ascertain whether there was any correlation between plasma VEGF levels and simultaneous random plasma glucose. The study comprised 19 children with new-onset diabetes mellitus between the ages of 3 and 18 yr. The control group comprised 55 healthy nondiabetic children with idiopathic short stature. RESULTS: Plasma VEGF concentrations were significantly elevated in children at diagnosis of diabetes, compared with healthy controls (P < 0.0002). Plasma VEGF levels (P < 0.01) and hemoglobin A(1C) (P < 0.0001) declined in diabetic children after insulin treatment. There was a highly significant correlation between reduction in plasma VEGF levels and hemoglobin A1C levels (r = 0.65, P = 0.0037). We did not find any correlation between the simultaneous plasma glucose values and basal VEGF. CONCLUSIONS: Presence of hyperglycemia and/ or insulin deficiency in children with new-onset of diabetes is associated with plasma VEGF elevation, even at the outset of disease, and this can be mitigated by insulin therapy.
Congenital absence of the pancreas is an extremely rare condition. We participated in the care of a patient with an unusual presentation of neonatal diabetes attributable to agenesis of the pancreas. Additional clinical features of the patient included cardiac septal defects, gall bladder agenesis and duodenal malrotation. Appropriate institution of insulin, exocrine pancreatic supplements and surgical repair of the cardiac and intestinal anomalies resulted in the infant's survival. Of the reported cases of congenital pancreas agenesis, two cases have been ascribed to mutations in the insulin promoter factor-1(Ipf-1) gene. Deletion of the Ipf-1-homolog pdx-1 in mice results in the failure of pancreas to develop. Analysis of both exons of the Ipf-1 coding sequence from the presented patient's genomic DNA, however, did not identify a mutation. These results suggest that a congenital or genetic perturbation occurred in this infant most likely before the appearance of dorsal pancreatic bud in the 3 mm long embryonic stage, around the embryonic day 25 in human development, before the onset of Ipf-1 expression.
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