The correlation of serum ferritin level to Ca-P metabolism and bone density study in thalassemic patients.
ABSTRACT A study of Ca-P related hormones and bone densities was carried out in seven homozygous beta-thalassemia patients. The levels of Ca, P, and Ca-P related hormones were not significantly changed except for 1,25(OH)2D3 which was significantly lower than in the control group and was inversely related to patients' ferritin levels. Bone densities were markedly reduced in all the thalassemic patients.
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ABSTRACT: Six children with severe forms of cyanotic congenital heart disease underwent a series of challenge studies in an attempt to clarify the pathophysiology of the hypoglycemia observed in these disorders. The glycemic response to intravenous glucagon positively correlated with the fasting glucose concentration (P<0.01). Following a 26-to 30-hour fast, two of six CCHD children developed hypoglycemia in the absence of significant differences in ketone body, alanine, or insulin concentrations when compared with controls. Blood lactate (P<0.05), pyruvate (P<0.02), plasma glucagon (P<0.05), and cortisol (P<0.01) values were increased at the end of the fast when compared to those in normal fasting children. Intravenous alanine challenge at the end of the fast demonstrated normal glycemic responses in all subjects, regardless of initial plasma glucose or lactate concentrations. One subject had an abnormal rise in lactate and β-hydroxybutyrate following alanine administration. Glucose and alanine turnover studies utilizing stable isotopes revealed normal glucose kinetics but lower alanine flux (8.4±1.1 μmol/kg·min vs 12.3±1.15 μmol/kg/min, P<0.05) and metabolic clearance rates (28.0±3.5 vs 51.0±7.4 ml/kg·min) in the CCHD subjects when compared to normal children. We conclude that hepatic glycogenolytic and glyconeogenic enzyme mechanisms are intact in patients with CCHD. The hypoglycemia is associated with glycogen depletion but not with abnormal secretion of glycoregulatory hormones. The decreased alanine metabolic clearance rate and the increased fasting lactate and pyruvate concentrations may reflect decreased hepatic uptake of these potential gluconeogenic substrates, leading to a functional defect in hepatic gluconeogenesis. These abnormalities may be secondary to decreased hepatic perfusion, but other mechanisms cannot be directly excluded.Journal of Pediatrics 09/1979; 95(2):220-7. DOI:10.1016/S0022-3476(79)80655-1 · 3.74 Impact Factor
- Journal of Pediatrics 06/1965; 66:964-74. · 3.74 Impact Factor
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ABSTRACT: Patients with severe thalassaemia major suffer endocrine and other abnormalities before their eventual death from iron overload due to repeated blood transfusions. The endocrine status of 31 thalassaemic patients aged 2-5 to 23 years was investigated. Exact data were available on the rate and duration of blood transfusion in all of them and in many the liver iron concentration was also known. Although the patients were euthyroid, the mean serum thyroxine level was significantly lower, and the mean thyrotrophic hormone level significantly higher, compared with the values found in normal children. Forty oral glucose tolerance tests with simultaneous insulin levels were performed in 19 children, of whom 5 developed symptomatic diabetes and one had impaired tolerance. Previous tests on all 6 patients were available and some showed raised insulin levels possibly due to insulin resistance. 2 patients had clinical hypoparathyroidism and are described. The parathyroid hormone levels determined by radioimmunoassay in 25 patients were below the mean for the age group in all and outside the reference range in 16. Nonfasting plasma calcium levels were not reduced. Puberty was delayed in some patients. Concentrations of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) measured in urine from 7 girls and 5 boys showed considerable variation. In the boys there was an overall tendency for FSH and LH excretion to be low with regard to age, but with respect to puberty rating FSH exretions were normal or low and LH normal or raised. The girls showed a tendency for LH but not FSH excretion to be raised in relation to puberty rating. The severity of the endocrine changes was related to the degree of iron loading and is discussed in relation to previous work in which the iron loading has rarely been accurately indicated nor parathyroid status assessed.Archives of Disease in Childhood 12/1976; 51(11):828-36. DOI:10.1136/adc.51.11.828 · 2.91 Impact Factor