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Biomedical subjects

H Schedewie

Publications and source records attributed to H Schedewie.

15 recordsLinked to original sources
PubMed1980

Glucose kinetics in leprechaunism: accelerated fasting due to insulin resistance.

Postprandial and postabsorptive glucose metabolism was studied in a 3-yr-old girl with leprechaunism by substrate and hormonal measurements and by quantifying hepatic glucose output during continuous infusion of D-[6-6-2H2]-glucose. Hepatic glucogen content and the activity of glycogen synthase and phosphorylase were also measured in the post-prandial state on a separate occasion. During the 4-h postprandial state, plasma glucose, alanine, lactate, beta-hydroxybutyrate, and glycerol were normal, as were hepatic glycogen, glycogen synthase, phosphorylase, and hepatic glucose output of 7.5 mg kg-1 min-1. Intravenous injection of glucagon (30 micrograms kg-1) caused an immediate almost 3-fold rise in glucose production consistent with brisk glycogenolysis. During the 8- to 12-h postabsorptive state, however, the patient had elevated levels of glycerol (330-508 microM) and beta-hydroxybutyrate (3291-3801 microM) and decreased levels of glucose 24-29 mg/dl) and alanine (121-135 microM) consistent with a much longer period of fasting in the normal child. Furthermore, hepatic glucose output was reduced to 3.9 mg kg-1 min-1, and iv glucagon injection failed to increase this rate; both of these observations are consistent with a hepatic state generally found only later in fasting in the normal child. From these observations we conclude that the hypoglycemia reported in the leprechaunism syndrome is due to an accelerated fasting state secondary to insulin resistance. As with long-fasted, glycogen-depleted normal children, gluconeogenesis alone is often not capable of adequately meeting the child's large noninsulin-dependent cerebral glucose requirements.

Abnormalities, Multiple↗
PubMed1979

The determination of bone age in the elbow as compared to the hand. A study in 390 children.

In a prospective study, bone age (BA) from both hand and elbow was assessed in 390 children, aged 6-15 years, in order to determine: (a) whether or not BA assessment from the hand and from the elbow give comparable results, and (b) whether the accuracy of predicting skeletal age from the hand may be improved by the additional BA determination in the elbow. BA assessment in the hand was performed according to the method of Greulich and Pyle while the standards of Schinz and Baensch were used for the elbow. Statistical analysis of data was carried out according to age groups as well as according to groups of clinical diagnoses. With only one exception in the group of so-called "healthy individuals", there was no difference between "hand age" and "elbow age". Thus, except in a very small group of subjects, both methods of BA assessment were found to be equivalent in predicting skeletal age in children between 6-15 years. Equivocal results were obtained regarding the question of whether the accuracy of BA assessment in the hand may be improved by the simultaneous BA determination from the elbow. Until further studies on larger statistical material provide more conclusive information in this matter, we feel that the combined determination of BA in the hand and elbow is not warranted for clinical purposes.

Adolescent↗
PubMed1978

Differential effects of cranial radiation on growth hormone response to arginine and insulin infusion.

The growth hormone responses to arginine infusion and to insulin-induced hypoglycemia were studied in 13 patients with neoplastic disease after treatment with radiation and chemotherapy. Patients who received intensive cranial radiation (greater than 2,400 rads) had no response to either arginine or insulin; those who received moderate cranial radiation (greater than or equal to 2,400 rads) had GH response to arginine but not to insulin; patients receiving no cranial radiation responded to both arginine and insulin. These data support the hypothesis that GH secretion in response to arginine infusion has a different mechanism in contrast to the response to insulin-induced hypoglycemia and that the latter is more vulnerable to cranial radiation.

Adolescent↗
PubMed1978

Improved iodination of peptides for radioimmunoassay and membrane radioreceptor assay.

Generally applicable methods for iodinating and purifying small peptide radiolabels for radioimmunoassay and membrane radioreceptor are described in detail. Resulting improvements in radioreceptor assay and radioimmunoassay, as well as results of analyses of specific activity, separation from unlabeled peptide, and storage characteristics, are presented for luliberin, corticotropin, melanotropin, and calcitonin.

Iodine Radioisotopes↗
PubMed1976

Experience with jejunoileal bypass for obesity.

Fifty-eight patients less than 30 years old but who are more than 300 pounds in weight were considered for investigation of jejunoileal bypass. Thirty patients had operations, and the functioning bypass included 40 centimeters of jejunum and 10 centimeters of ileum. In 14 patients, intestinal continuity was restored by either end-to-end or end-to-side anastomosis according to random choice, and in 16 patients, end-to-end anastomoses were used. At present, the follow-up period is one to four years, and the weight loss, so far, has not been significantly related to the type of reconstruction used. Weight loss correlated possitively with the preoperative weights up to one year after operation. Also, weight loss correlated positively with the total measured length of the small intestine during the first six postoperative months, but this correlation currently is showing a trend toward negativity. Food intake decreased by 2,682+/-690 calories per day at six months after operation as a result of the bypass. Chronic acidosis was common. Results of an endocrine evaluation revealed an unexpected significant decrease in parathormone levels within the normal range. A new complication, colonic pseudo-obstruction, has occurred one year or more after operation in five patients or 17 per cent of our group. This complication is related to the intestinal anaerobic flora in proximity of the defunctionalized limb; its symptoms and signs can be alleviated by giving antibiotics or Lactobacillus to change the intestinal flora. Although 90 per cent of our patients are pleased with their progress and the results of routine evaluation corroborate their satisfaction, detailed analysis of the research data available to us revealed that only 43 per cent have had beneficial effects from a jejunoileal bypass without any of the serious sequelae. We consider jejunoileal bypass for obesity justified when carried out by interdisciplinary groups dedicated to the long term follow-up study of the patient and periodic reporting of the results. The long term future for the operation is, as yet, not completely understood, and when the conditions we have recommended are not present, we cannot currently endorse the operation.

Adult↗
PubMedDate not supplied

Trimethylaminuria.

Abnormal urinary excretion of trimethylamine (TMA) signals the presence of trimethylaminuria, an inborn error of metabolism presenting with body odor of putrid fish. This article summarizes the nine reported cases (including three studied in our laboratory) and describes a rapid quantitative assay. Values of TMA are presented for 95 controls (72 children), three cases of TMA uria, and two mothers. Intestinal bacteria metabolize ingested choline to TMA which is normally converted in tissues to the non-odorous TMA-oxide. This latter step is apparently defective in cases of TMA uria. Oral choline loading greatly enhances urinary TMA, confirming questionable cases. Data on excretion of TMA post-choline load are presented for cases and mother as well as for one case during choline restriction in order to remove the odor totally. The severe psycho/social problems related to this odor and the therapeutic response to choline restriction make this a diagnosis that should not be missed--a goal requiring wider capability in the laboratory and greater awareness on the part of the physician.

Adolescent↗