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Biomedical subjects

M G Vogiatzi

Publications and source records attributed to M G Vogiatzi.

9 recordsLinked to original sources
PubMed2005

Prevalence of fractures among the Thalassemia syndromes in North America.

Historically, fractures are cited as a frequent problem in patients with Thalassemia prior to optimization of transfusion and chelation regimens. The aim of this study was to determine the prevalence of fractures in a contemporary sample of North American patients with Thalassemia. The North American Thalassemia Clinical Research Network (TCRN) database registry was used to gather historical data on 702 patients with common alpha and beta-Thalassemia diagnoses including Thalassemia Major (TM), Intermedia (TI), E/Beta, homozygous alpha Thalassemia (AT), Hemoglobin H disease (HbH) and HbH with Constant Spring (HbH/CS), who consented to a medical record chart review. Bone mineral density (BMD) measurements by DXA were available for review in a subgroup of patients (n = 312). The overall fracture prevalence among all Thalassemia syndromes was 12.1%, equally distributed between females (11.5%) and males (12.7%). Fractures occurred more frequently in TM (16.6%) and TI (12.2%) compared to E/Beta (7.4%) and alpha (2.3%). Prevalence increased with age (2.5% ages 0-10 years, 7.4% ages 11-19 years, 23.2% ages >20 years) and with use of sex hormone replacement therapy (SHRT) (P < 0.01). On average, BMD Z and T scores were 0.85 SD lower among patients with a history of fractures (mean Z/T score -2.78 vs. -1.93, 95% CI for the difference -0.49 to -1.22 SD, P = 0.02). Presence of other endocrinopathies (i.e. hypothyroidism, hypoparathyroidism and diabetes mellitus), anthropometric parameters, heart disease or hepatitis C were not significant independent predictors of fractures. These data indicate that fractures remain a frequent complication among the aging patients with both TM and TI beta-Thalassemia. However, the fracture prevalence has improved compared to published reports from the 1960s to 1970s. In addition, children with Thalassemia appear to have low fracture rates compared to the general population.

Adolescent↗
PubMed2001

Growth hormone therapy alone or in combination with gonadotropin-releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia.

Short stature in the adult patient with congenital adrenal hyperplasia (CAH) is commonly seen, even among patients in excellent adrenal control during childhood and puberty. In this study we examine the effect of GH therapy on height prediction in children with both CAH and compromised height prediction. Leuprolide acetate, a GnRH analog (GnRHa), was given to patients with evidence of early puberty. GH (n = 12) or the combination of GH and GnRHa (n = 8) was administered to 20 patients with CAH while they continued therapy with glucocorticoids. Each patient in the treatment group was matched according to age, sex, bone age, puberty, and type of CAH with another CAH patient treated only with glucocorticoid replacement. The match was made at the start of GH treatment. Of the 20 patients, 12 have completed 2 yr of therapy. After 1 yr of GH or combination GH and GnRHa therapy, the mean growth rate increased from 5 +/- 1.9 to 7.8 +/- 1.6 cm/yr vs. 5.4 +/- 1.7 to 5 +/- 2 cm/yr in the group not receiving GH (P < 0.0001). During the second year of treatment, the mean growth rate was 6 +/- 1.6 vs. 4.2 +/- 2.1 cm/yr in the group not receiving GH (P < 0.001). The height SD score for chronological age in the treatment group at the end of 1 and 2 yr of treatment improved significantly more than the nontreatment group (P < 0.01). A similar improvement in the height SD score for bone age was found in the treatment group after 1 (-1.4 +/- 0.9 vs. -1.7 +/- 0.9; P < 0.0001) and 2 yr of therapy (-0.67 +/- 0.68 vs. -1.7 +/- 1.2; P < 0.0004). The mean predicted adult height improved from 159 +/- 11 (baseline) to 170 +/- 7.5 cm (after 2 yr of therapy) closely approximating target height (173 +/- 8 cm). All patients continued the hydrocortisone treatment. In patients with CAH and compromised height prediction, treatment with GH or the combination of GH and GnRHa results in an improvement of growth rate and height prediction and a reduction in height deficit for bone age.

Adolescent↗
PubMed2001

Long term outcome in adult males with classic congenital adrenal hyperplasia.

The effects of classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency on final height and fertility were evaluated in 30 affected males, aged 17-43 yr. The mean adult height of these patients was 165.64 +/- 8.4 cm (mean +/- SD), with a mean SD score of -1.65 +/- 1.2 cm. The difference between the mean final height SD score and mean target height SD score was -1.67 +/- 1.0 cm. All patients had short stature and did not reach their estimated target heights. There was no difference in height SD score between the salt-wasting and simple virilizing CAH patients. No correlation between the final height and degree of hormonal control or bone age advancement was observed. Of the 30 subjects, 18 had testicular sonograms. Abnormal sonogram findings of testicular adrenal rests were present in 9 patients (group 1), whereas sonogram without adrenal rests comprised the remaining 9 patients (group 2). In group 1, 8 of 9 patients and in group 2, 4 of 9 patients were salt-wasters; the remainder were simple virilizers. In group 1, 7 of 9 patients had semen analysis, and all were judged infertile. Of the 6 patients in group 2 who had semen analysis, 1 was azoospermic, and the remainder were normal. During optimal adrenal hormone suppression, gonadotropins at baseline and after GnRH stimulation were significantly higher in group 1 than in group 2, reflecting the loss of Leydig cell function to secrete testosterone. In conclusion, adult males affected with CAH due to 21-hydroxylase deficiency do not achieve the height predicted from parental heights. The presence of adrenal rests within the testes of adult males with classic CAH are more frequent in the salt-wasting form and are associated with a higher risk for infertility.

Adolescent↗
PubMed1998

The short child.

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Adolescent↗
PubMed1997

Insulin does not stimulate protein synthesis acutely in prepubertal children with insulin-dependent diabetes mellitus.

Insulin treatment in adult type I diabetic patients decreases protein loss primarily by inhibiting protein breakdown without stimulating protein synthesis. In young growing rodents, insulin treatment has been reported to stimulate protein synthesis. We examined whether insulin stimulates protein synthesis in normally growing prepubertal children with insulin-dependent diabetes mellitus. Five prepubertal children with insulin-dependent diabetes mellitus (aged 8.6-11.25 yr) were studied in the postabsorptive state on two occasions: once during insulin deprivation (I-; blood glucose, 325 +/- 67.8 mg/dL; mean +/- SD) and once during insulin administration for 4 h (I+; blood glucose, 96 +/- 23.6 mg/dL). Leucine kinetics were measured using a 4-h primed continuous infusion of L-[1-13C]leucine. Serum insulin concentrations were lower (I- vs. I+, 0.6 +/- 0.3 vs. 7.5 +/- 4.3 microU/mL; mean +/- SD; P = 0.02), whereas serum beta-hydroxy-butyrate (I- vs. I+, 3.4 +/- 0.5 vs. 0.9 +/- 0.5 mg/dL; P < 0.001) and free fatty acid concentrations (I- vs. I+, 2.9 +/- 0.4 vs. 0.9 +/- 0.4 mEq/L; P < 0.001) were higher in the insulin-deprived state than during insulin administration. Leucine Ra, an index of protein breakdown (I- vs. I+, 200.5 +/- 23.4 vs. 167 +/- 17 mumol/kg.h; P = 0.008), and leucine oxidation (I- vs. I+, 56.5 +/- 20.7 vs. 29.6 +/- 9.3 mumol/kg.h; P = 0.03) were reduced by insulin treatment. Nonoxidative leucine disposal, an index of protein synthesis, was not affected by insulin treatment (I- vs. I+, 144 +/- 20.8 vs. 137.5 +/- 13.5 mumol/kg.h; P = 0.4). We conclude that the acute decline in net protein loss during insulin treatment in growing prepubertal children, like that in adults, is due primarily to an inhibition of protein breakdown without stimulation of protein synthesis.

Amino Acids↗
PubMed1997

Frequency and necessity of thyroid function tests in neonates and infants with congenital hypothyroidism.

OBJECTIVE: The American Academy of Pediatrics recommends frequent thyroid function tests in infants and children with congenital hypothyroidism (CH). Data supporting the recommended frequency are lacking. This review was conducted to assess the validity of these recommendations. METHODS: The thyroxine (T4) and thyroid-stimulating hormone (TSH) levels of 50 neonates diagnosed between 1988 to 1993 were reviewed to assess the length of time on a specific dose of levothyroxine. RESULTS: 1) Changes in the dose of levothyroxine occurred 35 times during the first year of life for the 39 children treated with .025 mg/day, five times during the first year of life for the 9 children treated with .0375 mg/day, and three times during the first year of life for the 2 children treated with .050 mg/day. 2) These dose changes occurred at varying time intervals. 3) The T4 and TSH levels obtained at visits requiring dose changes were statistically different from the T4 and TSH levels obtained at the previous two visits. The T4 and TSH levels at the two visits before the change in dosage did not differ statistically. CONCLUSIONS: 1) An initial levothyroxine dose of .0375 mg/day requires fewer dose changes than a dose of .025 mg/day. 2) A lack of statistical change in T4 or TSH levels obtained at visits before the change-in-dose visit and the variable time span between dose changes necessitate frequent monitoring regardless of the dose of levothyroxine, the previous T4 or TSH levels, or the length of time at a specific dose. 3) These data support the recommendations of the American Academy of Pediatrics regarding the frequency of thyroid function studies during the first 2 years of life.

Congenital Hypothyroidism↗
PubMed1996

Dehydroepiandrosterone in morbidly obese adolescents: effects on weight, body composition, lipids, and insulin resistance.

The hormone dehydroepiandrosterone (DHEA) has been reported to have beneficial effects on obesity, diabetes mellitus, and serum lipids in animal studies, but results in human studies are less clear. We conducted a randomized double-blind placebo-controlled trial to determine the effects of DHEA treatment on obesity and related physiologic conditions in adolescents and young adults. In this 10-week study, 13 morbidly obese subjects received a placebo for 2 weeks. After this run-in period, patients were randomized, with seven subjects (mean age, 15.5 years; body mass index [BMI, derived by dividing body weight in kilograms by height in meters squared], 48.2 +/- 9.7 [mean +/- SD]) receiving DHEA 40 mg sublingually twice daily for 8 weeks and six subjects (mean age, 18.0 years; BMI, 52.9 +/- 14) receiving placebo. Variables measured included body weight, body composition, resting metabolic rate (RMR), serum lipid levels, insulin sensitivity, and serum steroid levels. Treatment with DHEA resulted in a statistically significant increase in plasma DHEA and DHEA sulfate (DHEAS) concentrations (P < .01). Testosterone (T) levels were significantly increased in females who received DHEA. DHEA administration had no effect on body weight, sense of well-being, or any other measured variables. These findings suggest that DHEA 40 mg administered sublingually twice daily for 8 weeks has no positive effect on body weight, body composition, serum lipids, or insulin sensitivity in extremely obese adolescents and young adults.

Adolescent↗
PubMed1996

Gastrointestinal symptoms and diabetes mellitus in children and adolescents.

Because it may be difficult to evaluate gastrointestinal diseases in children with insulin-dependent diabetes mellitus (IDDM), this report highlights several clinical features unique to diabetes and emphasizes the relationship between gastrointestinal pathology and glycemic control. Two children with IDDM are described whose hyperglycemia, ketosis, and abdominal pain were the presenting features of H. pylori-positive duodenal ulcer disease and acute appendicitis, respectively. A third nondiabetic child developed persistent postprandial hyperglycemia as the initial manifestation of dumping syndrome. These patients illustrate the relationship between glycemic control and gastrointestinal pathology in children with diabetes and the effects of gastrointestinal dysfunction on glucose regulation in nondiabetic children. In children with IDDM, gastrointestinal pathology can be confused with ketoacidosis and complicate diabetes control and management. Early recognition and treatment of the underlying gastrointestinal disease often improves glycemic control. Furthermore, severe gastrointestinal dysfunction in nondiabetic children may deleteriously influence glycemic regulation and may be confused with childhood diabetes.

Adolescent↗