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Biomedical subjects

N Yordam

Publications and source records attributed to N Yordam.

At least 37 records · Page 2Linked to original sources
PubMed1995

Does gonadotropin-releasing hormone administration affect serum growth hormone levels in poorly controlled insulin-dependent and non-insulin-dependent diabetes mellitus?

Exaggerated growth hormone (GH) responses to various provocative stimuli have been reported previously in insulin-dependent diabetes mellitus (IDDM). Little is known about GH response to synthetic gonadotropin-releasing hormone (GnRH) in diabetes. It has been reported to be exaggerated in active acromegaly. We investigated GH, follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels after GnRH administration in seven IDDM and eight non-insulin-dependent diabetic (NIDDM) patients. They were poorly controlled from a metabolic point of view. Ten healthy subjects served as the control group. FSH and LH levels increased significantly after GnRH in all groups. In contrast, GnRH did not elicit significant GH increments above baseline levels in any group. Moreover, mean areas under the GH curves were comparable among the three groups. These results suggest that poorly controlled IDDM and NIDDM does not lead to inappropriate GH responses to GnRH.

Adolescent↗
PubMed1994

Serum 3 alpha-androstanediol glucuronide measurements in children with congenital adrenal hyperplasia.

To determine the value of 3 alpha-androstanediol glucuronide (3-AG) measurements in children with congenital adrenal hyperplasia, we compared serum 3AG, 17-hydroxyprogesterone (17-OHP), androstenedione (A), testosterone (T) and dihydrotestosterone (DHT) levels and 24-h urinary 17-ketosteroid (17-KS) excretion in 42 female children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, including 27 with the simple virilizing and 15 with the salt-losing form. Their mean age was 74.5 +/- 48.5 months (range, 6-194 months). Twenty-four-hour urinary 17-KS excretion and serum 3-AG, A, T, DHT and 17-OHP levels were measured in the patients. The values were less than the mean + 2 SD of the control group in 63%, 74%, 67%, 69%, 60% and 31% of the patients, respectively. Serum 3-AG levels correlated with 24-h urinary 17-KS excretion (r = 0.66) and plasma A (r = 0.80), 17-OHP (r = 0.56), T (r = 0.79) and DHT (r = 0.62) levels. We conclude that serum 3-AG is a useful metabolic index in the management of children with congenital adrenal hyperplasia.

17-alpha-Hydroxyprogesterone↗
PubMed1993

Growth response to growth hormone-releasing hormone(1-29)-NH2 compared with growth hormone.

To assess the growth-promoting effect of different doses of growth hormone-releasing hormone(1-29)-NH2 (GHRH(1-29)-NH2) in GH deficiency (GHD) of hypothalamic origin, 43 prepubertal children aged between 4.3 and 18.9 years (mean 10.4 +/- 2.9 years) were randomly assigned to three treatment regimens: low-dose GHRH(1-29)-NH2 (LD group; n = 15), high-dose GHRH(1-29)-NH2 (HD group; n = 12) and GH (GH group; n = 16). The LD group received GHRH(1-29)-NH2 at 30 micrograms/kg/day s.c. in three daily doses, the HD group received 60 micrograms/kg/day s.c. in three daily doses and the GH group received GH, 0.1 IU/kg/day s.c. once daily. All children were treated for a period of 6 months. Evaluation included anthropometry, bone age, intravenous and subcutaneous GHRH(1-29)-NH2 tests and determination of insulin-like growth factor I (IGF-I) levels. An increase in height velocity of 2 cm/year or more was observed in all except two children. Height velocity during treatment was lowest in the LD group, but comparable in the HD and GH groups. An increase in height SDS for bone age occurred only in the GH-treated group. GH responses to intravenous GHRH(1-29)-NH2 showed a priming effect of the LD GHRH(1-29)-NH2 treatment, while a decrease in response occurred in the GH-treated group. Following a subcutaneous test dose of one-third of the daily dose of GHRH(1-29)-NH2, GH levels remained unchanged in both the LD and HD groups. There was accumulation of GHRH immunoreactivity over time in the HD group, but there was no correlation between measured GHRH and GH levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

The incidence of non-classical 21-hydroxylase deficiency in hirsute adolescent girls.

Non-classical adrenal hyperplasia due to 21-hydroxylase enzyme deficiency (NC21OHD) causes hirsutism, acne and menstrual irregularities in women. Clinically, patients with NC21OHD may be indistinguishable from other hyperandrogenic women, as they all present with similar symptoms. An elevated response of cortisol precursors like 17 alpha-hydroxyprogesterone (17-OHP) to ACTH stimulation is a valuable diagnostic criteria. In this study, 32 hirsute adolescent girls, aged 13-19 years, underwent i.v. adrenocorticotrophic hormone (ACTH) (Synacthen 0.25 mg) stimulation test. The results were compared with those of the controls. The plasma levels of 17 alpha-hydroxyprogesterone, cortisol, dehydroepiandrosterone sulphate (DHEA-S), androstenedione, testosterone, follicle stimulating hormone (FSH), luteinizing hormone (LH) and prolactin were established before, and 60 min after the infusion of ACTH to both patients and controls. Six patients demonstrated an increase in both the 17 alpha-hydroxyprogesterone levels and the 17 alpha-hydroxyprogesterone/cortisol ratio on ACTH stimulation, almost twice that of the mean +/- 2SD in the control group and ten times that in one patient. Six patients with abnormal elevation of 17 alpha-hydroxyprogesterone were considered heterozygotes for 21-hydroxylase enzyme deficiency, and one patient was presumed to have NC21OHD. Human leukocyte antigen (HLA) analysis supported these diagnoses. In this study, the incidence of NC21OHD in hirsute adolescent girls in our population was investigated, and NC21OHD was found in only one of 32 patients.

17-alpha-Hydroxyprogesterone↗
PubMedDate not supplied

Brown tumour as a complication of secondary hyperparathyroidism in severe long-lasting vitamin D deficiency rickets.

UNLABELLED: Brown tumour is a localised form of fibrous-cystic osteitis associated with primary or secondary hyperparathyroidism. Despite the fact that secondary hyperparathyroidism occurs in vitamin D deficiency rickets, no cases of rickets with brown tumour have so far been described. We present a 2.9-year-old girl who had brown tumour of the mandible due to severe vitamin D deficiency rickets. Treatment with vitamin D3 corrected the hyperparathyroidism rapidly which was followed by gradual regression in tumour size. CONCLUSION: Brown tumour can develop in severe, long-standing vitamin D deficiency rickets and responds to vitamin D treatment.

Child, Preschool↗
PubMedDate not supplied

Age-related differences in serum insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 levels in congenital hypothyroidism.

It is well established that thyroid hormones play a fundamental role in normal growth and development. The complex relationship between thyroid hormone and the growth hormone-insulin-like growth factor axis is not completely understood. We investigated age-related differences in serum insulin-like growth factor-I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3) levels in 43 patients with primary congenital hypothyroidism. These patients were classified into five age groups (Group I: 0-1 month, Group II: 1 month-1 year, Group III: 1-5 years, Group IV: 5-9 years, Group V: 9-13 years). Patients diagnosed in the first month of life did not display a significant difference in serum IGF-I and IGFBP-3 levels compared to age-matched controls (p > 0.05). However, in groups II to V, IGF-I and IGFBP-3 levels were significantly lower than in controls (p < 0.05). Thyroid hormone replacement therapy increased serum IGF-I and IGFBP-3 levels significantly in 26 hypothyroid children (p < 0.05). Although serum IGF-I and IGFBP-3 levels increase in an age dependent manner in normal children, this increment was not observed in hypothyroid children.

Adolescent↗
PubMedDate not supplied

Height prognosis in children with late-diagnosed congenital hypothyroidism.

It is a general belief that early and adequate thyroid hormone replacement achieves normalization of growth as well as disappearance of clinical sings and symptoms of hypothyroidism. Due to the lack of comprehensive growth studies, height prognosis has remained controversial in late-diagnosed hypothyroidic children. The limited number of previous studies have suggested permanent height deficit in these children. In this study we present longitudinal growth and final height of 20 children (14 females and 6 males) in whom the duration of hypothyroidism before onset of therapy varied from three to 12.6 years. The etiological distribution of cases revealed ectopic thyroid tissue in nine cases, agenesis in seven, and dyshormonogenesis in four cases. At the time of the diagnosis all hypothyroidic children had severe growth retardation (mean height SDS +/- SD -3.95+/-1.07) due to prolonged hypothyroidism. Although the catch-up spurt corrected an important part of the initial height deficit in all patients, only nine patients reached or exceeded their target height, and the final height of five patients remained below 2 SD of mean. Despite treatment, prolonged hypothyroidism may result in compromised adult height in some patients. The contributing factors to this height deficit may include the duration of hypothyroidism, the height deficit at the time of the diagnosis, etiological differences and the diminished potential for catch-up growth in late-diagnosed hypothyroidism.

Body Height↗
PubMedDate not supplied

Congenital hypothyroidism in Turkey: a retrospective evaluation of 1000 cases.

In this retrospective investigation, 1000 cases of congenital hypothyroidism followed-up in the Pediatric Endocrinology Unit at Hacettepe University Children's Hospital between 1964-1989 were evaluated with respect to age at diagnosis, main complaints, symptoms and physical findings. The mean age at diagnosis was 49.22 months, with 55.4 percent of patients diagnosed after two years of age and only 3.1 percent during the neonatal period. The main complaints of the patients were growth failure (26.7%), inability to speak (21.4%), and inability to walk (18.1%). The physical signs and symptoms most commonly detected by the physician were hypotonia (72%), constipation (66.8%), cretinoid face (64.6%), and macroglossia (64.6%). These results emphasize the necessity for routine neonatal screening programs to be established in Turkey, with the aim of detecting congenital hypothyroidism.

Age Factors↗
PubMedDate not supplied

Diabetes mellitus, diabetes insipidus, optic atrophy and deafness (DIDMOAD syndrome).

Two patients with diabetes mellitus (DM), diabetes insipidus (DI), optic atrophy (OA), deafness (D) and dilatation of the urinary tract-the so-called DIDMOAD syndrome are presented. In one of the patients, the presenting components were DI and OA. In the second case, DM was the first manifestation to be diagnosed, and in this patient the course of the syndrome was complicated by associated epileptical activity disorders, and later septicemia. The admission of these patients led us to review the literature describing this syndrome.

Adolescent↗
PubMedDate not supplied

The epidemiology of juvenile-onset insulin-dependent diabetes mellitus in Turkish children. A retrospective analysis of 477 cases.

The hospital records of 477 patients under 18 years of age with insulin-dependent diabetes mellitus (IDDM) who were followed in the Endocrinology Unit of Hacettepe University Children's Hospital between the years 1969 and 1991 were analyzed for age, sex, residence at time of diagnosis, date of onset of symptoms, date of diagnosis, family history of IDDM, and consanguinity between parents. The distribution of age at diagnosis showed a small peak between 4 and 6 years of age and a main peak between 12 and 14 years. In girls, the main peak appeared between 10 and 12 years, and in boys between 12 and 14. A significant difference was not seen between sexes (239 males and 236 females). The frequency of diagnosis showed seasonal variations the lowest in autumn and the highest in winter. Consanguinity between parents was 23.9 percent, and 10.3 percent of the patients had IDDM in first degree relatives.

Adolescent↗
PubMedDate not supplied

Leprechaunism in two Turkish patients.

We report two cases of Leprechaunism with the classical features. The first case had hyperglycemia and severe hyperinsulinemia. The postmortem examination of the second child revealed enlargement of both ovaries, islet cell hyperplasia in the pancreas, and cholestasis and paucity of bile ducts in the liver. Cystic changes were noted in the ovaries, and the kidneys contained a few small cortical cysts. Both patients died at early ages.

Abnormalities, Multiple↗
PubMedDate not supplied

The results of long-term growth hormone replacement therapy in Turkish children with growth hormone deficiency.

From a total of 118 patients treated for growth hormone deficiency, 37 (23 boys, 14 girls) have reached their final height. Twenty-five patients had isolated growth hormone deficiency (IGHD) and 12 had multiple pituitary hormone deficiency (MPHD). Growth hormone deficiency was diagnosed and treated late in both boys and girls. The mean height standard deviation score (SDS) for chronological age (CA) increased significantly from -4.43 to -1.94 during the therapy. The target height was not achieved in boys or girls nor in MPHD and IGHD groups, although they have reached the third percentile of the normal Turkish population. The height and chronological age of the patients at the start of the treatment correlated significantly with final height in all patients. Therefore, early diagnosis and treatment is important to complete catch-up growth in growth hormone deficient patients. The height prognosis is improved with administration of a recombinant form of human growth hormone (GH) as daily subcutaneous injections with a dose of 0.1 IU/kg, when compared to the earlier studies with pituitary GH.

Age Determination by Skeleton↗