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Biomedical subjects

R Bundak

Publications and source records attributed to R Bundak.

At least 19 recordsLinked to original sources
PubMed2005

Metabolic control and educational status in children with type 1 diabetes: effects of a summer camp and intensive insulin treatment.

Our aim was to evaluate prospectively, in our diabetic patients, the impacts of a summer camp and intensive insulin treatment (IIT) on both metabolic control and disease-related educational level. Twenty-five patients participated in a 7-day-long summer camp. Before the camp, all patients were on therapy with short-acting human insulin (SAI) and intermediate-acting insulin (IAI) twice daily. On arrival, their insulin therapy regimen was changed by IIT including either SAI or rapid-acting insulin analogue (RAI) three times before meals supplemented by IAI at bedtime. Following the camp, all participants were given IIT with RAI plus IAI. Frequency of hypoglycaemia, insulin dose, body mass index (BMI) and glycohaemoglobin (HbA1c) levels were assessed at pre-camp and post-camp controls. To evaluate the effectiveness of camp-assisted education, all participants were regularly tested. We observed significant elevations in total daily dose of insulin and BMI at months 3 and 6 when compared with the pre-camp values but, by month 12, they were not significantly different from precamp values. The mean HbA(1c) level decreased significantly at months 6 and 12. Severe hypoglycaemic episodes and ketoacidosis were not detected during the camp and the following year. Significant improvements in knowledge about diabetes and self-management were determined at the end of the camp, after 6 and 12 months. Camp-assisted IIT with RAI improved metabolic control of diabetic children. Additionally, camp-assisted education has a positive effect on disease-related educational level and self-management.

Adolescent↗
PubMed2005

Response to growth hormone with respect to pubertal status on increased dose in idiopathic growth hormone deficiency: an analysis of Turkish children in the KIGS database (Pfizer International Growth Study).

AIM: To compare the growth response to growth hormone (GH) treatment in patients with idiopathic GH deficiency (IGHD) who were prepubertal with the response of those who were pubertal at the onset of GH therapy on an increased GH dose. PATIENTS AND METHODS: Among the Turkish patients enrolled in the Pfizer International Growth Study (KIGS) database with the diagnosis of IGHD, the growth data over 2 years of GH therapy were analyzed longitudinally of 113 (79 M) prepubertal (Group 1) and 44 (33 M) pubertal (Group 2) patients. Pubertal signs were reported to be present initially or to have appeared within 6 months of GH therapy in Group 2. Mean +/- SD age at onset of therapy was 8.7 +/- 3.5 and 13.5 +/- 1.8 years; height SDS -4.2 +/- 1.4 and -3.2 +/- 1.1 (p < 0.05) in Groups 1 and 2, respectively. Mid-parental height (MPH) SDS did not show a significant difference between the two groups (-1.5 +/- 1.1 vs -1.7 +/- 1.1). RESULTS: Delta height SDS over 2 years of therapy was significantly higher in Group 1 (1.1 +/- 1.0) than in Group 2 (0.7 +/- 0.6) (p <0.05) in spite of a significantly lower dose of GH (14.6 +/- 3.3 in Group 1 vs 17.0 +/- 3.1 IU/m2/week in Group 2, p < 0.05). Ht--MPH SDS showed an increase from -2.4 +/- 1.7 to -1.4 +/- 1.5 in Group 1 and from -1.5 +/- 1.5 to -0.8 +/- 1.3 in Group 2. Overall delta height SDS showed negative correlations with age (r = -0.32), height SDS (r = -0.41) and height--MPH SDS (r = -0.40) at onset of therapy (p < 0.001). CONCLUSIONS: These data show that in IGHD the slight increase (15-20%) in the dose of GH during puberty was not adequate to maintain height velocity at the same magnitude as in prepuberty, and thus was not cost effective.

Adolescent↗
PubMed2001

The effect of growth hormone treatment on biochemical indices in hypophosphatemic rickets.

BACKGROUND: Growth hormone (GH) in combination with conventional therapy in hypophosphatemic rickets (HR) has been shown to promote renal phosphate (P) conservation and to result in a better metabolic control. This study aimed at investigating the acute biochemical effects of GH in 7 patients (5 female, 2 male) with HR aged between 2.16 and 16 years. METHODS: Each patient received the following in a sequential design: oral P plus 1,25-dihydroxyvitamin D(3) [1,25-(OH)(2)D(3)] therapy to determine the optimum doses for baseline requirement followed by GH-only therapy and GH +1,25-(OH)(2)D(3) therapy and GH + P +1,25-(OH)(2)D(3) therapy each for 2 weeks with 1 washout week off treatment in between. GH was given at a dose of 0.03 mg/kg/day s.c. on a daily basis. The dose of oral P used ranged between 500 and 2,000 mg/day, and the dose of 1,25-(OH)(2)D(3) ranged between 0.25 and 0.5 microg/day and was kept constant for each child throughout the study. RESULTS: Laboratory investigations repeated at the end of each treatment, and the first washout period showed that the serum P level was highest (2.9 ng/ml) during the GH + P + 1,25-(OH)(2)D(3) period with higher serum 1,25-(OH)(2)D(3) levels: 50.9 +/- (SD) 23.4 ng/l. Parathyroid hormone and alkaline phosphatase levels did not show a significant difference between the periods. The tubular P reabsorption rate showed an insignificant increase during GH therapy periods. CONCLUSION: Considering the fixed dose of P and calcitriol, it may be concluded that GH added to conventional treatment in HR resulted in a slight improvement in the biochemical parameters without any side effects at the short term.

Adolescent↗
PubMed2001

Growth hormone treatment in short children with intrauterine growth retardation.

The aim of this prospective controlled study was to assess the effect of rhGH in short prepubertal children with intrauterine growth retardation and normal growth hormone status. Twenty-six children were randomized into treatment (12F, 4M) and control (6F, 4M) groups. Mean ages were 5.3 (1.3) yr and 4.3 (1.7) yr, respectively. rhGH (Genotropin) was used at a dose of 0.2 IU/kg/day as daily s.c. injections for two years. In the treated group, mean height SDS increased from -3.0 (0.5) to -1.9 (0.7) and height velocity SDS showed a significant increase from -1.3 (2.0) to 3.7 (1.8) in the first year (p < 0.001) and 1.6 (1.8) (p < 0.01) in the second year of treatment. In the controls, height SDS, initially -2.7 (1.4), and height velocity SDS, initially -0.9 (1.1), remained essentially the same during two years of follow-up. Height SDS for bone age changed by 0.6 in the treated group and 0.4 in the control group. Target height SDS--initial height SDS in the treated group improved by 1.1 SD but declined in the control group. IGF-I levels increased from 9.5 (4.2) nmol/l (72 [31.8] ng/ml) to 32.5 (27.0) nmol/l (244.4 [202.8] ng/ml) (p = 0.004) in the treated group while no change was observed in the controls. No adverse effects were encountered during rhGH therapy. It was concluded that rhGH treatment induces a significant increase in growth velocity in the short term. This outcome, as opposed to the unchanged indices in the control group over the same period, may be indicative of an improved height prognosis in short children born with intrauterine growth retardation treated with rhGH.

Body Height↗
PubMed2000

Summer camps for diabetic children: an experience in Antalya, Turkey.

To evaluate the effectiveness of diabetic summer camps with objective parameters, we examined the data relative to summer camps organized by our department in Antalya in the last two years. The duration of the camps was 10 days. Twenty-eight diabetic children with an average age of 13.6 +/- 2.9 years (range 8-20) participated in the first camp, fourteen of whom participated in both camps. The medical personnel consisted of three pediatric endocrinologists, one psychologist, two diabetes nurses and two dietitians. Despite a mean 10% reduction in insulin dosage and 10% increment in daily calorie intake at the beginning of the camp, hypoglycemia was common (mean, 2.4 hypoglycemic episodes per subject). Ketoacidosis was not encountered in any of the subjects during and after camps. An increment in weight in children whose weights, with respect to heights, were under the ideal weight and a decrement in weight of overweight children were observed at the end of the first camp. A significant improvement in knowledge and self-management of the disease was noted at the end of the camps. Improvement in nutrition and diabetic knowledge level of the children who participated in these consecutive camps was more obvious in the second compared with that in the first camp. No significant change in HbA1c level was observed at follow-up. In conclusion, summer camps are an invaluable way for diabetic children to gain skills in managing their disease.

Adolescent↗
PubMed2000

HLA-DR and -DQ associations with insulin-dependent diabetes mellitus in a population of Turkey.

Genetic susceptibility to insulin-dependent diabetes mellitus (IDDM) has been shown to be associated with MHC in many studies. To extend this data with a population with relatively low IDDM incidence, MHC DRB, DQA, and DQB have been investigated by polymerase chain reaction and sequence specific oligonucleotide probe hybridization (PCR/SSO) in 178 IDDM patients from Turkey and compared to 248 healthy controls. Significant differences are detected between IDDM and control groups in the frequencies of DRB1*0402 DQA1*03 DQB1*0302 (28.1% vs. 5.2%, p < 0.0001, OR: 7.1) and DRB1*0301 DQA1*0501 DQB1*02 (57% vs. 18.1%, p < 0.0001, OR: 6.1). Among the negative associations, the most strong ones are with DRB1*1401 DQA1*0101 DQB1*0503 (0.6% vs. 8.9%, p < 0.0001, OR: 0.1), DRB1*1502 DQA1*0103 DQB1*0601 (1.1% vs. 7.7%, p = 0.0023, OR: 0.1), DRB1*1301 DQA1*0103 DQB1*0603 (0.6% vs. 6.9%, p = 0.0039, OR: 0.2) and DRB1*1101 DQA1*0501 DQB1*0301 (3.9% vs. 12.1%, p < 0.0001, OR: 0.2). When the DRB, DQA or DQB genotypes of the susceptible alleles are compared, the most strong susceptibility marker of the disease is found to be DRB1*0301/*04 (31.4% vs. 2.8%, p < 0.0001, OR: 15.8) and among these, heterozygote genotype DRB1*0301/*0401 (4.5% vs. 0, p = 0.0008, OR: 24.8). These results confirm the positive associations with IDDM previously observed in other Caucasian populations and reveal many negative and strong associations which maybe underlining several characteristics that distinguish Turkish diabetics form other Caucasians.

Child↗
PubMed2000

A comparison of the use of premixed insulins in pen-injectors with conventional patient-mixed insulin treatment in children and adolescents with IDDM. Is there a decreased risk of night hypoglycemia?

Insulin injection is a problem in pediatric and adolescent age, and premixed insulin therapy given in pen-injectors (Novopen II) is expected to increase compliance. Compliance with treatment and safety of this kind of insulin substitution was investigated in 20 IDDM patients (8.2-19.6 years old). The study was of randomized cross-over design and its duration was 6 (2x3) months. Metabolic parameters were compared between premixed insulin therapy via pen-injector and patient-mixed insulin therapy via conventional syringe, and no differences were observed except for the postponing of night hypoglycemic attacks to 07.00 a.m. during premixed insulin therapy. No technical or medical problems occurred. Patients were more satisfied with the new therapy regimen as determined by questionnaire. We concluded that this kind of insulin substitution is safe in pediatric and adolescent IDDM patients.

Adolescent↗
PubMed2000

Bilateral ovarian steroid cell tumor in congenital adrenal hyperplasia due to classic 11beta-hydroxylase deficiency.

An 8.7 year-old patient, raised as a boy, presented with premature appearance of pubic hair and accelerated growth since 2 years of age and ambiguous genitalia noted at birth. There was first degree consanguinity between his parents. A similar problem was reported in a cousin. Examination of the external genitalia revealed complete scrotal fusion, a 5 cm long phallus, urogenital sinus at base of phallus with no gonads palpable. Pigmentation was increased. His blood pressure was 150/100 mm Hg. Pubic and axillary hair were at stage 3. Bone age was 17 years. Adrenal ultrasound was normal. Pelvic ultrasound showed relatively enlarged uterus and ovaries with normal echogenicity. Karyotype was 46,XX. Hormone profile was compatible with congenital adrenal hyperplasia (CAH) due to 11beta-hydroxylase deficiency (11-deoxycortisol: 11.5 nmol/l [400 ng/dl] [normal: 0.6-4.5 nmol/l [20-155 ng/ml]], androstenedione: 17.4 nmol/l [5 ng/ml] [normal: 0.1-1.2 nmol/l [0.03-0.35 ng/ml]]). Prednisolone and antihypertensive drugs were started. The patient underwent bilateral salpingo-oophorectomy and hysterectomy at 9.1 years. Histopathological examination of both ovaries revealed steroid cell tumor. The type of the tumor was "not otherwise specified" (NOS). Basal hormone levels and ACTH test performed 10 months after the operation and 7 days off treatment reconfirmed the diagnosis of 11beta-hydroxylase deficiency. Steroid cell tumors are extremely rare forms of steroid hormone-reducing ovarian neoplasms in childhood and may coexist with or imitate virilizing CAH.

Adrenal Hyperplasia, Congenital↗
PubMed1999

Deflazacort treatment in progressive diaphyseal dysplasia (Camurati-Engelmann disease).

Progressive diaphyseal dysplasia (PDD), a rare disorder of bones, in recent years has been accepted as a systemic disease within the spectrum of connective tissue disorders associated with immunological abnormalities. Steroids have been used in the treatment of PDD with variable success. In this report PDD is described in a 5-year-old boy who presented with leg pain, fatigue, headache and anorexia with an onset in infancy. Physical examination revealed a waddling gait, thorax deformity and thickening in the upper extremities. The diagnosis was made by radiologic demonstration of cortical thickening and a narrowed medullary cavity of the long bones of extremities. Bone scintigraphy showed areas of increased osteoblastic activity in the diaphyseal part of the long bones of extremities and the skull. Electron microscopic examination revealed myopathic and vascular changes. Serum immunoglobulin A, G and M levels were elevated and CD4 positive T cell numbers were low. Deflazacort, a steroid with a similar anti-inflammatory effect to prednisolone but with fewer adverse effects, was started in a dose of 1.2 mg/kg/day. Deflazacort treatment resulted in clinical and radiological improvement within 12 months with no side effects. In conclusion, steroids may be recommended as an effective method of treatment in PDD and deflazacort may be a safe alternative steroid.

Anti-Inflammatory Agents↗
PubMed1996

Spironolactone therapy in hypertrichosis.

Although regarded as a benign condition, simple hypertrichosis may be very disturbing to a child cosmetically. An abnormality in androgen metabolism has been implied in the etiology of simple hypertrichosis recently. This study was undertaken to investigate the effect of spironolactone therapy for its antiandrogenic property in 12 prepubertal girls with hypertrichosis with no underlying etiology. The girls, with a mean age of 6.9 (1.2) years, had normal height and bone age. Basal hormone levels and adrenocorticotropin stimulation test results were in the normal ranges. Pelvic and adrenal ultrasound did not reveal pathology. Total and medullary hair width were measured from hair taken from preauricular, anterior midthigh, distal and proximal forearm areas. Spironolactone was started at an oral dose of 25 and increased to 100 mg x m(-2) x day(-1) twice daily for 1 year. Total hair width decreased significantly in the preauricular and anterior midthigh regions up to 6-9 months of treatment. Medullary hair width, which was affected by therapy to a greater extent, decreased significantly in all regions up to 6 months. Both total and medullary hair width showed a tendency to increase afterwards. No side effects were encountered. Spironolactone may be used in the treatment of simple hypertrichosis; however, more data are needed to clarify the efficacy and safety of anti-androgen therapy in hypertrichosis.

Administration, Oral↗
PubMed1993

Estimation of annual height velocity based on short- versus long-term measurements.

We assessed the relationships between annual height velocity, calculated from two measurements taken at intervals of 6, 12 and 24 months, in 69 healthy prepubertal children (34 male, 35 female) aged 6.5 years (range 6.25-6.75 yrs) who were followed for 2.5 years and measured biannually. Initial height values for age were within normal ranges except for one child with a height SDS of -2.0. Mean annual velocities also conformed to British norms. Significant differences were observed between consecutive six-monthly velocities and these values failed to predict annual velocities calculated from measurements taken at 12- or 24-month intervals. Seasonal effects were thought to have some impact on the six-monthly velocity differences. Height velocity, based on measurements taken at intervals of 12 months, was essentially similar to two-year velocity values. The results indicate that in estimating annual height velocity, six-month intervals between measurements may be misleading while 12-monthly measurements are as reliable as height velocity values based on measurements taken at two-year intervals.

Age Factors↗
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↗
PubMed1988

Long-term auxologic effects of human growth hormone.

Seventy-eight children with idiopathic growth hormone deficiency treated with human growth hormone continuously for up to 5 years were studied; 58 were prepubertal at the start of treatment, and remained so throughout the treatment period, and 20 were showing pubertal signs at the start of treatment. Height velocity increased markedly in the prepubertal group over the first year of therapy, with a gradual decrease in the following years. The increased growth rate of the first treatment year continued in the pubertal group. Height standard deviation score for chronologic age increased significantly throughout all treatment years, but for bone age did not change in either group. The more frequent the treatment regimen, the better was the growth response; a dose effect independent of frequency was identified. Thus human growth hormone treatment cannot make up a deficit in height prognosis already present at diagnosis, but prevents further loss of stature, which is why early diagnosis is important.

Adolescent↗
PubMed1988

Body segments and growth hormone.

The effects of human growth hormone treatment for five years on sitting height and subischial leg length of 35 prepubertal children with isolated growth hormone deficiency were investigated. Body segments reacted equally to treatment with human growth hormone; this is important when comparing the effect of growth hormone on the growth of children with skeletal dysplasias or after spinal irradiation.

Anthropometry↗