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Biomedical subjects

S Darcan

Publications and source records attributed to S Darcan.

17 recordsLinked to original sources
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

Molybdenum cofactor deficiency associated with Dandy-Walker complex.

Molybdenum cofactor deficiency is a rare and devastating disease leading to intractable seizures in the neonatal period. Severe loss of neocortical neurons, gliosis, and cystic necrosis of cerebral white matter resulting in significant cerebral volume loss are the neuropathological findings. The mechanism of cerebral injury is unknown, but sulphite excess, and sulphate or uric acid deficiencies are possible factors. We present here a new case of Molybdenum cofactor deficiency associated with Dandy-Walker complex with a history of three dead siblings, the latter also having Dandy-Walker malformation. We speculate that severe cerebral volume loss due to the above mentioned mechanisms may lead to an appearance resembling Dandy-Walker malformation.

Brain↗
PubMed2001

Evaluation of growth hormone secretion after completion of therapy.

BACKGROUND: Growth hormone (GH) reserve in young adults previously diagnosed as having GH insufficiency, who were treated with human (h)GH replacement in childhood needs confirmation in adulthood. METHODS: Nine patients (seven males, two females; two empty cella, one hypoplasia of the hypophysis and six with idiopathic GH deficiency) diagnosed as having GH insufficiency by the insulin tolerance test (ITT) and dopamine stimulation test in childhood (mean age 12.8+/-2.6 years) were retested at completion of linear growth (mean age 21.0+/-3.0 years), 4.6+/-1.6 years after discontinuation of hGH therapy. RESULTS: At the initial diagnosis, seven had complete and two had partial GH deficiency. At diagnosis, the mean peak GH response to ITT and dopamine was 4.8+/-4.08 and 3.4+/-2.9 mU/L, respectively. At retesting, the mean GH response to ITT and dopamine stimulation was 3.5+/-2.5 and 3.3+/-3.1 mU/L, respectively (P=0.91 and 0.96, respectively). During hGH therapy, mean height velocity increased from 3.5+/-1.9 cm/year at diagnosis to 9.9+/-3.64 cm/year during the first year (P=0.002). One of nine children diagnosed as having GH insufficiency who was treated with hGH replacement had normal growth hormone secretion at completion of linear growth. CONCLUSIONS: All GH-insufficient children should be retested after completion of their hGH treatment and linear growth to identify those who are truly GH insufficient and who may benefit from GH therapy in adulthood.

Adolescent↗
PubMed2001

Association between serum leptin and anthropometric parameters at birth and at 15th day of life in infants born asymmetrically small for gestational age.

The aim of this study was to investigate changes in skinfold measurements taken at three sites, mid-arm circumference and umbilical circumference during the first 15 days of life; and to evaluate relationships between anthropometric measurements and umbilical cord blood serum leptin levels in infants born small for gestational age (SGA) and appropriate for gestational age (AGA) infants. Of 50 newborn infants, 25 were SGA and 25 were AGA. Neonates' weight, mid-arm circumference (MAC), umbilical circumference (UC), and triceps, subscapular and periumbilical skinfold thicknesses were measured (Holtain callipers) immediately after delivery. Anthropometric parameters were measured again at 15th days of age. At birth, mean birth weight, mean skinfold thickness, MAC and UC measurements in the AGA group were significantly higher than those of the SGA group. These differences were also found on the 15th day. Birth weight correlated with all skinfold thicknesses, MAC and UC at birth. Weight at 15th day of life correlated with skinfold thicknesses, MAC and UC at 15th day of life. Cord blood leptin level was significantly lower in the SGA than in the AGA infants. This difference continued on the 15th day. When cord blood leptin level was compared with that of the 15th day, we found that leptin levels in the cord blood were significantly higher. There were significantly positive correlations between leptin levels and birth weight and skinfold thicknesses when the infants were all grouped together. When the newborns were grouped according to birth weight, there were positive correlations between cord blood serum leptin levels and these parameters in the AGA group, but no correlation in the SGA group. At the 15th day of life serum leptin levels correlated with weight, subscapular and triceps skinfold thickness in the AGA group, but only with triceps skinfold thickness in the SGA group.

Anthropometry↗
PubMed2001

Localized aggressive periodontitis in a patient with type 1 diabetes mellitus: a case report.

BACKGROUND: Poor metabolic control of diabetes mellitus (DM) has often been associated with the severity of periodontal disease. The aim of this report is to present a 9-year-old female with localized aggressive periodontitis who had a history of type 1 DM and the outcome of her treatment. METHODS: The patient had received medical, clinical, and radiographic periodontal examinations. Peripheral blood analysis was done as well. She had non-surgical periodontal treatment, and medical management of her diabetes was performed at the same time. She was followed longitudinally for 5 years. RESULTS: Medical examination revealed no pathological findings except for growth retardation. Laboratory tests showed that she had poor metabolic control, with 497 mg/dl fasting blood glucose and 15.6% HbA1c. The random migration and neutrophil chemotaxis were significantly reduced. Periodontal treatment and metabolic control of her diabetes resulted in significant improvement in her periodontal condition. No incipient periodontal breakdown was observed around the teeth after 5 years from baseline. CONCLUSIONS: This report proves the efficiency of periodontal therapy in the prevention of future periodontal breakdown in a systemically compromised patient. It seems that in certain individuals who are predisposed to the aggressive forms of periodontitis, clinical and medical examinations and intervention to the systemic condition, in combination with periodontal treatment, are important in the management of these individuals.

Aggressive Periodontitis↗
PubMed2000

Vitamin A deficiency in healthy children aged 6-59 months in Izmir Province of Turkey.

Vitamin A deficiency even at subclinical levels is associated with increased childhood mortality. There have been few studies related to vitamin A status of children in Turkey. The aim of this study was to assess vitamin A status of children aged 6-59 months in Izmir, Turkey, and to evaluate the relationship of these levels with nutritional status. One hundred and sixty children were selected for the study using the cluster sampling method. Serum retinol levels were measured by high-performance liquid chromatography (HPLC) and ranged from 9.8 to 59.2 micrograms/dL (mean 29.3 +/- 9.5 micrograms/dL). Levels were below the lower limit of the normal range in 15.6% of the children. Deficient and marginal serum retinol among stunted children were observed in 16% and 42% respectively. There was a statistically significant relationship between low serum retinol and stunting (P < 0.05). Although xerophthalmia and other clinical signs of vitamin A deficiency are rarely seen, subclinical vitamin A deficiency is a public health problem in Izmir, Turkey.

Child, Preschool↗
PubMed1999

Determination of iodide amounts in urine and water by isotope dilution analysis.

Urinary iodide and iodine in drinking water were determined in 318 healthy children aged 0 to 18 yr living in Izmir and environmental rural and urban areas in the western part of Turkey. The method is based on substochiometric isotope dilution analysis. Iodide was precipitated by substoichiometric amounts of AgNO3. Iodide-131 was used as a tracer. Electrophoresis was performed to separate Ag131I from excess 131I-. The Ag131I zone was cut off the electrophoresis paper and counted with a NaI(Tl) scintillation counter. Count rates were plotted versus added KI concentrations. The unknown iodide amount was found by using these linear plots. Iodide concentration ranges were within 1.8-100.45 micrograms/L in the analyzed drinking water samples. The mean value was 44.14 +/- 17.33 micrograms/L and the median was 58.08 micrograms/L. Urinary iodide concentration ranges were 0.22-142.22 micrograms/L. The median of the distribution was 37.71 micrograms/L and the mean was 40.30 +/- 24.05 micrograms/L. The results show that the examined area suffers moderate iodine deficiency.

Adolescent↗
PubMed1998

Ingested interferon alpha suppresses type I diabetes in non-obese diabetic mice.

Type I diabetes mellitus is a chronic disorder that results from autoimmune destruction of the insulin-producing pancreatic beta cell. The non-obese diabetic mouse is a model of the human autoimmune disease Type I diabetes [1-3]. We have previously shown that ingested type 1 interferon inhibits chronic relapsing experimental autoimmune encephalomyelitis and the adoptive transfer of experimental autoimmune encephalomyelites by T cells, and decreases both antigen-specific and mitogen-induced pro-inflammatory cytokine secretion in this disorder. We therefore tried to determine whether ingested murine interferon alpha inhibits insulinitis and suppresses Type I diabetes mellitus in non-obese diabetic mice. Murine interferon alpha, given daily, decreased islet inflammation and suppressed diabetes. It increased the concanavalin A and ionomycin plus myristic acid palmitic ester-induced production of interleukin 4 and 10 and interferon gamma-secretion in spleen cells from treated mice. Adoptive transfer of unstimulated splenocytes secreting interleukin 4 and interleukin 10 from fed interferon alpha donors suppressed spontaneous diabetes mellitus in recipients. The protective effect of adoptively transferred unstimulated splenocytes shows the presence of ingested interferon alpha-activated regulatory splenic cell populations that may work via increased interleukin 4 or interleukin 10 production. Ingested interferon alpha administered during vulnerable periods in at-risk populations may potentially provide a continuous, convenient, non-toxic and effective treatment for Type I diabetes.

Adoptive Transfer↗
PubMed1998

Effect of enalapril on proteinuria, phosphaturia, and calciuria in insulin-dependent diabetes.

Elevated urinary calcium and phosphate excretion have been observed in children with insulin-dependent diabetes mellitus (IDDM). This may be related to a defect in tubular reabsorption. It is well known that converting enzyme inhibition decreases microalbuminuria and may prevent or retard diabetic nephropathy. We investigated whether enalapril also improves the defect in calcium and phosphate reabsorption. We studied 16 children and young adults (age 12-21 years) with IDDM and persistent microalbuminuria before and during 12 weeks of enalapril treatment. Before treatment microalbuminuria, urinary calcium excretion, and fractional tubular phosphorus reabsorption (TPR) were 153+/-53 microg/min, 5.5+/-0.9 mg/kg per day, and 71.4+/-3.6%, respectively. At the end of the 12th week, microalbuminuria had decreased to 20.3+/-7.9 microg/min and calcium excretion to 3.3+/-0.4 mg/kg per day (P<0.01), while the TPR increased to 80.1+/-3.8% (NS). The renal threshold phosphate concentration increased from 1.8+/-0.15 to 2.92+/-0.23 mg/dl (P<0.01). The fasting serum glucose and hemoglobin Alc levels did not change significantly during the study. Systolic and diastolic blood pressures were 120.4+/-2.2 / 79.3+/-1.4 mm Hg and 110.5+/-1.8 / 71.3+/-0.9 mm Hg before and after 12 weeks, respectively. We conclude that enalapril treatment improves not only microalbuminuria but also abnormal calcium and phosphate excretion in microalbuminuric children with IDDM.

Adolescent↗
PubMed1996

A case of pycnodysostosis with growth hormone deficiency.

Pycnodysostosis is a skeletal dysplasia characterized by short stature. Treatment of pycnodysostosis with growth hormone (GH) has not been reported so far. We describe a case of pycnodysostosis with growth hormone deficiency in addition to low mean insulin-like growth factor 1 (IGF-1) concentration. Complete GH deficiency was determined by two pharmacological provocative tests (insulin and L-dopa). A good height-velocity response was obtained after GH replacement treatment. Pycnodysostosis with GH deficiency and replacement therapy have not been reported previously, to the best of our knowledge.

Child↗
PubMedDate not supplied

Evaluation of bone mineral density in children with diabetes mellitus.

Multiple studies have documented reduction in peripheral bone mass in children with insulin dependent diabetes mellitus (IDDM). In this study, the bone mineral density (BMD) of the lumbar vertebrae (L2-L4) was measured by dual photon absorptiometry in 14 female and 16 male diabetic patients of age 11 to 16 years with varying clinical duration. Twenty three children between 11 to 16 years with normal anthropometric measurements between 10th and 97th percentile and no known history of metabolic bone disease served as a control group. BMD values, weight, height, body mass index, metabolic, biochemical and growth parameters of the study group were compared with those of the control group. BMD (L2 AP 0.732 +/- 0.15 gm/cm2, L2 lateral 0.534 +/- 0.09 gm/cm2 in the study group and 0.812 +/- 0.63 gm/cm2 and 0.619 +/- 0.20 gm/cm2 in the control group) and osteocalcin (10.10 +/- 3.40 ng/ml and 23.12 +/- 2.74 ng/ml in diabetes and control respectively) levels were significantly lower in diabetic patients (p < 0.05, p < 0.01 respectively). Within the study group BMD correlated positively with age but not with the duration of the disease nor with the level of metabolic control.

Absorptiometry, Photon↗
PubMedDate not supplied

Serum lipid and lipoprotein composition in infants of diabetic mothers.

Diabetes mellitus (DM) alters carbohydrate and lipid metabolism to a great extent. This study was planned to determine whether infants of insulin dependent and gestational diabetic mothers have abnormal lipid metabolism. Three groups of newborns were included in the study; group I consisted of 7 infants of diabetic mothers (IDM) with insulin dependent DM (Type 1 DM), group II of 18 infants of gestational diabetic mothers and group III of 20 control neonates whose mothers had no history of DM. Total cholesterol (TC), triglyceride (TG) and high density lipoprotein-cholesterol (HDL-C) values in groups I and II were no different compared to those in group III (p > 0.05). However, low density lipoprotein-cholesterol (LDL-C) and LDL-C/HDL-C ratio were similar between groups I and II (p > 0.05) but significantly higher in both infants of type 1 diabetic mothers and gestational diabetic mothers compared to control infants (p < 0.05). Apolipoprotein A-I (Apo A-1) and apolipoprotein B (Apo B) levels, Apo A-I/Apo B and HDL-C/Apo A-I ratios were similar in between groups. However, Apo B/LDL-C ratio was significantly lower in groups I and II compared to control group (p < 0.05). In conclusion, diabetes in pregnant women causes a tendency to LDL hypercholesterolemia in the offspring. These infants should be longitudinally followed up to assess whether this observation imposes an increased risk for atherosclerosis for advanced ages.

Adolescent↗
PubMedDate not supplied

Maternal and fetal serum insulin-like growth factor-I (IGF-I) IGF binding protein-3 (IGFBP-3), leptin levels and early postnatal growth in infants born asymmetrically small for gestational age.

This study was planned to investigate the relationship between birth weight and insulin-like growth factor-I (IGF-I), IGF binding protein-3 (IGFBP-3), and leptin levels in neonates with normal growth (appropriate for gestational age: AGA) and retarded growth (small for gestational age: SGA); and to evaluate these growth factors' effects in early postnatal growth. All newborns were full-term: gestational age 3,841 weeks. Of 50 neonates, 25 were SGA. IGF-I, IGFBP-3 and leptin levels were measured in maternal serum and venous cord blood at birth and at 15 days of life of neonates using specific RIAs. Maternal serum leptin concentrations were significantly higher than cord blood leptin concentrations (p < 0.001). Maternal serum IGF-I, IGFBP-3 and leptin levels did not show correlations with birth weight. In contrast, there were significantly positive correlations between birth weight and venous cord blood IGF-I, IGFBP-3 and leptin levels (p < 0.001). In the SGA group, the newborns with a slow postnatal growth pattern had lower umbilical cord serum IGF-I levels compared with newborns with a normal growth pattern. A similar result was also found in the AGA group. Similar results were not found for serum leptin and IGFBP-3. In conclusion, cord blood IGF-I, IGFBP-3 and leptin levels play an important role in the regulation of fetal and neonatal growth. It is likely that IGF-I has a more important role than the other factors in early postnatal growth.

Adult↗
PubMedDate not supplied

Familial brachyolmia.

Brachyolmia is characterized clinically by short stature and radiographically by generalized platyspondyly without significant long bone abnormalities. A healthy 13-(8/12) year-old girl was referred for evaluation of short stature. The parents were first cousins. She had two older brothers and a younger brother and sister. On examination, her height was 116.2 cm, height SDS -6.2, armspan 135 cm. She had completed puberty. Except for her short trunk and lower extremities and mild scoliosis, she appeared normal. At 12 years old, the younger brother had short stature. His height was 104.7 cm, height SDS -6.2, armspan 116 cm. The younger sister was 3 years old. Her height was 84.2 cm, height SDS -2.9, armspan 85 cm. Other findings were normal in the younger sister and brother. The other members of the family were of normal stature and appearance. The proband's growth hormone stimulation tests, thyroid function tests, sex steroids, gonadotropins and blood biochemistry were found normal. There were similar radiological findings in the three siblings. There was platyspondyly, narrowing of intervertebral spaces in all vertebral bodies. The iliac bones were broad. No metaphyseal irregularity and normal epiphyses were detected in all patients. No significant changes were seen in long bones and skull. According to the physical and radiological findings, the patients were evaluated as brachyolmia.

Adolescent↗
PubMedDate not supplied

A Turkish family with 6.7 Kb deletion associated with isolated growth hormone deficiency type 1A.

Familial growth hormone deficiency type 1A is an autosomal recessive disease caused by homogenous deletions of both alleles of growth hormone gene 1 (hGH1) in various patterns. The hGH1 gene deletion is an event that probably occurs between the 5' and 3' flanking regions by unequal recombination, and results in deletion of the hGH1 gene in different patterns. Deletions are mostly 6.7 kb and rarely 7.0 kb, 7.6 kb and 45 kb in size. A four-year-old girl diagnosed with growth hormone deficiency syndrome was send to us for further evaluation. DNA samples of the patient, her parents and controls were amplified by polymerase chain reaction (PCR); furthermore, restriction endonuclease analysis was done with Sma I enzyme and the patterns were evaluated. Our gel electrophoresis results show that the gene deletion pattern of the patient represents a homogenous 6.7 kb deletion, while her parents had a heterogeneous 6.7 kb deletion pattern.

Child, Preschool↗