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Biomedical subjects

Ozgur Pirgon

Publications and source records attributed to Ozgur Pirgon.

At least 19 recordsLinked to original sources

Psoriasis following growth hormone therapy in a child.

OBJECTIVE: To report a case of psoriasis that developed following growth hormone treatment. CASE SUMMARY: An 8-year-old boy was admitted to our pediatric endocrinology department because of short stature (<3rd percentile). A dopamine stimulation test and an insulin tolerance test revealed growth hormone (GH) deficiency with a normal cortisol response. His insulin-like growth factor 1 (IGF-1) level (9 ng/mL; reference range 113-261) was under the limits for his age and sex. Six months after the initiation of treatment with recombinant human GH 0.33 mg/kg/wk, the patient presented with a 10 day history of desquamation and a burning sensation in his right knee and hip. Lesions of erythematous papules consistent with plaques of psoriasis were present. Histologic findings from skin biopsies were consistent with psoriasis. The GH dose was reduced to 0.2 mg/kg/wk and treatment for psoriasis (including hydrocortisone and clemastine) was started. Three months after those interventions, the plaques had resolved. DISCUSSION: Previous studies proposed that the extent and severity of psoriasis correlate with GH levels, although psoriatic patients, in general, have normal GH and IGF-1 levels. The Naranjo probability scale indicated that the development of psoriasis was probably associated with GH therapy. CONCLUSIONS: This case suggests that the development of psoriasiform lesions in a previously unaffected individual represents an adverse effect of GH treatment, occurring at higher doses, with higher IGF-1 levels.

Child↗

Relation of in utero lead exposure with insulin-like growth factor-I levels and neonatal anthropometric parameters.

Our aim was to establish whether independent relationships exist between either anthropometric parameters or insulin-like growth factor-I (IGF-I) and cord blood lead levels in newborns. Umbilical cord blood samples and anthropometric data were obtained at delivery. Cord blood lead levels were analyzed by atomic absorption spectrophotometer. IGF-I levels were measured using RIA. Blood lead levels >or=100 microg/l were considered elevated in accordance with CDC guidelines. Data on all variables of interest were obtained for 54 term neonates. The mean cord blood lead level was 144+/-89 microg/l with a range of 51-355 microg/l. Twenty-nine (53.7%) neonates had blood lead levels >or=100 microg/l. However, only 5 (9.2%) of the neonates had blood lead levels of >or=250 microg/l. No statistically significant difference was found in case of anthropometric parameters and IGF-I levels between neonates with high lead levels (>or=100 microg/l) and with low lead levels (<100mug/l) in cord blood (p>0.05). There was a significant correlation between lead and birth weight in neonates with high lead levels (r: -0.49, p=0.01) but not other anthropometric data and IGF-I levels. A multivariate regression analysis using the full range of lead values adjusted for the relevant confounders such as gestational age and socioeconomic status was performed. In the model birth weight (p: 0.01, beta: -0.81), birth length (p: 0.05, beta: 0.41) and midarm circumferences (p: 0.05, beta: 0.30) were the best predictors of lead levels, with the total variance explained being 36%. Significant relationship was found between birth weight and lead burden in newborns. Whether serum lead level predicts the development of failure to thrive in these children remains to be determined in follow-up studies.

Biomarkers↗

Unusual cardiac features in cholestatic hepatitis A in an adolescent: improvement with corticosteroid treatment.

Hepatitis A is one of the most frequent infectious liver diseases affecting children worldwide. Extra-hepatic manifestations of acute hepatitis A virus are rare in pediatric age group. We report a case of a 16-year-old, otherwise healthy adolescent who had viral hepatitis A with cardiac manifestations. The patient is the first pediatric case of hepatitis A with a combination of hypotension, persistent bradycardia and progressive cholestasis.

Acute Disease↗

Arterial wall thickening and stiffening in children and adolescents with type 1 diabetes.

OBJECTIVE: We evaluated structural and functional characteristics of the common carotid artery (CCA), a marker of early carotid atherosclerosis, and investigated their relation to metabolic and anthropometric parameters in children and adolescents with type 1 diabetes. MATERIALS AND METHODS: Non-invasive ultrasonographic measurements were made in 45 type 1 diabetic patients and 33 controls. Age, sex, and body mass index were matched between patients and controls. We investigated intima-media thickness (IMT), compliance, distensibility, diastolic wall stress (DWS) and incremental elastic modulus (IEM) of the CCA. Metabolic and anthropometric parameters such as serum lipids, plasma glycated haemoglobin, body mass index, waist-hip ratio and blood pressure were assessed. RESULTS: The diabetic patients had significantly higher CCA-IMT than the controls (0.48+/-0.06mm versus 0.33+/-0.07mm; p<0.001). The diabetic children had significantly higher values than the controls for DWS (1.18+/-0.29mmHgx10(2) versus 0.81+/-0.25mmHgx10(2); p<0.001) and for IEM (1.26+/-0.57mmHgx10(3) versus 0.77+/-0.28mmHgx10(3); p<0.001). The difference was not significant between patients and controls for arterial compliance and for distensibility (p>0.05). In a multivariate regression model for all subjects, diabetic state was the best predictor of IMT (p<0.001), DWS (p<0.001) and IEM (p=0.001). CONCLUSION: Our results suggested that children and adolescent patients with type 1 diabetes are associated with early impairment of CCA structure and function and that diabetic state may be the main risk factor for CCA wall stiffening and thickening, which are of considerable concern as possible early events in the genesis of atheroma.

Adolescent↗

A case of Perlman syndrome presenting with hemorrhagic hemangioma.

The presence of enlarged, echogenic kidneys in a newborn with generalized macrosomia points toward diagnosis of an overgrowth syndrome. These include Beckwith-Wiedemann, Perlman and Simpson-Golabi-Behmel syndromes. Perlman syndrome is an autosomal recessively inherited overgrowth syndrome characterized by fetal gigantism, visceromegaly, unusual face, bilateral renal hamartomas with nephroblastomatosis, and Wilms tumor. We report a male infant who exhibits typical features of Perlman syndrome with an unusual presentation. In this report, it is emphasized that hemangioma in neonatal period may be an unusual finding in Perlman syndrome.

Abnormalities, Multiple↗

Possible effect of leptin on renal magnesium excretion in adolescent patients with type 1 diabetes.

BACKGROUND: Hypomagnesaemia and hyperleptinemia are common in patients with diabetes. Moreover, it has been demonstrated that leptin stimulates diuresis and natriuresis. The aim of this study was to evaluate the relationship between serum leptin, serum magnesium (Mg) and urinary Mg/urinary creatinine levels in patients with type 1 diabetes. METHODS: Serum leptin and Mg and urinary Mg/urinary creatinine levels were measured in 67 patients with diabetes (33 girls and 34 boys). The age, diabetes duration, anthropometric and metabolic parameters of the subjects were matched between girls and boys. The relation of serum leptin levels to serum and urinary Mg/urinary creatinine levels was assessed. RESULTS: Serum leptin levels of girls with diabetes were higher than those of the boys (14 +/- 5.3 microg/L vs 5.8 +/- 1.5 microg/L, P < 0.001, respectively). The differences for serum Mg and for urinary Mg/urinary creatinine levels were not significant between girls and boys with diabetes. Leptin levels were correlated with urinary Mg/urinary creatinine levels in both girls and boys (r = 0.39, P = 0.02 and r = 0.37, P = 0.03, respectively). In a multivariate regression model, leptin emerged as independent correlates for mean urinary Mg/urinary creatinine in both girls and boys with the total variance explained being 14%, and 15%, respectively. CONCLUSION: The data suggest that serum leptin might be related to increased urinary Mg loss in patients with type I diabetes.

Adolescent↗

Metabolic syndrome manifestations in Cohen syndrome: description of two new patients.

We report on sporadic cases that have the proposed diagnostic criteria for Cohen syndrome associated with metabolic syndrome. The patients, aged 14 and 16 years, had truncal obesity, mild mental retardation, hypotonia, narrow hands and feet, a high-arched palate, prominent upper central incisors, a high nasal bridge, and ocular abnormalities. Both had impaired glucose tolerance, with marked hyperinsulinemia exhibited by an oral glucose tolerance test. Moreover, they had the other metabolic syndrome features of hyperlipidemia and hypertension. We suggest that it is necessary to consider the possibility of metabolic syndrome in a case of Cohen syndrome.

Adolescent↗

Proton magnetic resonance spectroscopy findings and clinical effects of montelukast sodium in a case with Sjögren-Larsson syndrome.

Sjögren-Larsson syndrome is a rare hereditary metabolic disorder characterized by congenital ichthyosis, mental retardation, and spastic diplegia or tetraplegia. This genetic disease is caused by fatty acid aldehyde dehydrogenase deficiency, leading to an accumulation of long-chain alcohols. The role of enzyme in the degradation of leukotrienes paved the way to the development of a new therapeutic strategy for Sjögren-Larsson syndrome, leukotriene antagonists. We describe a 3-year-old boy with Sjögren-Larsson syndrome who had a lipid peak on proton magnetic resonance spectroscopy despite normal findings on cerebral magnetic resonance imaging. He benefited from treatment with montelukast sodium, especially with respect to the agonizing pruritus.

Acetates↗

Some effect of metformin on insulin resistance in an infant with leprechaunism.

Leprechaunism was first recognized in 1954 and is characterized by severe intrauterine and postnatal growth retardation, failure to thrive, lipoatrophy, dysmorphic features (globular eyes, large ears, and micrognathia), hirsutism and acanthosis nigricans. The presented infant, a 30 day-old boy, had multiple phenotypic anomalies, including low-set ears, prominent eyes, decreased subcutaneous fat, hirsutism, breast hyperplasia, and penile enlargement. We found persistent hyperglycemia with remarkably high immunoreactive insulin levels. His phenotypic and laboratory features were consistent with a diagnosis of leprechaunism. We observed some effect of treatment with metformin but not with insulin glargine.

Abnormalities, Multiple↗

Oral alendronate therapy for severe vitamin D intoxication of the infant with nephrocalcinosis.

Vitamin D intoxication is a well-known cause of hypercalcemia in children. We report here the use of oral alendronate for the treatment of hypercalcemia due to vitamin D intoxication in a 7 month-old infant with nephrocalcinosis. The serum calcium levels were normalized without complications. Oral alendronate therapy may be safely used in hypercalcemia due to vitamin. D intoxication in pediatric patients with nephrocalcinosis resistant to hydration, diuretics or corticosteroids.

Administration, Oral↗

The role of cord blood IGF-I levels in preterm osteopenia.

OBJECTIVE: Osteopenia is a frequent condition in preterm infants. This prospective study was designed to assess the relationship between cord blood insulin-like growth factor-I (IGF-I) levels and bone mineralization in healthy premature infants. METHODS: Twenty preterm infants (ten males and ten females) were studied. We determined the bone mineral density (BMD) and bone mineral content (BMC) of healthy premature infants by dual-energy X-ray absorptiometry and also studied the correlation between IGF-I and other parameters and the influence of cord blood IGF-I concentrations on bone mineralization in these infants. RESULTS: The mean concentration of IGF-I was 13.6 +/- 16.9 ng/ml and BMD and BMC were 0.249 +/- 0.06 g/cm2 and 3.09 +/- 1.18 g, respectively. Cord serum levels of IGF-I had significantly positive correlations with BMD (r = 0.605, p = 0.008), but not BMC (r = 0.242, p = 0.367). In stepwise regression analysis, IGF-I emerged as a significant predictor of BMD (beta = 0.595, p = 0.015) contributing to 35.4% of its variability. CONCLUSION: We found a relationship between cord blood IGF-I and BMD in preterm neonates, suggesting that even within an unremarkable population, IGF-I might be important to ensuring bone health.

Bone Density↗

Plasma homocysteine levels in children and adolescents with type 1 diabetes.

OBJECTIVE: Hyperhomocysteinemia has been established as a risk factor for cardiovascular disease. The objective was to investigate total plasma homocysteine concentrations in children and adolescents with type 1 diabetes and a control group. METHOD: Twenty-seven children with type 1 diabetes and 27 subjects of an age- and sex-matched control group were recruited. Fasting samples were collected for plasma total homocysteine, serum vitamin B12, folate, and creatinine. RESULTS: Fasting total homocysteine concentrations showed no difference between patients and controls (5.6 +/- 2.9 micromol/L vs 5.7 +/- 2.2 micromol/L; p greater than 0.05). The diabetic patients had significantly higher serum folate than the healthy controls (11.4 +/- 3.3 ng/mL vs 9.4 +/- 4.1 ng/mL; P = 0.02 and higher serum B12 than the control group (282.8 +/- 119 pg/mL vs 228.5 +/- 50.9 pg/mL; P = 0.03). Total plasma homocysteine concentration correlated with age (r = 0.44, P = 0.02), weight (r = 0.56, P = 0.002), body mass index (r = 0.57, P = 0.002), folate (r = -0.48, P = 0.01), and creatinine (r = 0.41, P = 0.03) in diabetic patients. In stepwise multivariate regression model for diabetics, the independent correlates for total plasma homocysteine concentration was folate (P = 0.002). CONCLUSION: We concluded that fasting plasma total homocysteine concentrations were within normal limits in children and adolescents with type 1 diabetes who were without any clinical evidence of microvascular and macrovascular complications.

Adolescent↗

Prevalence of metabolic syndrome in obese Turkish children and adolescents.

OBJECTIVE: The aim of our study was to assess the prevalence of metabolic syndrome and the other metabolic features in obese children. METHODS: We have studied 169 obese children and adolescents (body mass index>95th percentile), 100 prepubertal and 69 pubertal, aged between 7 and 18 years. Each subject was submitted to an oral glucose tolerance test. The diagnosis of impaired glucose tolerance, type 2 diabetes and metabolic syndrome were defined according to modified WHO criteria adapted for children. RESULTS: Metabolic syndrome was found in 27.2%, with a significantly higher rate among adolescents aged 12-18 years (37.6%) than among children aged 7-11 years (20%) (p < 0.001). There were no significant differences in the prevalence of metabolic syndrome by sex. The prevalences of insulin resistance, glucose intolerance and type 2 diabetes were 29, 19 and 2% among prepubertal children and 56.5, 27.5 and 4.3% among pubertal group, respectively. The prevalence of fasting hyperinsulinemia in pubertal group was significantly higher than prepubertal children (p < 0.001). Hyperinsulinemia was also more frequent in pubertal children with significant difference (20% versus 43.7%, p < 0.001). Hypertension was significantly more common in adolescents (31.8%) than children (15%) with obesity, as expected (p < 0.013). Overall, dyslipidaemia in prepubertal and pubertal groups was identified in 42 and 55%, respectively, with no significant differences (p = 0.085). CONCLUSIONS: Type 2 diabetes mellitus and metabolic syndrome prevalences among adolescents are quite high in the urban area of Konya, central Anatolia, with abnormal lipid profiles, obesity and nutritional mistakes.

Adolescent↗

Zimmermann-Laband syndrome in an infant with an atypical histologic finding.

Zimmermann-Laband syndrome is an inherited disorder that is characterized by abnormalities of the head, face, hands, and feet. Most children with this disorder have generalized hypertrichosis, large gingivae, and hypoplasia of the fingernails and toenails. We report a male infant who exhibited typical features of Zimmermann-Laband syndrome with an unusual histopathologic finding. Excised tissue from the infant's gingivae showed papillary projections that were composed of hyperplastic stratified squamous epithelium with different amounts of keratinization.

Abnormalities, Multiple↗

Serum magnesium concentrations in type 1 diabetic patients: relation to early atherosclerosis.

Hypomagnesemia and sub-clinical atherosclerosis are common in type 1 diabetic patients, and are especially common in poorly controlled and chronically treated diabetics. The aim of this study was to evaluate the relationships between serum magnesium (Mg) and intima-media thickness (IMT), and functions of common carotid artery (CCA), accepted as markers of early carotid atherosclerosis in type 1 diabetic patients. Serum magnesium levels were measured in 51 diabetic patients (26 girls and 25 boys). Age, duration of diabetes as well as major cardiovascular risk factors, including anthropometric and metabolic parameters were matched between girls and boys. CCA structure and functions were measured by ultrasonography as IMT, cross-sectional compliance (CSC), cross-sectional distensibility (CSD), diastolic wall stress (DWS), and incremental elastic modulus (IEM). The difference for serum Mg was significant between diabetic girls and boys (0.75+/-0.09 mmol/l versus 0.86+/-0.14 mmol/l; p=0.02). Mg levels were correlated with IMT (r=-0.58, p=0.004) and DWS (r=-0.49, p=0.01) in girls and with IMT (r=-0.55, p=0.007) and DWS (r=-0.46, p=0.03) in boys. In a multivariate regression model, Mg, systolic blood pressure and waist-hip ratio emerged as independent correlates for mean IMT in girls with the total variance explained being 63%, and Mg and triglycerides emerged as independent correlates for mean IMT in boys with the total variance explained being 68%. Mg did not emerge as independent correlates for mean DWS in both girls and boys. Serum magnesium levels in children and adolescent patients with type 1 diabetes are associated with early atherosclerosis.

Adolescent↗