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Teke M.Y.,Ulucanlar Eye Education and Research Hospital | Balikoglu-Yilmaz M.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Yuksekkaya P.,Ulucanlar Eye Education and Research Hospital | Citirik M.,Ulucanlar Eye Education and Research Hospital | And 3 more authors.
Retina | Year: 2014

PURPOSE:: To analyze the surgical outcomes and retinal redetachment frequency after silicone oil (SO) removal for complex retinal detachment. METHODS:: This institutional-based study included 894 consecutive patients who underwent pars plana vitrectomy with SO endotamponade for complicated retinal detachment. The effects of preoperative best-corrected visual acuity, vitreous base shaving, intraoperative scleral buckling, retinectomy, SO viscosity, duration of SO, and vitreous hemorrhage at the first postoperative week on the risk of redetachment were investigated. RESULTS:: During a mean follow-up of 39.9 months, anatomical success was not achieved in 118 patients (13.2%) after SO removal. On multivariate analysis, risk factors for recurrent retinal detachment included giant retinal tear (adjusted odds ratio [aOR], 12.39; P < 0.001), high myopia (aOR, 2.70; P = 0.011), surgeries without scleral buckling (aOR, 1.97; P = 0.039), inadequate vitreous base shaving (aOR, 117.62; P < 0.001), and vitreous hemorrhage at the first postoperative week (aOR, 12.13; P < 0.001). CONCLUSION:: Retinal detachment etiology, inadequate vitreous base shaving, lack of intraoperative scleral buckling, and vitreous hemorrhage at the first postoperative week after SO removal were significant risk factors for retinal redetachment after SO removal, but preoperative visual acuity, SO viscosity, and duration of SO had no significant effect on redetachment. Copyright © by Ophthalmic Communications Society, Inc.


Korkmaz H.A.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Hazan F.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Dizdarer C.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Tukun A.,Ankara University
JCRPE Journal of Clinical Research in Pediatric Endocrinology | Year: 2012

Hypochondroplasia (HCP) is an autosomal dominant skeletal dysplasia characterized by short extremities, short stature and lumbar lordosis, usually exhibiting a phenotype similar to but milder than achondroplasia (ACP). Fibroblast growth factor receptor 3 gene (FGFR3) mutations in the germline are well-known causes of skeletal syndromes. FGFR3 is a negative regulator of bone growth and all mutations in FGFR3are gain-of-function mutations that lead to skeletal dysplasias. We report a child who presented with short stature, a relatively long trunk, short legs, short arm span, radiographic evidence of HCP and mild mental retardation. Genetic analysis revealed a heterozygous 1620C>G (Asn540Lys) mutation in FGFR3. To our knowledge, ours is the first case report of HCP with a heterozygous 1620C>G (Asn540Lys) mutation in Turkey. ©Journal of Clinical Research in Pediatric Endocrinology.


Korkmaz H.A.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Demir K.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | KIlIc F.K.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Terek D.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | And 3 more authors.
Journal of Pediatric Endocrinology and Metabolism | Year: 2014

Aim: To assess the efficiency of oral desmopressin lyophilisate (ODL) in neonatal central diabetes insipidus (CDI). Methods: The characteristics of four newborns with CDI treated with ODL were evaluated. Results: Four newborns with polyuria and hypernatremia were included [male, 2 (50%); mean postnatal age, 19±17 days]. At the time of hypernatremia, the mean serum and urine osmolality values were 310±16 and 179±48 mOsm/kg, respectively. Antidiuretic hormone levels were undetectable (<0.5 pmol/L) in all cases. Magnetic resonance imaging revealed anatomical malformations in all cases. ODL (60 μg/tablet) dissolved in water (3-5 mL) was initiated with a dose of 5 μg/kg/day in two equal doses, together with limitation of water intake to avoid hyponatremia. Serum sodium levels returned to normal in a mean duration of 58±9.9 h with a mean decline rate of 0.37±0.1 mEq/L/h after desmopressin administration. Rehospitalization was required for one of the infants because of hypernatremia due to non-compliance. No episode of hyponatremia was encountered. Weight gain and growth of the infants were normal during the mean follow-up duration of 8.5±1 months. Conclusions: ODL appears to be practical and safe in the treatment of CDI during the first year of life. © 2014 by De Gruyter.


Dizdarer C.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Anil Korkmaz H.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Murat Buyukocak O.M.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Mohan Taranci S.M.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital | Coban A.,Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
JCRPE Journal of Clinical Research in Pediatric Endocrinology | Year: 2013

Objective: To assess insulin-like growth factor-1 (IGF-1)/IGF-binding protein-3 (IGFBP-3) axis and insulin resistance (IR) and the relationship of these parameters with growth in appropriate for gestational age (AGA) and small for gestational age (SGA) infants at birth and in early infancy. Methods: Postnatal blood samples for measurement of glucose, insulin, IGF-1, and IGFBP-3 were taken from 60 infants (30 AGA and 30 SGA) at birth and at one, three, and six months of age. Both SGA and AGA infants were divided into two groups: growing well and not growing well. Blood glucose, insulin, IGF-1, and IGFBP-3 values were assessed in all infants. Results: Homeostasis model assessment-IR (HOMA-IR) values in well-growing SGA infants in the third and sixth months were found to be higher than in not well-growing SGA infants (3.9±0.8 vs. 1.0±0.3 at 3 months and 3.3±0.9 vs. 2.4±0.9 at 6 months, p<0.05). IGF-1 levels in well-growing SGA infants at 3 and 6 months were found to be higher than those in not well-growing SGA infants (83.80±44.50 vs. 73.50±17.60 ng/mL at 3 months and 95.12±50.74 vs. 87.67±22.91 ng/mL at 6 months, p<0.05). The IGF-1 values were significantly lower in well-growing SGA infants than in well-growing AGA infants (83.80±44.50 vs. 103.31±30.81 ng/mL at 3 months and 95.12±50.74 vs. 110.87±26.44 ng/mL at 6 months, p<0.05). Conclusions: This study demonstrates the effects of accelerated early infant growth on IGF-1/IGFBP-3 axis in SGA-born infants. © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Journal Article | Journal: Central-European journal of immunology | Year: 2016

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon, life-threatening drug reaction. The basic findings are skin rash, multiorgan involvement, and eosinophilia. Most of the aromatic anticonvulsants, such as phenytoin, phenobarbital and carbamazepine can induce DRESS. Herein we report a 14-year-old patient with DRESS syndrome related to carbamazepine use. The patient presented with signs of involvement of the skin, lungs, liver, and microscopic hematuria. Carbamazepine treatment was discontinued; antihistamines and steroids were started. Hyperglycemia, commencing on the first dose of the steroid given, persisted even after the discontinuation of steroids and improvement of other signs. There were no signs of pancreatitis or type 1 diabetes clinically in laboratory tests. Her blood glucose levels were regulated at first with insulin and later with metformin. Within 1 year of follow-up, still regulated with oral antidiabetics, she has been diagnosed with type 2 diabetes. Formerly, long-term sequelae related to drug rash with eosinophilia and systemic symptoms syndrome such as hepatic and renal failure, type 1 diabetes mellitus, Graves disease, autoimmune hemolytic anemia, and lupus have also been reported. However, up to date, no cases with type 2 diabetes have been reported as long-term sequelae. To our knowledge, this is the first case in the literature presenting with type 2 diabetes as long-term sequelae.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Case Reports | Journal: Archivos argentinos de pediatria | Year: 2016

Wolfram syndrome (DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a rare neurodegenerative disorder. Mutations of the WFS1 (wolframin) on chromosome 4 are responsible for the clinical manifestations in majority of patients with Wolfram syndrome. Wolfram syndrome is also accompanied by neurologic and psychiatric disorders, urodynamic abnormalities, restricted joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotrophic hypogonadism in males and diabetic microvascular disorders. There are very limited data in the literature regarding cardiac malformations associated in children with Wolfram syndrome. A 5-year-old girl with Wolfram syndrome and tetralogy of Fallot is presented herein.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Case Reports | Journal: Journal of clinical research in pediatric endocrinology | Year: 2012

Hypochondroplasia (HCP) is an autosomal dominant skeletal dysplasia characterized by short extremities, short stature and lumbar lordosis, usually exhibiting a phenotype similar to but milder than achondroplasia (ACP). Fibroblast growth factor receptor 3 gene (FGFR3) mutations in the germline are well-known causes of skeletal syndromes. FGFR3 is a negative regulator of bone growth and all mutations in FGFR3 are gain-of-function mutations that lead to skeletal dysplasias. We report a child who presented with short stature, a relatively long trunk, short legs, short arm span, radiographic evidence of HCP and mild mental retardation. Genetic analysis revealed a heterozygous 1620C>G (Asn540Lys) mutation in FGFR3. To our knowledge, ours is the first case report of HCP with a heterozygous 1620C>G (Asn540Lys) mutation in Turkey.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Journal Article | Journal: Journal of clinical research in pediatric endocrinology | Year: 2016

The aim of this study was to examine the vitamin D status of children and to determine the factors influencing serum 25-hydroxyvitamin D [25(OH)D] concentration in Turkish infants living in zmir.In this study, we examined the serum 25(OH)D levels of 100 infants aged 1 to 24 months and of 22 mothers from zmir, Turkey. The study also included a questionnaire given to the mothers to acquire data on the demographic characteristics of the infants and their mothers as well as information on vitamin D supplementation, clothing habits, and sunlight exposure.Vitamin D deficiency was present in 31% of infants and 81.8% of mothers. Twenty-four male (42.9%) and 7 female (15.9%) infants were found to be vitamin D deficient (<20 mg/dL); 9 male (16.1%) and 17 female (38.6%) infants to be vitamin D insufficient (20-30 mg/dL); and 23 male (41.1%) and 20 female (45.5%) infants were vitamin D sufficient (>30 mg/dL). Only 63% of the infants were receiving vitamin D supplementation and 52% were said to be having regular exposure to sunlight. Mean serum vitamin D levels were lower in infants whose mothers were dressed according to the culture of traditional covered clothing (44%) compared to those infants whose mothers dressing style provided more exposure to sunlight.We conclude that low exposure to sunlight, inadequate use of vitamin D supplementation, and large family size are factors influencing the vitamin D status of Turkish children living in the inner city of zmir.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Journal Article | Journal: Journal of clinical research in pediatric endocrinology | Year: 2013

To assess insulin-like growth factor-1 (IGF-1)/IGF-binding protein-3 (IGFBP-3) axis and insulin resistance (IR) and the relationship of these parameters with growth in appropriate for gestational age (AGA) and small for gestational age (SGA) infants at birth and in early infancy.Postnatal blood samples for measurement of glucose, insulin, IGF-1, and IGFBP-3 were taken from 60 infants (30 AGA and 30 SGA) at birth and at one, three, and six months of age. Both SGA and AGA infants were divided into two groups: growing well and not growing well. Blood glucose, insulin, IGF-1, and IGFBP-3 values were assessed in all infants.Homeostasis model assessment-IR (HOMA-IR) values in well-growing SGA infants in the third and sixth months were found to be higher than in not well-growing SGA infants (3.90.8 vs. 1.00.3 at 3 months and 3.30.9 vs. 2.40.9 at 6 months, p<0.05). IGF-1 levels in well-growing SGA infants at 3 and 6 months were found to be higher than those in not well-growing SGA infants (83.8044.50 vs. 73.5017.60 ng/mL at 3 months and 95.1250.74 vs. 87.6722.91 ng/mL at 6 months, p<0.05). The IGF-1 values were significantly lower in well-growing SGA infants than in well-growing AGA infants (83.8044.50 vs. 103.3130.81 ng/mL at 3 months and 95.1250.74 vs. 110.8726.44 ng/mL at 6 months, p<0.05).This study demonstrates the effects of accelerated early infant growth on IGF-1/IGFBP-3 axis in SGA-born infants.


PubMed | Dr Behcet Uz Children Disease And Surgery Training And Research Hospital
Type: Case Reports | Journal: Journal of clinical research in pediatric endocrinology | Year: 2013

Maternal hypercalcemia suppresses parathyroid activity in the fetus resulting in impaired parathyroid responsiveness to hypocalcemia after birth. Resultant hypocalcemia may be severe and prolonged and rarely may lead to convulsions. Here, we present a newborn infant admitted to the pediatric emergency department at age two weeks with recurrent tonic convulsions due to asymptomatic maternal hyperparathyroidism and vitamin D deficiency. Physicians should be aware that undiagnosed maternal hyperparathyroidism can cause severe hypocalcemia in the newborn.

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