Yasemin Akın

Hypospadias in Istanbul: Incidence and risk factors

Background:  The aim of the present prospective study was to determine the incidence of hypospadias in newborns in one of the busiest teaching hospitals of Istanbul, and to investigate the risk factors.

Subacute Sclerosing Panencephalitis Presenting as Acute Disseminated Encephalomyelitis

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistant measles virus infection of the central nervous system. In majority of cases onset occurs from 5–15 years of age.1 In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years.2 SSPE generally occurs 5–10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, meales antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum, and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically beddiverse and a thorough differential diagnosis is mandatory for a definite diagnosis.

Effectiveness of a sleep education program for pediatricians

The high prevalence of sleep problems in children and long‐term consequences point to the need for early effective interventions, but health‐care providers have limited training in pediatric sleep medicine. The aims of this study were therefore to assess the effectiveness of a sleep health‐care education program and to develop a Turkish acronym for brief sleep history taking for pediatric primary caregivers in the ambulatory setting.

Reliability and validity of the Bladder and Bowel Dysfunction Questionnaireamong Turkish children

Background/aim: We developed a Turkish version of the Bladder and Bowel Dysfunction Questionnaire (BBDQ) and evaluated its psychometric properties among Turkish pediatric patients.Materials and methods: The BBDQ was translated into Turkish and then it was back-translated into English. A total of 193 patients were asked to complete the Turkish version of the BBDQ as well as the Dysfunctional Voiding and Incontinence Scoring System (DVISS). In addition, 39 children completed the same questionnaires twice at 2-week intervals for test/retest evaluation.Results: Cronbachs alpha coefficient of the BBDQ was 0.727. Reliability of the test/retest was 0.759 (P < 0.001). Area under the curve of the receiver operating characteristic plot was 0.765. There were statistically significant differences in BBDQ scores between the controls and patients (P < 0.001). Analysis demonstrated moderate convergent validity against the DVISS (r: 0.78, r2: 0.601, P < 0.0001).Conclusion: The Turkish version of the BBDQ is a reliable and valid instrument for Turkish pediatric patients with bladder and bowel dysfunction in clinical and research settings.

Necrotizing fasciitis of the perineum

BackgroundNecrotizing fasciitis is a severe soft tissue infection characterized by rapidly progressing necrosis, involving subcutaneous tissues. This rare condition carries a high mortality rate and requires prompt diagnosis and urgent treatment with radical debridement and antibiotics; but early diagnosis, which is essential to successful treatment, remains a challenge.MethodsPhysical examination findings, preoperative and operative findings, histopathological results of the structure, and follow-up results of the patient are discussed with related reports.ResultsA 15-month old girl had a history of trivial perineal dermatitis after treatment of anemia and pneumonia. Perineal dermatitis progressed fastly as necrotizing fasciitis which was successfully managed with intensive medical treatment, surgical debridement and reconstructive surgery.ConclusionsLack of cutaneous findings early in the disease makes the diagnosis challenging, so a high suspicion is essential. Recovery of the patient from this life-threatening condition needs a multi-disciplinary approach involving pediatrics, pediatric surgery, and plastic and reconstructive surgery.

Beta-ketothiolaz eksikliği: Olgu sunumu

Beta-ketothiolase deficiency is defect ofketone body and isoleucine metabolism. Mithocondrial acetoacetyl-CoA thiolase is responsible for the division of 2 methylacetoacetyl-CoA in isolasine methabolism, the formation of acetoacethyl-CoA in kethogenese and the division of acetoacetyl-CoA in ketolysis. This case was prepared to emphasize the importance of considering the Beta-ketotiolase deficiency for the diseases causing ketoacidosis which is resistant to the treatment.

Asfiksinin term yeni doğanlarda bilirubin düzeyine etkisi

Objective: In this study we evaluated the effect of perinatal asphyxia on serum bilirubin leves in term newborns. Materials and Methods: 21 term neonates hospitalized due to perinatal asphyxia and 30 healthy term newborns as a control group were included. This study applies a prospective approach and in orderto compare the senan bilirubin levels between asphyxiated and control group, 2nd, 3rd and 4th postnatal day bilirubin levels were obtained in each group. Conclusions: The average highest bilirubin concentration of asphyxiated and healthy newborns was 8.562 mg/dl(1.793) and 9.340 mg/dl (2.241) respectively. We could not find a statistical difference between the two groups. The average highest bilirubin concentration of 8 asphyxiated neonates treated with phenobarbital and the 13 neonates not treated with phenobarbital was 8.775 mg/dl (1.775) and 8.43 mg/dl (1.874) respectively. A significant statistical difference was not found between two groups. When we compared the average highest bilirubin concentration of 13 asphyxiated neonates with meconium stained amniotic fluid [8.977mg/dl(1.844)]: the 8 noeonates without meconium stained amniotic fluid [7.887mg/dl (1.587)] and the control group, we could not find any statistical difference either. Result: As a result, by using these statistical analysis, we decided that the asphyxia did not have any effect on bilirubin metabolism neither to decrease the serum bilirubine level nor to increase hyperbilirunubinemia in term newborns.