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Öğe The association of common atrium with smith-lemli-opitz syndrome in an infant(2008) Sert A.; Pirgon Ö.; Atabek M.E.; Dogan M.Smith-Lemli-Opitz syndrome is a rare syndrome presenting with multiple congenital anomalies/mental retardation associated with low plasma cholesterol levels. The spectrum of severity extends from prenatal death with holoprosencephaly or other lethal malformations, to patients with minimal physical abnormalities and normal intelligence or minimal intellectual impairment. Congenital heart defect is found in half of the Smith-Lemli-Opitz syndrome patients. To our knowledge, the association of common atrium and Smith-Lemli-Opitz syndrome has not been described before in the medical literature. We present a 4-month-old infant case of such association.Öğe Bacteremia Following Exchange Transfusion in Infants(2003) Atabek M.E.; Koc H.; Pirgon O.; Erkul I.The hypothesis is that transient bacteremia following exchange transfusion in infants may occur; however, it is not associated with clinical evidence of disease. Therefore, the objective of this study is to evaluate the significance of this bacteremia in terms of clinical evidence of infection. We studied 155 patients with jaundice requiring an immediate exchange transfusion (ET). Blood cultures were taken before, soon after completion of the procedure and at 24h later respectively. The clinical course of each patient after the procedure was monitored in terms of evidence of sepsis for minimum of five days. One hundred twenty of the 155 patients underwent a single ET whereas 35 patients required a second ET within 8 to 12 hours. Blood cultures obtained before ET (preexchange specimen) and at 24h later were sterile in all patients. Eighteen (11.6%) patients had positive blood cultures soon after completion of the procedure. Eleven of 120 (9.1%) patients with a single ET had positive blood cultures. Seven of 35 (20%) patients with the second ETs had positive blood cultures. There was a significant statistical difference in terms of bacteremia between the group of single ET and the group of second ETs. (p<0.001). The incidence of requirement for clinical infection was 1 out of 18 (5.5%) infants who developed bacteremia. Bacteremia resolved spontaneously in the majority of infants. However 1 in 18 (5.5%) of the bacteremic infants did develop symptoms and required treatment.Öğe Öğe Papillon-Lefevre Syndrome [Papillon-Lefevre Sendromu](2007) Pirgon Ö.; Atabek M.E.; Sert A.Papillon-Lefevre Syndrome is an extremely rare genetic disorder that typically effects infants of approximately one to 5 years of age. Papillon-Lefevre Syndrome is characterized by the development of palmar-plantar hyperkeratosis and early loss of the primary (deciduous) and permanent teeth due to rapidly progressive periodontopathy. The primary (deciduous) teeth frequently become loose and fall out by about five years of age. In the general population, the disorder occurs in approximately one to 4 individuals per 1.000.000. Here we present a Papillon-Lefevre Syndrome case, which is rarely seen, with a review of the literature.Öğe Prevalence of hepatitis A, B, C and E virus in adolescents with type-1 diabetes mellitus(Freund Publishing House Ltd, 2003) Atabek M.E.; Kart H.; Erkul I.The hypothesis for this study was that hepatitis virus infection could be associated with diabetes, because of the high frequency of injections. In this study, we aimed to determine the prevalence of hepatitis A, B, C and E viruses in type-1 diabetes mellitus. Methods: Sixty-three patients with the diagnosis of insulin-dependent diabetes mellitus and 63 healthy controls were included in this study. Serological markers of four different types of hepatitis (Anti-HAV IgM, total anti-HAV, HbsAg, anti-HBs, total anti-HBc, antiHBc IgM, anti-HCV and anti-HEV) were studied in all cases. None of the patients had a history of previous icterus or other signs of hepatitis, had received blood transfusions, or were on hemodialysis. Results: There was no difference between the patients and controls with respect to hepatitis A, B, C and E virus serology. The rate of seropositivity of patients within a month of the diagnosis was smaller than those of the patients whose diagnosis were older than one month, but the difference was not found to be statistically significant (p>0,05). Moreover, hepatitis virus infections did not seem to be related to duration of disease, nor age of onset, ketoacidosis, HbA1c, and insulin regimen. All of the sera were negative for anti-HCV in both patients and controls. Conclusion: Serological evidence of previous HAV, HBV, HCV and HEV infections was not significantly different between type-1 diabetes patients and healthy controls. Hepatitis virus infection was not associated with diabetes in spite of the high frequency of injections.Öğe Re: "Diabetes mellitus in Asian Indian children and adolescents", Nandkeoliar MK et al., JPEM 2007; 20: 1109-1114 [3](2008) Atabek M.E.[Abstract not Available]Öğe Re: "Effect of metformin and rosiglitazone in a prepubertal boy with Alström syndrome", Sinha SK et al., JPEM 2007; 20: 1045-1052 [5](2008) Atabek M.E.[Abstract not Available]Öğe Resuscitation of the newly born infant [Yenidoc?an resüsitasyonu](2004) Pirgon M.O.; Atabek M.E.Resuscitation of the newly born infant presents a different set of challenges than the resuscitation of the adult or even the older infant or child. Approximately 5-10% of the newly born population require some degree of active resuscitation at the birth. More than 1 million neonatal deaths from birth asphyxia occur worldwide each year. The need for resuscitation of the newly born infant often can be predicted allowing the selection of opportunities for an optimal setting and trained personnel.Öğe Two new Kabuki cases of Kabuki make-up syndrome(2006) Sert A.; Atabek M.E.; Pirgon Ö.Kabuki syndrome (Kabuki make-up syndrome, Niikawa-Kuroki syndrome) is a multiple congenital anomaly/mental retardation syndrome. We report on an 11-month-old girl with Kabuki make-up syndrome who has premature telarche, premature pubarche and epilepsy, and a 4-month-old boy with Kabuki make-up syndrome who had been operated due to diaphragmatic hernia and had mesocardia confirmed by echocardiography. In the study, we emphasize that careful phenotypic examination of children should be performed in every patient presenting with mental retardation and epilepsy to diagnose Kabuki syndrome and the patients diagnosed as Kabuki syndrome should be followed for precocius puberty. We suggest that mesocardia, which has not been reported in the literature yet, may be considered as one of the cardiological findings of Kabuki syndrome and all Kabuki patients should be evaluated for life-threatening complications of congenital diaphragmatic hernia.Öğe Unusual cardiac features in cholestatic hepatitis A in an adolescent: Improvement with corticosteroid treatment(2007) Atabek M.E.; Pirgon O.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. © 2006 The British Infection Society.
