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Background: Children with overgrowth syndromes including isolated hemihyperplasia have an increased risk for developing embryonal tumors, particularly Wilms' tumor and hepatoblastoma. Screening with sonography has been suggested as a method of identifying these tumors while they are still at an early stage. Case Presentation: We describe an infant diagnosed with Wilms tumor in whom isolated hemihyperplasia had been overlooked by several physicians prior to the tumor diagnosis. Conclusion: We recommend tumor surveillance for all patients with Beckwith-Wiedemann syndrome and isolated hemihyperplasia at least for the first six years of life since full molecular characterization of every patient is not readily available. [ABSTRACT FROM AUTHOR]

Abstract Pain after Cardiac Surgery (CS) is the most common patient complaint. However the first 48 h after surgery, when patients' pain is the most severe, is generally spent in the Intensive Care Unit (ICU). It is almost impossible for patients to report their pain because of their inadequate level of consciousness in the ICU. Many factors alter verbal communication with patients, such as administration of sedative medications, mechanical ventilation, and patients' changed level of consciousness. This descriptive study was conducted for the purpose of describing the experience of pain in CS patients in the ICU and determining situations that affect their pain. This research was conducted with 300 adult patients at a Ministry of Health Hospital who stayed in a cardiac surgery ICU post-operatively for a minimum of 48 h, had a sternal incision, chest tube, and required mechanical ventilation. The data were collected from the patients in face-to-face interviews by the researchers following transfer from the ICU to the surgical ward within 48 h of transfer. Most patients described their pain as aching (n = 177) and throbbing (n = 154). The presence of chest tubes (n = 95), endotracheal tube suctioning (n = 47), change of dressings (n = 27) and the use of air mattresses (n = 20) were also identified as painful experiences for patients. Based on these results it can be said that CS patients experience pain in the ICU, however they verbalized it with different words and identified different situations that decreased or increased their pain, which shows the subjective and complex nature of pain. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study is to evaluate clinical and radiological features of our cases with congenital muscular dystrophy. Material and Method: The data of cases who were diagnosed with congenital muscular dystrophy in the Division of Pediatric Neurology of Dokuz Eylül University School of Medicine between July 2005 and July 2008 were analysed retrospectively. Results: A total of 13 cases were evaluated. Among the 13 cases, seven (53%) were boys and six (47%) were girls. Seven patients (53%) were in the alfa dystroglycanopathy group, five patients (38%) were in collagen VI related congenital muscular dystrophy group and one patient (9%) was in 'rigid spine' group. The mean age of cases with alfa dystroglycanopathy and collagen VI related congenital muscular dystrophy were 3.57±2.92 (0.5-7) and 10.83±5.67 (1-16) years, respectively. In alfa dystroglycanopathy group, five cases (71%) had eye involvement and six cases (85%) had developmental malformations of the central nervous system. All cases had brainstem hypoplasia and six cases (85%) had cerebellar cysts. In collagen VI related congenital muscular dystrophy group, the main complaints were retardation in motor mile stones, contractures in proximal joints and hyperlaxicity in distal joints. The most important clinical features in the case diagnosed with 'rigid spine' syndrome were severe scoliosis and muscle atrophy. Conclusions: The clinical features of congenital muscular dystrophies are heterogenous and increase in number of cases diagnosed as congenital muscular dystrophy should give an opportunity to determine the incidence of different types of congenital muscular dystrophies in our country. [ABSTRACT FROM AUTHOR]