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İnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, Kamer S, Yaman Y, Kundak S, Vergin C. Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016; 58: 349-355. This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system, that predominantly affects the spinal cord and the optic nerve. Its key features include transverse myelitis, commonly associated with extensive inflammation spanning three or more consecutive vertebral segments. Longitudinal extensive spinal cord lesions can also occur in systemic autoimmune diseases, infections, vascular and metabolic disorders, subsequent to irradiation, intramedullary tumors and paraneoplastic myelopathies. We present a case study of an 8-year-old girl seropositive for antibodies against the aquaporin 4 who displayed longitudinal extensive spinal cord lesions, that was initially misdiagnosed as an intramedullary tumor. [ABSTRACT FROM AUTHOR]

Despite the relatively recent introduction of propranolol in the treatment of infantile hemangiomas, there can be little doubt of its efficacy. With regard to safety issues, there are no prior data for very low weight infants. In this study, we used propranolol in preterm and very low weight infants. We used clinical criteria to assess the response to the therapy. We noted all side effects expected from beta-adrenergic blocking drugs, and followed the patients' weight gain during propranolol treatment. Objective, clinical evidence of hemangioma regression was seen after two months in all patients. None of the patients required treatment discontinuation due to adverse side effects. During the propranolol treatment, weight gain was normal in all patients. To the best of our knowledge, this is the first report on the use of propranolol in preterm and very low weight infants, and also the first report from Turkey on the use of propranolol in infantile hemangiomas.

Possible clinical relevance of Nm23 expression, angiogenesis and proliferative activity were evaluated as prognostic parameters in childhood embryonal rhabdomyosarcoma (RMS). Specimens of 25 RMS cases were studied for Nm23 antigen immunohistochemically. Vascular surface density (VSD) and number of vessels per stroma (NVES) calculated by stereologic methods on labeling sections with CD34 antibody. For evaluation of proliferative activity of tumors, mitotic figures and Ki67 positive cells were investigated. All findings were searched statistically. Five patients were stage 1 (20%), two were stage 2 (8%), 15 were stage 3 (60%) and three were stage 4 (12%). The mean event free survival (EFS) was 20.8 and the mean overall survival (OS) was 25.9 months. Sixteen patients (64%) were alive and without disease. The percentage of Nm23 positivity was 52%. Log rank analysis showed Nm23 as a predictor for survival (p=0.0313). In Pearson correlation analysis, there was statistical significance between OS and presence of Nm23 expression (p=0.044). VSD was also positively related with EFS (p=0.040). Despite the present parameters in use, there is a need for new prognostic markers, especially to predict the outcome of patients. These findings suggested that Nm23 expression and VSD might be useful for follow-up in RMS.

Stool specimens taken from 50 children with malignancy and from 92 healthy children were investigated for intestinal parasites, using the modified formol ethyl acetate concentration method, and native-lugol, trichrome and Kinyoun acid-fast stain methods. Thirty-eight (76.0%) of the 50 patients had lymphoma or leukemia and were considered immunosuppressed. Several different parasites were found in 21 (42.0%) of the 50 patients with malignancy and in 16 (47.3%) of the 38 patients with immune deficiency compared to in only 16 (17.3%) of the 92 healthy children. The incidence of parasites in patients with malignancy or immunosuppression was significantly higher than in the healthy control group (p<0.01, p<0.01).