Chryseobacterium indologenes (C. indologenes) is nonmotile, oxidase-, and indole-positive gram-negative aerobic bacillus. Immunosuppression, comorbidities, use of broad-spectrum antibiotics are known risk factors for C. indologenes-related infections. We report a neutropenic fever caused by C. indologenes in a 16-month-old boy who was treated due to the neuroblastoma. According to the antimicrobial susceptibility test result, he was treated with cephaperazone/sulbactam.
Cases of neurofibromatosis type 1 (NF1)-associated medullary thyroid carcinoma (MTC) or C-cell hyperplasia are rarely associated with other endocrine tumors or cases with a multiple endocrine neoplasia type 2. In these patients, mutations were detected in the NF1 gene but no mutations were detected in the RET gene. Although vandetanib has been shown to improve progression-free survival in adults with advanced MTC, data in pediatric patients are limited. Herein, we report the use and outcome of vandetanib in a pediatric MTC case in which NF1 gene and RET proto-oncogen mutation were identified together.
Background. Hemangiondothelioma is a rare vascular tumor that can occur in the bone. Temporal bone involvement has been reported extremely rare in the literature. Case. Radiological examination of a one-year-old girl who was admitted due to facial paralysis revealed vascular tumor of the temporal bone and Galen vein aneurysm. Pathological examination showed retiform hemangioendothelioma. She was treated with propranolol, prednisolone, vincristine, and endovascular embolization followed by oral sirolimus. With sirolimus treatment, a partial response was obtained first, then the tumor remained stable and sirolimus treatment was discontinued. No progression was observed in the disease after discontinuation of treatment. Conclusion. In this article, a case of hemangioendothelioma originating from the temporal bone is discussed in the light of other case reports in the literature.
Introduction: Neurofibromatosis type 1 (NF 1) is an autosomal dominant neurocutaneous disease characterised by multisystemic involvement, including bone tissue. Deformities and reduced bone mass are the main bone manifestations in NF1. Quantitative computed tomography (QCT) provides true volumetric bone mineral density (BMD) measurement. This study aimed to evaluate bone metabolism parameters and BMD in children with NF1 using QCT. Methods: The data of 52 paediatric NF1 patients (23 female, 29 male) was evaluated retrospectively. We investigated anthropometric measurements, biochemical parameters like total calcium, phosphate, magnesium, alkaline phosphatase, 25-hydroxyvitamin D (25OHD), parathyroid hormone, calcitonin, urinary calcium/creatinine ratio, and QCT parameters like lumbar trabecular and cortical BMD, trabecular area and cortical thickness. Comparisons of gender and puberty status were performed. Results: 25% of patients had skeletal deformities and 42.3% had 25OHD inadequacy (< 20 ng/mL). The frequency of 25OHD inadequacy was significantly higher in pubertal/postpubertal patients than prepubertal patients (61.9% vs. 29.0%, p = 0.019). Trabecular BMD Z-score was < −2.0 in 11.5% of patients; all with low BMD were at the pubertal/postpubertal stage. There was a significant negative correlation between age and trabecular Z-score (r = −0.41, p = 0.003). Mean cortical BMD was statistically similar between the genders and puberty groups. Puberty status, anthropometric Z-scores, and biochemical and QCT parameters were statistically similar between the genders (p > 0.05). Conclusion: Paediatric NF1 patients may present with low BMD and 25OHD inadequacy, especially at puberty. QCT may be a useful tool to evaluate trabecular and cortical bone separately in NF1 patients.
INTRODUCTION: The aim of this study is to describe our institutional experience with pilomatrixoma in children, specifically examining its clinical presentation, associated conditions, radiological and pathological findings and attract attention to differential diagnosis for this tumor. METHODS: The medical records of 52 patients were reviewed retrospectively. RESULTS: There were a total of 62 tumors in 52 children. The median age at excision was 9.5 years old. Tumors were predominantly located in head and neck region (48.4%). One patient had a family history of pilomatrixoma. One patient had Turner Syndrome and one had Tuberous Sclerosis complex. Fifty-four lesions were examined by ultrasonography (USG). Pilomatrixoma was considered in the differential diagnosis in eight patients (15.3%) by a radiologist. DISCUSSION AND CONCLUSION: Pilomatrixoma is one of the most common causes of superficial masses in children. It should be kept in mind for differential diagnosis in children with superficial masses.
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