Primary spinal cord tumors of childhood: effects of clinical presentation, radiographic features, and pathology on survival

To determine the relationship between clinical presentation, radiographic features, pathology, and treatment on overall survival of newly diagnosed pediatric primary spinal cord tumors (PSCT). Retrospective analysis of all previously healthy children with newly diagnosed PSCT at a single institution from 1995 to present was performed. Twenty-five pediatric patients (15 boys, average 7.9 years) were diagnosed with PSCT. Presenting symptoms ranged from 0.25 to 60 months (average 7.8 months). Symptom duration was significantly shorter for high grade tumors (average 1.65 months) than low grade tumors (average 11.2 months) (P = 0.05). MRI revealed tumor (8 cervical, 17 thoracic, 7 lumbar, 7 sacral) volumes of 98–94,080 mm3 (average 19,474 mm3). Homogeneous gadolinium enhancement on MRI correlated with lower grade pathology (P = 0.003). There was no correlation between tumor grade and volume (P = 0.63) or edema (P = 0.36) by MRI analysis. Median survival was 53 months and was dependent on tumor grade (P = 0.05) and gross total resection (P = 0.01) but not on gender (P = 0.49), age of presentation (P = 0.82), duration of presenting symptoms (P = 0.33), or adjuvant therapies (P = 0.17). Stratified Kaplan–Meier analysis confirmed the association between degree of resection and survival after controlling for tumor grade (P = 0.01). MRI homogeneous gadolinium enhancement patterns may be helpful in distinguishing low grade from high grade spinal cord malignancies. While tumor grade and gross total resection rather than duration of symptoms correlated with survival in our series, greater than one-third of patients had reported symptoms greater than 6 months duration prior to diagnosis

Joubert syndrome: review and report of seven new cases

Joubert syndrome (JS) is an autosomal-recessive disorder, characterized by hypotonia, ataxia, global developmental delay and molar tooth sign on magnetic resonance imaging. A variety of other abnormalities have been described in children with JS, including abnormal breathing, abnormal eye movements, a characteristic facial appearance, delayed language, hypersensitivity to noise, autism, ocular and oculomotor abnormalities, meningoencephaloceles, microcephaly, low-set ears, polydactyly, retinal dysplasia, kidney abnormalities (renal cysts), soft tissue tumor of the tongue, liver disease and duodenal atresia. Even within siblings the phenotype may vary, making it difficult to establish the exact clinical diagnostic boundaries of JS. We review the clinical characteristics of seven cases that fulfill the criteria of JS

Investigation of the effectiveness of algan hemostatic agent in bleeding control using an experimental partial splenectomy model in rats [Deneysel parsiyel splenektomi modelinde sıçanlarda algan hemostatik ajanın kanama kontrolünde etkinliğinin araştırılması]

Objective: Algan hemostatic agent (AHA) is a plant-based hemostatic agent produced in Turkey. Although, there is a great improvement in the hemostatic technologies, more effective hemostatic products are required to be produced. The aim of this study was to demonstrate the efficacy of AHA in a partial splenectomy model in rats. In addition, in this model, postoperative abdominal adhesion was evaluated. Materials and Methods: In this study 5-7 weeks old 64 rats were used. Rats were randomly divided into 8 groups, each consisting of eight rats (4 groups heparinized and 4 groups non-heparinized). Experimental splenectomy was performed and the gauze impregnated with saline was applied to the control group for the hemorrhage control, the gauze impregnated with liquid AHA, gel and powder form of AHA, was applied to the experimental groups. Results: The time to reach complete homeostasis was significantly shorter in all AHA groups compared to the control group. The powder and the gel forms of AHA stopped the bleeding in heparinized and non-heparinized groups in 1 second. The AHA fluid (sponge) form stopped the bleeding in the first application in the control group less than 10 seconds and the second time application was not necessary. The bleeding was able to be controlled in the heparinized control group (saline impregnated sponge) by 55 seconds and in the non-heparinized control group by 38 seconds. Conclusion: This study showed that AHA is a highly effective hemostatic agent, which would be beneficial in controlling hemorrhage. © 2019, Marmara University. All rights reserved.This study was supported by the Algan Group Health Services (Algan Group Health Services Import and Export Industry and Trade Limited Company, Istanbul, Turkey). The company provided the study materials for the scientists, but had no role for the study design, data collection and analysis. The decision to publish and the preparation of the manuscript also belong to the authors

Guillain-Barre syndrome associated with Legionnella infection

This is the first report of Guillain-Barre syndrome (GBS) related to Legionnella pneumophilia infection. A 13-year-old boy presented with acute dysphagia and dyspnea. He lived in a rural area and had a history of drinking potable deep-hole water. The patient was intubated because of increased respiratory distress. A positive direct fluoresein antigen test confirmed L. pneumophilia infection in BAL. One week after the first admission, acute weakness was noticed including the lower extremities and was more prominent in the distal than the proximal portions. GBS was considered as the initial diagnosis. Tests for all causes known to trigger GBS were negative. Specific serology for L. pneumophilia IgG was positive. He was treated with intravenous immunoglobulins and discharged with minor weakness and difficulty in walking in the second month. On the basis of this case, L. pneumophilia should be included in the etiologic spectrum of GBS

Bone mineral density and bone metabolic markers' status in children with neurofibromatosis type 1.

AbstractBackground:Neurofibromatosis type 1 (NF1) is a multisystem disorder characterized by progressive manifestations, which is inherited in an autosomal dominant manner. The majority of patients with NF1 experience a diffuse, significant reduction in bone mass over time, with osteoporosis, osteopenia in the absence of severe scoliosis, or gross bone deformities. This study aimed to determine the bone mineral density (BMD) status, evaluate bone metabolism, and to determine the relevant factors in children with NF1.Methods:The study population included 33 pediatric NF1 patients (20 males and 13 females). Bone metabolic markers, such as total calcium, phosphorus, magnesium, alkaline phosphatase, parathyroid hormone, and 25-OH vitamin D, the urinary calcium/creatine ratio were measured. In addition, BMD was measured at both the lumbar spine (LS) and the femoral neck in all the patients.Results:All the patients had a low 25-OH vitamin D level, but it was significantly lower in the females than in the males (p&lt;0.009). Overall, 18.2% of the patients had skeletal abnormalities. The lumbar Z-score was ≤2 in 21.2% of the patients, whereas the femoral neck Z-score was ≤2 in 9.1%. The urinary calcium/creatine ratio was significantly higher in the female than in the male patients (p&lt;0.027). In all, six patients had skeletal abnormalities.Conclusions:It is widely known that bone mineral metabolism markers and BMD are significantly affected in NF1 patients; however, the present study did not identify any effective parameters that could be used to predict skeletal abnormalities, or diagnose early osteoporosis and osteopenia in pediatric NF1 patients.</jats:sec