Lumbar extradural infiltrating angiolipoma: a case report and review of 17 previously reported cases with infiltrating spinal angiolipomas

BACKGROUND CONTEXT: Spinal extradural angiolipomas are rare benign tumors, and most of them are noninfiltrating tumors located in the extradural space. However, there are 17 cases with extradural infiltrating spinal angiolipomas extending into the vertebral bodies or posterior vertebral arches in literature. These 17 tumors are mostly located at the thoracic region, and anterior or in the anterolateral extradural space, and they generally infiltrate only one vertebra. Only two of them are located at the lumbar region, four are mainly located in the posterior extradural space, and three infiltrate more than one vertebra. PURPOSE: To present an exceptional case with infiltrating extradural spinal angiolipoma involving two lumbar segments and mainly located in the posterior extradural space. STUDY DESIGN: A case report. METHODS: A 41-year-old woman with infiltrating spinal angiolipoma was treated by incomplete surgical removal of the tumor. RESULTS: There were no complaints nor recurrence after 18 months follow-up. CONCLUSIONS: Outcome after surgery for spinal angiolipomas is very good overall even in the cases with infiltrating tumors. Although complete removal is certainly preferred, outcomes remained favorable despite incomplete resections.     

Growth references for Turkish children aged 6 to 18 years

Aim: To create up-to-date reference standards for Turkish children, and to compare these with growth standards for US children (CDC 2000 Growth Charts) and with previous local data. Subjects and methods: Height and weight measurements of 1100 boys and 1020 girls were obtained by biannual visits to six schools located in relatively well-off districts of Istanbul city. All children came from well-to-do families and all were healthy. All measurements were made by two trained technicians. The LMS method was used in the analyses. Results: Heights of the boys and girls in all age groups were close to the updated 2000 USA growth references and showed an increase from data on Turkish children born 30 y earlier. Weight values were high compared to reference data on US children and to the older data on Turkish children.

Conclusion: These results indicate that height growth in Turkish school-age children of high socio-economic level conforms to the updated growth data on US children. The data also show a secular upward trend in Turkey. Weight-for-age values indicate an increase in obesity. The results also point to the value of collecting and evaluating local growth data periodically.     

Nocardia transvalensis infection in an immunocompetent patient reported from Turkey

Pulmonary nocardiosis is a rare infection mostly occurs in patients with immunosuppressive conditions. We report an immunocompetent case of pulmonary Nocardia transvalensis from Turkey, presented with bilateral pneumonia and bronchial dilatation treated six months with trimethoprim-sulfamethoxazole.     

Worster-Drought syndrome (congenital bilateral perisylvian syndrome) with posterior pituitary ectopia, pituitary hypoplasia, empty sella and panhypopituitarism: a patient report

Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature.     

Evaluation of glucose intolerance in adolescents relative to adults with type 2 diabetes mellitus

AIM: There is an increasing trend in the prevalence of type 2 diabetes mellitus (DM2) in childhood and adolescence, while positive family history of DM2 and obesity are the most important risk factors. To study the influence of family history and obesity on glucose intolerance in our country was the aim of this study. STUDY DESIGN AND METHODS: A total of 105 children and adolescents aged 10-18 years (mean 13.3 +/- 2.5 years) were included in the study. All children and adolescents were divided into three groups according to positive family history of DM2 and obesity, and an oral glucose tolerance test (OGTT) was performed for all. Prediabetes was defined as impaired glucose tolerance (IGT) and/or impaired fasting glucose (IFG). Insulin secretion and insulin resistance were estimated using the insulinogenic index; and the homeostatic model assessment for insulin resistance (HOMA-IR) and Matsuda index, respectively. RESULTS: The prevalence of prediabetes was 15.2% in the whole group, while it was 25.5% in obese children who also had a positive family history of DM2. The frequency of hyperinsulinism was 57.1% in all groups. Prediabetic children had significant insulin resistance (HOMA-IR 11.5 +/- 7.1 and 4.1 +/- 6.4, respectively, p = 0.034). CONCLUSIONS: Obesity and glucose intolerance are also a problem in developing countries. The risk of prediabetes in children is highest in obese children who also have a positive family history of DM2. There is a need for a lifelong preventive program starting in childhood to avoid DM2 and decrease cardiovascular risk factors     

Reevaluation of growth hormone deficiency during and after growth hormone (GH) treatment: diagnostic value of GH tests and IGF-I and IGFBP-3 measurements

Retesting of patients with growth hormone (GH) deficiency (GHD), especially those with idiopathic GHD, has yielded normalization of the results in several studies. The aim of this study was to reevaluate patients diagnosed as GHD at completion or reconfirm the diagnosis before completion of GH treatment by retesting with provocative tests, and to evaluate the value of IGF-I and IGFBP-3 levels in the diagnosis of GHD. Fifty (33 M, 17 F) patients with GHD (peak GH level <0.46 pmol/l (10 ng/ml]) in two pharmacological tests were retested and IGF-I and IGFBP-3 levels measured. The age of the patients at retest was 15.2+/-5.0 yr. Thirteen of 50 patients (26%) normalized their GH secretion. According to the initial diagnosis, 69% of those with partial GHD (peak GH level 0.32-0.46 pmol/l [7-10 ng/ml]), 43% with isolated GHD, 33% idiopathic and 11% of those with complete GHD (peak GH level <0.32 pmol/l [7 ng/ml]) normalized their GH level at retesting. None of the patients with multiple hormone deficiency and none with small pituitary on MRI normalized GH levels at retest. The sensitivities of IGF-I and of IGFBP-3 were 70% and 67%, respectively, and the specificities were 100%, when peak GH cutoff is taken as 0.46 pmol/l (10 ng/ml) for the diagnosis of GHD. The sensitivities of IGF-I and IGFBP-3 increased to 76.5% and 73.5% when the cutoff level for GHD is taken as 0.32 pmol/l (7 ng/ml). Those patients who normalized their GH levels at retest showed a satisfactory height velocity when GH therapy was discontinued. In conclusion, reevaluation of GH status may also be undertaken while patients are still on treatment as well as at completion of treatment, especially in patients with idiopathic, partial and isolated GHD, by retesting and by IGF-I and IGFBP-3 measurements. Lowering the cutoff level of GH peak at pharmacological tests to 0.32 pmol/l (7 ng/ml) will lower the number of false positive results in the diagnosis of GHD.