Emergency management of severe right ventricular inflow obstruction secondary to a metastatic cardiac tumor

We report the case of a 21-year-old female with previous history of primitive neuroectodermal tumor of the anterior abdominal wall who developed severe manifestations of right heart failure due to a mass obliterating the right ventricular cavity. She underwent emergent resection of the mass with histopathologic confirmation of metastatic neuroectodermal tumor. To the best of our knowledge, this is the first case of emergent surgical excision of a metastatic cardiac tumor of primitive neuroectodermal origin.     

The XXXIV Spanish Congress of Pediatric Nephrology 4-7 June 2008, Sevilla, Spain

Abstracts

The XXXIV Spanish Congress of Pediatric Nephrology 4-7 June 2008, Sevilla, Spain     

An estimation method for midwife demand

Manpower or human resource planning is a major component of health services planning. In this study, it was aimed to practice an estimation method for midwife demand in a selected region of Ankara, Turkey. In this study, the needs-based approach was used as a method of estimating manpower demand. The study was conducted in one of the central regions in Ankara. Calculations were done separately for 31 primary health care centers in the region. Midwife demand was calculated separately for each of the six specified duties in all health centers. By summing the calculated numbers, midwife demand for each health center was found In order to determine the approximate time required to deliver each service, 10 midwives from 10 different primary health care centers in the region were selected by random sampling and interviewed. According to the sample calculation presented in this study, the number of midwives required (160) was found to be equal to the current number of midwife posts available in the region. Although this finding was favorable, the fact that 29% of those posts were unfilled constituted a significant problem. Lack of human resources can lead to the lack of essential services, lack of quality in service delivery and loss of strength in primary care. In order to facilitate the optimal delivery of health services, an adequate number of personnel have to be allocated in the most appropriate positions.     

Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9?±?68.7?months (range: 2–202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3?days (1–67?days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1?=?2.3[1.48–3.43]) and etoposide usage (OR2?=?1.22 [1.14–1.89]). The patient’s 30-day survival was inferior among patients whose albumin level was 2?g/dL or less compared to those over 2?g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.     

Insulin resistance in type 2 diabetes mellitus may be related to bone mineral density

The mechanism of bone mineral density (BMD) changes in type 2 diabetes mellitus is not clear. We aimed to investigate the effect of insulin resistance in type 2 diabetics on BMD. Insulin resistance was determined using the homeostasis model assessment index (HOMA-IR). Nineteen type 2 diabetic patients with a HOMA-IR <2.7 (mean age, 51.5±9.6yr; body mass index [BMI], 27.3±5.1kg/m(2); duration of diabetes, 10.5±7.3yr) were included in Group A, and 30 BMI- and age-matched type 2 diabetic patients with a HOMA-IR ≥2.7 were included in Group B. The BMD was measured with dual-energy X-ray absorptiometry. Independent t-test was used for statistical analysis. The Group A values for mean fasting glucose and insulin levels were 160.1±77.0mg/dL and 4.79±2.89μU/L, respectively, whereas the Group B values were 195.1±58.9mg/dL (p>0.05) and 19.30±16.89μU/L (p=0.0001). Significantly higher total lumbar vertebra T-score (p=0.02) and total lumbar vertebra BMD in Group A were determined than Group B (p=0.033). The lumbar vertebra total Z-score was significantly lower in Group B (p=0.042). Marked insulin resistance may have a negative effect on BMD in type 2 diabetics, while the presence of hyperinsulinemia may be associated with the low BMD.     

Outcome after renal transplantation in children from native and immigrant families in Austria

Renal transplantation is the therapy of choice for children with end-stage renal disease (ESRD). Ethnicity affects the transplant survival rates substantially, but there has been no European academic evaluation of the effects of immigration on the pediatric renal transplantation outcome. The aim of this study was to compare the outcomes of renal transplantation between the children of immigrant families and the children of native families at the pediatric nephrology unit of the Medical University of Vienna, Austria. We conducted a retrospective study on all children who underwent renal transplantation at our center between January 1997 and June 2005. The patients were separated into two groups according to their immigration backgrounds. During the time frame of our study, 59 children underwent a total of 63 transplantations. Of these children, 42 were from native Austrian and 17 were from first-generation immigrant families. We analyzed the demographic data and outcome parameters for each of the 59 patients. We found no difference in patient and graft survival rates or long-term function between native and immigrant children. The two groups were also comparable in the rates of acute rejection episodes, 24-h blood pressure, and growth velocity. Living donor source had a positive influence on graft function (p=0.06), 24-h blood pressure (p=0.05), and growth velocity (p=0.02) only in the immigrant group. Our retrospective analysis shows no influence of the migration status on the patient or graft outcome, but we did find that immigrant children benefitted more than native children from living donation as opposed to deceased donation. To explain this fact, biological, heath-economical, psychosocial, and cultural background aspects must be investigated. Source of funding  Medical Scientific Fund of the Mayor of Vienna, project no. 2581.     

Right Ventricular Mass: Intracardiac Ectopic Thyroid Tissue: A Case Report

Ectopic thyroid tissue is a result of abnormal migration of thyroid tissue during the embryonic state. Most of these rare cases, almost 90% of them, are located in the base of the tongue and a minority of them is located anteriorly near the hyoid bone area. Below we report the case of a young patient with an intracardiac mass first diagnosed with three‐dimensional echocardiography and afterward successfully treated surgically. Once a careful histological examination has revealed a follicular adenoma, the prognosis is good especially if successful surgical excision is part of the management.