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Parenchymal Involvement (parenchymal + involvement)
Selected AbstractsDiffuse central nervous system protoplasmic astrocytoma,PEDIATRIC BLOOD & CANCER, Issue 5 2010ChB Hons, Sue Manley MB Abstract Protoplasmic astrocytoma is an extremely rare form of grade II low grade glioma which usually presents as a discrete mass lesion. We describe a 3-year-old female with diffuse protoplasmic astrocytoma with parenchymal involvement and leptomeningeal spread. This tumour proved extremely difficult to diagnose and followed a progressive course. Three superficial biopsies did not give the diagnosis and this was only confirmed 8 months from presentation from a larger fourth biopsy taken deeper from the cerebellum. To our knowledge this case represents the distinct presentation of protoplasmic astrocytoma presenting as extensive diffuse meningeal disease. Pediatr Blood Cancer 2010;54:768,769. © 2009 Wiley-Liss, Inc. [source] Early pulmonary involvement in Niemann-Pick type B disease: Lung lavage is not usefulPEDIATRIC PULMONOLOGY, Issue 2 2005Z.S. Uyan MD Abstract Niemann-Pick disease (NPD) is a rare, autosomal-recessively inherited lipid storage disease which is characterized by intracellular deposition of sphingomyelin in various body tissues. The disease is heterogeneous and classified into six groups. Pulmonary parenchymal involvement may be a feature of several subtypes of NPD, including type B. Progressive pulmonary involvement in NPD type B is a major cause of morbidity and mortality. It is usually diagnosed at older ages. Only a few cases with early pulmonary involvement have been reported. In this report, a patient with NPD type B, hospitalized with the diagnosis of pneumonia at age 3 months, is presented. Following treatment for pneumonia, she continued to have persistent respiratory symptoms and became oxygen-dependent. High-resolution computed tomography of the chest revealed diffuse interstitial changes. During follow-up, the patient developed hepatosplenomegaly. Lung, liver, and bone marrow biopsies showed characteristic findings for NPD. Biochemical studies also confirmed the diagnosis, and the sphingomyelinase enzyme level of the patient was low. Unilateral lung lavage was performed in order to decrease lipid storage as a treatment modality. However, there was no clinical or radiological improvement. The patient died at age 15 months due to progressive respiratory failure. Pulmonary involvement is a rare entity in early childhood in patients with NPD type B, but should be considered in the differential diagnosis of persistent interstitial lung disease. It may cause progressive respiratory failure, but the treatment options remain limited. Pediatr Pulmonol. 2005; 40:169,172. © 2005 Wiley-Liss, Inc. [source] Clinical spectrum of tuberculous pleural effusion in childrenPEDIATRICS INTERNATIONAL, Issue 3 2007CHIH-YUNG CHIU Abstract Background: The aim of this study was to describe the clinical characteristics and potentially diagnostic specimens of pediatric patients with tuberculous pleural effusion (TPE) to make a prompt diagnosis. Methods: Children who had TPE from September 1997 to December 2003 were retrospectively reviewed at a tertiary pediatric facility in northern Taiwan. Results: There were seven boys and six girls and their ages ranged from 10 to 17 years (average, 14.6 years). Tuberculosis contact history was identified in only six patients (46%). Fever (12/92%), cough (9/69%) and malaise (6/46%) were the most common symptoms. Normal leukocyte count was found in 12 patients (92%). Chest radiograph review showed unilateral pleural effusion in 12 patients (92%) but parenchymal involvement was found in nine patients (69%). Most of the pleural fluid analysis showed a lymphocytic exudative effusion (5/6). The acid-fast bacilli (AFB) stain of sputum, gastric washing, and pleural aspirate was positive in six of 11 (55%), two of seven (29%), and one of five (20%) patients, respectively. Culture of sputum, gastric washing, and pleural aspirate yielded Mycobacterium tuberculosis in four of 11 (36%), two of seven (29%), and two of five (40%) patients, respectively. A total of 6 to 9 months of multiple-drug therapy for tuberculosis was successful without sequale. Conclusions: Tuberculous pleural effusion usually presents as an acute illness and should always be considered in the differential diagnosis for older children and adolescents with pneumonia. A normal leukocyte count with a lymphocytic exudative effusion may provide a clue to the correct diagnosis of TPE. Diagnostic specimen of sputum seems more effective and sensitive in childhood TPE, especially those having pulmonary involvement. [source] Renal damage in vesico-ureteric refluxBJU INTERNATIONAL, Issue 4 2004P. Caione OBJECTIVE To detect the different extent of renal parenchymal involvement in primary vesico-ureteric reflux (VUR), and to evaluate the relationship between VUR grade, patient age and different patterns of parenchymal damage. PATIENTS AND METHODS This blinded retrospective study included 197 consecutive children (mean age 4.26 years, range 1 month to 13 years) with primary VUR detected by voiding cysto-urethrography (VCUG), 99mTc-dimercaptosuccinic acid (DMSA; 120 MBq/1.73 m2) renal scintigraphy, with scanning for 3 h after intravenous injection. An abnormal DMSA scan was classified into three subtypes: cortical defects as a single scar (SS), multiple cortical scarring (MS) and diffuse reduced uptake with small renal size. Renal absolute uptake (AU), and split-kidney relative uptake were evaluated in refluxing and nonrefluxing renal units, and correlated with parenchymal damage and patient age. Student's t -test and the chi-square test were used for the statistical analysis. RESULTS In all, 282 refluxing and 112 nonrefluxing units were assessed. Renal damage was detected in 188 of 282 units with VUR (67%) and in 18 of 112 (16%) contralateral nonrefluxing kidneys. The mean AU was 18.7% in kidneys with VUR and 29% in nonrefluxing units (P < 0.001). The mean (sd) AU decreased from lower to higher grades of VUR, i.e. grade 0 VUR (group A), 28.97 (9.71); grade 1,3 (group B), 21.28 (8.33); grade 4,5 (group C), 14.78 (8.02). The differences were statistically significant (A vs B, B vs C, both P < 0.001). Renal damage was differently distributed in the three groups: 69 of 109 kidneys (63%) in group C (MS prevalent), 39 of 173 (22.5%) in group B (SS prevalent) and 17 of 112 (15.2%) in group A. There was no significant difference in the distribution of renal damage subtypes in patients aged <,or >,2 years (SS 19.6% vs 17.9%, MS 29.6% vs 30.1%, small size 48.2% vs 46.3%). The VUR was severe (group C) in 65% of patients aged <,2 years and in 46% aged >,2 years (chi-square, P= 0.016). CONCLUSIONS VUR is commonly associated with renal damage. Age (< or >,2 years) did not significantly influence the kidney lesion subtype. Reduced parenchymal function (AU) progressively decreased with the severity of VUR. Focal MS, reduced size and relative uptake were significantly more common in severe VUR, leading to multifocal lesions and hypo-dysplasia. Renal scarring was present in up to 15% of contralateral nonrefluxing kidneys. Severe VUR behaved differently from lesser VUR in the renal scan parenchymal uptake. [source] | ||||||||||