Chronic granulomatous disease (CGD) is a very rare disease whose defect lies in an abnormal intracellular killing resulting in recurrent abscesses, lymphadenitis and granuloma formation. We describe 2 Malay male infants with CGD whom we believe to be the first report of this disorder in Malays. Both children presented with recurrent abscesses, pneumoniae and hepatosplenomegaly; lymphadenopathy was also present in one of the patients. The organisms isolated were catalase positive bacteria. Both neutrophil chemiluminescence (against fungal and bacterial antigens, phorbol myristate acetate) and intracellular killing assays were severely depressed. Recognition of CGD is important as great strides have been made in the treatment of this disease which include gamma interferon therapy besides the conventional prophylactic antibacterial therapy.
Anti-Bacterial Agents ; therapeutic use ; Fatal Outcome ; Granulomatous Disease, Chronic ; diagnosis ; drug therapy ; immunology ; Humans ; Immunity, Cellular ; Infant ; Interferon-gamma ; therapeutic use ; Malaysia ; Male ; Phagocytosis

<p>UNLABELLEDThe aim of this paper was to study the efficacy, side effects and complications of radiofrequency (RF) ablation of primary and metastatic liver malignancies.p><p>MATERIALS AND METHODSWe retrospectively reviewed 57 patients (39 men, 18 women; mean age, 63 years; age range, 44 to 83 years) who underwent RF ablation for liver malignancies from January 2002 to December 2004. A total of 87 tumours were ablated - 71 (81.6%) hepatocellular carcinomas and 16 (18.4%) metastases (from primaries in the colon, stomach and pancreas). RF ablation was performed either percutaneously (n = 71) under conscious sedation or intraoperatively (n = 16) under general anaesthesia. Follow-up ranged from 1 month to 41 months (mean, 15.2) and included computed tomography (CT) 1 day, 1 month and 3 months after ablation, and half-yearly thereafter. Patients were observed for local tumour progression and for the emergence of new tumours.p><p>RESULTSFour patients with a total of 5 tumours were lost to follow-up. Of the remaining 82 tumours treated, complete ablation was attained in 66 tumours after a single procedure, giving a primary effectiveness rate of 80.5%. Seven (8.5%) required 2 procedures to achieve complete ablation, giving a secondary effectiveness rate of 89% after 2 ablations. One tumour (1.2%) required 3 procedures to achieve complete ablation. One tumour required 4 procedures to date, with the latest follow-up CT still demonstrating incomplete ablation. Two tumours (2.4%) had an initial RF ablation and subsequent transarterial chemoembolisation (TACE). One tumour had an initial RF ablation followed by 32Phosphorus-biosilicon (BrachySil) injection, the latter as part of a Phase IIA trial. One tumour required 2 RF ablations and a subsequent TACE. Lastly, 3 tumours received initial RF ablation but subsequent local tumour progression was not treated as the patients were deemed unfit for repeat ablation. No procedure-related deaths or major complications were encountered. Minor complications were reported in 2 patients (3.8%) - subcapsular haematoma and thermal injury to the adjacent gastric antrum, both not necessitating surgical intervention.p><p>CONCLUSIONSRF ablation is an effective, safe and relatively simple procedure for the treatment of unresectable liver malignancies.p>
Adult ; Aged ; Aged, 80 and over ; Carcinoma, Hepatocellular ; mortality ; secondary ; surgery ; therapy ; Catheter Ablation ; Chemoembolization, Therapeutic ; Combined Modality Therapy ; Female ; Hospitals, General ; Humans ; Liver Neoplasms ; mortality ; secondary ; surgery ; therapy ; Male ; Middle Aged ; Retreatment ; Retrospective Studies ; Singapore ; Surgery, Computer-Assisted

<p>INTRODUCTIONAcute pancreatitis appears to be less prevalent in multi-ethnic Southeast Asia, where the aetiology also appears to be influenced by ethnicity. As with acute pancreatitis elsewhere, however, pancreatic necrosis is a cause of significant mortality and the aim of this study was to review our institutional experience with pancreatic necrosectomy.p><p>MATERIALS AND METHODSThe records of all patients who underwent pancreatic necrosectomy from January 2000 to December 2004 were analysed. Indications for surgery were the presence of infected necrosis, unresolving sepsis attributable to ongoing pancreatitis or the presence of gas in the pancreatic bed on imaging. Surgical debridement was achieved by debridement with closure over drains or by debridement with open packing.p><p>RESULTSThe cohort comprised 14 of 373 patients admitted for acute pancreatitis (3.8%), with an overall mortality rate of 29%. All patients had infected necrosis with positive bacteriological cultures. Eight patients (57%) underwent debridement with closure over drains and 6 patients (43%) underwent debridement with open packing. All mortalities occurred in patients who underwent open packing, who were also associated with a higher mean Acute Physiology and Chronic Health Evaluation (APACHE) II score. The mortality rate in patients who underwent debridement less than 4 weeks after admission was 33% (2 of 6), compared with 25% (2 of 8) in patients who underwent debridement after 4 weeks. There were no mortalities in patients operated on after 6 weeks.p><p>CONCLUSIONSurgical debridement with closure of drains and a policy of performing delayed necrosectomy are viable in our population.p>
APACHE ; Adult ; Aged ; Asia, Southeastern ; epidemiology ; Cohort Studies ; Debridement ; methods ; Drainage ; Female ; Humans ; Male ; Middle Aged ; Pancreatectomy ; methods ; Pancreatitis, Acute Necrotizing ; diagnosis ; mortality ; surgery ; Time Factors ; Tomography, X-Ray Computed

<p>INTRODUCTIONThis study aims to determine if the quantitative method of region-of-interest (ROI) analysis of lesion attenuation on CT may be a useful adjunct to the conventional approach of diagnosis by visual assessment in assessing tracer wash-out in hepatocellular carcinomas.p><p>MATERIALS AND METHODSFrom a surgical database of 289 patients from 2 institutions, all patients with complete surgical, pathological and preoperative multiphasic CT scans available for review were selected. For each phase of scanning, HU readings of lesion obtained (Lesion(arterial), Lesion(PV) and Lesion(equilibrium)) were analysed using receiver operating curves (ROC) to determine the optimal method and cut-off value for quantitative assessment of tumour wash-out (Lesion(arterial - equilibrium), Lesion(PV - equilibrium) or Lesion(peak - equilibrium)).p><p>RESULTSNinety-four patients with one lesion each met the inclusion criteria. The area under the curve (AUC) values for Lesion(arterial - equilibrium) (0.941) was higher than the AUC for Lesion(pv - equilibrium) (0.484) and for Lesion(peak - equilibrium) (0.667). Based on ROC analysis, a cut-off of 10HU value for Lesion(arterial - equilibrium) would yield sensitivity and specificity of 91.5% and 80.9%, respectively. ROI analysis detected 9/21 (42.9%) of lesions missed by visual analysis. Combined ROI and visual analysis yields a sensitivity of 82/94 (87.2%) compared to 73/94 (77.7%) for visual analysis alone.p><p>CONCLUSIONUsing a cut-off of 10 HU attenuation difference between the arterial and equilibrium phases is a simple and objective method that can be included as an adjunct to visual assessment to improve sensitivity for determining lesion wash-out on CT.p>
Adult ; Aged ; Aged, 80 and over ; Carcinoma, Hepatocellular ; diagnosis ; pathology ; surgery ; Confidence Intervals ; Databases, Factual ; Female ; Humans ; Liver ; pathology ; Liver Neoplasms ; diagnosis ; pathology ; surgery ; Male ; Middle Aged ; Preoperative Period ; ROC Curve ; Retrospective Studies ; Sensitivity and Specificity ; Tomography, X-Ray Computed ; instrumentation ; Young Adult