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Background Outcome in severe acute necrotizing encephalopathy of childhood is poor, with high mortality (30%) and moderate to severe disability in survivors despite the use of intravenous corticosteroids or immunoglobulins. Increased blood interleukin 6 level correlates with poor outcome. Methods We report the early use of tocilizumab, a monoclonal antibody against the interleukin 6 receptor, in three patients (aged five, eight, and 10 years) with severe acute necrotizing encephalopathy. Results All three patients experienced a rapid neurological deterioration associated with febrile viral illnesses and met criteria for severe acute necrotizing encephalopathy with a high risk for death or severe disability. Intravenous methylprednisolone and tocilizumab were administered at 18 to 32 hours of encephalopathy in addition to supportive medical therapy. No side effects were observed with this therapeutic strategy. The two patients with influenza A(H1N1)pdm09 virus-related acute necrotizing encephalopathy had a short illness with excellent clinical and radiological recovery. The patient with influenza B virus-related acute necrotizing encephalopathy and florid hemorrhagic brain lesions had a slow recovery with eventual mild disability despite focal encephalomalacia on follow-up neuroimaging. Conclusions The early use of interleukin 6 blockade in acute necrotizing encephalopathy is safe and may have a role in improving outcomes and preventing disability.

Supplemental digital content is available in the text., Autosomal recessive interleukin (IL)-12 p40 (IL-12p40) deficiency is a rare genetic etiology of Mendelian susceptibility to mycobacterial disease (MSMD). We report the genetic, immunologic, and clinical features of 49 patients from 30 kindreds originating from 5 countries (India, Iran, Pakistan, Saudi Arabia, and Tunisia). There are only 9 different mutant alleles of the IL12B gene: 2 small insertions, 3 small deletions, 2 splice site mutations, and 1 large deletion, each causing a frameshift and leading to a premature stop codon, and 1 nonsense mutation. Four of these 9 variants are recurrent, affecting 25 of the 30 reported kindreds, due to founder effects in specific countries. All patients are homozygous and display complete IL-12p40 deficiency. As a result, the patients lack detectable IL-12p70 and IL-12p40 and have low levels of interferon gamma (IFN-γ). The clinical features are characterized by childhood onset of bacille Calmette-Guérin (attenuated Mycobacterium bovis strain) (BCG) and Salmonella infections, with recurrences of salmonellosis (36.4%) more common than recurrences of mycobacterial disease (25%). BCG vaccination led to BCG disease in 40 of the 41 patients vaccinated (97.5%). Multiple mycobacterial infections were rare, observed in only 3 patients, whereas the association of salmonellosis and mycobacteriosis was observed in 9 patients. A few other infections were diagnosed, including chronic mucocutaneous candidiasis (n = 3), nocardiosis (n = 2), and klebsiellosis (n = 1). IL-12p40 deficiency has a high but incomplete clinical penetrance, with 33.3% of genetically affected relatives of index cases showing no symptoms. However, the prognosis is poor, with mortality rates of up to 28.6%. Overall, the clinical phenotype of IL-12p40 deficiency closely resembles that of interleukin 12 receptor β1 (IL-12Rβ1) deficiency. In conclusion, IL-12p40 deficiency is more common than initially thought and should be considered worldwide in patients with MSMD and other intramacrophagic infectious diseases, salmonellosis in particular.

Excellent short-term results have been reported in ABO-incompatible (ABOi) renal transplant recipients managed solely with antibody removal and conventional immunosuppression. However, long-term clinical outcomes with this regimen and predictive information from protocol biopsies are lacking.We compared outcome data in ABOi and ABO-compatible (ABOc) recipients receiving this regimen approximately 4 years posttransplant, and histology from biopsies approximately 12 months posttransplant.Patient and graft survivals among 54 ABOi recipients were 98.1% and 90.7%, respectively, at 4 years. Graft function was similar between ABOi (creatinine, 140.3 μmol/L) and ABOc recipients (creatinine, 140.2 μmol/L) (P = 0.99), with no significant change over the study period in either group (Δcreatinine, -0.83 vs 6.6 μmol/L) (P = 0.59). There was no transplant glomerulopathy in biopsies from either group. Interstitial fibrosis (IF) and tubular atrophy (TA) was present in 7 (28%) of 25 ABOi compared with 7 (20.6%) of 34 ABOc (P = 0.52). Progression of IF/TA from implantation was noted in 6 (24%) of 25 ABOi and 6 (17.6%) of 34 ABOc, respectively. C4d staining without antibody-mediated rejection was present in 13 (52%) 25 early posttransplant biopsies from ABOi recipients by immunohistochemistry, but in only 4 (16%) of 25 at 12 months.ABO-incompatible renal transplant performed with antibody removal and conventional immunosuppression continues to provide excellent patient and graft survival, and stable renal function over 4 years. Coupled with absent transplant glomerulopathy and low rates of progressive IF/TA on earlier biopsies, this suggests that ABOi with conventional immunosuppression and antibody removal, without rituximab or splenectomy, can achieve long-term outcomes comparable to ABO-compatible transplantation.

A 65-year-old man presented with a 7-month history of eight bleeding periungual lesions on both feet. The clinical diagnosis of multiple pyogenic granulomas was confirmed by histological examination. Historically, the pyogenic granulomas appeared 3 months after commencing 5-fluorouracil chemotherapy for rectal carcinoma, suggesting a possible causative relationship. Chemotherapy was ceased by the supervising oncologist. Resolution occurred after two lesions had been treated with curettage and diathermy, and the remaining lesions with occlusive dressings over Kenacomb ointment (triamcinolone acetonide 0.1%, neomycin sulphate 0.25%, gramicidin 0.025%, nystatin 100,000 U/g) topically twice daily for a period of 3 months.

Despite the prevalence of amoebiasis in many parts of the world, amoebomas are relatively uncommon. Amoebomas within the colorectum are indistinguishable from carcinomas either macroscopically or by barium enema examination and the presence of both conditions is much rarer still. Herein, we describe such a case and review the possible reasons for their coexistence.

A retrospective surgical case review.A tertiary-care, university teaching hospital.The authors describe a unique case operated on for a middle ear cholesterol granuloma, which had invaded the cochlea and vestibule causing profound sensorineural deafness.Extended radical mastoidectomy and labyrinthectomy with musculofascial seal.Successful postoperative outcome with no recurrence seen after 2 years of follow up.To the authors' knowledge, this is the first reported case of a primary middle ear cholesterol granuloma with direct invasion into the cochlea. Such invasion of the otic capsule by cholesterol granulomas is rare and presents a diagnostic challenge to the attending otologist and radiologist. Salient points of the case history, pathogenesis, imaging studies, histopathology, and management are presented with a review of the current literature.

We have compared the use of the laryngeal mask airway with the new modified laryngeal tube in a prospective randomized controlled study. Sixty ASA 1 or 2 patients, aged 18 to 65 years, scheduled for elective surgery and breathing spontaneously under general anaesthesia, were studied. After preoxygenation, anaesthesia was induced with fentanyl and propofol. The patients were randomized to receive either a laryngeal mask airway or a laryngeal tube. Anaesthesia was maintained with nitrous oxide, oxygen and isoflurane. We recorded the speed and the ease of insertion, the number of attempts needed to successfully secure the airway and intraoperative complications, such as partial airway obstruction needing airway manipulation. The airway devices were removed with the patients fully awake at the end of surgery. Systolic arterial blood pressure, heart rate and end-tidal CO 2 were recorded at various time intervals. Postoperative complications were recorded. We found that the incidence of partial airway obstruction needing intraoperative airway manipulation was higher with the laryngeal tube than with the laryngeal mask airway. We conclude that during spontaneous ventilation the modified laryngeal tube is not as reliable in providing a satisfactory airway and we consider it is not a suitable alternative to the laryngeal mask airway.

Purpose: To assess the long-term survival of patients with nasopharyngeal carcinoma (NPC) who were treated with conventional radical radiotherapy (RT) followed by adjuvant chemotherapy. Methods and Materials: Ninety-one newly diagnosed patients with Stage III and IV (American Joint Committee on Cancer, 1988) NPC, seen at the University of Malaya Medical Center, Kuala Lumpur, Malaysia between January 1992 and May 1997, were treated with RT followed by adjuvant chemotherapy. The tumor dose was 70 Gy delivered in 35 fractions, 5 fractions weekly. Three cycles of chemotherapy, each consisting of 5-fluorouracil, 1 g/m 2 /d on Days 1–4 and cisplatin 100 mg/m 2 on Day 1, were administered 3 weeks after RT completion. Thirty-six patients had Stage II, 10 had Stage III, and 45 had Stage IV disease (AJCC 1997 staging system). Results: After a median follow-up of 61 months, the 5-year overall survival rate for all 91 patients was 80.1%, the disease-free survival rate was 76%, and the locoregional control rate was 85%. The 3-year overall survival rate for Stage II was 94.3%; it was 80% for Stage III and 79.8% for Stage IV ( p = 0.0108). The 3-year DFS rate for Stage II was 90%; it was 80% for Stage II and 65% for Stage IV. The rate of distant failure for Stage IV was 8.9%. Conclusion: Radical RT followed by adjuvant chemotherapy was effective in our patients with locoregionally advanced NPC. The long-term results appear encouraging, even for patients with Stage IV disease. This single institution experience deserves further investigation in prospective trials.

Interleukin-12 (IL12) is a cytokine that is secreted by activated phagocytes and dendritic cells and that induces interferon-gamma production by natural-killer and T lymphocytes. It consists of two subunits, p35 and p40, which are encoded by IL12A and IL12B, respectively. The first reported patient with a genetic cytokine disorder was a Pakistani child, who was homozygous for a large loss-of-function deletion (g.482+82_856-854del) in IL12B. This IL12-deficient child suffered from infections caused by bacille Calmette-Guérin (BCG) and Salmonella enteritidis. We herein report 12 additional patients from five other kindreds. In one kindred from India, the same large deletion that was described elsewhere (g.482+82_856-854del) was identified. In four kindreds from Saudi Arabia, a recessive loss-of-function frameshift insertion (g.315_316insA) was found. A conserved haplotype encompassing the IL12B gene suggested that a founder effect accounted for the recurrence of each mutation. The two founder mutational events-g.482+82_856-854del and g.315_316insA-were estimated to have occurred approximately 700 and approximately 1,100 years ago, respectively. Among a total of 13 patients with IL12 deficiency, 1 child had salmonellosis only and 12 suffered from clinical disease due to BCG or environmental nontuberculous mycobacteria. One patient also had clinical disease caused by virulent Mycobacterium tuberculosis, five patients had clinical disease caused by Salmonella serotypes, and one patient had clinical disease caused by Nocardia asteroides. The clinical outcome varies from case to case, since five patients (aged 2-11 years) died of overwhelming infection, whereas eight patients (aged 3-12 years) are still in good health and are not currently taking antibiotics. In conclusion, IL12 deficiency is not limited to a single kindred, shows significant variability of outcome, and should be considered in the genetic diagnosis of patients with mycobacteriosis and/or salmonellosis. To date, two founder IL12B mutations have been identified, accounting for the recurrence of a large deletion and a small insertion within populations from the Indian subcontinent and from the Arabian Peninsula, respectively.

Idiopathic nephrotic syndrome of childhood is thought to be associated with T lymphocyte dysfunction often triggered by viral infections, with the production of circulating factor(s) resulting in proteinuria. In view of the conflicting evidence of T cell activation and Th1 or Th2 pattern of cytokine synthesis in this disease, this study examined the mRNA expression of interleukin-2 (IL-2), interferon-gamma, IL-4, and IL-13 from CD4+ and CD8+ T cells in steroid-responsive nephrotic patients in relapse and remission. Fifty-five children with steroid-responsive nephrotic syndrome were included in this study, together with 34 normal controls and 24 patient controls with viral infections. RNA was isolated from purified CD4+ or CD8+ cells from peripheral blood and subjected to reverse transcription-PCR. Cytokine mRNA expression was measured semiquantitatively, and a cytokine index was derived from densitometric readings, with cyclophilin as the housekeeping gene. Both cross-sectional and paired data showed an increased CD4+ and CD8+ IL-13 mRNA expression in patients with nephrotic relapse as compared to remission, normal, and patient controls (P < 0.008). This was also associated with increased cytoplasmic IL-13 expression in phorbol myristate acetate/ionomycin-activated CD3+ cells (6.66+/-3.39%) from patients with nephrotic relapse compared to remission (2.59+/-1.35%) (P < 0.0001). However, there was no significant difference in CD4+ or CD8+ IL-2, interferon-gamma and IL-4 mRNA expression. IL-13 is an important T cell cytokine with anti-inflammatory and immunomodulatory functions on B cells and monocytes. It is conceivable that IL-13 may act on monocytes to produce vascular permeability factor(s) involved in the pathogenesis of proteinuria in patients with relapse nephrotic syndrome.

Intravenous immunoglobulin (IVIg) is a blood product with immunomodulating properties that have been widely applied in the management of renal transplant recipients. In general, IVIg has been considered a relatively safe therapy, with most adverse events being mild and transient. Although rare, a serious and well-recognized complication of IVIg is large-vessel thrombotic events, which are thought to be related to hyperviscosity. We describe here two cases in which there was a temporal relationships between the administration of IVIg, an acute decline in allograft function, and the histologic finding of de novo thrombotic microangiopathy (TMA). In both cases, IVIg had been administered to facilitate immunosuppressive dose reduction in the context of BK nephropathy. We believe this is the first report of TMA associated with IVIg administration in renal allograft recipients.

Although post-mortem studies would suggest that cardiac metastases occur frequently, many of these metastases remain clinically silent. However, symptomatic lesions may also remain unrecognized due to overshadowing by other symptoms of the primary malignancy. Patients undergoing treatment for cancer are not routinely screened using echocardiography, unless their chemotherapeutic regimen includes cardiotoxic agents. The current era of research and development of targeted biological agents (such as trastuzumab and epidermal growth factor receptor inhibitors) for cancer may lead to prolonged survival of oncology patients. In future, metastases that were once rare may become increasingly recognized as these new treatments augment the natural history of the disease. There have been several case reports of small, asymptomatic left ventricular metastases, but clinically significant ventricular metastases are very rare. There are no reports in the current literature of a symptomatic ventricular metastasis, occurring in the absence of other metastatic disease. We report an unusual case of a large solitary ventricular metastasis, leading to left ventricular outflow tract obstruction and haemodynamic compromise. Echocardiographic imaging led to the diagnosis of a recurrence of soft-tissue fibrosarcoma 9 years after original resection.

We compared cell-mediated immune responses in two groups of patients on hemodialysis. One group of patients were chronic carriers of hepatitis B virus (HBV), and the patients of the other group were HBV antigen negative. Our results show that despite the presence of normal numbers of T cells and an increased CD4/CD8 ratio in both groups of patients compared to healthy controls (p less than 0.0001), only the group of patients who were chronic HBV carriers showed depressed lymphoproliferative responses to phytohemagglutinin (p less than 0.001) and concanavalin A (p less than 0.0001). In contrast, a control group of healthy adult HBV carriers showed normal T cell subsets and lymphoproliferative responses to mitogens, indicating that HBV infection per se did not result in depressed lymphoproliferative responses. These results further substantiate the notion that depressed cell-mediated immunity in chronic renal failure is an important factor in predisposing patients to HBV infection with subsequent development of the chronic carrier state.

The incidence of structural renal tract abnormalities in 109 children with documented congenital heart disease (CHD) was studied using real-time sonography. In these children, 11.9% had associated urologic anomalies, which included hydronephrosis, duplication, ectopia, agenesis, and dysplasia of kidneys. Children with associated extracardiac anomalies had a significantly higher incidence of renal tract anomalies (39.1%) compared to those with isolated CHD (4.7%). Therefore, sonographic screening of the renal tract should be performed routinely in patients with CHD, especially in those with multiple congenital defects.

Objective: To determine the efficacy and safety of oral desmopressin (DDAVP) treatment in Asian children with nocturnal enuresis. Methodology: This was a multicentre randomized placebo-controlled double-blind cross-over study. Patients were randomized to either active treatment with oral 400 mg DDAVP or placebo, with a 2-week medication-free period between the cross-over. Children with primary monosymptomatic nocturnal enuresis, aged between 7 and 18 years, with a minimum frequency of wetting of 6 nights or more during a 2-week observation period were recruited. Efficacy was measured by reduction in the average number of wet nights per week. Results: Of the 37 children initially recruited, the outcomes for 34 children were included in the final cross-over analysis, as they had complete data for both the treatment periods. Statistical analysis by anova showed that there was no significant difference between the medication-free period and the pretreatment period. However, the average number of wet nights per week for the DDAVP treatment period (2.5±2.7) was significantly lower than that of the placebo treatment period (4.5±2.1) (P

The effect of growth hormone (GH) on subclinical disease activity in a 15-year-old boy with previously quiescent lupus nephritis and chronic renal failure is described. Institution of supraphysiological doses of GH resulted in a rise in erythrocyte sedimentation rate, decrease in serum complement, rise in anti-DNA antibody titers, and increase in T-cell activation markers, all of which improved following cessation of GH treatment.

An outbreak of infection with the Nipah virus, a novel paramyxovirus, occurred among pig farmers between September 1998 and June 1999 in Malaysia, involving 265 patients with 105 fatalities. This is a follow-up study 24 months after the outbreak. Twelve survivors (7.5%) of acute encephalitis had recurrent neurological disease (relapsed encephalitis). Of those who initially had acute nonencephalitic or asymptomatic infection, 10 patients (3.4%) had late-onset encephalitis. The mean interval between the first neurological episode and the time of initial infection was 8.4 months. Three patients had a second neurological episode. The onset of the relapsed or late-onset encephalitis was usually acute. Common clinical features were fever, headache, seizures, and focal neurological signs. Four of the 22 relapsed and late-onset encephalitis patients (18%) died. Magnetic resonance imaging typically showed patchy areas of confluent cortical lesions. Serial single-photon emission computed tomography showed the evolution of focal hyperperfusion to hypoperfusion in the corresponding areas. Necropsy of 2 patients showed changes of focal encephalitis with positive immunolocalization for Nipah virus antigens but no evidence of perivenous demyelination. We concluded that a unique relapsing and remitting encephalitis or late-onset encephalitis may result as a complication of persistent Nipah virus infection in the central nervous system.

Cytomegalovirus (CMV) infection is a major concern following solid organ transplantation, especially in the pediatric population who remain at high risk of primary infection. CMV disease leads not only to increased patient and graft morbidity, but also to increased health care costs. This study describes the usefulness of a quantitative CMV polymerase chain reaction (PCR) technique for monitoring peripheral blood CMV DNA in pediatric recipients of kidney and liver allografts who had recurrent CMV retinitis. The incidence of CMV disease in 28 pediatric transplant recipients was 28.6%, one-half of whom developed retinitis. Two of these patients had recurrent retinitis on cessation of anti-viral treatment. A peripheral blood CMV DNA copy number of > or =500/microg of DNA was associated with recrudescence of the retinitis in these patients. We conclude that the measurement of peripheral blood CMV DNA by PCR is a useful tool for the surveillance of disease resolution and recurrence. This is particularly important in patients with CMV retinitis, who may remain asymptomatic for a period of time, despite recurrences.

This study was undertaken to determine whether tailoring the dialysis prescription in Asian children on nightly intermittent peritoneal dialysis (NIPD), without adding high-dose therapy for cost-savings purposes, was able to achieve dialysis adequacy and improvement in nutrition. Eight children (age range: 5.5-20 years) on NIPD for a mean of 2.1 +/- 0.6 years, were studied at baseline and at 3 months and 9 months after their dialysis dose was tailored. Dialysis adequacy was measured by weekly Kt/Vurea and creatinine clearance (CCr). Fat-free mass (FFM) and percent body fat (%FAT) as measured by bioelectrical impedance, together with anthropometric measurements, serum total protein, and albumin, were used as indicators of nutrition. After the dialysis prescription was tailored, the mean weekly Kt/Vurea increased from 1.89 +/- 0.35 to 2.12 +/- 0.54 at 9 months, and total CCr increased from 36.4 +/- 11.51 L/1.73 m2 to 48.30 +/- 14.30 L/1.73 m2. The increase occurred despite a decline in residual renal function and was attributable to significant improvements in the peritoneal clearances of urea and creatinine (p0.05). The mid arm muscle circumference (MAMC) increased significantly (p = 0.006), while FFM increased from 25.68 +/- 7.92 kg to 26.95 +/- 9.83 kg, and %FAT decreased from 21.56% +/- 8.41% to 18.66% +/- 8.16%. The increase in FFM correlated significantly with a decrease in serum creatinine (r = -0.94, p = 0.005). In conclusion, tailoring the dialysis prescription in NIPD, without adding high-dose therapy, resulted in a trend of improvement in dialysis adequacy and some nutritional parameters.

Postprandial lipoproteins are raised in diabetes and there is increasing evidence for the atherogenicity of the chylomicron remnant. Increased postprandial cholesteryl ester transfer has also been demonstrated in diabetes and may contribute to the atherogenic lipoprotein profile. The present study examined the effect of improving metabolic control on postprandial lipoproteins in 13 Type 2 diabetic patients. Blood was taken fasting and at 2-h intervals following a high fat, 1100 kcal meal. Patients were brought into good control by intensified dietary advice and oral hyperglycaemic agents or insulin if blood glucose failed to respond. Fasting and postprandial cholesteryl ester transfer protein (CETP) and lecithin:cholesteryl acyltransferase (LCAT) were determined in six patients. Lipoproteins were isolated by sequential ultracentrifugation. Chylomicron and very low density lipoprotein (VLDL) apolipoprotein B-48 and apolipoprotein B-100 were isolated by polyacrylamide gradient gel electrophoresis and quantified by densitometric scanning. CETP and LCAT were determined by an endogenous method which determined cholesterol esterification and transfer between the patients’ lipoproteins. There was a significant reduction in postprandial chylomicron apo B-48 (PB0.005), apo B-100 (PB0.0005) and chylomicron cholesterol (PB 0.001) following improved diabetic control. The chylomicron lipid:apo B ratio increased with improved control (PB 0.01). Postprandial CETP and LCAT were significantly reduced in good control (PB0.01 and PB 0.05, respectively) and there were significant changes in HDL composition. The study shows that improvement in metabolic control in Type 2 diabetic patients leads to a reduction in postprandial chylomicron particles and less transfer of cholesterol to apo B-containing lipoproteins. © 2000 Elsevier Science Ireland Ltd. All rights reserved.

Dysplastic gangliocytoma or Lhermitte-Duclos disease (LDD) is a hamartomatous malformation of the cerebellar hemisphere that usually presents with signs of increased intracranial pressure or symptoms of cerebellar dysfunction. In this paper, we report a case of LDD presenting with tinnitus, and postulate a probable mechanism for this unusual presentation.

The determinants for recurrence of Crohn's disease remain controversial. Eight factors that might predict recurrence were studied in 130 patients operated on for Crohn's disease over a 24-year period.Separate survival curves were estimated for each variable. A multivariate analysis was then carried out in which several selected explanatory variables were included simultaneously in a proportional hazards regression model.Operation for recurrent disease was necessary in 36 patients. Univariate analysis revealed a highly significant relationship between the presence of granulomas and subsequent recurrence (P = 0.003). There was a trend towards increased recurrence in patients with ileocolonic disease and segmental colectomy (P = 0.11 and P = 0.1 respectively). Age, sex, length of history, indication for operation and affected lines of transection were not associated with recurrence. After multivariate analysis, the association of granulomas with recurrence remained significant (P = 0.03). This association persisted when death was regarded as a treatment failure (P = 0.02).The presence of granulomas in patients with Crohn's disease is significantly associated with recurrence.

A retrospective cohort study was undertaken to evaluate the diagnostic value of the renal medulla in acute renal allograft rejection (ARAR). One hundred and ninety-five biopsies from 98 patients were randomly selected out of 565 transplant biopsies. Biopsies were graded blindly from Grade 0 (no rejection) to Grade 3 (severe rejection) using standard criteria; ARAR was confirmed by a fall in all cases of mean serum creatinine concentration from 0.331 +/- 0.182 to 0.184 +/- 0.079 mmol/l, with anti-rejection therapy. In the 43 biopsies which contained both cortex and medulla, the ARAR grades and the intensities of mononuclear cell, plasma cell, polymorphonuclear cell and eosinophil infiltrates, and of interstitial oedema and haemorrhage, were similar in cortex and medulla (Spearman's Rank Correlation r = 0.55-0.81, P0.001). The sensitivity, specificity and overall accuracy of medullary changes in predicting ARAR changes in the cortex were 77%, 100% and 38%, respectively. Acute vascular rejection changes could not be compared between renal cortex and renal medulla because of the anatomical differences between cortex and medulla. Further evaluation of ARAR in the all 195 biopsies, of which 188 had cortical tissue and 50 had medullary tissue, showed no significant differences in histological features (P0.05), except for more cortical biopsies with plasma cells (29%) than medullary biopsies with plasma cells (10%; P0.02). It is concluded that: (1) ARAR histological changes are similar in cortex and medulla; (2) the predictive value of ARAR medullary changes for cortical rejection changes has low sensitivity (77%) and high specificity (100%).(ABSTRACT TRUNCATED AT 250 WORDS)

This report describes a patient with acute renal failure that resulted from the ingestion of djenkol beans. Features of acute djenkolism include nausea, vomiting, bilateral loin pain, gross hematuria, and oliguria. The blood urea level was 16.2 mmol/L and the serum creatinine was 460 mumol/L. Phase contrast microscopy of the urinary sediment indicated that the hematuria was nonglomerular. Ultrasound of the kidneys showed slightly enlarged kidneys with no features of obstruction. Renal biopsy showed acute tubular necrosis similar to the single animal study reported in the literature. With conservative therapy, which included rehydration with normal saline and alkalinization of the urine with sodium bicarbonate, the acute renal failure resolved. Based on its chemistry, djenkol bean-associated acute renal failure may be analogous to acute uric acid nephropathy.

The risk of renal papillary necrosis and renal dysfunction due to the chronic use of nonsteroidal anti-inflammatory drugs (NSAIDs) is unknown. In a prospective study of 259 heavy analgesic users seen in a general medical hospital over an 11-year period beginning in January 1982, 69 new cases of analgesic nephropathy with renal papillary necrosis were confirmed by intravenous urogram (26.6%), ultrasonography (30.4%), and/or computed tomography (43%). Twentynine of these patients (42%) had consumed excessive quantities of NSAIDs alone; an additional nine patients (13%) had consumed NSAIDs predominantly in combinations with paracetamol, aspirin, phenacetin, caffeine, and/or traditional herbal medications. Of those patients who consumed NSAIDs alone, 17 had consumed only a single type of NSAID and the remaining 12 had consumed multiple types of NSAIDs. The amount of NSAIDs administered ranged from 1,000 to 26,600 capsules or tablets over a 2- to 25-year period. Renal impairment (serum creatinine, 126 to 778 μmol/L) was noted in 26 of these 38 patients (64.8%). The reasons given for consuming NSAIDs include gouty arthritis (18 patients), osteoarthritis (seven patients), rheumatoid arthritis (six patients), chronic headache (three patients), gouty arthritis plus chronic headache (three patients), and chronic backache (one patient). All patients were prescribed these drugs and were followed medically. The occurrence of analgesic nephropathy was predominantly in males (male to female ratio, 1.9:1). Most of the patients did not have the characteristic psychological profile attributed previously to analgesic abuse nephropathy. Associated addictive habits, such as the use of psychotropic drugs and sleeping tablets, purgative abuse, and alcoholism, were absent. We conclude that excessive consumption of NSAIDs can lead to analgesic nephropathy with chronic renal impairment. The frequency of this problem related to the size of the population chronically receiving prescribed and nonprescribed NSAIDs needs to be carefully discerned by controlled prospective studies as part of any public health strategy to prevent end-stage renal disease.

Despite the tremendous health-care advances in recent years, external compression of the tracheobronchial tree by cardiovascular anomalies still remains a significant cause of morbidity and potential mortality in neonates and infants. As the initial presenting symptoms can be deceivingly mild, the diagnosis is easily missed without a high index of suspicion by the investigating clinician utilising the appropriate investigations. Four case studies are presented, (i) illustrating the importance of tracheobronchoscopy in assessing any neonate or infant with suspected airway obstruction; (ii) reviewing the current literature, and (iii) suggesting a comprehensive diagnostic approach by way of relevant investigations.

A comparative study was made on two groups of children comprising 20 patients with renal hypoplasia/dysplasia in one group and 12 patients with chronic glomerulonephritis (GN) in the other, presenting with chronic renal failure (CRF) in the Department of Paediatrics, Singapore General Hospital and National University Hospital between 1975 and 1989. The age of onset of CRF, the progression of renal failure and the presence of various clinical complications were analysed and compared. The mean age of onset of CRF was earlier in patients with renal hypoplasia/dysplasia (p less than 0.001) but the progression of renal failure in these patients were slower (p less than 0.005). Hypertension occurred more frequently in the chronic GN group (p less than 0.001) while urinary tract infection (UTI) occurred more frequently in the renal hypoplasia/dysplasia group (p less than 0.004). With the early onset of renal failure and slow deterioration of renal function in patients with renal hypoplasia/dysplasia, the provision of good conservative treatment for renal failure is most important in the management of these patients. In the chronic GN patients however, with the rapidity of deterioration of renal function, early preparation for replacement therapy becomes more imminent. However, renal replacement therapy in end-stage renal failure (ESRF) is costly and not readily available, it is more prudent to delay the onset of ESRF by providing effective conservative treatment of renal failure which includes the early recognition and treatment of hypertension in chronic GN and UTI in renal hypoplasia/dysplasia.

Complications of chemotherapy for a synovial sarcoma in an eight-year old boy included cisplatinum nephrotoxicity and adriamycin cardiotoxicity. The resultant renal failure and fluid overload necessitated peritoneal dialysis. Intractable hypomagnesemia was also a prominent feature.

Feeding gastrostomies were placed in three children treated with chronic peritoneal dialysis at our center because of persistent, severe malnutrition and inadequate growth. Two had frequent fungal infections of the gastrostomy site and all three developed Candida peritonitis which occurred at 1 month, 2 months and 2 years after insertion of gastrostomy. Complications included multiple intra-abdominal adhesions, abscess formation and loss of peritoneal function necessitating transfer to hemodialysis. The presence of a gastrostomy may predispose to the development of fungal peritonitis with its high morbidity and should be avoided in children on chronic peritoneal dialysis.

OBJECTIVE To report a case of isolated renal mucormycosis in a previously healthy man, and to briefly review the literature relating to this rare condition. CLINICAL FEATURES An 18-year-old man presented with a two-month history of fevers followed by two weeks of severe left loin pain. Initial treatment with antibiotics failed and abdominal computed tomography and renal perfusion scans were requested, demonstrating a non-functioning left kidney. INTERVENTION AND OUTCOME Nephrectomy was performed, the histology of which was consistent with mucormycosis. Amphotericin B was administered for one month. The patient made a complete recovery and no underlying disorder was found. CONCLUSIONS This man is believed to be the first reported Australian with isolated renal mucormycosis, and only the second person worldwide in whom an identifiable underlying disorder was absent.

Summary A patient who presented with cutaneous nodules had carcinoma of the stomach. The histochemistry of the dermal infiltrate gave a clue to the primary site. Autopsy revealed metastases in all the organs of the chest and abdominal cavity. The invasion of the testis and the gingiva was unusual. Cutaneous metastases from a visceral malignancy probably indicate an early fatal termination. Metastatic lesions to the skin from internal malignancies are rare and the incidence has been reported to vary from one to 2·7% (Gates, 1937; Beerman, 1957). Most reports quote lung and the breast as the primary site of malignancy although other sites including the stomach are not uncommon. The metastases, either single or multiple, may be the initial manifestations of the internal malignancy.

The first living patient with a Schistosoma japonicum-type infection who presented with the nephrotic syndrome is reported in detail. It is not clear whether the nephrotic syndrome was due to the schistosome infection or to the deposition of hepatitis B antigen and antibody complexes. This is the tenth case of schistosomiasis reported from aborigines in Malaysia and a sylvatic source of infection is suggested.

mesangium of the kidney. C3 has been consistently found deposited in a similar distribution. In addition, IgG and IgM may also be present in the mesangium. The clinical course is generally benign. Gross hematuria following an upper respiratory infection and intermittent or persistent microhematuria are the hallmarks of the disease. Occasionally, there is proteinuria (usually less than 1 gm/24 hours), hypertension, or nephrotic syndrome. Renal failure has been reported in children, ~ but is more commonly found in adults who have had the disease for many years. Literature search revealed three children reported to have transient moderate reduction in creatinine clearances, which returned to normal without therapy. 3,4 Our patient with immunofiuorescent-proven IgA nephropathy had an atypical course. Three episodes of renal insufficiency have been documented over a period of 13 months. All episodes were preceded by upper respiratory tract infections and gross hematuria. One episode required peritoneal dialysis. Serum creatinine values returned to normal after each episode. It is suggested that acute renal failure in IgA nephropathy should be treated conservatively because of the excellent chance of complete recovery.

The prevalence rate of 749 cases of oral squamous cell carcinomas (SCC) recorded between 1978-1984 was studied. Results showed that the Indians formed the dominant group (63.8%) followed by the Malays (19.6%) and Chinese (16.6%). The peak incidences were generally located between the sixth and eighth decades. Only in the Chinese group a male preponderance was noted. In the Indians, the buccal mucosa was the most prevalent site; in the Malays and Chinese males, the gingiva and alveolus; and the Chinese female, the tongue. In all three groups, the well-differentiated SCC was the commonest type and the poorly differentiated least common.

Congenital pyloric atresia is a very rare condition and, to our knowledge, has never been described in siblings. Two siblings (a male and a female) are described; the first sibling died from peritonitis, after operation to close gastric perforation. In the other sibling, the diagnosis was made by gastric aspiration of the last feeding just prior to the next, and gastroduodenostomy was successful. The possibility is raised of this being a genetically determined condition.