Empyema Thoracis in Children: A Short Term Outcome Study.

This study prospectively evaluates clinical course of pyogenic empyema thoracis in 25 children (2 mo – 12 y) treated with injectable antibiotics and chest tube drainage, and followed for 6 weeks. The median (range) age at presentation was 3 y (4 mo to 11 y). The pleural fluid culture was positive in 24% of patients. Staphylococcus aureus was the most commonly isolated organism. The median (range) duration of injectable antibiotics was 14(14-52) d; median duration of total antibiotics (injectable and oral) was 4 weeks. The median (range) duration of chest tube insertion and hospital stay was 8(5-45) and 14(14-56) days, respectively. All patients were discharged without any surgical intervention besides chest tube drainage. At discharge, pleural thickening was present in 84% and crowding of ribs was seen in 60% of the subjects on radiological examination. All these patients were asymptomatic at discharge. Chest deformity was present in 20% of the patients at 6-weeks follow up. Antibiotics and chest tube drainage is an effective method of treating pyogenic empyema thoracis in children in resource-poor settings.

Disease Course in Steroid Sensitive Nephrotic Syndrome.

Objective: To review the disease course in patients with steroid sensitive nephrotic syndrome (SSNS) and the factors that determine outcome Design: Retrospective, analytical Setting: Pediatric Nephrology Clinic at referral center in North India Participants/patients: All patients with SSNS evaluated between 1990 and 2005 Intervention: None Main outcome measures: Disease course, in patients with at least 1-yr follow up, was categorized as none or infrequent relapses (IFR), frequent relapses or steroid dependence (FR), and late resistance. Details on complications and therapy with alternative agents were recorded. Results: Records of 2603 patients (74.8% boys) were reviewed. The mean age at onset of illness and at evaluation was 49.7±34.6 R E S E A R C H P A P E R INDIAN PEDIATRICS 881 VOLUME 49__NOVEMBER 16, 2012 and 67.5±37.9 months respectively. The disease course at 1-yr (n=1071) was categorized as IFR in 37.4%, FR in 56.8% and late resistance in 5.9%. During follow up, 224 patients had 249 episodes of serious infections. Alternative medications for frequent relapses (n=501; 46.8%) were chiefly cyclophosphamide and levamisole. Compared to IFR, patients with FR were younger (54.9±36.0 vs. 43.3±31.4 months), fewer had received adequate (≥8 weeks) initial treatment (86.8% vs. 81.7%) and had shorter initial remission (7.5±8.6 vs. 3.1±4.8 months) (all P<0.001). At follow up of 56.0±42.6 months, 77.3% patients were in remission or had IFR, and 17.3% had FR. Conclusions: A high proportion of patients with SSNS show frequent relapses, risk factors for which were an early age at onset, inadequate initial therapy and an early relapse.

Typhoid fever and viral hepatitis.

Feco-oral route is the most common mode of transmission for both enteric fever and hepatitis A and E, and thus infection by these agents may occur concurrently especially in tropical endemic areas like India. In this scenario, there occurs a diagnostic dilemma with respect to differentiation from 'typhoid hepatitis', the hepatic manifestations of typhoid fever. We herein present such a case and discuss pertinent issues.

Nephrotic syndrome in children.

Nephrotic syndrome is an important chronic disease in children, characterized by minimal change disease in the majority. Research on pathogenesis has emphasized the importance of T lymphocyte dysregulation and vascular permeability factors that might alter podocyte function and permselectivity. While mutations in genes that encode important podocyte proteins have also been identified, a hypothesis unifying available evidence on pathogenesis is yet to be proposed. Patients with nephrotic syndrome are at risk for life threatening infections and thromboembolic episodes. Long-term effects of persistent hyperlipidaemia and prolonged steroid therapy are increasingly recognized. Remission of proteinuria following corticosteroid therapy has greater prognostic value, in relation to long-term outcome, than the precise renal histology. Prospective studies show that prolonged duration of therapy for the initial episode results in sustained remission and reduced frequency of relapses. Treatment with levamisole, cyclophosphamide, cyclosporine and mycophenolate mofetil is beneficial in a variable proportion of patients with frequent relapses or steroid dependence. The management of steroid-resistant nephrotic syndrome is difficult; most patients failing to achieve remission show progressive renal damage. Calcineurin inhibitors (cyclosporine, tacrolimus) are capable of inducing remission in a significant proportion of patients, but at risk of nephrotoxicity. Reduction of proteinuria is also possible, in children, using angiotensin converting enzyme inhibitors and/or angiotensin receptor blockers. Prospective trials are necessary to identify effective and safe therapies for patients with frequent relapses, steroid dependence and resistance.

Long-term outcome in children with primary distal renal tubular acidosis.

OBJECTIVE: To evaluate complications in adequately treated children with distal renal tubular acidosis (RTA) and to identify factors influencing their development. DESIGN: Records of patients with primary distal RTA followed for 2 or more years at this hospital were reviewed. Case records were examined for age at onset of symptoms and at initiation of treatment, treatment details, follow-up and complications. Height, weight and growth velocity were expressed as standard deviation score (SDS) during different periods of follow-up. Regression analysis was performed to evaluate factors influencing increase in height and weight SDS. P value of less than 0.05 was considered significant. RESULTS: Of 18 patients (eleven boys), the diagnosis was established at the median (range) age of 6 yr (1.5-13 yr). These patients were followed up for a median (range) period of 4 yr (2-18.5 yr). Short stature (height SDS <-2) was noted in all patients at the time of diagnosis with median (range) height SDS of -5.2(-7.5 - -0.4). All patients had failure to thrive with median (range) weight SDS of -3(-5.7 - -1.5). Height SDS increased by median (range) of 2 (1.2-5.5) to become -2.7(-4.8 - -1.1) at last follow-up. Weight SDS increased by median (range) of 0.9 (-0.6 - 2.8) to become -2.4 (-4 - -0.5). Median (range) growth velocity SDS decreased from 3 (1-16) during first year of treatment to 1(-0.3 - 7) at four years with an increase in mean height SDS by 1.3 during the first two years of treatment. Height SDS at last follow-up was not influenced by the age at initiation of treatment, follow-up duration, initial height SDS or severity of acidosis at diagnosis. Increase in height SDS correlated negatively with base excess and height SDS at diagnosis, and positively with follow-up duration on univariate analyses. Initial height SDS was the only factor that influenced increase in height SDS on multivariate analyses. Increase in weight SDS was negatively correlated with base excess and initial weight SDS with significant association with initial weight SDS, on multivariate analyses. All patients had rickets at the time of diagnosis with pathological fractures in four. Rickets resolved without treatment with vitamin D in seventeen patients after a median (range) period of 4 mo (1-12 mo). Eight patients (44.4%) had nephrocalcinosis at diagnosis. Twenty-four hour urine calcium excretion was higher in those with nephrocalcinosis (p = 0.01). Creatinine clearance remained normal in all except one who progressed to renal failure. CONCLUSION: There is a need for early diagnosis, appropriate treatment and regular follow-up of patients with distal RTA for improving outcome.

Non-azotemic refractory rickets in Indian children.

In order to examine the etiology of refractory rickets, we evaluated the case records of patients presenting between 1990 and 2002. Subjects with impaired renal functions were excluded. Of 131 patients, 25.9 % each had hypophosphatemic rickets and distal renal tubular acidosis (RTA), 19.6 %vitamin D dependent rickets (VDDR), 11.3 % proximal RTA, 9.1 % liver disease and 6.1 % malabsorption. A significant proportion of patients with VDDR and proximal RTA showed deformities in the first year of life, whereas those with distal RTA and hypophosphatemic rickets presented later. Patients with hypophosphatemic rickets had predominant involvement of lower limbs; hypercalciuria was found in 4. Distal RTA was associated with marked rickets and normal levels of alkaline phosphatase. Hypophosphatemia and low tubular reabsorption of phosphate, though characteristic of hypophosphatemic rickets, was also seen in patients with VDDR (19.2%) and distal RTA (17.6 %). Our findings suggest that application and interpretation of appropriate investigations are useful in determining the cause of non-azotemic refractory rickets allowing initiation of specific therapy.

Renal tubular dysgenesis.

A neonate with acute renal failure due to renal tubular dysgenesis is described. The diagnosis was confirmed on renal histology, characterized by short and poorly developed proximal renal tubules.

Short term efficacy of intravenous dexamethasone and methylprednisolone therapy in steroid resistant nephrotic syndrome.

OBJECTIVE: To compare the short term efficacy of intravenous pulses of methylprednisolone and dexamethasone in treatment of steroid resistant nephrotic syndrome in children. METHOD: We prospectively treated 81 children with idiopathic steroid resistant nephrotic syndrome with six alternate-day pulses of intravenous dexamethasone (5 mg/kg) or methylprednisolone (30 mg/kg). Fifty-nine patients received dexamethasone and 22 were treated with methylprednisolone. Two patients in dexamethasone and one in methylprednisolone group developed serious infection during administration of alternate-day pulses and could not complete the therapy. RESULTS: The median age at treatment was 38 (36-74.7) months. Of patients who completed therapy, 20 (35.1 percent) (95 PERCNT CI 22.9-48.9) and 7 (33.1 percent) (95 percent CI 14.6-56.9) patients in dexamethasone and methylprednisolone group, respectively achieved complete remission. Following alternate day pu1ses the median urinary albumin to creatinine ratio decreased from 9.2 to 1.5 (P less tha 0.005) in dexamethasone group and from 12.1 to 0.7 (P less than 0.005) in methylprednisolone group. The median reduction in urinary albumin to creatinine ratio was 54.1 PERCNT (95 percent CI 32.7- 83.9) and 63.2 percent (95 percent CI 23.5- 100) in dexamethasone and methylprednisolone group respectively. The chief side effects of therapy were transient hypertension or worsening of preexisting hypertension, which occurred in 31 (54.4 percent) patients in dexamethasone group and 10 (47.6 percent) in the methylprednisolone group. The hypertension was satisfactorily controlled on antihypertensive drugs. One or more side effects were observed in 66.7 percent (95 percent CI 52.9-78.6) children receiving dexamethasone therapy and 61.9percent (95 percent CI 38.4-81.9) receiving methylprednisolone, which was comparable. CONCLUSIONS: We conclude that intravenous dexamethasone is as effective as methylprednisolone in inducing remission in patients with steroid resistant nephrotic syndrome.

Management of edema in nephrotic syndrome.

Nephrotic syndrome is a recurrent or chronic disorder characterized by heavy proteinuria, hypoalbuminemia and edema. In most patients, the edema can be managed satisfactorily with judicious oral administration of loop diuretics, most often frusemide. The treatment of patients with intractable edema is more difficult and comprises a series of measures including salt restriction, treatment with intravenous frusemide, additional use of thiazide and potassium sparing diuretics, and intravenous albumin. Ultrafiltration and head out water immersion are beneficial for treatment of patients with edema, which is unresponsive to the above measures.

Chronic renal failure in children.

OBJECTIVE: To determine the etiology of chronic renal failure amongst children attending the Pediatric Nephrology services at a tertiary care center. SUBJECT: We reviewed the records of 305 children, diagnosed to have chronic renal failure (CRF) over a 7-year period. CRF was defined as glomerular filtration rate (GFR) below 50-mL/1.73 m2/min persisting for more than 3 months. RESULTS: The mean age at onset and presentation of CRF in these patients was 5.9 and 8 years respectively. Ninety-six children were below 5 years of age. Mean (SD) levels of blood urea and creatinine at presentation were 150 and 4.6 mg/dL respectively. The median GFR at presentation was 18.5 mL/min/1.73 m(2) while 25.3 patients were already in end stage renal failure indicating that these patients were referred late. The mean (SD) hemoglobin at presentation was 7.6 (2.6) g/dL. The mean height and weight SD scores were 2.9 and 2.4 respectively. Obstructive uropathy was the commonest cause of CRF present in 97 children. Other causes included chronic glomerulonephritis in 84, reflux nephropathy in 51, hereditary nephritis in 20, renal dysplasia in 15 and hemolytic uremic syndrome in 5 children. The mean (SD) duration of follow-up was 11(15) months. Peritoneal or hemodialysis was performed in 63 patients. Fifteen patients underwent a live-related renal transplantation. The rest opted out of dialysis program and were conservatively managed due to financial constraints. CONCLUSIONS: The commonest causes of CRF were obstructive and reflux nephropathy. A significant proportion of patients presented late; had severe CRF and were malnourished and stunted. Majority of these were managed conservatively due to lack of financial resources.

Management of urinary tract infections.

Urinary tract infection (UTI) is common in childhood. It may result in long-term complications due to renal scaring. Younger children are at higher risk of renal scarring. The diagnosis of UTI is based on urine culture. The bacterial count for diagnosis of UTI depends on the method of urine collection. Urinalysis is useful for making a presumptive diagnosis of UTI and allows initiation of empirical treatment in high-risk patients, after urine culture has been obtained. The treatment of UTI is guided by the severity of illness and age of the patient. Following a UTI, investigation should be performed to identify an underlying urinary tract anomaly. Recurrence of UTI occurs in 30-50% children. Important predisposing factors include VUR, urinary tract obstruction, voiding dysfunction and constipation. Vesicoureteric reflux (VUR) is seen in 30-50% children with UTI. The cornerstone of management of VUR is long-term antibiotic prophylaxis, which has been found to be as effective as surgical reimplantation.