Physical growth in children with reflux nephropathy with normal or mildly impaired renal function.

Ten children aged 11 months to 10 years (means 5.7 years) with reflux nephropathy, vesicoureteric reflux (VUR) and normal or mildly impaired renal function having GFR more than 50 ml/min/1.72 m2, were included in the study. The hematological and biochemical parameters were within normal limits. Height standard deviation score (HZ score) was reduced at entry and, decreased further during follow-up (-2.2 and -2.6 at 0 and 12 months, respectively). Weight for height index (WHI) improved significantly (p=0.0004) during follow-up. The basal and stimulated peak growth hormone levels of these patients were found to be elevated, 18.53 ± 11.36 μg/L and 34.20 ± 5.86 μg/L, respectively. The IGF-1 levels were low ranging from 45.00 to 84.40 ng/dl (mean ± SD 61.54 ± 10.21 ng/dl) compared to 51.80 to 247.50 ng/dl (mean ± SD111.20 ± 70.24 ng/dl) in age and sex matched controls, indicating partial insensitivity to growth hormone.

Multicentric Castleman's disease in two cases.

Castleman's disease is an atypical lymphoproliferative disorder having two types of presentation--the localized and the multicentric form. The localized form presents as a slowly growing mass with a relatively benign course. Multicentric Castleman's disease has a more aggressive clinical course with diffuse lymph node enlargement and systemic illness. It is rarely seen in childhood and only thirteen cases have been reported in literature. This is the first report of 2 cases from the Indian subcontinent with a maximum follow-up of 44 months one of whom had asplenia.

Urinary tract infections: evaluation and treatment.

Urinary tract infections (UTI) are the second most common bacterial infection in children after those of the respiratory tract. These infections are important in view of their acute morbidity and the long-term risk of renal scarring. Occurrence of UTI below two years of age, delay in starting treatment and presence of vesicoureteric reflux or obstruction are the chief risk factors associated with renal scarring. The classical features of UTI are absent in young children, who often present with few signs or symptoms other than fever. Since the diagnosis of UTI warrants a thorough evaluation subsequently, empiric treatment based on symptoms or urinalysis alone should be avoided. Therapy with appropriate antibiotics is started only after obtaining a urine culture. The distinction between upper and lower urinary tract infections is difficult and the choice of therapy guided by the patient's age and severity of clinical manifestations. All children with UTI should be investigated to identify those with an underlying urinary tract anomaly.

Hypocalcemic heart failure masquerading as dilated cardiomyopathy.

Hypocalcemia is a rare, but reversible, cause of congestive heart failure. We report a 4-month-old boy diagnosed as dilated cardiomyopathy who had prolonged QoTc with low blood levels of calcium, normal phosphate, elevated alkaline phosphatase and findings suggestive of rickets. In view of non response to calcium and vitamin D3, a possible diagnosis of VDDR I (Vitamin D-dependent rickets) was made and he was treated with calcium and calcitriol. The serum calcium levels normalised within 10 days, along with resolution of the signs and symptoms of heart failure, near normal left ventricular function and normalisation of QoTc. Pediatricians should be aware of the association of hypocalcemia with cardiac dysfunction and should keep it as a possible reversible cause of heart failure in children.

Role of supravital staining of urine sediment and bright field microscopy in diagnosis of acute renal failure in bedside medicine.

BACKGROUND: An early accurate etiological categorization of acute renal failure (ARF) into acute glomerulonephritis (AGN), acute tubular necrosis (ATN) and acute interstitial nephritis (AIN) is very important in clinical medicine. METHODS: To evaluate the efficacy of a simple, cheap supravital staining method developed in our laboratory, we examined urine of 32 cases of ARF at their initial presentation in oliguric phase, which were later biopsied. The stain consisted of 1% crystal violet and 0.5% safranin in normal saline. The coverslip preparations of coded and stained urine sediments were examined under ordinary bright field microscope (BFM) by two independent observers. RESULTS: The renal biopsy showed 12 cases of AGN, 12 ATN and 8 AIN. The diagnosis could be predicted by supravital staining method in 75% cases with 95% uniformity among two observers with a sensitivity of 85.7% for AGN followed by AIN (80%) and ATN (75%). CONCLUSION: Thus this simple supravital staining technique can be used with ordinary BFM for accurate urine sediment analysis in cases of ARF in bedside medicine.

Sustained hypertension in children.

OBJECTIVE: To study the etiology and clinical profile of children with sustained hypertension. DESIGN: Retrospective hospital-based study. SETTING: Tertiary care, referral center. SUBJECTS: 246 children with sustained hypertension presenting between January 1983 and December 1996. RESULTS: The mean age at presentation was 8.2+/-3.9 yr; range 2 months-16 yr. There were 180 boys. An underlying cause for hypertension was identified in 242 (98.4%); 4 patients were considered to have essential hypertension. The chief causes included chronic glomerulonephritis (GN) in 121 (49.2%), obstructive uropathy in 39 (15.8%), reflux nephropathy in 30 (12.2%), thrombotic microangiopathy in 15 (6.1%) and renovascular disease in 14 (5.7%). Takayasu's disease was the most common cause of renovascular hypertension. Coarctation of aorta was the commonest cause of hypertension in infancy, being present in 53.3% of cases. In 198 subjects (80.5%) hypertension was detected as a feature of a known underlying disease. Thirty-five patients however, presented for the first time with complications of severe hypertension, including congestive cardiac failure in 21 and encephalopathy in 23. Thirteen patients presented with nonspecific symptoms and hypertension was detected on clinical examination. CONCLUSIONS: Most patients with sustained hypertension have an underlying etiology. A significant proportion of patients with renovascular and endocrine conditions may present, for the first time, with complications of hypertension.

An indigenously developed nitrite kit to aid in the diagnosis of urinary tract infection.

OBJECTIVE: To evaluate the utility of an indigenously developed nitrite kit for the rapid diagnosis of urinary tract infection (UTI) METHODS: 1018 urine specimens were collected from all cases where there was clinical suspicion of UTI. Samples were cultured as per standard microbiological protocol. Presence of nitrites was indicated by the development of purple color on addition of color developing solution and compared with the set of graded positive and negative controls also provided in the Kit. RESULTS: The results of the nitrite kit were compared with the semi-quantitative urine culture as the gold standard. The sensitivity, specificity, positive predictive and negative predictive values were 47%, 87%, 31% and 93%, respectively. CONCLUSION: Nitrite kit as a screening test can decrease the work load in the clinical bacteriology laboratory. More importantly in a field set up that is devoid of culture facilities, it can be used to correctly predict the absence of UTI.

Clinical profile of pulmonary nocardiosis.

Pulmonary nocardiosis mimics pulmonary tuberculosis in both clinical symptoms, being chronic in nature and radiological characteristics, and it is often wrongly treated with anti-tuberculosis drugs. The present study was undertaken to determine the prevalence of pulmonary nocardial infection in patients having chronic chest symptoms and to study their clinical response to specific chemotherapeutic agents. All the patients, who had a negative sputum for AFB on direct smear examination consecutively, were investigated for nocardiosis by examining the sputum with KOH preparation and modified Ziehl-Neelsen method. This was later confirmed by fungal culture of the sputum and inoculation on McClung's broth for paraffin baiting technique. Fibreoptic bronchoscopy was performed on all the suspected cases and the bronchial aspirate was examined similarly. The confirmed cases of nocardiosis were treated with cotrimaxazole and doxycycline for a total duration of six months. The prevalence of pulmonary nocardiosis in the present study was 1.9 percent. All the patients were immunocompetent. All the patients showed a good clinical response to chemotherapy at the end of six months of treatment. No relapse has been observed on follow up.

McCune Albright syndrome and hypophosphatemic rickets.

A 4 year-old girl had coxa vara, tibial bowing and hyperpigmented macules. The x-ray showed polyostotic fibrous dysplasia and evidence of rickets. Biochemical investigations confirmed hyperphosphaturic hypophosphatemic rickets. The literature has been reviewed.

Management of acute renal failure.

Acute renal failure (ARF) is defined as an abrupt decline in the renal regulation of water, electrolytes, and acid-base balance. It continues to be an important factor contributing to the morbidity and mortality of critically ill infants and children. The frequency of specific diseases that result in ARF differs among different age-groups and geographical areas. The common causes in Indian children include hemolytic uremic syndrome, acute tubular necrosis, glomerulonephritis and urinary tract obstruction. Though the hallmark of renal failure is oliguria, there is increasing recognition of non-oliguric ARF often associated with the use of nephrotoxic drugs. The basic principles of management are avoidance of life-threatening complications, maintenance of fluid and electrolyte balance and nutritional support. Specific management of the underlying disorder is possible only in a minority of cases. All the major dialysis modalities--peritoneal dialysis (PD), hemodialysis (HD) and continuous hemofiltration--can be used to provide equivalent solute clearance and ultrafiltration. Peritoneal dialysis requires minimal equipment and infrastructure, and is easy to perform; this makes it the favoured modality in developing countries where resources for HD or continuous therapies may not be accessible. However, continuous hemofiltration is an excellent alternative to PD in patients with ARF and severe fluid overload. The prognosis of children with renal failure depends on the underlying condition and associated medical complications.

Evaluation and treatment of chronic renal failure.

Chronic renal failure (CRF) is the irreversible deterioration of renal function that gradually progresses to end stage renal disease (ESRD). The chief causes of CRF include obstructive uropathy, primary glomerular diseases, reflux nephropathy and hypoplastic or dysplastic kidneys. Progressive hyperperfusion and hyperfiltration causes increasing glomerular injury and further renal damage. Symptoms of CRF are usually seen when GFR is between 10-25% of normal. Children with severe CRF often suffer from failure to thrive, growth retardation, acidosis, anemia and renal osteodystrophy. Management of CRF aims at retarding progression of renal damage and treatment of complications related to renal dysfunction. Measures suggested to retard progression include protein restriction, strict control of hypertension, use of angiotensin converting enzyme inhibitors and control of hyperlipidemia. Appropriate amounts of protein and calories are recommended to prevent growth failure. Nutritional supplements are often required. The availability of recombinant erythropoietin, calcitriol and human growth hormone has significantly improved the management of these patients. Once ESRD supervenes, renal replacement therapy in the form of chronic peritoneal or hemodialysis and transplantation is necessary.

Urinary net charge in hyperchloremic metabolic acidosis.

OBJECTIVE: (i) To examine the usefulness of urinary net charge (UNa + UK - UCl) in the evaluation of hyperchloremic metabolic acidosis secondary to diarrhea, distal RTA and proximal RTA and (ii) To characterize the type of distal RTA on the basis of the underlying defect. SETTING: Pediatrics division of a tertiary referral center. SUBJECTS: Thirty four children with hyperchloremic metabolic acidosis secondary to diarrhea (n = 16), distal RTA (n = 11) and proximal RTA (n = 7). Ten normal children with ammonium chloride induced acidosis were also studied. METHODS: All subjects underwent urine collection of 30-60 minutes duration for measurement of Na, K, Cl, pH and pCO2. The measurements were also made on the blood samples collected at the midpoint of urine collection. The urinary net charge was calculated by subtracting Cl values from the sum of the Na and K. RESULTS: Patients with proximal and distal RTA had a positive urine net charge. Patients with diarrhea and ammonium chloride induced acidosis showed negative urine net charge. Patients with diarrhea with extremely low urine sodium levels showed an inappropriately high urine pH despite persistent metabolic acidosis. All patients with distal RTA were found to have a secretory type of defect. CONCLUSION: Measurement of urine net charge is helpful in the initial evaluation of a patient with hyperchloremic metabolic acidosis.

Spontaneous lung hernia.

A case of spontaneous thoracic type of acquired lung hernia in a diabetic obese patient is presented in this report. The patient has been on conservative medical therapy for the last six months.

Peripheral blood lymphocyte subsets in idiopathic nephrotic syndrome of childhood.

Lymphocyte subsets in the peripheral blood were examined in 34 patients with nephrotic syndrome [27 with minimal change nephrotic syndrome (MCNS), 7 with significant lesions] and 10 normal children. The investigations were done at the onset of disease before administering corticosteroids (n = 34), during the subsequent remission (n = 27), and the first relapse (n = 12). The proportions of T cell subsets (CD3, CD4 and CD8) were determined by immuno-alkaline phosphatase staining; interleukin-2 receptor (IL-2 R) positive cells, natural killer (NK) cells and B cells were studied by indirect immunofluorescence. No significant differences were found in the proportion of CD3, CD4, CD8 and IL-2 R positive cells in patients with MCNS as compared to controls. An increased proportion of NK and B cells was not specific to MCNS and was detected even in those with significant renal lesions. Our findings suggest that T lymphocyte subsets in MCNS are similar to those in normal children.