Clinical Profile and Outcome of Emergencies in Pediatric Chronic Kidney Disease

Objective: To describe the clinical profile and outcome of emergencies in children with chronic kidney disease (CKD). Methods: This retrospective analysis studied children with CKD presenting with acute emergencies. The clinical profile, renal and patient outcomes were compared between incidentally diagnosed - iCKD, previously diagnosed not on dialysis - pCKD and those on maintenance dialysis - dCKD groups. Results: 82 children (67 boys, median age – 8 years) with 99 visits were included. Uremic encephalopathy was the most common emergency in iCKD (64.7%) and pCKD (38.4 %), and access-related infections (32.1%) in dCKD group. Children with iCKD had higher Pediatric Risk of Mortality score (P<0.001), emergent initiation of dialysis (P=0.03) and discontinuation of treatment (P<0.001) when compared to the pCKD group. Conclusion: Uremic encephalopathy and access-related infections were the most common emergencies in children with CKD. Incidentally diagnosed CKD had a worse clinical profile and outcome.

NPHS2 Mutations in Indian Children with Sporadic Early Steroid Resistant Nephrotic Syndrome.

We examined the frequency and spectrum of podocin NPHS2 mutations in Indian children with sporadic steroid resistant nephrotic syndrome (SRNS). Of 25 children screened, only one (4%) had a pathogenic mutation resulting in a stop codon. The allele and genotype frequencies of the four known single nucleotide polymorphisms detected in the cohort were similar to that of controls. This finding emphasizes the need to screen for mutations in other genes involved in the pathogenesis of SRNS.

Profile of neonates admitted in pediatric ICU and validation of Score for Neonatal Acute Physiology (SNAP).

A retrospective study was conducted in the Pediatric Intensive Care Unit (PICU) of a tertiary care hospital in northern India to profile neonatal admissions and to assess the validity of the Score for Neonatal Acute Physiology (SNAP) in predicting the outcome in terms of mortality and length of hospital stay (LOS). Neonatal sepsis (51%) and birth asphyxia (11.2%) were the commonest indications for admission. Thirty-seven (38.1%) of the neonates died. The mean SNAP score in babies who died was 18.8 +/- 9.8 and 10.1 +/- 6.4 in survivors (P<0.001). There was no correlation between SNAP score and mean length of stay in hospital (P=0.5). We conclude that SNAP correlates well with mortality in neonates admitted to the PICU.

Acute lung injury and acute respiratory distress syndrome.

Acute lung injury and acute respiratory distress syndrome are an important challenge for pediatric intensive care units. These disorders are characterized by a significant inflammatory response to a local (pulmonary) or remote (systemic) insult resulting in injury to alveolar epithelial and endothelial barriers of the lung, acute inflammation and protein rich pulmonary edema. The reported rates in children vary from 8.5 to 16 cases / 1000 pediatric intensive care unit (PICU) admissions. The pathological features of ARDS are described as passing through three overlapping phases - an inflammatory or exudative phase (0-7 days), a proliferative phase (7-21 days) and lastly a fibrotic phase (from day 10). The treatment of ARDS rests on good supportive care and control of initiating cause. The goal of ventilating patients with ALI/ARDS should be to maintain adequate gas exchange with minimal ventilator induced lung injury. This can be achieved by use of optimum PEEP, low tidal volume and appropriate FiO2. High frequency ventilation can improve oxygenation but does affect the outcomes. Prone positioning is a useful strategy to improve oxygenation. Pharmacological strategies have not made any significant impact on the outcomes. Preliminary data suggests some role for use of corticosteroids in non-resolving ARDS. The mortality rates have declined over the last decade chiefly due to the advances in supporting critically ill patients.

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.