Author Response.

How to cite this article: Agrwal S, Pallavi, Jhamb U, Saxena R. Author Response. Indian J Crit Care Med 2024;28(5):518-519.

Childhood Steroid-resistant Nephrotic Syndrome: Long-term Outcomes from a Tertiary Care Center.

Introduction: Steroid-resistant nephrotic syndrome (SRNS) is a rare condition that accounts for about 10% to 20% of all nephrotic syndromes in children. While calcineurin inhibitors induce remission in the majority, the data on long-term outcomes are limited. This retrospective study aimed to look at the clinical profile, biopsy findings, and long-term treatment outcomes in children with SRNS. Methods: The records of all children (1-18 years) with SRNS with biopsy findings of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangioproliferative glomerulonephritis, who received treatment for a minimum period of 12 months and were in follow-up during the years 2007-2018 at a tertiary care teaching hospital were retrieved. The clinical, histopathological, and biochemical factors and treatment outcomes were recorded and analyzed. Results: Ninety-one (72 boys) children with a median (interquartile range [IQR]) age of onset of nephrotic syndrome as 48 (24-87) months were included. MCD and FSGS were the most common histopathological types (57.1% and 36.3%, respectively) and 62 (68.1%) patients had late steroid resistance. Calcineurin inhibitors (CNIs) were used in 86.8% of the children, and response rates with cyclosporine and tacrolimus for complete remission (CR) were 80% and 73.7%, respectively, with median (IQR) time to response being 3 (2-4) months. The presence of MCD on histology and the use of CNIs were significantly associated with CR (P < 0.01). At a median (IQR) follow-up of 5 (3-7) years, 76 (83.5%) children had either CR or partial remission, four (4.4%) developed chronic kidney disease and five (5.5%) died (three due to end-stage renal disease and two of infective complications). Conclusion: SRNS children with MCD on biopsy, late resistance, and response to CNIs have better long-term outcomes. Most patients respond to CNIs within the first 6 months of use and need therapy for at least 24 to 36 months.

Paroxysmal Sympathetic Hyperactivity in Neurocritical Children: A Pilot Study.

Background: Paroxysmal sympathetic hyperactivity (PSH) is characterized by the abnormal excessive sympathetic response to acute cerebral insult. There is a paucity of data about this condition in children. This study was planned to analyze the incidence of PSH among children requiring neurocritical care and its association with the outcome. Materials and methods: The study was conducted in the pediatric intensive care unit (PICU) of a tertiary care hospital over a period of 10 months. Children of age 1 month to 12 years admitted with neurocritical illnesses were included. Children who were declared brain dead after initial resuscitation were excluded from the study. The criterion laid by Moeller et al. was used for the diagnosis for PSH. Results: During the study period, 54 children requiring neurocritical care were included in the study. The incidence of PSH was 5/54 (9.2%). Additionally, 30 (55.5%) children had less than four criteria for PSH and were termed as "incomplete PSH." Children with all four criteria for PSH had a significantly longer duration of mechanical ventilation, PICU stay, and higher PRISM III scores. Children with less than four criteria for PSH also had a longer duration of mechanical ventilation and stay. However, there was no significant difference in mortality. Conclusion: Paroxysmal sympathetic hyperactivity is common in children with neurological illnesses admitted to the PICU and is associated with longer mechanical ventilation and stay in PICU. They also had higher illness severity scores. Timely diagnosis of the condition and appropriate management is required to improve the outcome of these children. How to cite this article: Agrwal S, Pallavi, Jhamb U, Saxena R. Paroxysmal Sympathetic Hyperactivity in Neurocritical Children: A Pilot Study. Indian J Crit Care Med 2022;26(11):1204-1209.

Chronic Hepatitis B and Nephrotic Syndrome in Children: Treatment Outcomes.

Hepatitis B-related glomerulonephritis (GN) is an uncommon but important cause of renal morbidity in children. While immunosuppressive therapy has been tried along with antivirals for treatment, some children may undergo spontaneous remission or achieve remission with antivirals alone. We retrospectively studied the outcomes of children with nephrotic syndrome (NS) and chronic hepatitis B infection treated at our nephrology clinic over a five years period; seven children were included of which six (86%) presented with NS and one with nephritic syndrome. Renal biopsy (done in 5 children) showed membranous GN in two (40%), membranoproliferative GN in one (20%), and focal segmental glomerulosclerosis in two (40%). Entecavir therapy was started in 6/7(86%) and four (57%) achieved remission after a median period of 2.7 months and achieved hepatitis B e-antigen seroconversion after mean duration of 1.2 years of treatment with entecavir; the remaining achieved remission with immunosuppression with calcineurin inhibitors.

Nephrotic Syndrome with Central Retinal Artery Occlusion: A Unique Presentation.

Childhood nephrotic syndrome is associated with significant morbidity because of recurrent relapses, infections, and episodes of thromboembolism. Thromboembolism in nephrotic syndrome may involve any major blood vessel. Timely recognition of symptoms and early initiation of anticoagulation therapy are important to avoid end-organ damage. We present here a case of a child with steroid-resistant nephrotic syndrome (SRNS) with bilateral central retinal artery occlusion (CRAO), whose vision improved with anticoagulation therapy.

Acute kidney injury in children hospitalized with a relapse of nephrotic syndrome: A short-term outcome study.

Children with nephrotic syndrome (NS) have a number of potential risk factors for the development of acute kidney injury (AKI) including intravascular volume depletion, infection, exposure to nephrotoxic medication, and renal interstitial edema. This study was aimed to determine the incidence of AKI in children hospitalized with a relapse of NS and its short-term outcome. This prospective observational study was conducted from February 2017 to January 2018 at a tertiary care teaching hospital. A total of 54 children and adolescents (1-18 years) hospitalized with a diagnosis of NS and relapse with/or without other complications were enrolled. Clinical data and examination were recorded. AKI was defined using the Kidney Disease Improving Global Outcomes (KDIGO) serum creatinine criteria and Pediatric Risk, Injury, Failure, Loss, End-Stage Renal Disease (p-RIFLE) classification. Children who developed AKI during the first two weeks of hospitalization were followed up till recovery or six weeks whichever was earlier to determine the outcome and factors predisposing to AKI. The mean age of the study population was 59.5 months and 35 (64.8%) patients were male. Of the 54 patients hospitalized, 42 (77.8%) were admitted with infection-associated relapses while 22.2% of children had relapse alone. Diarrhea and spontaneous bacterial peritonitis were the most common infections (26.1% each) followed by urinary tract infections in 19% and pneumonia in 14.3%. Twenty-three (42.6%) children developed AKI according to the KDIGO definition and 27 (50%) using the pRIFLE classification. Fourteen (60.9%) had stage 2 AKI while 21.7% had stage 3 AKI. Infections [odds ratio (OR) 1.24] and use of angiotensin-converting enzyme inhibitors (ACEI) (OR 2.3) were the most common predisposing factors for AKI. The mean recovery time for AKI was 7.34 days. Development of AKI was associated with prolonged hospital stay (12.57 vs.8.55 days P <0.01) and delayed recovery. At the end of follow-up all children recovered from AKI. The incidence of AKI in children hospitalized with complications of NS is high. While the occurrence of these AKI episodes may appear transient, a recurrence of such episodes may be detrimental to the long-term outcome of children with NS. Infections and the use of ACEI during relapses are risk factor for the occurrence of AKI.

Purulent pericardial effusion in children: Experience from a tertiary care center in North India.

BACKGROUND: Purulent pericarditis, if not recognized and managed timely, it can lead to significant morbidity and mortality. There are no guidelines for the management of purulent pericardial effusion in pediatric patients. AIM: The study describes our experience with the management of 22 patients admitted with a primary diagnosis of purulent pericardial effusion seen over a 7-year period. MATERIALS AND METHODS: Hospital records of 22 children admitted to the pediatric intensive care unit with purulent pericardial effusion during January 2012-December 2018 were retrospectively analyzed. RESULTS: The mean age of presentation was 4.6 years. The most common presentation was fever. History of antecedent trauma was present in 27.27% of patients. Empyema was the most common associated infection. Staphylococcus aureus was the most commonly isolated organism. Out of 22, pericardial drainage was done in 13 patients (59%). Only one of these patients required pericardiectomy later on. Six (27.2%) patients responded to antibiotics alone. Three (13.6%) patients died before any intervention could be planned. CONCLUSION: Echocardiography-guided percutaneous pericardiocentesis and pigtail catheter placement are a safe and effective treatment for purulent pericardial effusion. When pericardial drainage is not amenable, close monitoring of the size of effusion by serial echocardiography is required. Small residual pericardial effusion may be managed conservatively.

National Comprehensive Cancer Network Guidelines for Pediatric Acute Lymphoblastic Leukemia.

The National Comprehensive Cancer Network has recently published the first pediatric guidelines for the management of children, adolescent and young adults with acute lymphoblastic leukemia (ALL). The recommendations for diagnosis, work up, genetic evaluation, treatment and follow up of pediatric ALL have been provided. Genetic risk factors and newer therapeutic agents have been discussed. We highlight the major points in the guidelines.

AN UNUSUAL CASE OF FAMILIAL SYSTEMIC LUPUS ERYTHEMATOSUS WITH DISTAL RENAL TUBULAR ACIDOSIS AND HEMOLYTIC ANEMIA.

Systemic lupus erythematosus (SLE) in children is associated with renal involvement in a majority. While glomerular involvement is a common manifestation, tubular involvement is rare. Tubular dysfunctions previously described with SLE are renal tubular acidosis (Type 1 and less commonly type 4). Isolated renal tubular acidosis without glomerular involvement has not been reported in childhood SLE. We report an adolescent girl with SLE, autoimmune thyroiditis and distal RTA who subsequently developed autoimmune hemolytic anemia. During a follow up of almost 7 years the girl never developed any proteinuria while the RTA persisted. Also during this period her mother was also diagnosed with SLE manifesting as thrombocytopenia.

Goldston syndrome with congenital hepatic fibrosis: A rare cause of neonatal cholestasis.

Goldston syndrome (GS) is a rare association of Dandy-Walker malformation (DWM) and cystic renal dysplasia with or without hepatic fibrosis. It is considered to be a milder variant of Meckel Gruber syndrome (MGS) and shares features with Miranda syndrome. We reported a 22 day old infant with DWM and autosomal recessive polycystic kidney disease (ARPKD) who presented with cholestasis and acholic stools. Ultrasonography and magnetic resonance cholangiopancreatography (MRCP) confirmed the diagnosis of congenital hepatic fibrosis (CHF). The child improved with supportive treatment. CHF is a rare condition which may present as a syndromic association.

Hemophagocytic lymphohistiocytosis with neurological manifestations in an infant with scrub typhus: a rare fatal occurrence.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of unchecked activation of the immune system leading to phagocytosis of blood cells and proliferation of histiocytes in solid organs. HLH can be primary or secondary to infective, autoimmune and malignant conditions. Scrub typhus is an infective illness caused by Orientia tsutsugamushi, transmitted by mite. The illness ranges from mild fever with rash to severe multisystem illness. Scrub typhus has rarely been associated with secondary HLH. We report an infant with scrub typhus who progressed to develop HLH with central nervous system involvement with fatal outcome.