Steroid Sensitive Nephrotic Syndrome: Revised Guidelines.

JUSTIFICATION: Steroid sensitive nephrotic syndrome (SSNS) is one of the most common chronic kidney diseases in children. These guidelines update the existing Indian Society of Pediatric Nephrology recommendations on its management. OBJECTIVE: To frame revised guidelines on diagnosis, evaluation, management and supportive care of patients with the illness. PROCESS: The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by review of literature and evaluation of evidence by experts in two face-to-face meetings. RECOMMENDATIONS: The initial statements provide advice for evaluation at onset and follow up and indications for kidney biopsy. Subsequent statements provide recommendations for management of the first episode of illness and of disease relapses. Recommendations on the use of immunosuppressive strategies in patients with frequent relapses and steroid dependence are accompanied by suggestions for step-wise approach and plan of monitoring. Guidance is also provided regarding the management of common complications including edema, hypovolemia and serious infections. Advice on immunization and transition of care is given. The revised guideline is intended to improve the management and outcomes of patients with SSNS, and provide directions for future research.

Hemolytic uremic syndrome in a developing country: Consensus guidelines.

BACKGROUND: Hemolytic uremic syndrome (HUS) is a leading cause of acute kidney injury in children. Although international guidelines emphasize comprehensive evaluation and treatment with eculizumab, access to diagnostic and therapeutic facilities is limited in most developing countries. The burden of Shiga toxin-associated HUS in India is unclear; school-going children show high prevalence of anti-factor H (FH) antibodies. The aim of the consensus meeting was to formulate guidelines for the diagnosis and management of HUS in children, specific to the needs of the country. METHODS: Four workgroups performed literature review and graded research studies addressing (i) investigations, biopsy, genetics, and differential diagnosis; (ii) Shiga toxin, pneumococcal, and infection-associated HUS; (iii) atypical HUS; and (iv) complement blockade. Consensus statements developed by the workgroups were discussed during a consensus meeting in March 2017. RESULTS: An algorithm for classification and evaluation was developed. The management of Shiga toxin-associated HUS is supportive; prompt plasma exchanges (PEX) is the chief therapy in patients with atypical HUS. Experts recommend that patients with anti-FH-associated HUS be managed with a combination of PEX and immunosuppressive medications. Indications for eculizumab include incomplete remission with plasma therapy, life-threatening features, complications of PEX or vascular access, inherited defects in complement regulation, and recurrence of HUS in allografts. Priorities for capacity building in regional and national laboratories are highlighted. CONCLUSIONS: Limited diagnostic capabilities and lack of access to eculizumab prevent the implementation of international guidelines for HUS in most developing countries. We propose practice guidelines for India, which will perhaps be applicable to other developing countries.

Profile of Acute Kidney Injury in Hospitalized Children with Idiopathic Nephrotic Syndrome.

OBJECTIVE: To determine the incidence, risk factors and outcome of acute kidney injury (AKI) in hospitalized children with nephrotic syndrome. METHODS: All consecutive hospitalized children (aged 1-14 years) with diagnosis of nephrotic syndrome between February 2016 and February 2017 were enrolled for the study. Children (aged 1-14 years) with features of nephritis, underlying secondary causes of nephrotic syndrome as well as children admitted for diagnostic renal biopsy and intravenous cyclophosphamide or rituximab infusion were excluded. RESULTS: A total of 73 children (81 admissions) were enrolled; incidence of AKI was 16% (95% CI, 9-23). On multivariate logistic regression analysis, furosemide infusion was observed as an independent risk factor for acute kidney injury (OR 23; 95% CI, 3-141; P<0.001). Out of 13 children with AKI, three died. CONCLUSIONS: Acute kidney injury in hospitalized children with nephrotic syndrome has high risk of mortality. Children receiving furosemide infusion should be closely monitored for occurrence of acute kidney injury.

Aplasia cutis congenita: a rare case with extensive symmetrically distributed lesions.

Aplasia cutis congenita is a rare developmental disorder of the skin of neonates, usually presenting as a solitary lesion over the scalp. We report an interesting presentation of AC along with the histopathological features in a neonate with extensive lesions over scalp as well as in bilaterally symmetrical areas over trunk and thighs; such symmetrical distributions being rarely reported.

Acute transverse myelitis: an unusual complication of typhoid fever.

Typhoid fever is associated with a wide spectrum of neurological complications. Acute transverse myelitis is a rare complication with only a few reports in adults and none in children. A 15-year-old boy with typhoid fever is reported who developed acute transverse myelitis in the 3rd week of illness. He was treated with antibiotics and corticosteroids and made a complete recovery.

Steroid-resistant nephrotic syndrome associated with steroid sulfatase deficiency-x-linked recessive ichthyosis: a case report and review of literature.

UNLABELLED: Nephrotic syndrome associated with X-linked recessive ichthyosis due to steroid sulfatase deficiency has rarely been reported in English literature. We describe a 4 and a half-year-old boy presenting with steroid-resistant nephrotic syndrome (SRNS) with an underlying ichthyotic skin present since birth. Renal biopsy revealed minimal change disease. As many of the male members of the family also showed similar skin manifestations, genetic analysis was done on the patient, which revealed deletion of the steroid sulfatase (STS) gene spanning both the 3' as well as the 5'ends. The patient was thus diagnosed with SRNS associated with X-linked recessive ichthyosis. He was started on cyclosporine regimen, and remission was achieved in 5 weeks. We speculate that the deficiency of STS resulting in increased cholesterol sulfate accumulation interferes with the integrity of adherens junctions present between glomerular epithelial cells of the slit diaphragm, and this results in proteinuria and nephrotic syndrome. The nephrotic syndrome remitted with a calcineurin inhibitor medication. CONCLUSION: We suggest that the deficiency of STS is another one in an increasing list of genetic causes of podocytopathy and nephrotic syndrome. Remission of proteinuria in such a case may be achieved with immunosuppressive medication.

Clinico-socio-demographic profile and predictors of poor outcome in children with liver abscess: a hospital-based study in northern India.

Liver abscess (LA) is a common health problem among children in tropical countries. We conducted a prospective, observational study at a tertiary-care medical college in order to examine the clinico-socio-demographic profile and assess a possible association between certain predictors of a poor outcome in children admitted with LA. Of a total of 53 children, only 24.4% were malnourished. Staphylococcus aureus was the most common organism observed. Amoebic LA was noted in 7.5%. Right-sided pleural effusion was the most common complication recorded (20.7%). The most successful treatment modality was therapeutic percutaneous aspiration with antimicrobials (90.5% success). An elevated alkaline phosphatase and open surgery were associated with a poor outcome (P = 0.04 in each case). The rate of hospital admissions of children with LA remains high and most are of pyogenic origin. Of the different treatment modalities, therapeutic aspiration along with antimicrobials achieved the best results. Raised alkaline phosphatase and open surgery were predictors of a poor outcome.