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Background: Hematopoietic stem cell transplantation in primary immunodeficiency disorders has come a long way since the first transplant in 1968. In India, pediatric stem cell transplantation long-term survival outcomes range from 62.5% to 75%, compared to 90% in high-income countries. Objective: We present single-center data of primary immunodeficiency transplants with immune-reconstitution evaluation after transplantation from a charitable trust hospital. Methods: Retrospective data of children transplanted for primary immunodeficiency disorders from March 2019 to March 2022 in a newly established transplant unit were collected. Data of pretransplant infections and comorbidities, surveillance for carbapenem-resistant Enterobacteriaceae, transplant characteristics, donor source, graft-versus-host disease, posttransplant infections, immune reconstitution, overall survival at 1 year, and immunodeficiency-free survival were collated. Results: Twenty-one patients underwent transplantation for primary immunodeficiency disorders. The median age at transplantation was 3 years and 5 months (range, 7 months to 17 years). Seventy-five percent of the cohort had organ involvement, with lung being the most common organ involved, followed by central nervous system. Fifty-two percent of children had peritransplant infections, with most of them recognized at the pretransplant assessment. Among 20 of 21 children with engraftment, 94% had complete chimerism initially, with 33% developing mixed chimerism over time. The median duration of immunosuppression was 3 months after transplantation, and only 1 child required systemic graft-versus-host disease treatment for more than a year. Immune-reconstitution showed good T-cell recovery at 3 months and naive T-cell production at 6 months. There was no regimen-related or sepsis-related mortality. Overall survival of the cohort was 95% at 1-year follow-up. Immunodeficiency-free survival was 86% after a median follow-up of 20 months. Conclusions: Immunodeficiency-free and graft-versus-host disease-free survival can be achieved in the majority of children with primary immunodeficiencies using enhanced supportive care and the latest transplantation algorithms.
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Background. Methemoglobinemia is a potentially life-threatening condition which presents with cyanosis and characteristic "chocolate-coloured blood." Although a co-oximetry would give a prompt diagnosis, there have been multiple reports of misdiagnosing this treatable condition-from being diagnosed as sepsis to asthma and even being operated for "ruptured ectopic pregnancy." Here, we report a case which presented without the classical signs of poisoning and methemoglobinemia-without vomiting, cyanosis, or chocolate-coloured blood. We also discuss the common misconceptions regarding anemia physiology and the pitfalls in diagnosing this condition and warn the reader regarding the reflexive use of antidotes like methylene blue. Case Presentation. A well-grown 3-year old boy presented with an acute history of irritability, cola-coloured urine, and desaturation on examination. The child was pale, with tachypnoea and in failure. Blood smear was suggestive of severe hemolytic anemia. Methemoglobinemia was diagnosed on co-oximetry. By focussing on physiologic principles of management rather than a specific antidote, the child was discharged home, well and active within 3 days of intensive care admission.
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BACKGROUND: Multisystem inflammatory syndrome (MIS) associated with severe acute respiratory syndrome coronavirus (SARS-CoV-2) (MIS-C) in children is being increasingly reported across the world. MATERIALS AND METHODS: Children fulfilling the World Health Organization criteria of MIS-C needing pediatric intensive care unit between April 15 and July 26, 2020 were studied. RESULTS: There were 21 patients with median age of 7 years [interquartile range (IQR) 1.9-12.1], of which 11 were females. SARS-CoV-2 real-time polymerase chain reaction positive in 8/21 and/or antibody positive 16/21. Fever was present in all patients, and gastrointestinal symptoms being second most frequent (16/21). One child had aplastic anemia, while the rest had no comorbidities. Nearly all presented with shock (n = 20/21) and 90% needed vasoactive drugs with a median Vasoactive Inotropic Score of 40 (IQR 20-95). Thirteen children needed ventilatory support and one needed peritoneal dialysis. Nine children had left ventricular dysfunction and five had dilatation of coronaries on echocardiography. Inflammatory markers C-reactive protein [98 mg/dL (IQR 89-119)], serum ferritin [710 mg/dL (IQR 422-1,609)], and serum interleukin-6 levels [215 ng/L (IQR 43-527)] were uniformly elevated. Eighteen children received pulse methyl-prednisolone, eleven intravenous immunoglobulins, and four tocilizumab. Eighteen children (86%) were discharged home while three died. CONCLUSION: In our cohort, MIS-C was seen in previously healthy children with fever, gastrointestinal symptoms, and shock. Early and aggressive management of shock and immune modulation with methyl-prednisolone and intravenous immunoglobulin were used. HOW TO CITE THIS ARTICLE: Shobhavat L, Solomon R, Rao S, Bhagat I, Prabhu S, Prabhu S, et al. Multisystem Inflammatory Syndrome in Children: Clinical Features and Management-Intensive Care Experience from a Pediatric Public Hospital in Western India. Indian J Crit Care Med 2020;24(11):1089-1094.
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Subacute combined degeneration of the spinal cord (SACD), a condition resulting from vitamin B12 deficiency, generally affects elderly people. We present a 12-year-old girl, who, despite a non-vegetarian diet and no obvious etiological factors, developed SACD.
