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Summary Background Vitamin B12 deficiency has been associated with a very wide spectrum of neurologic manifestations and the majority of cases occur in infants, pregnant women, and elderly people. In children, common neurological complications include neuropathy, neuropsychiatric features, infantile tremor syndrome, developmental delay, cognitive decline, spastic paraparesis due to subacute combined degeneration of cord, seizures, encephalopathy, extrapyramidal features, and neuropathy. Vitamin B12 is known to cause sensory ataxia, along with impaired position and vibration sense, as well as variable spasticity, as a part of subacute combined degeneration of the spinal cord. However, only a few cases of isolated cerebellar ataxia caused by vitamin B12 deficiency have been reported in published literature. Methods and results We are reporting a case of isolated cerebellar ataxia progressing over months in a 13-year-old boy. He also had associated knuckle hyperpigmentation, megaloblastic anemia and his magnetic resonance imaging of the brain was normal. Complete blood count showed hemoglobin of 8.6 gm/dl and peripheral smear showed macrovalocytes and few hypersegmented neutrophils. Serum vitamin B12 level was low (134 pg/mL). He was started on daily intramuscular vitamin B12 supplementation and he showed a favorable response after the first week. Conclusions Clinicians need to consider vitamin B12 deficiency as one of the rare etiological possibilities in children presenting with isolated subacute onset/chronic ataxia, as supplementation of this vitamin is likely to cause a complete reversal of ataxia in such children.

Early identification of children with decompensated chronic liver disease (DCLD) at risk of short-term mortality helps improve outcome. We aimed to evaluate the predictors of outcome and role of Child-Pugh, pediatric end-stage liver disease (PELD) and pediatric chronic liver failure sequential organ failure assessment (pCLIF-SOFA) score for prognosticating 28-day mortality in children with DCLD.DCLD children were prospectively evaluated with a clinico-laboratory proforma and followed for 28 days to determine outcome. Child-Pugh, PELD and pCLIF-SOFA were calculated at admission. Univariate and multivariate analysis was performed to identify the best predictors of outcome.A total of 110 children (74 boys, 96 [4-204] months) were enrolled and 37 (33.6%) died at 28 days. Significant risk factors for mortality were a higher international normalized ratio (hazard ratio [HR] 1.17; 95% CI 1.04-1.31; p 0.001) and bilirubin (HR 1.04; 95% CI 1.01-1.08; p 0.001), lower albumin (HR 0.46; 95% CI 0.27-0.77; p = 0.03) and sodium (HR 0.93; 95% CI 0.89-0.98; p = 0.01), absence of treatable etiology (HR 2.00; 95% CI 1.40-2.87; p = 0.001) and presence of organ failure (HR 3.22; 95% CI 1.98-10.58; p 0.001). Organ failure and serum sodium were independent predictors of poor outcome on multivariate analysis. pCLIF-SOFA (16 [9-22] vs. 9 [5-15]), Child-Pugh (11 [9-15] vs. 10 [8-14]) and PELD (22.2 [7.5-45.3] vs. 15.3 [4.5-23.9]) scores were significantly higher in non-survivors. The area under the curve was 0.977 for pCLIF-SOFA, 0.815 for Child-Pugh score, and 0.741 for PELD score. A pCLIF-SOFA score of ≥11 identified 28-day mortality with a sensitivity and specificity of 94.9% and 91.5%, respectively.Thirty-four percent of children with DCLD have a poor short-term outcome. Organ failure and low serum sodium are independent predictors of outcome. pCLIF-SOFA performs better than Child-Pugh and PELD in prognostication of 28-day mortality. Our study supports the use of scores based on organ failure in prognosticating children with DCLD.The ability to predict the course of a disease is an important part of the assessment, enabling timely interventions that improve outcomes. We evaluated the outcome (death vs. survival) and compared three different scoring systems for their ability to predict mortality within 28 days in children with decompensated chronic liver disease (DCLD). One-third of children with DCLD died within 28 days and the pediatric chronic liver failure sequential organ failure assessment score, which considers the main organ systems of the body (lungs, liver, brain, kidney, blood and cardiac) fared better for identification of children with a poor outcome than the Child-Pugh and pediatric end-stage liver disease score which comprise of only liver-related parameters. Our study supports the use of scores based on organ failure in prognosticating children with DCLD.

Background and aims Risk of infections is increased in patients with Acute Liver Failure (ALF) and Decompensated Chronic Liver Disease (DCLD). We evaluated the frequency, site, type and risk-factors for bacterial infections in children with ALF and DCLD and its effect on outcome. Methods ALF or DCLD children were enrolled prospectively. Clinical and laboratory details were recorded. Cultures (blood, urine and ascites) and chest X-ray were done at admission followed by weekly surveillance cultures. Results 173 patients, 68 ALF and 105 DCLD were enrolled. Infections were more common in DCLD than ALF (60/105 [57.1%] vs. 27/68 [39.7%]; P = 0.02). Ascitic fluid infection, pneumonia, urinary tract infection and bacteremia were seen in 19%, 17.9%, 13.2% and 12.1% patients respectively. Healthcare-Associated (HCA) infections were most frequent (39/87, 44.8%), followed by Nosocomial (NC, 32%) and Community-Acquired (CA, 23%). Nearly 3/4th of bacterial isolates were resistant to cephalosporins and quinolones, 23% being Multiresistant Bacteria (MRB). DCLD patients with infection had higher Child–Pugh Score (10 [6–14] vs. 7 [6–14]; OR 3.2 [1.77–5.10]: P = 0.007), need for ICU care (26/60 vs. 3/45; OR 10.70 [2.98–38.42]: P = 0.01), in-hospital mortality (24/60 vs. 8/45;OR 3.08 [1.22–7.75]: P = 0.04) and mortality at 3 month follow-up (32/60 vs. 9/45; OR 4.57 [1.87–11.12]: P = 0.00). Infection did not affect the outcome in ALF. Conclusion Infections develop in 40% ALF and 57% DCLD children. HCA and NC infections account for 77% of infections. Most culture isolates are resistant to cephalosporins and fluoroquinolones and 23% have MRB. Risk of infections is higher in DCLD patients with advanced liver disease.