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n among Indian population post HSCT and was associated with significant mortality. In majority of the cases, the cause is multifactorial and pose a diagnostic dilemma and challenges in therapy.
The objective of this study was to determine the outcome of children with tyrosinemia type 1 from India.
A retrospective observational study was conducted on 11 patients diagnosed with type I tyrosinemia under our care. Age at symptoms, age at diagnosis, age at starting 2-nitro-4-trifluoromethylbenzoyl-1,3-cyclohexanedione (NTBC), duration between diagnosis and initiation of NTBC, dose given, total duration of NTBC, and outcomes were noted.
Eleven children with a median age of 1.1 years (0.51-1.52) at onset of symptoms were included in the study. The median age at diagnosis was 1.76 years (0.95-2.43). Their current median age is 5.44 (2.36-8.80) years. Common clinical features at presentation were chronic liver diseasein 8 (72.72%), rickets in 2 (18.18%), and fulminant liver disease in 1 (9.09%) patient. Hepatomegaly was observed in all children, growth retardation in 9 (81.81%), coagulopathy in 8 (72.72%), and abdominal distention in 6 (54.54%) patients. The median duration of NTBC therapy was 13.5 (7-21.25) months. The median dose of NTBC was 1 (0.77-1) mg/kg/day. One (9.09%) patient died due to liver cell failure. Penicillin-Streptomycin However, she had received NTBC only for a month. Another patient developed hepatocellular carcinoma (HCC) and underwent liver transplantation. He could receive NTBC only for 2 months, although he was diagnosed to have tyrosinemia for over a 1 year. Eight patients are on treatment with NTBC and are doing well, and 1 patient is not on NTBC and continues to have renal tubular acidosis.
NTBC therapy is effective and improves the prognosis of tyrosinemia. A long-term follow-up is required to determine progression to HCC and need for liver transplantation.
NTBC therapy is effective and improves the prognosis of tyrosinemia. A long-term follow-up is required to determine progression to HCC and need for liver transplantation.
With ageing population and higher prevalence of nonalcoholic steatohepatitis (NASH) and hepatocellular carcinoma (HCC) in older patients, more and more living donor liver transplants (LDLTs) are being considered in this group of patients as eligibility for deceased donor liver transplant is restricted to those aged 65 years and younger. However, the short- and long-term outcomes of this group have not been reported from India, which does not have a robust national health scheme. The aim of this study was to provide guidelines for transplant in this group.
All patients aged 60 years and older (group 1) who underwent LDLT in our centre between January 2006 and December 2017 were studied. A propensity score-matched group in 12 ratio was created with comparable sex and Model for End-Stage Liver Disease score (group 2). The 2 groups were compared for duration of hospital stay, surgical complications, hospital mortality and 1-, 3- and 5-year survival.
Group 1 consisted of 207 patients, and group 2 had 414 patients. The number of patients in group 1 gradually increased with time from 4 in 2006 to 33 in 2017 accounting for 15% of total cases. Group 1 had more patients with viral hepatitis, NASH and HCC, and they had a higher 30-day mortality due to cardiorespiratory complications. Although 1- and 3-year survival was similar, the 5-year survival was significantly lower in group 1.
Five-year survival was lower in the elderly group due to cardiorespiratory complications and recurrence of HCC. Outcomes in the elderly group can be improved with better patient selection and preventing HCC recurrence.
Five-year survival was lower in the elderly group due to cardiorespiratory complications and recurrence of HCC. Outcomes in the elderly group can be improved with better patient selection and preventing HCC recurrence.
Diabetes has been linked with poorer outcomes in coronavirus disease (COVID-19) patients. However, the question to whether continue or withdraw metformin therapy in COVID-19 patients with type 2 diabetes mellitus remains contentious. This study aims to investigate the association between metformin and poor COVID-19 outcomes.
Eligible studies published up to 21 October 2020 were included and appraised for validity, importance, and applicability. The included studies were further ranked according to the level of evidence (LOE).
Nine studies were included for further assessments, of which seven studies stated that metformin was not associated with poor COVID-19 outcomes (LOE II-V), while the other two with poorer designs stated otherwise (LOE V). Although metformin may increase the risk of developing acidosis and lactic acidosis (LOE IV), the observed risks were more accentuated in patients with severe COVID-19 disease or kidney impairment and in patients with > 2 daily metformin doses. Interestingly, one study revealed that metformin may even yield therapeutic role in reducing the risk of COVID-19 mortality (LOE II), although further studies are required to confirm these findings.
Our findings indicated that metformin may be safely continued in COVID-19 patients. The benefit of metformin therapy with simultaneous continuous monitoring of COVID-19 severity and kidney function may outweigh the risks of lactic acidosis, of which incidence is relatively rare.
The online version contains supplementary material available at 10.1007/s13410-021-00924-w.
The online version contains supplementary material available at 10.1007/s13410-021-00924-w.
Resident milestones are objective instruments that assess the resident's growth, progression in knowledge, and clinical diagnostic reasoning; but they rely on the subjective appraisal of the supervising attending. Little is known about the use of standardized instruments that may complement the evaluation of resident diagnostic skills in the academic setting.
Evaluate a modified bronchiolitis severity assessment tool by appraising the inter-rater variability and reliability between pediatric attendings and pediatric residents.
Cross-sectional study of children under 24 months of age who presented to a Community Hospital's Emergency Department with bronchiolitis between January-June 2014. A paired pediatric attending and resident evaluated each patient. Evaluation included age-based respiratory rate (RR), retractions, peripheral saturation, and auscultation. Cohen's kappa (K) measured inter-rater agreement. Inter-rater reliability (IRR) was assessed using a one-way random, average measures intra-class correlation (ICC) to evaluate the degree of consistency and magnitude of disagreement between inter-raters.
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