CLINICAL PROFILE OF WILSON DISEASE IN CHILDREN IN A TERTIARY CARE CENTRE IN SOUTH INDIA

2021 ◽  
pp. 29-30
Author(s):  
Sumathi Bavanandam

Background: Wilson disease (WD) is the most common metabolic liver disease in Indian children with late presentation mandating early identication and treatment to prevent disease related morbidity and mortality. Aim: To study the clinical prole of Wilson disease in a tertiary referral care centre for children in South India. Material & Methods: Retrospective descriptive analysis of medical records of children with Wilson disease over ve years from January 2014 to January 2018 admitted in Paediatric Gastroenterology department, Institute of child health & Hospital was done. Results: There were 75 children, 36 male 39 Female (1:1.08) with age ranging from 4 to 12 years. Clinical features include asymptomatic stage 7(9.3%). hepatic phenotype in 53 (70.6%) and neurophenotype in 15 (20%) children. Growth retardation was observed in 64 (85%), Kayser Fleischer ring in 30 (40%), Jaundice in 45 (60%), chronic liver disease in 40 (65.3%) out of which 21 (52.5%) children presented with decompensated liver disease and 14 children died during the study period with 18.7% mortality. Laboratory tests showed mean Hb 9.8 gm/dl SGOT 254 IU/L, SGPT 154 IU/L, albumin 2.2 gm/dl, low serum ceruloplasmin < 20mg/dl in 49 (65.3%), 24 hours urinary copper after D. Penicillamine challenge 680.4 µgm /day. Majority tolerated oral chelation therapy with D. Penicillamine except in 3. Conclusion:WD is the most common metabolic liver disease in children with chronic liver disease with 18.7%h mortality rate

2021 ◽  
Vol 5 (5) ◽  
pp. 01-05
Author(s):  
Parveen Malhotra ◽  
Vani Malhotra ◽  
Yogesh Sanwariya ◽  
Isha Pahuja ◽  
Ajay Chugh ◽  
...  

Introduction: It is important to determine the epidemiological factors like etiology, age, sex, mode of clinical presentation and pattern of development of complications of chronic liver disease, so as to design optimal and cost effective preventive and treatment strategies for the same. Aim: To determine etiology of Chronic liver disease in Northern India. Material and Methods: This was a prospective study done at Medical Gastroenterology Department, PGIMS,Rohtak conducted over a period of ten years i.e. 01.01.2011 to 31.12.2020, on 1000 confirmed patients of chronic liver disease (CLD). Results: The most common etiology seen was Alcoholic liver disease (48.9%), followed by Nonalcoholic liver disease (26.4%), Chronic Hepatitis B (12.3%), Chronic Hepatitis C (9%), Cryptogenic (2.7%) and Autoimmune liver disease related (0.7%). Conclusion: The present study reveals that alcohol is most common cause of chronic liver disease in Northern India. The males of middle age group with rural background are at significant risk of developing CLD, thus requiring immediate social and medical intervention.


2020 ◽  
Vol 5 (4) ◽  
pp. S48-S49
Author(s):  
Julius Scott ◽  
Deenadayalan M ◽  
Naresh Shanumgam ◽  
Mukul Vij ◽  
Priya Ramachandran ◽  
...  

2021 ◽  
Vol 12 (1) ◽  
pp. 1-14
Author(s):  
Lubomir Skladany ◽  
Tomas Koller ◽  
Svetlana Adamcova Selcanova ◽  
Janka Vnencakova ◽  
Daniela Jancekova ◽  
...  

AbstractChronic liver disease management is a comprehensive approach requiring multi-professional expertise and well-orchestrated healthcare measures thoroughly organized by responsible medical units. Contextually, the corresponding multi-faceted chain of healthcare events is likely to be severely disturbed or even temporarily broken under the force majeure conditions such as global pandemics. Consequently, the chronic liver disease is highly representative for the management of any severe chronic disorder under lasting pandemics with unprecedented numbers of acutely diseased persons who, together with the chronically sick patient cohorts, have to be treated using the given capacity of healthcare systems with their limited resources. Current study aimed at exploring potentially negative impacts of the SARS CoV-2 outbreak on the quality of the advanced chronic liver disease (ACLD) management considering two well-classified parameters, namely, (1) the continuity of the patient registrations and (2) the level of mortality rates, comparing pre-COVID-19 statistics with these under the current pandemic in Slovak Republic. Altogether 1091 registrations to cirrhosis registry (with 60.8% versus 39.2% males to females ratio) were included with a median age of 57 years for patients under consideration. Already within the very first 3 months of the pandemic outbreak in Slovakia (lockdown declared from March 16, 2020, until May 20, 2020), the continuity of the patient registrations has been broken followed by significantly increased ACLD-related death rates. During this period of time, the total number of new registrations decreased by about 60% (15 registrations in 2020 versus 38 in 2018 and 38 in 2019). Corresponding mortality increased by about 52% (23 deaths in 2020 versus 10 in 2018 and 12 in 2019). Based on these results and in line with the framework of 3PM guidelines, the pandemic priority pathways (PPP) are strongly recommended for maintaining tertiary care uninterrupted. For the evidence-based implementation of PPP, creation of predictive algorithms and individualized care strategy tailored to the patient is essential. Resulting classification of measures is summarized as follows: The Green PPP Line is reserved for prioritized (urgent and comprehensive) treatment of patients at highest risk to die from ACLD (tertiary care) as compared to the risk from possible COVID-19 infection. The Orange PPP Line considers patients at middle risk of adverse outcomes from ACLD with re-addressing them to the secondary care. As further deterioration of ACLD is still probable, pro-active management is ascertained with tertiary center serving as the 24/7 telemedicine consultation hub for a secondary facility (on a physician-physician level). The Red PPP Line is related to the patients at low risk to die from ACLD, re-addressing them to the primary care. Since patients with stable chronic liver diseases without advanced fibrosis are at trivial inherent risk, they should be kept out of the healthcare setting as far as possible by the telemedical (patient-nurse or patient- physician) measurements. The assigned priority has to be monitored and re-evaluated individually—in intervals based on the baseline prognostic score such as MELD. The approach is conform with principles of predictive, preventive and personalized medicine (PPPM / 3PM) and demonstrates a potential of great clinical utility for an optimal management of any severe chronic disorder (cardiovascular, neurological and cancer) under lasting pandemics.


2013 ◽  
Vol 2013 ◽  
pp. 1-6
Author(s):  
Vani Chandrashekar

The aim of this study was to identify common stool parasites in patients attending a tertiary care centre in South India. We evaluated 2355 stool samples and parasites were detected in 7.9% of samples. 41.1% of our patients were in the 45–58-year age group. Protozoal infections were the commonest seen in 7.8% of samples. Entamoeba histolytica was the commonest protozoa (4.6%) followed by Entamoeba coli (1.2%) and Giardia (0.8%). Entamoeba histolytica and Entamoeba coli were together seen in 0.63%, and they were the commonest organisms seen in samples with multiple-organism infection. Both were equally detected in diarrheal samples.


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