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UNASSIGNED: Adherence to medication(s) is an essential component of holistic management in any chronic disease including in post liver transplant (LT) patients. Thus, this study aimed to assess adherence to medications in Indian pediatric liver disease patients (including post LT recipients) and to identify variables affecting its occurrence.
UNASSIGNED: A cross-sectional study was conducted among pediatric (<18 years of age) subjects with Wilson disease (WD) and autoimmune liver disease (AILD) along with post LT recipients from May 2021 to October 2021. Structured tools using prevalidated questionnaires (Medication adherence measure and the Child & Adolescent Adherence to Medication Questionnaire) were used to collect data related to nonadherence prevalence (based on missed and late doses) and factors influencing the adherence.
UNASSIGNED: A total of 152 children were included in the study (WD 39.5%, AILD 32.9%, and post LT 27.6%). Prevalence of missed and late dose nonadherence (at a cut-off of ≥20%) was 12.5% and 16.4%, respectively. Older age (odd\'s ratio/O.R 1.185), stay in a rural area (O.R 5.08), and barriers like bad taste of medication (O.R 4.728) and hard to remember the medication (O.R 7.180) were independently associated with nonadherence (P < 0.05).
UNASSIGNED: Overall, nonadherence was seen in 12-16%, i.e., around one-sixth of the patients, with least nonadherence seen in post LT recipients (0-2.4%). Older age of the patient, rural place of stay and personal barriers like hard to remember/forgetfulness and bad medication taste were identified as factors independently leading to nonadherence.

UNASSIGNED: Wilson disease (WD) manifesting as seizure is rare. Rolandic epilepsy as presenting feature of WD has been reported only once before.
UNASSIGNED: A 6-year-old girl of nonconsanguineous parentage presented with focal seizures. There was associated fatty hepatomegaly and elevated aminotransferases.
UNASSIGNED: Brain magnetic resonance imaging (MRI) was unremarkable. Electroencephalogram demonstrated bilateral centrotemporal spike classical of Rolandic epilepsy. Serum ceruloplasmin was low and 24-h urinary copper levels were elevated. Genetic mutational analysis showed she carried the rare homozygous p.Asn1270Ser genetic mutation. Administration of d-penicillamine gradually halted seizure activity together with near normalization of serum aminotransferases.
UNASSIGNED: Rolandic epilepsy associated with elevated liver enzymes should undergo evaluation for WD. Chelators have a salutary effect on seizure activity, as well as elevated serum aminotransferases.

Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.

UNASSIGNED: Low serum ceruloplasmin levels can occur in patients without Wilson\'s disease (WD) liver disorders. When present, extensive, costly, and potentially harmful additional investigations for WD may be undertaken. The purpose of this study was to document the prevalence of low serum ceruloplasmin levels in adult patients without WD and describe the features commonly associated with this finding.
UNASSIGNED: Serum ceruloplasmin levels were measured by an enzymatic assay in 3040 adult patients attending an urban, liver diseases outpatient clinic.
UNASSIGNED: A total of 122 (4.0%) patients without WD had serum ceruloplasmin levels less than the lower limit of normal documented at their initial visit. Their mean age was 44 ± 14 years, and 80 (66%) were men. The Model for End-stage Liver Disease (MELD) score was 9.0 ± 4.0. Approximately, one half (65/122, 53%) had underlying viral hepatitis (52% hepatitis B and 48% hepatitis C). When compared with 64 MELD-matched control patients with normal or elevated serum ceruloplasmin levels, there were no significant differences in liver enzyme/function tests, ferritin, creatinine values, or survival. However, the low serum ceruloplasmin cohort patients were younger (43 ± 14 versus 52 ± 13 years, p = 0.0002), less often men (66% vs. 88%, p = 0.001), and viral hepatitis was significantly more common (53% versus 27%, p = 0.0005).
UNASSIGNED: Low serum ceruloplasmin levels were documented in 4.0% of adult patients without WD attending this urban liver diseases outpatient clinic. These patients tend to be younger, less often men, and more often have viral hepatitis as the underlying cause of their liver disease.

UNASSIGNED: Acute on chronic liver failure (ACLF) is an emerging entity whose unique pathogenesis, presentation, and outcome are different from those with decompensated cirrhosis. Patients with Wilson disease (WD) often present with ACLF. The outcome in this setting and predictors of mortality have not been well delineated. We describe the clinical features, laboratory characteristics, and prognostic factors in patients with WD with ACLF. We compared the outcome in those without criteria for ACLF.
UNASSIGNED: We analyzed the admission characteristics of 68 patients with WD presenting with features of ACLF among a cohort of WD patients from 1997 to 2017. WD was diagnosed as per European association for the study of the liver (EASL)/Leipzig criteria and ACLF by the Asia-Pacific Association of Study of Liver and World Gastroenterology Organization consensus criteria. Factors associated with mortality were analyzed by univariate followed by multivariate analysis and receiver operating characteristic curve.
UNASSIGNED: Of the 272 patients with WD, 68 fulfilled criteria for ACLF. The mean age was 14.4 years (Range 5-42 years). Males constituted 38/68 (56%). Acute viral or drug induced hepatitis as precipitating factors was seen in 11.7%. Forty-nine patients (49/67; 73%) died including 30/32 (93.8%) with encephalopathy and 45/62 (72.6%) with ascites. Prognostic factors on univariate analysis significant for mortality included encephalopathy, international normalized ratio, white blood cell count and model for end-stage liver disease (MELD) score. On multivariate analysis, only encephalopathy was significant with 82% accuracy in differentiating survivors versus non-survivors. Post mortem liver biopsy in 21 patients and explant biopsy in 2 patients showed features of cirrhosis in all.
UNASSIGNED: WD with ACLF is associated with a high mortality Precipitating factors such as viral and drug-induced hepatitis was seen in 11.7% patients. Liver histology in patients subjected to biopsy showed cirrhosis in all. Only encephalopathy is a prognostic marker of non-survival with an accuracy of 82%.

Hepatic encephalopathy (HE) is a complex syndrome of neurological and psychiatric signs and symptoms that is caused by portosystemic venous shunting with or without liver disease irrespective of its etiology. The most common presentation of Wilson disease (WD) is liver disease and is frequently associated with a wide spectrum of neurological and psychiatric symptoms. The genetic defect in WD leads to copper accumulation in the liver and later in other organs including the brain. In a patient presenting with Wilsonian cirrhosis neuropsychiatric symptoms may be caused either by the metabolic consequences of liver failure or by copper toxicity. Thus, in clinical practice a precise diagnosis is a great challenge. Contrary to HE in neurological WD consciousness, is very rarely disturbed and pyramidal signs, myoclonus dominate. Asterixis and many other clinical symptoms may be present in both disease conditions and are quite similar. However details of neurological assessment as well as additional examinations could help in differential diagnosis.