Disfunção hepática em pacientes de unidade de terapia intensiva médica prevê desfecho desfavorável / Hepatic dysfunction in medical intensive careunit patients predicts poor outcome

ABSTRACT Background: A large number of patients admitted to the medical intensive care unit (MICU) have abnormal liver function tests (LFT). This includes patients with critical illness with or without preexisting liver disease and patients with acute primary liver injury. There are very few studies which have investigated the spectrum of liver disease, clinical profile and outcome in patients admitted to the MICU. Objective To evaluate the occurrence, etiology, clinical profile, laboratory profile and outcome of hepatic dysfunction in patients admitted to the MICU. To evaluate the utility of model for end-stage liver disease (MELD) score on admission as a predictor of adverse short term outcome in patients with hepatic dysfunction admitted in MICU. Methods: It was a prospective observational study, conducted from December 2017 to December 2018 in a tertiary care hospital. Two hundred and two patients admitted to the MICU with LFTs as per the inclusion criteria were analyzed and their short-term outcome at 7 days was studied in relation to various parameters. Results: LFT abnormalities were present in 202/1126 (17.9%) of the patients admitted to MICU. Critical illness associated liver dysfunction was found in 172 (85.2%) patients, chronic liver disease in 11 (5.4%) patients and acute viral hepatitis in 19 (9.4%) patients. Most common symptom was fever (68.3%) followed by vomiting (48.0%). Among LFT abnormalities, elevated transaminases, raised international normalized ratio and high MELD score on admission correlated with poor short-term outcome. Requirement for inotropes and mechanical ventilation correlated with poor short-term outcome. Mortality did not differ significantly between patients with chronic liver disease, patients with acute viral hepatitis and patients with critical illness associated hepatic dysfunction. Hepatic dysfunction in MICU was associated with poor outcome and a high short-term mortality of 56.4% (114/202). Conclusion: Liver function abnormality is common in patients who are admitted to the MICU and its presence is an indicator of poor short-term outcome.

RESUMO Contexto: Um grande número de pacientes internados na unidade de terapia intensiva (UTI) tem testes de função hepática anormais (TFH). Isso inclui pacientes com doença crítica com ou sem doença hepática pré-existente e pacientes com lesão hepática primária aguda. Há poucos estudos que têm investigado o espectro da doença hepática, perfil clínico e desfecho em pacientes admitidos em UTI. Objetivo Avaliar a ocorrência, etiologia, perfil clínico, perfil laboratorial e desfecho de disfunção hepática em pacientes internados na UTI médica. Avaliar a utilidade do modelo para doença hepática em estágio terminal (MELD). Escore na admissão como preditor de desfecho adverso a curto prazo em pacientes com disfunção hepática admitida em UTI. Métodos: Foi realizado um estudo observacional prospectivo, de dezembro de 2017 a dezembro de 2018 em um hospital de atenção terciária. Foram analisados 202 pacientes internados na UTI com TFH conforme os critérios de inclusão e seu desfecho a curto prazo de 7 dias foi estudado em relação a diversos parâmetros. Resultados: Anormalidades dos testes estiveram presentes em 202/1126 (17,9%) dos pacientes internados na UTI. Doença crítica associada à disfunção hepática foi encontrada em 172 (85,2%) pacientes, doença hepática crônica em 11 (5,4%) pacientes e hepatite viral aguda em 19 (9,4%) pacientes. O sintoma mais comum foi a febre (68,3%), seguido de vômito (48,0%) casos. Entre as anormalidades do TFH, transaminases elevadas, INR e escore MELD elevados na admissão correlacionaram-se com desfecho ruim de curto prazo. Exigência de inotrópicos e ventilação mecânica correlacionaram-se com desfecho de curto prazo ruim. A mortalidade não diferiu significativamente entre pacientes com doença hepática crônica, pacientes com hepatite viral aguda e pacientes com doença crítica associada à disfunção hepática. A disfunção hepática em UTI esteve associada a um desfecho ruim e à uma alta mortalidade a curto prazo de 114/202 (56,4%). Conclusão: A anormalidade da função hepática é comum em pacientes que são admitidos nas unidades de tratamento intensivo e sua presença é um indicador de desfecho de curto prazo ruim.

Spontaneous Ascitic Fluid Infection: Are we Experiencing an Epidemiological shift in Causative Organisms?

Background: Spontaneous ascitic fluid infection (SAI) is common in cirrhotic patients leading to significant morbidity and mortality. Third-generation cephalosporins are currently recommended as first-line therapy. This is a retrospective observational study that aims to determine bacterial etiology, susceptibility patterns of SAI, and its correlation with model for end-stage liver disease-sodium (MELD-Na) and Child–Turcotte–Pugh (CTP) score. Materials and methods: The present study was conducted on 274 consecutive cases admitted in Bombay Hospital and Medical Research Centre, Mumbai, India. Cases of cirrhosis (irrespective of etiology) with ascites between the ages of 18–85 years were included in this study. Ascitic fluid of every patient was aspirated under all aseptic measures and was sent for biochemical, culture, and cytological analysis. Results: Of the 274 patients studied, 34 (12.4%) patients were diagnosed to have SAI. Culture-negative neutrocytic ascites (CNNA) was present in 27 patients, spontaneous bacterial peritonitis (SBP) was present in six patients, and monomicrobial bacteriascites was seen in one patient. Mean age of patients enrolled was 56.05 ± 2.47 years. Eighty-two percent were males and 18% were females. Alcohol (45.45%) was the leading cause of cirrhosis followed by nonalcoholic steatohepatitis (NASH) related cirrhosis (26.47.7%) and hepatitis C virus (HCV) related cirrhosis (11.46%) and cryptogenic cirrhosis (8.82%). Average MELD-Na score was 25 and the CTP class C was most common. Klebsiella pneumoniae was the most commonly isolated organism followed by Escherichia coli. The various factors that predispose to development of SBP include low ascitic fluid protein concentration, a high level of serum bilirubin, deranged serum creatinine, high Child–Pugh score, and high MELD-Na score. Conclusion: Ascitic fluid analysis remains the single most important test for identifying and assessing a course of SBP. Early diagnosis and treatment will reduce the mortality rate in these patients.

Pattern of liver function test variations in COVID-19 infection & its clinical significance: A study from a dedicated COVID-19 tertiary care centre from India

Background & objectives: Coronavirus disease 2019 (COVID-19) affects respiratory, gastrointestinal, cardiovascular and other systems disease. Studies describing liver involvement and liver function test (LFT) abnormalities are sparse from our population. This study was undertaken to estimate the LFT abnormalities in patients with COVID-19 in a tertiary care set up in India. Methods: In this retrospective study conducted at a tertiary care centre in Mumbai, India, all consecutive patients with proven COVID-19 by reverse transcriptase–PCR from March 23 to October 31, 2020 were enrolled. Of the 3280 case records profiled, 1474 cases were included in the study. Clinical characteristics, biochemical parameters and outcomes were recorded. Results: Overall 681 (46%) patient had deranged LFTs. Hepatocellular type of injury was most common (93%). Patients with deranged LFTs had more probability of developing severe disease (P<0.001) and mortality (P<0.001). Advanced age (P<0.001), male gender (P<0.001), diabetes mellitus (P<0.001), lower oxygen saturation levels at admission (P<0.001), higher neutrophil–lymphocyte ratio (P<0.001), history of diabetes mellitus and cirrhosiss were associated with deranged LFTs. Acute liver injury was seen in 65 (4.3%) cases on admission and 57 (3.5%) cases during hospital stay. On multivariate analysis for predicting mortality, age >60 yr serum creatinine >2 mg%, PaO2/FiO2 ratio ?200 and raised AST >50 IU/l (OR: 2.34, CI: 1.59-3.48, P<0.001) were found to be significant. Interpretation & conclusions: In COVID-19, LFT abnormalities were common, and derangement increased as severity progressed. The presence of deranged LFT worsens the clinical outcome and predicts in-hospital mortality.

Artificial Intelligence in Medicine

Artificial intelligence as the name suggests is intelligence given to machines by man. AI learns and performs like humans without human instructions. We encounter AI in daily activities, like music and video suggestions in video applications, and personalize what we see on Facebook, Twitter, Instagram. AI makes our day-to-day life easier, efficient and it increases the speed and accuracy of our efforts. In this article, we reviewed previous articles and internet sites to understand the general principles of artificial intelligence. We included general articles and few articles related to medicine to understand the basics of AI and its application in the medical field. A literature search was done using the following search terms: ‘AI’ and ‘AI in medicine’

Gallbladder Polyp: Review and Proposed Algorithm for Management

Gall bladder polyp is a lesion which is often an incidentally reported finding on ultrasonography. Such patients may or may not have symptoms of gall bladder disease. Although majority of polyps are cholesterol polyps, some are malignant. The challenge is early detection of malignant polyp and cholecystectomy before it becomes invasive or if invasive to carry out adequately wide surgical excision; while avoiding surgery in asymptomatic patients with benign disease. Following are the characteristics suggestive of malignancy in polyp: - size more than 10 mm, solitary lesions, increase in size over time, adenomatous polyps, sessile lesions and associated gallbladder wall thickening. Incidence of malignancy is higher in patients with Indian ethnicity, age more than 50 years, associated primary sclerosing cholangitis or gall stone disease. Pre-operative tissue diagnosis is difficult to obtain and review ultrasonography, contrast enhanced CT scan, endosonography and a regular follow-up can help the surgeon take the appropriate decision.

Combined ns5a & ns5b nucleotide inhibitor therapy for patients with chronic hepatitis c with stage 5 chronic kidney disease on hemodialysis / Terapia inibidora de nucleotídeo NS5A& NS5B combinada para pacientes com hepatite C crônica com doença renal crônica em estágio 5, em hemodiálise

ABSTRACT BACKGROUND: Hepatitis C virus (HCV) infection is the most common hepatotropic viral infection affecting the patients on maintenance hemodialysis. Treatment of chronic HCV infection in stage 4 and 5 CKD includes a combination of elbasvir/grazoprevir and glecaprevir/pibrentasvir, which are not available in many countries. OBJECTIVE: Hence, we have conducted this study to look for the safety and efficacy of sofosbuvir combination therapy in this difficult to treat population. METHODS: We conducted a single-center, prospective, open-label study in which Stage 5 CKD patients on maintenance hemodialysis with HCV infection. Total of 18 patients was included. sofosbuvir with daclatasvir or ledipasvir was used according to genotype for 12 weeks. HCV RNA, genotype, transient elastography (TE) was considered for every patient. HCV RNA was quantified at 4th week, 12th week and 12 weeks post-treatment to look for sustained virologic response (SVR 12). RESULTS: Infection due to genotype 1 was seen in 12 (66.7%) patients followed by genotype 3 in 4 (22.3%) with each patient of genotype 2 and 5. The median value of HCV RNA was 2,35,000 IU/mL. On TE, all had liver stiffness of <9.4 KPa. All patients had HCV RNA of <15 IU/mL at 4th and 12th week of treatment and 12 weeks post-treatment. No significant change in hemoglobin, eGFR and liver stiffness was observed. CONCLUSION: Full dose sofosbuvir i.e. 400 mg, in combination with NS5A inhibitors daclatasvir or ledipasvir is found to be safe and effective in patients with end stage renal disease, who are on maintenance hemodialysis.

RESUMO CONTEXTO: A infecção pelo vírus da hepatite C (HCV) é a infecção viral hepática mais comum que afeta pacientes em hemodiálise de manutenção. O tratamento da infecção crônica por HCV no estágio 4 e 5 da doença renal crônica inclui uma combinação de elbasvir/grazoprevir e glecaprevir/pibrentasvir, que não estão disponíveis em muitos países. OBJETIVO: Portanto, realizamos este estudo para procurar a segurança e eficácia da terapia combinada de sofosbuvir nesta população de difícil tratamento. MÉTODOS: Realizamos um estudo de centro único, prospectivo e aberto, no qual pacientes com doença renal crônica em estágio 5 em hemodiálise de manutenção com infecção por HCV. Um total de 18 pacientes foi incluído. Sofosbuvir com daclatasvir ou ledipasvir foi usado de acordo com o genótipo por 12 semanas. O HCV RNA, genótipo, elastografia transitória foi considerado para cada paciente. O HCV RNA foi quantificado na 4ª semana, 12ª semana e 12 semanas após o tratamento para procurar uma resposta virológica sustentada. RESULTADOS: A infecção por genótipo 1 foi observada em 12 (66,7%) pacientes, seguido pelo genótipo 3 em 4 (22,3%), em um paciente do genótipo 2 e em outro, 5. O valor mediano do HCV RNA foi de 2.35.000 IU/mL. Na elastografia transitória, todos tinham rigidez hepática de <9.4 KPa. Todos os pacientes tinham RNA HCV <15 IU/mL na 4ª e 12ª semana de tratamento e 12 semanas após o tratamento. Não foi observada nenhuma alteração significativa na hemoglobina, eGFR e rigidez hepática. CONCLUSÃO: A dose completa sofosbuvir ou seja, 400 mg, em combinação com inibidores NS5A daclatasvir ou ledipasvir foi considerada segura e eficaz em pacientes com doença renal em estágio final, que estão em manutenção hemodiálise.

Utilidade clínica dos escores de risco no sangramento varicoso / Clinical utility of risk scores in variceal bleeding

ABSTRACT BACKGROUND: Variceal bleeding remains important cause of upper gastrointestinal bleed. Various risk scores are used in risk stratification for non-variceal bleed. Their utility in variceal bleeding patients is not clear. This study aims to compare probability of these scores in predicting various outcomes in same population. OBJECTIVE: This study aims to compare probability of these scores in predicting various outcomes in same population. To study characteristics and validate AIMS65, Rockall, Glasgow Blatchford score(GBS), Progetto Nazionale Emorragia Digestiva (PNED) score in variceal Upper Gastrointestinal Bleed (UGIB) patients for predicting various outcomes in our population. METHODS: Three hundred subjects with UGIB were screened prospectively. Of these 141 patients with variceal bleeding were assessed with clinical, blood investigations and endoscopy and risk scores were calculated and compared to non-variceal cases. All cases were followed up for 30 days for mortality, rebleeding, requirement of blood transfusion and need of radiological or surgical intervention. RESULTS: Variceal bleeding (141) was more common than non variceal (134) and 25 had negative endoscopy. In variceal group, cirrhosis (85%) was most common etiology. Distribution of age and sex were similar in both groups. Presence of coffee coloured vomitus (P=0.002), painless bleed (P=0.001), edema (P=0.001), ascites (P=0.001), hemoglobin <7.5 gms (P<0.001), pH<7.35 (P<0.001), serum bicarbonate level <17.6 mmol/L (P<0.001), serum albumin<2.75 gms% (P<0.001), platelet count <1.2 lacs/µL (P<0.001), high INR 1.35 (P<0.001), BUN >25mmol/L (P<0.001), and ASA status (P<0.001), high lactate >2.85 mmol/L (P=0.001) were significant. However, no factor was found significant on multivariate analysis. Rockall was found to be significant in predicting mortality and rebleed. AIMS65 was also significant in predicting mortality. GBS was significant in predicting blood transfusion and need of intervention. PNED score was significant in all events except mortality. CONCLUSION: All four scores had lower predictive potential in predicting events in variceal bleed. However, AIMS65 & Rockall score were significant in predicting mortality, while GBS in predicting need of transfusion and intervention. PNED score was significant in all events except mortality.

RESUMO CONTEXTO: O sangramento varicoso permanece como importante causa de sangramento gastrointestinal superior. Vários escores são utilizados na estratificação do risco para sangramento não varicoso. Sua utilidade em pacientes de sangramento varicoso não é clara. OBJETIVO: Este estudo tem como objetivo comparar a probabilidade desses escores em prever vários desfechos na mesma população. Estudar característica e validar o AIMS65, o Rockall, a Pontuação de Glasgow Blatchford (GBS), o escore Progetto Nazionale Emorragia Digestiva (PNED), na pontuação em hemorragia gastrointestinal varicosa superior (UGIB) em pacientes para prever vários resultados em nossa população. MÉTODOS: Um total de 300 indivíduos com UGIB foram rastreados prospectivamente. Destes, 141 pacientes com sangramento varicoso foram submetidos à avaliação clínica, hematológica e endoscopia tendo seus escores de risco calculados e comparados aos casos não-varicosos. Todos os casos foram acompanhados por 30 dias para mortalidade, necessidade de transfusão sanguínea por ressangramento ou de necessidade de intervenção radiológica ou cirúrgica. RESULTADOS: O sangramento varicoso (141) foi mais comum do que não varicoso (134) e em 25 teve endoscopia negativa. No grupo varicoso, a cirrose foi a etiologia mais comum (85%). A distribuição da idade e do sexo foi semelhante em ambos os grupos. Presença de vômito colorido em borra de café (P=0,002), sangramento indolor (P=0,001), edema (P=0,001), ascite (P=0,001), hemoglobina <7,5 GMS (P<0,001), pH <7,35 (P<0,001), nível de bicarbonato sérico <17,6 mmol/L (P<0,001), albumina sérica <2,75 GMS% (P<0,001), contagem plaquetária <1,2 Lacs/μL (P<0,001), INR elevada 1,35 (P<0,001), Bun >25 mmol/L (P<0,001) e estado ASA (P<0,001), lactato elevado >2,85 mmol/L (P=0,001) foram significativos. Entretanto, nenhum fator foi encontrado como significativo na análise multivariada. Rockall foi significativo em prever a mortalidade e ressangrar. O AIMS65 também foi significante na predição da mortalidade. O GBS foi significativo na predição de transfusão sanguínea e necessidade de intervenção. O escore de PNED foi significante em todos os eventos, exceto mortalidade. CONCLUSÃO: Todos os quatro escores apresentaram menor potencial preditivo na predição de eventos em sangramento varicoso. Entretanto, o AIMS65 e o escore de Rockall foram significantes na predição da mortalidade, enquanto o GBS na predição da necessidade de transfusão e intervenção. O escore de PNED foi significante em todos os eventos, exceto mortalidade.

Perfil clínico, teste padrão da resistência à droga e resultados do tratamento de pacientes com tuberculose abdominal da Índia ocidental / Clinical profile, drug resistance pattern and treatment outcomes of abdominal tuberculosis patients in western india

ABSTRACT BACKGROUND: Abdominal tuberculosis is an increasing problem in developing world. OBJECTIVE: The objective of the study was to describe the clinical presentations, drug resistance pattern and treatment outcomes of abdominal tuberculosis in Western India. METHODS: All the cases of abdominal tuberculosis from May 2014 to April 2017, diagnosed on the basis of clinical profile and gross morphological findings at endoscopy, imaging, followed by histology and/or GeneXpert and MGIT culture were included. All patients received antitubercular drug (AKT) therapy according to national protocol. Patients were followed from diagnoses till completion of treatment and various parameters were studied. RESULTS: Out of the 176 patients, 48% were males. Abdominal pain was most common complaint in 83.5%. On colonoscopy terminal ileum and ileocaecal valve were most commonly involved segments. Upper gastrointestinal tract was involved in four patients. Overall ulceronodular lesions were most common followed by ulcerative/nodular lesion. Strictures in bowel were seen in 28 (15.9%) patients with ileocaecal valve being most commonly involved, of which 23 had symptomatic relief with AKT and only three required dilatation. Histopathology showed granuloma in 80.8% cases. MGIT was positive in 43 (35.80%) cases and GeneXpert was positive in 35 (26.1%) cases. Eight patients had multi drug resistant tuberculosis. Only two patients required surgical management. CONCLUSION: Abdominal tuberculosis with wide spectrum of presentation, can still be managed with early diagnosis and treatment even in patients with sub acute intestinal obstruction. Weight gain or resolving symptoms were considered early markers of treatment response. Patients with stricture can become asymptomatic with medical treatment alone.

RESUMO CONTEXTO: A tuberculose abdominal é um problema crescente no mundo em desenvolvimento. OBJETIVO: O objetivo do estudo foi descrever as apresentações clínicas, o padrão de resistência a fármacos e os desfechos de tratamento da tuberculose abdominal na Índia ocidental. MÉTODOS: Foram incluídos todos os casos de tuberculose abdominal de maio de 2014 a abril de 2017, diagnosticados com base no perfil clínico e nos resultados morfológicos brutos na endoscopia, imagem latente, seguido pela histologia e/ou pela cultura de GeneXpert e de MGIT. Todos os pacientes receberam a terapia antitubercular da droga (AKT) de acordo com o protocolo nacional. Os pacientes foram acompanhados de diagnósticos até a conclusão do tratamento e vários parâmetros foram estudados. RESULTADOS: Dos 176 pacientes, 48% eram do sexo masculino. A dor abdominal foi a queixa mais comum em 83,5%. Na colonoscopia, o íleo terminal e a válvula íleo-cecal foram os segmentos mais comumente envolvidos. O trato gastrointestinal superior foi envolvido em quatro pacientes. As lesões ulceronodulares totais foram as mais comuns seguidas pela lesão ulcerosa/nodular. As estenoses foram observadas em 28 (15,9%) pacientes priciplamente com a válvula íleo-cecal envolvida, dos quais 23 tiveram alívio sintomático com AKT e somente três necessitaram de dilatação. A histopatologia mostrou o granuloma em 80,8% casos. O MGIT foi positivo em 43 (35,80%) e GeneXpert foi positivo em 35 (26,1%) casos. Oito pacientes tiveram tuberculose resistente a múltiplas drogas. Apenas dois pacientes necessitaram de manejo cirúrgico. CONCLUSÃO: A tuberculose abdominal com amplo espectro de apresentação, ainda pode ser manuseada com diagnóstico precoce e tratamento mesmo em pacientes com obstrução intestinal sub-aguda. Ganho de peso ou sintomas resolvidos foram considerados marcadores precoces de resposta ao tratamento. Pacientes com estenose podem se tornar assintomáticos com tratamento médico isoladamente.

Nutritional status and prognosis in cirrhotic patients.

Background and aims: Malnutrition is commonly associated with chronic liver disease. The presence of protein–calorie malnutrition has been shown to be associated with increased short- and long-term mortality in patients with acute and chronic liver disease. We undertook this study to assess the prognostic value of nutritional status in predicting survival in cirrhotic patients. The aim of our study was to determine whether assessment of nutritional status using the RFH-SGA score adds significantly to CP (Child-Pugh) and MELD scores in predicting patient prognosis and survival in cirrhotic patients. Methods: Diagnosed cases of cirrhosis were enrolled and their nutritional assessment was done using the RFH-SGA score. All patients were followed up for a period of 6 months. The mortality rates in the various groups were compared with respect to their nutritional status. Multivariate analysis was used to determine the factors associated with mortality. Results: A total of 73 cirrhotic patients were taken up for this study. Of these, 23 patients (31.5%) were well nourished, 21 (28.8%) had mild to moderate malnourishment and 29 (39.7%) were severely malnourished. Multivariate analyses of various parameters identified poor nutritional status, increased CP grade, increased creatinine, lower sodium levels and longer prothrombin time as being independently associated with poorer survival. Conclusions : RFH-SGA is a simple and inexpensive tool for assessing the nutritional status in cirrhotic patients and can reliably predict their disease prognosis and survival.

An open-label, prospective clinical study to evaluate the efficacy and safety of TLPL/AY/01/2008 in the management of functional constipation.

Functional constipation is one of the most common gastrointestinal symptoms across the globe. Its high prevalence rate, economic burden, and adverse implications on the quality of life make constipation a major public health issue. Though various treatment options are available for the management of constipation, evidence for their efficacy and safety are limited. An open-label, prospective, interventional, and exploratory clinical trial was carried out to evaluate the efficacy and safety of “TLPL/AY/01/2008” in 34 patients suffering from functional constipation. “TLPL/AY/01/2008” is an Ayurvedic proprietary polyherbal formulation in powder form, containing Isabgol husk, Senna extract, and Triphala extract. Administration of “TLPL/AY/01/2008” for 14 days showed a significant increase in mean weekly bowel movements from 10.19 ± 05.64 to 18.29 ± 05.72 (P<0.05). The mean average time spent on toilet for bowel evacuation reduced significantly from 11.02 ± 05.43 minutes (baseline value) to 08.70 ± 04.72 minutes on day 14 (P<0.05). Mean stool form score assessed on Bristol stool form scale was improved from 02.97 ± 00.48 (baseline value) to 04.61 ± 00.84 (P<0.05) on day 14. A significant improvement (P<0.05) was also noted in straining during defecation, sensation of incomplete evacuation, sensation of anorectal blockage, and other associated symptoms of functional constipation. The significant improvement in most of the above symptoms was endured for a post-treatment observatory period of one week. All the study patients showed an excellent tolerability to the study drug. These findings suggest that “TLPL/AY/01/2008” is an effective, safe, and nonhabit- forming herbal laxative formulation for the management of constipation. Comparative clinical studies with larger sample size would be able to confirm the above findings.

Neonatal presentation of a rare metabolic liver disease.

Tyrosinemia is a rare paediatric metabolic liver disorder. A 15-days-old neonate born of a third degree consanguineous marriage presented with jaundice due to tyrosinemia, which progressed to fatal hepatic encephalopathy. The diagnosis was based on very high alpha-fetoprotein level, with urine aminoacidogram revealing tyrosine spot and liver biopsy depicting cirrhosis. Very early neonatal presentation and rapid progression were the unusual features of this case.

Hypercoagulable state in idiopathic ulcerative colitis: role of hyperhomocysteinemia and hyperfibrinogenemia.

BACKGROUND: Previous reports on hypercoagulable factors in inflammatory bowel diseases involve heterogeneous populations and patients on various medications. AIMS: To determine the frequency of thrombotic complications in ulcerative colitis (UC); to evaluate for hyperhomocysteinemia and its relationship to vitamin B12 and folate levels and methylene tetrahydrofolate reductase (MTHFR) mutation; and to evaluate for hyperfibrinogenemia and factor V Leiden mutation. METHODS: Eighty-six adult patients with UC were seen during the study period; 28 of them underwent blood tests and constituted the study population. Patients who received medications that affect these factors were among the 58 excluded. Tests were obtained at baseline and after 2 months during remission. Patients received folic acid in addition to treatment for UC. RESULTS: Vascular thrombotic events were noted in 4 patients during follow up. Hyperhomocysteinemia was detected in 11 (39.3%) patients (controls 15/100, p=0.007). Heterozygous state for MTHFR C677T mutation was found in 5 (17.9%) patients (controls: 0.2% homozygous, 13.6% heterozygous, p>0.05). Plasma homocysteine did not correlate with extent, severity or duration of disease, or with MTHFR C677T heterozygous state, but correlated with serum folic acid level (p=0.003) and BMI (p=0.03). With folate supplementation, homocysteine decreased significantly in patients who had hyperhomocysteinemia at baseline. Hyperfibrinogenemia was detected in 3 patients (none in 100 controls). Plasma fibrinogen was not affected by duration, extent or severity of UC and did not decrease with remission of disease. Only one patient had heterozygous factor V Leiden mutation. CONCLUSION: Vascular thrombosis occurred in less than a fifth of the UC population studied. Hyperhomocysteinemia reversible by folate supplementation and hyperfibrinogenemia were observed, but their contribution and that of factor V Leiden mutation appear to be insignificant.

Effect of liver disease on maternal and fetal outcome--a prospective study.

BACKGROUND: Liver disease in pregnancy can have serious consequences. Its prevalence, profile and effect on outcome of pregnancy have not been documented systematically in India. We prospectively determined the frequency, causes and outcome of liver disease in pregnant women. SETTING: Tertiary-care teaching hospital. METHODS: Consecutive pregnant women with liver disease attending the inpatient or outpatient services of the Departments of Gastro-enterology and Obstetrics and Gynecology between December 2002 and October 2004 were evaluated and followed up till 2 weeks postpartum or death. RESULTS: Liver disease was found in 107 (0.9%) of 12,061 pregnancies. Of these, fifty six (52.3%) had pregnancy-specific liver disorders (pregnancy-induced hypertension [PIH]-associated liver dysfunction 36--including HELLP syndrome 22 and pre-eclamptic liver dysfunction 14; intrahepatic cholestasis of pregnancy 10; hyperemesis gravidarum 7; acute fatty liver of pregnancy 3). Liver disorders not specific to pregnancy included hepatitis E (16), hepatitis B, non A-E hepatitis and chronic liver disease (5 each) and others (14); in 6 patients no cause could be found. Ninety-six patients completed follow up. Overall maternal and perinatal mortality rates were 19.7% and 35.4%, respectively. CONCLUSIONS: PIH-associated liver dysfunction was the most common cause of liver disease in pregnancy. This is associated with significant maternal and perinatal morbidity and mortality.

Endoscopic biliary drainage in patients with amebic liver abscess and biliary communication.

BACKGROUND: Percutaneous drainage or surgery is required when amebic liver abscess (ALA) fails to respond to medical management. In some of these patients, non-response may be due to communication of ALA with the biliary tree. This report describes our experience with the use of endoscopic biliary draining in such patients. METHODS: Medical records of patients with ALA undergoing either needle aspiration or percutaneous pigtail drainage were retrieved; the indications for drainage were: abscess volume exceeding 250 mL, a thin rim of tissue (< 1 cm thick) around the abscess, systemic toxic features and failure to improve on medical treatment. Patients with abscess drain output >25 mL/day persisting for 2 weeks or presence of bile in the drain fluid underwent endoscopic biliary drainage. RESULTS: A total of 115 patients with ALA underwent percutaneous treatment. None of the 25 patients with needle aspiration needed any further treatment. Of the 90 who underwent catheter drainage, the catheter could be removed within one week in 77 patients; the remaining 13 patients (median age 42 years, range 24-65; all men) had an abscess-biliary communication. In them, the median catheter output was 88 mL/day (range 45-347) and 54 mL/day (28-177) at 2 days and 2 weeks after catheter placement. The drain fluid contained bile in all 13 patients and in addition contained pus in 10 patients. Eleven patients had a solitary abscess and two had multiple abscesses. Cholangiogram showed biliary communication in all 13 patients. All patients were treated with placement of 10F biliary endoprosthesis or 10F nasobiliary drain. Pigtail catheter was removed within 1 week in 11 of 13 patients. CONCLUSION: In patients with amebic liver abscess communicating with the biliary tree, biliary stenting may hasten clinical recovery and allow early removal of liver abscess catheter drain.

Thrombophilic conditions in non-cirrhotic portal vein thrombosis.

OBJECTIVE: To study the prevalence of thrombophilic conditions in patients with acute and chronic portal vein thrombosis (PVT) and to compare it with those in patients suffering from deep vein thrombosis (DVT) after lower limb arthroplasty and in healthy subjects. METHODS: Twenty-six patients with spontaneous PVT (20 chronic, 6 acute) with normal liver function and not receiving anticoagulants were evaluated for thrombophilic conditions. Levels of protein C, protein S and antithrombin were compared with those in 50 healthy controls. Factor V gene 'Leiden' mutation (FVL) and high homocysteine levels were looked for in patients with PVT and in 18 patients developing post-arthroplasty lower limb DVT despite anticoagulation. RESULTS: Of 26 patients with PVT, 19 had at least one thrombotic condition (acute PVT 5/6, chronic PVT 14/20) and 12 had more than one such condition; in comparison, of 18 patients with DVT, eight had one thrombophilic condition and one had two such conditions (p=0.03). Patients with PVT had significantly lower levels of protein C, protein S and antithrombin than healthy subjects and those with DVT. Six patients had Factor VIII levels above 150%; four had elevated homocysteine levels and three had detectable anti-cardiolipin antibodies. Three patients with PVT (acute 2, chronic 1) were heterozygous for FVL mutation. CONCLUSIONS: Underlying thrombophilic conditions are common in Indian patients with spontaneous PVT. In many patients, multiple thrombophilic conditions are present and these may play a role in the pathogenesis of PVT.