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[Autoimmune hepatitis in children. Initial presentation as fulminant hepatic failure].

UNLABELLED: There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. METHODS: Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7%) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). EVOLUTION: Patients 1 and 3 died while waiting for liver transplant (LT)

Natural history of hepatitis C.

It is now widely accepted that 85% or more of individuals with acute hepatitis C virus (HCV) infection progress to chronic hepatitis, and chronic hepatitis C is a known risk factor for cirrhosis and hepatocellular carcinoma (HCC). However, there has been much controversy about the inevitability of developing cirrhosis and HCC and the time frames in which they are likely to occur. Natural history studies have provided varying estimates of the risk of progression in chronic hepatitis C. Part of this variation may be a result of viral-specific, host, and/or environmental factors, but much of it u

Fatal outcome of dengue fever with multi-organ failure and hemorrhage: A case report.

Dengue fever is a prevalent viral disease caused by a single-stranded positive RNA virus belonging to the Flaviviridae family, genus flavivi … old male with EDS who presented at a Tertiary Care Hospital in Karachi and died a week later due to liver failure.

Intestinal Hypoganglionosis Leading to Intestinal Failure and the Compassionate Use of Omegaven™.

Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkalin

Cirrhotic cardiomyopathy: pathogenesis and clinical relevance.

Cirrhosis is known to cause alterations in the systemic haemodynamic system. Cirrhotic cardiomyopathy designates a cardiac dysfunction that includes impaired cardiac contractility with systolic and diastolic dysfunction, as well as electromechanical abnormalities in the absence of other known causes of cardiac disease. This condition is primarily revealed by inducing physical or pharmacological stress, but echocardiography is excellent at revealing diastolic dysfunction and might also be used to detect systolic dysfunction at rest. Furthermore, measurement of circulating levels of cardiac biom

Updates on the biology and management of dyskeratosis congenita and related telomere biology disorders.

Dyskeratosis congenita (DC) is a cancer-prone inherited bone marrow failure syndrome caused by aberrant telomere biology. The mucocutaneous triad of nail dysplasia, abnormal skin pigmentation and oral leukoplakia is diagnostic, but is not always present; DC can also be diagnosed by the presence of very short leukocyte telomeres. Patients with DC are at high risk of bone marrow failure, pulmonary fibrosis, liver disease, cancer and other medical problems. Germline mutations in one of nine genes associated with telomere maintenance are present in approximately 60% of patients. DC is one among th

Predictive factors associated with the progression to hepatic failure caused by lamivudine-resistant HBV.

The aims of this study were to select the patients with a potential for progression to hepatic failure due to lamivudine-resistant HBV and to standardize the treatment for patients with lamivudine-resistant HBV. Patients (n = 47) with reactivated hepatitis due to lamivudine-resistant HBV were classified into two groups, with and without potential for progression to hepatic failure, according to the criteria using the data of serum bilirubin level and prothrombin activity after the reactivated hepatitis. Multivariate analysis showed that prothrombin activity at the initiation of lamivudine ther

Branched-chain amino acids.

The branched-chain amino acids (BCAA), isoleucine, leucine and valine, are unique in that they are principally metabolized extrahepatically in the skeletal muscle. This observation led to the investigation of these nutrients in a number of clinical scenarios. By far the most intensively studied applications for BCAA have been in patients with liver failure and/or patients in catabolic disease states. However, the resulting studies have not demonstrated a clear clinical benefit for BCAA nutritional supplements. In patients with liver failure, the BCAA did improve nitrogen retention and protein …

Острый вирусный гепатит.

Основным средством лечения острого вирусного гепатита является поддержка, поскольку в большинстве случаев оно носит ограниченный характер. Общие меры по всем видам острого вирусного гепатита включают постель, если пациент очень симптоматичен, высококалорийную диету, отказ от гепатотоксичных препаратов и воздержание от алкоголя в надежде на то, что большинство пациентов выздоровеет неравномерно. В тяжелых случаях для внутривенной регидратации может потребоваться госпитализация, если пациент не в состоянии поддерживать достаточное количество перорального вскармливания из-за тошноты и рвоты или е

Совпадение в аннотации оригинала
The mainstay of treatment for acute viral hepatitis is supportive care, as most cases are self-limited. General measures in all types of acu … pha therapy in acute hepatitis C may decrease the risk of developing chronic hepatitis C. Fulminant liver failure due to acute viral hepatitis is uncommon, but orthotopic liver transplantation occasionally may be life saving.

Nutritional support in hepatic encephalopathy.

Hepatic encephalopathy (HE) is a syndrome of global cerebral dysfunction resulting from underlying liver disease or portal-systemic shunting. HE can present as one of four syndromes, depending on the rapidity of onset of hepatic failure and the presence or absence of preexisting liver disease. The precise pathogenesis is unknown but likely involves impaired hepatic detoxification of ammonia as well as alterations in brain transport and metabolism of amino acids and amines. The etiology of malnutrition in hepatic failure is multifactorial. Nutritional deficits may be clinically manifest as mara

[Isoniazid-inducedtoxic hepatitis].

Hepatotoxicity is a well-known side effect to isoniazid treatment with the risk of progression to liver failure. This case report describes a 39-year-old male, who received standard isoniazid treatment for latent TB infection (LTBI) and developed severe isoniazid-induced acute hepatitis. Liver transplantation was considered, but the patient slowly recovered with full hepatic regeneration. With increasing focus on treating LTBI in Denmark, routine follow-up including biochemical monitoring should be implemented for patients receiving LTBI treatment to prevent severe complications.

New treatments/targets for primary biliary cholangitis.

Primary biliary cholangitis (PBC) is an autoimmune, cholestatic, chronic liver disease that ultimately progresses towards cirrhosis and liver failure if untreated. While ursodeoxycholic acid has been established as standard of care for PBC in the last few decades, significant advances in second-line treatment options have recently been made and new therapeutic developments are currently under evaluation. The purpose of this article is to provide the clinician with an overview of the current treatment options and future opportunities for patients with PBC.

Acute-Onset Optic Neuropathy in Wilson's Disease.

Wilson's disease (WD), also known as hepatolenticular degeneration, is a rare autosomal recessive condition of excess copper accumulation that is most commonly associated with hepatic, neurologic, psychiatric, and ocular manifestations. While Kayser-Fleischer rings and sunflower cataracts are well known in WD, visual impairment is very rare. We report the case of a 20-year-old female who presented with acute liver failure and associated monocular vision loss. WD was found to be a cause of her liver disease and decreased vision.

A short case of a splenic arteriovenous fistula coexisting with portal hypertension secondary to hepatitis C: Radiologic diagnosis and treatment.

Summary Splenic arteriovenous fistula (SAVF) is a rare but potentially curable condition. Only a few cases have been reported in the English literature. SAVF can cause portal hypertension, ascites, gastrointestinal bleeding, and heart failure. An early diagnosis is essential to avoid life threatening complications. We hereby present a case of SAVF in a young female patient, with hepatitis C liver cirrhosis who presented with recurrent severe upper gastrointestinal bleeding. Such an association of liver cirrhosis and SAVF has not been previously reported.

[The first Danish child with small bowel transplantation].

A girl with extensive Hirschsprung's disease was operated, leaving her with a 35 cm short bowel. She received long-term parenteral nutrition through a central catheter. Progressive liver failure and intravenous access problems developed and at the age of 5.5 years she was transplanted in Birmingham with 270 cm small bowel, a reduced liver and pancreas. After the transplantation she received immunosuppressive medication, ursodeoxycholic acid and antibiotic prophylaxis. She is now well and on enteral nutrition, 32 months after the transplantation.

[Autoimmune hepatitis in patients with a diagnosis of multiple sclerosis].

Autoimmune hepatitis (AIH) is a chronic necroinflammatory liver disorder associated with hypergammaglobulinemia and circulating autoantibodies. Two patients previously diagnosed with multiple sclerosis who developed AIH are reported. One patient showed acute presentation with fulminant hepatic failure requiring liver transplantation. Serum autoantibodies were negative in both patients but a characteristic clinical course in the first patient as well as the hepatic histological features with typical pathological changes of AIH in both patients and a score compatible with AIH established the dia

Recombinant interferon alfa 2a in hepatic hemangiomatosis with congestive heart failure: a case report.

A 45-day-old patient was admitted with dyspnea, hepatomegaly, tachycardia, holosystolic murmur in the precordial region, and continuous murmur at the right hypochondrium. Four cutaneous angiomas were noted. Instrumental examinations revealed congestive heart failure and multiple focal lesions in the liver with typical features of hemangiomas. The therapy with subcutaneous interferon-alfa-2a (IFN-alpha) was administered for 12 months with progressive regression of cutaneous hemangiomas, liver lesions, and cardiological alterations. IFN-alpha therapy was effective without any significant adverse

Non-inflammatory herpes simplex hepatitis in an adult with chronic neutropenia.

We report the case of a young woman with chronic neutropenia, in whom hepatitis, extensive herpetic eruption and herpes simplex viremia developed after genital herpetic ulceration. Although severe liver necrosis was present, the patient's death did not result from hepatic failure. No inflammatory cell infiltration was found circumscribing the multiple necrotic foci in the liver. This absence of inflammatory cell infiltration reflects the host's inability to normally restrain herpes simplex virus dissemination and, in this patient, might be the consequence of chronic neutropenia.

Disseminated aspergillosis complicating hepatic failure.

Disseminated aspergillosis is not generally known as a complication of hepatic failure. Three patients with subacute hepatic necrosis died of clinically unrecognized disseminated aspergillosis. All of the patients had pulmonary aspergillosis and Aspergillus infections of the CNS. Focal neurologic deficits were clinically misconstrued as intracerebral hemorrhages caused by the coagulopathy of liver failure. Signs of invasive pulmonary aspergillosis were ascribed to refractory bacterial pneumonia. Each patient had a hospital course of longer than three weeks and each received treatment with cort…

Utility of N-acetylcysteine in Non-Acetaminophen-Induced Liver Injury Secondary to Influenza A Infection.

Influenza A, which belongs to the Orthomyxoviridae viral family, is a known causative agent of respiratory illness and systemic inflammation. Annual influenza immunizations are crucial in reducing the incidence and severity of the flu. Intravenous (IV) N-acetylcysteine (NAC) is a pharmaceutical agent indicated for hepatic injury, particularly to address oxidative stress and inflammation secondary to acetaminophen toxicity. The authors present a case of a young female with acute liver injury and impending liver failure in the setting of viral influenza A infection, successfully treated with IV-…