alpha 1-antitrypsin deficiency/fetma

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Alpha-1 Antitrypsin Deficiency Liver Disease, Mutational Homogeneity Modulated by Epigenetic Heterogeneity With Links to Obesity.

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Alpha-1 antitrypsin deficiency (AATD) liver disease is characterized by marked heterogeneity in presentation and progression, despite a common underlying gene mutation, strongly suggesting the involvement of other genetic and/or epigenetic modifiers. Variation in clinical phenotype has added to the

Yield of diagnostic tests in obese children with an elevated alanine aminotransferase.

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OBJECTIVE Nonalcoholic fatty liver disease (NAFLD) is associated with obesity and affects roughly 10% of children. However, NAFLD is often diagnosed by exclusion - that is, obese children with an elevated alanine aminotransferase (ALT) are screened for other liver diseases in the absence of a

Clinical and histologic features of adults with alpha-1 antitrypsin deficiency in a non-cirrhotic cohort.

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OBJECTIVE Alpha-1 antitrypsin deficiency (AATD) is an uncommonly recognized cause of liver disease in adults, with descriptions of its natural history limited to case series and patient-reported data from disease registries. Liver pathology is limited to selected patients or unavailable. Therefore,

Liver cirrhosis associated with heterozygous alpha-1-antitrypsin deficiency type Pi MS and autoimmune features.

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Patients with homozygous protease inhibitor (Pi) type ZZ or a few rare M-like types may develop liver cirrhosis due to intracellular storage of alpha-antitrypsin (AAT), whereas some patients with heterozygous Pi MZ or SZ normally present with transient abnormal liver function tests in childhood. We

Asthma as aetiology of bronchiectasis in Finland.

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By definition bronchiectasis (BE) means destructed structure of normal bronchus as a consequence of frequent bacterial infections and inflammation. In many senses, BE is a neglected orphan disease. A recent pan-European registry study, EMBARC, has been set up in order to better

Hypertransaminasemia in childhood as a marker of genetic liver disorders.

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BACKGROUND The widespread use of routine biochemical assays has led to increased incidental findings of hypertransaminasemia. We aimed to evaluate the prevalence of different causes of raised aminotransferase levels in children referred to a university department of pediatrics. METHODS We

The clinical significance of slightly to moderately increased liver transaminase values in asymptomatic patients.

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BACKGROUND Our aim was to study liver disorders in asymptomatic patients with slightly to moderately increased liver transaminase values in a population living in an area with a low prevalence of viral and hereditary liver diseases. METHODS One hundred and fifty consecutive patients with slightly to

[Role of autophagy in the pathogenesis of liver diseases].

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Autophagy is a self-digestion process that plays an important role in the development, differentiation and homeostasis of cells, helping their survival during starvation and hypoxia. Accumulated mutant proteins in the endoplasmic reticulum can be degraded by autophagy in alpha-1 antitrypsin

Nonalcoholic fatty liver disease.

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NAFLD likely is the most common liver disease in children and is responsible for significant progression to cirrhosis, portal hypertension, and the need for liver transplantation in adults and even in some adolescents. Early diagnosis and lifestyle interventions appear to be our best hope for

Weaning of immunosuppression in long-term liver transplant recipients.

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Seventy-two long-surviving liver transplant recipients were evaluated prospectively, including a baseline allograft biopsy for weaning off of immunosuppression. Thirteen were removed from candidacy because of chronic rejection (n = 4), hepatitis (n = 2), patient anxiety (n = 5), or lack of

Liver Phenotypes of European Adults Heterozygous or Homozygous for PiZ Variant of AAT (PiMZ vs Pi*ZZ genotype) and Non-carriers.

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Homozygosity for the Pi*Z variant of the gene that encodes the alpha-1 antitrypsin peptide (AAT), called the Pi*ZZ genotype, causes a liver and lung disease called alpha-1 antitrypsin deficiency. Heterozygosity (the Pi*MZ genotype) is a risk factor for cirrhosis in individuals with

Autophagy in liver diseases.

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Autophagy, or cellular self-digestion, is a cellular pathway crucial for development, differentiation, survival, and homeostasis. Its implication in human diseases has been highlighted during the last decade. Recent data show that autophagy is involved in major fields of hepatology. In liver

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alpha 1-antitrypsin deficiency/fetma

Alpha-1 Antitrypsin Deficiency Liver Disease, Mutational Homogeneity Modulated by Epigenetic Heterogeneity With Links to Obesity.

Yield of diagnostic tests in obese children with an elevated alanine aminotransferase.

Clinical and histologic features of adults with alpha-1 antitrypsin deficiency in a non-cirrhotic cohort.

Liver cirrhosis associated with heterozygous alpha-1-antitrypsin deficiency type Pi MS and autoimmune features.

Asthma as aetiology of bronchiectasis in Finland.

Hypertransaminasemia in childhood as a marker of genetic liver disorders.

The clinical significance of slightly to moderately increased liver transaminase values in asymptomatic patients.

[Role of autophagy in the pathogenesis of liver diseases].

Nonalcoholic fatty liver disease.

Weaning of immunosuppression in long-term liver transplant recipients.

Liver Phenotypes of European Adults Heterozygous or Homozygous for Pi*Z Variant of AAT (Pi*MZ vs Pi*ZZ genotype) and Non-carriers.

Autophagy in liver diseases.

Den mest kompletta databasen med medicinska örter som stöds av vetenskapen

Liver Phenotypes of European Adults Heterozygous or Homozygous for PiZ Variant of AAT (PiMZ vs Pi*ZZ genotype) and Non-carriers.