Related Experiment Videos

Fibrinogen storage disease without hypofibrinogenemia associated with estrogen therapy

Z Simsek1, O Ekinci, M Cindoruk

  • 1Gastroenterology Department, Gazi University, Faculty of Medicine, Ankara, Turkey. zahidesimsek@yahoo.com

BMC Gastroenterology
|November 17, 2005
PubMed

Insights

Estrogen replacement therapy can cause fibrinogen storage disease, a type of endoplasmic reticulum storage disease (ERSD). Liver biopsies are crucial for diagnosing this rare condition, especially in patients with unexplained liver enzyme abnormalities.

Area of Science:

  • Hepatology
  • Endocrinology
  • Pathology

Background:

  • Cytoplasmic inclusion bodies in hepatocytes can indicate Endoplasmic Reticulum Storage Diseases (ERSD).
  • ERSD involves abnormal protein accumulation in the endoplasmic reticulum, leading to liver dysfunction.
  • Fibrinogen storage disease is a specific type of ERSD.

Observation:

  • A case of secondary fibrinogen storage disease linked to estrogen replacement therapy is presented.
  • Histological, immunohistochemical, and ultrastructural analyses of liver biopsies confirmed the diagnosis.
  • Paired liver biopsies taken during and after therapy demonstrated the drug's causal role.

Findings:

  • Estrogen replacement therapy can induce fibrinogen storage disease.
  • Cytoplasmic inclusion bodies, specifically fibrinogen deposits, were identified in hepatocytes.
  • The condition is a secondary ERSD, acquired due to medication.

Implications:

  • Liver biopsies should be examined for inclusion bodies in cases of idiopathic liver enzyme abnormalities.
  • Fibrinogen storage disease, though rare, should be considered in the differential diagnosis.
  • Understanding drug-induced ERSD is vital for accurate patient management and diagnosis.
Abstract

Related Concept Videos

Venous Thrombosis I: Introduction01:30

Venous Thrombosis I: Introduction

Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Cirrhosis I: Introduction01:23

Cirrhosis I: Introduction

Cirrhosis is a chronic, irreversible liver disease characterized by the widespread replacement of healthy liver tissue with fibrotic scar tissue and the formation of regenerative nodules.Etiology of cirrhosisCirrhosis results from sustained liver injury that triggers progressive fibrosis and structural remodeling. The underlying causes are diverse, encompassing common and less frequent clinical conditions. Regardless of the origin, all causes lead to chronic inflammation, hepatocyte loss, and...
Venous Thrombosis III: Interprofessional Care01:29

Venous Thrombosis III: Interprofessional Care

Venous thrombosis requires effective prevention and treatment strategies to improve patient outcomes and reduce potential complications.Prevention StrategiesHealthcare providers must prioritize preventing venous thromboembolism (VTE) for all adult patients upon admission. Interventions depend on bleeding and thrombosis risk, medical history, current medications, diagnoses, planned procedures, and patient preferences. Patients on bed rest should change positions every two hours and, if not...
Oogenesis02:07

Oogenesis

In human women, oogenesis produces one mature egg cell or ovum for every precursor cell that enters meiosis. This process differs in two unique ways from the equivalent procedure of spermatogenesis in males. First, meiotic divisions during oogenesis are asymmetric, meaning that a large oocyte (containing most of the cytoplasm) and minor polar body are produced as a result of meiosis I, and again following meiosis II. Since only oocytes will go on to form embryos if fertilized, this unequal...
Fibril-associated Collagen01:11

Fibril-associated Collagen

Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...