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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Heart failure (HF) manifests primarily as dyspnea, fatigue, and fluid retention, resulting in peripheral and pulmonary edema. Symptoms may vary depending on which ventricle is more affected, left or right.Left-Sided Heart FailureAlso known as left ventricular failure, this condition results from the left ventricle's inability to fill or eject sufficient blood into the systemic circulation. It leads to pulmonary congestion, which occurs when the left ventricle fails to eject blood effectively...
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Severe Ascites in Common Variable Immunodeficiency.

Guilherme Camões1, Diogo A Fernandes2, Diana M Ferreira1

  • 1Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, PRT.

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Summary
This summary is machine-generated.

Common variable immunodeficiency (CVI) can cause liver disease, including nodular regenerative hyperplasia and portal hypertension. Early diagnosis and management are crucial for patients with CVI presenting with ascites.

Keywords:
ascitescommon variable immunodeficiencyhepatologyidiopathic non-cirrhotic portal hypertensionnodular regenerative hyperplasia

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Area of Science:

  • Hepatology
  • Immunology
  • Internal Medicine

Background:

  • Liver disease is a known complication of common variable immunodeficiency (CVI).
  • Manifestations range from mild enzyme elevation to severe conditions like cirrhosis and portal hypertension.
  • Nodular regenerative hyperplasia is the most common histologic finding in CVI-related liver disease.

Observation:

  • A 48-year-old woman with CVI and hemolytic anemia presented with rapidly progressing ascites and asthenia.
  • Ascites were attributed to portal hypertension, confirmed by imaging showing permeable hepatic and portal veins.
  • Liver biopsy revealed nodular regenerative hyperplasia without cirrhosis.

Findings:

  • The patient was diagnosed with idiopathic non-cirrhotic portal hypertension secondary to CVI.
  • Treatment adjustment led to significant improvement in ascites.

Implications:

  • Idiopathic non-cirrhotic portal hypertension is an under-recognized complication of CVI.
  • A high index of suspicion is necessary for diagnosing this condition, particularly in CVI patients with ascites.
  • This case highlights the importance of considering CVI in the differential diagnosis of non-cirrhotic portal hypertension.