Non-Wilson's Disease-Associated Hypoceruloplasminemia

Area of Science:

• Hepatology
• Clinical Biochemistry

Background:

• Low serum ceruloplasmin levels can be found in patients without Wilson's disease (WD).
• This finding may lead to extensive, costly, and potentially harmful investigations for WD.
• Understanding the prevalence and associated features of low ceruloplasmin in non-WD patients is crucial.

Purpose of the Study:

• To determine the prevalence of low serum ceruloplasmin levels in adult patients without Wilson's disease.
• To describe the clinical and demographic features associated with low serum ceruloplasmin in this population.

Main Methods:

• Serum ceruloplasmin levels were measured using an enzymatic assay.
• 3040 adult patients attending an urban liver diseases outpatient clinic were included.
• Comparison was made with MELD-matched control patients.

Main Results:

• 4.0% of patients without WD (122/3040) had low serum ceruloplasmin.
• These patients were younger, less often male, and more frequently had viral hepatitis (53%) compared to controls.
• No significant differences were observed in liver function tests, ferritin, creatinine, or survival between groups.

Conclusions:

• Low serum ceruloplasmin levels are present in 4.0% of adult patients without WD in this urban clinic setting.
• The key associated features are younger age, being less often male, and a higher prevalence of viral hepatitis.
• This data can aid in differentiating low ceruloplasmin causes and potentially reduce unnecessary WD investigations.