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Hypothyroidism Associated with ATP8B1 Deficiency.

Liting Li1, Maesha Deheragoda2, Yi Lu1

  • 1Department of Pediatrics, Shanghai Medical College, Fudan University, Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai, China.

The Journal of Pediatrics
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Hypothyroidism is an extrahepatic feature of ATPase, aminophospholipid transporter, class I, type 8B, member 1 (ATP8B1) deficiency. Thyroid function monitoring is recommended for these patients.

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Area of Science:

  • Endocrinology
  • Hepatology
  • Genetics

Background:

  • ATPase, aminophospholipid transporter, class I, type 8B, member 1 (ATP8B1) deficiency is a genetic disorder.
  • Extrahepatic manifestations of ATP8B1 deficiency are not well-defined.

Purpose of the Study:

  • To investigate if hypothyroidism is an extrahepatic feature of ATP8B1 deficiency.

Main Methods:

  • Retrospective analysis of clinical data and thyroid function tests in children with normal gamma-glutamyltransferase cholestasis.
  • Comparison of hypothyroidism prevalence between patients with ATP8B1 deficiency, ATP-binding cassette, subfamily B (MDR/TAP), member 11 (ABCB11) deficiency, and those without mutations.

Main Results:

  • Hypothyroidism and subclinical hypothyroidism were significantly more frequent in patients with ATP8B1 deficiency (5/13) compared to controls (0/19 and 0/15).
  • Thyroid function normalized with hormone replacement, but cholestasis did not improve.

Conclusions:

  • Hypothyroidism is a potential extrahepatic manifestation of ATP8B1 deficiency.
  • Regular thyroid function monitoring is advised for individuals with ATP8B1 deficiency.