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Metachromatic leukodystrophy and its effects on the gallbladder: a case report
Paul M Rodriguez-Waitkus1, Robert Byrd, John Hicks
1Department of Pathology & Immunology, Baylor College of Medicine and Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA.
Ultrastructural Pathology
|November 17, 2011
Summary
Metachromatic leukodystrophy (MLD), a genetic disorder, can affect the gallbladder, causing inflammation and distinct structural changes. Ultrastructural analysis aids in diagnosing MLD by revealing characteristic cellular patterns.
Area of Science:
- Biochemistry
- Genetics
- Pathology
Background:
- Metachromatic leukodystrophy (MLD) is an inherited lysosomal storage disease due to arylsulfatase A deficiency.
- Sulfatide accumulation in MLD impacts the central nervous system and other organs, including the gallbladder.
Observation:
- A 9-year-old patient with late-infantile MLD presented with cholecystitis symptoms.
- Radiographic and gross examination revealed gallbladder enlargement, wall thickening, papillary projections, and mucin-filled cystic spaces.
Findings:
- Microscopic analysis showed papillary projections and dilated Rokitansky-Aschoff sinuses filled with mucin.
- Ultrastructural examination identified secondary lysosomes with lamellar material, exhibiting a "herringbone" or "tuffstone" pattern.
Implications:
- This case highlights gallbladder involvement in MLD, presenting unique pathological features.
- Ultrastructural examination can be a valuable diagnostic tool for MLD, particularly in atypical presentations.
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