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Radiological findings in Wolfram syndrome
Azmy M Hadidy1, Nadim S Jarrah, Maha I Al-Till
1Department of Radiology, Jordan University Hospital, Amman, Jordan.
Saudi Medical Journal
|May 13, 2004
Summary
Radiologic assessment reveals urinary tract dilation in Wolfram syndrome (WFS) patients. Peptic ulcers are more common in WFS-2, with no significant radiologic differences between WFS-1 and WFS-2 subtypes.
Area of Science:
- Radiology
- Genetics
- Neurology
Background:
- Wolfram syndrome (WFS) is a rare genetic disorder.
- Precise radiologic findings are crucial for clinical evaluation and defining reference populations.
Purpose of the Study:
- To precisely define radiologic findings in Wolfram syndrome (WFS) patients.
- To utilize objective imaging techniques for a better clinical evaluation.
Main Methods:
- Study included 16 WFS patients (WFS-1 and WFS-2) from 4 families.
- Radiologic techniques included urography, ultrasonography, barium meal, endoscopy, and MRI of the brain and pituitary glands.
Main Results:
- Urinary tract dilation (hydronephrosis, hydroureter) was observed in WFS patients.
- Peptic ulcers and Helicobacter pylori were frequent, especially in WFS-2.
- Brain atrophy (cerebellum, brainstem, optic tracts) was noted in all patients.
Conclusions:
- Urinary tract dilation is a common radiologic finding in both WFS-1 and WFS-2.
- Peptic ulcer disease is more prevalent in WFS-2 patients.
- No significant radiologic distinctions were identified between WFS-1 and WFS-2.