Later, this was more appropriately renamed as trichorrhexis invaginata (bamboo hair) for a ball-and-socket–type hair-shaft deformity at the suggestion of Wilkinson et al. In 1974, Mevorah et al. established the clinical relationship between ichthyosis linearis circumflexa and Netherton syndrome, and an atopic diathesis was found to occur in approximately 75% of patients with Netherton syndrome. See also
Trichomegaly
List of cutaneous conditions

References


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Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced. Sclerosing cholangitis in critically ill patients, however, is associated with rapid disease progression and poor outcome. Serologic testing, radiological imaging and histological analysis can help diagnose SSC. Treatment
First lines of treatment can include mass spectrum antibiotics or drainage of the bile duct that is infected followed by close monitoring. Endoscopic surgery is favored over open procedures to reduce infection and quicker recovery times. If these fail a liver transplant may be necessary. References


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