Sarcoglycanopathy is a more frequent form of LGMD whereas alpha type is the most common among the SGP. The four types of SGP do not differ in the pattern of muscle involvement. A relatively earlier onset, selective weakness of knee flexors and a very high CK may help differentiate SGP from other forms of LGMD. Immunohistochemistry is very useful in classifying the different types of LGMD prior to genetic analysis.
We report a case of spontaneous hemobilia in a hemophilia patient presenting with classical Sandblom's triad of symptoms. Endoscopic retrograde cholangiography was helpful in establishing the diagnosis. Hemobilia subsided with factor VIII replacement and endoscopic nasobiliary drainage. Endoscopic nasobiliary drainage resulted in lysis of the clots in the biliary tree and relief of obstructive jaundice.
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