title: Successful spleno-renal shunt and splenectomy in two patients with alpha-1-antitrypsin deficiency.
abstract: Recent evidence suggests that the cirrhosis of alpha-1-antitrypsin deficiency is not invariably fatal as it was previously thought. Portal hypertension is often the major determinant of survival. The few reports of porta-systemic venous anastomosis in this disorder have shown poor results or uncertain outcome. Thus, doubts exist as to whether porta-systemic shunts should be performed in alpha-1-antitrypsin deficiency. Two patients with alpha-1-antitrypsin deficiency (PiZZ) and associated portal hypertension, cirrhosis, and hypersplenism underwent splenorenal shunt and splenectomy 8 yr ago, and both have done well. One of the patients has chronic severe headaches, diarrhea, exudative enteropathy, sinusitis, and hematuria, all uncommon in alpha-1-antitrypsin deficiency but possibly related to the antienzyme deficiency. She also has a higher trypsin inhibitory capacity than is generally reported in ZZ individuals. Based on the experience with these 2 patients, it appears that alpha-1-antitrypsin deficiency with cirrhosis is not a valid contraindication to the performance of a portasystemic shunt.
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D006971:Hypersplenism / Q000209:etiology / Q000601:surgery; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery; D008297:Male; D010375:Pedigree; D010641:Phenotype; D011170:Portasystemic Shunt, Surgical; D013156:Splenectomy; D013164:Splenorenal Shunt, Surgical; D019896:alpha 1-Antitrypsin Deficiency