Abstract
The hepatitis associated with AlAT deficiency is no longer considered an invariably fatal disorder. For some patients portal hypertension may be the major determinant of survival. The few reports of porta-systemic venous anastomoses have shown poor results or uncertain outcome, and doubts exist as to whether such shunts should be performed. Two patients with AlAT deficiency (PiZZ) and associated portal hypertension, cirrhosis (proven by biopsy) and hypersplenism underwent spleno-renal shunt and splenectomy six years ago, and both are doing well, attending school, and have practically normal liver function. One of the patients has chronic severe headaches, diarrhea, exudative enteropathy (20% of Cr51-labelled albumin recovered in stools in 4 days), sinusitis and hematuria, all uncommon in A1AT deficiency but likely related to the primary disorder. She also has a higher trypsin inhibitory capacity (0.45 mg trypsin inhibited/ml serum) than is generally reported in ZZ individuals. Based on the experience with these two patients, we conclude that AlAT deficiency is not a valid contraindication to the performance of a porta-systemic shunt.
Supported in part by the John W. Champion Center.
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Cutler, E., Romshe, C., Calworthy, H. et al. 519 SUCCESSFUL SPLENO-RENAL SHUNT AND SPLENECTOMY IN TWO PATIENTS WITH ALPHA-1-ANTITRYPSIN DEFICIENCY. Pediatr Res 12 (Suppl 4), 450 (1978). https://doi.org/10.1203/00006450-197804001-00524
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DOI: https://doi.org/10.1203/00006450-197804001-00524
