Successful spleno-renal shunt and splenectomy in two patients with alpha-1-antitrypsin deficiency

Sotos, Juan F.; Cutler, Edward A.; Romshe, Carolyn A.; Clatworthy, H. William

doi:10.1016/s0022-3468(81)80107-8

Cited by 9 publications

(4 citation statements)

References 13 publications

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“…The stable condition of the three patients reported here for several years after portacaval shunt compared with their progressive deterioration before operation is consistent with this prediction. Similar long survival of two children after central splenorenal shunt for variceal haemorrhage has been described by Sotos et al 16 However, the most objective evidence that the history of the hepatic disease was favourably altered came from the histopathological studies. The number of hepatocytes with cytoplasmic alpha-1-antitrypsin globules fell in both patients for whom repeat biopsy samples were available.…”

Section: Discussionsupporting

confidence: 73%

Reversal of Hepatic Alpha-1-Antitrypsin Deposition After Portacaval Shunt

et al. 1983

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End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 3 1/2 to almost 7 years. Postoperative liver biopsy material from two of the patients showed the typical histopathological changes caused by portal diversion, as well as an apparent reduction in the quantity of alpha-1-antitrypsin particles in the hepatocytes. The metabolic changes caused by portal diversion have apparently created a more favourable equilibrium between the synthesis and excretion of the abnormal alpha-1-antitrypsin.

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Section: Discussionsupporting

confidence: 73%

Reversal of Hepatic Alpha-1-Antitrypsin Deposition After Portacaval Shunt

et al. 1983

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“…Ages at surgery were 8, 10, and 11 years. Long-term successful surgical portasystemic shunt was previously reported in one child with a-1-antitrypsin deficiency, operated on at 7 years, with an 8-year followup after surgery (19). These results have to be compared with those obtained with liver transplantation (20; Peters, R. L. et al, Gastroenterology 1975; 69:852, Abstract).…”

Section: Long-term Follow-upmentioning

confidence: 99%

α-Antitrypsin Deficiency

Alagille

1984

Hepatology

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“…The progressive and inexorable course that this pathogenesis implies may have discouraged atte~pts to treat complications of the portal hypertension (such as varIceal hemorrhage) with portal-systemic shunts. In addition, the results with portal diversion have been poor.17R-180 Only Sotos et al 181 have reported encouraging results, and their observations were limited to two children.…”

Section: Portal Diversion For (Xi-antitrypsin Deficiencymentioning

confidence: 99%