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6.
Clin Genet ; 49(6): 296-9, 1996 Jun.
Article in English | MEDLINE | ID: mdl-8884077

ABSTRACT

Four siblings with achalasia, alacrimia and other problems involving the autonomic nervous system involvements are reported. Achalasia and alacrimia were present in all of them. Their parents are first cousins and have four other healthy children. Electrophysiological tests showed that autonomic dysfunction has progressed with age. Blood cortisol levels were normal in all four affected children. Depending on those findings of our case and previous reports, we conclude that triple-A syndrome and achalasia, alacrimia with or without neurological abnormalities could be variable manifestations of the same autosomal recessive gene defect.


Subject(s)
Esophageal Achalasia/genetics , Nervous System Diseases/genetics , Tears/metabolism , Adolescent , Child , Child, Preschool , Esophageal Achalasia/complications , Female , Genes, Recessive , Humans , Male , Nervous System Diseases/complications , Pedigree
7.
J R Coll Surg Edinb ; 38(2): 82-5, 1993 Apr.
Article in English | MEDLINE | ID: mdl-8478839

ABSTRACT

Forty-three patients with unilocular hydatid cyst of the liver were divided into three groups. Omentoplasty (n = 15) was performed in group 1, introflexion (n = 18) in group 2 and a combination of both (n = 10) in group 3. Patients with omentoplasty developed fewer complications and had a significantly shorter hospitalization than those with introflexion. The results indicate that omentoplasty is superior both to introflexion and to introflexion plus omentoplasty for the management of hydatid liver cysts.


Subject(s)
Echinococcosis, Hepatic/surgery , Omentum/surgery , Adolescent , Adult , Aged , Combined Modality Therapy , Female , Humans , Length of Stay , Male , Middle Aged , Recurrence , Surgical Procedures, Operative/methods , Treatment Outcome
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