Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gaucher disease is a genetic disorder in which the enzyme activity of lysosomal acid beta-glucocerebrosidase is impaired, causing excessive lysosomal storage of glucocerebrosidase in monocytes and macrophages. Coagulation and multiorgan disorders can develop during pregnancy in women with this disorder, affecting anesthetic management. We report the case of a woman with type 1 Gaucher disease who was scheduled for cesarean section. The blood workup showed evidence of hepatosplenomegaly and thrombocytopenia, osteopenia, and gastroesophageal reflux. Epidural anesthesia-analgesia was provided. Surgical conditions and analgesia were both satisfactory and no complications developed after withdrawal of the epidural catheter. We conclude that the management of anesthesia for pregnant women with Gaucher disease will depend on the degree of systemic involvement. Regional anesthesia has been used in many such patients, but there is insufficient literature on which to base guidelines or protocols.
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PMID:[Anesthetic management in a pregnant woman with type 1 Gaucher disease]. 2049 4

Management of a patient with portal hypertension and hypersplenism who is scheduled for elective surgery is a challenge for anesthesia providers. We report a case that was scheduled for elective surgery in which preoperative splenic artery embolization improved the hematologic parameters. A 32-year-old woman was scheduled for total abdominal hysterectomy for multiple fibroids and menorrhagia with pancytopenia. She also had diagnoses of chronic liver disease with cirrhotic changes, portal hypertension, and grade IV Manageesophageal varices with hepatosplenomegaly. In view of pancytopenia, splenic artery partial embolization was done preoperatively. Her hematologic parameters improved, and she underwent surgery under general anesthesia with intrathecal morphine analgesia, uneventfully. The condition of patients with portal hypertension with associated hypersplenism and pancytopenia who are scheduled for elective surgery can be optimized with preoperative partial splenic artery embolization.
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PMID:Role of splenic artery partial embolization in a patient with portal hypertension and pancytopenia undergoing hysterectomy under anesthesia. 2258 77