Partial Splenectomy In Type 1 Gaucher's Disease

Year 1991, Volume: 44 Issue: 2, 259 - 266, 30.06.1991

Ercan Tutar

Abstract

Gaucher's disease, the inherited deficiency of glucocerebrosidase, is the most freguenily encountered lipidosis. Disease is conventionaliy classified into three types according to the presence and rate of progression of neurologic abnormalities. Type 1, is the most comman form of the disease, hematologic findines dominate the clinical picture of patients in this category. In this paper, we have reported a child with Type 1 Gaucher's disease. Partial splenectomy was performed to the patient but subseguentiy she underwent total splenectomy due to bleeding from the remnant of the spleen.

Keywords

Gaucher , Type 1 , Splenectomy

Ethical Statement

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Supporting Institution

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Thanks

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Geniş Deri Kayıplarında Mesh Greft Uygulamaları

Abstract

Glukoserebrozidaz enziminin kalıtsal eksikliği sonucu oluşan Gaucher hastalığı, en sık görülen lipidozdur. Hastalık nörolojik bozuklukların varlığı ve ilerleme hızına göre üç tipe ayrılmaktadır. Tip 1, hastalığın en sık görülen formu olup klinik tabloya hematolojik bulgular egemendir. Burada, Tip 1 Gaucher hastalıklı bir çocuk sunulmaktadır. Hastaya parsiyel splenektomi yapılmış fakat sonradan, kalan dalak dokusunda oluşan kanama sebebiyle total splenektomiye gidilmiştir.

Keywords

Gaucher Hastalığı , Tip 1 , Splenektomi (Dalak Ameliyatı)

Ethical Statement

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Supporting Institution

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Thanks

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References

• 1.Bar, Maor JA Yehudain JG ; Partia isplenectomy in children with Gaucher's disease, Pediatrics, 76 : 398, 1985.
• 2. Caffey's Pediatric X-Ray Diagnosis, Lipid Storage Diseases, Ed. Silverman FN: Year Book Medical Pub. Inc. : 699, 1985.
• 3. Goldblatt J: Type 1 Gaucher disease, J. Med. Genet., 25 : 415-418, 1988.
• 4.Kolodny EH Boustany RM : Storage discases of the reticuloendothelial system, Hematology of Infancy and Childhood, Ed. Nathan DG, Oski FA : W.B. Saunders Company : 1212-1230, 1987.
• 5.Martin BM Sidransky E Ginns El : Gauchers disease : Advances and Challenges, Adv, Pediatr., 36 : 277-306, 1989.
• 6.Pearson HA : The spleen and disturbances of splenic function, Hematology of Infancy and Childhood, Ed. Nathan DG, Oski FA : W.B. Saunders Company : 000-917, 1987.
• 7.Rubin M ve ark. : Partial splenectomy in Gaucher's disease, J. Pediatr. Surg, 21; 125-128, 1986.
• 8.Swaiman KF ; Lysosomal Diseases-Gaucher's Disease, Pediatric Neurology Principles and Practice, Ed. Swaiman KF ; C.V, Bosby Company : 1034-1036, 1989,
• 9.Zimran A ve ark : Prediction of severity of Gaucher's disease by identification of mutations at DNA level, Lancet, i : 349-352, 1989,