Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gaucher's disease is an autosomal recessive disorder caused by deficiency of beta glucocerebrosidase, resulting in an accumulation of glucocerebroside in the reticuloendothelial system. These patients have massive splenomegaly and bone pain, but may have normal life expectancy. Traditionally, splenectomy has been used to treat hypersplenism, but may be associated with a high incidence of postsplenectomy sepsis and accelerated hepatic and bone lipid deposition. Two children are reported who had partial splenectomy for symptoms of Gaucher's disease. Both patients had laboratory evidence of hypersplenism. A 90% splenectomy was performed, and the residual splenic fragment was wrapped in Vicryl mesh. Both patients are currently asymptomatic with normal hematologic parameters. Postoperative radionuclide scans demonstrate increase in the size of the residual splenic fragment. Partial splenectomy may benefit patients with Gaucher's disease, but long-term follow-up care is necessary.
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PMID:Partial splenectomy for Gaucher's disease. 359 12

Splenomegaly is a common problem. In the absence of systemic illness or malignancy splenic cysts must be considered, especially the epithelial variety. For large cysts total splenectomy has long been recommended. Recognition of the risk of an overwhelming postsplenectomy infection (OPSI), especially in children, has led to spleen conserving surgery. We describe the use of an absorbable Vicryl net after partial splenectomy with total cystectomy in the management of splenic epithelial cysts.
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PMID:Splenic epithelial cysts and splenomegaly: diagnosis and management. 976 57

A wandering spleen is a rare condition with an unknown incidence. The lack of short gastric vessels and suspensory ligaments is thought to result from a fusion anomaly of the dorsal mesogastrium of the spleen. The major risk in performing a splenectomy for patients with a wandering spleen is overwhelming postsplenectomy infection (OPSI). The incidence of OPSI is 0.13% to 8.1%, with a mortality rate of 30% to 60%. Laparoscopic splenopexy provides the benefits of minimally invasive surgery while avoiding the complications of splenectomy. The reported case presents a patient with a wandering spleen. The patient, an 18-year-old woman, experienced a malarial infection at the age of 5 years and had a computed tomography (CT) scan documenting her spleen in the normal anatomic position. When she was 18 years old, a CT scan showed her spleen located in the right lower quadrant (RLQ). At laparoscopy, the presence of the spleen in the RLQ was confirmed. The spleen could not be easily manipulated with laparoscopic instruments, so a hand port was used to mobilize the spleen to the left upper quadrant (LUQ). No evidence of attenuated suspensory ligaments was seen. The spleen was secured in the left subdiaphragmatic location by the use of a Vicryl mesh bag. An omental sling was used to support the spleen further. A 1-year follow up CT confirmed that the spleen still was located in the correct anatomic position. This is a unique case in that the patient was known to have a normally located spleen at a young age and then found to have a wandering spleen later in life. This could have resulted from a congenital fusion anomaly or attenuation of the patient's suspensory ligaments caused by her previous malarial infection and splenomegaly.
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PMID:Video. Hand-assisted laparoscopy for wandering spleen. 2051 9

Wandering spleen with torsion, a rare clinical diagnosis, was found to be the cause of chronic abdominal pain in an 11-year-old female with a history of congenital diaphragmatic hernia repaired at three days of age. Doppler ultrasound revealed patent vessels with splenomegaly, and computed tomography (CT) showed an absence of the spleen in the left subphrenic space with torsion at the splenic hilum. Due to the chronicity of pain and risk of ischemia from torsion, open splenopexy with Vicryl mesh was performed. This case report/review of the literature discusses the rarity of this condition, and the importance of timely diagnosis and intervention.
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PMID:Splenic torsion after congenital diaphragmatic hernia repair: case report and review of the literature. 2348 Sep 45