A Four-year Follow-up Case of Oligomeganephronia Detected Early by School Screening Urinalysis

Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7- year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.

A Case of Splenic Embolization Followed by Splenectomy with Preservation of Accessory Spleen in Gaucher's Disease

Gaucher's disease is an autosomal recessive genetic disorder of lipid metabolism. A deficiency of beta-glucocerebrosidase causes an accumulation of glucocerebroside in the reticulo-endothelial system and bone marrow. Total or partial splenectomy has been used in case of massive splenomegaly with hypersplenism and/or mechanical pressure symtoms. Partial splenectomy is preferred to prevent susceptibility to overwhelming postsplenectomy sepsis and to delay the massive deposition of glucocerebroside in the liver and bones. We report the case of a 20-year-old woman with Gaucher's disease and who had a splenic embolization 4 years ago. The spleen cross the midline of the abdomen reached to the true pelvis and elevated the left diaphragm. Angiotensin-converting enzyme, acid phophatase and ESR were increased but beta-glucocerebrosidase was normal. Osteosclerotic changes of the distal femur was observed. Hepatomegaly and splenomegaly with mutiple accessory spleens were seen on abdominal CT. On isotope scan for liver and spleen, multiple accessory spleens had isotope uptake, but spleen did not. We noted severe adhesion of spleen to neighboring structure and no viable splenic tissue for preservation. Total splenectomy with preservation of four accessory spleens was performed. We needed multiple transfusion during dissection and bleeding was continuous for 3 days postoperatively. The patient was discharged without problems on the postoperative 15 th day.

Endoscopic Fine Needle Aspiration Cytology in the Diagnosis of Upper Gastrointestinal Malignancies / 대한소화기내시경학회지

The endoscopic fine needle aspiration cytology may add to the diagnostic accuracy of endoscopic biopsy and brush cytology. It is also of particular value in submucosal, infiltrative and ulceronecrotic tumors. Endoscopic fine needle aspiration cytology was done with sclerotherapy needle(23 gauge) in. the 17 patients with submucosal tumor(18 cases), extrinsic compression(2 cases), infiltrative gastric cancer(one case) and cancer of the ampulla of Vater(one case) on the upper gastrointestinal endoscopy. Endoscopic fine needle aspiration cytology established the diagnosis in 6 cases(submucosal tumor of the stomach; 2 cases, submucosal tumor of the duodenum; one case, extrinsic mass of the duodenum; one case, infiltrative cancer of the stomach; one case, and the cancer of ampulla of Vater; one case) of 17 cases. There were negative results for malignancy in 7 cases and material insufficiency in 4 cases. Five cases of the positive results with endoscopic fine needle aspiration cytology were not diagnosed with endoscopic forceps biopsies. There was no complication. We conclude that endoscopic fine needle aspiration cytology is a simple and safe technique and is of particular value in submucosal tumor, extrinsic compression of the upper gastrointestinal tract by tumor, and infiltrative gastric cancer.