5 alpha-reductase deficiency resulting in male pseudohermaphroditism is a rare disease characterized by clitoral-like phallus, bifid scrotum, urogenital sinus, testis cited in labioscrotal folds. Evaluation of plasma T/DHT ratios in infancy, particularly after hCG stimulation of the testes and elevated urinary tetrahydrocortisol (THF) to 5 alpha-tetrahydrocortisol(5 alpha-THF) ratios provide a valuable dianostic test for 5 alpha-reductase deficiency. We report one case of 5 alpha-reductase deficiency who were presented with ambiguous genitalia and elevated T/DHT ratio before and after hCG stimulation.
46, XY Disorders of Sex Development ; Cholestenone 5 alpha-Reductase* ; Disorders of Sex Development ; Plasma ; Rare Diseases ; Scrotum ; Testis ; Tetrahydrocortisol

The angle of the femoral anteverison has been recognized as an important consideration in the orthopedic Geld. The angle of the femoral anteversion can be measured roentgenographically by 3 methods, such as fluoroscopic, biplanar and axial methods. It is the purpose of this study to investigate the comparative accuracy of the roentgenographic methods determining the angle of the femoral anteversion. The measurements by the fluoroscopic(Rogers), biplanar (Magilligan) and axial (Dunn) methods were compared with the direct measurement of 42 adult dried femora and the measured values were statistically analyzed. The results were as follows: l. Among fluoroscopic, biplanar and axial methods, the biplanar method using Magilligan technique was the statistically reliable method to determine the angle of the femoral anteversion. 2. The axial method showed tendency to underestimate the angle of the femoral anteversion. 3. Though the fluoroscopic method was not so accurate as the biplanar method statistically, the 2 methods showed no difference in the ratio of measurement error within ±5°and ±10° range.
Adult ; Humans ; Methods ; Orthopedics

Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Blood Glucose ; Congenital Hyperinsulinism* ; Diazoxide ; Glucose ; Humans ; Hypoglycemia ; Infant, Newborn* ; Insulin ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Somatostatin

Primary hemangioma of the lymph node is an extremely rare vascular neoplasm, and only four cases on the subject have been reported in the literature. We describe a case of cavernous hemangioma in an axillary lymph node that was incidentally found in 70-year-old woman who underwent a modified radical mastectomy for infiltrating duct carcinoma of the left breast. Brief review of the literature regarding vasoformative lesions occupying lymph node is made with special regard to differential diagnosis from reactive-proliferative processes and other true neoplasms.
Female ; Humans ; Diagnosis, Differential ; Hemangioma

No abstract available.
Replantation*

Although the reported number of malignant lymphoma of the thyroid has been increased, primary malignant lymphoma of the thyroid associated with chronic lymphocytic thyroiditis has been sporadically reported since its first description by Graham in 1931. The apparent coexistence of these two conditions has undoubtedly been noted by other observers. And they suggested that malignant lymphoma of the thyroid might arise from the lymphoid tissue ina wide variety of preexisting thyroidal disease. This report was made to record a case of primary thyroid lymphoma recently seen in this department with special emphasis on the associated thyroiditic changes in the uninvolved portion of the gland, which suggest the antecedent presence of chronic lymphocytic thyroiditis.

This report describes the pathologic features of 17 cases of Castleman's disease, examined at the Department of Pathology, Seoul National University Hospital during a period from 1973 to 1989. The lesions in 12 cases were hyaline-vascular type and the remainders plasma cell type. The pathologic features favoring the plasma cell type over the hyaline vascular type included a sufficient number to large-sized follicles. However, a histologic overlapping between two types was present. In the hyaline vascular type the age of the patients ranged from 7 to 76 years and they appeared to be no particular sex predominence. The majority of the lesions occurred in the neck and within the chest. Almost all cases presented with a solitary mass except three cases. Neither conventional symptoms nor systemic manifestations were associated. The plasma cell type was characterized by presentation of constitutional symptoms, involvement of intra abdominal and inguinal lymphnodes, in association with unusual clinicopathologic features including IgA nephropathy, diabetes mellitus, systemic progressive sclerosis, peripheral neuropathy, and anemia. Immunohistochemical study was performed in three cases of the plasma cell type. Two cases revealed poly-clonal plasma cell infiltration. In a patient with IgA nephropathy, however, serum IgA was increase and a strong immunoreactivity to IgA heavy chain was found. Another case, associated with systemic progressive sclerosis and neuropathy, revealed monoclonal plasma cell infiltration (IgG and lambda light chain). The above results support a possibility that in some of the plasma cell type an altered immune mechanism is involved in its pathogenesis.

PURPOSE: Caliceal diverticular calculi are mostly asymptomatic. In certain cases they may be associated with chronic or recurrent pain, recurrent urinary tract infections, progressive renal damage and hematur ia. The treatment of symptomatic caliceal diverticular calculi has evolved from open surgery to less invasive procedures, such as extracorporeal shock wave lithotripsy (SWL), percutaneous techniques, retrograde ureteroscopy and laparoscopy, but it remains controversial. Percutaneous techniques are frequently used to address the diverticular stone burden, to fulgurate the diverticular wall, to dilated the diverticular neck and improve drainage. For this reason, we reviewed 33 patients with caliceal diverticular calculi to determine whether they could be treated successfully by percutaneous nephrolithotomy (PCNL) as the initial treatment. MATERIALS AND METHODS: A total of 33 patients with caliceal diverticular calculi underwent PCNL between February 1990 and February 2000. The majority of diverticula were located at the upper pole. Access was gained via a direct target puncture. After sequential dilatation of the tract, stones were removed and the diverticular wall was fulgurated. The diverticular neck was dilated with the Amplatz dilator upto 24-30 Fr. and a 20 Fr. nephrostomy catheter was placed. RESULTS: PCNL was performed successfully in 30 patients (90.9%), but puncture was failed in 3. Mean operative time was 105 minutes and mean hospital stay was 4.5 days. All patients who were treated successfully with PCNL became stone-free without any complication. Mean follow-up was 20 months (ranged from 1 to 108 months). Of the 26 patients with symptoms preoperatively, 25 patients were rendered symptom-free (96.2%). Twenty-four patients were assessed with an intravenous urogram at 1 or 3 months and 6, 12 months and there was no stone recurrence. The diverticula were obliterated or had improved drainage in 95.8% (23/24) of assessable cases. CONCLUSIONS: We confirmed that PCNL is a safe, less invasive and effective procedure and should be considered an acceptable form of primary management of patients with caliceal diverticular stone.
Calculi ; Catheters ; Dilatation ; Diverticulum ; Drainage ; Follow-Up Studies ; Humans ; Laparoscopy ; Length of Stay ; Lithotripsy ; Neck ; Nephrostomy, Percutaneous ; Operative Time ; Punctures ; Recurrence ; Shock ; Ureteroscopy ; Urinary Tract Infections

A total of 66 cases of thymoma(57 surgically resected cases and 9 incisional biopsy cases) were reviewed with an attempt to correlate pathomorphologic features and clinical presentations. Criteria of benign or invasive thymoma were primarily determined by operative clinical and pathologic findings. Of them, 21 cases were invasive thymomas. The mean age of patients at the time of surgery was 47 years and it occurred largely in the sixth decade. Myasthenia gravis was accompanied in 29 cases(43%). One patient died during folow-up period, and five of the remainder suffered from recurrence. Microscopically, mixed type was the most common one(33 cases), being followed by predominantly epithelial type(17 cases) and predominantly lymphocytic type(16 cases). Thirty four cases of thymomas were cortical type, 29 mixed type, and the remaining 3 medullary type. None of the histologic type were significantly correlated with tumor invasiveness, Myasthenia gravis was more frequently associated with mixed and cortical type, respectively.
Biopsy

Forty-five patients with primary neoplasm of the spine, disgnosed and operated at Dept. of Orthopedic Surgery, Seoul National University Hospital, from 1970 to 1988, were reviewed in an attempt to analyze the incidence of the primary spine tumors and effectiveness of surgical treatment. Mean follow-up was 2.5 years. Among 29 benign tumors, 7 cases of osteoid osteoma, 6 cases of giant cell tumor, 4 cases of osteoblastoma and aneurysmal bone cyst, and 2 cases of fibrous dysplasia and osteochondroma, and others were identified. Of 16 malignant tumors, 6 cases of eosinophilic granuloms, 4 cases of chordoma, 3 cases of solitary plasmacytoms, and others were noted. 17 cases occurred in the anterior compartment, 18 cases in the posterior compartment, and 10 cases involved both compartments. Surgical treatment consisted of complete or near complete excision, decompression, and additional stabilization procedure when the stability of the vertebral column was compromised. Stablization was achieved either by fusion, strut bone graft, or by additional Zielke, Cotrel-Dubousset, or Luque-Harringtion instrumentation. The results were astisfactory in most of the cases with benign tumors except cases of giant cell tumor. The results in malignant tumor were satisfactory especially in eosinophilic granuloma and solitary plasmacytoma with respects to symptoms and survival, and this fact was attaibuted to good response to adjuvant chemotherapy and radiation therapy. Recent application of Cotrol-Dubousset or Zielke instrumentation after agressive resection made early ambulation a clinical reality.
Aneurysm ; Bone Cysts ; Chemotherapy, Adjuvant ; Chordoma ; Decompression ; Early Ambulation ; Eosinophilic Granuloma ; Eosinophils ; Follow-Up Studies ; Giant Cell Tumors ; Humans ; Incidence ; Orthopedics ; Osteoblastoma ; Osteochondroma ; Osteoma, Osteoid ; Plasmacytoma ; Seoul ; Spine ; Transplants