A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan sulfate and decreased enzyme activity of -iduronidase revealed that she has mild form of mucopolysaccharidosis type I. Her weakness was improved with cervical laminectomy and duraplasty. In this case, progressing quadriparesis resulted from cervical spinal cord compression by thickened dura mater. Earlier surgical decompression could have been better for the patient.
Decompression, Surgical ; Dermatan Sulfate ; Dura Mater ; Female ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Mucopolysaccharidoses* ; Mucopolysaccharidosis I* ; Quadriplegia ; Spinal Cord Compression* ; Spinal Cord* ; Spine ; Young Adult

PURPOSE:To report two cases of multicentric Castleman's disease involving the orbital area. CASE SUMMARY: A 52-year-old man presented with discomfort of the right eye on eyeball movement that began 4~5 months ago. He had been diagnosed as Castleman's disease upon the biopsy of his neck lymph nodes 2 years ago. Proptosis was found in his right eye, and orbital computed tomography revealed an enlarged mass in the inferior orbit compared to the former image. An incisional biopsy confirmed Castleman's disease. A 57-year-old man presented with left eyelid swelling that began 3 months ago. Orbital and chest computed tomography revealed a mass in the left superotemporal orbit and the enlarged lymph nodes of the mediastinal area. Castleman's disease was confirmed by biopsy of the lymph nodes in the mediastinal area. CONCLUSIONS: Steroid therapy was prescribed for the 52-year-old man. The 57-year-old man was placed under close observation.
Biopsy ; Exophthalmos ; Eye ; Eyelids ; Giant Lymph Node Hyperplasia ; Humans ; Lymph Nodes ; Middle Aged ; Neck ; Orbit ; Thorax

No abstract available.

The cytolytic activity of CD4' T cells, both human and murine, has been clearly demonstrated in the immune response to mycobacterial infection and suggested to play a significant role in the protection and immunopathology. However, Uttle is known about the differentiation of CD4' CTL. In order to address this issue, we examined the influences of some factors on the generation of CD4' CTL specific to mycobacterial antigens. After 7 days' stimulation of PBMCs from healthy tuberculin reactors with mycobacterial antigens, the cytolytic activity of purised CD4' T cells toward autologous macrophages infected with mycobacteria was measured by Cr release assay. First, we found that both of live M. tubeiculosis and soluble antigens (ST-CF) induced the cytolytic activity of CD4' T cells, although the inducibility of the former was slightly greater than the latter. Second, the cytolytic activity was maximally induced at the relatively low antigen concentration (0.2:1 bacteria:monocyte ratio or 0.5 mg/ml of ST-CF). Finally, in the presence of increasing amounts of neutralizing anti-IL-12 or anti-IFN-r MoAb, the cytolytic activity of CD4+ T cells was decreased in a dose-dependent manner. These results suggest that low dose of antigen, its particulate type give mycobacteria), IL-12, and IFN-r give some positive signals for the generation of CD4+ CTL.
Humans ; Interleukin-12 ; Macrophages ; T-Lymphocytes* ; Tuberculin

No abstract available.
Gastroschisis* ; Prenatal Diagnosis*

PURPOSE: We report clinical and radiologic outcomes after metacarpal extension osteotomy for mild osteoarthritis of the thumb carpometacarpal joint. MATERIALS AND METHODS: From 1999 to 2008, 11 patients were diagnosed with mild thumb carpometacarpal arthritis (Eaton stage I, II), and extension osteotomies were performed. Of these, seven patients with at least 6 years follow-up were analyzed retrospectively. Male to female ratio was 2:5, and mean age at time of surgery was 38.9 years old. Symptom onset period was a mean of 11.2 months. Two patients were I, and five patients were II in Eaton stage. Preoperative visual analogue scale (VAS) and disabilities of the arm, shoulder and hand scale (DASH) scores were 3.7 points (3-4 points), and 40.1 points (32-51 points). Radial abduction was 38.5degrees (30degrees-45degrees), and volar abduction was 42.1degrees (40degrees-45degrees). Grip strengths and pinch powers, compared with the normal contralateral side were 82% (64%-90%) and 72% (40%-100%), respectively. RESULTS: The mean follow-up period was 8.5 years, and all patients except one maintained their occupational activity during the follow-up period. Final VAS and DASH scores were 0.7 points (0-2 points) and 11.7 points (8-16 points), respectively, and were statistically significant. Volar abduction, grip strengths, and pinch power were improved to 45degrees (40degrees-50degrees), 92.3% (73%-117%), and 94.4% (75%-117%) with statistical significances. In five patients, Eaton stages did not change, and two patients advanced to the next stage (stage I to II in one patient, stage II to III in one patient). CONCLUSION: Among the various treatment options for mild thumb carpometacarpal arthritis, metacarpal extension osteotomy may be considered as an effective treatment.
Arm ; Arthritis* ; Carpometacarpal Joints ; Female ; Follow-Up Studies ; Hand ; Hand Strength ; Humans ; Male ; Osteoarthritis ; Osteotomy* ; Retrospective Studies* ; Shoulder ; Thumb*

Purpose: The goal of this study was to evaluate the efficacy of suction drains following total hip arthroplasties, by comparing the post-operative results between the group with suction drains and the group without suction drains. Materials and Methods: Eighty-six patients, who underwent primary total hip arthroplasties from June 2001 to June 2004, were divided into two groups: group 1 (48 patients), with suction drains; and group 2 (38 patients), without suction drains. We assessed the perioperative hemoglobin and platelet levels, the amount of total blood loss, the amount of post-operative blood transfusions, post-operative ranges of motion (ROMs, at 6 weeks), wound problems, and general conditions. Results: Although the postoperative hemoglobin level was greater in group 2 than in group 1, there was no statistically significant difference. The amount of total blood loss and blood transfusions in group 1 were statistically greater than in group 2 (p<0.05). There were no statistically significant differences in the post-operative ROMs and wound complications between groups 1 and 2. Conclusion: There were no limitations of hip motion and no wound complications in the patients without suction drains after total hip arthroplasties. However, they required fewer post-operative blood transfusions than did the patients with suction drains, due to less post-operative blood loss; and the absence of a suction drain might prevent retrograde tube infections. In addition, patients without suction drains after total hip arthroplasties seemed to recover better did than those with suction drains. Therefore, suction drains might provide no benefit in total hip arthroplasties.
Arthroplasty ; Arthroplasty, Replacement, Hip* ; Blood Platelets ; Blood Transfusion ; Hip ; Humans ; Suction* ; Wounds and Injuries

OBJECTIVE: The aim of this study were to compare the effects of EG and PROH on cryopreservation of mouse and human embryos, and to find the optimal protocol for embryo freezing. METHODS: Human embryos derived from fertilized eggs showing 3 pronuclei (PN) and mouse embryos were divided into two groups respectively: dehydrated with 1.5 M EG+0.2 M sucrose or 1.5 M PROH+0.2 M sucrose using the slow freezing method. Moreover mouse embryos were controlled the exposure time of cryoprotectant during dehydration or rehydration steps. RESULTS: The survival rates of human embryos were 79.2% (84/106) in EG group and 77.9% (88/113) in PROH group. In mouse embryos, the survival and development rates up to blastocyst were 70.6% (245/347), 44.1% (123/279) in EG group and 62.1% (198/319), 45.1% (123/279) in PROH group, respectively. However, in EG group, partially damaged embryos after thawing were decreased compared to PROH group. In combination group, when the exposure time during dehydration and rehydration were reduced, the survival and embryonic developments were increased slightly, but not significant. CONCLUSION: Cryopreservation of mouse and human embryos at cleavage stage by using EG or PROH exhibited no statistical difference in the survival rate and/or developmental rate to blastocyst. However, the use of EG for cryopreservation of embryos might reduce the exposure time of the cryoprotectant because of a high permeation of EG and result in lessen its toxic effects.
Animals ; Blastocyst ; Cryopreservation ; Dehydration ; Embryonic Development ; Embryonic Structures ; Ethylene Glycol* ; Female ; Fluid Therapy ; Freezing ; Humans* ; Mice* ; Pregnancy ; Propylene Glycol* ; Sucrose ; Survival Rate ; Zygote

PURPOSE: Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. METHODS: The study group consisted of 73 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. RESULTS: The age(mean+/-SD) of patients was 4.53+/-2.53 years old. The male to female ratio was 52:21. In 95%, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables:age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.006). CONCLUSION: The rapidity of response is expected to be one of the predictive factors for relapse in children with SRNS.
Biopsy ; Child* ; Diagnosis ; Female ; Gyeongsangbuk-do ; Hematuria ; Humans ; Male ; Medical Records ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Pediatrics ; Recurrence* ; Retrospective Studies

Fibroepithelial polyp (FEP) and lymphangioma circumscriptum (LC) of the vulva are rare diseases that occur generally in young to middle-aged women. The pathogenesis of FEP is not clearly understood. Several previous cases have suggested that a hormonal influence may be a predisposing condition for developing FEP with or without chronic inflammation. A 68-year-old postmenopausal woman presented with a history of multiple raised lesions on the vulva with a 1-year duration. Additionally, a pedunculated mass had appeared 6 months earlier in the left lower labia minora. The patient underwent simple excision by electrocautery. The final pathological diagnosis was FEP with LC. In this case, the development of FEP was associated only with chronic inflammation induced by LC. Therefore, the case supports the hypothesis that a chronic inflammatory process may be related to the development of FEP even without hormonal influence.
Aged ; Diagnosis ; Electrocoagulation ; Female ; Humans ; Inflammation ; Lymphangioma* ; Neoplasms, Fibroepithelial ; Polyps* ; Polytetrafluoroethylene ; Postmenopause ; Rare Diseases ; Vulva*