Pfeiffer syndrome, an unusual type of acrocephalosyndactyly, is a complex of associated malformations, first described by Pfeiffer in 1964. In addition to the common head and face anomalies seen in other acrocephalosyndactylies, its characteristics are broad thumbs and big toes, minimal syndactyly and normal intelligence. It is inherited in autosomal dominant pattern and shows various clinical features. The author's case was a 7-year old boy, who had been managed since birth for bilateral congenital resistant clubfeet including cast correction for six months and two operations. At present he represents not only the common features described above but also some unique features, e.g. pectus excavatum, posterior dislocation of both elbows, mild genu valgum, metatarsus adductus and complex malalignment of carpal and tarsal bones. To our knowledge, there is no report on Pfeiffer syndrome in Korea. The authors report a case of Pfeiffer syndrome with review of literatures.
Acrocephalosyndactylia ; Dislocations ; Elbow ; Funnel Chest ; Genu Valgum ; Head ; Humans ; Intelligence ; Korea ; Male ; Metatarsus ; Parturition ; Syndactyly ; Tarsal Bones ; Thumb ; Toes

There are several considerations in performing free vascularized fibular graft for the treatment of the large bone defect, e.g., bone fixation, additional bone graft, and management of complication. Authors have analyzed 25 cases treated with free vascularized fibular graft at Holy Family Hospital between Jun. 1985 and Dec. 1994. The mean follow up was 27.4 months The results were as follows: 1. The causes of the defect were traumatic defect with infection of 16 cases, bone tumor of 4 cases, congenital pseudoarthrosis of tibia of 3 cases and osteomyelitis of 2 cases. 2. Hypertrophy of the graft was more common in the lower extremity and in the patient under the age of 15. 3. Nonunion of graft occurred in 4 cases(16%). These cases were fixed with screws and/or pin ini tially and subsequently treated with rigid internal fixation. 4. Stress fracture of graft occurred in 3 cases(12%). Two cases of them were treated with internal fixation. 5. Additional bone graft were performed in 6 cases(24%), among 17 cases who had bone defect of lower extremity in adult. In conclusion, authors emphasize that rigid internal fixation and additional bone graft in performing free vascularized fibular graft are recommended for obtaining early solid bony union and achieving early rehabilitation.
Adult ; Follow-Up Studies ; Fractures, Stress ; Humans ; Hypertrophy ; Lower Extremity ; Osteomyelitis ; Pseudarthrosis ; Rehabilitation ; Tibia ; Transplants

Incidence of non-contiguous multiple spine fracture was increased from 3.2%~4.5% at 1960th and 70th to 5.7% ~23.8% at 80th and 90th. But there is no reports in Korea which has high incidence of traffic and industrial accidents. In 345 consecutive patients with acute spine fracture, we found that 43 patients (12.5%) had multiple non-contiguous spine fractures. At the 43 patients studies 37 (87.4%) sustained fractures at 2 levels while 6 had more than 3 levels of injury, 26 were injured by fall from heights, 27 (62.8%) had 2.33 associated injuries on an average, and 12 (27.9%) had a neurologic deficit. There are five patterns account for 26 (50%) of the total number of patients. In 19 patients (44.2%), there was a mean 29.3 days delay (from 3 to 233 days) in diagnosis of the minor lesions. In 19 delay diagnosed minor lesions, it confirmed by bone scan in 12 patinets(63.6%). Two patients died within 24 hours after accident, and 41 patients (41 major and 50 minor fractures) were treated in our hospital. Among them, 21 (51.2%) needed modification of treatment by the presence of non-con- tiguous multiple spine fractures. According to above findings, the physician must be aware of the possible noncontiguous multiple spine fractures when examine and treat the patients with spine fracture.
Accidents, Occupational ; Diagnosis ; Humans ; Incidence ; Korea ; Neurologic Manifestations ; Spine

No abstract available.
Neck* ; Posture* ; Rabbits*

BACKGROUND: Xenologous or synthetic graft materials are commonly used as an alternative for autografts for guided bone regeneration. The purpose of this study was to evaluate effectiveness of carbonate apatite on the critical-size bone defect of rat's calvarium. METHODS: Thirty-six critical-size defects were created on 18 adult male Sprague-Dawley rat calvaria under general anesthesia. Calvarial bones were grinded with 8 mm in daimeter bilaterally and then filled with (1) no grafts (control, n = 10 defects), (2) bovine bone mineral (Bio-Oss®, Geistlich Pharma Ag. Swiss, n = 11 defects), and (3) hydroxyapatite (Bongros®, Bio@ Inc., Seongnam, Korea, n = 15 defects). At 4 and 8 weeks after surgery, the rats were sacrificed and all samples were processed for histological and histomorphometric analysis. RESULTS: At 4 weeks after surgery, group 3 (42.90 ± 9.33 %) showed a significant difference (p < 0.05) compared to the control (30.50 ± 6.05 %) and group 2 (28.53 ± 8.62 %). At 8 weeks after surgery, group 1 (50.21 ± 6.23 %), group 2 (54.12 ± 10.54 %), and group 3 (50.92 ± 6.05 %) showed no significant difference in the new bone formation. CONCLUSIONS: Bongros®-HA was thought to be the available material for regenerating the new bone formation.
Adult ; Anesthesia, General ; Animals ; Autografts ; Bone Regeneration ; Carbon ; Durapatite* ; Gyeonggi-do ; Heterografts ; Humans ; Korea ; Male ; Miners ; Osteogenesis ; Rats ; Rats, Sprague-Dawley ; Skull ; Transplants

PURPOSE: Patients requiring urethral reconstruction due to failed hypospadias repairs present a considerable technical challenge. Herein we report the experience of redo urethroplasties for complicated recurrent hypospadias. MATERIALS AND METHODS: From 1987 to 1997, we performed redo urethroplasty in 11 cases with complicated recurrent hypospadias. Types of recurrent hypospadias were coronal (1 case), distal shaft (4 cases), mid-shaft (1 case), and penoscrotal (5 cases) hypospadias. The interval between the last operation and redo operation was distributed from 8 months to 5 years. We analysed preoperative status, operative methods and their complications retrospectively. RESULTS: We performed urethroplasties using scrotal skin flap or buccal mucosal graft in only 2 cases. In spite of a paucity of usable penile skin, we performed urethroplasties with maximal use of remaining penile skin. Mathieu method was used in one case with coronal type. Mustarde operation was used in 4 cases with distal shaft hypospadias. And in 5 cases with penoscrotal type, we used Thiersch-Duplay method. Six of 11 patients had complications such as urethrocutaneous fistulas (4 cases) or recurrent hypospadias (2 cases) which were managed successfully later. CONCLUSIONS: Urethroplasty using penile skin maximally such as Mathieu, Mustarde, or Thiersch-Duplay methods appears to achieve success in patient with recurrent hypospadias subsequent to previous failed repairs.
Female ; Fistula ; Humans ; Hypospadias* ; Male ; Mustard Plant ; Retrospective Studies ; Skin ; Transplants

Renal cell carcinoma takes up 85 per cent of primary malignant renal tumors. It frequently metastasizes to the adrenal gland, lung, bone, liver and nephrectomy site but rarely to the head and neck area. When metastasizes to head and neck area, it involves the gingiva, tongue, palate, parotid gland, uvula, mandible, and lip. When tumors are found in the salivary gland consisting of clear cells, it is impartant to carry out differential diagnosis of various primary tumors with clear cell and metastatic renal cell carcinoma. Patients' medical history, review of system, and pathologic findings would be helpful factors in the differential diagnosis. In this paper, we report on a case of renal cell carcinoma which had metastasized to the psubmandibular gland after a radical nephrectomy.
Adrenal Glands ; Carcinoma, Renal Cell* ; Diagnosis, Differential ; Gingiva ; Head ; Lip ; Liver ; Lung ; Mandible ; Neck ; Nephrectomy ; Palate ; Parotid Gland ; Salivary Glands ; Submandibular Gland* ; Tongue ; Uvula

Inhibition of CD36, a fatty acid transporter, has been reported to prevent glucotoxicity and ameliorate high glucose induced beta cell dysfunction. Ezetimibe is a selective cholesterol absorption inhibitor that blocks Niemann Pick C1-like 1 protein, but may exert its effect through suppression of CD36. We attempted to clarify the beneficial effect of ezetimibe on insulin secreting cells and to determine whether this effect is related to change of CD36 expression. mRNA expression of insulin and CD36, intracellular peroxide level and glucose stimulated insulin secretion (GSIS) under normal (5.6 mM) or high glucose (30 mM) condition in INS-1 cells and primary rat islet cells were compared. Changes of the aforementioned factors with treatment with ezetimibe (20 μM) under normal or high glucose condition were also assessed. mRNA expression of insulin was decreased with high glucose, which was reversed by ezetimibe in both INS-1 cells and primary rat islets. CD36 mRNA expression was increased with high glucose, but decreased by ezetimibe in INS-1 cells and primary rat islets. Three-day treatment with high glucose resulted in an increase in intracellular peroxide level; however, it was decreased by treatment with ezetimibe. Decrease in GSIS by three-day treatment with high glucose was reversed by ezetimibe. Palmitate uptake following exposure to high glucose conditions for three days was significantly elevated, which was reversed by ezetimibe in INS-1 cells. Ezetimibe may prevent glucotoxicity in pancreatic β-cells through a decrease in fatty acid influx via inhibition of CD36.
Animals ; Anticholesteremic Agents/*pharmacology ; Antigens, CD36/antagonists & inhibitors/genetics/*metabolism ; Cells, Cultured ; Ezetimibe/*pharmacology ; Flow Cytometry ; Glucose/toxicity ; Insulin/genetics/metabolism/secretion ; Insulin-Secreting Cells/cytology/*drug effects/metabolism ; Male ; Palmitic Acid/metabolism ; RNA, Messenger/metabolism ; Rats ; Rats, Sprague-Dawley ; Reactive Oxygen Species/metabolism ; Real-Time Polymerase Chain Reaction

BACKGROUND: Acute lung injury (ALI) is a commonly encountered respiratory disease and its prognosis is poor when the treatment is not provided promptly and properly. However no specific pharmacologic treatment is currently available for ALI, although recently several supportive drugs have been under scrutiny. We studied anti-inflammatory effects of pentoxifylline (PF), a methylated xanthine, and ONO-5046, a synthetic neutrophil elastase inhibitor on lipopolysaccharide (LPS)-induced ALI in vitro. METHODS: To establish an in vitro model of LPS-induced ALI, primary rat alveolar macrophages and peripheral neutrophils in various ratios (1:0, 5:1,1:1,1:5,0:1) were co-cultured with transformed rat alveolar epithelial cells (L2 cell line) or vascular endothelial cells (IP2-E4 cell line) under LPS stimulation. Each experiment was divided into five groups-control, LPS, LPS+PF, LPS+ONO, and LPS+PF+ONO. We compared LPS-induced superoxide anion productions from primary rat alveolar macrophages and peripheral neutrophils in various ratios, and the resultant cytotoxxicity on L2 cells or IP2-E4 cells between groups. In addition we also compared the productions of tumor necrosis factor (TNF)-α, interleukin (IL)-1β, monocyte chemotactic protein(MCP)-1, IL-6, and IL-10 as will as mRNA expressions of TNF-α, inducible nitric oxide synthetase(iNOS), and MCP-1 from LPS-stimulated primary rat alveolar macrophages between groups. RESULTS: (1) PF and ONO-5046 in each or both showed a trend to suppress LPS-induced superoxide anion productions from primary rat alveolar macrophages and peripheral neutrophils regardless of their ratio, except for the LPS+PF+ONO group with the 1:5 ratio, although statistical significance was limited to a few selected experimental conditions. (2) PF and ONO-5046 in each or both showed a trend to prevent IP2-E4 cells from LPS-induced cytotoxicity by primary rat alveolar macrophages and peripheral neutrophils regardless their ratio, although statistical significance was limited to a few selected experimental conditions. The effects of PF and/or ONO-5046 on LPS-induced L2 cell cytotoxicity varied according to expaerimental conditions. (3) PF showed a trend to inhibit LPS-induced productions of TNF-α, MCP-1, and IL-10 from primary rat alveolar macrophages. ONO-5046 alone didnot affect the LPS-induced productions of proinflammatory cytokines from primary rat alveolar macrophages but the combination of PF and ONO-5046 showed a trend to suppress LPS-induced productions of TNF-αand IL-10 PF and ONO-5046 in each or both showed a trend to increase LPS-induced IL-β and IL-6 productions from primary rat alveolar macrophages. (4) PF and ONO-5046 in each or both showed atrend to attenuate LPS-induced mRNA expressions of TNF-α and MCP-1 from primary rat alveolar macrophages but at the same time showed a trend increase iNOS mRNA expression. CONCLUSION: These results suggest that PF and ONO-5046 may play a role in attenuating inflammation in LPS-induced ALI and that further study is needed to use these drugs as a new supportive therapeutic strategy for ALI.
Acute Lung Injury* ; Animals ; Cytokines ; Endothelial Cells ; Epithelial Cells ; Inflammation ; Interleukin-10 ; Interleukin-6 ; Interleukins ; Leukocyte Elastase* ; Macrophages, Alveolar ; Monocytes ; Neutrophils* ; Nitric Oxide ; Pentoxifylline* ; Prognosis ; Rats ; RNA, Messenger ; Superoxides ; Tumor Necrosis Factor-alpha ; Xanthine

MRI findings of extramedullary hematopoiesis of the spleen have not been described in the literature. Wereport the MRI features of this condition, as seen in two patients and confirmed by fine needle biopsy. Threesmall masses(< or =3cm) were isointense on T1WI, hyperintense on T2WI, and enhanced after the injection of gadolinium.Two 6cm-sized masses were hypointense on both T1WI and T2WI, and showed no contrast enhancement.
Biopsy, Fine-Needle ; Hematopoiesis, Extramedullary* ; Humans ; Magnetic Resonance Imaging* ; Primary Myelofibrosis* ; Spleen*