Blue rubber bleb nevus syndrome is a rare disease of cutaneous hemangioma of a variant of the carvenous type which is clinically characterized by multiple, protuberant, dark blue, generally soft, rubbery, and compressible cutaneous masses usually associated with similar lesions of the gastrointestinal tract. Most cases are sporadic, although autosomal dominant inheritance has been described. We report herein a cases of blue rubber bleb nevus syndrome in a 49-year-old, who had multiple, dark blue, slightly tender, easily compressible vascular tumors on the face, right postauricular area, tongue, neck, and right had and foot, measuring from 0.5 to 1.0 cm in diameter. Endoscopic examination also revealed several similar lesions on the upper third of the esophagus. Biopsy specimen from the lesion of the neck showed irregularly dilated vessels containing red blood cells and fibrinous material. The vascular space was lined by a single layer of flattened endothelial cells.
Biopsy ; Blister* ; Endothelial Cells ; Erythrocytes ; Esophagus ; Fibrin ; Foot ; Gastrointestinal Tract ; Hemangioma ; Humans ; Middle Aged ; Neck ; Nevus* ; Rare Diseases ; Rubber* ; Tongue ; Wills

We report a case of infantile acute hemorrhagic edema in a 15-month-old boy. The patient showed multiple, bean to walnut-sized, red to purple-colored, indurated purpura with a cockade pattern on the face, earlobes, and extremities. Edema of the hands, feet, and earlobes was also observed. Laboratory examinations showed thrombocytosis, decreased serum CH50, and ketone in the urine. A skin biopsy taken from indurated purpura on the lower leg exhibited findings of acute leukocytoclastic vasculitis. Direct immunofluorescence examination showed deposition of IgM along the dermo-epidermal junction and C3 in the dermal vessel walls. With-out specific management except for fluid therapy to correct dehydration, the skin lesions cleared completely in 2 weeks, and recurrence has not observed.
Biopsy ; Dehydration ; Edema* ; Extremities ; Fluid Therapy ; Fluorescent Antibody Technique, Direct ; Foot ; Hand ; Humans ; Immunoglobulin M ; Infant ; Leg ; Male ; Purpura ; Recurrence ; Skin ; Thrombocytosis ; Vasculitis

BACKGROUND: The changes in lipid composition during epidermal differentiation has been reported in human and animal models. Because of the difficulties in getting adeguate specimens from human subjects, the authors used easily obtainable circumcised prepuce for lipid analysis. OBJECTIVE: To investigate the changes in lipid composition duriig cornification of the epidermis, the lipid compositions of whole epidermis and stratum corneum were analyzed by thin layer chromatography(TLC). METHODS: From circumcied prepuce whole epidermis and stratum orneum were separated by 10mM EDTA(ethylene diamine tatraacetate) in PBS(phosphate-buffered saline) or heat(60C), and 0.5% trypsin in PBS respectively. Lipids were extracted with methanolctloroform-HO mixture(4:2:1.6, v/ v, Bligh-Dyer solvent), TLC was performed and lipid composition was quantitated by photodensitometer. RESULTS: In the composition of stratum corneum lipids, sphingoliids were the highest(33.3+2.9%) followed by cholesterol, free fatty acids and cholesterol esters in cleceasing order, there were small percentages of triglycerides, cholesterol sulfate and squalene. CONCLUSION: In this study the lipid composition of epidermis was similar to that of stratum corneum rather than those of previous reports on epidermal lipids, which may indicate the regional characteristics of epidermal/stratum orneum lipids in hyperkeratotic prepuie.
Cholesterol ; Cholesterol Esters ; Epidermis* ; Fatty Acids, Nonesterified ; Humans ; Models, Animal ; Squalene ; Triglycerides ; Trypsin

From January 1988 to January 1996, 42 infants and children (44 renal units) had undergone surgical management to correct ureteropelvic junction obstruction. Median patient age was 8.5 years (range from 2 months to 17 years) and 11 patients were less than 1 year old at operation. Of 44 renal units surgically managed, 30 were on the left side and 10 were right side. 2 patients had undergone bilateral surgical management. Presenting symptoms were febrile urinary tract infection in 14 cases, abdominal pain in 14 cases, abdominal mass in 5 cases, gross hematuria in 3 cases and 3 cases were detected prenatally. We used imaging antegrade pyelography (AGP) in 15 cases, additional retrograde pyelography (RGP) in 12 cases and both AGP and RGP were performed in 1 case. To correct ureteropelvic junction obstruction, we performed dismembered pyeloplasty in 33 renal units, ureterolysis in 2 renal units, ureterocalycostomy in 1 renal unit, endopyelotomy in 1 renal unit and nephrectomy in 7 renal units. To diverge the urinary flow, we used nephrostomy in 19 renal units, ureteral stenting in 6 renal units and both nephrostomy and ureteral stenting were used in 10 renal units. As postoperative complications, restenosis was developed in 5 renal units, delayed open in 5 renal units, urinary tract infection in 2 renal units and wound infection, prolonged urine leakage, ureteral stone in each 1 renal unit. Postoperative success rate in followed-up patients, who had undergone pyeloplasty, was 91.6%. The success rate in children, who was less than 1 year old, was 100% and in children, who was more than 1 year old, was 88.4%. Finally we suggest that the surgical correction is safe and proper method for ureteropelvic junction obstruction in children. Additionally early operation of ureteropelvic junction obstruction is recommendable.
Abdominal Pain ; Child* ; Hematuria ; Humans ; Infant ; Nephrectomy ; Postoperative Complications ; Stents ; Ureter ; Urinary Tract Infections ; Urography ; Wound Infection

Background: The present study investigates whether modifiable behavioral factors of current cigarette smoking, heavy alcohol consumption, and regular exercise are associated with risk of lower extremity amputation (LEA) in diabetic patients. Methods: A total of 2,644,440 diabetic patients (aged ≥20 years) was analyzed using the database of the Korean National Health Insurance Service. Cox proportional hazard regression was used to assess adjusted hazard ratios (HRs) for the behavioral factors with risk of LEA under adjustment for potential confounders. Results: The risk of LEA was significantly increased by current cigarette smoking and heavy alcohol consumption (HR, 1.436; 95% confidence interval [CI], 1.367 to 1.508 and HR, 1.082; 95% CI, 1.011 to 1.158) but significantly decreased with regular exercise (HR, 0.745; 95% CI, 0.706 to 0.786) after adjusting for age, sex, smoking, alcohol consumption, exercise, low income, hypertension, dyslipidemia, body mass index, using insulin or oral antidiabetic drugs, and diabetic duration. A synergistically increased risk of LEA was observed with larger number of risky behaviors. Conclusion Modification of behaviors of current smoking, heavy alcohol intake, and exercise prevents LEA and can improve physical, emotional, and social quality of life in diabetic patients.

PURPOSE: To evaluate the hemodynamic status of varicocele, 99m Tc-RBC venography was performed in 29 varicocele patients and 10 normal controls from January to July 1996. MATERIALS AND METHODS: The mean age of varicocele patients was 23 years old and of normal controls was 27 years old. Among 29 varicocele patients, 26 had varicocele in left side and 3 had in both side. In unilateral varicocele group. 2 cases had grade I, 8 cases had grade II and 16 cases had grade III varicocele. In bilateral varicocele group, all had grade III in le% side and grade II varicocele in right side. RESULTS: The mean+/-SD of left to right radionuclide uptake ratio in pampiniform plexus during resting and Valsalva maneuver state was 1.01 +/-0.04:1 and 1.05+/-0.05:1 in normal control, 1.05+/-0.02:1 and 1.10:1 in varicocle grade I, 1.21+/-0.19:1 and 1.21 +/-0.12:1 in varicocele glade II, 1.60+/-0.63:1 and 1.27+/-0.18:1 in varicocele grade III, 1 18+/-0.06:1 and 1.26 in bilateral varicocele. As varicocele grade be higher, the mean left to right radionuclide uptake ratio be larger during resting and Valsalva maneuver state. CONCLUSIONS: 99mTc-RBC venography can quantify the hemodynamic state of varicocele so we suggest that it is useful as a method of diagnosis and follow up in varicocele patient.
Adult ; Diagnosis ; Follow-Up Studies ; Hemodynamics* ; Humans ; Phlebography* ; Valsalva Maneuver ; Varicocele* ; Young Adult

Renal angiomyolipomas are uncommon benign neoplasms composed of mature adipose tissue, thick walled blood vessels and smooth muscle in varying proportions. Accurate preoperative diagnosis of renal angiomyolipoma is essential if conservative resection or angiographic embolization is to be used. In recent years, abdominal computerized tomography (CT) and renal ultrasonography have resolved the diagnostic dilemma, making detection of angiomyolipomas possible in almost all cases. Recently, we performed selective renal angioembolizations in 3 patients (2 females, 1 male). After followup period of 6 months to 1 year, all patient got better in clinical symptoms. In two of them the lesions decreased in size on followup CT but one patient showed suspicious malignant change and we performed the exploration to rule out renal malignancy. In our opinion, it is appropriate to perform the selective renal angioembolization for initial treatment of renal angiomyolipomas.
Adipose Tissue ; Angiomyolipoma* ; Blood Vessels ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Muscle, Smooth ; Ultrasonography

Clear cell carcinoma of the ovary is a clinicopathologic entity with distinct morphologic characteristics and known to be associated with poor prognosis irrespective of stage and prognostic benefits of chemotherapy have not been evidently demonstrated. This represents 2-3.6% of all ovarian malignancies. From October, 1991 to January, 1998, nine patients with primary clear cell carcinoma of the ovary treated in the deptartment of Gynecology and Obstetrics, University of Ulsan, Asan Medical Center were identified, and clinical and histologic review(including architectural pattern, mitotic activity, nuclear grading) was performed retrospectively. The age of the patients ranged from 30 to 67 year(mean+S.D.; 46.0+11.8) and the mean parity was 1.2 (+1.3). Five(55.6%) of nine patients had elevated level of CA 125. The size of the tumors varied from 3 cm to 20 cm with the mean maximal diameter of 12.1 (+6.3) cm. The FIGO stages of the patients were IC(6 cases, 66.7%), IIC (2 cases, 22.2%) and IV(1 case, 11.1%). All patients except one were treated by total abdominal hysterectomy and bilateral salpingoophorectomy with or without omentectomy and pelvic lymph node dissection. All except two were received postoperative chemotherapy with regimens such as CEC (cyclophosphamide + epirubicin + carboplatin), TC(taxol + carboplatin), CC(cyclophosphamide + carboplatin) or CAP (cyclophosphamide + adriamycin + cisplatin). The mean follow-up duration was 14.6 (+ 8.6) months and 8 patients were free of disease and one patient with stage IV disease died of disease during postoperative chemotherapy(4 months after surgery).
Chungcheongnam-do ; Doxorubicin ; Drug Therapy ; Epirubicin ; Female ; Follow-Up Studies ; Gynecology ; Humans ; Hysterectomy ; Lymph Node Excision ; Obstetrics ; Ovary* ; Parity ; Prognosis ; Retrospective Studies ; Ulsan

Birth brachial plexus palsy (BBPP) is usually caused by plexus traction during difficult delivery. Although the possibility of complete recovery is relatively high, 5% to 25% of BBPP cases result in prolonged and persistent disability. In particular, muscle imbalance and co-contraction around the shoulder and elbow cause abnormal motor performance, osseous deformities, and joint contracture. Physical and occupational therapies have most commonly been used, but these conventional therapeutic strategies have often been inadequate, in managing the residual muscle imbalance and muscle co-contraction. Therefore, we attempted to improve the functional movements, by using botulinum toxin type A, to reduce the abnormal co-contraction of the antagonist muscles.
Botulinum Toxins* ; Botulinum Toxins, Type A ; Brachial Plexus Neuropathies ; Brachial Plexus* ; Congenital Abnormalities ; Contracture ; Elbow ; Joints ; Muscles ; Occupational Therapy ; Paralysis* ; Parturition* ; Shoulder ; Traction

Cough is one of the most common symptoms that causes patients to seek outpatient medical care. If cough persists longer than 8 weeks, common causes of chronic cough, such as upper airway cough syndrome, asthma, and gastroesophageal reflux disease (GERD), should be considered. Although not a common cause of chronic cough, achalasia may cause symptoms very similar to reflux that can lead to its misdiagnosis as GERD. In this report, a 40-year-old woman presenting with chronic cough was initially diagnosed with GERD; however, her symptoms were refractory to conventional GERD treatment. Finally, she was diagnosed with achalasia. Her cough improved completely after pneumatic dilatation. Achalasia is a rare disease accompanied by dysphagia or regurgitation. If cough presumably due to GERD does not respond to treatment, or if the cause of chronic cough is uncertain, physicians should suspect achalasia.
Adult ; Asthma ; Cough* ; Deglutition Disorders ; Diagnostic Errors ; Dilatation ; Esophageal Achalasia* ; Female ; Gastroesophageal Reflux* ; Humans ; Outpatients ; Rare Diseases