Klippel-Trenaunay-Weber syndrome is a rare, sporadically occuring disorder characterized by hemihypertrophy (unilateral limb hypertrophy), varicose veins, hemangiomata and occasionally arteriovenous malformation. With the development of ultrasonography, this syndrome can be detected by antenatal sonography. We report a case of Klippel-Trenaunay-Weber syndrome that demonstrates huge mass with increased blood flow on left thigh detected by ultrasonography.
Arteriovenous Malformations ; Extremities ; Klippel-Trenaunay-Weber Syndrome* ; Thigh ; Ultrasonography* ; Varicose Veins

The clinical data of 27 patiets with hypophosphatemic rickets treated with phosphate and 1alpha-hydroxyvitamin D were analysed retrospectively. The median age at diagnosis was 4 years, and the main clinical manifestations were bowleg and short stature. Among total 24 families, 5 families (21%) had X-linked dominant mode of inheritance, 1 family (4%) had autosomal dominant mode and 17 families (71%) had no family history, The serum phosphorus concentration rose from initial value of 2.7+/-0.13mg/dl to 3.5+/-0.19mg/dl. The serum alkaline phosphatase was reduced from 871+/-63IU/L to 393+/-41IU/L. Healing of rickets was demonstrated by radiography. Patients treated for at least two years before the onset of puberty had an increase in the mean height SD score from -1.58 to -0.79. Orthopedic surgeries for severe lower extremity deformity were performed in 11 patients, of whom 8(73%) were dignosed over 5 years of age. Complications of therapy were as follows; 12 patients (44%) developed more than one episode of hypercalciuria, 5 patients (19%) developed more than one episode of hypercalcemia, and nephrocalcinosis was noted in 5(33%) out of 15 patients by renal ultrasound. The group with nephrocalcinosis had a higher incidence of hypercalemic episodes than the group without nephrocalcinosis. In conclusion, treatment of hypophosphatemic rickets results in healing of rickets and acceleration of growth, and we must evaluate complications of therapy such as hypercalcemia and nephrocalcinosis.
Acceleration ; Adolescent ; Alkaline Phosphatase ; Congenital Abnormalities ; Diagnosis ; Humans ; Hypercalcemia ; Hypercalciuria ; Incidence ; Lower Extremity ; Nephrocalcinosis ; Orthopedics ; Phosphorus ; Puberty ; Radiography ; Retrospective Studies ; Rickets ; Rickets, Hypophosphatemic* ; Ultrasonography ; Wills

No abstract available.
Chorion* ; Chorionic Villi Sampling* ; Chorionic Villi* ; Female ; Humans ; Pregnancy

No abstract available.
Groin* ; Vulvar Neoplasms*

No abstract available.

A case of autonephrectomized tuberculosis of right kidney in 14-year old boy, complaining of abdominal pain, fever, and fist sized mass in right upper abdomen was reported with a brief review of the related literature.
Abdomen ; Abdominal Pain ; Adolescent ; Fever ; Humans ; Kidney* ; Male ; Tuberculosis*

Inactivation or loss of suppressor genes on a specific chromosome plays an important role in the development and progression of cancer. Recent studies have shown that p53 gene acts as a tumor suppressor gene and that its mutation appears to be related to the aggressiveness of transitional cell carcinoma of the bladder. To investigate the significance of p53 gene mutations in transitional cell carcinoma of the renal pelvis (renal pelvis tumor), 28 tumors with various stages and grades were examined for p53 gene mutations in exon regions 5 to 8 using polymerase chain reaction single-strand conformation polymorphism analysis. Seven (25%) of 28 pelvis tumors were found to have p53 gene mutations. Three of 12 superficial tumors including pTis, pTa, and pT1 were found to have p53 gene mutations. And only four of 16 invasive tumors with pT2, pT3, and pT4 were found to have p53 mutations. In the respect of tumor grade, p53 gene mutation was found in four of the 14 tumors with grade I and II, while three of 14 tumors with grade III, and IV were found to have p53 gene mutations. These observations suggest that, in contrast to bladder cancer, the incidence of p53 gene mutations does not related to the tumor stages and grades in transitional cell carcinoma of the renal pelvis. These results further indicate that p53 gene mutation may not represent a genetic marker of malignant potentials in transitional cell carcinoma of the renal pelvis.
Carcinoma, Transitional Cell* ; Exons ; Genes, p53* ; Genes, Suppressor ; Genes, Tumor Suppressor ; Genetic Markers ; Incidence ; Kidney Pelvis* ; Pelvis ; Polymerase Chain Reaction ; Urinary Bladder ; Urinary Bladder Neoplasms

Localized fibrous tumor is an uncommon submesothelial origin tumor found in pleura most commonly. Sixty five-year-old man with right lower quadrant pain was admitted. He was treated with resection of tumor and was diagnosed as localized fibrous tumor of pelvic cavity. He is now being followed up without any evidence of recurrence for 34 months. This case is presented with reviewing references.
Pleura ; Recurrence

An outbreak of dermatosis occurred in a livestock fodder factory at Kyongju in May 1994. Authors conducted a questionnaire survey on 60 workers in the factory and a dermatologist examined their skin lesion. Authors also collected mites and identified them. The obtained results were summarized as follows; 1. Twentyeight cases of dermatosis were identified with attack rate of 46.7%. Attack rate was not different by department, age, sex, educational level and employment duration. Attack rate was 67.5% in productive worker and 5.0% in clerical workers (p<0.01) but was not significantly different between departments among productive workers (p>0.05). 2. Three cases among 28 dermatosis cases and one subject among 32 non-cases had a history of same dermatosis last year. Only one of dermatosis cases had a history of dermatosis among family members. History of other skin lesion and allergy was very rare in both cases and non-cases. 3. Skin lesions of the cases were rice-sized erythematous papules or vesicles with a central biting point. Onset date of dermatosis was between May 1 and June 10. Duration was from one day to more than 30 days. Skin lesion was most frequent at the back(75.0%). and also observed at the arms(64.3%). abdomen(60.7%), legs(57.1%), chest(32.1%) and neck (25.0%). Skin lesion was aggravated while workers are sweeping the floor(35.7%), working at the workplace(21.4%) and in bed at night(28. 6%). 4. Total l,637 mites were collected and identified into 3 suborder, 7 families and 17 species. Dermafophagoides farinae was most frequent Nnd most of the species identified were blood sucking mites. Authors concluded that the outbreak of dermatosis was brought about by mite-bites and grain beetle parasitizing Acarophenax tribolii was the most suspected species. Further studies to identify the specific species causing dermatosis and route of import are needed.
Beetles ; Edible Grain ; Employment ; Epidemiologic Studies* ; Gyeongsangbuk-do ; Humans ; Hypersensitivity ; Korea ; Livestock ; Mites* ; Neck ; Surveys and Questionnaires ; Skin ; Skin Diseases*

Hailey-Hailey disease is a rare hereditary dermatosis that begins in the 2nd or 3rd decade of life. The skin lesion is characterized by a localized, recurrent eruption of small vesicles on an erythematous base. It courses remissions and exacerbations. It seldom begins in early childhood, and main treatment modalities are conservative ones. We report a case of Hailey-Hailey disease that began on a 7-month old infant and improved by surgical treatment. In according to review of the previous reports, it is probably the earliest onset age and it is may be the first case which was treated with surgery in Korea.
Age of Onset ; Humans ; Infant ; Korea ; Pemphigus, Benign Familial* ; Skin ; Skin Diseases