Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by concurrent nevus flammeus (capillary malformation) and pigmentary nevus. According to current research, the major pathophysiologic mechanism in PPV is venous dysplasia with resultant compensatory collateral channels and venous hypertension. Arterial involvement is rare. We herein report our experience on renovascular hypertension, intermittent claudication, and severe rhabdomyolysis due to diffuse stenosis of multiple arteries in a patient with PPV type IIb associated with SWS.
Arteries ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular ; Intermittent Claudication ; Intracranial Aneurysm ; Neurocutaneous Syndromes* ; Nevus ; Port-Wine Stain ; Rhabdomyolysis* ; Sturge-Weber Syndrome* ; Vascular Diseases

No abstract available.
Captopril* ; Child* ; Humans ; Nephrotic Syndrome*

No abstract available.
Captopril* ; Child* ; Humans ; Nephrotic Syndrome*

A case of Aicardi syndrome with cleft lip and palate was experienced at the Department of Pediatrics, College of Medicine, Hallym University, and the patient's infantile spasm was treated with ACTH. In previous studies, four cases of Aicardi syndrome accompanied by cleft lip and palate were reported. We present the fifth case of Aicardi syndrome with cleft lip and palate in the world. Thus, facial clefts may be found as an occasional manifestation of Aicardi syndrome.
Adrenocorticotropic Hormone ; Aicardi Syndrome* ; Cleft Lip* ; Infant ; Infant, Newborn ; Palate* ; Pediatrics ; Spasms, Infantile

Congenital aural atresia (CAA) with variable degrees of microtia are combined with many other otologic or facial anomalies. Among them, cholesteatoma developed behind atretic plate is rarely reported. Since Von Luders had first reported about this condition at 1912, several authors had described CAA associated with cholesteatoma. In their reports, atretic canal cannot prevent the development of cholesteatoma. We reviewed 4 cases of CAA associated with cholesteatoma with medical records and preoperative temporal bone CTs retrospectively. All the cases were unilaterally presented and corrected by canaloplsty. Keratin materials were demonstrated at the medial side of atretic ear canal.
Cholesteatoma* ; Ear Canal ; Medical Records ; Retrospective Studies ; Temporal Bone

We report a case of B-cell lymphoma with multiple asymmetrical cranial nerve palsies as the initial presentation. A 70-year-old woman complained of chin numbness, diplopia, dysarthria, and headache that had developed insidiously over the previous 2 months. The neurological examination showed multiple cranial nerve dysfunction, including right V and left VI nerve palsies. Her cerebrospinal fluid was normal, while her bone marrow biopsy revealed CD20-positive B-cell lymphoma. Five days after starting R-CHOP (rituximab-cyclophosphamide, adriamycin, vincristine, prednisolone) chemotherapy, her cranial nerve palsies and pain had improved markedly. Twenty days after starting R-CHOP chemotherapy, however, she was rehospitalized due to general worsening paralysis. In the hospital, the general paralysis progressed rapidly and she lapsed into delirium. No additional treatment was given based on the directives of her guardian and herself, and she was allowed to leave the hospital for hospice care.
Aged ; Antibodies, Monoclonal, Murine-Derived ; Antineoplastic Combined Chemotherapy Protocols ; B-Lymphocytes ; Biopsy ; Bone Marrow ; Chin ; Cranial Nerve Diseases ; Cranial Nerves ; Cyclophosphamide ; Delirium ; Diplopia ; Doxorubicin ; Dysarthria ; Female ; Headache ; Hospice Care ; Humans ; Hypesthesia ; Lymphoma ; Lymphoma, B-Cell ; Neurologic Examination ; Neurosyphilis ; Paralysis ; Prednisone ; Skull ; Skull Base ; Vincristine

Following the Korean War, presence of foreign troops in Korea, economic difficulty and changing moral Standards have brought about the development of concentrations of prostitutes around army camps known as "villes". The problems with venereal disease among the prostitutes living in the villes and consequently among soldiers has been a problem which has perplexed both American and Korean health authorities. Research into the problems and efforts to solve the V.D. problem have yielded only fragmentary results. Fortunately, with the cooperation of the U.S. Army Research and Development Group, an extensive research project has been undertaken to determine the root causes and best methods to prevent venereal disease in the villes around American military installations. The villes around large military installations were selected as the target of this research. In particular, the Pyeongtaek ville, the Gunsan "Silver Town" area and the "Texas Town" area of Busan were studied. The study took two years, from April, 1973 to March, 1975. Microbiological and serological studies were undertaken to determine the venereal disease infection rate and the efficacy of different antibiotics both in treatment and in sensitivity tests. In addition to the microbiological and serological studies other factors related to venereal diseases control such as social environment, health facilities, knowledge of venereal disease and its control were studied. The following results were obtained. 1. An overall gonorrheal morbidity rate of 14.1% was found among the 1,282 girls studied. This shows e rather dramatic rise compared to other studies. The majority of the increase is attributed to an actual increase of the V.D. rate, but more precise methods of isolation and culture using Thayer-Martin media also contributed to the increase recorded. 2. In Pyeongtaek, a gonorrhea morbidity rate of 5.39/0 (endocervical), the lowest in any of the villes was recorded. The Pyeongtaek police of enforced hospitalization until cure was effected tended to eliminate contact with infected girls and also make the girls more aware of and concerned about prevention of venereal disease. The Texas town prostitutes in Busan had a 15.5% (endocervical) morbidity rate, the highest of any of the areas. This might be attributed to sexual contact with many foreign sailors. 3. In culturing for gonorrhea, it was found that rectal as well as cervical cultures are necessary. Rectal cultures revealed 71 cases(39.2% to total positive cases) some of which were negative by endocervical culture, and endocervical cultures yielded 181 positives among the 1,282 cases surveyed. 4. A total of 988 serological tests for syphilis were carried out. By the V.D.R.L. method, 11.5% reactive cases were recorded, Kolmer C-F showed 5.9% reactive and RPCF test showed a 4.3% reactive rate. The RPR card test was used to retest 151 persons who were demonstrated to be V.D.R.L. (in prostitutes and general entertainers) reactive and confirmatory reactive results were shown in 74.0% of the cases. The FTA-ABS test was done on 57 RPCF tes/; reactive cases and showed confirmatory reactive results in 96.5% of the cases. It was concluded that the RPR card test, although it is less sensitive than the V.D.R.L test was both simple and effective for use in mass screening. However it is suggested that RPR card test rectors be reconfirmed using either the RPCF test or the FTA-ABS test. 5. Penicillin treatment failed to cure 40% of the gonorrhea patients in clinical tests and 16% of the strains used in the sensitivity tests showed resistance to penicillin. Therefore, penicillin was judged to be rather ineffective against gonorrhea. Ampicillin, however, is still quite effective, and spectinomycin was the most effective antibiotic against gonorrhea. 6. The socio-medical study of prostitutes revealed that their average age 25.5 years and the average number of years of education was 6. 8, implying the completion of primary school. Living conditions were generally poor, for example, in Pyeongtaek, 37% of the girls did not have running water and depended on well water. Data on the toilet facilities revealed that 78% of the prostitutes living in the control area in Wanweoldong, Busan were still using traditional Korean privy style toilets, and in Pyeongtaek 91% also were using privy toilets. Over two-thirds of the prostitutes only bathed once or twice a week. Therefore, improvement of living conditions was considered a critical problem. 7. Generally speaking, the girls had some know/edge of syphilis and gonorrhea and the measures necessary to prevent these diseases. However, over half of the girls abused antibiotics in venereal disease treatment and prevention attempts and only 65% of them required their partner to use a condom. Therefore, much improvement is needed in this area. 8. A survey of knowledge about contraceptive measures showed that 96% of the girls had good knowledge about oral contraceptives, 90 knew about condoms and 88% had knowledge of the douche method. In actual practice however, 95% of the girls used the douche method and 62% used condoms for contraception.
Ampicillin ; Anti-Bacterial Agents ; Baths ; Busan ; Condoms ; Contraception ; Contraceptives, Oral ; Education ; Female ; Fluorescent Treponemal Antibody-Absorption Test ; Gonorrhea ; Gyeonggi-do ; Health Facilities ; Hospitalization ; Humans ; Jeollabuk-do ; Korea* ; Korean War ; Mass Screening ; Military Personnel ; Penicillins ; Police ; Running ; Serologic Tests ; Sex Workers* ; Sexually Transmitted Diseases* ; Social Conditions ; Social Environment ; Spectinomycin ; Syphilis ; Texas ; Toilet Facilities

To examine the effectiveness and safety of erythropoietin on the anemia of end-stage chronic renal failure, we administered recombinant human erythropoietin(rHuEPO), Eporon(R), to 66 patients with anemia and chronic renal failure who were undergoing hemodialysis or peritoneal dialysis. All received Eporon(R) intravenously, two or three times per week at 150unit/kg, and then the dose of Eporon(R) was adjusted to 75-300unit/kg/week according to the hemoglobin response. The results of this study judged 19(39.6%) of 48 patients as being in a "markedly improved" condition while 23(47.9%) of 48 patients were judged as being in an "improved" condition. At results, the response rate of this study was 87.5%(42/48 patients). This was the same as the target response rate, 90%(p= 0.564). Serious adverse events occured in 6 cases during the study, but all proved to be unrelated to Eporon(R). These events included itching 1(1.5%), urticaria 1(1.5%) and headache 1(1.5%). No patients discontinued to participate in the study except one patient who withdrew from the study voluntarily. The symptoms of all adverse events eventually decreased and disappeared during the clinical study. No factors in the clinical laboratory tests changed significantly (p<0.05) except for the decrease of TSR and ferritin, a consequence of using iron hemoglobin synthesis caused by Eporon(R) administration. The significant increase in blood pressure, which could not be proved to be related to Eporon(R), disappeared during the study. Anti-Epo antibody was not detected in any samples. These results demonstrate that Eporon(R) is effective in many patients with the anemia of end-stage chronic renal failure.
Anemia* ; Blood Pressure ; Erythropoietin ; Ferritins ; Headache ; Humans* ; Iron ; Kidney Failure, Chronic* ; Peritoneal Dialysis ; Pruritus ; Renal Dialysis ; Urticaria

BACKGROUND AND OBJECTIVES: Several studies have attempted to show the reasons for ototoxicity induced by cisplatin, but the cochlear ototoxicity remained poorly understood. Recently, it is considered that free radicals play an important role in cisplatin ototoxicity. Heat shock proteins (HSP) are consistently present at some level in normal tissues and increased synthesis following stress. They have been implicated in the role of cellular protection during sub-lethal stressors. Many studies have demonstrated the increased synthesis of HSP following hyperthermia, ischemia, surgical injury and noise exposure. Free radicals are also considered as important inducer of HSP. The purpose of this study was to demonstrate the increase of HSP after cisplatin injection. MATERIALS AND METHOD: Thirty six Albano guinea pigs were used in this study. The animals were injected with a dose of 8 mg/kg cisplatin intraperitoneally. Cochleae are harvested 1, 3, 5 or 6, 12, 24 hours after injection. Immunocytochemistry and surface preparation method were used to detect the expression of HSP 72 in the cochlear tissues. RESULTS: The level of HSP 72 immunoreactivity began increasing by 3 hours after injection and continued to increase thereafter to reach maximal levels at 6 hours. Twelve hours after injection, the level of HSP 72 seemed to decrease to its normal levels. The increase of HSP 72 was mainly detected in Deiters' cells. CONCLUSION: Cisplatin induces a HSP in the guinea pig cochlea, particularly in the organ of Corti. However, further studies including quantitative analysis should be followed.
Animals ; Cisplatin* ; Cochlea ; Fever ; Free Radicals ; Guinea Pigs ; Heat-Shock Proteins* ; Hot Temperature* ; HSP72 Heat-Shock Proteins* ; Immunohistochemistry ; Intraoperative Complications ; Ischemia ; Noise ; Organ of Corti

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by concurrent nevus flammeus (capillary malformation) and pigmentary nevus. According to current research, the major pathophysiologic mechanism in PPV is venous dysplasia with resultant compensatory collateral channels and venous hypertension. Arterial involvement is rare. We herein report our experience on renovascular hypertension, intermittent claudication, and severe rhabdomyolysis due to diffuse stenosis of multiple arteries in a patient with PPV type IIb associated with SWS.
Arteries ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular ; Intermittent Claudication ; Intracranial Aneurysm ; Neurocutaneous Syndromes ; Nevus ; Port-Wine Stain ; Rhabdomyolysis ; Sturge-Weber Syndrome ; Vascular Diseases