BACKGROUND: Congenital hypotrichosis is a non-specific, descriptive term for structural abnormalities of hairs showing variable clinical features. We recently have encountered a group of eleven patients exhibiting abnormal hairs showing similar patterns. OBJECTIVES: Our purpose was to clarify the entity of this structural hair disorder. METHODS: Dermatologic examination with routine histopathology, trichograms along with scanning electron-microscopic examination and laboratory studies were undertaken. RESULTS: All cases except one were female, and hair abnormalities had developed at birth or within a year. Diffuse distribution of thin, sparse, soft and hypopigmented hairs were noticed. On hair mounts, four patients showed tapering of roots. The majority of the patients exhibited cuticular changes, as well as pitting and longitudinal axial twisting by scanning electron-microscopic examination; one case demonstrated trichorrhexis nodosa, and another, trans-verse fracture. CONCLUSION: Although our cases bear some similar points with woolly hair, some differences were noted between this type of congenital hypotrichosis and other previously described syndromes.
Female ; Hair ; Humans ; Hypotrichosis* ; Parturition

39 patients with tibial shaft fracture had treated by open reduction and internal fixation with AO DCP and screws applying on the medial surface of the tibia, at the Department of Orthopedic Surgery, Ulsan Dongkang Hospital, from January, 1983 to December, 1985. A clinical study was done on all the 39 cases with the follow-up check over 1 year. In general, because the lateral surface of the tibia is well covered by rich soft tissue, it is popularized to apply the plate on the lateral aspect of the tibia. In our department, we applied the plate on the medial aspect of the tibia, which resulted in mimi-zing soft tissue injuries and, by inserting the screws perpendicular to the surface of the bone, increased stability of the fixation; and therefore resulted in relatively short operation time, relatively low incidences of infection and non-union; but there had been some drawbacks such as focal skin necrosis, hematoma, adhesion after fixatives removal, and cosmetic disfiguring. But there were no problems during the follow up periods. So, this is a recommandable procedure of internal fixation with the plate for the tibial shaft fractures.
Clinical Study ; Fixatives ; Follow-Up Studies ; Hematoma ; Humans ; Incidence ; Necrosis ; Orthopedics ; Skin ; Soft Tissue Injuries ; Tibia ; Ulsan

No abstract available.
Cicatrix, Hypertrophic ; Toes

No abstract available.
Skin Diseases

BACKGROUND: The occurrence of cutaneous malignant tumors has been increasing worldwide due to changes in various environmental factors. OBJECTIVE: Our study aimed to analyze the overall tendency of clinical characteristics in single-center patients with cutaneous malignant tumors according to sex, age, duration, size, anatomic site, treatment, and concomitant diseases. METHODS: We retrospectively reviewed the medical records and clinical photographs of 319 patients diagnosed with cutaneous malignant tumors (basal cell carcinoma, squamous cell carcinoma, melanoma, lymphoma, Paget's disease, metastatic skin cancer, mycosis fungoides, angiosarcoma, dermatofibrosarcoma protuberans, Kaposi sarcoma, malignant fibrous histiocytoma, Merkel cell carcinoma) and 109 patients diagnosed with premalignant tumors (Bowen's disease) between January 2007 and January 2017. RESULTS: The average annual incidence of malignant cutaneous tumors was 2.4%. In total, 158 males (36.9%) and 270 females (63.1%) were included with a mean age of onset of 66 years. Among the malignant tumors, basal cell carcinoma (30.8%) was the most common, while the incidences of squamous cell carcinoma (18.9%), melanoma (6.3%), and lymphoma (4.4%) were also high. The predominantly involved anatomic sites were the face (67.3%), trunk (11.5%), and legs (10.9%). Most cases involved solitarily existing tumors (77.3%), and pruritus (21.7%) was the most common accompanying symptom. Among the 69.6% of patients who received treatment for their tumors, the most common method was surgical removal. No significant increase in concomitant diseases or environmental factors was detected. CONCLUSION: Our study is meaningful as an overall and recent review of various types of cutaneous malignancies that provides preliminary data for further large-scale nationwide studies.
Age of Onset ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Chungcheongnam-do ; Dermatofibrosarcoma ; Female ; Hemangiosarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Incidence ; Leg ; Lymphoma ; Male ; Medical Records ; Melanoma ; Methods ; Mycosis Fungoides ; Pruritus ; Retrospective Studies ; Sarcoma, Kaposi ; Skin Neoplasms ; Statistics as Topic

No abstract available.
Erythrokeratodermia Variabilis

No abstract available.
Herpes Zoster*

Nephrogenic diabetes insipidus (NDI) is a disorder in which the secretion of antidiuretic hormone is normal, but the response of the renal collecting tubules to vasopressin is impaired. Compared with acquired NDI (a-NDI), which is secondary to chronic bilateral incomplete urinary tract obstruction with hydronephrosis, congenital NDI (c-NDI) is a very rare heritable disorder that usually follows the X- linked recessive pattern. Clinical symptoms of c-NDI can be non specific, and often the disease ultimately results in failure to thrive, or mental retardation. Recently, the diagnosis can be confirmed by direct sequencing analysis of the peripheral blood specimens. The long-term results of treatment for c-NDI are not satisfactory. Reports on the follow up of c-NDI cases are rare and there is no report on the cases treated with combinations of three drugs. We report herein a case of severe c-NDI in an 8 year-old-boy with a severely dysconfigurated urinary tract system. The patient and his mother showed a frameshift mutation on the AVPR2 gene on chromosome Xq28: .847_851delTGCTG (p.C283fsX90). The patient showed normal growth and development by treatment with combinations of hydrochlorothiazide (65 mg/m2), amiloride (0.3 mg/kg/d) and indomethacin (100 mg/m2), yet after five years he needed adjuvant cystostomy to relieve him from the residual symptoms of urgency with polyuria.
Amiloride ; Cystostomy ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Failure to Thrive ; Follow-Up Studies ; Frameshift Mutation ; Growth and Development ; Humans ; Hydrochlorothiazide ; Hydronephrosis ; Indomethacin ; Intellectual Disability ; Mothers ; Polyuria ; Urinary Tract ; Vasopressins

PURPOSE: In order to evaluate microstructural changes after shock wave exposure, gross, light microscopic and transmission electron microscopic findings were analyzed with rabbit gallgladders. MATERIALS AND METHODS: A preliminary study(2 rabbits) was performed to determine the dosage intensity of shock waves needed to inflict damage, using a EDAP LT 01 piezoelectric extracorporeal shock wave Iothotriptor. The gallbladders of three different groups of rabbits were given shock waves of various intensity. A storage value of 100, 50, 25 at rate of 20/sec under 80% power were given to group I (4 rabbits), group II( 4 rabbits), and group III(3 tabits), respectively. The rabbits were sacrified 6--12 hours later. RESULTS: The observed pathologic changes in the transmission electron microscopy were vaculization of cytoplasm and swelling of epithelial cells with dilatation and structural alteration of intracellular organelles, especially endoplasmic reticulum. Cell membrane rupture and necrosis were observed at the markedly affected area. The structural changes of intracellular organelles were minimally found at a storage value of 25. However, above pathologeic changes with dilatation and structural alterations of endoplasmic reticulums were more profund at value of 100. CONCLUSION: Early histologic changes induced by shock waves are dose dependent and the findings of cellular damage caused by ESWL might be explained as above.
Cell Membrane ; Cytoplasm ; Dilatation ; Endoplasmic Reticulum ; Epithelial Cells ; Gallbladder* ; Microscopy, Electron, Transmission ; Necrosis ; Organelles ; Rabbits ; Rupture ; Shock*