We reported two cases of elephantiasis neurofibromatosa in a family. The first case was the son of the second case. Both patients showed huge masses and bone changs. They were treated by surgical excision due to functional and cosmetic problems. The histopathologic findings of the two cases were consistent with neurofibroma.
Elephantiasis* ; Humans ; Neurofibroma ; Neurofibromatoses*

Basaloid follicular hamartoma (BFH) is a benign adnexal tumor with a wide spectrum of clinical appearance and is characterized histologically by the presence of branching cords and thin strands of undifferentiated anasomosing, basaloid proliferations that are embedded in a fibrous stroma. It has been often misdiagnosed and treated as basal cell carcinoma. We report a 57-year-old woman with BFH on the face which is similar to basal cell carcinorna clinically that turned out to be a solitary BFH histologically. There has been no recurrence during one year of follow up after excisional biopsy.
Biopsy ; Carcinoma, Basal Cell ; Female ; Follow-Up Studies ; Hamartoma* ; Humans ; Middle Aged ; Recurrence

No abstract available.
*Bile Duct Diseases/diagnosis ; *Bile Ducts, Intrahepatic ; Female ; *Hamartoma/diagnosis ; Humans ; Liver Diseases/diagnosis ; Middle Aged

BACKGROUND: Herpes zoster is characterized by vesicular skin lesions over the unilateral sensory dermatomes being caused by the reactivation of varicella-zoster virus and its incidence seems to be increasing recently. OBJECTIVE: The purpose of this study was to elucidate the epidemiology and the clinical characteristics of herpes zoster. METHODS: The authors observed 877 cases of herpes zoster clinically for annual incidence, age, sex, monthly distribution, predilection site, associated disease, and complications at the Department of Dermatology in Chung-Ang University Hospital during a 10 years period(1982.9-1992.8). RESULTS: 1) The annual incidence averaged over 10 years was 2.26%(877 cases of total 38.717 outpatients) being on the increase recently. 2) The age distribution was in the range of 2-86 years old being most prevalent in the 6th decade and there was equal sex ratio(444:433). 3) There was no statistically significant monthly or seasonal variation in the incidence of herpes zoster. 4) The most common anatomical distribution was thoracic dermatome(51.7%), followed by trigeminal(17.1%), cervical(16.1%), lumbar (10.6%), sacral(1.4%)and multiple deramatomic involvement(3.1%). Left or right side was affected in about the same ratio(439:437), and bilateral involvement was in 1 case. 5) Associated disease of herpes zoster were observed in 210 patients(23.9%) ; hypertension(7.0%), diabetes mellitus(4.1%), tuberculosis(1.8%), postoperative status(0.7%), malignancy(0.7%), gastric ulcer(0.7%), and so on, 6) The most common complication of herpes zoster was postherpetic neuralgia(7.4%), followed by eye complication(2.1%), bacterial infection(1.4%), scar formation(0.7%), neurogenic bladder(0.2%), Ramsay-Hunt syndrome(0.2%), motor paralysis(0.1%), herpes zoster generalisatus(0.1%) 7) The incidence of postherpetic neuralgia increased with age and was highest in trigeminal dermatome. 8) Recurrence of herpes zoster was observed in 4 patients(0.5%). CONCLUSION: According to this study, the annual incidence of herpes zoster showed a tendency to increase recently but there was no significant monthly or seasonal variation. The anatomical distribution was most commom on the thoracic dermatome. The incidenc of postherpetic neuralgia increased with age being most common in trigeminal nerve.
Age Distribution ; Cicatrix ; Dermatology ; Epidemiology ; Herpes Zoster* ; Herpesvirus 3, Human ; Incidence ; Neuralgia, Postherpetic ; Recurrence ; Seasons ; Skin ; Trigeminal Nerve

OBJECTIVE: The aim of this study was to evaluate the role of lower uterine segment thickness in predicting an actual delivery date and to determine the factors affecting the depth of lower uterine segment. METHODS: Sixty patients with singleton gestation were weekly measured for their lower uterine segment (LUS), cervical length, cervical gland thickness and AFI by ultrasonography from 36 weeks. The regression analysis was carried to find out the relevance between LUS and the remaining days to birth and Pearson correlation analysis was performed for relevance between LUS and other factors such as maternal age, parity, cervical length, cervical gland thickness, and AFI. RESULTS: LUS became thin as cervical length decreasing. However, the relevance between LUS and other factors such as age, parity, cervical gland thickness, and AFI was not found. While individual LUS thickness is decreasing as delivery date becomes closer, LUS thickness as a group is found not to be related with the delivery date. CONCLUSION: In term pregnancy, LUS thickness decreases with cervical change but it cannot predict the delivery date.
Female ; Humans ; Maternal Age ; Parity ; Parturition ; Pregnancy* ; Ultrasonography

OBJECTIVE: The objective of this study is to determine whether the club foot of the affected fetus is related to the location, size, and rupture state of the spinal neural tube defect lesion. METHODS: Ultrasound and medical records of 21 fetuses with spinal neural tube defect were reviewed. At the time of diagnosis 7 fetuses had clubfoot and 14 fetuses didn't have. All the fetuses with spinal neural tube defect were divided into two groups, one with clubfoot fetuses, and the others without clubfoot. RESULTS: The mean lesion size of the clubfoot group was significantly larger than normal foot group (4.1+/-1.22 cm vs 2.1+/-1.59 cm). And the level of lesion is significantly higher in club foot group. But the rate of sac rupture is lower in club foot group. CONCLUSION: The fetuses with clubfoot detected at the time of diagnosis had larger size and higher level of lesion.
Clubfoot ; Diagnosis ; Fetus* ; Foot* ; Medical Records ; Neural Tube Defects* ; Neural Tube* ; Rupture ; Ultrasonography ; Ultrasonography, Prenatal*

We report a case of transient abnormal myelopoiesis in a Down syndrome fetus diagnosed at 28(+3) weeks of gestation that rapidly progressed to intrauterine death 10 days later. Fetal hepatosplenomegaly with cerebral ventriculomegaly, although not specific, may be a suggestive finding of Down syndrome with transient abnormal myelopoiesis. Prompt fetal blood sampling for liver function test and chromosomal analysis are mandatory for early detection and management.
Adult ; Down Syndrome/*ultrasonography ; Female ; Fetal Blood/cytology ; Fetal Death ; Fetal Diseases/*diagnosis ; Hepatomegaly/ultrasonography ; Humans ; Leukocytosis/diagnosis ; *Myelopoiesis ; Pregnancy ; *Prenatal Diagnosis ; Splenomegaly/ultrasonography ; Thrombocytopenia/diagnosis

PURPOSE: Speech and language development is an useful indicator of the overall development of children. Since speech and language delay can lead to emotional, social, and learning problems, the early intervention is very important. We examined 137 children with speech or language problems including 22 children of functional articulation disorders of their clinical features. METHODS: 137 children with speech or language problems in the Department of Pediatrics, Uijeongbu St. Mary's Hospital from January 2004 to December 2007 were reviewed for sex, age, developmental and language test findings retrospectively. Especially, in 22 children diagnosed with functional articulation disorders, articulation test findings were analyzed. RESULTS: 1) The mean age of 137 children was 46.8 months, the ratio of male to female was 2.5:1, and the most frequent age group was 24-35 months. The chief complaints included language delay(67.2%), mispronouncing speech sound(27%), stuttering(3.6%), learning disability(2.2%). The diagnoses of them were developmental language disorder(70.8%), functional articulation disorders(16.1%), mental retardation(5.8%), stuttering(2.9%), tongue tie(0.7%), normal language pattern(3.6%). 2) The mean age of 22 patients with functional articulation disorders was 63 months, the ratio of male to female was 1.4:1, and the most frequent age group was 60-71 months. The mean percentage of consonant correct of Picture Consonant Articulation Test was higher in older age group. In the types of phonetic errors substitutions were most common. Distortions, omissions, and additions were followed in the order of frequency. Substitutions and distortions were common in word initial and medial but omissions in word final. It is appeared that the patients had the difficulties in pronouncing alveolar fricative, palatal affricate, liquid, and velar plosive which mature lately in older children. Mainly palatal affricate, alveolar fricative, and velar plosive were substituted for alveolar plosive and the liquid and alveolar fricative were distorted as gliding. CONCLUSION: Categorizing the cause of language and speech delay is essential to make an adequate treatment plans and decrease the late complications. The future studies for the early screening, more suitable tests in Korean, treatment guidelines or prognosis are needed.
Articulation Disorders ; Child ; Early Intervention (Education) ; Female ; Humans ; Language Development ; Language Development Disorders ; Language Tests ; Learning ; Male ; Mass Screening ; Pediatrics ; Prognosis ; Retrospective Studies ; Tongue

Between January, 1990, and December, 1994, 105 elderly patients(over the age of sixty) were referred to the Division of Nephrology at the Seoul National University Hospital as acute renal failure(ARF) (serum creatinine >1.7mg/dL, patients who had been diagnosed to have acute on chronic renal failure were excluded). To find out the characteristics of ARF in the elderly, we made a retrospective study of our data. Sufficient data for analysis were available in 101 of these. Among these patients, prerenal failure occured in 5% of the cases, ischemic ATN 34%, toxic ATN 11%, renovascular obstruction 5%, glomerular disease 5%, postrenal failure 15%. Dialysis was required in 31 patients(31%). Twenty four patients were treated by hemodialysis, it was carried out in 1 patient by the peritoneal route, and the other 6 patients were treated by CAVH. The most common indication was hypervolemia(77%). Twenty seven patients died during the period of acute renal failure. The most common cause of death was infection(15 patients), and the others were underlying diseases, pulmonary complications and cardiovascular complications. Oliguria, and chronic underlying disease were poor prognostic factors. There were significant differences between living and died group in APACHE II score(P<0.05). We conclude that ischemic ATN is a more common cause of ARF in the elderly than in the younger, and presence of oliguria and chronic underlying disease are poor prognostic factors.
Acute Kidney Injury* ; Aged* ; APACHE ; Cause of Death ; Creatinine ; Dialysis ; Hemofiltration ; Humans ; Kidney Failure, Chronic ; Lung Diseases ; Mortality ; Nephrology ; Oliguria ; Renal Dialysis ; Retrospective Studies ; Seoul

BACKGROUND: Dendritic epidermal T cells(DETC) are skin specific members of the epithelial gammadelta-T cell family that reside normally in mouse epidermis. Although the mechanisms of antigen recognition by alpabeta-T cells have become better defined, the physiological role of the gammadelta-T cells and ligands they recognize are still poorly understood. OBJECT: In the present study we sought to elucidate the antigen specificity and whether the DETC recognize transformed keratinocyte derived antigen. METHOD: We used a DETC and TCR(-) DETC which were obtained by 1600R gamma-irradiation and sorting, in addition to transformed keratinocyte cell lines. We performed coculture experiments of DETC/transformed keratinocyte cell lines to detect direct evidence that DETC recognize the keratinocyte-derived antigen. RESULT: 1. TCR negative variants of DETC do not respond to concanavalin(Con)-A, but respond to phobol myristate acetate(PMA)/ionomycin. 2. PAM 212, UV-irradiated PAM 212 and heat shocked PAM 212 cells stimulate DETC. PAM 212 cells could perform as stimulator of DETC even in the absence of stress signal. 3. UV irradiated XB2 cells stimulate DETC, but XB2 and heat shocked XB2 cells could not stimulate DETC. 4. DETC do not respond to fibroblast, UV-irradiated fibroblast and heat shocked fibroblast 5. TCR negative variants of DETC are no longer stimulated by PAM 212 cells, suggests that PAM 212 cells mediate their effects through the TCR CONCLUSION: The above results strongly suggest that DETC recognize specific, transformed and stressed keratinocyte-derived antigens and may play a role as an immune surveillant for cellular damage. Therfore, DETC may play critical roles during the induction of immune reaction in the skin.
Animals ; Cell Line* ; Coculture Techniques ; Epidermis ; Fibroblasts ; Hot Temperature ; Humans ; Keratinocytes* ; Ligands ; Mice* ; Myristic Acid ; Receptors, Antigen, T-Cell ; Sensitivity and Specificity ; Shock ; Skin