The tongue is located inside the oral cavity at rest, and the structural interrelations between the tongue and the oral cavity is essentially important for normal tongue function. Macroglossia is clinically diagnosed whenever the tongue does not fit the oral cavity, thus patients complain of noisy breathing, drooling, speech and swallowing dysfunction, malocclusion, and social ridicule. Partial glossectomy for macroglossia volume has been assumed to be difficult. In this study, we attempted to measure the tongue volume with the plaster tongue model in a 7-year-old macroglossia patient. This method is reliable and simple for measuring the tongue volume in macroglossia.
Child ; Deglutition ; Glossectomy* ; Humans ; Macroglossia* ; Malocclusion ; Mouth ; Respiration ; Sialorrhea ; Tongue*

No abstract available.
Down Syndrome*

Adenomyosis is a common disease of middle-aged women and adenomyoma is a variety of adenomyosis that formed localized tumor. Cystic degeneration of an adenomyoma is a rare clinical manifestation. A 30-year-old parous woman suffered from severe dysmenorrhea and menorrhagia for about 5 months, was operated under the impression of endometriosis of the pelvis. Following the operation, cystic degeneration of an adenomyoma was found incidentally. The authors experienced a case of adenomyosis that formed cystic tumor of uterus and presented with a pertinent literatures.
Adenomyoma ; Adenomyosis* ; Adult ; Dysmenorrhea ; Endometriosis ; Female ; Humans ; Menorrhagia ; Pelvis ; Uterus

A Bednar tumor is a variant of dermatofibrosarcoma protuberans(DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP except for the presence of melanin-containing cells scattered within the lesion, so called pigmented DFSP. The majority of Bednar tumors are seen as DFSP present as multinodular protuberant masses in the skin. They can also present as an atrophic depressed scar-like lesions without any nodularity. We report an uncommon clinical presented case of a Bednar tumor on the back in a 22-year-old female. She presented with an asymptomatic, firm, bluish, chestnut sized, depressed and atrophic lesion on the back which had been present for 6 years. A Histopathological examination revealed massive proliferation of spindle-shaped cells arranged in a tight storiform pattern mixed with scattered pigmented cells. On immunohistochemical staining, the tumor cells were positive for vimentin, a -l-antitrypsin, and CD34, but were negative for cytokeratin, neurofilament, and factor XIIIa. The majority of the tumor cells was negative and the pigment cells were positive for the S-100 protein. The patient was treated by a wide local excision of the lesion. There has been no evidence of recurrence after 20 months post-operative follow up.
Dermatofibrosarcoma* ; Factor XIIIa ; Female ; Follow-Up Studies ; Humans ; Keratins ; Recurrence ; S100 Proteins ; Skin ; Vimentin ; Young Adult

Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and acrocephaloyndactyly was named by Apert in 1906. Since then, more than 200 cases have been reported in the world upto 1970. Recently, we have experienced three for typical Apert syndrome and made a brief related literature review
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Skull ; Syndactyly

Malignant eccrine poroma, or eccrine porocarcinoma, is a rare malignant cutaneous appendageal tumor arising from the intraepidermal eccrine sweat duct(acrosyringium), and usually develops ia an eccrine poroma of long-standing. We report a case of malignant eccrine poroma on the left buttock in a 72 year-old female. She was presented with a slightly pruritic, well-defined, reddish, firm, 4.5 x 4.5 x 0.7cm sized, protruding, ulcerated tumor. Histopathological examination revealed well-defined tumor cell nests in the epidermis and dermis. The tumor nests consisted of areas of eccrine poroma cells with benign appearance adjoining areas of anaplastic cells. Duct-like structures were observed within the tumor nests and showed a PAS-positive, diastase-resistant reaction. On immunohistochemical staining, the tumor cells were, positive for EMA, and most of tumor cells were negative but the duct-like structures were positive for CEA. She was treated with surgical excision. During the three year follow up period after excision, there was no recurrence.
Aged ; Buttocks ; Dermis ; Eccrine Porocarcinoma* ; Epidermis ; Female ; Follow-Up Studies ; Humans ; Poroma ; Recurrence ; Sweat ; Ulcer

OBJECTIVE: To evaluate the changing pattern of serum leptin level and the correlation between estradiol level during the clomiphene citrate challenge test(CCCT) or controlled ovarian hyperstimulation(COH) cycle. METHODS: Twenty-seven women who underwent CCCT and fourteen women who underwent controlled ovarian hyperstimulation were recruited to measure the serum leptin level. After correction of serum concentration with body mass index(BMI), changes of leptin level and correlation with serum estradiol level during CCCT and COH cycle were analyzed. RESULTS: Circulating leptin levels were significantly correlated with BMI at each time point in CCCT cycle(P<0.01). In CCCT cycle, leptin/BMI level was significantly increased at midluteal phase compared to that of menstrual cycle day 3 and 10(p<0.05). In women with regular menstruation, leptin/BMI level at midluteal phase was significantly higher than that of menstrual day 3 and 10, but this difference was not seen in women with irregular menstruation. The leptin/BMI level in COH cycle showed increasing tendency throughout ovarian stimulation. But there was no significant correlation between leptin/BMI and estradiol level in CCCT and COH cycle. CONCLUSION: There is a significant correlation between BMI and circulating leptin level. Midluteal leptin level is significantly higher than that of follicular phase in CCCT cycle, and there is an increasing tendency in leptin level after ovarian stimulation in CCCT and COH cycle without statistical significance. These findings suggest that circulating estradiol concentration has no major influence on circulating leptin level.
Clomiphene* ; Estradiol ; Female ; Follicular Phase ; Humans ; Leptin* ; Menstrual Cycle ; Menstruation ; Ovulation Induction

PURPOSE: In order to recognize predictive factors of the recurrent seizure attacks with benign epilepsy of childhood with centrotemporal spike(BECCT), we have studied the factors such as age of seizure onset, sex of the patient, frequency and duration of seizure Prior to therapy, initial antiepileptic drug, which are presumed to influence the recurrence of seizure attack. METHODS: Among the children with BECCT, who visited the department of pediatrics, Chuncheon Sacred Heart hospital, Hallym University from Jan. 1985 to Dec. 1997, 52 patients who were followed up for more than 12 months after disappearance of the seizure, have been studied. The patients were divided into three groups, (group I; seizure duration is less than 3 months, groups II; seizure duration less than 12 months, group III; seizure duration more than 12 months) by duration of seizure recurrence, and compared with difference of seizure recurrence by predicting factor in each group. Data were analysed by using DBSTAT version 1.0 by ANOVA. RESULTS: 1) Age onset of seizure The age onset of seizure in group I is 8.6+/-2.0 years and 7.9 +/-3.5 years in group II, 6.2+/-1.7 years in group III. The younger the seizure standted, the more seizures recurred(p<0.05). Our study revealed no age differences of the seizure remission in 3 groups. 2) Sex : Among the 52 patients, 30 patients were male, 22 patients were female. The ratio of the male to female was 1:1(12/12) in group I, 1:1.5(2/3) in group II, 2.3: 1(16/7) in group III. The recurrence rate was higher in male than in female. But no statistically difference in recurrence rate by the sex was revealed. 3) Seizure duration and seizure frequency prior to therapy : In group I, pretreatment duration was 1.6+/-3.5 months, frequency of the attacks was 2.0+/-1.0. In group II, duration 1.3+/-1.2 months, frequency 3.2+/-0.5. In group III, duration 3.5+/-6.9 months, frequency 3.5+/-1.8. Our study revered statistically significant differences in values of frequencies and durations in each groups(p<0.05). 4) Initial anticonvulsant and seizure recurrence : Seizure recurred in 36% of patients treated by carbamazepine, and 55.6% of valproate, 66.7% of phenobarbital, 37.5% of diphenylhydantion. There was no significant relation between initial anticonvulsant and seizure recurrence in each group. CONCLUSION: Our data support that age onset and seizure frequency were predictive factor fort seizure recurrence. BECCT Is a age dependent genetic disorder with variable penetrance, having longer duration of seizure with earlier onset. Clinical seizures and abnormal EEG trait tend to remit at certain age, regardless of onset age and treatment.
Age of Onset ; Carbamazepine ; Child ; Electroencephalography ; Epilepsy ; Epilepsy, Rolandic* ; Female ; Gangwon-do ; Heart ; Humans ; Male ; Pediatrics ; Penetrance ; Phenobarbital ; Recurrence* ; Seizures* ; Valproic Acid

Kasabach-Merritt syndrome is the association of thrombocytopenia, disorder of coagulation, spontaneous bleeding and enlargement of a hemangima or extensive hemangiomatosis, which can be often life threatening. We experienced a three years-old girl with suddenly enlarging hemangioma of right cheek, complicated by severe thrombocytopenia, consumptive coagulopathy and hemorrhagic cystitis. Generalized petechia and ecchymosis were noted on the overlying skin of hemangioma, trunk and extremities. But the study of MRI and angiography, hypevascular was located within the right masseter muscle extending to temporalis muscle and anterior to parotid gland. So selective embolization with PVA (polyvinyl alcohol) was performed through the distal branch of internal maxillary artery and facial artery. She was medicated with oral corticosterioid for 3 weeks. After embolizationn, blood flow to hemangioma and facial swelling were markedly decreased. Patient was followed up for 7 years and she showed normal contour of face and no bleeding diathesis.
Angiography ; Arteries ; Cheek ; Cystitis ; Disease Susceptibility ; Ecchymosis ; Extremities ; Female ; Hemangioma ; Hemorrhage ; Hemorrhagic Disorders* ; Humans ; Kasabach-Merritt Syndrome* ; Magnetic Resonance Imaging ; Masseter Muscle ; Maxillary Artery ; Parotid Gland ; Skin ; Thrombocytopenia

No abstract available.
Lymphomatoid Granulomatosis*