PURPOSE: Differential diagnosis of sexual precocity is important. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS: We reviewed the etiology and age incidence of precocious puberty in 14 boys and 89 girls examined between 1994. 11. 1-2002. 7. 31 at Samsung Seoul Hospital. All underwent standard anthropometric measures. They were assigned to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: In 89 girls, 27 had idiopathic precocious puberty, 3 had precocious puberty with organic brain lesion, 12 had gonadotropin-independent precocious puberty, 44 had premature thelarche, 2 had premature menarche, 1 had premature adrenarche. The majority of idiopathic precocious puberty girls(78%) were aged between 7-7.9 years. In 14 boys, 2 had idiopathic precocious puberty, 3 had precocious puberty with organic brain lesion, 6 had gonadotropin-independent precocious puberty, 3 had gynecomastia. CONCLUSION: Sexual precocity occurs more frequently in girls than boys. Premature thelarche is the most common form of sexual precosity. In boys, majority of central precocious puberty had organic brain lesion, so brain imaging study should be performed. In girls, majority of idiopathic precocious puberty were aged between 7-7.9 years. Reexamination of the age limit for defining when puberty should be considered precocious in Korean girls is necessary.
Adolescent ; Adrenarche ; Brain ; Diagnosis, Differential ; Female ; Gynecomastia ; Humans ; Incidence* ; Male ; Menarche ; Neuroimaging ; Puberty ; Puberty, Precocious* ; Seoul ; Ultrasonography

PURPOSE: To understand natural course of transient hyperthyrotropinemia, so that the unnecessary treatment could be avoided. METHODS: Total 182 healthy newborns whose TSH levels in newborn screening test had been higher than 20 microIU/mL were recalled. Their serum levels of TSH, T4, free T4 were checked. RESULTS: Among 182 newborns, 10 newborns had congenital hypothyroidism. 68.1 % of healthy newborns who had high TSH levels were normalized within 2 months and all of the rests were normalized within 5 months. CONCLUSION: Transient hyperthyrotropinemia in newborns improves spontaneously. The unnecessary treatment of transient hyperthyrotropinemia should be avoided.
Congenital Hypothyroidism ; Follow-Up Studies* ; Humans ; Infant, Newborn ; Mass Screening ; Neonatal Screening*

Many dialysis modalities such as peritoneal dialysis (PD), hemodialysis (HD) and continuous hemofiltration or hemodialysis (CRRT) are available for the management of pediatric patients with acute renal failure (ARF). PD is a relatively simple, inexpensive modality and can be used in hemodynamically unstable patients. But, it may not be the optimal therapy for patients with severe volume overload or life threatening hyperkalemia. HD is the preferred modality for the treatment of severe volume overload, severe hyperkalemia, but it needs vascular access. Improvements in the HD equipment have allowed HD to be performend in small children. Recents technological improvements in CRRT therapies have enabled pediatric patients who are less stable to be treated. CRRT is becoming the preferred method of acute therapy in pediatric intensive care units. A sound knowledge of the underlying principles of dialysis and awareness of recent technological advancements in differnet dialysis modalities will hopefully result in improved management of children with ARF.
Acute Kidney Injury* ; Child* ; Dialysis ; Hemofiltration ; Humans ; Hyperkalemia ; Intensive Care Units, Pediatric ; Peritoneal Dialysis ; Renal Dialysis ; Renal Replacement Therapy*

An ossifying fibromyxoid tumor of soft parts is a rare, recently described, fibro-osseous neoplasm of uncertain histogenesis. It occurs most frequently within the subcutis or skeletal muscle of the extremities. Its biologic behavior is generally regarded as benign with at worst a locally aggressive clinical course. But, atypical and malignant variants have been recently reported. Herein we report a case of a benign ossifying fibromyxoid tumor which occurred in the left upper back of 41-year-old man. The tumor is composed of uniformly round or polygonal cells arranged in cords or nests which are separated by myxoid and hyalinzed fibrous matrix and associated with irregular bony trabeculae. The tumor cells are strong positive for vimentin. Ultrastructural findings and a review of literatures are added.
Adult ; Extremities ; Humans ; Muscle, Skeletal ; Vimentin

An ossifying fibromyxoid tumor of soft parts is a rare, recently described, fibro-osseous neoplasm of uncertain histogenesis. It occurs most frequently within the subcutis or skeletal muscle of the extremities. Its biologic behavior is generally regarded as benign with at worst a locally aggressive clinical course. But, atypical and malignant variants have been recently reported. Herein we report a case of a benign ossifying fibromyxoid tumor which occurred in the left upper back of 41-year-old man. The tumor is composed of uniformly round or polygonal cells arranged in cords or nests which are separated by myxoid and hyalinzed fibrous matrix and associated with irregular bony trabeculae. The tumor cells are strong positive for vimentin. Ultrastructural findings and a review of literatures are added.
Adult ; Extremities ; Humans ; Muscle, Skeletal ; Vimentin

PURPOSE: Polyglutamine diseases are a group of diseases caused by the expansion of a polyglutamine tract in the protein. The present study was performed to verify if polyglutamine disease transgenic Drosophila models show similar dysfunctions as are seen in human patients. METHODS: Polyglutamine disease transgenic Drosophila were tested for their climbing ability. And using genetic methods, the effects of anti-apoptotic gene bcl-2 and chemical chaperones on neurodegeneration were observed. Also, spinocerebellar ataxia 2 (SCA2) transgenic Drosophila lines were generated for future studies. RESULTS: Expanded forms of spinocerebellar ataxia 3 (SCA3) transgenic protein causes characteristic locomotor dysfunction when expressed in the nervous system of Drosophila but the anti-apoptotic gene bcl-2 shows no evidence of ameliorating the deleterious effect of the expanded protein. However, Glycerol, a chemical chaperone, seemed to reduce the toxicity, at least in the eyes of the transgenic flies. The level SCA2 expression is too weak in the transgenic SCA2 Drosophila for evaluation. CONCLUSION: SCA3 transgenic Drosophila show ataxic behavior as observed in human patients. Chemical chaperones such as glycerol may prove beneficial in this class of genetic disease, which has no current method of cure.
Diptera ; Drosophila* ; Glycerol ; Humans ; Machado-Joseph Disease ; Nervous System ; Spinocerebellar Ataxias

Lacrimal sac diverticulum is a rare condition, and its various symptoms complicate differential diagnosis. We present cases of a peculiar type of lacrimal diverticulum. A 5-year-old girl and a 50-year-old woman presented with a protruding mass inferior to the medial canthus. Each lacrimal system was patent to irrigation. The masses compressed and distorted the lacrimal passage and had no apparent connection with the lacrimal sac in dacryocystography or computed tomography. Surgical exploration and complete excision of the masses were completed. Each patient had an inverted Y- and an inverted V-shaped multilobular cystic mass that was pathologically confirmed as a lacrimal sac diverticulum. Lacrimal sac diverticula may rarely take the form of a multilobular cyst and can present as a lower lid mass. We speculate that an abnormality in lacrimal embryogenesis resulted in multiple blind pouches, a peculiar type of lacrimal sac diverticulum.
Child, Preschool ; Diagnosis, Differential ; Diverticulum/radiography/*surgery ; Eyelids/radiography/surgery ; Female ; Humans ; Lacrimal Apparatus Diseases/radiography/*surgery ; Middle Aged ; Tomography, X-Ray Computed

Granulomatous mastitis is a rare inflammatory breast lesion, usually presented with rapidly enlarging palpable mass in young parous women and can simulate carcinoma. Unnecessary surgical procedure can be avoided if the nature of the lesion were defined by fine needle aspiratio(FNA) cytology. We experienced a case of bilateral granulomatous mastitis diagnosed by FNA cytology. The patients was a 31-year-old woman with one month history of a rapidly enlarging mass in the left breast. considered clinically to be malignant. After 4 years. she presented with a palpable mass in the right breast. The FNA cytology smears contained numerous aggregates of epithelioid histiocytes admixed with Langhans' and foreign body giant cells, lymphoytes, neutrophils and apoptotic debris leading to a diagnosis of granulomatous mastitis. The subsequent lumpectomy of the left breast confirmed the diagnosis of granulomatous mastitis. The FNA cytology smears from right breast showed identical cytologic findings.
Adult ; Biopsy, Fine-Needle* ; Breast ; Diagnosis ; Female ; Giant Cells, Foreign-Body ; Granulomatous Mastitis* ; Histiocytes ; Humans ; Mastectomy, Segmental ; Needles ; Neutrophils

PURPOSE: The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). METHODS: Forty-one patients who had been treated with GH for more than two years (24 boys and 17 girls, mean age 7.3+/-3.3 years during treatment initiation) were enrolled for this study. RESULTS: After 2 years of GH therapy, the height and weight standard deviation scores (SDS) increased significantly (- 1.19+/-1.37 vs. - 0.02+/-1.45, and 1.02+/-2.42 vs. 1.63+/-2.22, P<0.002); however the percentage body fat decreased (44.6+/-9.9% vs. 38.1+/-10.5%, P<0.001). Further, no change was observed in the thyroid and serum glucose levels, but the total cholesterol level decreased. GH therapy did not impact glucose control in the patients with diabetes. The most common adverse effects of GH therapy were the progression of scoliosis and adenoid hypertrophy. CONCLUSION: GH therapy improved the height SDS and body composition in patients with PWS. However, GH should be used with caution in patients with scoliosis and adenoid hypertrophy.
Adenoids ; Adipose Tissue ; Body Composition ; Child ; Cholesterol ; Glucose ; Growth Hormone ; Humans ; Hypertrophy ; Prader-Willi Syndrome ; Scoliosis ; Thyroid Gland

Verruca vulgaris is commonly seen on skin, but extremely rare in other areas, especially in larynx, where it could be not uncommonly misdiagnosed as verrucous carcinoma. In the recent studies, we noted that verruca vulgaris of the larynx is related to human papilloma virus type 6, 11, which is different from human papilloma virus type 2, 4, which is found on skin. Verruca vulgaris of the larynx occurs in old age with the average of 56 years, and it looks white in color on laryngoscopic examination, which is limited to the true vocal cords. It recurs infrequently. Pathologically, it is heavily keratinized and it contains prominent keratohyaline granules and exhibits koilocytosis. We experienced a case of verruca vulgaris of the larynx in a patient with hoarseness lasted for 3 months. So we report this case with a review of literature.
Carcinoma, Verrucous ; Hoarseness ; Humans ; Larynx* ; Papilloma ; Skin ; Vocal Cords ; Warts*