We report a patient with a cutis laxa-like, generalized PXE without systemic involvement. A 28-year-old woman had loose, pendulous skin of the neck, axillae, thighs, trunk and body folds which resulted in a prematurely aged appearance. She had no family history of related diseases. Histological examination showed considerable accumulations of swollen and irregularly clumped fibers in the middle and lower dermis and von Kossa s stain revealed calcium deposits along the altered elastic fibers.
Adult ; Axilla ; Calcium ; Dermis ; Elastic Tissue ; Female ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Skin ; Thigh

We report a patient with a cutis laxa-like, generalized PXE without systemic involvement. A 28-year-old woman had loose, pendulous skin of the neck, axillae, thighs, trunk and body folds which resulted in a prematurely aged appearance. She had no family history of related diseases. Histological examination showed considerable accumulations of swollen and irregularly clumped fibers in the middle and lower dermis and von Kossa s stain revealed calcium deposits along the altered elastic fibers.
Adult ; Axilla ; Calcium ; Dermis ; Elastic Tissue ; Female ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Skin ; Thigh

No abstract available.
Humans ; Moral Development* ; Students, Medical*

Intraspinal tumors in children are of especially interest to orthopaedic surgeons for two reasons; First, musculoskeletal abnormalities, are often treated for long periods before the correct diagnosls is suspected; Second, important orthopaedic problems are encountered in almost everychild treated for intraspinal tumor, not only during the immediate postoperative management but all through subsequent growth. We report a case presentation of the intraspinai tumor, dermoid cyst, successfully treated with operation.
Child ; Dermoid Cyst ; Humans ; Musculoskeletal Abnormalities ; Surgeons

No abstract available.
Female ; Humans ; Middle Aged* ; Myofibroblasts

No abstract available.
Anal Canal* ; Paget Disease, Extramammary* ; Radiotherapy* ; Vulva*

No abstract available.
Stevens-Johnson Syndrome*

Neurocristopathy is characterized as having a common origin in aberrant neural crest development. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by marked depression of respiratory drive during sleep and normal ventilation while awake because of no response to both hypercapnea and hypoxia. The girl was full-term, weighing 3020 grams. The girl had poor respiratory effort at birth, but improved with oxygen supply and stimulation. abdominal distention and calcification were noted. During laparotomy transitional zone was found at distal jejunum; a jejunostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an apnea work-up, including brain sonography, echocardiogram, were normal. The girl died of sepsis at 37 days of age. para-aortic ganglioneuroblastoma was found on autopsy. We experienced a newborn with congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma representative of neurocristopathy.
Anoxia ; Apnea ; Autopsy ; Brain ; Depression ; Female ; Ganglioneuroblastoma* ; Hirschsprung Disease* ; Humans ; Hypoventilation* ; Infant, Newborn* ; Jejunostomy ; Jejunum ; Laparotomy ; Neural Crest ; Oxygen ; Parturition ; Sepsis ; Ventilation

Hemangiopericytoma was first described by Stout & Murray in 1942 and was an uncommon soft tissue tumor thought to be derived from vascular pericytes. Approximately 10-15% of the cases occur in children have a propensity to develop in the region of head, neck and lower extremities. We experienced a case of congenital hemangiopericytoma of small bowel in a male neonate who showed vomiting and abdorninal distension. We reported a case of congenital hemangiopericytoma of small bowel with brief review of related literature.
Child ; Head ; Hemangiopericytoma* ; Humans ; Infant, Newborn ; Intestinal Obstruction* ; Intestine, Small ; Lower Extremity ; Male ; Neck ; Pericytes ; Vomiting

Ventriculoperitoneal shunt (VP shunt) for hydrocephalus is thought to inhibit the closure of processus vaginalis by increasing intraabdominal pressure, thus it promotes the inguinal hernia. We reviewed the incidence and characteristics of the inguinal hernia in VP shunted patients, and tried to estimate the patency rate of processus vaginalis in early childhood. A reprospective review of patients undergone insertion of VP shunt between January 1980 and May 1998 at Seoul National University Children Hospital was done. 262 patients were included in this study. Among them, 28 patients developed inguinal hernia (10.7%). Six patients developed inguinal hernia before the insertion of VP shunt. According to the age of VP shunt, the inguinal hernia developed in 16.2% (12/74) of patients who had undergone VP shunt before 6 months old, 12.4% (11/89) between 6 months and 2 years old and 5.1% (5/99) after 2 years old. Among 22 patients excluding 6 patients who developed hernia before VP shunt, the incidence of inguinal hernia after VP shunt was 8.6% (22/256) with male predominance (M:F=18:4). 8 patients developed inguinal hernia bilaterally (36.4%). It is suggested that at least 14% of processus vaginalis is patent until 2 years old.
Child ; Child, Preschool ; Hernia ; Hernia, Inguinal* ; Humans ; Hydrocephalus ; Incidence ; Infant ; Male ; Seoul ; Ventriculoperitoneal Shunt*