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

BACKGROUND: Pertechnetate ( Tc) has been widely employed for thyroid imaging. While pertechnetate and radioiodide have usually similar results in identifying thyroid nodules, occasionally differences have been noted. We intended to observe that the thyroid nodules which appeared to be hot on pertechnetate and to compare them with the images by radioiodide. METHODS: 'I scan was performed to thirty-eight cases (mean age: 48.9 +/- 13.2) presenting as hot nodule on Tc scan. Thyroid function test and pathologic diagnosis were obtained in all patients. RESULTS: Of the 38 patients, 24 had euthyroidism, 13 had hyperthyroidism, and 1 had hypothyroidism. Thirty patients had adenomatous goiter, 4 papillary carcinoma, 3 Hashimotos thyroiditis, and 1 had HQrthle cell tumor. 28 of 38 patients showed similar images, but the remaining 10 patients(26.3%) revealed discordant images on Tc and 131I scan. Among the concordant cases, 23 had adenomatous goiter, 3 had papillary carcinoma, and 2 had Hashimotos thyroiditis. Among the discordant cases, 7 had adenomatous goiter, 1 had papillary carcinoma, 1 had Hashimotos thyroiditis, and 1 had HQrthle cell tumor. The incidence of malignancy was 10.7% of concordant cases, and 20% of discordant cases and was revealed statistically insignificant (p>0.05). CONCLUSION: We observed higher incidence of malignancy in patients presenting hot nodules on 99mTc scan than ever reported. Fine needle aspiration should be performed to all patients with hot nodules and the 'I scan would not be recommended for further diagnostic study.
Biopsy, Fine-Needle ; Carcinoma, Papillary ; Diagnosis ; Goiter ; Humans ; Hyperthyroidism ; Hypothyroidism ; Incidence ; Sodium Pertechnetate Tc 99m ; Thyroid Function Tests ; Thyroid Gland* ; Thyroid Nodule ; Thyroiditis

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