No abstract available.
Hepatolenticular Degeneration*

No abstract available.
Spinal Cord Injuries* ; Spinal Cord*

Disseminated intravascular coagulation (DIC) is a pathological syndrome in which activation of coagulation cascade leads to fibrin clot formation, consumption of platelets and coagulation factors, and secondary fibrinolysis. We report a case of severe postoperative hemorrhagic diathesis due to DIC. A 59-year-old man was scheduled for reduction of tibia fracture and anatrophic nephrolithotomy of staghorn calculi. On the fifth postoperative day, second operation was performed for nephrectomy due to perirenal hematoma. Two days later, third operation was performed for hemostasis because of the continuous bleeding. Coagulation tests showed positive DIC profiles of thrombocytopenia, hypofibrinogenemia, increased fibrin degradation products, and prolonged prothrombin time and thrombin time. The patient recovered uneventfully and discharged on the 59th postoperative day.
Blood Coagulation Factors ; Calculi ; Dacarbazine ; Disseminated Intravascular Coagulation* ; Fibrin ; Fibrin Fibrinogen Degradation Products ; Fibrinolysis ; Hematoma ; Hemorrhage* ; Hemorrhagic Disorders ; Hemostasis ; Humans ; Middle Aged ; Nephrectomy ; Postoperative Complications ; Prothrombin Time ; Thrombin Time ; Thrombocytopenia ; Tibia

No abstract available.

Short umbilical cord syndrome, also known as the limb-body wall malformation complex and the body stalk anomaly, is a poorly defined sporadic group of congenital anomaly charaterized by a complex set of disruptive abnormalities having in common the failured closure of the ventral body wall. This disorder is charaterized by a short or absent umbilical cord and disruption of the lateral body wall, spine, limbs, face, and cranium, isolated or in combination. Recently, we present a case of short umbilical cord syndrome which found in a term baby, so we report a case of short umbilical cord syndrome with brief review of literature.
Extremities ; Skull ; Spine ; Umbilical Cord*

A patient with extraskeletal cervical epidural Ewing's sarcoma who presented with cervical radiculopathy is reported. A 58-year-old woman presented with right posterolateral neck pain and upper extremity radiculopathy. The computed tomography myelography showed epidural and paravertebral masses on right side, with widening of the ipsilateral neural foramina at the C2-C3 level. On magnetic resonance images, the mass was isointense to soft tissue on T1-weighted images, hyperintense on T2-weighted images, and showed diffuse enhancement on GdTA enhanced T1-weighted images. The staged, combined posterior and anterior approach were done and the tumor mass was subtotally removed. During postoperative adjuvant chemotherapy with Cytoxane, Adriamycin, Vincristine, Prednisolone regimen, the patient died of severe compression of upper cervical cord by recurrent, extended tumor mass in the spinal canal.
Chemotherapy, Adjuvant ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Female ; Humans ; Middle Aged ; Myelography ; Neck Pain ; Prednisolone ; Radiculopathy ; Sarcoma, Ewing* ; Spinal Canal ; Upper Extremity ; Vincristine

Phakomatosis pigmentova.scularis is a disease complex with dermal melanocyto sis such as nevus of Ota, nevus of Ito, or Mongolian spots and extensive hemangiomatous change such as nevus flammeus, which are associated with systemic organ involvement. We report a 23-year-old female, who showed bilateral nevus of Ota and nevus of Ito with widespread nevus flammeus and Mongolian spots since birth. She was also associated with megalocornea due to congenitol glaucoma and cataract.
Cataract ; Female ; Glaucoma ; Humans ; Mongolian Spot ; Neurocutaneous Syndromes* ; Nevus ; Nevus of Ota ; Parturition ; Port-Wine Stain ; Young Adult

Hyperkeratosis of the nipple and areola is a rare condition. We report a case of hyperkeratosis of the nipple and areola occurrinp in man with no underlying endocrirop thy or synthetic estrogenic drug therapy. The patient was not associated with epidermal nevus orichthyosis and so fitted into the nevoid form of hyperkeratosis of the nipple and areola. The clinical appearance and histological findings of the lesion were the same as those reported before. He showed a slight improvement in the color and hyperkeratcsis of the nipple and areola with 6 months use of a topical keratolytic agent and corticosteroid crearn.
Drug Therapy ; Estrogens ; Humans ; Nevus ; Nipples*

We found a mistake in our recently published article.

We are reporting a case of molluscum contagiosum in a 3-year-old boy who showed a good response to diphenylcyclopropenone (DPCP) immunotherapy. The lesions were multiple, 2 to 7mm in size, centrally umbilicated, normal skin-colored papules of 7 months' duration which were distributed on the penoscrotal area, extremities and trunk. In spite of several treatments using extraction and curettage, new lesions developed continuously. The patient was sensitized with 0.1ml of 1% DPCP solution in acetone on his right shoulder and there-after challenged with solution of varying concentrations (0.05 to 0.1%) on his left shoulder once or twice a week to maintain a mild eczema. Clinical improvement was noted 1 week after sensitization and almost all of the lesions cleared after 8 week's treatment.
Acetone ; Child, Preschool ; Curettage ; Eczema ; Extremities ; Humans ; Immunotherapy* ; Male ; Molluscum Contagiosum* ; Shoulder