Wiskott-Aldrich syndrome is an X-linked combined immunodeficiency disorder characterized by severely decreased number of platelets which are small in size, eczema resembling atopic dermatitis and recurrent infection. The serum of the patient contains elevated concentrations of IgA and IgE, whereas the IgG level is usually normal and IgM level is decreased. The patients also shows skin test anergy and progressive T-lymphocytopenia. Bleedings and recurrent infections are the main causes of death and the patients usually die before age 10. Bone marrow transplantation is accepted to be the only radical therapy. We experienced a case compatible with Wiskott-Aldrich syndrome in a 5 year old male child who accompanied above clinical manifestations and laboratory findings.
Bone Marrow Transplantation ; Cause of Death ; Child ; Child, Preschool ; Dermatitis, Atopic ; Eczema ; Humans ; Immunoglobulin A ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulin M ; Male ; Skin Tests ; Thrombocytopenia ; Wiskott-Aldrich Syndrome* ; X-Linked Combined Immunodeficiency Diseases

No abstract available.
Byssinosis*

Purpose: Radiation-induced oral mucositis limits delivery of high-dose radiation to targeted cancers. Therefore, it is necessary to develop a treatment strategy to alleviate radiation-induced oral mucositis during radiation therapy. We previously reported that inhibiting reactive oxygen species (ROS) generation suppresses autophagy. Irradiation induces autophagy, suggesting that antioxidant treatment may be used to inhibit radiation-induced oral mucositis. Materials and Methods: We determined whether treatment with N-acetyl cysteine (NAC) could attenuate radiation-induced buccal mucosa damage in vitro and in vivo. The protective effects of NAC against oral mucositis were confirmed by transmission electron microscopy and immunocytochemistry. mRNA and protein levels of DNA damage and autophagy-related genes were measured by quantitative real-time polymerase chain reaction and western blot analysis, respectively. Results: Rats manifesting radiation-induced oral mucositis showed decreased oral intake, loss of body weight, and low survival rate. NAC intake slightly increased oral intake, body weight, and the survival rate without statistical significance. However, histopathologic characteristics were markedly restored in NAC-treated irradiated rats. LC3B staining of rat buccal mucosa revealed that NAC treatment significantly decreased the number of radiation-induced autophagic cells. Further, NAC inhibited radiation-induced ROS generation and autophagy signaling. In vitro, NAC treatment significantly reduced the expression of NRF2, LC3B, p62, and Beclin-1 in keratinocytes compared with that after radiation treatment. Conclusion NAC treatment significantly inhibited radiation-induced autophagy in keratinocytes and rat buccal mucosa and may be a potentially safe and effective option for the prevention of radiation-induced buccal mucosa damage.

No abstract available.
Brain* ; Meningitis* ; Tuberculoma*

Bladder urothelial carcinoma typically occurs in individuals in their sixties or seventies: it rarely occurs in persons <20 years old. Moreover, in young patients, bladder urothelial carcinoma lesions are reported to be solitary and nonmuscle invasive, with low malignant potential. However, 13% of lesions in this age group are reported to be noninvasive high-grade papillary urothelial carcinoma but these are extremely rare in children <15 years. There is no specific consensus regarding treatment of bladder urothelial carcinoma in children and adolescent. In particular, method has been suggested for follow-up of high-grade bladder urothelial carcinoma. Therefore we report our experience of one case the, discuss surveillance methods, and provide a brief review of the literature.
Adolescent ; Child* ; Consensus ; Follow-Up Studies ; Humans ; Methods ; Pediatrics ; Urinary Bladder*

Between March 1994 and March 1995, 10 patients with thoracolumbar burst fractures underwent a one-stage operation consisting of anterior decompression, reduction, bony fusion with iliac bone and stabilization with Kaneda device. The mean follow-up was 6.4 Months. Most patients with incomplete neurologic lesions showed postoperative improvement and were upgraded one or two steps in the Frankel scale. No patient showed neurological deterioration after surgery. Loss of reduction was 5.5 degree during follow-up period. Anterior decompression and strut fusion was used to effectively recover the neurological deficit and reduce the pain in a thoraco-lumbar burst fracture.
Decompression ; Follow-Up Studies ; Humans

We have experienced 19 patients of the cerebral arteriovenous malformation with subarachnoid hemorrhage, who were admitted to the Presbyterian Hospital, Daegu from January 1966 to July 1968, and to the Catholic Medical Center, Seoul from August 1968 to August 1971. All of the patients, who have the cerebral arteriovenous malformation, were proved by cerebral angiography. Since the site of lesions were considered to have close relation to the neurologic deficit and the result of surgery, various analysis of the clinical manifestations and the cerebral angiographic findings were attempted. Of the 19 patients, surgery was performed on 12 patients and its results were analyzed correlating to various types of surgical procedure. Following are the results. 1. From January 1966 to August 1971, there were 100 cases of cerebral vascular anomalies which were proved by cerebral angiography. Among the 100 cases, there were 71 intracranial aneurysms, 19 cerebral arteriovenous malformations, one cavernous angioma, 2 telangiectasis, 5 cerebral rete mirabile, and s Sturge Weber-Dimitris disease. The ratio of arteriovenous malformatons to aneurysms was 1: 3.7. 2. Age distribution of the bleeding arteriovenous malformations was ranged from 8 to 54, and 42 per cent of them were in the third decade. 73 percent of the group had bleeding from the cerebral arteriovenous malformation before the age of 40. The ratio of male to female was 1.9:1. 3. The parietal region was most commonly involved by the malformations. There were 8 parietal lesion, 4 temoroparietal lesions, one temporal lesion, one occipital lesion, and one tela chorioidea lesion. Simultaneous involvement of the both hemispheres occurred in one case. In lesions involving the cerebral hemisphere, there was a predominance of the left side. 4. In the past history, convulsive seizure was recorded in two cases and recurrent hemorrhages were reported in two. One patient was recorded to have suffered fourth hemorrhage and the other have sufferd third hemorrhge. 5. Meningeal irritation signs were most frequently observed in bleeding arteriovenous malformations. The incidence of weakness of extremities was higher in lesions involving the parietal area. Unconsciousness was occurred in 6 patients at the onset of symptom, of which transient loss of consciousness was seen in 3 patients. Transient hypertension at the onset was observed in two patients. 6. The feeding arteries of the malformations were most frequently seen from the middle cerebral artery and the draining veins from the malformations were into the superior sagittal sinus and internal cerebral vein in many cases. It was evident that the carotid artery of the sites of arteriovenous malformations was markedly dilated in 5 patients. 7. Ligation of the vessels feeding the arteriovenous malformations was carried out in 8 patients. One patient had neurologic deficit after the operation. Evacuation of intracerebral hematoma, resection of the anomaly or carotid ligation were carried out in 4 patients. Of the 12 patients who underwent various types of surgery, there was one death after evacuation of a large intracereral hematoma. 8. In our opinion, the ligation of feeding artery a choice of procedure to reduce the incidence of rebleeding, if the resection of the lesion was considered to have operative risk by the location of malformations.
Age Distribution ; Aneurysm ; Arteries ; Arteriovenous Malformations ; Carotid Arteries ; Cerebral Angiography ; Cerebral Veins ; Cerebrum ; Daegu ; Extremities ; Female ; Hemangioma, Cavernous ; Hematoma ; Hemorrhage ; Humans ; Hypertension ; Incidence ; Intracranial Aneurysm ; Intracranial Arteriovenous Malformations ; Ligation ; Male ; Middle Cerebral Artery ; Neurologic Manifestations ; Protestantism ; Rabeprazole ; Seizures ; Seoul ; Subarachnoid Hemorrhage ; Superior Sagittal Sinus ; Telangiectasis ; Unconsciousness ; Veins

A case of T8-9 disc herniation presenting with signs of spinal cord compression is reported. The patient was subjectd to a transpedicle approach through a midline incision and was cured completely. The other surgical techniques and the diagnostic value of the spine CT are also discussed.
Humans ; Spinal Cord Compression ; Spine

Two cases with intraventricular arachnoid cysts are reported and a brief review of the relevant literature is presented. Arachnoid cysts are benign developmental cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane. They are usually extracerbral or extraventricular. Intraventricular arachnoid cysts are rare: except the two cases reported here, only five cases have been described. The following characteristics were noted in these patients: all were young; headache was the initial symptom; the cyst was in the body of a lateral ventricle.
Arachnoid Cysts ; Arachnoid* ; Axis, Cervical Vertebra ; Headache ; Humans ; Lateral Ventricles ; Membranes

A case of traumatic intracranial occlusion of the right internal carotid artery is reported, and the mechanism and management of occlusion is reviewed to make realize the importance of vascular lesions in patients with head injury. Considering that CT became the principal diagnostic method in head injured patients, it is important to realize the possibility of vascular injury in patients with head injury who have unexplained neurological deficits and to pursue the evaluation of the patients with an angiography.
Angiography ; Carotid Artery, Internal* ; Craniocerebral Trauma ; Head ; Head Injuries, Closed* ; Humans ; Thrombosis ; Vascular System Injuries