No abstract available.
Bronchiectasis*

Spontaneous bleeding due to vascular involvement of neurofibromatosis type 1 is rare but potentially fatal. Herein, we report a case of a lethal spontaneous hemomediastinum in a patient with neurofibromatosis type 1. The bleeding was caused by rupture of an aberrant bronchial artery arising from the ipsilateral subclavian artery, which was successfully treated using transarterial embolization with coils and N-butyl-2-cyanoacrylate.

Sjogren syndrome is a chronic, slowly progressive, autoimmune disease in which the exocrine glands are damaged by lymphocytic infiltration, resulting in xerostomia and xerophthalmia. Sjogren syndrome may occur in 2 forms: primary Sjogren syndrome, when the clinical manifestations of the syndrome are seen alone, and secondary Sjogren syndrome, when associated with another autoimmune disease, such as rheumatoid arthritis, systemic lupus erythromatosus, or scleroderma. Approximately one third of patients present with extraglandular manifestations: arthritis, Raynaud phenomenon, lymphadenopathy, lung involvement, vasculitis and peripheral nervous system involvement. About 10-50% of patients with Sjogren syndrome had evidence of thyroid disease, mainly hypothyroidism. Several inflammatory thyroid diseases are also considered to be autoimmune in origin. In this respect, the histologic picture of primary Sjogren syndrome exocrine glands and autoimmune thyroid glands show great similarities. Here, we report a new case of Sjogren syndrome accompanying with hyperthyroidism which affected a 10-year-old girl.
Arthritis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Child ; Exocrine Glands ; Female ; Humans ; Hyperthyroidism* ; Hypothyroidism ; Lung ; Lymphatic Diseases ; Peripheral Nervous System ; Raynaud Disease ; Sjogren's Syndrome* ; Thyroid Diseases ; Thyroid Gland ; Vasculitis ; Xerophthalmia ; Xerostomia

1.050 patients who visited orthodontic dental department from 1983 to 1994, were surveyed on the yearly tendency of orthodontic patient distribution and state by means of Angle's classification. The results were as follows : 1. There was increased visiting rate of patient per year and higher visiting rate in female than in male. 2. 8-15 age group was 61.4% in total visiting patients and over 20 age group was 18.5%, under 7 age group was 8.1%. 3. Class I malocclusion was 42.2%, class II div 1 was 22.5%, class II-2 was 3.9%, class III was 29.1% and cleft lip & palate was 2.0% in total visiting patient. 4. As showed the living distribution, Namgu and Susunggu's patients were 43.7% of the total patients. 5. There was increased tendency for the number of the patient to be received orthognathic surgery.
Cleft Lip ; Dentistry* ; Female ; Humans ; Male ; Malocclusion* ; Orthodontics* ; Orthognathic Surgery ; Palate

Functional recovery after mandibular condyle fracture was a contradictory result of many authors. The treatment goal of condyle fracture has been directed primarily toward restoration of functional movement of the mandible. We selected some patients who requested functional therapy in many cases of condylar fracture, depend on pattern of fracture, patient's demand, occlusion, age. Without intermaxillary fixation, we induced the patients to rapid healing of temporomandibular function and normal mandibular protrusive, lateral movement as a result of early functional therapy by activator. So, the authors report the cases with review of concerned literature.
Humans ; Mandible ; Mandibular Condyle

BACKGROUND: The Hetzer procedure for the correction of Ebstein's anomaly has the advantages of technical feasibility and incorporation of the atrialized right ventricle (RV) into the functional RV. MATERIAL AND METHOD: We preformed a retrospective review of 11 patients with Ebstein's anomaly and they had undergone a Hetzer procedure between March 2002 and December 2006. RESULT: The median age at operation was 19.8 years (range: 6 months~56 years). There were 4 males and 7 females. All patients showed severe tricuspid regurgitation (TR) preoperatively, and arterial desaturation (<95%) was present in 3 patients. The original Hetzer technique was employed in 6 patients with the Carpentier type A anomaly. In the remaining 5 patients with the Carpentier type B or C anomalies, valve competence was restored at the level of the displaced tricuspid valve mechanism. Adjunct bidirectional cavo-pulmonary shunt, or one and a half ventricle repair strategy was employed for all the patients. The median follow-up was 8.6 months (range: 0.8~51.9 months). There was no early or late death, and the immediate postoperative TR was trivial to mild in 8 patients. The median cardio-thoracic ratios on chest X-ray at the preoperative period and at postoperative 0, 1 and 6 months were 65%, 62%, 55% and 55%, respectively. CONCLUSION: The original or modified Hetzer procedure for Ebstein's anomaly shows excellent intermediate-term outcomes.
Ebstein Anomaly* ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Ventricles ; Humans ; Male ; Mental Competency ; Preoperative Period ; Retrospective Studies ; Thorax ; Tricuspid Valve ; Tricuspid Valve Insufficiency

Subclavian steal syndrome caused by an acute thrombus is very rare. We present a case of cerebellar infarction with proximal subclavian artery thrombosis. A 56-year-old woman was admitted for sudden vertigo. One day prior to admission, she received a shoulder massage comprised of chiropractic manipulation. On examination, her left hand was pale and radial pulses were absent. Blood pressure was weak in the left arm. Downbeat nystagmus and a right falling tendency were observed. Brain MRI showed multiple acute infarctions in the left cerebellum. The findings of Doppler ultrasonography in the left vertebral artery were compatible with a partial subclavian artery steal phenomenon. Digital subtraction angiography demonstrated a large thrombus in the left subclavian artery. After heparin infusion, thrombus size markedly decreased. Cerebellar infarction caused by acute subclavian thrombosis following minor trauma is rare, but the thrombus can be successfully resolved with anticoagulation.
Brain Infarction/*diagnosis/etiology/pathology ; Cerebellar Diseases/diagnosis/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Thrombosis/*complications

No abstract available.
Cranial Nerve Diseases ; Uveitis*

PURPOSE: Common conditions, such as dehydration or respiratory infection can aggravate hypoxia and are associated with interstage mortality in infants who have undergone palliative surgery for congenital heart diseases. This study was done to evaluate the efficacy of a home monitoring program (HMP) in decreasing infant mortality. METHODS: Since its inception in May 2010, all infants who have undergone palliative surgery have been enrolled in HMP. This study was a prospective observational study and infant outcomes during HMP were compared with those of previous comparison groups. Parents were trained to measure oxygen saturation, body weight and feeding volume and to contact the hospital through the hotline for emergency situations. Telephone counseling was conducted by clinical nurse specialists every week post discharge. RESULTS: Forty-one infants were enrolled in HMP. Nine hundred telephone counseling sessions were conducted. Seventy-three infants required telephone triage with the most common conditions being gastrointestinal (50.7%) and respiratory symptoms (32.9%). With HMP intervention, interstage mortality decreased from 18.6% (8/43) to 9.8% (4/41) (chi2=1.15, p=.283). CONCLUSION: Results indicate that active measures and treatments using the HMP decrease mortality rates, however further investigation is required to identify various factors that contribute to hemodynamic complications during the interstage period.
Body Temperature ; Body Weight ; Caregivers/education/psychology ; Counseling ; Female ; Heart Defects, Congenital/mortality/prevention & control/*surgery ; Heart Rate ; Hospitalization ; Hotlines ; Humans ; Infant ; Infant, Newborn ; Male ; Monitoring, Physiologic/*methods ; Oxygen Consumption ; Palliative Care ; *Program Evaluation ; Prospective Studies ; Time Factors

OBJECTIVES: Bisphenol A Di Glycidyl Ether (BADGE) is the major component in commercial liquid epoxy resins, which are manufactured by co-reacting bisphenol A with epichlorohydrin. The authors investigated the acute toxicity of BADGE. METHODS: BADGE was administered by a gavage to 8 week old SPF Sprague Dawley rats in a single dose of 0 (negative control), 0.37 (Diethylstilbesterol, DES), 1000, 2000, 4000, and 8000 mg/kg/day of BADGE. Each treatment group contained 7 rats. The general status and weight of the rats were observed for 14 days. The rats were anesthetized by ether at 14 days, and the changes in morphology, organ weight, sperm count and motility, and hormone level were measured. RESULTS: All the rats treated with BADGE had diarrhea on the 1st day. The rats administered BADGE at 1000, 2000, and 4000 mg/kg/day showed a soiled perineal region and soft stools with diarrhea until the 3rd day. The 8000 mg/kg/day BADGE rats had diarrhea for two days followed by emaciation, soiled fur, a soiled perineal region, staining around the mouth and were moribund for three to eight days. No weight gain was observed after the 1st day in the 2000, 4000, and 8000 mg/kg/day BADGE rats and after the 7th day in all the treatment groups compared with the control groups. Some treatment groups were observed to have a decrease in the weight of the heart (BADGE 1000, 2000 and 4000 mg/kg/day), liver (BADGE 1000, 2000, 4000 and 8000 mg/kg/day) and prostate (BADGE 4000 mg/kg/day) compared with control group. The weight of the liver was significantly lower in all treatment groups compared with the control group. The relative weight of the liver (BADGE 1000 and 4000 mg/kg/day) was significant lower than the control. No pathological changes were observed in the brain, liver, thyroid, heart, spleen, kidney, lung and prostate. The number of spermatid in the seminiferous tubule in the testes was lower in all treatment groups than the control. The sperm motility tended to decrease with increasing concentration but the sperm count was similar in all treatment groups. The plasma Estrogen and testosterone level were similar in the control and treatment groups. CONCLUSIONS: These results suggest that BADGE induces general, hepatic and reproductive toxicity at 1000 mg/kg/day.
Animals ; Brain ; Diarrhea ; Emaciation ; Epichlorohydrin ; Epoxy Resins ; Estrogens ; Ether* ; Heart ; Humans ; Kidney ; Liver ; Lung ; Male* ; Mouth ; Organ Size ; Plasma ; Prostate ; Rats ; Rats, Sprague-Dawley* ; Seminiferous Tubules ; Soil ; Sperm Count ; Sperm Motility ; Spermatids ; Spleen ; Testis ; Testosterone ; Thyroid Gland ; Weight Gain