The deletion 9p syndrome is a well characterized syndrome with about one hundred cases having been reported. Most patients have dysmorphic facial features, cardiac anomalies, and mental retardation. We report on a female infant with micrognathia, corneal opacity, cleft palace, cardiac anomaly, left polycystic kidney, and deletion 9p. Chromosome analysis and fluorescence in situ hybridization (FISH) showed her to have a derived chromosome 9 inherited from a maternal t(9;16) (p22;p13.2) by adjacent I segregation There are few reports of this particular chromosome rearrangement. We review deletion Sp syndrome.
Chromosomes, Human, Pair 9 ; Corneal Opacity ; Female ; Fluorescence ; Humans ; In Situ Hybridization ; Infant ; Intellectual Disability ; Polycystic Kidney Diseases

This study was undertaken for the clinical analysis and evaluation on 121 patients with incompetent internal os of the cervix, who were admitted and treated with McDonald operation or Shirodkar operation at the Soonchounhyang Medical Center from January 1991 to December 1995. The results of this study were as follows : 1. The incidence of this IIOC was 1.1% of 11,116 cases of total delivery. 2. The mean age of IIOC was 31.7 years old. 3. The average number of gravida was 3.2. 4. The most common contributary factor was previous history of artificial abortion (51.2 %), and midtrimester abortion (17.4 %), cervical laceration due to previous vaginal delivery (8.3 %) etc. was followed. 5. The success rate of operation was 76 %, and the highest success rate (85.7 %) was reveald with period from 15th weeks to 16th weeks of gestation. 6. When cervical dilatation was abscent or small, the success rate of operation was high. 7. The factors of failed operation were preterm labor (58.7 %), PROM (34.5 %), and PIH, bleeding. 8. The delivery method after operation was vaginal delivery in 83 cases (68.6%) and cesarean section in 38 cases (31.4 %).
Cervix Uteri* ; Cesarean Section ; Female ; Hemorrhage ; Humans ; Incidence ; Labor Stage, First ; Lacerations ; Obstetric Labor, Premature ; Pregnancy ; Pregnancy Trimester, Second

No abstract available.
Education ; Humans

No abstract available.
Humans ; Hypophosphatemia*

Periodic alternating nystagmus(PAN) is a form of spontaneous nystagmus with a specific pattern. It may be of congenital origin in many cases, and may be related to vestibulo-cerebellar system disease. It usually causes not only many ophthalmic problems such as decreased vision but also neurologic symptoms such as ataxic gait. So, ophthalmologist must perform both ophthalmic and neurologic examinations. Nystagmus can be treated with muscle relaxant. We report our experience about 30-year-old woman who complained of involuntary ocular movement and ataxic gait, and were diagnosed as PAN associated with Chiari-1 malformation and syringomyelia through electronystagmography and brain MRF.
Adult ; Brain ; Electronystagmography ; Female ; Gait ; Humans ; Neurologic Examination ; Neurologic Manifestations ; Nystagmus, Pathologic* ; Syringomyelia*

Objective and METHOD: In order to identify the aggravating agents for intrinsic asthma, we performed ASA- and food additive-challenge tests on 182 subjects diagnosed as having intrinsic asthma. The following tests were performed: Lysine-aspirin bronchoprovocation test to confirm aspirin-sensitivity, sodium bi-sulfite (40-200mg) oral provocation test for sulfite sensitivity, tartrazine oral provocation test (50mg) for tartrazine sensitivity, and sodium benzoate (400mg) oral provocation test for sodium benzoate sensitivity. Positive reaction was defined as decrease in FEV, by more than 20% from the baseline value after the provocation. RESULT: Seventy-five (41.2%) of 182 subjects showed positive responses to more than one agent among the aspirin and three food additives challenged. The prevalence of aspirin-sensitivity was the highest (22.5%), followed by sulfite-sensitivity (8.8%), and then concurrent sensitivity to both aspirin and sulfite (6.0% ), to both aspirin and tartrazine (1.6% ), to aspirin, sulfite and tartrazine (1.1%) and to aspirin, sulfite and sodium benzoate (0.5%). Rhino-sinusitis was noted in 62.5% of aspirin-sensitive asthmatic subjects, 60% of sulfite-sensitive ones, and 80% of tartrazine-sensitive ones. Urticaria was noted in 21.4% of aspirin-sensitive asthmatic subjects, 16.6% of sulfite-sensitive ones and 6.3% of tartrazine-sensitive ones. Thirty-seven to 83% of positive responders had no adverse reaction history. CONCLUSION: These findings suggest that ASA and food additive challenge tests should be considered as a screening test to evaluate any aggravating factors in subjects with intrinsic asthma, even though they may not have experienced any adverse reactions.
Aspirin* ; Asthma* ; Food Additives* ; Mass Screening ; Prevalence ; Sodium ; Sodium Benzoate ; Tartrazine ; Urticaria

BACKGROUND AND OBJECTIVES: Fulminant invasive fungal sinusitis (IFS) is an aggressive, destructive disease most commonly affecting the immunocompromised hosts. This study aimed to investigate the clinical features of invasive fungal sinusitis and to determine its prognostic factors. MATERIALS AND METHOD: Fifteen cases of invasive fungal sinusitis were retrospectively reviewed. Prognosis was analyzed according to predisposing factors including the underlying disease, the extent of disease, the mycotic species, and treatment modalities. RESULTS: Overall 5-year survival rate of the patients with IFS was 60%. There was significant survival gain in the group for which underlying disease was well controlled (p=0.04). There was no difference in survival in terms of mycotic species and extent of disease at presentation. No statistically significant difference in survival was observed between the conservative surgery and the radical surgery group. CONCLUSION: Survival is usually determined at the very early period of treatment. The most important prognostic factor is not the surgical modalities including wide surgical resection or conservative debridement, but the optimal control of underlying disease.
Causality ; Debridement ; Fibrinogen ; Humans ; Immunocompromised Host ; Prognosis ; Retrospective Studies ; Sinusitis* ; Survival Rate

Polymyositis(PM) is one of idiopathic inflammatory myopathy, characterized by proximal muscle weakness, myalgia and muscle enzyme elevation. Currently the main pathogenesis is well documented, the cell-mediated immunity. We experienced a case of polymyositis associated with hepatitis, developed after hepatitis B virus(HBV) infection. This virus-induced autoimmunity seems to result from the cross-reactivity between muscle protein and B-viral antigen, so called antigenic mimicry. This relation of PM and HBV is more significant in Korea because of the epidemicity of HBV infection.
Autoimmunity ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Immunity, Cellular ; Korea ; Molecular Mimicry ; Muscle Proteins ; Muscle Weakness ; Myalgia ; Myositis ; Polymyositis*

Diverticular disease of the colon is frequently involved in Western countries, which in korea, it is regarding as rare disease. In Western the diverticulosis is mainly affected on left side and increasing in according to age and false type is more frequent than ture type. While in oriental countries, the diverticulosis of the colon is mainly affected on right side and it is more frequent in younger patients. Cecal diverticulitis is known to a very rare disease and very difficult to be differenciated from acute scopy has purnished another useful tool in the differential diagnosis of diverticular disease. We experienced a case of cecal diverticultitis which had been diagnosed by colonofiberscopy and reviewed literatures briefly.
Colon ; Diagnosis, Differential ; Diverticulitis* ; Diverticulum ; Humans ; Korea ; Rare Diseases

BACKGROUND AND OBJECTIVE: IgG subclass deficiency has been reported in patients with bronchial asthma and is associated with recurrent respiratory tract infections. This study was done to identify prevalence of IgG subclass deficiency and to evaluate the possible difference between atopic and non-atopic asthmatics. Subjects and METHODS: We measured serum levels of IgG and IgG subclass in 35 asthmatic patients and 50 healthy controls using nephelometry. Reference values of each IgG subclass was defined as cumulative percentile between 2.5% to 97.5% of controls. RESULTS: Total IgG, IgG1 and IgG2 of asthmatics were significantly lower than for those of controls(p<0.05, respectively). In atopic asthmatics, compared with non-atopic asthmatics, IgG4 level was significantly higher (p<0.05). The frequency of IgG subclass levels below the reference value was eight (22.9%) of 35 asthmatics. CONCLUSION: IgG, IgG1 and IgG2 were significantly lower in asthmatic patients. Some patients had IgG subclass levels below reference value. Further studies will be needed to evaluate their clinical significance.
Asthma* ; Humans ; Immunoglobulin G* ; Nephelometry and Turbidimetry ; Prevalence ; Reference Values ; Respiratory Tract Infections