Osteogenesis imperfecta is a rare congenital disease. It is a heterogeneous group of inherited disorders characterized by multiple bone fracture, blue sclera, hearing loss, abnormalities of dentition and widespread connective tissue ahnormality. We experienced a case of osteogenesis imperfecta diagnosed in utero by ultrasonogram and confirmed hy postnatal radiograph after delivery. We present the case with a hrief review of the literature.
Connective Tissue ; Dentition ; Fractures, Bone ; Hearing Loss ; Osteogenesis Imperfecta ; Sclera ; Ultrasonography

Cytomegalovirus (CMV) infection is more frequent in immunocompromised patients those with acquired immunodeficiency syndrome (AIDS), malignant disease, steroid therapy. However, CMV can infect a healthy person who has normal immunity. Most cases of CMV infections are due to reactivation of latent virus. We report a case of cytomegalovirus colitis in a 73 years old woman who has congestive heart failure with normal immunity. Sigmidoscopy reveals cobble stone like mucosa and deep ulceration. CMV infection produces a cytomegalic cell containing a intranuclear inclusion, which is surrounded by clear halo in Hematoxylin-Eosin stain. Immunohistochemical stain for CMV reveals focal positive in cytoplasm and in nuclei of large cells. We diagnosed CMV colitis with histopathologic finding and immunohistochemistry through sigmoidoscopic mucosal biopsy.
Acquired Immunodeficiency Syndrome ; Aged ; Biopsy ; Colitis* ; Cytomegalovirus* ; Cytoplasm ; Female ; Heart Failure ; Humans ; Immunocompromised Host ; Immunohistochemistry ; Intranuclear Inclusion Bodies ; Mucous Membrane ; Ulcer

BACKGROUND/AIMS: The role of autonomic dysfunction in patients with functional dyspepsia has not been completely understood. The purposes of our study are (1) to prospectively assess the abnormalities of the autonomic function in patients with functional dyspepsia and (2) to assess whether the presence of autonomic dysfunction in patients with functional dyspepsia correlates with the presence of visceral hypersensitivity or with the severity of dyspeptic symptoms. METHODS: Twenty eight patients with functional dyspepsia (4 men and 24 women; age range, 29-57) and 14 healthy volunteers without gastrointestinal symptoms (6 men and 8 women; age range, 23-61) were included in this study. All patients and controls were submitted to a battery of five standard cardiovascular autonomic reflex tests and gastric barostat tests. A modified version of the Glasgow Dyspeptic questionnaire was used in this study. RESULTS: (1) Autonomic function tests showed that both sympathetic and parasympathetic scores of dyspeptic patients were significantly higher than those of the control group. (2) Visceral hypersensitivity could be confirmed in some of our dyspeptic patients in response to proximal gastric distension, demonstrating lower pain threshold in this group. (3) We could not find significant association between the presence of autonomic dysfunction and the presence of visceral hypersensitivity or severity of dyspeptic symptoms in patients with functional dyspepsia. CONCLUSION: Autonomic dysfunction was more prevalent in dyspeptic patients than in the control group. However, it did not correlate with the presence of visceral hypersensitivity or severity of dyspeptic symptoms. It is suggested that a defect in the spinal region or at the CNS level may be a major mechanism of visceral hypersensitivity in functional dyspepsia.
Dyspepsia* ; Female ; Healthy Volunteers ; Humans ; Hypersensitivity ; Male ; Pain Threshold ; Prospective Studies ; Reflex ; Surveys and Questionnaires

Previous studies have demonstrated that rottlerin, a specific PKCdelta inhibitor, potentiates death receptor- mediated apoptosis through a cytochrome c-dependent or -independent pathway. However, its ability to regulate necrotic cell death, as well as the underlying mechanism, remains unknown. We found that in murine fibrosarcoma L929 cells, treatment with rottlerin protected the cells against TNF-induced necrosis, whereas it sensitized the cells to apoptosis induced by co-treatment with Hsp90 inhibitor geldanamycin and TNF, in a manner independent of its ability to inhibit PKC-delta. TNF treatment induced rapid accumulation of mitochondrial superoxide (O2") through the Nox1 NADPH oxidase when cells undergo necrosis. Moreover, pretreatment with rottlerin failed to induce the GTP-bound form of small GTPase Rac1 by TNF treatment, and subsequently suppressed mitochondrial O2(-) production and poly(ADP-ribose) polymerase activation, thus inhibiting necrotic cell death. Therefore, our study suggests that Nox1 NADPH oxidase is a new molecular target for anti-necrotic activity of rottlerin upon death-receptor ligation.
Acetophenones/*pharmacology ; Animals ; Benzopyrans/*pharmacology ; Cell Death/*drug effects ; Cell Line, Tumor ; Mice ; Protein Kinase Inhibitors/*pharmacology ; Superoxides/metabolism ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors/pharmacology

BACKGROUND/AIMS: Menetrier's disease is a poorly defined condition that is of unknown origin, characterized by giant folds in the stomach. The histologic features are foveolar hyperplasia and cystic dilatation of the gland. We presented the characteristic findings of Menetirer's disease in Korea with a review of literatures to understand the Menetirer's disease more precisely. METHODS: The sixteen cases of Menetrier's disease was reported in Korea. We analyzed their age, sex, symptoms, signs, laboratory findings and treatments, retrospectively. RESULTS: The average age was 46 years. There were 11 men and 4 women. The most common symptom was epigastric pain (94%). The most common sign were epigastric tenderness (69%) and pretibial pitting edema (63%). Patients were often associated with the hypoalbuminemia (73%). All patients showed hypertrophic folds on either gastrofiberscopy or upper gastrointestinal series. All patients showed foveolar hyperplasia histologically. Three patients were operated to control a massive upper gastrointesinal bleeding. Two patients were operated to control the intractable edema. Two patients were operated to exclude gastric malignancy. CONCLUSIONS: Menetrier's disease showed broad clinical features such as epigastric pain, hypoalbuminemia, massive hematemesis and mimicking gastric malignancy. The giant gastric folds and foveolar hyperplasia were the most commom and important findings in the Menetrier's disease.
Dilatation ; Edema ; Female ; Gastritis, Hypertrophic* ; Hematemesis ; Hemorrhage ; Humans ; Hyperplasia ; Hypoalbuminemia ; Korea* ; Male ; Retrospective Studies ; Stomach

Background/Aims: Prokinetics such as mosapride citrate CR (conventional-release; Gasmotin) are commonly used in functional dyspepsia (FD). This study aims to evaluate the efficacy and safety of once-a-day mosapride citrate SR (DWJ1252), a sustained-release formulation of mosapride citrate, compared with mosapride citrate CR 3 times a day, in patients with FD. Methods: In this multicenter, randomized, double-blind, active-controlled, non-inferiority study, 119 patients with FD (by the Rome III criteria, 60 for mosapride citrate SR and 59 for mosapride citrate CR) were randomly allocated to mosapride citrate SR once daily or mosapride citrate CR thrice daily for 4 weeks in 16 medical institutions. Primary end point was the change in gastrointestinal symptom (GIS) score from baseline, assessed by GIS questionnaires on 5-point Likert scale after 4-week treatment. Secondary end points and safety profiles were also analyzed. Results: The study included 51 and 49 subjects in the mosapride citrate SR and mosapride citrate CR groups, respectively. GIS scores at week 4 were significantly reduced in both groups (mean ± SD: − 10.04 ± 4.45 and − 10.86 ± 5.53 in the mosapride citrate SR and mosapride citrate CR groups, respectively; P < 0.001), and the GIS changes from baseline did not differ between the 2 groups (difference, 0.82 point; 95% CI, − 1.17, 2.81; P = 0.643). Changes in GIS at weeks 2 and 4 and quality of life at week 4, and the improvement rates of global assessments at weeks 2 and 4, did not differ between the groups. Adverse events were similar in the 2 groups, and there were no serious adverse events. Conclusion In patients with FD, mosapride citrate SR once daily is as effective as mosapride citrate CR thrice daily, with a similar safety profile.

This study compared the efficacy of DA-9601 (Dong-A ST Co., Seoul, Korea) and its new formulation, DA-5204 (Dong-A ST Co.), for treating erosive gastritis. This phase III, randomized, multicenter, double-blind, non-inferiority trial randomly assigned 434 patients with endoscopically proven gastric mucosal erosions into two groups: DA-9601 3 times daily or DA-5,204 twice daily for 2 weeks. The final analysis included 421 patients (DA-5204, 209; DA-9601, 212). The primary endpoint (rate of effective gastric erosion healing) and secondary endpoints (cure rate of endoscopic erosion and gastrointestinal [GI] symptom relief) were assessed using endoscopy after the treatment. Drug-related adverse events (AEs), including GI symptoms, were also compared. At week 2, gastric healing rates with DA-5204 and DA-9601 were 42.1% (88/209) and 42.5% (90/212), respectively. The difference between the groups was −0.4% (95% confidence interval, −9.8% to 9.1%), which was above the non-inferiority margin of −14%. The cure rate of gastric erosion in both groups was 37.3%. The improvement rates of GI symptoms with DA-5204 and DA-9601 were 40.4% and 40.8%, respectively. There were no statistically significant differences between the two groups in both secondary endpoints. AEs were reported in 18 (8.4%) patients in the DA-5204 group and 19 (8.8%) in the DA-9601 group. Rates of AE were not different between the two groups. No serious AE or adverse drug reaction (ADR) occurred. These results demonstrate the non-inferiority of DA-5204 compared to DA-9601. DA-5204 is as effective as DA-9601 in the treatment of erosive gastritis. Registered randomized clinical trial at ClinicalTrials.gov (NCT02282670)
Artemisia ; Double-Blind Method ; Drug-Related Side Effects and Adverse Reactions ; Endoscopy ; Gastritis* ; Humans ; Seoul

BACKGROUND/AIMS: To evaluate the efficacy and safety of a controlled release, once-daily formulation of mosapride (UI05MSP015CT) in patients with functional dyspepsia (FD). METHODS: Patients with FD were randomly assigned (1:1) to receive either UI05MSP015CT (15 mg once a day, study group) or mosapride (5 mg three times a day, control group) and corresponding placebo for 4 weeks. The primary endpoint was a change in the gastrointestinal symptom score (GIS) evaluated at enrollment and after 4 weeks. Secondary endpoints were changes in the Nepean Dyspepsia Index-Korean version (NDI-K), rate of satisfactory symptom relief, and rate of adverse events. RESULTS: A total of 138 patients were enrolled (female, 73.9%; mean age, 44.0±15.4 years). After excluding patients who violated the study protocol, 59 and 58 patients from the study and control groups, respectively, were included in the per-protocol analysis. No difference was observed in drug compliance between the control and study groups (97.07%±4.52% vs 96.85%±6.05%, p=0.870). Changes in GIS scores were 9.69±6.44 and 10.01±5.92 in the study and control groups. The mean difference in GIS change between groups was 0.33 (95% confidence interval, 1.75 to 2.41), demonstrating non-inferiority of UI-05MSP015CT (p=0.755). The rate of satisfactory symptom relief was not different between the study and control groups (39.0% vs 56.9%, p=0.053). No differences in change in NDI-K score (14.3 vs 16.9, p=0.263) or rates of adverse events (12.9% vs. 4.4%, p=0.062) were observed between the study and control groups. CONCLUSIONS: Once-daily mosapride is not inferior to conventional mosapride in efficacy and is safe in patients with FD.
Compliance ; Dyspepsia* ; Humans

BACKGROUND/AIMS: The aims of this study were to determine subgoups of functional dyspesia and to evaluate the short-term effect of cisapride in patients with functional dyspepsia in Korea. METHODS: 1025 patients, with a mean age of 42.6 years, with symptoms of functional dyspepsia, were recruited consecutively and upper gastrointestinal symptoms were investigated by interview in 41 hospitals in Korea. In an open, multicenter trial, 1025 patients received Smg of cisapride three times a day (TID) for at least .2 weeks for the treatment of symptoms of functional dyspepsia. When necessary, the dose of cisapride was increased to 10mg TID and the duration of therapy was extended to 4 weeks. RESULTS: The most frequently reported symptoms of functional dyspepsia were epigastric discomfort or fullness (85%), bloating (70%), belching (53%), early satiety (52%) and epigastric pain (46%) retrospectively. Subgroups of functional dyspepsia were as follows; dysmotility-like 73.5%, ulcer-like 39.7%, reflux-like 13.0%, and unspecified dyspepsia 14.0%. However, 33.2% of subjects with functional dyspepsia could be classified into more than one subgroup. Upper gastrointestinal symptoms were decreased to average 50.3% (range; 42.2 to 59.2%) after 2 weeks of cisapride treatment and to 25% (19.2 to 29.9%) after 4 weeks. cisapride therapy resulted in good or excellent improvement in 59.0% of the patients after two weeks, in 75% of patients after 4 weeks. Adverse events were occurred in 52 patients (5.8% of all patients), most commonly, loose stools or diarrhea (3.5%), abdominal pain (1.1%), and dizziness (0.3%). The majority of adverse events was mild and transient in nature and led to premature discontinuation of treatment in 4 patients. CONCLUSIONS: Although the majorities of patients with functional dyspepsia have dysmotility like symptoms in Korea, there is such overlap among the dyspepsia subgroups. Most patients responded well to a short therapeutic trial with cisapride without significant side effects.
Abdominal Pain ; Cisapride* ; Diarrhea ; Dizziness ; Dyspepsia* ; Eructation ; Humans ; Korea* ; Retrospective Studies