No abstract available.
Humans ; Mothers*

Trisomy 9p syndrome was first described by Rethore, et al in 1970 and about 150 cases have been reported. Trisomy 9p has been reported as either partial or complete. The term "duplication 9p syndrome" instead of "trisomy 9p syndrome" is used since most of the reported patients had only partial duplication rather than the whole arm duplication of 9p. Duplication of 9p syndrome is characterized by growth and developmental retardation, microbrachycephaly, deep and wide set eyes with down-slanting palpebral fissures, "globular" nose, down-turned corners of the mouth, prominent apparently low-set ears, and short fingers and toes with small nails. A 10- month-old male was referred to our department of pediatrics because of hypotonia and delayed development. Karyotype revealed 46, XY, dup(9)(p12p24) by GTC-Banding. We report a case of a duplication 9p syndrome diagnosed by GTC-banding.
Arm ; Ear ; Fingers ; Growth and Development ; Humans ; Karyotype ; Male ; Mouth ; Muscle Hypotonia ; Nose ; Pediatrics ; Toes ; Trisomy*

We have experienced three cases of mucinous ductal ectasia of the pancreas. They showed the characteristic duodenoscopic findings and underlying pathology was hyperplasia in two cases and adenocarcinoma in one case. When endoscopic retrograde pancreatography was performed, bulging ampulla of Vater, patulous ampullary orifice and mucus leakage from papillary orifice were noted. Also cyst-like dilatation of main duct or side branch of the uncinate process were observed.
Adenocarcinoma ; Ampulla of Vater ; Dilatation ; Dilatation, Pathologic* ; Hyperplasia ; Mucins* ; Mucus ; Pancreas* ; Pathology

Campomelic syndrome is a very rare skeletal dysplasia with a characteristic pattern of deformity involving the proximal and distal extremities, pelvic and shoulder girdles, thoracic cage and palate. Respiratory compromise often leads to death in early infancy. Etiology has not been determined although evidence suggests genetic heterogeneity. Cytogenetic study revealed high incidence of a 46,XY karyotype in phenotypic females. Recently, we had experienced a case of campomelic dysplasia at amenorrhea 30weeks and termination was done, so we report with a brief review of literature.
Amenorrhea ; Campomelic Dysplasia* ; Congenital Abnormalities ; Cytogenetics ; Extremities ; Female ; Genetic Heterogeneity ; Humans ; Incidence ; Karyotype ; Palate ; Shoulder

Hyperacute or acute accelerated rejection caused by preformed antibody in sensitized patients resulted in increased waiting period and complicated posttransplant hospital course. Intravenous immunoglobulin (IVIG) has known to have anti cytotoxic effect by blocking the anti HLA antibody. PURPOSE: We investigated the effect of IVIG on hyperacute and acclerated rejection of the heart graft in the presensitized rat. METHODS: Recipients (Wistar) were sensitized from repeated allo (Lewis) skin graft and followed by heterotopic allo cardiac transplantation. A guinea pig was used for the xenotransplantation model. IVIG (Green Cross kappa, 400 mg/kg in allotransplantation, 800 mg/kg in xenotransplantation) was given just before heart transplantation. Graft survival and donor specific IgG, IgM and complement were measured. RESULTS: Graft survival was 7.2 days in non sensitized allogenic heart transplantation (n=9), 1.3 days in sensitized allogenic recipients (n=7). Graft survival was prolonged from 1.3 days to 4.4 days with IVIG treatment (n=5). As for xenogenic transplantation, graft survival was prolonged from 30 min to 7.4 hr with IVIG treatment (n=5). Donor specific IgG and IgM and complement increment were blocked by IVIG during the IVIG treatment. Donor specific IgG and Ig M and complement were increased after the cessation of IVIG treatment. CONCLUSION: IVIG was able to prolong the graft survival of the sensitized allograft and xenograft. Suppression of the donor specific IgG, IgM and complement might be one of the underlying mechanisms. A further studies have to follow to clarify the more detailed mechanism.
Allografts ; Animals ; Complement System Proteins ; Graft Survival ; Guinea Pigs ; Heart Transplantation* ; Heart* ; Heterografts ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Rats* ; Skin ; Tissue Donors ; Transplantation, Heterologous ; Transplants

Persistent hiccups are described as a recurring troublesome series of involuntary inspirations accompanied by glottic closure, lasting longer than a month. Recently, baclofen, the most effective treatment for intractable hiccups was found to be a useful drug for the management of chronic hiccups, and that substituting gabapentin for baclofen can be effective. Moreover, gabapentin was used successfully in combination with baclofen. We report here on three patients with persistent hiccups, where gabapentin was used successfully as an "add-on" with baclofen.
Baclofen* ; Hiccup* ; Humans

No abstract available.
Bile Ducts* ; Bile* ; Carcinoma, Hepatocellular*

PURPOSE: The purpose of this study was to analyze effects of a community-based case management program for clients with hypertension living in the community. METHODS: The research design was a one group pre and post-test design with 30 participants with hypertension who agreed to participate in the 8-12 week case management program provided by case managers from the National Health Insurance Corporation in 2002. Data were collected three times, before and after the case management services, and 6 months later. Outcomes included changes in blood pressure, knowledge of hypertension and daily life practices, including alcohol consumption, smoking, exercise, and medication adherence. RESULTS: Repeated-measures ANOVA and post-hoc tests of means revealed significant differences before and after service for systolic blood pressure, daily life practices (monitoring body weight and BP, low salt and cholesterol and high vegetable diet, and stress-relief practices), and exercise. The goal for medication adherence was attained after service. Significant improvements from baseline to 6 months after service were observed in measures of salt and vegetables in diet. There were no significant differences on hypertension knowledge, alcohol consumption or smoking behavior between before service and after, and at 6 months. CONCLUSION: The findings provide preliminary evidence that case management intervention can have positive outcomes on BP control, daily life practices, exercise, and medication adherence for clients with hypertension. However, additional interventions are needed to sustain long-term effects.
Activities of Daily Living ; Aged ; Alcohol Drinking/prevention & control/psychology ; Blood Pressure ; Case Management/*organization & administration ; Community Health Services ; Diet, Sodium-Restricted ; Female ; Humans ; Hypertension/psychology/*therapy ; Korea ; Male ; Medication Adherence/psychology ; Middle Aged ; Patient Education as Topic ; Smoking Cessation/psychology

PURPOSE: To identify prognostic factors influencing progression-free survival (PFS) of aggressive fibromatosis (AF) after postoperative radiotherapy (PORT) and assess correlations between immunohistochemistry (IHC) features of β-catenin/smooth muscle actin (SMA) and PFS. MATERIALS AND METHODS: Records of 37 patients with AF treated by PORT from 1984 to 2015 were retrospectively reviewed. Fifteen patients underwent wide excision for AF and 22 patients received debulking operation. The median total dose of PORT was 59.4 Gy. IHC staining results of β-catenin and SMA were available for 11 and 12 patients, respectively. RESULTS: The median follow-up duration was 105.9 months. Five-year PFS rate was 70.9%. Tumor size or margin status was not related to PFS in univariate analysis (p = 0.197 and p = 0.716, respectively). Multivariate analysis showed that increased interval from surgery to PORT (>5.7 weeks) was a marginal risk factor for PFS (p = 0.054). Administration of PORT at the initial diagnosis resulted in significantly improved PFS compared to deferring PORT after recurrence (p = 0.045). Patient with both risk factors of deferring PORT after recurrence and interval from surgery to PORT >5.7 weeks had significantly lower 5-year PFS than patients without risk factor (34.1% vs. 100.0%; p = 0.012). Nuclear β-catenin intensity tended to inversely correlate with 5-year PFS, although it did not reach statistical significance (62.5% at low vs. 100.0% at high; p = 0.260). SMA intensity was not related to PFS (p = 0.700). CONCLUSION: PORT should be performed immediately after surgery irrespective of margin status or tumor size especially in recurrent case. Nuclear β-catenin staining intensity of IHC might correlate with local recurrence.
Actins ; beta Catenin ; Diagnosis ; Disease-Free Survival ; Fibromatosis, Aggressive ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Multivariate Analysis ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Risk Factors

Purpose: The purpose of this study was to examine the psychological distress related to quality of life (QoL) of patients with colorectal cancer receiving 5-fluorouracil (5-FU) chemotherapy at home with disposable Elastomeric infusion pumps. Methods: In this study, 179 colorectal outpatients were recruited between September 2019 and January 2021. National Cancer Center Psychological Symptom Inventory scores, general self-efficacy, and the EORTC QLQ-C30 scores were measured. Data were analyzed using Independent t-test, One-way ANOVA with Bonferroni post hoc analysis, and hierarchical multiple linear regression with the SPSS/WIN 26.0 programs. Results: The overall prevalence of psychological distress was 52.0% in colorectal patients. In multiple regression, psychological distress (β=-.20, p=.005), appetite loss (β=-.20, p=.001), chemotherapy cycles (β= .19, p=.002), fatigue (β=-.16, p=.035), physical functioning (β=-.16, p=.024), and emotional functioning (β=-.15, p=.025) were significant factors of QoL, and the final model explained 45.0% of the total variance of QoL. Conclusion Supporting patients toward decreased psychological distress and increased physical and emotional functioning, especially in the first or second cycle of chemotherapy, could be used to improve their QoL. To consider the thresholds for clinical importance, it is necessary to increase the interpretation of psychological distress in clinical practice and further research.