Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

No abstract available.
Arthritis, Rheumatoid* ; Humans ; Hyperplasia* ; NF-kappa B*

No abstract available.
Gastroschisis*

It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital hypothyroidism can ameliorate and possibly reverse its consequences. Treatment with L-thyroxine is started in case of suspicious hypothyroidism at the first visit. Serum concentration of total and free thyroxine become normal within 1week after start of therapy but TSH values become normal from 2 months to 6 months after L-thyroxine therapy. The possible explanations for prolonged TSH elevation in congenital hypothyroidism are poor compliance for therapy, an inadequate dose of L-thyroxine, elevated threshold for thyrotropin suppression and two novel mutations in the thyrotropin (TSH) receptor gene in a child with resistance to TSH. Authors have experienced two cases of prolonged TSH elevation with normal T3 and T4 levels till 18months and 27 months of age after optimal L-thyroxine therapy and literature were reviewed.
Child ; Compliance ; Congenital Hypothyroidism* ; Humans ; Hypothyroidism ; Infant, Newborn ; Mass Screening ; Thyrotropin ; Thyroxine

PURPOSE: To identify the effects of spousal support on the relationship between acculturative stress and sense of parenting competence among married Vietnamese immigrant women. METHODS: The sample for this study consisted of 175 married Vietnamese immigrant women in Gyeongsangnam-do. Data were analyzed using descriptive statistics, t-test, ANOVA, Scheffé test, Pearson correlations, and hierarchical multiple regression with SPSS/WIN 23.0. RESULTS: (a) sense of parenting competence was negatively correlated with acculturative stress, whereas a positive correlation was observed with spousal support; (b) spousal support mediated the relationship between acculturative stress and sense of parenting competence. CONCLUSION: Results of the study suggest that spousal support decreases the negative effects of acculturative stress on sense of parenting competence. Therefore, we need to find ways to strengthen spousal support for married Vietnamese immigrant women.
Acculturation ; Asian Continental Ancestry Group* ; Emigrants and Immigrants* ; Female ; Gyeongsangnam-do ; Humans ; Mental Competency* ; Parenting* ; Parents* ; Spouses

Objectives: This research aimed to identify the level of perception, knowledge, preventive behavior, and factors affecting preventive behavior of patients with asthma and chronic obstructive pulmonary disease against particulate matter. Methods: This research was a descriptive survey research, and the subjects were chronic pulmonary disease patients over the age of 19 and under 80 who visited a university hospital in Daegu City. Data was collected by convenience sampling through structured self-administered questionnaire survey from December 2019 to January 2020, and a total of 212 copies were used for analysis. Results: Out of 212 total subjects, 112 were asthma patients (52.8%) and 100 were chronic obstructive pulmonary disease patients (47.2%). The average score (out of 10) of perception, knowledge and preventive behavior of patients with asthma for particulate matter was 7.92, 6.99, and 7.10, respectively, while those with chronic obstructive pulmonary disease scored 7.72, 6.24, and 6.80, respectively. The knowledge score was significantly higher in patients with asthma than those with chronic obstructive pulmonary disease (p=0.007). Factors affecting particulate matter preventive behavior were perception score, knowledge score, and age for asthma patients, and perception score for chronic obstructive pulmonary disease patients. Conclusions As a result of the above, the factors relate to the preventive behavior of patients with asthma and chronic obstructive pulmonary disease were perception score, knowledge score, and age for asthma, and perception score for chronic obstructive pulmonary disease. Raising the level of particulate matter preventive behavior can prevent the deterioration of chronic pulmonary diseases caused by particulate matter, so the development of arbitration programs considering the characteristics of the patients according to the disease and continuous and repetitive education are required.

Background: This study evaluated the effects of a mobile diabetes management program called “iCareD” (College of Medicine, The Catholic University of Korea) which was integrated into the hospital’s electronic medical records system to minimize the workload of the healthcare team in the real clinical practice setting. Methods: In this retrospective observational study, we recruited 308 patients. We categorized these patients based on their compliance regarding their use of the iCareD program at home; compliance was determined through self-monitored blood glucose inputs and message subscription rates. We analyzed changes in the ABC (hemoglobin A1c, blood pressure, and low-density lipoprotein cholesterol) levels from the baseline to 12 months thereafter, based on the patients’ iCareD usage patterns. Results: The patients comprised 92 (30%) non-users, 170 (55%) poor-compliance users, and 46 (15%) good-compliance users; the ABC target achievement rate showed prominent changes in good-compliance groups from baseline to 12 months (10.9% vs. 23.9%, P<0.05), whereas no significant changes were observed for poor-compliance users and non-users (13.5% vs. 18.8%, P=0.106; 20.7% vs. 14.1%, P=0.201; respectively). Conclusion Implementing the iCareD can improve the ABC levels of patients with diabetes with minimal efforts of the healthcare team in real clinical settings. However, the improvement of patients’ compliance concerning the use of the system without the vigorous intervention of the healthcare team needs to be solved in the future.

Background: Recent studies have shown that oral administration of probiotics may improve the immune imbalance caused by dysbiosis of the gut microbiome in atopic dermatitis (AD). This study aimed to investigate the clinical and immunological effects of Lactobacillus pentosus in children with mild to moderate AD. Methods: Children aged 2–13 years with AD were randomized to receive either 1.0 × 1010 colony-forming units of L. pentosus or placebo, daily, for 12 weeks. The clinical severity of AD and transepidermal water loss were evaluated. Blood eosinophil counts, serum total immunoglobulin E (IgE), and cytokine levels were measured. The diversity and composition of the gut microbiota were also analyzed. Results: Eighty-two children were recruited, and 41 were assigned to the probiotics intervention group. The mean scoring of atopic dermatitis (SCORAD) indices at baseline were 30.4 and 34.3 for the probiotics and placebo groups, respectively. At week 12, the mean indices were 23.6 and 23.1 for the probiotics and placebo groups, respectively. Clinical severity decreased significantly over time in both groups, with no significant difference between the two groups. In both groups, there were no significant differences in cytokine levels, microbial diversity, or the relative abundance of the gut microbiota at week 12 compared with the corresponding baseline values. The mean subjective scores of SCORAD indices after intervention for the probiotics group were significantly lower than those for the placebo group in IgE sensitized AD (P = 0.019). Conclusion Our results show improved symptoms in the probiotics and placebo groups, and we could not find additional effects of L. pentosus in AD. However, the mean subjective scores of SCORAD indices for the probiotics group are significantly improved compared with those for the placebo group in allergen-sensitized AD.

Osteochondral lesions of the femoral head are uncommon and few studies have reported their imaging findings. Since joints are at risk of early degeneration after osteochondral damage, timely recognition is important. Osteochondral lesions of femoral head may often be necessary to differentiate from avascular necrosis. Here, we report a case of osteochondral lesions on bilateral femoral heads. This lesion manifested as subchondral cysts in initial radiographs, which led to further evaluation by computed tomography arthrography and magnetic resonance imaging, which revealed overlying cartilage defects.
Female ; Hip Joint/abnormalities/*radiography ; Humans ; Magnetic Resonance Imaging ; Osteochondritis/*diagnosis ; Patients ; Sports ; Tomography, X-Ray Computed ; Young Adult