Incontinentia pigmenti(IP), so called Bloch-Sulzberger syndrome is a rare hereditary neurocutaneous syndrome and was described first by Bardach in l925. This disorder is known as a systemic disease caused by a defect at the developmental stage of organs originated from ectoderm or mesoderm. Incontinentia pigmenti is presented by characteristic linear hyperpigmented skin lesions, often associated with central nervous system involvement, dysplasia in the dental system, and ocular abnormalities. This disorder occurs almost in female infants, usually lethal in males, inherited as X-linked dominantly. Thirty percent of the patients suffer from central nervous system complications such as mental retardation, encephalopathy, delayed development, seizure, spastic paralysis and microcephaly. We report a case of a 12 month-old female with incontinentia pigmenti who was hospitalized with linear hyperpigmented skin lesions and delayed development.
Central Nervous System ; Ectoderm ; Female ; Humans ; Incontinentia Pigmenti ; Infant ; Intellectual Disability ; Male ; Mesoderm ; Microcephaly ; Muscle Spasticity ; Neurocutaneous Syndromes ; Paralysis ; Seizures ; Skin

Purpose: The purpose of this study was to examine the associations of aerobic physical activity and musclestrengthening exercise with mental health, respectively. Furthermore, the relationship between combined exercise with depression, stress, and suicidal thoughts was also examined. Methods: The association between adherence to physical activity guidelines and mental health was analyzed among 27,268 adults who participated in the 2014– 2018 Korea National Health and Nutrition Examination Survey. Logistic regression was used to predict the relationship between adherence to physical activity guidelines and mental health. Results: The odds ratio for depression and suicidal thoughts in groups that met both aerobic physical activity and muscle-strengthening exercise guidelines decreased by 27% (95% confidence interval [CI], 0.56– 0.94) and 40% (95% CI, 0.39– 0.93), respectively, compared to those that did not meet both. Stress awareness decreased by 22% (95% CI, 0.67– 0.90) in those who met muscular exercise guidelines, but did not meet aerobic physical activity guidelines. For those who met only the aerobic physical activity guidelines, stress awareness decreased by 12% (95% CI, 0.82 – 0.95). In addition, the odds ratio decreased by 29% (95% CI, 0.62 – 0.80) in groups that met both guidelines. Conclusion The most positive relationships between adherence to physical activity guidelines and mental health were found when both aerobic and muscular exercise guidelines were met.

PURPOSE: Recently, researchers at medical schools have shown increasing interest in developing a curriculum for medical humanities and improving 'humanity' in existing curriculums. This study introduces the process of developing the 'doctor & leadership' curriculum at K University from 2002. The results of the three-year implementation were analyzed. The effectivenessof the curriculum development is discussed. METHODS: The curriculum development followed the ADDIE model. A sixteen-week curriculum with three subjects was developed. The main methods of instruction were self-regulation learning and collaboration learning. The instruction method was a blended learning approach composed of both on-line and off-line instructions. Students were assessed through a portfolio assessment. 'Leadership self-assessment' and 'satisfaction index' of the overall curriculum were measured. RESULTS: The results on pre- and post- leadership self-assessment shows that leadership increased significantly after the learning process. Of the 4 sub-factors of the satisfaction index, the satisfaction indices on the blended learning, professor-student interaction, and instruction design had the greatest influence on the total satisfaction index (p<.001) CONCLUSION: Our outcome indicates that the 'doctor & leadership' curriculum is significantly effective. And generally, to increase the satisfaction index of courses, collaboration learning based on team projects and blended learning, as well as, professor-student interaction are helpful in encouraging the application of skills.
Cooperative Behavior ; Curriculum* ; Humanities ; Humans ; Leadership ; Learning ; Schools, Medical ; Self-Assessment

Background: and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. Methods: The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. Results: The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. Conclusions The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

Background: and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. Methods: The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. Results: The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. Conclusions The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.