Caudal regression syndrome (CRS) is a rare neural tube defect that affects the terminal spinal segment, manifesting as neurological deficits and structural anomalies in the lower body. We report a case of a 31-month-old boy presenting with constipation who had long been considered to have functional constipation but was finally confirmed to have CRS. Small, flat buttocks with bilateral buttock dimples and a short intergluteal cleft were identified on close examination. Plain radiographs of the abdomen, retrospectively reviewed, revealed the absence of the distal sacrum and the coccyx. During the 5-year follow-up period, we could find his long-term clinical course showing bowel and bladder dysfunction without progressive neurologic deficits. We present this case to highlight the fact that a precise physical examination, along with a close evaluation of plain radiographs encompassing the sacrum, is necessary with a strong suspicion of spinal dysraphism when confronting a child with chronic constipation despite the absence of neurologic deficits or gross structural anomalies.

Caudal regression syndrome (CRS) is a rare neural tube defect that affects the terminal spinal segment, manifesting as neurological deficits and structural anomalies in the lower body. We report a case of a 31-month-old boy presenting with constipation who had long been considered to have functional constipation but was finally confirmed to have CRS. Small, flat buttocks with bilateral buttock dimples and a short intergluteal cleft were identified on close examination. Plain radiographs of the abdomen, retrospectively reviewed, revealed the absence of the distal sacrum and the coccyx. During the 5-year follow-up period, we could find his long-term clinical course showing bowel and bladder dysfunction without progressive neurologic deficits. We present this case to highlight the fact that a precise physical examination, along with a close evaluation of plain radiographs encompassing the sacrum, is necessary with a strong suspicion of spinal dysraphism when confronting a child with chronic constipation despite the absence of neurologic deficits or gross structural anomalies.

No abstract available.
Echolalia ; Serotonin Uptake Inhibitors ; Stroke

Objectives: ：To describe the differences in long-term outcomes in Alzheimer’s disease (AD) patients according to initial dementia severity. Methods: ：A retrospective chart review of AD patients from a dementia clinic at the University Hospital in Korea was conducted from April 2010 to March 2017. There were 168 patients enrolled, who were divided into three groups based on initial Clinical Dementing Rating (CDR). There were 55 in the very mild group (CDR=0.5 ; mean age 80.64±6.57), 93 in the mild group (CDR=1 ; mean age 80.57±7.28) and 20 in the moderate group (CDR=2 ; mean age 83.00±9.07). Participants were treated with donepezil±memantine. The observation period was 2.44±0.50 years. Cognitive function and severity of dementia were initially assessed by the Consortium to Establish a Registry for Alzheimer’s Disease Neuropsychological Assessment Battery (CERAD-NP) and were annually assessed by Mini-Mental State Exam (MMSE), CDR and CDR-Sum of boxes (CDR-SB). Results: ：The annual decline rate of MMSE score was -0.82 and those of very mild, mild, moderate groups were -0.63, -0.80, -1.96 respectively, while the annual change in CDR-SB score was 0.98, very mild group 0.86, mild group 1.03, moderate group 1.26. Education level, male, initial CDR were found to be significant potential factors in the annual change in MMSE, while initial CDR was a significant potential factor in the annual change in CDR-SB. Conclusion ：It is meaningful that we studied long-term outcomes of anti-dementia medications in real-world clinical setting. The higher the initial severity of AD, the higher the cognitive decline rate.

Translationally controlled tumor protein (TCTP) is also known as histamine releasing factor as it has the ability to activate mast cells. To investigate the role of TCTP in the pathogenesis of chronic spontaneous urticaria (CSU), we evaluated serum level of TCTP and effect of TCTP on basophil and mast cell degranulation. TCTP levels in the sera from 116 CSU patients and 70 normal healthy controls (NCs) were measured by ELISA. CD203c expression on basophils from CSU patients and β-hexosaminidase release from Laboratory of Allergic Disease 2 mast cells were measured upon stimulation monomeric and dimeric TCTP. Non-reducing Western blot analysis was used for detecting dimeric TCTP. No difference was observed in serum TCTP levels between CSU patients and NCs (p=0.676). However, dimeric TCTP intensity on Western blot was stronger in CSU patients than in NCs. TCTP levels were higher in patients with severe CSU (p=0.049) and with IgG positivity to FcɛRIα (p=0.038). A significant positive correlation was observed between TCTP and eosinophil cationic protein levels (Spearman's rho=0.341; p=0.001). Both basophil and mast cell degranulation were significantly increased after stimulation with dimeric TCTP, but not with monomic TCTP. The ability of TCTP to activate basophil and mast cells is dependent on dimerization, suggesting that the inhibition of TCTP dimerization can be a therapeutic option for CSU. Association between TCTP levels and the presence of IgG to high affinity Fc epsilon receptor I alpha subunit in CSU patients indicates that autoimmune mechanisms may be involved in the dimerization of TCTP.
Basophils ; Blotting, Western ; Dimerization ; Enzyme-Linked Immunosorbent Assay ; Eosinophil Cationic Protein ; Histamine ; Humans ; Immunoglobulin G ; Mast Cells ; Urticaria

BACKGROUND/OBJECTIVES: Diabetes is diagnosed after an average of 10–12 years of diabetic development. Strict glycemic control in diabetic patients promotes the normalization of blood glucose and reduces cardiovascular diseases (CVDs) and diabetic complications. Therefore, early diagnosis in non-aware individuals is very important. SUBJECTS/METHODS: Clinical indices and nutrient intakes in Korean diabetic adults aged 19–64 years were examined according to the awareness of diabetes, using 2012 and 2013 Korea National Health and Nutrition Examination Survey (KNHANES) data. The aware group was defined as individuals who were aware of having diabetes from diagnosis by physician before the survey and the non-aware group as individuals who were not aware of having diabetes. RESULTS: The average age was higher in the aware group compared to the non-aware group in both men (P = 0.002) and women (P = 0.004). The prevalences of hypertension and dyslipidemia were not different between the two groups, but the diagnosis rate was significantly lower in the non-aware group. In the non-aware group, total and LDL-cholesterol were significantly higher (P < 0.001), the risk for total cholesterol over 240 mg/dL was 3.4 times (95% CI: 1.58–7.52) higher (P = 0.002) and the risk for LDL-cholesterol over 160 mg/dL was 4.59 times (95% CI: 2.07–10.17) higher (P < 0.001). The calorie intake of the female non-aware group was significantly higher compared to the female aware group (P = 0.033). CONCLUSION: The results suggested that the recommendation of screening test is necessary even for young adults. Studies on the methodology for early diagnosis of diabetes are also needed.
Adult ; Blood Glucose ; Cardiovascular Diseases ; Cholesterol ; Diabetes Complications ; Diagnosis ; Dyslipidemias ; Early Diagnosis ; Female ; Humans ; Hypertension ; Korea ; Male ; Mass Screening ; Nutrition Surveys ; Prevalence ; Young Adult

No abstract available.
Infarction ; Stroke, Lacunar

No abstract available.
Abscess ; Carotid Arteries ; Stents

Wernicke encephalopathy is a syndrome caused by thiamine deficiency whose three typical symptoms are ophthalomoplegia, ataxia, and confusion. There are also rare reports of bilateral hearing loss, which can be caused by damage to the bilateral inferior colliculus or thalamic lesions, or by energy failure of the cochlea. This case suggests that thiamine should be administered based on the possibility of Wernicke encephalopathy occurring in malnourished or alcoholic patients with sudden bilateral hearing loss.
Alcoholics ; Ataxia ; Cochlea ; Hearing Loss ; Hearing Loss, Bilateral ; Humans ; Inferior Colliculi ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy