Neonatal cholestasis is caused by various forms of liver injury and has a complex etiological background. Among these, cases of severe cholestasis due to primary hemolytic disease are rare. Herein, we report a case in which thalassemia-induced severe hemolysis caused bile duct injury by hemosiderosis, with cholestasis occurring shortly after birth and lasting for >4 months. In addition, complete recovery of liver pathology was observed both biochemically and histologically. Hence, clinicians should consider hemolytic disease as a rare cause of neonatal cholestasis in the differential diagnosis of neonates as well as the advisability of conservative treatment based on case progression.

Background and Objectives: Although several studies have compared the characteristics of Ramsay Hunt syndrome (RHS) with Bell’s palsy (BP), the differences in comorbid symptoms and prognosis according to symptoms have not been determined. This study therefore evaluated the differences in otologic symptoms and prognosis between patients with these two conditions.Subjects and Method The medical records of 118 patients with RHS and 215 patients with BP were retrospectively reviewed. Factors compared in these two groups included otologic symptoms, general health condition, electroneurography (ENoG) and House-Brackmann grades. Results: Age, sex, body mass index, lipid profiles, ENoG, rate of diabetes, and side of palsy did not differ significantly between patients with RHS and BP (p>0.05). The rates of hearing disturbance, tinnitus, vertigo, and postauricular pain were significantly higher in RHS (p<0.05 each). Hearing disturbance was more frequent in patients with severe Bell’s facial palsy than with moderate Bell’s facial palsy (p<0.05). The prognosis of patients with BP and RHS who had otologic symptoms did not differ from those who had not (p>0.05). Additionally, in patients with facial paralysis, diabetes was associated with hearing disturbance and vertigo symptoms and dyslipidemia was associated with postauricular pain (p<0.05 each). Conclusion Otologic symptoms were more common in RHS than in BP. However, the prognosis of RHS and BP were not related to otologic symptoms. In patients with facial palsy hearing disturbance and vertigo were associated with diabetes and hypertension. Also, dyslipidemia was associated with post auricular pain.

Due to the inconsistent fluctuation of blood supply for transfusion, much attention has been paid to the development of artificial blood using other animals. Although mini-pigs are candidate animals, contamination of mini-pig T cells in artificial blood may cause a major safety concern. Therefore, it is important to analyze the cross-reactivity of IL-7, the major survival factor for T lymphocytes, between human, mouse, and mini-pig. Thus, we compared the protein sequences of IL-7 and found that porcine IL-7 was evolutionarily different from human IL-7. We also observed that when porcine T cells were cultured with either human or mouse IL-7, these cells did not increase the survival or proliferation compared to negative controls. These results suggest that porcine T cells do not recognize human or mouse IL-7 as their survival factor.

Due to the inconsistent fluctuation of blood supply for transfusion, much attention has been paid to the development of artificial blood using other animals. Although mini-pigs are candidate animals, contamination of mini-pig T cells in artificial blood may cause a major safety concern. Therefore, it is important to analyze the cross-reactivity of IL-7, the major survival factor for T lymphocytes, between human, mouse, and mini-pig. Thus, we compared the protein sequences of IL-7 and found that porcine IL-7 was evolutionarily different from human IL-7. We also observed that when porcine T cells were cultured with either human or mouse IL-7, these cells did not increase the survival or proliferation compared to negative controls. These results suggest that porcine T cells do not recognize human or mouse IL-7 as their survival factor.

Central hypoventilation syndrome is a rare and fatal condition resulting from various central nervous system disorders that is characterized by a failure of automatic breathing. We report a case of central hypoventilation syndrome following posterior circulation stroke whose pulmonary function was improved by respiratory rehabilitation. A 59-year-old woman with a history of hemorrhagic stroke of the bilateral cerebellum was hospitalized due to pneumonia. A portable ventilator was applied via tracheostomy, recurrent episodes of apnea and hypercapnia impeded weaning. A respiratory rehabilitation program including chest wall range of motion exercise, air stacking exercise, neuromuscular electrical stimulation (NMES) on abdominal muscles, upper extremity ergometer, locomotor training, high-frequency chest wall oscillator, mechanical insufflation, and exsufflation was employed, as spirometry showed a severe restrictive pattern. A spontaneous breathing trial was started, and a portable ventilator was applied for 8 hours, only during nighttime, to prevent sudden apneic event. After 4 weeks of treatment, follow-up spirometry showed much improved respiratory parameters. This case suggests that respiratory rehabilitation can improve pulmonary function parameters and quality of life in central hypoventilation syndrome.
Abdominal Muscles ; Apnea ; Central Nervous System Diseases ; Cerebellum ; Electric Stimulation ; Female ; Follow-Up Studies ; Humans ; Hypercapnia ; Hypoventilation ; Insufflation ; Middle Aged ; Pneumonia ; Quality of Life ; Range of Motion, Articular ; Rehabilitation ; Respiration ; Respiratory Center ; Spirometry ; Stroke ; Thoracic Wall ; Tracheostomy ; Upper Extremity ; Ventilators, Mechanical ; Weaning

STUDY DESIGN: A cross-sectional study. OBJECTIVES: To provide reference data for the study and treatment of thoracic scoliosis. SUMMARY OF LITERATURE REVIEW: There have been no reports on the prevalence of thoracic scoliosis in Korea. MATERIALS AND METHODS: From August 2011 to October 2012, radiographs of patients under 80 years of age who underwent routine chest radiographs were retrospectively reviewed. Based on their age when the chest radiographs were obtained, the patients were divided into 8 groups. The prevalence and angle of the curve of thoracic scoliosis were investigated in each age group, and the prevalence of thoracic scoliosis according to sex, the direction of the curve, number of vertebrae in the major curve, the location and rotation of the apical vertebrae, and osteophyte location were examined. RESULTS: The prevalence of thoracic scoliosis was 2.4% (621 patients), and female patients (3.0%, 375 of 12471) showed a higher prevalence than male patients (1.8%, 246 of 13654) (p<0.001). Right curvature was present in 445 patients and left curvature in 176 patients. In each age group, the prevalence and degree of thoracic scoliosis were 1.1% (14.2°±3.2°), 2.3% (17.4°±7.7°), 2.5% (17.0°±8.9°), 1.9% (15.8°±5.9°), 1.3% (15.5°±6.6°), 2.1% (18.0°±13.6°), 2.9% (14.3°±3.6°), and 6.1% (16.2°±4.8°), respectively. The mean curvature in all scoliosis patients was 16.0°±7.0°. The angle of the curve was significantly different by sex (15.4°±7.1° for males, 16.8°±7.6° for females). The average curve angle of patients with thoracic scoliosis was 16.0°±7.0°, among whom it was 10°–20° in 533 patients, 20°–30° in 64, 30°–40° in 11, and over 40° in 13. CONCLUSIONS: This study could be used as a reference point for the study and treatment of thoracic scoliosis.
Cross-Sectional Studies ; Female ; Humans ; Korea ; Male ; Osteophyte ; Prevalence ; Radiography ; Radiography, Thoracic ; Retrospective Studies ; Scoliosis ; Spine ; Thoracic Vertebrae ; Thorax

OBJECTIVES: To provide reference data for the study and treatment of thoracic scoliosis.SUMMARY OF LITERATURE REVIEW: There have been no reports on the prevalence of thoracic scoliosis in Korea. MATERIALS AND METHODS: From August 2011 to October 2012, radiographs of patients under 80 years of age who underwent routine chest radiographs were retrospectively reviewed. Based on their age when the chest radiographs were obtained, the patients were divided into 8 groups. The prevalence and angle of the curve of thoracic scoliosis were investigated in each age group, and the prevalence of thoracic scoliosis according to sex, the direction of the curve, number of vertebrae in the major curve, the location and rotation of the apical vertebrae, and osteophyte location were examined. RESULTS: The prevalence of thoracic scoliosis was 2.4% (621 patients), and female patients (3.0%, 375 of 12471) showed a higher prevalence than male patients (1.8%, 246 of 13654) (p<0.001). Right curvature was present in 445 patients and left curvature in 176 patients. In each age group, the prevalence and degree of thoracic scoliosis were 1.1% (14.2°±3.2°), 2.3% (17.4°±7.7°), 2.5% (17.0°±8.9°), 1.9% (15.8°±5.9°), 1.3% (15.5°±6.6°), 2.1% (18.0°±13.6°), 2.9% (14.3°±3.6°), and 6.1% (16.2°±4.8°), respectively. The mean curvature in all scoliosis patients was 16.0°±7.0°. The angle of the curve was significantly different by sex (15.4°±7.1° for males, 16.8°±7.6° for females). The average curve angle of patients with thoracic scoliosis was 16.0°±7.0°, among whom it was 10°–20° in 533 patients, 20°–30° in 64, 30°–40° in 11, and over 40° in 13. CONCLUSIONS This study could be used as a reference point for the study and treatment of thoracic scoliosis.

Subependymomas are rare benign tumors located in the ventricular system. Intraparenchymal subependymoma is extremely rare; only 6 cases have been reported, and all were located in the supratentorial region. We describe a case of infratentorial, intraparenchymal subependymoma in a 28-year-old man with intermittent headache. Imaging revealed a well-demarcated cystic and solid cerebellar mass near the fourth ventricle. The mass had a microcystic component and calcification without contrast enhancement. Complete surgical excision was performed, and histopathology confirmed a subependymoma.
Adult ; Calcinosis/diagnosis ; Cerebellar Neoplasms/*diagnosis/surgery ; Fourth Ventricle ; Glioma, Subependymal/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Rare Diseases/*diagnosis/surgery ; Tomography, X-Ray Computed

Recent studies recommend pegylated interferon alpha2a as the first choice treatment for chronic hepatitis B, especially when the patient is negative for HBeAg. Pegylated interferon alpha is known to show variable side effects including skin symptoms, with alopecia as the most common dermatologic side effect experienced for 10% up to 36%. pcc) Most alopecia cases related to pegylated interferon alpha were presented with telogen effluvium pattern, but alopecia areata cases are reported rarely. In this case, typically localized alopecia areata was presented with pegylated interferon alpha2a treatment in hepatitis B patient, and the course of alopecia areata seemed to be related with administration and discontinuation of pegylated interferon alpha2a.
Alopecia Areata* ; Alopecia* ; Hepatitis B ; Hepatitis B e Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Interferon-alpha ; Interferons* ; Skin

Skin hyperpigmentation is one of the most common skin disorders caused by abnormal melanogenesis. The mechanism and key factors at play are not fully understood. Previous reports have indicated that cystamine (CTM) inhibits melanin synthesis, though its molecular mechanism in melanogenesis remains unclear. In the present study, we investigated the effect of CTM on melanin production using ELISA reader and the expression of proteins involved in melanogenesis by Western blotting, and examined the involvement of transglutaminase-2 (Tgase-2) in SK-MEL-2 human melanoma cells by gene silencing. In the results, CTM dose-dependently suppressed melanin production and dendrite extension in alpha-MSH-induced melanogenesis of SK-MEL-2 human melanoma cells. CTM also suppressed alpha-MSH-induced chemotactic migration as well as the expressions of melanogenesis factors TRP-1, TRP-2 and MITF in alpha-MSH-treated SK-MEL-2 cells. Meanwhile, gene silencing of Tgase-2 suppressed dendrite extension and the expressions of TRP-1 and TRP-2 in alpha-MSH-treated SK-MEL-2 cells. Overall, these findings suggested that CTM suppresses alpha-MSH-induced melanogenesis via Tgase-2 inhibition and that therefore, Tgase-2 might be a new target in hyperpigmentation disorder therapy.
Blotting, Western ; Cystamine ; Dendrites ; Enzyme-Linked Immunosorbent Assay ; Gene Silencing ; Humans ; Hyperpigmentation ; Melanins ; Melanoma* ; Skin