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Kim, Sungsu,Kwak, Choong Hwan,Jung, Jaehoon,Baek, Jong Ha,Jung, Jung Hwa,Park, Ki-Jong,Kim, Kyongyoung,Kim, Soo Kyoung,Kang, Dawon,Hahm, Jong Ryeal Hindawi 2018 BioMed research international Vol.2018 No.-
<P>The mechanisms by which hypoglycemia increases cardiovascular mortality remain unclear. The aim of the study is to investigate changes in serum electrolytes, norepinephrine concentrations, electrocardiography, and baroreflex sensitivity (BRS) and associations between corrected QT (QTc) intervals and the changes in serum electrolytes during combined pituitary stimulation test (CPST). We recruited the subjects who were admitted to the Gyeongsang National University Hospital to undergo CPST between September 2013 and December 2014. Participants were 12 patients suspected of having hypopituitarism. Among 12 patients, cardiac arrhythmia in two patients occurred during hypoglycemia. There were significant differences in serum levels of potassium (<I>P</I> < 0.001), sodium (<I>P</I> = 0.003), chloride (<I>P</I> = 0.002), and calcium (<I>P</I> = 0.017) at baseline, hypoglycemia, and 30 and 120 minutes after hypoglycemia. Also, there was a significant increase in heart rate (<I>P</I> = 0.004), corrected QT (QTc) interval (<I>P</I> = 0.008), QRS duration (<I>P</I> = 0.021), and BRS (<I>P</I> = 0.005) at hypoglycemia, compared to other time points during CPST. There was a positive association between QTc intervals and serum sodium levels (<I>P</I> < 0.001) in 10 patients who did not develop arrhythmia during CPST. This study showed that there were significant changes in serum levels of potassium, sodium, chloride, and calcium, as well as heart rate, QTc interval, QRSd, and BRS during CPST. It was revealed that QTc intervals had a significant association with concentrations of sodium.</P>
Kim Minji,Lee Mi-Hee,Hong Soo-Jong,Yu Jinho,Cho Joongbum,서동인,Kim Hyung Young,Kim Hye-Young,Jung Sungsu,Lee Eun,Lee Sooyoung,Jeong Kyunguk,Shim Jung Yeon,Kim Jeong Hee,Chung Hai Lee,Jang Yoon Young,Kwo 대한천식알레르기학회 2023 Allergy, Asthma & Immunology Research Vol.15 No.6
Purpose: Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder that leads to secondary ciliary dysfunction. PCD is a rare disease, and data on it are limited in Korea. This study systematically evaluated the clinical symptoms, diagnostic characteristics, and treatment modalities of pediatric PCD in Korea. Methods: This Korean nationwide, multicenter study, conducted between January 2000 and August 2022, reviewed the medical records of pediatric patients diagnosed with PCD. Prospective studies have been added to determine whether additional genetic testing is warranted in some patients. Results: Overall, 41 patients were diagnosed with PCD in 15 medical institutions. The mean age at diagnosis was 11.8 ± 5.4 years (range: 0.5 months-18.9 years). Most patients (40/41) were born full term, 15 (36.6%) had neonatal respiratory symptoms, and 12 (29.3%) had a history of admission to the neonatal intensive care unit. The most common complaint (58.5%) was chronic nasal symptoms. Thirty-three patients were diagnosed with transmission electron microscopy (TEM) and 12 patients by genetic studies. TEM mostly identified outer dynein arm defects (alone or combined with inner dynein arm defects, n = 17). The genes with the highest mutation rates were DNAH5 (3 cases) and DNAAF1 (3 cases). Rare genotypes (RPGR, HYDIN, NME5) were found as well. Chest computed tomography revealed bronchiectasis in 33 out of 41 patients. Among them, 15 patients had a PrImary CiliAry DyskinesiA Rule score of over 5 points. Conclusions: To our knowledge, this is the first multicenter study to report the clinical characteristics, diagnostic methods, and genotypes of PCD in Korea. These results can be used as basic data for further PCD research.
SoxF Transcription Factors Are Positive Feedback Regulators of VEGF Signaling
Kim, Kangsan,Kim, Il-Kug,Yang, Jee Myung,Lee, Eunhyeong,Koh, Bong Ihn,Song, Sukhyun,Park, Junseong,Lee, Sungsu,Choi, Chulhee,Kim, Jin Woo,Kubota, Yoshiaki,Koh, Gou Young,Kim, Injune Grune & Stratton 2016 Circulation research Vol.119 No.7
<P>Rationale: Vascular endothelial growth factor (VEGF) signaling is a key pathway for angiogenesis and requires highly coordinated regulation. Although the Notch pathway-mediated suppression of excessive VEGF activity via negative feedback is well known, the positive feedback control for augmenting VEGF signaling remains poorly understood. Transcription factor Sox17 is indispensable for angiogenesis, but its association with VEGF signaling is largely unknown. The contribution of other Sox members to angiogenesis also remains to be determined. Objective: To reveal the genetic interaction of Sox7, another Sox member, with Sox17 in developmental angiogenesis and their functional relationship with VEGF signaling. Methods and Results: Sox7 is expressed specifically in endothelial cells and its global and endothelial-specific deletion resulted in embryonic lethality with severely impaired angiogenesis in mice, substantially overlapping with Sox17 in both expression and function. Interestingly, compound heterozygosity for Sox7 and Sox17 phenocopied vascular defects of Sox7 or Sox17 homozygous knockout, indicating that the genetic cooperation of Sox7 and Sox17 is sensitive to their combined gene dosage. VEGF signaling upregulated both Sox7 and Sox17 expression in angiogenesis via mTOR pathway. Furthermore, Sox7 and Sox17 promoted VEGFR2 (VEGF receptor 2) expression in angiogenic vessels, suggesting a positive feedback loop between VEGF signaling and SoxF. Conclusions: Our findings demonstrate that SoxF transcription factors are indispensable players in developmental angiogenesis by acting as positive feedback regulators of VEGF signaling.</P>
The first Vietnamese patient with fulminant type 1 diabetes mellitus.
Kim, Hee Jin,Kim, Ho-Su,Hahm, Jong Ryeal,Jung, Jung Hwa,Kim, Soo Kyoung,Lee, Sang Min,Kim, Sungsu,Chung, Soon Il,Jung, Tae Sik Japanese Society of Internal Medicine 2012 Internal medicine Vol.51 No.17
<P>A 23-year-old pregnant woman had a stillbirth at 30 weeks gestation due to abrupt diabetic ketoacidosis. The patient had a normal HbA1c, severe hyperglycemia, negative islet cell autoantibodies, and very low insulin secretion capacity. The viral markers associated with fulminant type 1 diabetes were negative. The patient's human leukocyte antigen genotypes were DRB1*04:06 and DQB1*03:01/05:02, not common subtypes for fulminant type 1 diabetes. This is the first Vietnamese patient with fulminant type 1 diabetes mellitus.</P>
Case of 36-Year-Old Man with Normokalemic Thyrotoxic Periodic Paralysis
Hee Jin Kim,Tae Sik Jung,Jong Ryeal Hahm,Jung Hwa Jung,Soo Kyoung Kim,Sang Min Lee,Sang Su Lee,Ho Su Kim,Sungsu Kim,Soon Il Chung 대한갑상선학회 2011 International Journal of Thyroidology Vol.4 No.2
Thyrotoxic periodic paralysis is not a rare complication of hyperthyroidism in Asian people, but the cases of paralysis with normal serum potassium levels are very rare. A 36-year-old Korean man who had been diagnosed with Graves’ disease had experienced recurrent paralysis for 9 months in spite of normokalemia. We measured the patient’s serum potassium levels nine times at the time of paralysis. All measurements fell in the range 3.3∼4.7 mmol/L. We treated the patient with methimazole, Lugol’s solution, beta-blockers, and radioactive iodine. Thyroid function was normalized after anti-thyroid treatment and then no further paralytic attacks have occurred.
( Kyong Young Kim ),( Jaehoon Jung ),( Soo Kyoung Kim ),( Bo Ra Kim ),( Sungsu Kim ),( Jung Hwa Jung ),( Jong Ryeal Hahm ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Endocrine diseases, including pituitary and adrenal conditions, are frequently accompanied by diabetes mellitus. Treatment of an underlying endocrine disease often improves insulin resistance and glucose control. The co-occurrence of acromegaly and Cushing syndrome in a single individual is extremely rare. We describe here a patient who showed dramatic improvement in glucose control following treatment for coexisting acromegaly and Cushing syndrome. A 56-year-old woman who underwent evaluation of uncontrolled diabetes mellitus and an incidentally discovered adrenal mass was diagnosed with acromegaly and a unilateral cortisol-producing adrenal adenoma. She showed the typical features of acromegaly and Cushing syndrome, but had never been examined for other endocrine disease. Upon hospitalization, her blood glucose was poorly controlled despite a high dosage of insulin. After adrenalectomy for Cushing syndrome, her insulin dosage decreased by almost half. Insulin treatment was eventually discontinued following the subsequent surgical removal of the pituitary macroadenoma responsible for acromegaly. These findings show the deleterious effects of excess growth hormone and cortisol on glucose metabolism and the importance of clinical suspicion of endocrine diseases in patients with poorly controlled diabetes mellitus.