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Townes-Brocks Syndrome Associated with Hypothyroidism in a Korean Newborn
Shin Young Park,Woo Ryoung Lee 대한의학유전학회 2008 대한의학유전학회지 Vol.5 No.2
Townes-Brocks 증후군은 이형성 귀, 청력 상실, preaxial polydactyly, triphalangeal thumbs, 쇄항, 신기형, 선천성 심질환, 정신 지체 등을 동반하는 다기형의 상염색체 우성 유전질환이다. 그러나 갑상선 기능저하증은 Townes-Brocks 증후군의 흔한 특징은 아니다. 현재까지 갑상선 기능저하증과 동반된 Townes-Brocks 증후군은 3례가 보고된 바 있다. 저자들은 한국에서 최초로 갑상선 기능저하증과 동반된 Townes-Brocks 증후군 1례를 보고한다. Townes-Brocks syndrome (TBS) is an autosomal dominant disorder with multiple malformations which include dysplastic ears, hearing loss, preaxial polydactyly and/or triphalangeal thumbs, imperforate anus, renal anomalies, congenital heart defects, and mental retardation. However, hypothyroidism is not a common feature of TBS. There have been only three reported cases of TBS associated with hypothyroidism. We report the first case of TBS associated with hypothyroidism in Korea.
Conserved Virulence Factors of Pseudomonas aeruginosa are Required for Killing Bacillus subtilis
Shin-Young Park,Yun-Jeong Heo,Young-Seok Choi,Eric Deziel,조유희 한국미생물학회 2005 The journal of microbiology Vol.43 No.5
The multi-host pathogen, Pseudomonas aeruginosa, possesses an extraordinary versatility which makes it capable of surviving the adverse conditions provided by environmental, host, and, presumably, competing microbial factors in its natural habitats. Here, we investigated the P. aeruginosa-Bacillus subtilis interaction in laboratory conditions and found that some P. aeruginosa strains can outcompete B. subtilis in mixed planktonic cultures. This is accompanied by the loss of B. subtilis viability. The bactericidal activity of P. aeruginosa is measured on B. subtilis plate cultures. The bactericidal activity is attenuated in pqsA, mvfR, lasR, pilB, gacA, dsbA, rpoS, and phnAB mutants. These results suggest that P. aeruginosa utilizes a subset of conserved virulence pathways in order to survive the conditions provided by its bacterial neighbors.
Extended distal pancreatectomy for advanced pancreatic neck cancer
Shin-Young Park,Woo Young Shin,Yun-Mee Choe,Keon-Young Lee,Seung-Ik Ahn 한국간담췌외과학회 2014 한국간담췌외과학회지 Vol.18 No.3
Backgrounds/Aims: We investigated the clinical application of extended distal pancreatectomy in patients with pancreatic neck cancer accompanied by distal pancreatic atrophy. In this study, we have emphasized on the technical aspects of using the linear stapling device for a bulky target organ. Methods: From March 2010 to September 2013, 46 patients with pancreatic adenocarcinoma, who underwent pancreatic resection with radical intent at our institute, were reviewed retrospectively. Among them, three patients (6.5%) underwent extended distal pancreatectomy. A linear stapling device and vise-grip locking pliers were used for en bloc resection of the distal pancreas, first duodenal portion, and distal common bile duct. The results were compared with those after standard pancreatectomy. Results: All three patients presented with jaundice, and the ratio of pancreatic duct to parenchymal thickness of the pancreatic body was greater than 0.5. Grade A pancreatic fistula developed in all of the cases, but none of these fistulae were lethal. Pathological staging was T3N1M0 in all of the patients. The postoperative daily serum glucose fluctuations and insulin requirements were comparable to those in patients who received pancreaticoduodenectomy or distal pancreatectomy. At the last follow-up, two patients were alive with liver metastasis at 4 and 10 months postoperatively, respectively, and one patient died of liver metastasis at 5 months postoperatively. Conclusions: While the prognosis of advanced pancreatic neck adenocarcinoma is still dismal, extended distal pancreatectomy is a valid treatment option, especially when there is atrophy of the distal pancreas. Also, the procedure is technically feasible, and further refinement is necessary to improve patient survival.