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강직성 척추염으로 오인된 지연형 척추 골단 이형성증 1예
오일환 ( Il Hwan Oh ),송준석 ( June Seok Song ),임동휘 ( Dong Hwi Rim ),최종욱 ( Jong Wook Choi ),이승훈 ( Seung Hun Lee ),이주현 ( Joo Hyun Lee ),김태환 ( Tae Hwan Kim ) 대한류마티스학회 2011 대한류마티스학회지 Vol.18 No.4
The spondyloepiphyseal dysplasia tarda (SEDT) is a hereditary arthropathy that progressively leads to deformities of small and large joints, irregularities of the end plates of vertebral bodies, which causes joint restriction, short stature, and gait difficulties. The typical radiographic findings of SEDT are generalized platyspondyly and dysplasia of the epiphyses, resulting in premature arthrosis. Clinically SEDT is manifested as a form of short-trunk dwarfism and early arthrosis in the period from late childhood to adolescence. The major clinical importance of this rare disease is similarity to juvenile idiopathic arthritis (JIA), which has a rather different prognosis and treatment. A few cases of SEDT have been published. However, no cases have been reported in South Korea. We describe the case of a 29-year old man who suffered from back and multiple joint pain, who was misdiagnosed as having ankylosing spondylitis. We evaluated the patient clinically and radiographically in greater detail, and changed his diagnosis to SED tarda.
오일환 ( Il Hwan Oh ),김근호 ( Gheun Ho Kim ) 대한내과학회 2014 대한내과학회지 Vol.86 No.6
Hyponatremia results from a relative excess of total body water compared with the sodium content. Except for primary polydipsia, vasopressin activation plays a major role in pathogenesis of water retention. Consequently, the increase of solute-free water clearance by inactivating vasopressin action would be a more reasonable therapeutic approach than the addition of sodium. The V2 vasopressin receptor is mainly localized to the collecting ducts in the kidney and causes water reabsorption via water channels. Selective V2 receptor antagonists or vaptans were recently introduced to clinical practices and may be useful for correcting dilutional hyponatremia. Clinical trials have shown that vaptans are effective in increasing the serum sodium concentration in patients with syndrome of inappropriate anti-diuresis and congestive heart failure and that they might be safe as long as patients are allowed free accesses to water. However, the indications for using vaptans need to be more refined, and the question of their long-term cost-effectiveness should be answered. In addition, the potential roles of vaptans in ameliorating the growth of cysts in polycystic kidney disease, saving diuretics in edematous disorders, and retarding the progression of chronic kidney disease are being explored. (Korean J Med 2013;85:686-694)