Freeman-Sheldon Syndrome is one of the very rare genetic diseases which primarily affects face, hands and feet. At first, Freeman and Sheldon described this syndrome as cranio-carpo-tarsal dystrophy in 1938 and later Burian, as "whistling face" syndrome in 1963. There were 60 cases of reports in the world up to now, and only one paper with 5 cases in a family was reported in Korea. The authors report 2 cases of Freeman-Sheldon Syndrome associated with bilateral inguinal hernias and undescended tests in a family, briefly review the literature and alert orthopaedic surgeons to this condition.
Cryptorchidism ; Foot ; Hand ; Hernia, Inguinal ; Humans ; Korea ; Male ; Surgeons

No abstract available.
Child* ; Humans ; Infant* ; Urinary Tract Infections* ; Urinary Tract*

We experienced a case of adenocarcinoma arising in sacrococcygeal teratoma. The patient was a 52-year-old woman. She was admitted due to one month of sacral pain. She had a sacral mass since birth. On physical examination, anal fistula was present at the perianal area and pus drainage was noted. MR image showed multiple variable-sized cysts with inhomogeneous density. Resected specimen, mesuring 12.5 7.0 cm in diameter, showed multiple variable-sized cystic lesions admixed with grayish solid portion. The cysts contained mucoid material. The microscopic examination showed mature teratoma composed of cysts lined by pseudostratified ciliated columnar epithelium, intestinal mucosa, mature cartilage, bone, and fat tissue. A moderately differentiated adenocarcinoma developed from the cystic area in the mass.
Adenocarcinoma* ; Adult ; Cartilage ; Drainage ; Female ; Humans ; Intestinal Mucosa ; Middle Aged ; Mucous Membrane ; Parturition ; Physical Examination ; Rectal Fistula ; Suppuration ; Teratoma*

PURPOSE: This study aimed to explore the constituents and structure of adaptation experiences in their everyday life among senior patients of a nursing home in order to provide nursing intervention data for the satisfaction and the improvement of life of senior patients by appreciating the nature of their adaptational experience. METHODS: The participants were five female and one male senior patients who were 65-years old or older and admitted at a nursing home in a metropolitan city. The interview data were analyzed by the Giorgi's phenomenological analysis method. RESULTS: As the results of analysis, the following three constituents have been found out: retrospective focus based on the meaning of admission, expanding a view and facing up to the reality, reconstructing views about the meaning of life and the world. CONCLUSION: With the increasing number of senior in facilities, nurses not only play a key role in caring for seniors but also in managing their maladaptation. Thus, it is considered that the results obtained from the this study provide valuable information for both the senior patients and their families as well as for the nurses, by presenting the data about senior patients' adaptational experiences of nursing home admission.
Aged ; Female ; Humans ; Male ; Nursing Homes* ; Nursing* ; Retrospective Studies

Thin basement membrane nephropathy(TBMN) is defined histologically as follows: 1) By light rnicroscopy only minor abnormalities are detected in the glomeruli at most minor mesangial widening. 2) By electron microscopy, diffuse thinning of glomerular basement rnembrane is demonstrated. 3) By immunofluorescence, absence of immunoglobulins and complement components is demonstrated. 4) Alport's syndrome and systemic diseases that may affect the glomerular structure have been excluded. TBMN presented frequently with recurrent or persistent microscopic hematuria. Massive proteinuria such as in nephrotic syndrome rarely occurs in TBMN. We reported two cases of TBMN presented with typical minimal change nephrotic syndrome.
Basement Membrane* ; Complement System Proteins ; Fluorescent Antibody Technique ; Hematuria ; Immunoglobulins ; Microscopy, Electron ; Nephritis, Hereditary ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Proteinuria

Restenosis and stent thrombosis remain major concerns after percutaneous coronary intervention for the treatment of coronary artery disease. The present review was undertaken in order to highlight the various coronary stents that have been investigated in our Heart Research Center, and how far we have come from the first heparin-coated stent first used in the late 1990s. Thereafter, from the abciximab-coated stent to the current gene-delivery stent and other newer agents, our group has applied a range of techniques in this field. However, in groups similar to ours, the restenosis rates of such stents are still high for second-generation drug-eluting stents (DESs). Moreover, our nation imports almost all of these types of stents from other countries. Thus, we need to develop domestic coronary stents. Research into newer DESs are warranted in Korea so as to achieve improved safety and efficacy outcomes.
Coronary Artery Disease ; Heart ; Korea ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Stents ; Thrombosis

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by recurrent abscesses, nodules, and fistulous tracts. Recently, novel staging systems, including sonographic scoring system of hidradenitis suppurativa (SOS-HS), have been widely introduced in HS. Objective: To assess the efficacy of SOS-HS compared with Hurley staging and modified sartorius scoring (MSS) system and determine the necessity of ultrasound evaluation. Methods: A radiologist performed an ultrasound evaluation, and three dermatologists reviewed the images and videos. Clinical characteristics, including staging and demographic data, were recorded by three dermatologists. The correlation of each staging system and the significance of vascularity (Doppler mode) and lesion depth on sonography were statistically analyzed. Results: Among the 46 patients, 34.8% were classified as stage 1 and 3, and 30.4% were classified as stage 2 in the SOS-HS system, which was statistically higher than the Hurley staging (p＜0.0005). MSS score increased according to the SOS-HS stages (p＜0.0005, p=0.001). Vascularity and depth of involvement also increased with SOS-HS and Hurley staging. Conclusion Sonographic examination can be applied in HS severity assessment and documentation of the clinical characteristics of each lesion in detail. Special tools, such as Doppler mode and depth assessment, can also be useful in HS evaluation.

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by recurrent abscesses, nodules, and fistulous tracts. Recently, novel staging systems, including sonographic scoring system of hidradenitis suppurativa (SOS-HS), have been widely introduced in HS. Objective: To assess the efficacy of SOS-HS compared with Hurley staging and modified sartorius scoring (MSS) system and determine the necessity of ultrasound evaluation. Methods: A radiologist performed an ultrasound evaluation, and three dermatologists reviewed the images and videos. Clinical characteristics, including staging and demographic data, were recorded by three dermatologists. The correlation of each staging system and the significance of vascularity (Doppler mode) and lesion depth on sonography were statistically analyzed. Results: Among the 46 patients, 34.8% were classified as stage 1 and 3, and 30.4% were classified as stage 2 in the SOS-HS system, which was statistically higher than the Hurley staging (p＜0.0005). MSS score increased according to the SOS-HS stages (p＜0.0005, p=0.001). Vascularity and depth of involvement also increased with SOS-HS and Hurley staging. Conclusion Sonographic examination can be applied in HS severity assessment and documentation of the clinical characteristics of each lesion in detail. Special tools, such as Doppler mode and depth assessment, can also be useful in HS evaluation.