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*

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

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*

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

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

BACKGROUND/AIMS: We evaluated the management and educational status of adult anaphylaxis patients at emergency departments (EDs). METHODS: Anaphylaxis patients who visited ED from 2011 to 2013 were enrolled from three hospitals. We analyzed clinical features, prior history of anaphylaxis, management and provided education for etiology and/or prevention. For analyzing associated factors with epinephrine injection, Pearson chi-square test was used by SPSS version 21 (IBM Co.). RESULTS: A total of 194 anaphylaxis patients were enrolled. Ninety-nine patients (51%) visited ED by themselves. Time interval from symptom onset to ED visit was 62 ± 70.5 minutes. Drug (56.2%) was the most frequent cause of anaphylaxis. Forty-seven patients (24.2%) had prior history of anaphylaxis and 33 patients had same suspicious cause with current anaphylaxis. Cutaneous (88.7%) and respiratory (72.7%) symptoms were frequent. Hypotension was presented in 114 patients (58.8%). Mean observation time in ED was 12 ± 25.7 hours and epinephrine was injected in 114 patients (62%). In 68 patients, epinephrine was injected intramuscularly with mean dose of 0.3 ± 0.10 mg. Associated factor with epinephrine injection was hypotension (p = 0.000). Twenty-three patients (13%) were educated about avoidance of suspicious agent. Epinephrine auto-injectors were prescribed only in five patients. Only 34 (19%) and 72 (40%) patients were consulted to allergist at ED and outpatient allergy department respectively. CONCLUSIONS: We suggested that management and education of anaphylaxis were not fully carried out in ED. An education and promotion program on anaphylaxis is needed for medical staff.
Adult* ; Anaphylaxis* ; Education ; Educational Status* ; Emergencies* ; Emergency Medical Services ; Emergency Service, Hospital* ; Epinephrine ; Humans ; Hypersensitivity ; Hypotension ; Medical Staff ; Outpatients ; Patient Care Management

Cutaneous papillomas or acrochorda usually appear after the 4th decade of life in areas with skin folds. Conventional methods of removal are associated with bleeding problems, pain and prolonged sequelae. Thus, acrochorda removal with lasers has gained attention. In this study, we compared the efficacy of two popular laser types with different wavelengths and pulse widths for removal of skin tags. A 60-year-old Korean male noticed skin tags on his neck bilaterally. All tags were treated in a single session, on one side with a picosecond (ps)-domain 532 nm Nd:YAG laser and on the contralateral side with a long-pulsed (LP) 755 nm alexandrite laser. The endpoint for the ps-532 laser was immediate whitening, while that for the LP and quasi-LP (QLP) 755 lasers were visible changes on the surface of the lesion. Antibiotic ointment was applied, dressing was done and clinical photographs were taken. Both lasers effectively removed the skin tags at all settings in a single session without bleeding and with minimal discomfort. Crust formation occurred on both sides with natural shedding within 1 to 2 weeks. Transient erythema lasted longer in the tags treated with the ps-532 laser. At the 5th month of follow-up, residual lesions were detected on the field treated with the ps-532 laser. No persistent side effects such as scarring or postinflammatory hyperpigmentation (PIH) were observed. In conclusion, both the ps-532 nm Nd:YAG and the 755 nm alexandrite lasers ensured safe and effective removal of skin tags in a single session without adverse sequelae.

A 78-year-old male presented with an asymptomatic pinkish multi-nodular mass on his frontal scalp. The lesion had recurred twice after incomplete surgical excision. Initial punch biopsy was diagnosed with cylindroma. He revisited after one year with exophytic enlargement of the mass, and two staged Mohs micrographic surgery identified well-differentiated malignant cylindroma. Histopathology in the lower dermis and periosteal layer showed atypical cells with mitosis and hyperchromatic nucleoli with increased Ki-67 index of 10% to 30%. The postoperative wound was successfully treated with negative wound pressure therapy (NPWT) and secondary intentional healing. We report this case showing malignant transformation of solitary cylindroma, and good result for secondary intention healing using NPWT for postoperative defect.