The purpose of this study was to evaluate the bone age and hand anomaly in Down syndrome patients. We reviewed radiographs in 52 children and youths with Down syndrome, aged from 2 to 23 years. The characteristic findings of hand anomaly were observed in the middle phalanx of the fifth finger, with the following incidence; brachymesophalangia (44%), clinodactyly (48%), and dysmesophalangia (15%). Pseudoepiphysis (21%) was found typically at the distal end of the first metacarpal bone and proximal end of the second metacarpal bone. Up to 8.5 years of age, bone maturation was retarded and thereafter bone age accelarated in advance of chronologic age. These findings may provide valuable information relative to the diagnosis and treatment of hand anomaly in Down syndrome.
Adolescent ; Child ; Diagnosis ; Down Syndrome* ; Fingers ; Hand* ; Humans ; Incidence

PURPOSE: The purpose of this study was to compare hospitalized patients in comprehensive nursing care units and general care units as to satisfaction with nursing care and factors influencing their intent to revisit the hospital METHODS: A cross-sectional study was conducted with 178 patients who had been hospitalized in a comprehensive nursing care unit and a general care unit in one hospital. Participants completed self-report questionnaires. Data were analyzed using SPSS 21.0. RESULTS: There was a significant difference between the comprehensive nursing care unit and general care unit for intent to revisit the hospital (p=.036). Factors influencing intent to revisit the hospital for patients in the comprehensive nursing care unit were 'satisfaction with nursing care' (p<.001) and 'use of additional costs for comprehensive nursing care' (p=.041). The factor influencing intent to revisit hospital for patients in the general care unit was 'satisfaction with nursing care' (p<.001). CONCLUSION: Findings indicate that comprehensive nursing care service in which all care is provided by nursing staff only, without family or private caregivers, increases intent to revisit the hospital. These results can be used as the foundation of reviewing the operation and expansion of comprehensive nursing care service.
Caregivers ; Cross-Sectional Studies ; Humans ; Nursing Care* ; Nursing Staff ; Nursing* ; Patient Satisfaction

We have experienced a case of infantile nephritic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.
Biopsy ; Edema ; Eyelids ; Female ; Humans ; Infant ; Intellectual Disability ; Joint Instability ; Nephrotic Syndrome* ; Proteinuria

We report four cases of hyalinosis cutis et mucosae. They developed hosrseness in their infancy and all had past history of frequent skin infections with accompanying varioliform scars. On physical examination, all the patients had beaded papules along their eyelid margins, hoarseness, varioliform scars and various skin and mucous membrane infiltrations. Pathologic examinations performed on skin infiltrations and eyelid papules revealed diastase resistant periodic acid Schiff positive materials deposited mainly in upper dermis. We have given three of four patients 1g/day of dimethyl sulphoxide from 6 to 12 months with no significant side effects nor remarkable clinical improvement.
Amylases ; Cicatrix ; Dermis ; Dimethyl Sulfoxide ; Eyelids ; Hoarseness ; Humans ; Lipoid Proteinosis of Urbach and Wiethe* ; Mucous Membrane ; Periodic Acid ; Physical Examination ; Skin

Meningioma of unusual age of onset, location, histogenesis and histologic type is reported. The patient, 4 year-old girl, had an intradural spinal meningioma arising from lumbar nerve root with no dural attachement. The meningioma revealed glycogen-rich, clear cell type with extensive and blocky hyalinization of the stroma. The tumor shared common fibrous sheath with attached lumbar nerve, and nerve fibers were scattered within the tumor. Ultrastructurally, the tumor cells had abundant glycogen particles, intermediate filaments and intercellular desmosomes. Hyalinized material revealed large amianthoid collagen fibers.
Child ; Male ; Female ; Humans ; Meningioma

No abstract available.
Alcoholism* ; Primary Health Care*

A 78-year-old male presented with early gastric cancer and a 5.5 cm-sized infrarenal abdominal aortic aneurysm, detected during regular screening. Endovascular aneurysm repair (EVAR) was performed first, followed by laparoscopic distal gastrectomy. After gastrectomy, the patient underwent computed tomography (CT) scan due to persistent fever, which showed increased perigraft fluid collection around the right iliac limb graft. Echocardiography also revealed mitral valve vegetation, consistent with infective endocarditis. Despite intensive antibiotic treatment, the patient had persistent fever and showed fluid extension to the psoas muscle on CT scan. On the 49th post operative day (POD) after EVAR, stent graft explantation and aortic reconstruction with the left superficial femoral vein was performed. Ligation of the right iliac artery for infection control and simultaneous femoro-femoral bypass was also performed. The patient was discharged on the 46th POD after graft removal without any events.
Aged ; Aneurysm ; Aortic Aneurysm, Abdominal ; Blood Vessel Prosthesis ; Echocardiography ; Endocarditis ; Endovascular Procedures ; Extremities ; Femoral Vein* ; Fever ; Gastrectomy ; Humans ; Iliac Artery ; Infection Control ; Ligation ; Male ; Mass Screening ; Mitral Valve ; Prosthesis-Related Infections ; Psoas Muscles ; Stomach Neoplasms ; Tomography, X-Ray Computed ; Transplants

BACKGROUND: Alpha hydroxy acids (AHAs) are known to diminish corneocyte cohesion at the innermost levels of the stratum corneum and have been used in the treatment of various disorders of keratinization. However, their effect on skin barrier function and their irritant potential is not fully understood. OBJECTIVE: Our study was done to evaluate the skin irritancy of AHAs in normal human skin. METHODS: Patches with 1%, 5% and 10% solutions of lactic acid (LA) and glycolic acid (GA) were applied to the volar forearm of 20 healthy volunteers for 24 hours using large Finn chambers with filter paper. Visual scores, erythema (E-) index and transepidermal water loss (TEWL) were measured at 30 min, 24 h and 48 h after removal of the patches. RESULTS: The results are summarized as follows. 1. Visual scores were 0.1+/-0.3 (1%), 0.5+/-0.6 (5%) and 1.1+/-0.8 (10%) at 24 h after removal of LA, and were 0.2+/-0.4 (1%), 0.6+/-0.6 (5%) and 1.0+/-0.7 (10%) at 24 h after removal of GA. They were increased in proportion to the concentrations and there were significant differences in skin responses between the control and each concentration of the solutions. 2. E-indices were 9.1+/-2.1 (control), 8.8+/-1.8 (1%), 9.0+/-2.6 (5%) and 10.5+/-3.9 (10%) at 24 h after removal of LA, and were 9.4+/-1.8 (control), 9.3+/-2.3 (1%), 10.0+/-3.0 (5%) and 11.1+/-3.5 (10%) at 24 h after removal of GA. They were not increased in the patch areas of 1% and 5% solutions in both the LA and GA group, but were significantly increased in the patch areas of 10% solutions in both the LA and GA group. 3. TEWL values were 7.3+/-2.3 (control), 8.3+/-4.0 (1%), 9.8+/-4.5 (5%) and 16.7+/-9.1 (10%) at 24 h after removal of LA, and were 8.1+/-3.2 (control), 7.8+/-3.8 (1%), 8.6+/-3.0 (5%) and 10.9+/-4.1 (10%) at 24 h after removal of GA. They were not increased in the patch areas of 1% LA, 1% GA and 5% LA, but there were high significant differences between the controls and 10% solutions of both LA and GA. CONCLUSION: Visual scores were increased in all concentrations of AHAs tested, but the increase in E-index and TEWL values were not significant or minimal in 1% and 5% solutions of AHAs. These findings suggest that AHAs could be classified as non-corrosive irritants.
Erythema ; Forearm ; Healthy Volunteers ; Humans* ; Hydroxy Acids* ; Irritants ; Lactic Acid ; Skin*

Chronic granulomatous disease (CGD) is one of congenital immunodeficient disease and a rare X-linked or autosomal recessive disease characterized by recurrent life- threatening infections and granuloma formation. We observed clinical features, laboratory findings and genetic subgroups of 33 children who were diagnosed with chronic granulomatous disease in the Department of Pediatrics, Seoul National University Children's Hospital. There were 23 males and 10 females. Activated NBT test of all patients revealed 0% positive cell and mothers of 15 patients had 25%- 75% normal neutrophils in the activated NBT test. According to the result of activated NBT test and family history, the ratio of X-linked and autosomal recessive inheritance was 2:3. There was a significant difference for the age at onset of the first infection in the different genetic subgroups. The X-linked group had the mean onset at 1.98 months of age and autosomal recessive group had a mean onset as late as 3.82 months (p<0.05). The most common type of the first infection was lymphadenopathies (41%) and other infections were skin pustules, fever, perianal abscess, pneumonia and chronic diarrhea. However, the age at diagnosis was not significant in the different genetic subgroups. Lymphadenitis (27%) was the most common infection, and pneumonia, gastrointestinal tract infection, skin infection were also common. The most common infectious agent was Candida sp. (5%) and other microorganisms involved were BCG, coagulase-negative staphylococcus, S. aureus, K/ebsiella pneumoniae, Aspergi/lus sp., and Enterococcus faecium. Chronic condition associated with CGD were hepatomegaly (59%), splenomegaly, and anemia of chronic disease, underweight, and lymphadenopathy. The leukocyte count of patients at diagnosis was within normal limit except in three patients and leukopenia was not observed in any of the patients. The humoral and cellular immunity and complement system were normal, but the level of Ig E in four patients was elevated. Early diagnosis of CGD can be made by suspicion if there are lymphadenitis after BCG vaccination and recurrent pyogenic infections under the first year of age. Though progression in the treatment of CGD, like gene therapy, is concerned, genetic counseling and prenatal diagnosis by carrier detection and molecular genetic analysis is thought to be necessary.
Abscess ; Anemia ; Candida ; Child ; Chronic Disease ; Complement System Proteins ; Diagnosis ; Diarrhea ; Early Diagnosis ; Enterococcus faecium ; Female ; Fever ; Gastrointestinal Tract ; Genetic Counseling ; Genetic Therapy ; Granuloma ; Granulomatous Disease, Chronic* ; Hepatomegaly ; Humans ; Immunity, Cellular ; Korea* ; Leukocyte Count ; Leukopenia ; Lymphadenitis ; Lymphatic Diseases ; Male ; Molecular Biology ; Mothers ; Mycobacterium bovis ; Neutrophils ; Pediatrics ; Pneumonia ; Prenatal Diagnosis ; Seoul ; Skin ; Splenomegaly ; Staphylococcus ; Thinness ; Vaccination ; Wills

No abstract available.
Congenital Hypothyroidism*