Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder usually showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. However, in some reported cases, Birbeck granules could not be demonstrated and only cytoplasmic dense bodies were seen. If the lesion is more advanced, Birbeck granules are transformed to lysosomes, i.e., 'unique phagosomes', in which they are degraded. A 2-month-old Korean girl presented with congenital, numerous red-brown pigmented papules on the left side of trunk and upper extremity without systemic symptoms. A biopsy specimen demonstrated papillary dermis containing epidermotropic infiltrates of histiocytes with abundant eosinophilic cytoplasm. Some had kidney-shaped nuclei and PAS-positive cytoplasmic inclusions. Immunohistochemically, infiltrating cells expressed S-100 protein and ultrastructurally, no Birbeck granules but many dense laminated bodies and unique phagosomes were found. It was ten months since the skin lesions developed that they have started resolving.
Biopsy ; Cytoplasm ; Dermis ; Diagnosis ; Eosinophils ; Female ; Histiocytes ; Humans ; Inclusion Bodies ; Infant ; Lysosomes ; Microscopy, Electron ; Phagosomes ; S100 Proteins ; Skin ; Upper Extremity

sodium hyaluronate in beagle dogs. METHODS:In group 1, hGH(Eutropin, r-hGH) 0.29mg/kg was injected subcutaneously to 6 beagle dogs everyday for 7 days. In group 2, 1mg/kg in sustained- release formulation using sodium hyaluronate(SR-hGH), was injected subcutaneously to 6 beagle dogs. In group 3, 2mg/kg of the same formulation(SR-hGH) was injected subcutaneously to 6 beagle dogs. Blood samplings were done for the measurement of GH and IGF-1 concentrations with ELISA kit(Diagnostic Systems laboratories, Inc., USA) RESULTS:GH concentration in group 1 was below 0.5ng/ml before injection and elevated to 98.1+/-15.7 at 1 hr, 124.2+/-15 at 2 hr, 57.8+/-18.1 at 4 hr, 23.8+/-4.8 at 6hr, 10.8+/-3.7 at 8 hr, 2.8+/-1.6 at 10 hr, 1.0+/-0.7 at 12 hr, and 0.5+/-0.1ng/ml at 24hr after injection. Peak GH concentration was observed in 2 hr and thereafter decreased progressively and returned to basal level at 10 hr after injection. From the 2nd day GH concentration was measured only at 6 hr after daily GH injection, indicating the values of 20.9+/-8.7, 16.2+/-14.9, 23.1+/-8.5, 34.3+/-9.9, 16.1+/-7.0, and 21.8+/-13.0ng/ml at 2nd, 3rd, 4th, 5th 6th, and 7th day, respectively. GH concentrations in group 2(SR- hGH 1mg/kg) were 136.7+/-22.8 at 1hr, 149.3+/-29.9 at 2hr, 110.6+/-17.8 at 4hr, 103.7+/-18.2 at 6hr, 108.3+/-21.0 at 8hr, 91.4+/-21.4 at 10hr, 79.6+/-15.9 at 12hr, 23.7+/-8.3 at 24hr, 5.5+/-1.5 at 30hr, 0.7+/-0.2 at 48hr, 1.4+/-1.4 at 54hr, and 0.5+/-0.1ng/ml at 72hr after injection. GH concentration was elevated above the basal level for 72hr with the peak at 2hr after injection of SR-hGH of 1mg/kg. GH concentrations in group 3(SR-hGH 2.0mg/kg) were 196.7+/-45.2 at 1hr, 219.4+/-39.8 at 2hr, 198.1+/-38.0 at 4hr, 196.0+/-31.4 at 6hr, 179.2+/-28.3 at 8hr, 151.8+/-19.5 at 10hr, 141.3+/-23.1 at 12hr, 72.9+/-14.7 at 24hr, 43.7+/-14.2 at 30hr, 3.8+/-1.6 at 48hr, 1.6+/-0.5 at 54hr, 0.8+/-0.5 at 72hr, 0.5+/-0.1 at 78hr, and 0.5+/-0.2ng/ml at 120hr. Peak GH concentration occurred at 2hr after injection and remained high concentration till 72hr and returned to basal level thereafter. IGF-1 concentrations in group 1 changed from 190.5+/-68.1ng/ml before injection, to 326.4+/-96.2, 346.4+/-79, 391.4+/-86.9, 417.0+/-96.1, 422.1+/-92.0, 429.9+/-86.4, and 478.0+/-90.2ng/ml at 12hr, 30hr, 54hr, 78hr, 102hr, 126hr, and 150hr, respectively. IGF-1 concentrations in group 2 were 128.5+/-37.0 ng/ml before and 268.0+/-64.2, 307.6+/-63.1, 374.8+/-55.3, 335.5+/-39.4, 301.9+/-44.8, 288.5+/-42.5, 272.8+/-51.8, 273.9+/-46.0, 251.1+/-40.9, and 239.2+/-45.0ng/ml at 24hr, 30hr, 48hr, 54hr, 72hr, 78hr, 96hr, 102hr, 126hr, and 150hr, respectively after injection. Peak IGF-1 concentration was measured at 48hr and remained in high concentration till 150hr after injection. IGF-1 concentrations in group 3 were 116.0+/-68.9ng/ml before and 365.5+/-118.6, 400.0+/-135.1, 463.6+/-138, 450.2+/-140.0, 337.2+/-122.4, 301.4+/-113.4, 236.3+/-89.1, 226.3+/-75.5, 148.9+/-55.2, and 129.8 48.4ng/ml at 24hr, 30hr, 48hr, 54hr, 72hr, 78hr, 96hr, 102hr, 126hr, and 150hr, respectively after injection. Peak IGF-1 concentration was at 48hr and remained in high concentration till 150 hr after injection. There was no significant difference in IGF-I conc between group I and group 3. CONCLUSION: Sustained-release form(1mg or 2mg/kg) of hGH with sodium hyaluronate released GH for 72 hours with the peak level at 2 hours and higher concentration of IGF-I above baseline maintained for 150 hour after injection with peak level at 48 hour. There was no difference in IGF-1 concentration between SR-hGH 1mg/kg and 2mg/kg injection. So sustained release form 1mg/kg will be more effective for GH therapy as weekly injection mode. More extensive study is needed to permit for new therapeutic application.
Animals ; Dogs* ; Drug Delivery Systems ; Enzyme-Linked Immunosorbent Assay ; Growth Hormone* ; Hyaluronic Acid ; Insulin-Like Growth Factor I ; Sodium

BACKGROUND: Chronic actinic dermatitis comprises a spectrum of chronic photosensitivity disorders. Treatment includes avoidance of UV light, application of broad-spectrum topical sunscreens, PUVA therapy, corticosteroid, azathioprine and cyclosporine. OBJECTIVE: Our purpose was to determine the efficacy of cyclosporine in the treatment of chronic actinic dermatitis. METHODS: Six patients with chronic actinic dermatitis refractory to conventional treatment were treated with cyclosporine 100-200mg a day for four to eighteen weeks. RESULTS: In all six patients improvement of the skin lesions and itching were dramatic, but in three of them hyperterision developed during the cyclosporine treatment. After stopping the cyclosporine therapy, their blood pressures normalized within two to five weeks. Other side effects of cyclosporine were not found. Although the skin lesions of all of the six patients were aggravated more or less after stopping the cyclosporine therapy, we could maintain their improved states with topical corticosteroids and oral antihistamines. CONCLUSION: 1. Cyclosporine is a good alternative in treating chronic actinic dermatitis patients who are suffering from severe symptoms refractory to conventional therapy. 2. Hypertension is the frequent side effect of cyclosporine.
Adrenal Cortex Hormones ; Azathioprine ; Cyclosporine* ; Histamine Antagonists ; Humans ; Hypertension ; Photosensitivity Disorders* ; Pruritus ; PUVA Therapy ; Skin ; Sunscreening Agents ; Ultraviolet Rays

PURPOSE: To investigate the fundus examination rate in diabetic patients and the public health factors associated with the fundus examination rate. METHODS: We analyzed the results of the third Korean National Health and Nutrition Examination Survey. We investigated the fundus examination rate in patients who had diabetes mellitus for more than 3 months during the previous year and determined the relationship of public health factors with the fundus examination rate. RESULTS: In all 547 patients (38.1%) received the fundus examination out of 1,437 total patients with diabetes mellitus. We found that many public health factors influenced the higher fundus examination rate, including patients who were residents in urban areas, women, office workers, university graduates, patients with a higher family income or more than 5 years duration of diabetes mellitus, patients with current insulin treatment, treatment of diabetes mellitus, education about diabetes mellitus, subjective visual problems or subjective health problems. However, age, marital status, health insurance and residential district did not influence the fundus examination rate. CONCLUSIONS: The Korean National Health and Nutrition Examination Survey is the largest health survey in Korea. This study is meaningful as a population-based study, not a clinic-based cross-sectional study and it suggests the basic data for improving the fundus examination rate in diabetes mellitus patients.
Diabetes Mellitus ; Female ; Health Surveys ; Humans ; Insulin ; Insurance, Health ; Korea ; Marital Status ; Nutrition Surveys ; Public Health

OBJECTIVES: To evaluate the urinary concentrations of hydroxyridinium crosslinks of collagen in patients with osteoarthritis(OA) or rheumatoid arthritis(RA), and to compare its clinical correlation with the classic indices of the disease activity of RA. METHODS: Concentrations of urinary pyridinoline (Pyd) and deoxypyridinoline(Dpd) were measured in urinary samples collected from 18 control patients, 35 patients with OA, 45 patients with RA by competitive enzyme immunoassay using microplate coated with monoclonal antibody. RESULTS: 1) Mean urinary concentrations of Pyd in OA patients were 33.5nmol/mmol creatinine, in RA patients were 50.0nmol/mmol creatinine which were higher than the values in controls (25.1 nmol/mmol creatinine). Also, mean concentrations of Dpd in OA patients were 9.2nmol/mmol creatinine, in RA patients were 10.1nmol/mmol creatinine which were higher than the values in controls(5.6nmol/mmol creatinine)(p<0.01). 2) Mean urinary concentration of Pyd was 50.0 nmol/mmol creatinine in RA patients, which was significantly higher than the values in OA(33.5 nnmol/mmol creatinine)(p<0.05), but the mean Dpd concentratians were not significantly different between the two groups. 3) The concentrations of urinary Pyd in RA patients was significantly correlated with the biologic markers indicating inflammatory activity such as ESR(r=0.68, p<0.001), CRP(r=0.72, p<0.001) and the number of tender joint(r=0.66, p<0.01) CONCLUSION: Urinary concentrations of Pyd and Dpd were significantly higher in OA and RA patients than in controls, Especially urinary Pyd concentrations were significantly increased in RA patients than in OA patients, and strongly correlated with disease activity index of rheumatoid arthritis. The mean Dpd concentration, bone specific analogue, in RA patients was not significantly different from that of OA patients and it was not correlated with disease activity index Thus measurement of urinary Pyd might provide a sensitive, noninvasive biochemical marker for studying activity of RA.
Arthritis, Rheumatoid ; Biomarkers ; Collagen ; Creatinine ; Humans ; Immunoenzyme Techniques ; Osteoarthritis

No abstract available.
DNA* ; Humans* ; Papilloma* ; Respiratory System*

A clinico-pathologic study with an immunohistochemical examination for c-erbB-2 expression in 54 cases of ductal carcinoma in situ and 16 cases of Paget's disease of the breast was performed. c-erbB-2 oncoprotein overexpression was observed in 45% (24/54) and 88% (14/16) of ductal carcinoma in situ and Paget's disease, respectively. The overexpression of c-erbB-2 oncoprotein was significantly correlated with the nuclear grade of tumors and inversely with the status of the estrogen receptor. c-erbB-2 was positive in 4 out of 5 patients with metastasis to axillary lymph nodes and 3 out of 4 patients who died of the disease. Prognostic significance of c-erbB-2 oncoprotein in ductal carcinoma in situ was highly suggested. The expression of c-erbB-2 oncoprotein in Paget's disease was well correlated with coexisting infiltrating or in situ ductal carcinoma. The high positive rate of c-erbB-2 oncoprotein in ductal carcinoma with Paget's disease could be understood with a recent hypothesis that c-erbB-2 oncoprotein is involved in promotion of cell motility and the spread of carcinoma cells.
Neoplasm Metastasis

No abstract available.
Heart Valve Prosthesis* ; Heart Valves*

No abstract available.
Esophageal Atresia* ; Tracheoesophageal Fistula*

BACKGROUND: Laryngomicrosurgery has some special characteristics. It is stressful due to intubation and direct laryngoscopy during a short operation time. Therefore both adequate anesthesia and quick recovery for the slience therapy after the operation are needed. This study compared the cardiovascular responses and recovery pattern between propofol and Thiopental-Enflurane anesthesia. METHODS: Sixty outpatients of ASA class 1 or 2 for microlaryngoscopy were randomly assigned to receive either anesthesia with propofol (Group P, n = 30) and thiopental-enflurane (Group E, n = 30). Group P was induced with propofol 2 mg/kg and succinylcholine 1 mg/kg and maintained with vecuronium 0.04 mg/kg, propofol 10 - 6 mg/kg/h, and N2:O2/3 L/min:2 L/min. Group E was induced with thiopental 5 mg/kg and succinylcholine 1 mg/kg and maintained with vecuronium 0.04 mg/kg, enflurane 1 3 vol%, and N2O:O2/3 L/min:2 L/min. Ketorolac (30 mg) and hydrocortisone (100 mg) were added for postoperative pain in both groups. The changes in blood pressure and heart rate, pre and post induction, were compared in both groups. In addition, we compared energence time and the state of recovery (Steward's score) 5 minutes and 15 minutes after extubation and the frequencies of other complications. RESULTS: No significant differences in age, wt, sex and anesthesia time of the two groups were observed. Mean arterial pressures were significantly different after anesthesia and after intubation between the two groups. However the heart rates were not different among the groups. The extubation time was significantly shorter in Group P. The recovery score at 5 min and 15 min after extubation was significantly higher in Group P. CONCLUSIONS: We conclude that propofol with nitrous oxide may be useful in laryngeal microsurgery, especially, when silence therapy is needed.
Anesthesia* ; Arterial Pressure ; Blood Pressure ; Enflurane ; Heart Rate ; Humans ; Hydrocortisone ; Intubation ; Ketorolac ; Laryngoscopy ; Microsurgery ; Nitrous Oxide ; Outpatients ; Pain, Postoperative ; Propofol* ; Succinylcholine ; Thiopental ; Vecuronium Bromide