The May-Hegglin anomaly is a rare autosomal dominant trait characterized by platelet abnormalities in the peripheral blood and large (up to 5mu) pale blue staining inclusions in the cytoplasm of neutrophils, eosinophils, basophils, and monocytes. We experienced a case of May-Hegglin anomaly in a 7 year old male and another case out of his family. His initial complaints at the admission were petechiae and intermittent epistaxis. Diagnosis was confirmed by peripheral blood smear and family study. We report the case with brief review of related literature.
Basophils ; Blood Platelets ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Epistaxis ; Humans ; Male ; Monocytes ; Neutrophils ; Purpura

No abstract available.

Strumal carcinoma of the ovary is rare tumor of germ cell origin characterized by intimate mixture of thyroid tissue and carcinoid. We report a strumal carcinoid tumor associated with ipsilateral follicular cyst and contralateral mature cystic teratoma of the ovary in a 47-year-old woman. Histologically it was composed largely of trabeculae partly of insular carcinoid and focally of individual thyroid follicles. Many cells in the carcinoid areas and even some follicular areas contained abundant argyrophile granules, and they also, in the corresponding areas, demonstrated thyroglobulin. Electron microscopic findings revealed abundant neurosecretory granules and colloid material in the same cell. We agree that this tumor is derived from hybrid showing thyroid and neuroendocrine differenciation.
Female ; Humans ; Cysts

There are frequently limitations to general wound dressing in some cases. Dermabond(R) has been available as a skin closure alternative. The purpose of this study was to apply this topical skin adhesive in postoperative wound management. Dermabond(R) was used for postoperative dressing in total 62 cases; group A includes wounds of the perineum & anus, which have greater chance of contamination(N=16), group B includes wounds located in hairy areas, which are difficult to cover up (N=21), group C includes wounds in children or bed-ridden patients, who have poor compliance (N=6), and group D includes wounds in patients who underwent free flap operations(N=19). There were no infections in group A by separating the wounds from infection sources. Dressings in group B were done much more simply and comfortable. There was increased compliance of patients in group C, as patients could wash around the wound. It was possible to carry out real time monitoring in group D as the simply coverage of the wound. Using Dermabond(R) after primary closure was found to be efficient in the management of wounds near areas with greater chance of being contaminated, wounds which are hard to cover up with dressing materials, wounds in patients with poor compliance, and wounds requiring frequent observation.
Child ; Male ; Female ; Humans

PURPOSE: To evaluate the accuracy of preoperative keratometers used in cataract surgery with toric intraocular lens (IOL). METHODS: Twenty-five eyes received an AcrySof toric IOL implantation. Four different keratometric methods, a manual keratometer, an IOL master, a Pentacam and an auto keratometer, were performed preoperatively in order to evaluate preexisting corneal astigmatism. Differences between the true residual astigmatism and the anticipated residual astigmatism (keratometric error) were compared at one and three months after surgery by using a separate vector analysis to identify the keratometric method that provided the highest accuracy for astigmatism control. RESULTS: The mean keratomeric error was 0.52 diopters (0.17-1.17) for the manual keratometer, 0.62 (0-1.31) for the IOL master, 0.69 (0.08-1.92) for the Pentacam, and 0.59 (0.08-0.94) for the auto keratometer. The manual keratometer was the most accurate, although there was no significant difference between the keratometers (p > 0.05). All of the keratometers achieved an average keratometric error of less than one diopter. CONCLUSIONS: Manual keratometry was the most accurate of the four methods evaluated, although the other techniques were equally satisfactory in determining corneal astigmatism.
Analysis of Variance ; Astigmatism/complications/*surgery ; *Cataract Extraction ; Female ; Humans ; Lens Implantation, Intraocular/*methods ; *Lenses, Intraocular ; Magnetic Resonance Imaging ; Male ; Prospective Studies ; Refraction, Ocular ; Reproducibility of Results ; Treatment Outcome ; Visual Acuity

Richer's syndrome is a development of a high grade malignant lymphoma in a patient with preexisting chronic 1ymphocytic leukemia, small lymphocytic lymphoma or Waldenstrom's macroglobulinemia. A rare case of Richer's syndrome arising in the spleen of a 35-year-old-man was studied by morphology, immunohistochemistry and gene rearrangement study. He has had weight loss and night sweat for last 6 months. Hepatosplenomegaly and abdominal lymphadenopathy were noted on CT scanning. Especially an ovoid radiolucent mass was found within the image of splenomegaly. Lymph nodes and liver biopsy, bone marrow aspiration and splenectomy were done. In the lymph nodes, liver and bone marrow, well differentiated small lymphocytic infiltrations were found but, in the spleen, pleomorphic, large cells with occasional multinucleated giant cells formed a nodular mass surrounded by diffuse, extensive infiltration of small well differentiated lymphocytes. The two distinctive areas in the spleen had positive staining for B-cell marker (HLA-DR and L26), negative staining for T-cell marker (UCLH1), and positive staining for IgM heavy chain and kappa light chain by immuohistochemical study. so this case was diagnosed as a diffuse large cell 1ymphoma transformed from small lymphocytic lymphoma. We made an another effort to clarify their clonality. Gene rearrangement method usingcomplementarity.determining region 3(CDR3) of immunoglobulin heavy chain (IgH) gene and T-cell receptor gamma (TCRgamma) gene by polymerase chain reaction (PCR) technique was done. The two lymphomas in the spleen demonstrated the same rearrangement pattern in both IgH and TCRgamma gene. We think these findings strongly suggest that the large cell lymphoma has the same clonality with that of the small lymphocytic lymphoma.
Male ; Humans

Endothelin (ET) is a potent vasoconstrictive 21-amino acid peptide hormone released from vascular endothelium The effects of ET-1 on coronary and systemic hemodynamics in comparison with Bay K 8644, a Ca2+ agonist, were studied in halothane-anesthetized dogs. The modification of ET-1 effects by nicardipine, a voltage-dependent Ca2+ antagonist, was also investigated. Single bolus ET-1 (100 ng/ kg) and Bay K 8644 (30 ug) were administered consecutively into left circumflex coronary artery during intracoronary infusion of either 0.9% saline (0.5 ml/kg/h, n=11) or nicardipine (1 ug/kg/min, n=10). Coronary and systemic hemodynamic parameters were measured just prior to (baseline), during saline or nicardipine infusion and 1, 5, 10, 20, 30, 45, and 60 min after ET-1 injection. Also electrocardiographic changes were observed continuously. The results are as follows: 1) Both ET-1 and Ray K 8644 produced a marked and immediate reduction in coronary blood flow and an increase in coronary vascular resistance. 2) ET-1 evoked coronary vasoconstrictions were long-lasting as compared with transient actions of Bay K 8644. 3) ET-1 reduced peak systolic intramyocardial pressure (IMP), mean aortic pressure (MAP), and cardiac index (CI), in contrast Bay K 8644 increased IMP without any changes in MAP and CL 4) Nicardipine (1 ug/kg/min, i.c) produced a significant increase (2-fold) in coronary blood flow and a reduction (46%) in coronary vascular resistance, whereas other hemodynamic parameters remained unchanged. 5) Nicardipine partially attenuated coronary vascular and systemic effects of ET-1, but it completely prevented those of Bay K 8644. 6) ET-1 (100 ng/kg, i.c.) produced a significant ST segment elevation in electrocardiogram in all cases of the saline group, but in none of the nicardipine group. These findings suggest that ET-1 is a potent and long-lasting coronary vasoconstrictor and that its vasoconstrictive effect is mediated in part by promoting Ca2+ influx through a voltage-dependant Ca2+ channel since nicardipine only partially attenuated ET-1 induced cardiovascular effects.
3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester ; Animals ; Arterial Pressure ; Coronary Vessels ; Dogs* ; Electrocardiography ; Endothelin-1* ; Endothelins ; Endothelium, Vascular ; Hemodynamics* ; Nicardipine* ; Vascular Resistance ; Vasoconstriction

PURPOSE: It is well known that a high proportion of patients with cerebral palsy have neurogenic bladder. However, cystometry performed to determine the presence and type of neurogenic bladder is an invasive procedure. We evaluated the validity of uroflowmetry and postvoid residual urine volume as a screening tool for neurogenic bladder in children with cerebral palsy. MATERIALS AND METHODS: 34 children with cerebral palsy (range 6-13 years) were randomly selected for this study. Uroflowmetry and residual urine volume were deter mined at least twice in 22 children. Uroflow curve pattern was classified into normal, tower, plateau and staccato type. All patients underwent cystometry with sphincter elec tromyography. We analyzed relationship between the results of uroflowmetry and cystometry. RESULTS: Of the 34 patients, 23 (67.6%) children displayed upper motor neuron lesion of bladder. 11 (32.4%) had uninhibited contraction, 9 (26.5%) small capacity and con comitant detrusor-external sphincter dyssnergia was found in 3 (8.8%). Of 22 children who performed uroflowmetry, 13 showed abnormality. Staccato, tower, and plateau type of pattern was seen 11 (84.6%), 1 (7.7%), and 1 (7.7%) patient, respectively. The re maining 9 (40.9%) children were normal. Eleven of the 13 children with abnormal uroflow curve on uroflowmetry showed upper motor neuron lesion of bladder. Among 13 children with abnormal cystometric results, 11 reveal abnormal uroflow curves. 2 children (22.2%) displayed upper motor neuron lesion of bladder among the 9 children with normal uroflow curve. So, uroflowmetry reveal sensitivity 84.6%, specificity 77.8% for neurogenic bladder in cerebral palsied children. CONCLUSIONS: These results show a high rate of neurogenic bladder in children with cerebral palsy as in previous reports. Abnormalities on uroflow curve correlated well with abnormal cystometry findings. We believe that uroflowmetry is a viable noninvasive screening tool for the detection of neurogenic bladder in children with cerebral palsy.
Cerebral Palsy* ; Child* ; Humans ; Mass Screening* ; Motor Neurons ; Sensitivity and Specificity ; Urinary Bladder ; Urinary Bladder, Neurogenic* ; Urodynamics

No abstract available.
Paragonimiasis*

PURPOSE: The aim of this study was to compare the factors of health-related quality of life in older adults according to the type of residency. METHODS: A cross-sectional comparative study was conducted. The subjects were 114 institutionalized older adults and 99 community dwelling older adults. Data were collected through face-to-face interviews by nurses using structured questionnaires composed of SF-36 (ver.2) and CGA-SF. RESULTS: The institutionalized older adults had more health problems and experienced lower quality of life compared to community dwelling older adults. Factors influencing health-related quality of life for institutionalized older adults were social support, educational level, and ADL, which explained about 25.7% of the total variance, while thoseof community dwelling older adults were IADL, experiencing fall, and weight loss, which had explanatory power of 31.8%. CONCLUSION: These results indicate that differentiated nursing strategies according to the type of residency are required to promote health-related quality of life for older adults.
Activities of Daily Living ; Adult ; Aged ; Humans ; Internship and Residency ; Quality of Life ; Residence Characteristics ; Weight Loss ; Surveys and Questionnaires