No abstract available.
Amnion* ; Cell Line* ; Cyclooxygenase 2* ; Protein Kinases*

Cyclic Neutropenia is a benign, unusual hematologic disorder characterized by regularly recurring episodes of severe neutropenia occurring approximately every 21 days. Beside neutrophils, monocytes, lymphocytes, platelets and reticulocytes all cycle with strict periodicity suggest that this disease should be viewed as cyclic hematopoiesis, not merely as cyclic neutropenia. During neutropenic periods, patients regularly experience aphthous stomatitis, fever, malaise, cervical lymphadenitis, cutaneous infections and occasional pneumonia and otitis media. The exact cause of cyclic neutropenia is unknown. But it is strongly suggested that cyclic neurtopenia is due to an abnormality in the regulation of early hematopoietic precursor cells. We have documented a case of cyclic neutropenia for a period of 2.5 years in a 11 year-old boy who had suffered from recurrent fever, stomatitis, gingival swelling, cervical lymphadenitis and skin infections at 3 weeks intervals since 5 years of age. A brief review of the related literatures is presented.
Child ; Fever ; Hematopoiesis ; Humans ; Lymphadenitis ; Lymphocytes ; Male ; Monocytes ; Neutropenia* ; Neutrophils ; Otitis Media ; Periodicity ; Pneumonia ; Reticulocytes ; Skin ; Stomatitis ; Stomatitis, Aphthous

No abstract available.
Thanatophoric Dysplasia*

No abstract available.
Pierre Robin Syndrome*

This is a report of clinical study on the 63 patients of symptomatic urinary tract infection who were hospitalized at the Department of Pediatrics in Kangnam General Hospital during the period between the May of 1989 and the September of 1992. The observation results were as follows: 1) The frequency of urinary tract infection (UTI) was the highest in children under the age of one year (66.7%). 35 patients were male, while the other 28 were female. The ratio of male and female patients was 1.25:1. 2) Fever was observed in most of the cases. Systemic nonspecific manifestation was predominant in the infancy and early childhood, whereas local symptom of UTI was predominant in the late childhood. Among the 35 male patients, 32 cases were phimoses. As for the other 3 cases, Phimoses were not identified. 3) E. coli had the most frequency (65.3%), followed by Klebsiella, Enterobacter, Pseudomonas in descending order. 4) In the vitro antimicrobial susceptibility test, Gram negative organisms such as E. coli Klebsiella, Enterobacter were sensitive to cefotaxime, amikacin, and ampicillin/sulbactam. Meanwhile, Gram positive organisms such as Staphylococcus, Enterococcus were sensitive to vancomycin and cephradine. 5) The renal ultrasonography test was performed for 59 patients, 13 cases of whom showed abnormal findings. 6 cases out of the 13 abnormal patients had hydronephrosis. 6) DMSA renal scan was performed for 40 patients within 2 months of an acute pyelonephritic attack. Signs of pyelonephritic change were found in 18 patients. DMSA scan was repeated 4~12 months later in 4 of these patients. This showed renal cortical scarring in all patients. 7) Voiding cystourethrographic findings in 38 patients showed vesicoureteral reflux in 13 patients. 8) We performed urine culture again after 48 hours from the beginning of therapy and 85.5% of the cases became sterile. 9) The recurrent percentage of UTI was 23.8% with the ratio of 1:1.5 between male and female. Most of the patients were clildren under the age of one year. 10) Operations were made on two cases showing the increase of reflux during the follow-up of unilateral Grade ll and Grade 3 reflux, respectively. Operations were also performed on other two cases with bilateral Grade IV reflux. All the cases were good after the operations. In the meantime, as for another two cases having the right reflux of Grade lland the bilateral reflux of Grade lll, the refluxes were able to be reduced by prophylaxis only, in the course of following up.
Amikacin ; Cefotaxime ; Cephradine ; Child* ; Cicatrix ; Enterobacter ; Enterococcus ; Female ; Fever ; Follow-Up Studies ; Hospitals, General ; Humans ; Hydronephrosis ; Infant* ; Klebsiella ; Male ; Pediatrics ; Phimosis ; Pseudomonas ; Staphylococcus ; Succimer ; Ultrasonography ; Urinary Tract Infections* ; Urinary Tract* ; Vancomycin ; Vesico-Ureteral Reflux

The aim of the present study was to determine whether oncologic outcomes and adverse events associated with active on/off intermittent antiandrogen monotherapy (daily bicalutamide, 50 mg per day) are comparable with those of standard external beam radiation therapy (EBRT) or combined androgen blockade (CAB) therapy in prostate cancers with positive surgical margins after radical prostatectomy. Two hundred twenty-three patients with positive surgical margins post-radical prostatectomy who underwent active surveillance (AS, n = 32), EBRT without hormone therapy (n = 55), intermittent antiandrogen monotherapy without EBRT (IAAM, n = 50), or CAB without EBRT (n = 86), between 2007 and 2014, were reviewed retrospectively. Pathologic outcomes, biochemical recurrence rates, radiological disease progression, and adverse events were collected from medical records. Biochemical recurrence rates, biochemical recurrence-free survival rates, and radiological recurrence were not different between the groups (P = 0.225, 0.896, and 0.284, respectively). Adverse event rates and severities were lower for IAAM compared with EBRT or CAB (both P < 0.05), but were comparable to those for AS (P = 0.591 and 0.990, respectively). Grade ≥3 adverse events were not reported in the IAAM or AS groups. Erectile dysfunction and loss of libido rates were lower in the IAAM group compared with the EBRT and CAB groups (P = 0.032). Gastrointestinal complications were more frequently reported in the EBRT group (P = 0.008). Active on/off IAAM treatment might be an appropriate treatment option for patients with positive surgical margins after radical prostatectomy. Furthermore, regarding oncologic outcomes, IAAM was comparable to standard EBRT but had a milder adverse event profile.
Aged ; Aged, 80 and over ; Androgen Antagonists/adverse effects* ; Anilides/adverse effects* ; Antineoplastic Agents/adverse effects* ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Chemotherapy, Adjuvant/adverse effects* ; Disease-Free Survival ; Humans ; Male ; Neoplasm Recurrence, Local/blood* ; Neoplasm, Residual ; Nitriles/adverse effects* ; Prostate-Specific Antigen/blood* ; Prostatectomy ; Prostatic Neoplasms/therapy* ; Radiotherapy, Adjuvant/adverse effects* ; Retrospective Studies ; Tosyl Compounds/adverse effects*

We present a case of erosive adenomatosis of the nipple in a 22-year-old woman. The clinical features of erosive adenomatosis of the nipple are similar to Paget's disease in that it has erythematous erosions with oozing and fissured crusts on one nipple. However, histologic findings are characteristic, consisting of dilated tubular structures that are lined by a peripheral layer of cuboidal cells and a luminal layer of columnar cells showing secretary properties into the lumen. The clinical course is benign, so simple mastectomy is enough for treatment.
Female ; Humans ; Mastectomy, Simple ; Nipples* ; Phenobarbital ; Young Adult

BACKGROUND: The automated border detection(ABD) echocardiography has the ability of recognizing the endocardial-blood interface, and therefore, on-line estimation of the left ventricular(LV) volume every cardiac cycle. Compared with the off-line conventional 2-dimensional echocardiographic method that requires tracing the endocardial border manually, the ABDd system can be a convenient and objective method in the estimation of the LV volume and the ejection fraction(EF). The purpose of this study is to compare the LV volume and EF between the on-line ABD system and the convertional off-line echocardiographic method. METHODS: In two weeks, 83 adult patients older than 16 years of age were referred to our echocardiographic laboratory. Among these 83 patients, 64 patients who had a normal sinus rhythm were included to our study. Using the Hewlett-Packard SONOS 1500, a 2.5 MHz transducer was placed dat the cardiac apex. Patients with an apical 4 chamber view of the LV in which at least 75% of the endocardium was clearly seen were selected for study. On that view, the ABD system was turned on, and the reansmit power and the time-gain compensation controls were adjusted in order to approximate the automated border to the visually apparent endocardial surface. The LV end-diastolic volume(LVESV) and LV end-systolic volume(LVESV) were calculated by the method of disc. LVEDV, LVESV, and EF were displayed every cardiac cycle. Also the off-line estimation of the LV volume was performed by the method of disc, after manually tracing the endocardial border on the apical 4 chamber view. RESULTS: 44 patients(69%) of 64 patients had > or =75% of the LV endocardium visualized. LVEDV, LVESV, and Ef with the ABD system were highly correlated with those with the off-line, manually traced method(r=0.95, 0.8, respectively), but LVEDV and EF with toe ABD system were significantly less than those with the latter(p<0.01). The limits of agreement between tow methods(off-line, manually traced method-ABD system) were somewhat wide. Those of LVEDV, LVESV and EF were +22~-10ml(mean 6ml), +15~-14ml(mean 0.1ml), and +19~-12%(mean 3.8%), respectively. CONCLUSION: LVEDV,LVESV, and EF measurements by the ABD system and the off-line manually traced methods thve a strong correlation, The ABD system should habe clinical applications in setting, in which measurements of LV volume and Ef are important, But, the comparison with a more reliable method is necessary.
Adult ; Compensation and Redress ; Echocardiography* ; Endocardium ; Humans ; Toes ; Transducers

Plasmapheresis has been used for some conditions during pregnancy and puerperium, such as hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. We present one case of the HELLP syndrome which was treated with plasmapheresis and also review the indications, complications and guidelines for repetitive plasma exchange.
Blood Platelets ; Female ; HELLP Syndrome* ; Hemolysis ; Liver ; Plasma Exchange ; Plasmapheresis* ; Postpartum Period* ; Pregnancy

PURPOSE: Homocysteine is a strong and independent risk factor for cardiovascular disease. The deleterious effects of homocysteine included endothelial dysfunction, arterial intimal-medial thickening, wall stiffness and procoagulant activity. However, the precise mechanism responsible for homocysteine release in children with coronary artery disease is still unknown. The purpose of this study was to investigate serum homocysteine and tumor necrosis factor(TNF)-alpha levels and identify whether these levels had any association with the development of coronary artery lesions in Kawasaki disease(KD). METHODS: Serum homocysteine and TNF-alpha levels were measured in 24 KD patients(group 1, eight patients with normal coronary artery; group 2, 16 patients with coronary artery lesions) and 21 controls(group 3, 10 afebrile controls; group 4, 11 febrile controls). Blood samples were drawn from each study group before and after intravenous immunoglobulin(IVIG) therapy and in the convalescent stage. RESULTS: The homocysteine levels before IVIG therapy were significantly higher in group 1 than in group 3, and in group 2 than in group 3 and 4. The TNF-alpha levels before IVIG therapy were significantly higher in group 2 than group 3 and 4. Serum homocysteine and TNF-alpha levels were highest in group 2 before IVIG therapy. In the acute KD patients, serum homocysteine levels correlated significantly with TNF-alpha levels. CONCLUSION: The increased serum homocysteine levels in the acute stage increase the susceptibility to coronary arterial lesions in KD. TNF-alpha may also play an important role in the formation of coronary arterial lesions in KD.
Cardiovascular Diseases ; Child ; Coronary Artery Disease ; Coronary Vessels ; Homocysteine* ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Necrosis ; Risk Factors ; Tumor Necrosis Factor-alpha*