Ninety two cases of culture proved neonatal sepsis who had been admitted to pediatric department, National medical Center, during 7 years from Jan. 1984 to Dec. 1990 were reviewed clinically and the following results were obtained. 1) The frequency of neonatal sepsis was 3.1% and male to female sex ratio was 1.9:1, Sepsis was more prevalent in premature infants (9.9%) than in full term infants (1.9%). 2) The seasonal incidence was more prevalent in summer (32.6%). 3) The weight distribution showed 50 cases with the weight less than 2500 gram and 42 cases more than 2500 gram. 4) In 76 cases the onset was before 7 days old and in 16 cases were developed after 7 days old. 5) The major associated perinatal conditions in neonatal sepsis were institutional baby (23.9%), premature rupture of membranes (11.9%) and placenta previa (4.3%) in the order of frequency. The clinical manifestations on admission were jaundice (50.0%), poor activity (46.7%), respiratory difficulty (35.9%), poor feeding (22.8%), cyanosis (22.7%), gastrointestional symptoms (21.5%), fever (15.2%) and convulsion (13.0%) in the order of frequency. 6) The associated diseases were urinary tract infection (31.5%), hyaline membrane disease (19.6%), congenital disorder (18.5%), pneumonia (15.2%), anemia (13.0%), meningitis (9.8%), omphalitis (7.6%), DIC (6.5%), necrotizing enterocolitis (5.4%) and intracranial hemorrhage (5.4%) in the order of frequency. 7) Causative organisms were gram positive organisms in 27 cases (25.7%) and gram negative organisms in 79 cases (74.3%). The main organisms were Serratia marcescens (18.5%). Enterobacter spp (17.4%), Klebsiella pneumoniae (12.0%), Staphylococcus aureus (10.9%), Acinetobactor calcoaceticus (8.7%), Coagulase (-) staphylococcus (8.7%), E. coli (8.7%), Enterococcus (6.5%), Group B beta-hemolytic streptococcus (5.4%) and Pseudomonas (5.4%) in the order of frequency. The sensitivity to antibiotics were: Serratia marcescens: 70.6% sensitive to Amikacin 58. 9% sensitive to Cefotaxime 59. Enterobacter spp: 87.5% sensitive to Amikacin 68.8% sensitive to Cefotaxime Klebsiella pneumoniae: 100% sensitive to Amikacin 91. 0% sensitive to Cefotaxime Staphylococcus aureus: 100% sensitive to Cefazolin 90. 0% sensitive to Cefotaxime Acinetobacter calcoaceticus: 88.9% sensitive to Amikacin Coagulase (-) Staphylococcus: 100% sensitive to Amikacin 87. 5% sensitive to Cefotaxime E. coli: 100% sensitive to Amikacin, Cefotaxime Enterococcus: 50% sensitive to Gentamicin, Ampicillin, Amikacin Group B beta-hemolytie Streptococcus: 100% sensitive to Ampicillin, Penicillin Pseudomonas: 100% sensitive to Amikacin, Gentamicin, Tobarmycin 8) Mortality cases were 32 cases (34.8%).
Acinetobacter calcoaceticus ; Amikacin ; Ampicillin ; Anemia ; Anti-Bacterial Agents ; Cefazolin ; Cefotaxime ; Coagulase ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Cyanosis ; Dacarbazine ; Enterobacter ; Enterococcus ; Enterocolitis, Necrotizing ; Female ; Fever ; Gentamicins ; Humans ; Hyaline Membrane Disease ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature ; Intracranial Hemorrhages ; Jaundice ; Klebsiella pneumoniae ; Male ; Membranes ; Meningitis ; Mortality ; Penicillins ; Placenta Previa ; Pneumonia ; Pseudomonas ; Rupture ; Seasons ; Seizures ; Sepsis* ; Serratia marcescens ; Sex Ratio ; Staphylococcus ; Staphylococcus aureus ; Streptococcus ; Urinary Tract Infections

No abstract available.

"Fetus in fetus" is a rare pathologic feature consisting of a parasitic twin included within the body of the other twin, which most likely arises from inclusion of a monozygotic, diamniotic twin pregnancy. The exact embryogenesis of fetus in fetus is controversial. Some investigators propose that it is a highly organized teratoma. Since the condition was first described by Meckel in the late 18th centry, approximately 100 cases have been reported in the literature. Most cases present as an abdominal mass during the first year of life, with a few cases being detected prenatally by ultrasound examination. So we report one case of fetus in fetus detected by ultrasound examination prenatally.
Embryonic Development ; Female ; Fetus* ; Humans ; Pregnancy ; Pregnancy, Twin ; Research Personnel ; Teratoma ; Ultrasonography

BACKGROUND: Cyclin-dependent kinase-associated phosphatase (KAP) is a human dual-specificity protein phosphatase that dephosphorylates Cdk2 on threonine160 in a cyclin-dependent manner and that is known as an up-regulated molecule in some malignant tumors. We investigated the expression and clinicopathologic significance of KAP protein in relation to tumorigenesis of colorectal carcinoma. METHODS: The expression patterns of KAP protein in tumor tissue were examined by reverse transcription-PCR and immunohistochemical staining. RESULTS: An enhanced transcriptional level of KAP mRNA was observed in 11 out of 12 colorectal carcinoma specimens. Immunohistochemical examination showed that KAP protein was more highly expressed in the tumors than that in the adjacent non-neoplastic mucosal tissues for 52 of 102 colorectal cancer tissues. The statistical analysis showed that an increased level of KAP protein in the colorectal cancer tissues was inversely correlated with the histologic grade, tumor size and Duke's stage. CONCLUSION: The present study suggests that alteration of KAP might play a role, at least in part, in the tumorigenicity of colorectal carcinoma through the mechanism of cell cycle regulation.
Carcinogenesis ; Cell Cycle ; Colorectal Neoplasms* ; Humans ; Immunohistochemistry ; Mucous Membrane ; RNA, Messenger

PURPOSE: Skin-sparing mastectomy (SSM) involves the resection of the nipple-areolar complex (NAC) along with the breast parenchyma, and this improves aesthetic outcome for breast cancer patients. Yet most patients desire preservation of the NAC. The purpose of this study was to determine the associated risk factors of NAC involvement and to identify the value of preoperative breast magnetic resonance imaging (MRI) for measuring the tumor-nipple distance (TND). METHODS: This prospective study was carried out in 92 breast cancer (3 patients with bilateral breast cancer) patients who underwent MRI and they had undergone modified radical mastectomy or SSM at the Department of Surgery in Soonchunhyang University Hospital from November of 2003 to March of 2006. The patients were divided into two groups: nipple-positive for malignancy (group 1; n=9) and nipple-negative for malignancy (group 2; n=86). We analyzed the risk factors of NAC involvement, including tumor size, nuclear grade, lymph node invasion, muticentricity, TND, the hormone recepor status, and lymphovascular invasion. The TND was measured by preoperative breast MRI (the imaging distance) in all patients and by the distance of specimens which were obtained postoperatively (the real distance) in 31 patients. RESULTS: The overall frequency of malignant nipple involvement was 9 of 95 (9.4%). There were no differences in tumor size, nuclear grade, lymph node invasion, muticentricity, the hormone recepor status and lymphovascular invasion between the two groups. The TND was identified as an independent predictor of malignant NAC involvement: the mean TND by breast MRI was 0.7 cm in group 1 and 2.7 cm in group 2 (p=0.01) the mean TND by pathologic measuring the specimen was 0.7 cm in group 1 and 3.2 cm in group 2 (p=0.02). In all the nipple involved cases, the tumor was within 2 cm of the nipple. In measuring the TND, there was no significant difference between the imaging distance of MRI and real distance of the sepcimen (p=0.166). CONCLUSION: The only predictive factor for malignant NAC invasion was TND in our study. NAC preservation would be appropriate for the patients with tumor located more than 2 cm away from the nipple. The TND as determined by preoperative MRI is considered to be helpful when deciding whether to preserve the NAC.
Breast Neoplasms* ; Breast* ; Humans ; Lymph Nodes ; Magnetic Resonance Imaging ; Mastectomy ; Mastectomy, Modified Radical ; Nipples* ; Prospective Studies ; Risk Factors

PURPOSE: The purpose of this study was to evaluate the relationship between osteoporosis and skin thicknessas shown by CT scanning. MATERIALS AND METHODS: Eighty- six women with osteoporosis (mean age, 52) and 51 normalcontrols (mean age, 50) participated in the study. For a quantitative CT examinations, a CT scanner(Somatom Plus,Siemens) was used. Osteoporosis was defined as present when spinal bone mineral density was more than 2.5 standarddeviations below young normal density, as determined by quantitative CT. Patients with endocrinologic, malignantor collagen disease and undergoing antimetabolite or steroid therapy were excluded. The thickness of back skin wasretrospectively measured at the third lumbar vertebra level, as seen on CT films, using a conventional magnifier.For statistical analysis, Students' t test and Spearman's rank correlation were used. RESULTS: On the basis of CTscans, the mean thickness of back skin in the osteoporotic group(0.50+/-0.20 mm) was significantly less than innormal control subjects(0.80+/-0.23 mm) (p<0.001). Significant correlation was observed between skin thickness andbone mineral density(r=0.523, p<0.0001). Sensitivity, specificity, accuracy, and positive and negative predictivevalues were measured as 76, 78, 76, 88, 62% with a cut-off value of 0.6 and 84, 61, 77, 81, 66% with a cut-offvalue of 0.7, respectively. CONCLUSION: The present study demonstrated that the thickness of back skin, asmeasured by CT scanning, is predictive of osteoporosis.
Bone Density ; Collagen Diseases ; Female ; Humans ; Osteoporosis* ; Sensitivity and Specificity ; Skin* ; Spine ; Tomography, X-Ray Computed*

Klippel-Trenaunay-Weber syndrome is a congenital vascular disorder consisted of a variety of vascular malformations, enlargement of the involved limb, and varicose veins. We report a case of Klippel-Trenaunay-Weber syndrome presenting portal hypertension and varices bleeding caused by hypoplasia of the portal vein. Portal hypertension was caused by portal vein hypoplasia associated with Klippel-Trenaunay-Weber syndrome. There were three episodes of variceal bleeding, and hemostasis were achieved by endoscopic band ligation, Sugiura operation, and splenic artery embolization respectively. Although successful hemostasis was achieved, an additional procedures to reduce portal hypertension were needed to prevent repeated episodes of variceal bleeding.
Adult ; English Abstract ; Esophageal and Gastric Varices/*complications ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Hypertension, Portal/complications ; Klippel-Trenaunay-Weber Syndrome/*complications ; Male ; Portal Vein/abnormalities

Background: Pigmented purpuric dermatosis (PPD) is a chronic disorder characterized by distinct petechial hemorrhage and brownish pigmentation. The cause of PPD is unclear, but several underlying conditions are associated with it. Previous reports suggest that venous insufficiency (VI) might be related to PPD; however, a clear correlation remains unelucidated. Objective: To elucidate the causal relationship between PPD and VI. Methods: A total 118 patients diagnosed with PPD in the Department of Dermatology, Pusan National University Hospital from November 2006 to July 2019 were retrospectively reviewed. Doppler ultrasonography of the lower extremities was performed in 56 PPD patients, who were then divided into two groups: PPD with and without VI. We compared the clinical features between the two groups. In the PPD with VI group, we assessed the correspondence ratios between PPD and VI lateralities, and between the PPD distribution and the veins involved. Results: VI was detected in 35 of the 56 patients (62.5%). The PPD with VI group was significantly associated with wider distribution, darker coloration and longer disease duration. There was a positive correlation of laterality between PPD and VI, and between PPD distribution and the vein involved. Conclusion This findings suggest that VI is a clear provoker of PPD.

Rab40 proteins are an atypical subgroup of Rab GTPases containing a unique suppressor of the cytokine signaling (SOCS) domain that is recruited to assemble the CRL5 E3 ligase complex for proteolytic regulation in various biological processes. A nonsense mutation deleting the C-terminal SOCS box in the RAB40B gene was identified in a family with axonal peripheral neuropathy (Charcot-Marie-Tooth disease type 2), and pathogenicity of the mutation was assessed in model organisms of zebrafish and Drosophila. Compared to control fish, zebrafish larvae transformed by the human mutant hRAB40B-Y83X showed a defective swimming pattern of stalling with restricted localization and slower motility. We were consistently able to observe reduced labeling of synaptic markers along neuromuscular junctions of the transformed larvae. In addition to the neurodevelopmental phenotypes, compared to normal hRAB40B expression, we further examined ectopic expression of hRAB40B-Y83X in Drosophila to show a progressive decline of locomotion ability. Decreased ability of locomotion by ubiquitous expression of the human mutation was reproduced not with GAL4 drivers for neuron-specific expression but only when a pan-glial GAL4 driver was applied. Using the ectopic expression model of Drosophila, we identified a genetic interaction in which Cul5 down regulation exacerbated the defective motor performance, showing a consistent loss of SOCS box of the pathogenic RAB40B. Taken together, we could assess the possible gain-of-function of the human RAB40B mutation by comparing behavioral phenotypes in animal models; our results suggest that the mutant phenotypes may be associated with CRL5-mediated proteolytic regulation.

To elucidate the genes responsible for constitutive human skin color, we measured the extent of skin pigmentation in the buttock, representative of lifelong non-sun-exposed skin, and conducted a gene mapping study on skin color in an isolated Mongolian population composed of 344 individuals from 59 families who lived in Dashbalbar, Mongolia. The heritability of constitutive skin color was 0.82, indicating significant genetic association on this trait. Through the linkage analysis using 1,039 short tandem repeat (STR) microsatellite markers, we identified a novel genomic region regulating constitutive skin color on 11q24.2 with an logarithm of odds (LOD) score of 3.39. In addition, we also found other candidate regions on 17q23.2, 6q25.1, and 13q33.2 (LOD > or = 2). Family-based association tests on these regions with suggestive linkage peaks revealed ten and two significant single nucleotide polymorphisms (SNPs) on the linkage regions of chromosome 11 and 17, respectively. We were able to discover four possible candidate genes that would be implicated to regulate human skin color: ETS1, UBASH3B, ASAM, and CLTC.
Adolescent ; Adult ; Asian Continental Ancestry Group/*genetics ; Child ; *Chromosome Mapping ; Color ; Female ; Genome-Wide Association Study ; Humans ; Male ; Mongolia ; Pedigree ; Polymorphism, Single Nucleotide ; Skin/metabolism ; *Skin Pigmentation ; Young Adult