No abstract available.
Animals ; Dogs* ; Neurons*

Partial Trisomy of 3p (Trisomy of 3p2, dup (3) (p23-->pter)) is a characteristic syndrome of chromosomal duplication of distal part of 3p, but breakpoints seem to vary in location. This syndrome shows multiple congenital anomalies with severe mental retardation, characteristic craniofacial change and absence of other gross external abnormalities. The craniofacial dysmorphism includes frontal bossing and temporal indentation, square face, marked hypertelorism, thick and short nose, full lips and a large mouth with downturned corners. Congenital heart defect, most frequently ASD and VSD, are found in most patients. In the majority of patients, the 3p2 duplication is the unbalanced product of a parental autosomal translocation involving 3p2 and another chromosome. We report a case of female baby who has facial dysmorphism, ASD and hyptonia and was found to have 3p2 duplidation (46XX-9, +der(9)t (3:9)(p23:p24)) by chromosomal analysis. Also we found her father was a carrier of blanced translocation of 3p2 and chromosome 9p (46XY, t(3:9)(p23:p24)).
Chromosome Duplication ; Fathers ; Female ; Heart Defects, Congenital ; Humans ; Hypertelorism ; Intellectual Disability ; Lip ; Mouth ; Nose ; Parents ; Trisomy*

BACKGROUND: The authors conducted the present study to identify clinical and radiological prognostic factors in infants and neonates with septic arthritis of the hip. METHODS: The authors retrospectively reviewed the records of 31 patients with septic arthritis of the hip. All of the patients were younger than 18 months old. Follow-up periods ranged from 5 to 17 years. The following potential variables for predicting the prognosis were included in the assessment: gender, age, underlying diseases, duration of symptoms, changes of hip joint in X-ray, concomitant osteomyelitis, elevation of erythrocyte sedimentation rate and C-reactive protein, sepsis, pus drainage, synovial fluid culture, and infecting organisms. Clinical and radiological prognoses were analyzed at the final follow-up. RESULTS: Univariate analysis demonstrated that radiological prognoses were poorer in patients who had underlying diseases, a longer duration of symptoms, and pus drainage. However, on multivariate analysis, only the variable-duration of symptoms-was found to be statistically related with a poor radiological prognosis. CONCLUSIONS: Although poor prognosis for patients with several underlying diseases and radiological changes has already been established, a favorable outcome might be expected with prompt surgical drainage and appropriate antibiotics.
Arthritis, Infectious/*diagnosis/microbiology/therapy ; Female ; Follow-Up Studies ; *Hip Joint ; Humans ; Infant ; Infant, Newborn ; Male ; Prognosis ; Retrospective Studies

PURPOSE: This study was performed to assess potential improvements in clinical outcomes when applying recent advanced hip arthroplasty surgical techniques and understand the potential relationship between bone mineral density (BMD) and surgical outcomes. MATERIALS AND METHODS: Among 37 cases of periprosthetic femoral fractures after hip arthroplasty treated between March 2014 and September 2016, all included a follow-up of at least one year and were included in this study. Outcomes were evaluated using the Beals and Tower's criteria. BMD was examined in 27 of 37 cases and the relationship between osteoporosis and treatment outcomes was analyzed. Advanced hip arthroplasty surgical approaches varied depending on the fracture type: i) open reduction with wiring for Vancouver A, ii) open reduction with double plate fixation for Vancouver B1, iii) revision THA with long stem for Vancouver B1-Nonunion, B2 and B3, and iv) open reduction with double plate fixation for Vancouver C. RESULTS: When assessed using the Beals and Tower's criteria, 33 out of 37 (89.2%) patients were excellent and 4 (10.8%) were poor. These outcomes were an improvement compared with series I (81.8%). When analyzed according to the Vancouver classification, patients with type A (n=8), type B1 (n=16), and type B2 (n=2) were all excellent, the patients with type B3 were excellent (n=1) and poor (n=1), and the patients with type C were excellent (n=6) and poor (n=3). The mean BMD was −2.6 (T-score) in 27 of 37 cases and −4.4 in 4 cases with poor prognosis. Osteoporosis was statistically correlated to those classified as poor by Beals and Tower. CONCLUSION: The results of the analysis suggest that applying new surgical hip arthroplasty treatment approaches leads to improved outcomes compared with the author's previous study. When treating periprosthetic femoral fractures following total hip arthroplasty, an appropriate internal fixation method should be selected, at least in part based on the Vancouver classification. In addition, osteoporosis may be a major prognostic factor for the outcomes of surgical treatment.
Arthroplasty ; Arthroplasty, Replacement, Hip ; Bone Density ; Classification ; Femoral Fractures ; Follow-Up Studies ; Hip ; Humans ; Methods ; Osteoporosis ; Periprosthetic Fractures ; Prognosis

Nonsurgical endoscopic or percutaneous dilatation and insertion of an endoprosthesis is the treatment of choice in the majority of patiens with incurabie malignant biliary obsturction. But these palliative treatment of extrahepatic cholestasis with an endoscopic or percutaneous biliary endoprosthesis is limited by clogging. One of the factors thought to be of importance is the diameter of the stent. So in order to avoid being limited by the size of the instrumentation channel of the endoscope, expandable stents have been developed. Wallstent is braided in the form of a tubular mesh from surgical grade stainless alloy. This prosthesis is geometrically stable, pliable and self expanding. Its elastic properties are such that its diameter can be substantially reduced by moderate elongation. The stent is constrainded on a small diameter delivery catheter(total outside diameter: 9 French). During the implantation procedure the final position of the partially released endoprosthesis can be adjusted by gradual removal of the delivery catheter. If full expansion to 30 French occurs, the stent will be shortened by approximately 30% to their normal length range between 34-102mm. Now in this article we report a new method for endoscopic retrograde placement of biliary Wallstent in a patient with obsturctive jaundice due to periampullary choangiocacrcinoma.
Alloys ; Catheters ; Cholestasis, Extrahepatic ; Dilatation ; Endoscopes ; Humans ; Jaundice ; Palliative Care ; Prostheses and Implants ; Stents

Many reports have been published on the tissue damage of a shock wave with respect to histopatological changes in light microscopy and various imaging modalities. However, the studies on the electron microscopic findings and cause of renal functional change such as parenchymal obstructive pattern following extracorporeal shock wave lithotripsy (ESWL) have not been elucidated. In order to evaluate the microstructural changes after shock wave exposure, gross, light microscopic and trasmission electron microscopic findings were analyzed with rabbit kidneys. Preliminary study (n=2) was performed to determine the dosage intensity of shock waves to inflict damage, using a EDAP LT 01 piezoelectric extracorporeal shock wave lithotriptor. A shoke wave of various intensities were given to the left kidneys of 3 different groups of rabbits. Storage value of 100, 50, 25 at rate of 20/sec under 87% power were given to group I (n=4), group II (n=4), and group III(n=3) respectively. The right kidneys were preserved as the control group. The rabbits were killed 6-12 hours later. In gross, there were a few subcapsular hemorrhagic foci and mild congestion of corticomedullary junction without a large hematoma formation. No significant differences were noted between each group. Light microscopic findings were mainly hydropic changes in the proximal convoluted tubules and congestion without significant necrotic changes. The observed pathologic changes in the transmission electron microscopy were vacuolization of cytoplasm with swelling of epithelial cells especially porximal convoluted tubules. There were also tubular obstruction due to swelling and desquamation of epithelial cells into tubular lumen. The structural changes of intracellular organelles were not found at storage values of 25 and 50. But dilatation and structural alterations of endoplasmic reticulums were noted of 100 with cell membrane rupture. The findings of this study suggest that tubular obstructions with or without structural change of intracellular organelles is one cause of renal functional change such as parenchymal obstructive pattern after ESWL.
Cell Membrane ; Cytoplasm ; Dilatation ; Endoplasmic Reticulum ; Epithelial Cells ; Estrogens, Conjugated (USP) ; Hematoma ; Kidney* ; Lithotripsy ; Microscopy ; Microscopy, Electron, Transmission ; Organelles ; Rabbits ; Rupture ; Shock*

OBJECTIVE: The aim of this study was to compare midtrimester maternal plasma concentrations of angiopoietin 1, angiopoietin 2, and placental growth factor between pregnant women who subsequently developed preeclampsia and those who did not. METHODS: Midtrimester maternal plasma was collected and stored at -70degrees C when genetic amniocentesis was performed. Cases included 37 samples of individual who subsequently developed preeclampsia, and matched controls were from individuals who did not develop preeclampsia. Angiopoietin 1, angiopoietin 2, and placental growth factor concentrations were measured by the enzyme-linked immunosorbent assay method and were compared using the Mann-Whitney U-test. A P-value <0.05 was considered significant. RESULTS: In pregnant women who subsequently developed preeclampsia, midtrimester maternal plasma concentrations of angiopoietin 1 and angiopoietin 2 were significantly higher and placental growth factor concentrations were significantly lower than in women who did not develop preeclampsia (angiopoietin 1: 10.6 [3.1-19.7] vs. 7.8 [0.9-24.4] ng/mL, P=0.031; angiopoietin 2: 31.0 [4.7-81.2] vs. 18.4 [4.2-49.7] ng/mL, P<0.001; placental growth factor: 87.1 [14.2-774.3] vs. 148.8 [57.2-425.6] pg/mL, P<0.001). Within the case group who subsequently developed preeclampsia, the placental growth factor was significantly lower in those who had fetal growth restrictions than in those who did not (placental growth factor: 72.5 [14.2-774.3] vs. 140.9 [44.2-257.5] pg/mL, P=0.003). CONCLUSION: Midtrimester maternal plasma concentrations of angiopoietin 1, angiopoietin 2, and placental growth factor may be associated with the subsequent development of preeclampsia.
Amniocentesis ; Angiopoietin-1* ; Angiopoietin-2* ; Enzyme-Linked Immunosorbent Assay ; Female ; Fetal Development ; Humans ; Plasma* ; Pre-Eclampsia* ; Pregnancy ; Pregnancy Trimester, Second* ; Pregnant Women*

No abstract available.
Animals ; Rats* ; Suprachiasmatic Nucleus* ; Vasoactive Intestinal Peptide*

BACKGROUND: Urinary trypsin inhibitors (UTI) have been widely used for the treatment of diseases including disseminated intravascular coagulation, shock, and pancreatitis. Since UTI synthesis is likely to be reduced in patients who have undergone liver resection, the incidence of inflammatory reactions may be increasing accordingly. For such patients, the liver enzyme increases after the operation can reflect liver damage. The purpose of this study was to examine if ulinastatin can inhibit liver enzyme increases after liver resection. METHODS: After receiving Institutional Review Board approval, a retrospective chart review was performed on 201 patients who underwent hepatic resection from 2006 to 2010. We divided the records into the control (n = 69) and ulinastatin (n = 132) groups according to the use of intraoperative ulinastatin and compared the preoperative and postoperative laboratory test results. The number of patients who had > 400 U/L elevation of aspartate transaminase (AST) level after surgery was compared between the 2 groups. RESULTS: The mean AST, alanine transaminase (ALT), and total bilirubin levels after liver resection were significantly lower in the ulinastatin group than in the control group. The number of patients who showed an AST > 400 U/L after liver resection was significantly higher in the control group (odds ratio = 3.02). CONCLUSIONS: Ulinastatin attenuates the elevation of hepatic enzymes and bilirubin after liver resection.
Alanine Transaminase ; Aspartate Aminotransferases ; Bilirubin ; Disseminated Intravascular Coagulation ; Ethics Committees, Research ; Glycoproteins ; Hepatectomy ; Humans ; Incidence ; Liver ; Liver Function Tests ; Pancreatitis ; Retrospective Studies ; Shock ; Trypsin ; Trypsin Inhibitors

Purpose: Neuregulin 1 (NRG1) gene fusion is a potentially actionable oncogenic driver. The oncoprotein binds to ERBB3-ERBB2 heterodimers and activates downstream signaling, supporting a therapeutic approach for inhibiting ERBB3/ERBB2. However, the frequency and clinicopathological features of solid tumors harboring NRG1 fusions in Korean patients remain largely unknown. Materials and Methods: We reviewed archival data from next-generation sequencing panel tests conducted at a single institution, specifically selecting patients with in-frame fusions that preserved the functional domain. The clinicopathological characteristics of patients harboring NRG1 fusions were retrospectively reviewed. Results: Out of 8,148 patients, NRG1 fusions were identified in 22 patients (0.27%). The average age of the patients was 59 years (range, 32 to 78 years), and the male-to-female ratio was 1:1.2. The lung was the most frequently observed primary site (n=13), followed by the pancreaticobiliary tract (n=3), gastrointestinal tract (n=2, stomach and rectum each), ovary (n=2), breast (n=1), and soft tissue (n=1). Histologically, all tumors demonstrated adenocarcinoma histology, with the exception of one case of sarcoma. CD74 (n=8) and SLC3A2 (n=4) were the most frequently identified fusion partners. Dominant features included the presence of fewer than three co-occurring genetic alterations, a low tumor mutation burden, and low programmed death-ligand 1 expression. Various clinical responses were observed in patients with NRG1 fusions. Conclusion Despite the rarity of NRG1 fusions in Korean patients with solid tumors, identification through next-generation sequencing enables the possibility of new targeted therapies.