Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity, insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed.
Acanthosis Nigricans ; Child ; Diabetes Mellitus* ; Failure to Thrive ; Female ; Humans ; Hypertrichosis ; Insulin ; Jaw ; Mouth

Clear cell sarcoma of tendon and aponeurosis is a rare malignant tumor. It occurs chiefly in young adults, predominates in women and is most common in the regions of the foot and ankle. We report a case of clear cell sarcoma of tendon and aponeurosis in s 22-year-old man. he pstient had had a asymptomatic, normal skin colored, relativerly hard, dome shsped nodule on the anterior chest for 6 months. Histopsthologic findings revealed uniform pattern composed of compact nests of round or fusiform cells which had clear cytoplasm and were surrounded by delicate framework of fibrocollagenous tissue, and the individual tumor cell had a fairly regular appearance of possessing round to avoid vesicular nucleus with prominent basophilic nucleolus. One year after surgical excision and post operative radiotherapy, there was no recurrence.
Ankle ; Basophils ; Cytoplasm ; Female ; Foot ; Humans ; Radiotherapy ; Recurrence ; Sarcoma, Clear Cell* ; Skin ; Tendons ; Thorax* ; Young Adult

No abstract available.
Myocardial Infarction ; Obesity

PURPOSE: The purpose of this study was to investigate the radiologic and serologic factors related to postoperative union using intramedullary (IM) internal fixation in atypical femoral fractures (AFF), which are closely related to bisphosphonates (BPs) for osteoporosis. MATERIALS AND METHODS: From February 2008 to December 2016, 65 patients (71 cases) who had undergone IM nail fixation after diagnosis of AFF were enrolled in this study. Patients were divided into group A, who experienced union within 6 months and group B, who did not experience union within 6 months. They were evaluated for duration of BPs use, radiologic factors and serological factors. RESULTS: The mean duration of BPs use was 6.17 years in group A and 8.24 years in group B (p=0.039). In the subtrochanteric area, there were 14 cases (27.5%) in group A and 14 cases (70.0%) in group B. In the femoral shaft, there were 37 cases (72.5%) in group A and 6 cases (30.0%) in group B (p=0.001). On the preoperative, the flexion in the coronal plane was 5.9° (2.1°–9.2°) in group A and 8.0° (3.1°–12.1°) in group B (p=0.041). On the postoperative, conversion to valgus was 15 cases (29.4%), 8 cases (40.0%); conversion to neutral was 34 cases (66.7%) and 8 cases (40.0%); conversion to varus was 2 cases (3.9%) and 4 cases (20.0%), each (p=0.037). The fracture site gap was 1.5 mm (0–2.9 mm) on the front side and 1.2 mm (0–2.2 mm) on lateral side and 2.2 mm (0.9–4.7 mm) and 1.9 mm (0.5–3.5 mm), each (p=0.042, p=0.049). Among serological factors, there was no significant difference between the two groups. CONCLUSION: Factors adversely affecting the union should be recognized before surgery, such as longterm BPs use or a severe degree of bending of the femur in the coronal plane. During surgery, proper reduction and spacing of the fracture site on the coronal plane should allow adequate reduction of the anterior and posterior surfaces. Obtaining anatomic reduction would be most beneficial for union, but if that is not possible, obtaining congenital valgus rather than varus on the coronal plane may be helpful for union.
Diagnosis ; Diphosphonates ; Femoral Fractures ; Femur ; Humans ; Osteoporosis

The renin-angiotensin system plays an important role in renal growth and development. Exposure of the fetus or neonate to angiotensin converting enzyme (ACE) inhibitors increases mortality, growth retardation, and results in renal anomalies. This study was designed to investigate the effects of ACE inhibition in the neonatal rat on the expression of genes known to modulate renal cellular proliferation, cell interactions, and extracellular matrix. Newborn rat pups were treated with enalapril (30mg/ kg/day) or vehicle for 14 days, and kidneys were removed for determination of mRNA for transforming growth factor-beta 1 (TGF- B1) and prepro epidermal growth factor (EGF). Enalapril treatment resulted in 40Yo mortality by day 14 as well as reduced body and kidney weight (P<0.05 vs vehicle group). Also enalapril decreased renal TGF-Bl and EGF mRNA expression (P<0.05). These results indicate that ACE inhibition in the developing kidney reduces the renal expression of critical growth factors, which may account for renal growth impairment.
Angiotensins* ; Animals ; Cell Communication ; Cell Proliferation ; Enalapril ; Epidermal Growth Factor ; Extracellular Matrix ; Fetus ; Growth and Development ; Humans ; Infant, Newborn ; Intercellular Signaling Peptides and Proteins ; Kidney* ; Mortality ; Peptidyl-Dipeptidase A* ; Rats* ; Renin-Angiotensin System ; RNA, Messenger ; Transforming Growth Factor beta1

The measurements of B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP), when taken together with conventional clinical assessment, may assist in making the prognosis and also for making serial adjustment of such treatment. But although such commercial assays are currently approved for the diagnosis of heart failure, the role of the natriuretic peptides for monitoring the success of congestive heart failure (CHF) therapy has not as yet been submitted for regulatory approval. Moreover, because of the intra-individual biologic variation of the BNP or because of multiple factors that affect the BNP levels, the magnitude of the change of BNP levels must be large to confidently interpret BNP changes within an individual, and just how large has not been determined. Yet the levels of plasma BNP and NT-pro BNP are well correlated with the concurrent haemodynamic measurements and indicators of left ventricular systolic function. Also, BNP and NT-pro BNP serve as significant prognostic information and it is possible that adjustment of anti-heart failure therapy according to serial measurements of BNP (in addition to the standard clinical assessment) may offer improved outcomes. Better understanding of the test characteristics is needed before we can effectively use this valuable test to guide therapeutic strategies.
Diagnosis ; Heart Failure* ; Heart* ; Humans ; Natriuretic Peptide, Brain* ; Natriuretic Peptides ; Plasma ; Prognosis

PURPOSE: Recently, it has been reported that the disease with secondary surfactant deficiency such as pneumonia and adult respiratory distress syndrome (ARDS) improved in arterial blood gas analysis and pulmonary mechanics by surfactant treatment. In this study, we investigated the effect of surfactant in the experimentally induced E. coli pneumonia in rats. METHODS: 0.25 mL (0.5x109) E. coli suspension was injected intratracheally to the rats. After ventilating rats for 1 minute, 0.25 mL Surfactant TA (60 mg/mL phospholipid) was administered to the study group and normal saline to the control group. In about 12 hours, pneumonia symptoms developed, and the arterial blood gas analysis was performed with the blood obtained from abdominal aorta accessed by laparotomy. And then, bronchial lavage fluid (BAL) was obtained to perform cell count with differentials and E. coli culture, and to measure protein concentrations. The lungs were fixed in formalin for histological examination to compare the degree of inflammation. RESULTS: There were no significant differences in PaO2, cell count, differential count, E. coli culture between the study group and the control group. The protein concentrations of BALs in the surfactant-treated group were significantly lower than those in the control group (277+/-164 mg/dL vs 1,030+/-410 mg/dL). The inflammatory changes were found in E. coli-infected lung tissues from both groups, but less prominent in the surfactant-treated group than in control. CONCLUSION: Surfactant treatment decreased both the protein concentration of BALs and the inflammatory changes of lung tissue in an experimental model of E. coli pneumonia in rats.
Animals ; Aorta, Abdominal ; Blood Gas Analysis ; Bronchoalveolar Lavage Fluid ; Cell Count ; Formaldehyde ; Inflammation ; Laparotomy ; Lung ; Mechanics ; Models, Theoretical ; Pneumonia* ; Rats* ; Respiratory Distress Syndrome, Adult

PURPOSE: In a developing kidney, the renin-angiotensin system(RAS) is markedly activated and is thought to play an important role in postnatal renal growth and maturation. We previously demonstrated that angiotensin converting enzyme(ACE) inhibition in a developing rat kidney increases apoptosis and decreases its related gene expressions, which may account for renal growth impairment. Among the mitogen-activated protein kinases(MAPK) family members, c-jun N terminal kinase(JNK) and p38 MAPK(p38) are thought to inhibit cell growth and induce apoptosis. This study was designed to investigate the relationship between the RAS and the MAPK family during neonatal renal development. METHODS: Forty-nine neonatal Spargue-Dawley rats were separated into two groups. The enalapril group was treated with ACE inhibitor(enalapril:30 mg/kg/day) and the control group with normal saline for seven days. Their kidneys were removed for immunohistochemical stain and western blot analysis of JNK-2 and p38. RESULTS: In the enalapril group, JNK-2 expression was strongly detected in the dilated cortical tubular epithelial cells, and JNK-2 protein expression was significantly increased compared to the control group. p38 expression was noted in the dilated tubular epithelial cells by ACE inhibitor and also p38 protein expression was significantly increased. CONCLUSION: These results suggest that the expressions of the MAPK family, especially JNK and p38, are modulated by ACE inhibition in the neonatal rat kidney. In regard of the correlation between MAPK activations and the occurrence of apoptosis in renal growth impairment by ACE inhibition, JNK and p38 may be implicated to participate in angiotensin II related intracellular signaling pathways of renal apoptosis in developing kidney.
Angiotensin II ; Angiotensins* ; Animals ; Apoptosis ; Blotting, Western ; Enalapril ; Epithelial Cells ; Gene Expression ; Humans ; Kidney* ; Mitogen-Activated Protein Kinases ; Models, Animal ; p38 Mitogen-Activated Protein Kinases ; Peptidyl-Dipeptidase A* ; Protein Kinases* ; Rats* ; Renin-Angiotensin System

No Abstract Available.
Humans ; Kidney Failure, Chronic* ; Rupture* ; Tendons*

Background: This study compared the neutralizing antibody kit using the Enzyme-Linked Immunosorbent Assay (ELISA) method with the rapid antibody diagnostic kit using the Lateral Flow Immunoassay (LFIA) method to evaluate the clinical effectiveness of the COVID-19 Biokit IgG/IgM regarding evaluation of antibody formation after COVID-19 vaccination. Methods: The neutralizing antibody test was performed with antibody detection kit of diagnostic medical devices for the qualitative method using the standard ELISA method. The rapid antibody diagnostic kit was measured with the COVID-19 Biokit IgG/IgM using the LFIA method. Based on the results of the neutralizing antibody measurement test of the standard test method, the test results of the rapid antibody diagnostic kit are compared and analyzed to confirm its the sensitivity and specificity. Results: When the consistency was determined as positive and negative for the two test results, 118 cases were matched and two cases were inconsistent, showing a 98.3% consistency rate. That is, sensitivity 98%, specificity 100% and correctly classified proportion 98%. Conclusions Although the positive results of antibody formation of this kit would mean that individual has immunity to COVID-19, the result cannot be used to confirm or evaluate for re-infection. But the strong agreement between rapid antibody diagnostic kit results and ELISA results suggests that the kit used in this study is available as a screening test for antibody and neutralizing antibody responses, which could help evaluate the need for additional vaccinations, collect data quickly and cheaply and monitor individual immune responses.