An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures

We experienced an extremely rare case of proximal epithelioid sarcoma (PES) of the vulva in a 77-year-old woman. After history taking and physical examination, the patient was tentatively diagnosed as having Bartholin’s cyst in the right labium. Based on histopathological and immunohistochemical (IHC) findings, however, a final diagnosis of PES of the vulva was made. After receiving CyberKnife treatment, the patient survived but with recurrent episodes and poor prognosis. In conclusion, our case indicates that patients with PES of the vulva should be appropriately managed with radiotherapy after a differential diagnosis based on histopathological and IHC findings.

An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures

We experienced an extremely rare case of proximal epithelioid sarcoma (PES) of the vulva in a 77-year-old woman. After history taking and physical examination, the patient was tentatively diagnosed as having Bartholin’s cyst in the right labium. Based on histopathological and immunohistochemical (IHC) findings, however, a final diagnosis of PES of the vulva was made. After receiving CyberKnife treatment, the patient survived but with recurrent episodes and poor prognosis. In conclusion, our case indicates that patients with PES of the vulva should be appropriately managed with radiotherapy after a differential diagnosis based on histopathological and IHC findings.

An extremely rare case of prenatally diagnosed absent both aortic and pulmonary valves

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.

An extremely rare case of prenatally diagnosed absent both aortic and pulmonary valves

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.

Prevalence of Plasmid-mediated Quinolone Resistance and Its Association with Extended-spectrum Beta-lactamase and AmpC Beta-lactamase in Enterobacteriaceae / 대한진단검사의학회지

BACKGROUND: We investigated the prevalence of plasmid-mediated quinolone resistance and its association with extended-spectrum beta-lactamase (ESBL) and AmpC beta-lactamase in Enterobacteriaceae. METHODS: A total of 347 non-duplicated isolates of Enterobacteriaceae were collected between August and October 2006 from 2 hospitals. Qnr determinant screening was conducted using PCR amplification, and all positive results were confirmed by direct sequencing. Qnr-positive strains were determined on the basis of the presence of ESBL and AmpC beta-lactamase genes. RESULTS: The qnr gene was detected in 47 of 347 clinical Enterobacteriaceae isolates. Among the 47 qnr-positive strains, Klebsiella pneumoniae (N=29) was the most common, followed by Escherichia coli (N=6), Enterobacter cloacae (N=6), Citrobacter freundii (N=5), and Enterobacter aerogenes (N=1). These isolates were identified as qnrA1 (N=6), 8 qnrB subtypes (N=40), and qnrS1 (N=1). At least 1 ESBL was detected in 38 of the 47 qnr-positive strains. Qnr-positive strains also showed high positive rates of ESBL or AmpC beta-lactamase, such as TEM, SHV, CTX-M, and DHA. DHA-1 was detected in 23 of 47 qnr-positive strains, and this was co-produced with 1 qnrA1 and 22 qnrB4. Strains harboring MIR-1T and CMY were also detected among the qnr-positive strains. Antimicrobial-resistance rates of qnr-positive strains to ciprofloxacin, levofloxacin, norfloxacin, nalidixic acid, and moxifloxacin were 51.1%, 46.8%, 46.8%, 74.5%, and 53.2%, respectively. CONCLUSIONS: The qnr genes were highly prevalent in Enterobacteriaceae, primarily the qnrB subtypes. They were closely associated with EBSL and AmpC beta-lactamase.

Non-invasive prediction of clinical infection in women with preterm labor / 대한산부인과학회지

OBJECTIVE: To develop a model based on non-invasive variables to predict the probability of subsequent maternal and/or neonatal clinical infection in women admitted to hospital for preterm labor with intact membranes. METHODS: Transvaginal ultrasound for measurement of cervical length was performed and maternal blood was collected for the determination of white blood cell (WBC) count at admission in 165 consecutive women with preterm labor (between 20.0 and 35.0 weeks). Clinical infection was defined as the presence of clinical chorioamnionitis at delivery or early onset neonatal sepsis. Receiver operating characteristic (ROC) curves and logistic regression analysis were used for statistical analyses. RESULTS: The prevalence of clinical infection was 5% (8/165). Women who developed clinical infection had a significantly lower median gestational age at admission, a lower shorter median cervical length, and a higher median WBC count as compared to those who did not develop clinical infection. Logistic regression analysis was performed and a final model was chosen, which included maternal blood WBC, cervical length, and gestational age as the best predictors of clinical infection. A risk score was calculated containing these 3 variables for each patient. The model was shown to have an adequate goodness of fit (P=0.202), and the area under the ROC curve was 0.822, indicating reasonably good discrimination. CONCLUSION: In women admitted to hospital for preterm labor with intact membranes, the risk for the subsequent maternal and/or neonatal clinical infection can be predicted non-invasively with a risk score based on cervical length at admission, maternal blood WBC, and gestational age.

A case of borderline clear cell fibroadenoma / 대한산부인과학회지

Clear cell tumor of borderline malignancy has been reported to account for 5 to 8% of all clear cell neoplasms, and <1% of borderline malignancies of the ovary. Borderline clear cell fibroadenoma with or without microinvasion mostly occurs in women with middle age, and the presenting symptoms associated with this tumor are non-specific. The clinico-pathologic features of borderline clear cell fibroadenoma are not well known because of its rarity. Furthermore, there is no consensus for treating the disease. Thus, we report a case of borderline clear cell fibroadenoma presenting as ovarian fibroadenoma with a review of literature.

A variant of complete septate uterus with double cervix and vagina: A case report / 대한산부인과학회지

Rare mullerian anomalies without any present classification were sometimes reported. A 30-year-old nulligravid woman was referred to our hospital with 2-year history of primary infertility. Laparoscopic examination revealed a relatively intact uterine fundus with both patent fallopian tubes. Hysteroscopic exam confirmed the presence of double vagina and cervix, as well as complete uterine septum with opening at the lower segment. Hysteroscopic septotomy was successfully performed through the right-sided cervix. A variant of complete septate uterus with double cervix that communicated at the isthmic portion could be successfully treated by hysteroscopic operation.

Clinical and sonographic predictors for successful labor induction in preterm women / 대한산부인과학회지

OBJECTIVE: To identify the clinical and sonographic parameters which predict the likelihood of successful labor induction in preterm women. METHODS: This prospective observational study enrolled 103 consecutive preterm women (<37 weeks gestation) with singleton gestations scheduled for induction of labor. Transvaginal ultrasound for measurement of the cervical length was performed and the Bishop score was determined by digital examination. The parameters studied were gestational age at induction, parity, maternal age, Bishop score, sonographic cervical length, and current body mass index (BMI). Univariate and multivariate statistical methods were used for data analysis. RESULTS: Successful induction of labor occurred in 45 (44%) of women. Multiple logistic regression analysis identified parity, maternal BMI, and gestational age at induction as the independent predictors of successful labor induction in preterm women, although gestational age did not reach statistical significance (P=0.056). However, maternal age, sonographic cervical length, and Bishop score did not provide independent contribution to success of induction. CONCLUSION: In preterm women undergoing induction of labor parity, maternal BMI, and gestational age at induction were independent parameters in predicting successful induction of labor. However, sonographic cervical length and Bishop score had poor predictive values for success of labor induction.

A case of primary transitional cell carcinoma of the ovary / 대한산부인과학회지

Primary ovarian transitional cell carcinoma (TCC) is extremely rare type of tumor and resembles transitional cell carcinoma of the bladder. Primary ovarian TCC has been classified as a different subtype from malignant Brenner tumor for it's histologic and clinical characteristics. It usually presents at an advanced stage .Though more aggressive than malignant Brenner tumor, it shows more favorable prognosis because of better response to the chemotherapy than other epithelial ovarian carcinomas. We experienced a case of primary ovarian transitional cell carcinoma in a premenopausal woman who underwent staging operation and chemotherapeutic treatment, and herein report the case with a brief review of related literatures.

Clinical study on obstetric variables affecting perinatal mortality in placental abruption / 대한산부인과학회지

OBJECTIVE: To evaluate obstetric variables in the placental abruption that affect on perinatal mortality. METHODS: We reviewed clinical data of all singleton deliveries complicated with placental abruption between January 2000 and December 2007, in Department of Ob. & Gyn., Sanggye Paik Hospital. RESULTS: Placental abruption complicated 54 cases (0.55%) of all deliveries (n=9,903) from January 2000 to December 2007. The peak age was 26-30 years (42.1%). The most common symptom was vaginal bleeding (57.4%) and intrauterine fetal death had already occurred in 9.3%. Perinatal mortality rate was 13.0% and there was no maternal death. 38.9% of placental abruption occurred between 33 to 36weeks of gestational age. 38.9% of placental abruption was diagnosed before delivery with ultrasonography and 9.3% was chronic placental abruption. 33.3% of placental abruption was associated with preeclampsia, and then associated with PPROM (24.1%), uterine myoma (3.7%), chronic hypertension (1.9%) and smoking (1.9%). When the hypertensive disorders were associated, fetal distress rate was higher than normotensive pregnancy (63.2% vs 20.0%, P-value: 0.005). Mean gestational age (days) (194+/-32.8 vs 248.12+/-28.0, P-value<0.001) and the severity of placental abruption (P-value: 0.005) affect perinatal mortality in placental abruption. The most common complication of placental abruption was DIC (16 cases, 29.6%), followed by Couvelaire uterus (4 cases, 7.4%) and uterine rupture (1 case, 1.9%). CONCLUSION: Obstetric variables that affect perinatal mortality were gestational age at the diagnosis and the severity of placental abruption.

A Case of Posterior Reversible Encephalopathy Syndrome with Post Streptoccocal Glomerulonephritis / 대한소아신경학회지

Posterior Reversible Encephalopathy Syndrome(PRES) mainly develops in patients under immunosuppressive therapy after transplantation, or patients who suffer hemato-oncologic diseases, eclampsia, acute hypertensive encephalopathy related with nephrotic disease. Sudden headache, nausea, mental derangement, convulsion, vision problems are the main symptoms shown in PRES. It typically shows edema of occipito-parietal area of cortex or subcortex in Magnetic Resonance Imaging(MRI). A 10-year-old male was hospitalized with the chief complaint of headache, vomiting and dizziness for 3 days. He was treated for upper respiratory infection for 1 week before the hospitalization. Initial blood pressure was 145/95 mmHg, which was high for his age. Uninalysis showed microscopic hematuria(3+). The hypertension persisted even after the hospitalization, and re-checked blood pressure was 175/115 mmHg. The patient complained of headache, and after that, he suffered from alteration of mental status with dysarthria and generalized type of seizure events. The MRI showed lesions suggestive of PRES. He recovered to alertmental status after the blood pressure was controlled. Antistreptolysin-O(ASO) titer was increased and complement 3(C3) titer was decreased. The follow up MRI taken 1 month after the event became normal. We report a case of PRES related with contemporary hypertensive event in Acute Post-Streptococcal Glomerulonephritis(APSGN).

A Premature Infant Case on the Treatment of Candidemia, Candidal Meningitis and Multiple Brain Microabscesses with Amphotericin B and Fluconazole

Identified risk factors for neonatal candidemia are low-birth weight, use of a central venous catheter, parenteral nutrition, and broad spectrum antibiotics. Candidemia is also the source of considerable morbidity endophthalmitis, meningitis, brain abscess, endocarditis, and renalare all examples of the potential consequences of candidemia abscess. In this study, we report a premature infant case whose candidemia involving candidal meningitis and multiple brain microabscesses was completely remedied through antifungal therapy without any onset of neurodevelopmental disability.

Suppression of VEGF and STAT3 by Lipoic acid in Experimental Diabetic Rat Retina

PURPOSE: We evaluated whether lipoic acid as antioxidant could inhibit expression of VEGF and STAT3 in experimental diabetic rat retina. METHODS: Diabetes was induced chemically by injection of streptozotocin in 12 rats of 18 Sprague-Dawley rats. After induction of diabetes, lipoic acid was injected into the peritonium in 6 rats. So all rats were divided into 3 groups, normal group (n=6), diabetes mellitus (DM) group (n=6), lipoic acid treated group (n=6). The ocular tissue of the rats were collected on 8 weeks after diabetes induction. Difference of VEGF and STAT3 expression was investigated by immunohistochemistry, RT-PCR, western blot. The change of VEGF and STAT3 in lipoic acid treated group were evaluated in these experimental model. RESULTS: The VEGF and STAT3 expression was elevated in diabetic rat retina. The active form STAT3, phosphorylated STAT3 was also elevated. The VEGF and STAT3 expression in lipoic acid treated group was lower than DM group. CONCLUSIONS: The lipoic acid could inhibit the VEGF and STAT3 expression in diabetic rat retina.

Effect of Rhodiola Sachalinensis Administration and Endurance Exercise on Insulin Sensitivity and Expression of Proteins Related with Glucose Transport in Skeletal Muscle of Obese Zucker Rat

Peripheral insulin resistance in obese/type II diabetes animals results from an impairment of insulin-stimulated glucose uptake into skeletal muscle. Insulin stimulate the translocation of GLUT4 from intracellular location to the plasma membrane. Soluble N-ethylmaleimide-sensitive factor attachment protein receptors (SNAREs) is implicated in mediation of fusion of GLUT4-containing vesicle with the plasma membrane. Present study investigated regulatory effects of Rhodiola sachalinensis administration and exercise training on the expression of GLUT4 protein and SNAREs protein in skeletal muscles of obese Zucker rats. Experimental animals were randomly assigned into one of five groups ; lean control (LN), obese control (OB), exercise-treated (EXE), Rhodiola sachalinensis-treated (Rho), combine of Rho & EXE (Rho-EXE). All animals of exercise training (EXE, Rho-EXE) performed treadmill running for 8 weeks, and animals of Rho groups (Rho, Rho-EXE) were dosed daily by gastric gavage during the same period. After experiment, blood were taken for analyses of glucose, insulin, and lipids levels. Mitochondrial oxidative enzyme (citrate synthase, CS ; beta-hydroxyacyl-CoA dehydrogenase, beta-HAD) activity were analysed. Skeletal muscles were dissected out for analyses of proteins (GLUT4, VAMP2, syntaxin4, SNAP23). Results are as follows. Exercise and/or Rhodiola sachalinensis administration significantly reduced body weight and improved blood lipids (TG, FFA), and increased insulin sensitivity. Endurance exercise significantly increased the activity of mitochondrial enzymes and the expression of GLUT4 protein, however, administration of Rhodiola sachalinensis did not affect them. The effect of exercise and/or Rhodiola sachalinensis administration on the expression of SNARE proteins was unclear. Our study suggested that improvement insulin sensitivity by exercise and/or Rhodiola sachalinensis administration in obese Zucker rats is independent of expression of SNARE proteins.

The Clinical Results of 6-mm Iris-Fixated Phakic IOL

PURPOSE: To evaluate the clinical results of 6 mm iris-fixated phakic intraocular lens (Artisan(R) lens) implantation in myopic patients. METHODS: Forty eyes of 23 myopic patients underwent 6-mm Artisan(R) phakic IOL implantation and were followed-up for over 6 months. We prospectively analyzed the efficacy, stability, predictability, change of astigmatism, endothelial cell count, pupil diameter, degree of decentration, subjective satisfaction and complications. RESULTS: The preoperative mean spherical equivalent was -9.46 D, and postoperative spherical equivalent was -0.70D at 1 month, -0.55D at 3 months, -0.54D at 6 months, -0.78D at 12 months and remained stable during the follow-up period. The preoperative mean astigmatism was -1.88D and postoperative astigmatism was -0.87D at 6 months. Postoperative uncorrected visual acuity (UCVA) was more than 0.8 in 85% of the eyes at 1 month, 91% at 3 months, 95% at 6 months, and 89% at 12 months. The spherical equivalent refraction after surgery was within 1.0D of emmetropia in 78% of eyes at 1 month, 80% at 3 months, 88% at 6 months, and 64% at 12 months. There was no significant decrease in the endothelial cell count during the follow-up period. Preoperative scotopic pupil diameters were significantly decreased at 1 month and the mean decentration of the lens was 0.38 mm. Patient satisfaction was generally high. Complications included the transient elevation of intraocular pressure in 1 eye, corneal edema in 4 eyes, iritis in 1 eye, traumatic dislocation in 1 eye and iris atrophy in 2 eyes. CONCLUSIONS: The 6-mm Artisan(R)phakic IOL implantation may be an effective surgical procedure for myopic patients who cannot undergo corneal refractive surgery, as it provided good visual results, predictability, patient satisfaction, and short-term safety.

A Case of Del 13(q24) Syndrome with Multiple Anomalies / 소아과

It has been estimated that chromosomal aberrations account for 2.3% to 3% of normal pregnancies, and of them, 85% are aborted. Therefore, the survival rate of neonates with chromosomal aberrations is very low. Among them, patients with partial deletion of the long arm of chromosome 13 are very rare. The natural history of deletion of the long arm is dependent on the deleted segment. It has been known that patients with proximal deletions not extending into q32 usually show mild to moderate mental retardation, variable minor anomalies, and growth retardation. Patients with more distal deletions, including at least part of q32, usually have severe mental retardation, growth deficiency, and major malformations including microcephaly and CNS defects, distal limb anomalies, eye defects, and gastrointestinal malformation. We report a case of a 13(q24) deletion male infant who showed intrauterine growth retardation, imperforate anus, CNS anomalies, hydronephrosis, clubfoot, clinodactyly and developmental delay, although his deletion site was proximal to q32.

Diagnosis and Clinical Features in Children Referred to Developmental Delay Clinic

OBJECTIVE: To determine the phenomenologic, etiologic diagnosis and clinical features of children with developmental delay. METHOD: One hundred seventy-one children, referred to Developmental Delay Clinic which was multidisciplinary clinic for the evaluation of a suspected developmental delay, were prospectively enrolled. Diagnostic yield was ascertained after the completion of the questionnaire, clinical assessments and laboratory investigations by the physiatrist, pediatric neurologist and pediatric psychiatrist. RESULTS: One hundred fifty-one children met study criteria. The common phenomenologic diagnoses of children with developmental delay were mental retardation, delayed language disorder, autism and cerebral palsy. The etiologic diagnosis was determined in 44 (28.02%) children. The diagnoses were hypoxic-ischemic encephalopathy, malformation of cortical development, dysmorphic syndrome, chromosomal abnormalities, and neuomuscular disorders in the order of frequency. In many cases, the chief complaint of parent was not in accordance with final diagnosis. CONCLUSION: In the clinical assessment and management of children with developmental delay, the most important thing is integrative and comprehensive approach including all the developmental territories. And also, the settlement of paradigm for systematic evaluation of these children with other specialists will be needed.

A Case of Microcephaly and Early-onset Nephrotic Syndrome: Galloway-Mowat Syndrome

The Galloway-Mowat syndrome, a rare inherited disorder, is characterized by congenital microcephaly with various neurological abnormalities and early onset of nephrotic syndrome with unresponsiveness to treatment, progressive deterioration in renal function and death in early lifetime. In this report, we describe a girl with microcephaly, seizures. and psychomotor retardation who developed nephrotic syndrome at 17 months of age.

A Case of Microcephaly and Early-onset Nephrotic Syndrome: Galloway-Mowat Syndrome

The Galloway-Mowat syndrome, a rare inherited disorder, is characterized by congenital microcephaly with various neurological abnormalities and early onset of nephrotic syndrome with unresponsiveness to treatment, progressive deterioration in renal function and death in early lifetime. In this report, we describe a girl with microcephaly, seizures. and psychomotor retardation who developed nephrotic syndrome at 17 months of age.