Suwon Catholic University

Umbilical cord cysts are relatively common which are identified in approximately 1% of first trimester sonograms. However, such cysts coexisting with patent urachus are rare. We present a case of patent urachus misdiagnosed as an umbilical cord cyst measured 32 x 24mm at 17 weeks of gestation which gradually decreased in size and disappeared after 25 weeks of gestation. An urachus is a remnant of allantois in embryo development constituting a connection between the dome of the bladder and umbilicus. A patent urachus occurs when the entire urachus fails to close during embryonic period and is a rare anomaly with an incidence of 1∼2.5 per 100,000. The prenatal diagnosis of patent urachus is challenging, however, in distinct cases, prenatal sonographic findings of an opening between the bladder and the umbilical cord cyst, megacystis and a large umbilical cord cyst may be key features of diagnosis (figure 1). We present a case of patent urachus misdiagnosed as an umbilical cord cyst which disappeared in the second trimester, however postnatally found to be a patent urachus. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Introduction: Endoscopic remission has been recognized as the treatment goal of ulcerative colitis (UC). However, microscopic inflammation exists even in endoscopically normal mucosa. Fecal calprotectin (FC) reflects low-grade inflammation which cannot be detected on endoscopy. We aim to investigate fecal calprotectin as a biomarker in predicting the relapse in UC patients with endoscopic remission. Methods: We performed a prospective study of patients with UC in endoscopic remission (Mayo endoscopic subscore of 0) from March 2014 to February 2016. Stool sample was collected near the time of the endoscopy and FC measured using a quantitative enzyme-linked immunosorbent assay. Patients were followed until the last outpatient clinic visit or the development of a relapse. Results: Of the 33 patients in endoscopic remission, 7/33 (21%) relapsed after a median follow-up of 8 months (range 2-20 months). FC was significantly higher in patients who relapsed compared with patients who maintained remission (148 mcg/g vs. 70 mcg/g; p = 0.039). A receiver operating characteristic analysis estimated a cutoff level of > 135mcg/g for FC (area under the curve, 0.758 and confidence interval 95%, 0.579-0.937) for predicting relapse during follow-up with 71.4% sensitivity, 76.9% specificity, 85.7% positive predictive value and 76.9% negative predictive value. Conclusion: High FC levels can predict relapse in patients with UC. Therefore, FC measurement should be considered in UC patients, even if they have endoscopic remission.

Gastric cancer is supposed to be a result of inflammation induced by Helicobacter pylori (H. pylori) infection. Nucleotide-binding oligomerization domain 1 (NOD 1) is required for the innate immune response to H. pylori. We aim to investigate whether single nucleotide polymorphism (SNP) in NOD 1 gene is associated with H. pylori-induced gastric mucosal inflammation in a healthy Korean population. The study was conducted on 412 adults who visited two different healthcare centers for health examinations. The G796A (E266K) NOD 1 SNP was detected by using polymerase chain reaction/restriction fragment length polymorphism. A gastritis score was calculated by the summed values of the grade and the activity of gastritis scored according to the updated Sydney system. The expression of IL-8 and COX-2 mRNA was assessed by quantitative reverse transcription polymerase chain reaction. In the group with H. pylori infection, the complete screening of the genes comprising the cag PAI was performed. The genotype frequencies were 26.7% (AA type), 58.3% (GA), and 15.0% (GG). In H. pylori-positive patients, gastritis score of the AA genotype was significantly higher than those of the others (p = .04). Also, the IL-8 and COX-2 mRNA levels increased in the AA genotype. In the group with H. pylori infection, 31.9% were found to carry the complete cag PAI. When the subjects were infected with intact cag PAI, the IL-8 and COX-2 mRNA levels were significantly high in AA genotype. G796A (E266K) NOD 1 polymorphism is closely correlated with H. pylori-associated gastric mucosal inflammation in the Korean population.

To evaluate the antenatal cord coiling index obtained at 22 ∼ 28 weeks of gestation as a predictor of perinatal outcomes. Two hundred twenty six women who had a fetal anatomic survey 22 to 28 weeks of gestation were evaluated for umbilical cord coiling. The antenatal umbilical cord coiling index was calculated as a reciprocal value of distance between a pair of coils (cord coiling index = 1/distance in cm) and was correlated with the following adverse pregnancy outcomes. Total 226 patients were included. The average delivery weeks of the hypocoiled cord group was 36.8 ± 2.34 weeks, which was shorter than 38.3 ± 1.82 weeks of the normocoiled cord group or 38.9 ± 1.72 weeks of the hypercoiled cord group (P = 0.02). In regard to premature delivery cases before 37 weeks, similarly, cases with the hypocoiled cord was 36.4%, and a premature delivery rate higher than 7.7% of the normocoiled cord group or 16.7% of the hypercoiled cord group was shown (P = 0.041). The incidence of low birth weight neonates (lower than 2,500 g) in the hypocoiled cord group was 36.4%, and it was significantly higher than 10% of the normocoiled cord group and 20% of the hypercoiled cord group (P = 0.044). Admission to the neonatal intensive care unit in the hypocoiled cord group was 27.3%, and in comparison with 6.8% of the normocoiled cord group and 0% of the hypercoiled cord group, a statistically significantly higher admission rate was shown (P = 0.041). Cases who were treated for hyperbilirubinemia after birth in the hypocoiled cord group were 54.5%, the normocoiled cord group were 20.5%, and the hypercoiled cord group were 30%, and it was statistically significantly high (P = 0.044). When the gestational weeks at birth were adjusted, low birthweight neonates, admission to the intensive care unit and the incidence of hyperbilirubinemia were not statistically significant. When the umbilical cord coiling index was measured in the late second semester of gestation, the hypocoiled cord was associated with preterm labor.

It has not been explicitly addressed whether new biomarkers, in addition to readily available clinical data, make a contribution to the prediction of liver fibrosis. A total of 209 patients with chronic hepatitis B who underwent liver biopsy were recruited. The Clinical Score (CS) model using only routine clinical data and the Biomarker Score (BS) model using 7 putative biomarkers were derived from the derivation set (n=105), and these models were applied to a separate patients group (n=104) to investigate whether the addition of BS improved the diagnostic accuracy in predicting significant fibrosis beyond an assessment based solely on CS. The most informative biomarkers for predicting significant fibrosis were hyaluronic acid and matrix metalloproteinase-2. The BS was an independent predictive factor for significant fibrosis even after accounting for CS in both sets. Among the derivation set, the incorporation of the BS into the CS model did not significantly increase the receiver operating characteristic area, with only a small improvement of about 2% (P=0.11). Similarly, in the validation set, a combined model with CS and BS showed no superior diagnostic accuracy over the CS model alone, with an improvement of approximately 2% [0.83 (0.75 to 0.92) vs. 0.81 (0.70 to 0.91); P=0.16]. The simultaneous addition of several biomarkers adds only modestly to clinical predictive factors for risk assessment of individual patients. These results highlight the need for the models to be validated in another cohort with a broader distribution of fibrosis severity.

Objective. To determine the degree and risk factors for decreased bone mineral density (BMD) in patients with systemic lupus erythematosus (SLE). Methods. One hundred and one patients with SLE and 57 age- and gender- matched healthy controls were enrolled in this study. The BMD was measured by dual energy X-ray absorptiometry (DXA). The laboratory findings and clinical variables evaluated in the SLE patients consisted of disease duration, SLE disease activity index (SLEDAI), and medications, including mean and cumulative dose of glucocorticoid. At the time of the clinical and laboratory assessment, the levels of serum osteocalcin, serum FSH/ LH, urine deoxypyridinoline (DPD), and serum cytokines, such as IL-6 and soluble receptor activator of NF-kB ligand (RANKL), were determined in SLE patients using a enzyme-linked immunosorbent assay. Results. The BMD T score decreased in patients with SLE compared to the healthy controls (−1.11 versus −0.41, p=0.001 at lumbar spine, −0.84 versus −0.01, p＜0.001 at femur neck, −1.20 versus −0.45, p＜0.001 at total hip, respectively). Osteoporosis and osteopenia was present in 16.8% and 46.5% of patients, respectively. Multiple regression analysis revealed a low BMD in the lumbar spine to be associated with increased FSH, low BMI and cumulative glucocorticoid dose. A low BMD in the hip and femur neck was associated with increased FSH, low BMI, and duration of glucocorticoid. On the other hand, the levels of osteocalcin, deoxypyridinoline (DPD), IL-6, and soluble RANKL were similar in patients with a low BMD and those with normal BMD. Conclusion. Osteoporosis and osteopenia are more common in young Korean SLE patients than in control subjects. Elevated FSH, low BMI, and the use of glucocorticoid are independent risk factors linked to a decreased BMD in Korean patients with SLE.

Conclusions: Ramsay Hunt syndrome (RHS) with multiple involvement of cranial nerves is more severe and intractable than RHS without such involvement. Objectives: Typically, RHS involves VII and VIII nerves and unilaterally, and RHS accompanied by multiple cranial neuropathy is very rare. We describe 11 patients who developed RHS with multicranial nerve involvement and we analyzed their clinical characteristics and compared them with those of patients with RHS not accompanied by multiple cranial neuropathy. Methods: During the period 1995–2009, we treated 339 patients with RHS; of these, 11 patients had concurrent multiple cranial neuropathy. We assessed the clinical characteristics of RHS patients with and without multiple cranial neuropathy. Results: The mean age of the 11 patients with multiple cranial neuropathy (6 men, 5 women) was 49.2 ± 19.4 years, although 7 were aged 50 years or older. Eight patients had right-sided and three had left-sided facial paralysis. The initial degree of facial paralysis was House-Brackmann (HB) grade IV in four patients (36.4%) and HB grade V in seven (63.6%). Six patients showed improvement in symptoms, whereas five (45.6%) showed no improvement. The recovery rates from facial paralysis in patients with and without multiple cranial neuropathy were 54.5% and 82.9%, respectively, and the complete recovery rates were 27.3% and 67.7%, respectively.

The purpose of this study was to describe the prenatal sonographic features of ilojejunal atresia. We identified neonates with surgically confirmed ilojejunal atresia that had prenatally sonography in our institution from January 1, 1995, to June 30, 2009. Prenatal sonography images and reports were reviewed. 13 neonates were surgically confirmed ileojejunal atresia in our institution for five years. Three fetuses (23.1%) did not showed antenatally any intestinal abnormalities on the ultrasonography. Ten fetuses (76.9%) showed various sonographic features of ileojejunal atresia including multiple dilated bowel loops, ascites, cystic lesions, polyhydramnios with or without associated anomalies. Six of ten fetuses showed multiple dilated bowel loops and two fetuses showed ascites. Two fetuses showed a large cystic mixed ehogenic bowel dilatation. In one fetus, a large ehogenic bowel dilatation was showed at 38 weeks of gestation, which was initially sinlge small anehogenic cyst and gradually increased in size during the second trimester and no visualized in the late third trimester. Hyperehogenic bowel was showed in another fetus at early second trimester. However, the sign was disappeared and a large cystic echogenic bowel dilatation was suddenly showed at 28 weeks of gestation. Polyhydroamnios was associated in two fetuses. Heart anomaly, kidney anomaly or hydrops were associated in four fetuses. The prenatal sonographic features of ilojejunal atresia is various. The most common sonographic feature is multiple dilated bowel loops. However, although there are not any clue for ilojejunal atresia, and if a small cyst or hyperechogenic bowel was showed in early second trimester, there is some possibility of ileojejunal atresia.