Hypoplastic Left Heart Syndrome with Ventricular Septal Defect and Patent Arterial Duct (SNUCH CPC 95-3)

Nine days old male was admitted to the intensive care unit because of the jaundice and suspicious cardiac disease. He was delivered at 39 +5 weeks of gestation without peripartum events. His birth weight was 3.4 kg. He was the second baby with healthy brother. The family history was not contributory. He was initially transferred to the other hospital because of the jaundice at 2 days of age. Cardiac murmur was detected at that hospital. He was asymptomatic besides mild dyspnea. He was able to suck the milk of 70 ml per feeding. He was referred to another hospital because the echocardiographic examination showed a small left ventricular size

Juvenile Angiodysplasia of Gut

Angiodysplasia or arteriovenous malformation of the gastrointestinal tract in younger patients is different from "classic" angiodysplasia in older patients, by age of onset, clinical presentation, location of the lesion and diverse histologic features, We report two cases of juvenile angiodysplasia of the gut. The diagnosis was suspected by radionuclide blood pool scan and was confirmed by resection and pathological examination, Both cases were girls of 5 years and 12 years of age, and the lesions were in the jejunum and ileum, Grossly, one case showed multiple petechiae and another case showed a hemorrhagic polypoid mass, Microscopically, the lesions were composed of irregularly dilated vascular channels in mucosa and submucosa with abnormal proliferation of arteries and veins in submucosa, Both cases are free of recurrence after local resection

Colorectal Lymphoid Polyposis in a Child

Lymphoid polyposis is a lymphoid hyperplasia of the gastrointestinal tract that usually presents as multiple small polyps in the colon during childhood. This should be differentiated from other neoplastic or familial polyposis of the intestine. We report a case of benign lymphoid polyposis of the colon in a 17-month-old boy who presented with perianal fistula and mucosal ulceration. Colon study and rectal examinations showed multiple polyps in the sigmoid colon and rectum. Segmental resection of the sigmoid colon and rectum showed over 100 smallt 3 - 7 mrn) sessile or pedunculated polyps that were diffusely scattered through out the removed segment. The polyps consisted of mature lymphoid tissue with numerous germinal centers, that was located mostly in the lamina propria and submucosa

Visceral Myopathy of Intestinal Pseudoobstruction

Intestinal pseudoobstruction is a syndrome complex caused by a variety of disorders of various etiology. It can be classified pathologically as visceral myopathy and visceral neuropathy. The sporadic form of visceral myopathy is characterized histologically by vacuolar degeneration and fibrosis of smooth muscle but differs from the familial form only by the absence of other affected family members. We studied 6 cases with intestinal pseudoobstruction classified as sporadic visceral myopathy. They were four boys and two girls, and were two neonates, two infants and two children. The duration of symptoms ranged from two days to two years. Two babies were dead from pneumonia and sepsis. Others were alleviated after surgical resection of the bowel. Both small and large intestines were found affected in autopsy cases. Histopathologic features were vacuolar degeneration of muscularis propria, disproportionate hypoplasia of outer muscle layer, abnormal muscle direction of muscularis propria, submucosal and/or interstitial fibrosis and extra muscle layering. It is presumed that a variety of histopathologic features accounts for visceral myopathy of intestinal pseudoobstruction

Identification of MYC as an antinecroptotic protein that stifles RIPK1-RIPK3 complex formation

The underlying mechanism of necroptosis in relation to cancer is still unclear. Here, MYC, a potent oncogene, is an antinecroptotic factor that directly suppresses the formation of the RIPK1-RIPK3 complex. Gene set enrichment analyses reveal that the MYC pathway is the most prominently down-regulated signaling pathway during necroptosis. Depletion or deletion of MYC promotes the RIPK1-RIPK3 interaction, thereby stabilizing the RIPK1 and RIPK3 proteins and facilitating necroptosis. Interestingly, MYC binds to RIPK3 in the cytoplasm and inhibits the interaction between RIPK1 and RIPK3 in vitro. Furthermore, MYC-nick, a truncated form that is mainly localized in the cytoplasm, prevented TNF-induced necroptosis. Finally, down-regulation of MYC enhances necroptosis in leukemia cells and suppresses tumor growth in a xenograft model upon treatment with birinapant and emricasan. MYC-mediated suppression of necroptosis is a mechanism of necroptosis resistance in cancer, and approaches targeting MYC to induce necroptosis represent an attractive therapeutic strategy for cancer

Ethanol extract of Angelica gigas inhibits croton oil-induced inflammation by suppressing the cyclooxygenase - prostaglandin pathway

The anti-inflammatory effects of an ethanol extract of Angelica gigas (EAG) were investigated in vitro and in vivo using croton oil-induced inflammation models. Croton oil (20 µg/mL) up-regulated mRNA expression of cyclooxygenase (COX)-I and COX-II in the macrophage cell line, RAW 264.7, resulting in the release of high concentrations of prostaglandin E2 (PGE2). EAG (1~10 µg/mL) markedly suppressed croton oil-induced COX-II mRNA expression and PGE2 production. Application of croton oil (5% in acetone) to mouse ears caused severe local erythema, edema and vascular leakage, which were significantly attenuated by oral pre-treatment with EAG (50~500 mg/kg). Croton oil dramatically increased blood levels of interleukin (IL)-6 and PGE2 without affecting tumor-necrosis factor (TNF)-α and nitric oxide (NO) levels. EAG pre-treatment remarkably lowered IL-6 and PGE2, but did not alter TNF-α or NO concentrations. These results indicate that EAG attenuates inflammatory responses in part by blocking the COX-PGE2 pathway. Therefore, EAG could be a promising candidate for the treatment of inflammatory diseases

Effects of a safflower tea supplement on antioxidative status and bone markers in postmenopausal women

We conducted this study to examine the effects of safflower seed granular tea containing physiologically active polyphenols on antioxidative activities and bone metabolism. Forty postmenopausal women ages 49 to 64-years were recruited from Daegu and Gyeongbuk and were randomly assigned to either a safflower tea supplement (Saf-tea) group (n = 27) or a placebo group (n = 13). The Saf-tea group received 20 g of safflower seed granule tea per day containing a 13% ethanol extract of defatted safflower seeds, whereas the placebo group received a similar type of tea that lacked the ethanol extract. No significant changes in nutrient intake for either the placebo or Saf-tea groups were observed before or after the study period, except vitamin A intake increased after 6 months in the Saf-tea group. Dietary phytoestrogen intakes were similar in the Saf-tea group (60.3 mg) and placebo group (52.5 mg). Significant increases in plasma genistein and enterolactone were observed in the Saf-tea group. After 6 months of supplementation, serum levels of antioxidant vitamins such as α-tocopherol and ascorbic acid increased significantly, and TBARS levels decreased in the Saf-tea group compared to the placebo group. Serum osteocalcin levels were reduced (P < 0.05) in the Saf-tea group after 6 months, whereas serum osteocalcin did not change in the placebo group. Urinary deoxypyridinoline/creatinine excretion was not different between the two groups at baseline, and did not change in either group after 6 months. Bone mineral density decreased significantly in the placebo group (P < 0.01) but not in the supplemented group. It was concluded that polyphenols (72 mg/day), including serotonin derivatives, in the Saf-tea had both antioxidant and potential bone protecting effects in postmenopausal women without liver toxicity

Why Are Outcomes Different for Registry Patients Enrolled Prospectively and Retrospectively? Insights from the Global Anticoagulant Registry in the FIELD-Atrial Fibrillation (GARFIELD-AF).

Background: Retrospective and prospective observational studies are designed to reflect real-world evidence on clinical practice, but can yield conflicting results. The GARFIELD-AF Registry includes both methods of enrolment and allows analysis of differences in patient characteristics and outcomes that may result. Methods and Results: Patients with atrial fibrillation (AF) and ≥1 risk factor for stroke at diagnosis of AF were recruited either retrospectively (n = 5069) or prospectively (n = 5501) from 19 countries and then followed prospectively. The retrospectively enrolled cohort comprised patients with established AF (for a least 6, and up to 24 months before enrolment), who were identified retrospectively (and baseline and partial follow-up data were collected from the emedical records) and then followed prospectively between 0-18 months (such that the total time of follow-up was 24 months; data collection Dec-2009 and Oct-2010). In the prospectively enrolled cohort, patients with newly diagnosed AF (≤6 weeks after diagnosis) were recruited between Mar-2010 and Oct-2011 and were followed for 24 months after enrolment. Differences between the cohorts were observed in clinical characteristics, including type of AF, stroke prevention strategies, and event rates. More patients in the retrospectively identified cohort received vitamin K antagonists (62.1% vs. 53.2%) and fewer received non-vitamin K oral anticoagulants (1.8% vs . 4.2%). All-cause mortality rates per 100 person-years during the prospective follow-up (starting the first study visit up to 1 year) were significantly lower in the retrospective than prospectively identified cohort (3.04 [95% CI 2.51 to 3.67] vs . 4.05 [95% CI 3.53 to 4.63]; p = 0.016). Conclusions: Interpretations of data from registries that aim to evaluate the characteristics and outcomes of patients with AF must take account of differences in registry design and the impact of recall bias and survivorship bias that is incurred with retrospective enrolment. Clinical Trial Registration: - URL: http://www.clinicaltrials.gov . Unique identifier for GARFIELD-AF (NCT01090362)

Risk profiles and one-year outcomes of patients with newly diagnosed atrial fibrillation in India: Insights from the GARFIELD-AF Registry.

BACKGROUND: The Global Anticoagulant Registry in the FIELD-Atrial Fibrillation (GARFIELD-AF) is an ongoing prospective noninterventional registry, which is providing important information on the baseline characteristics, treatment patterns, and 1-year outcomes in patients with newly diagnosed non-valvular atrial fibrillation (NVAF). This report describes data from Indian patients recruited in this registry. METHODS AND RESULTS: A total of 52,014 patients with newly diagnosed AF were enrolled globally; of these, 1388 patients were recruited from 26 sites within India (2012-2016). In India, the mean age was 65.8 years at diagnosis of NVAF. Hypertension was the most prevalent risk factor for AF, present in 68.5% of patients from India and in 76.3% of patients globally (P < 0.001). Diabetes and coronary artery disease (CAD) were prevalent in 36.2% and 28.1% of patients as compared with global prevalence of 22.2% and 21.6%, respectively (P < 0.001 for both). Antiplatelet therapy was the most common antithrombotic treatment in India. With increasing stroke risk, however, patients were more likely to receive oral anticoagulant therapy [mainly vitamin K antagonist (VKA)], but average international normalized ratio (INR) was lower among Indian patients [median INR value 1.6 (interquartile range {IQR}: 1.3-2.3) versus 2.3 (IQR 1.8-2.8) (P < 0.001)]. Compared with other countries, patients from India had markedly higher rates of all-cause mortality [7.68 per 100 person-years (95% confidence interval 6.32-9.35) vs 4.34 (4.16-4.53), P < 0.0001], while rates of stroke/systemic embolism and major bleeding were lower after 1 year of follow-up. CONCLUSION: Compared to previously published registries from India, the GARFIELD-AF registry describes clinical profiles and outcomes in Indian patients with AF of a different etiology. The registry data show that compared to the rest of the world, Indian AF patients are younger in age and have more diabetes and CAD. Patients with a higher stroke risk are more likely to receive anticoagulation therapy with VKA but are underdosed compared with the global average in the GARFIELD-AF. CLINICAL TRIAL REGISTRATION-URL: http://www.clinicaltrials.gov. Unique identifier: NCT01090362

Improved risk stratification of patients with atrial fibrillation: an integrated GARFIELD-AF tool for the prediction of mortality, stroke and bleed in patients with and without anticoagulation.

OBJECTIVES: To provide an accurate, web-based tool for stratifying patients with atrial fibrillation to facilitate decisions on the potential benefits/risks of anticoagulation, based on mortality, stroke and bleeding risks. DESIGN: The new tool was developed, using stepwise regression, for all and then applied to lower risk patients. C-statistics were compared with CHA2DS2-VASc using 30-fold cross-validation to control for overfitting. External validation was undertaken in an independent dataset, Outcome Registry for Better Informed Treatment of Atrial Fibrillation (ORBIT-AF). PARTICIPANTS: Data from 39 898 patients enrolled in the prospective GARFIELD-AF registry provided the basis for deriving and validating an integrated risk tool to predict stroke risk, mortality and bleeding risk. RESULTS: The discriminatory value of the GARFIELD-AF risk model was superior to CHA2DS2-VASc for patients with or without anticoagulation. C-statistics (95% CI) for all-cause mortality, ischaemic stroke/systemic embolism and haemorrhagic stroke/major bleeding (treated patients) were: 0.77 (0.76 to 0.78), 0.69 (0.67 to 0.71) and 0.66 (0.62 to 0.69), respectively, for the GARFIELD-AF risk models, and 0.66 (0.64-0.67), 0.64 (0.61-0.66) and 0.64 (0.61-0.68), respectively, for CHA2DS2-VASc (or HAS-BLED for bleeding). In very low to low risk patients (CHA2DS2-VASc 0 or 1 (men) and 1 or 2 (women)), the CHA2DS2-VASc and HAS-BLED (for bleeding) scores offered weak discriminatory value for mortality, stroke/systemic embolism and major bleeding. C-statistics for the GARFIELD-AF risk tool were 0.69 (0.64 to 0.75), 0.65 (0.56 to 0.73) and 0.60 (0.47 to 0.73) for each end point, respectively, versus 0.50 (0.45 to 0.55), 0.59 (0.50 to 0.67) and 0.55 (0.53 to 0.56) for CHA2DS2-VASc (or HAS-BLED for bleeding). Upon validation in the ORBIT-AF population, C-statistics showed that the GARFIELD-AF risk tool was effective for predicting 1-year all-cause mortality using the full and simplified model for all-cause mortality: C-statistics 0.75 (0.73 to 0.77) and 0.75 (0.73 to 0.77), respectively, and for predicting for any stroke or systemic embolism over 1 year, C-statistics 0.68 (0.62 to 0.74). CONCLUSIONS: Performance of the GARFIELD-AF risk tool was superior to CHA2DS2-VASc in predicting stroke and mortality and superior to HAS-BLED for bleeding, overall and in lower risk patients. The GARFIELD-AF tool has the potential for incorporation in routine electronic systems, and for the first time, permits simultaneous evaluation of ischaemic stroke, mortality and bleeding risks. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier for GARFIELD-AF (NCT01090362) and for ORBIT-AF (NCT01165710)