Adult Pelvic Sarcomas: A Heterogeneous Collection of Sarcomas?

Introduction. Adult pelvic soft tissue sarcomas are a rare group of heterogeneous malignancies. These sarcomas differ from extremity and trunk soft tissue sarcomas in presentation, characteristics and response to treatment

The impact of diagnosis and trait anxiety on psychological distress in women with early stage breast cancer: A prospective study

ObjectivesHigh trait anxiety (HTA) determines depressive symptoms and state anxiety in women with breast cancer (BC) or benign breast disease (BBD). Before implementing screening for psychological counselling in these women, it is important to evaluate whether high state anxiety and depressive symptoms are determined by (the threat of) having BC or solely by HTA. Therefore, we compared women with a lump in the breast with women with gallstone disease (GD). MethodsWomen diagnosed with BC (n=152), BBD (n=205), and GD (n=128) were included in a prospective longitudinal study. Questionnaires concerning trait anxiety (baseline), state anxiety, and depressive symptoms were completed before diagnosis was known (BC and BBD) or the laparoscopic cholecystectomy and 6months later. ResultsPre-diagnosis BC patients scored higher on state anxiety (p=.001) and depressive symptoms (p ConclusionsThe severity of diagnosis (BC) in combination with HTA determined the level of state anxiety and depressive symptoms. Therefore, we recommend identifying women with HTA and offering them a tailor-made follow-up protocol during and after the diagnostic process for BBD or BC

Early Detection of Neonatal Cholestasis: Inadequate Assessment of Stool Color by Parents and Primary Healthcare Doctors

IntroductionEarly diagnosis and surgery (<60 days of age) improve outcomes in children with biliary atresia. Only 56% of patients undergo timely surgery in the Netherlands. Lack of acquaintance with symptoms such as discolored stools might underlie this delay. We analyzed whether Dutch parents, youth healthcare doctors, or general practitioners recognized discolored stools and evaluated the effect of the Infant Stool Color Card (ISCC) on recognizing discolored stools. Material and MethodsWe asked 100 parents, 33 youth healthcare doctors, and 50 general practitioners to classify photographs of stools as normal or abnormal. Subsequently, we asked whether parents would seek medical help and doctors would refer the patient for medical investigation. Finally, parents scored stools using the ISCC. ResultsTwo-third of both parents and youth healthcare doctors recognized all discolored stools. Only half of them would seek medical help for all discolored stools resp. refer patient for medical investigation. Only one-third of the general practitioners recognized all discolored stools and would refer for medical investigation for all discolored stools. Using the ISCC, the percentage of parents recognizing all discolored stool increased from 66 to 87% (p ConclusionNeither parents nor youth healthcare doctors nor general practitioners reliably recognize discolored stool. The ISCC is an effective screening method for discolored stool. Our data indicate that the ISCC should be accompanied by unequivocal advices regarding referral for medical investigation upon detection of discolored stools

Necrotizing Enterocolitis in a Dutch Cohort of Very Preterm Infants: Prevalence, Mortality, and Long-Term Outcomes

INTRODUCTION:  To improve counseling of parents and to guide care strategies, we studied the disease course and outcomes of necrotizing enterocolitis (NEC) up to 2 years of corrected age (CA) from a multidisciplinary perspective. MATERIALS AND METHODS:  This was a retrospective cohort study in preterm infants (birth weight < 1,500 g, gestational age < 32 weeks), diagnosed with NEC (Bell's stage ≥ II) from 2008 through 2020. Data on prevalence, mortality, surgery, intestinal failure (IF), growth, and neurodevelopment at 2-year follow-up were separately analyzed for medically and surgically treated children. RESULTS:  Of 3,456 preterm infants, 200 (6%) were diagnosed with NEC, of whom 135 developed an indication for surgery within 7 days after the diagnosis; 28/135 died before surgery, and 37/107 died after an open-and-close procedure. An enterostomy was constructed in 62 patients and an end-to-end anastomosis in 15. The postoperative course was described for 77 patients, of whom 23 developed surgical complications (12/23 incisional hernias, 9/23 anastomotic strictures), 13/77 a short bowel, and 25/77 IF. Sixty-day survival after birth for medical NEC patients was 88% (hazard ratio [HR]: 0.698; p = 0.318), and for surgically treated NEC patients was 40% (HR: 3.729; p < 0.001). At 2-year follow-up, one patient received parenteral nutrition. Severe delay in weight for age, motor, and cognitive development was seen in 3, 6, and 2%, respectively. CONCLUSION:  In this cohort, the mortality rate was high, especially in surgically treated NEC patients. The surgical complication rate is comparable to previous studies, but in surviving patients, persisting IF and severe delay in growth and neurodevelopment at 2 years CA were relatively rare

Liver fibrosis during the development of biliary atresia:Proof of principle in the murine model

Background: The murine model of biliary atresia (BA) is used for examining the pathogenesis of BA. The aim of the study was description of the morphological features and illustrating the detailed development of fibrosis using the Biliary Atresia Research Consortium (BARC) system. Methods: Neonatal mice were injected intraperitoneally with rhesus rotavirus (RRV) strain (N = 17). Healthy mice were the control group (N = 29). All mice were sacrificed at 7 or 14 days after birth. Two pathologists examined the morphological features using the BARC system; CK19, alpha SMA and collagen type I were assessed by immunohistochemistry. Results: In RRV mice, portal fibrous expansion with focal bile duct proliferation and strong portal cellular infiltrate was found in contrast to healthy mice. In RRV mice, CK19 bile duct staining was significantly less or absent (p <0.01), with stronger portal staining of collagen type I (p = 0.02). Expansion of staining for alpha SMA was more in RRV mice (p <0.01), but aSMA portal staining was stronger in healthy mice (p = 0.02). Conclusions: The morphological features observed in the murine model of BA correspond with the BA characteristics according to the BARC criteria. Fibrosis is an important feature of the model. Therefore, this murine model is useful for investigating the pathogenesis of BA. (C) 2015 Elsevier Inc. All rights reserved

Health Status and Quality of Life in Adult Biliary Atresia Patients Surviving with Their Native Livers

IntroductionGaining an insight into the quality of life (QOL) in long-term biliary atresia (BA) survivors is becoming more important. Identifying patients with limitations might make tailor made interventions possible. This is the first study investigating the health status (HS) and QOL in adults surviving BA with their native livers, and comparing them with healthy peers. MethodsBA patients surviving with their native liver were identified in the Netherlands Study Group on Biliary Atresia Registry database. The RAND-36 and the World Health Organization Quality of Life assessment instrument-100 (WHOQOL-100) were used to measure HS and QOL, respectively. Correlation between the RAND-36 and WHOQOL-100 was also assessed. Hospital Anxiety and Depression Scale (HADS) and the Impact Event Scale (IES) were also completed. ResultsIn total, 25 (83%) of the 30 eligible patients after Kasai portoenterostomy completed the questionnaires (median age 23.2 years). A lower perceived level of general health in HS was found as compared with the reference group and a higher score on the social domain was reported in QOL. Correlations between HS and QOL questionnaires were moderate to good. For the group, overall HADS and IES scores were good, though individual patients did score above the cutoff of both the questionnaires. ConclusionAdult BA patients surviving with their native liver have similar HS and QOL as compared with their healthy peers. RAND-36 and WHOQOL-100 questionnaires are not interchangeable, but complementary to assess the patients' outcomes. The overall scores did not point to increased levels of anxiety and depression. However, on an individual level, three and six patients had a score above the cutoff of the HADS subscales demonstrating clinically relevant levels of anxiety and depression, respectively