European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Clinical risk factors for testicular torsion and a warning against falsely reassuring ultrasound scans: a 10-year single-centre experience.

BACKGROUND: We sought to determine which demographic, clinical and ultrasonography characteristics are predictive of testicular torsion (TT) and to determine factors associated with time to treatment. METHODS: We retrospectively reviewed all medical records of patients (0-17 years) with acute scrotal syndrome (ASS) who were treated in our hospital in Lithuania between 2011 and 2020. We extracted patients' demographic data, in-hospital time intervals, clinical, US and surgical findings. TT was determined at surgery or clinically after manual detorsion. Test characteristics of demographic, clinical and US findings for the diagnosis of TT versus other causes of ASS were determined. We performed a multivariate analysis to identify independent clinical predictors of torsion, and factors associated with surgical delay. RESULTS: A search of medical records yielded 555 cases: 196 (35%) patients with TT and 359 (65%) patients with other ASS causes. Multivariate logistic regression analysis showed that age between 13 and 17 years (OR 8.39; 95% CI 5.12 to 13.76), duration of symptoms <7 hours (OR 3.41; 95% CI 2.03 to 5.72), palpated hard testis (OR 4.65; 95% CI 2.02 to 10.67), scrotal swelling (OR 2.37; 95% CI 1.31 to 4.30), nausea/vomiting (OR 4.37; 95% CI 2.03 to 9.43), abdominal pain (OR 2.38; 95% CI 1.27 to 4.45) were independent clinical predictors of TT. No testicular blood flow in Doppler US had a specificity of 98.2% and a positive predictive value of 94.6%. However, 75 (41.7%) patients with TT had normal testicular blood flow, yielding low sensitivity (58.3%) and negative predictive value of 81.3% for this US finding. In-hospital waiting time for surgery was longer in patients with TT with normal testicular blood flow by USS (195 min) compared with no blood flow (123 min), p<0.01. Higher orchiectomy rates were associated with longer duration of symptoms (p<0.001) and longer waiting time for USS (p=0.029) but not with false-negative US. CONCLUSIONS: Pubertal age, symptoms duration of <7 hours, nausea/vomiting, palpated hard testis, abdominal pain and scrotal swelling are predictive factors for TT. Time lost between symptom onset and seeking medical care, and between arrival and US are associated with the need for orchiectomy. Preserved blood flow in USS does not rule out TT and may contribute to delays to surgery.

Does Multisystem Inflammatory Syndrome Only Mimic Acute Appendicitis in Children or Can It Coexist: When Should We Suspect MIS-C?

Background and Objectives: Acute abdominal pain in children has been noticed to be a primary reason to seek medical attention in multisystem inflammatory disorder (MIS-C), which can prevail separately or together with acute appendicitis. Our aim was to distinguish regular appendicitis cases from MIS-C and to suggest the best clinical and laboratory criteria for it. Materials and methods: Cases of patients, admitted to the Pediatric Surgery Department over a six-month period in 2021, were retrospectively analyzed. Confirmed MIS-C or acute appendicitis cases were selected. MIS-C cases were either separate/with no found inflammation in the appendix or together with acute appendicitis. Acute appendicitis cases were either regular cases or with a positive COVID-19 test. Four groups were formed and compared: A-acute appendicitis, B-MIS-C with acute appendicitis, C-MIS-C only and D-acute appendicitis with COVID-19. Results: A total of 76 cases were overall analyzed: A-36, B-6, C-29 and D-5. The most significant differences were found in duration of disease A­1.4 days, B­4.5 days, C­4 days, D­4 days (p < 0.0001), C reactive protein (CRP) values A-19.3 mg/L B-112.5 m/L, C-143.8 mg/L and D-141 mg/L (p < 0.0001), presence of febrile fever A-13.9%, B-66.7%, C-96.6% and D-40% (p < 0.0001) and other system involvement: A 0%, B 100%, C 100% and D 20%. A combination of these factors was entered into a ROC curve and was found to have a possibility to predict MIS-C in our analyzed cases (with or without acute appendicitis) with an AUC = 0.983, p < 0.0001, sensitivity of 94.3% and specificity of 92.7% when at least three criteria were met. Conclusions: MIS-C could be suspected even when clinical data and performed tests suggest acute appendicitis especially when at least three out of four signs are present: CRP > 55.8 mg, symptoms last 3 days or longer, febrile fever is present, and any kind of other system involvement is noticed, especially with a known prior recent COVID-19 contact, infection or a positive COVID-19 antibody IgG test.

Dignity Violations and Barriers to Dignity Assurance for Terminally Ill Patients at the End of Life: A Cross-Sectional Analysis.

Background and Objectives: Investigation into forms of behavior that violate dignity is not the typical way to look for means of dignity preservation, but it may be the optimal way to prevent improper behavior. Numerous studies document that maintaining and improving patient dignity at the end of life require an understanding of factors posing threats to dignity in health care organizations. This study aimed to assess associations between dignity-violating behaviors and barriers to the assurance of dignity in health care settings from the perspective of health professionals. Materials and Methods: An anonymous survey of health professionals was conducted in Lithuania in May 2021 by using a convenience sampling method (N = 168). Two scales were developed and included in the questionnaire. One scale measured respondents' perceptions of Dignity Violations that they had witnessed. The other scale measured their opinions about Barriers to Dignity Assurance of terminally ill patients in clinical settings. Data analysis began with descriptive statistics, followed by exploratory principal component analysis (PCA) to identify the underlying structure of each scale. The variables assigned to distinct components in the PCA were combined into reflective latent variables in a path model. The path model of the relationships between the latent constructs was tested for significant links by implementing the partial least squares structural equation modeling technique. Results: Dehumanization, Humiliation, Inattentiveness, Control, Demonization, and Manipulation were identified as major forms of dignity-violating behavior. In addition, Organizational Barriers and Patient as an Obstacle were identified as two major types of barriers to the assurance of patient dignity. Both organizational and patient-oriented barriers were directly or indirectly associated with all forms of violations of patient dignity. Conclusions: The Dignity Violations scale showed potential for estimating professionals' observations of dignity violations in health care settings. Perceived high workloads, staff shortages, insufficient resources, and lack of organizational support were identified as negative organizational factors that may result in increased risk of seeing patients as obstacles to providing care that preserves the dignity of terminally ill patients.

Challenging diagnosis of abdominal tuberculosis in children: case report.

Tuberculosis (TB) remains a significant cause of morbidity and mortality in the modern world. Abdominal TB is a rare form of extrapulmonary TB that has been found to affect children without comorbidities in particular, although exact numbers are unavailable because of lack of data and its rarity. The diagnosis of abdominal TB remains a challenge because of its unspecific clinical features and unclear recommendations regarding the best diagnostic tools. We report 4 cases of children with abdominal TB diagnosed at The Hospital of Lithuanian University of Health Sciences Kaunas clinics from 2008 to 2018 at the Department of Paediatric Surgery. All these cases are exceptional. The final diagnosis of abdominal TB was confirmed only after diagnostic laparoscopy and biopsy. Moreover, we suggest that QuantiFERON test and ascitic fluid analysis with serum ascites albumin gradient and adenosine deaminase tests should be performed before more invasive interventions. Laparoscopy with biopsy should be performed only in unclear cases.

Did the COVID-19 Pandemic Prolong the Time Till Diagnosis and Worsen Outcomes for Children with Acute Appendicitis?

Background and Objectives: Our aim was to see if the COVID-19 pandemic led to an increase of time until diagnosis, operation, and time spent in Emergency room (ER), and if it resulted in more cases of complicated appendicitis and complication rates in children. Materials and Methods: We conducted a retrospective analysis of patients admitted to the Pediatric Surgery Department with acute appendicitis during a 4-month period of the first COVID-19 pandemic and compared it to the previous year data-the same 4-month period in 2019. Results: During the pandemic, the time spent in the ER until arriving at the department increased significantly 2.85 vs. 0.98 h p < 0.001, and the time spent in the department until the operation 5.31 vs. 2.66 h, p = 0.03. However, the time from the beginning of symptoms till ER, operation time and the length of stay at the hospital, as well as the overall time until operation did not differ and did not result in an increase of complicated appendicitis cases or postoperative complications. Conclusions: The COVID-19-implemented quarantine led to an increase of the time from the emergency room to the operating room by 4 h. This delay did not result in a higher rate of complicated appendicitis and complication rates, allowing for surgery to be postponed to daytime hours if needed.

Epigenetic Regulation of APAF-1 Through DNA Methylation in Pancreatic Cancer.

BACKGROUND/AIM: Apoptotic peptidase activating factor 1 (APAF-1) is essential regulator of apoptosis and inactivation by DNA methylation is common event in numerous cancer types. We investigated the regulation of APAF-1 through DNA methylation in pancreatic cancer. MATERIALS AND METHODS: Datasets from 44 patients after pancreatoduodenectomy and the pancreatic adenocarcinoma (PDAC) cell lines Capan-2 and MIA PaCa-2 treated with decitabine were analyzed by RT-PCR, immunoblotting, methylation-specific PCR analysis, apoptosis and viability assays to identify effects of APAF-1 regulation. RESULTS: APAF-1 mRNA and protein levels were significantly down-regulated, and APAF-1 methylation status was associated with perineural invasion in PDAC. Decitabine inhibited cell viability and increased apoptosis rates, however failed to restore APAF-1 mRNA and protein levels in cells. CONCLUSION: APAF-1 gene hypermethylation may contribute to the progression of PDAC through perineural invasion. Decitabine could sensitize pancreatic cancer cells to apoptosis and growth retardation, however, not directly through the APAF-1 demethylation process.

Human antigen R mediated post-transcriptional regulation of inhibitors of apoptosis proteins in pancreatic cancer.

AIM: To determine the association of human antigen R (HuR) and inhibitors of apoptosis proteins (IAP1, IAP2) and prognosis in pancreatic cancer. METHODS: Protein and mRNA expression levels of IAP1, IAP2 and HuR in pancreatic ductal adenocarcinoma (PDAC) were compared with normal pancreatic tissue. The correlations among IAP1/IAP2 and HuR as well as their respective correlations with clinicopathological parameters were analyzed. The Kaplan-Meier method and log-rank tests were used for survival analysis. Immunoprecipitation assay was performed to demonstrate HuR binding to IAP1, IAP2 mRNA. PANC1 cells were transfected with either anti-HuR siRNA or control siRNA for 72 h and quantitative reverse transcription polymerase chain reaction (RT-PCR), western blot analysis was carried out. RESULTS: RT-PCR analysis revealed that HuR, IAP1, IAP2 mRNA expression were accordingly 3.3-fold, 5.5-fold and 8.4 higher in the PDAC when compared to normal pancreas (P < 0.05). Expression of IAP1 was positively strongly correlated with HuR expression (P < 0.05, r = 0.783). Western blot analysis confirmed RT-PCR results. High IAP1 expression, tumor resection status, T stage, lymph-node metastases, tumor differentiation grade, perineural and lymphatic invasion were identified as significant factors for shorter survival in PDAC patients (P < 0.05). Immunohistological analysis showed that HuR was mainly expressed in the ductal cancer cell's nucleus and less so in cytoplasm. RNA immunoprecipitation analysis confirmed IAP1 and IAP2 post-transcriptional regulation by HuR protein. Following siHuR transfection, IAP1 mRNA and protein levels were decreased, however IAP2 expression levels were increased. CONCLUSION: HuR mediated overexpression of IAP1 significantly correlates with poor outcomes and early progression of pancreatic cancer. Further studies are needed to assess the underlying mechanisms.

Doppler flowmetry of the superior mesenteric artery and portal vein: impact for the early prediction of necrotizing enterocolitis in neonates.

PURPOSE: Necrotizing enterocolitis (NEC) is a serious gastrointestinal disorder in newborns. Early diagnosis and rapid treatment is essential for seeking good outcome for neonates. The aim of our study was to evaluate intestinal blood flow in superior mesenteric artery (SMA) and portal vein (PV) in neonates with suspected or confirmed NEC and investigate the prognostic cut-off values to develop NEC. METHODS: Doppler flowmetry of SMA and PV was performed for 62 newborns. Resistive (RI) and pulsatility (PI) indexes were measured in SMA as well as Volumetric blood flow (Vflow) in PV. ROC curves were applied to estimate sensitivity and specificity and to identify cut-off values. RESULTS: There were 93.5% preterm neonates. 29 patients (46.8%) were diagnosed with NEC and 33 (53.2%) formed a control group. 96.3% NEC patients had RI >0.75 with sensitivity of 96.3% and specificity of 90.9% (OR 260). 88.9% NEC patients had PI >1.85 with sensitivity of 88.9% and specificity of 78.8% (OR 29). Portal Vflow lower than 37 ml/min was present in 89.7% patients with NEC (OR 11.7). CONCLUSION: Intestinal blood flow Dopplerography can be a useful tool for diagnosing and predicting NEC.

Assessing the effects of pharmacists' perceived organizational support, organizational commitment and turnover intention on provision of medication information at community pharmacies in Lithuania: a structural equation modeling approach.

BACKGROUND: As a member of a pharmacy organization, a pharmacist is not only bound to fulfill his/her professional obligations but is also affected by different personal and organizational factors that may influence his/her behavior and, consequently, the quality of the services he/she provides to patients. The main purpose of the research was to test a hypothesized model of the relationships among several organizational variables, and to investigate whether any of these variables affects the service of provision of medication information at community pharmacies. METHODS: During the survey, pharmacists working at community pharmacies in Lithuania were asked to express their opinions on the community pharmacies at which they worked and to reflect on their actions when providing information on medicines to their patients. The statistical data were analyzed by applying a structural equation modeling technique to test the hypothesized model of the relationships among the variables of Perceived Organizational Support, Organizational Commitment, Turnover Intention, and Provision of Medication Information. RESULTS: The final model revealed that Organizational Commitment had a positive direct effect on Provision of Medication Information (standardized estimate = 0.27) and a negative direct effect (standardized estimate = -0.66) on Turnover Intention. Organizational Commitment mediated the indirect effects of Perceived Organizational Support on Turnover Intention (standardized estimate = -0.48) and on Provision of Medication Information (standardized estimate = 0.20). Pharmacists' Turnover Intention had no significant effect on Provision of Medication Information. CONCLUSIONS: Community pharmacies may be viewed as encouraging, to some extent, the service of provision of medication information. Pharmacists who felt higher levels of support from their organizations also expressed, to a certain extent, higher commitment to their organizations by providing more consistent medication information to patients. However, the effect of organizational variables on the variable of Provision of Medication Information appeared to be limited.

Estimating cancer risk from dental cone-beam CT exposures based on skin dosimetry.

The aim of this study was to measure entrance skin doses on patients undergoing cone-beam computed tomography (CBCT) examinations, to establish conversion factors between skin and organ doses, and to estimate cancer risk from CBCT exposures. 266 patients (age 8-83) were included, involving three imaging centres. CBCT scans were acquired using the SCANORA 3D (Soredex, Tuusula, Finland) and NewTom 9000 (QR, Verona, Italy). Eight thermoluminescent dosimeters were attached to the patient's skin at standardized locations. Using previously published organ dose estimations on various CBCTs with an anthropomorphic phantom, correlation factors to convert skin dose to organ doses were calculated and applied to estimate patient organ doses. The BEIR VII age- and gender-dependent dose-risk model was applied to estimate the lifetime attributable cancer risk. For the SCANORA 3D, average skin doses over the eight locations varied between 484 and 1788 µGy. For the NewTom 9000 the range was between 821 and 1686 µGy for Centre 1 and between 292 and 2325 µGy for Centre 2. Entrance skin dose measurements demonstrated the combined effect of exposure and patient factors on the dose. The lifetime attributable cancer risk, expressed as the probability to develop a radiation-induced cancer, varied between 2.7 per million (age >60) and 9.8 per million (age 8-11) with an average of 6.0 per million. On average, the risk for female patients was 40% higher. The estimated radiation risk was primarily influenced by the age at exposure and the gender, pointing out the continuing need for justification and optimization of CBCT exposures, with a specific focus on children.