Postoperative outcomes of acute-on-chronic liver failure in infants and children with biliary atresia.

INTRODUCTION: Acute-on-chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse. METHOD: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra-hepatic organ failure in children with decompensated cirrhosis. RESULTS: Of 107 patients (65% female; median age 14 [9-31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4-8] vs. 9 [6-24] months; p < .001) and at LT (8 [8-11] vs. 16 [10-40] months, p < .001) compared to no-ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20-53] vs. 10 [6-19] mL/kg; p < .001) and blood transfusion requirements (33 [21-69] vs. 18 [7-25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45-151] vs. 13 [7-30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1-year post-LT survival rates were comparable between the groups. CONCLUSION: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT.

Estimating risk of prolonged mechanical ventilation after liver transplantation in children: PROVE-ALT score.

BACKGROUND: Children at high risk for prolonged mechanical ventilation (PMV) after liver transplantation (LT) need to be identified early to optimize pulmonary support, allocate resources, and improve surgical outcomes. We aimed to develop and validate a metric that can estimate risk for Prolonged Ventilation After LT (PROVE-ALT). METHODS: We identified preoperative risk factors for PMV by univariable analysis in a retrospective cohort of pediatric LT recipients between 2011 and 2017 (n = 205; derivation cohort). We created the PROVE-ALT score by mapping multivariable logistic regression coefficients as integers, with cutoff values using the Youden Index. We validated the score by C-statistic in a retrospectively collected separate cohort of pediatric LT recipients between 2018 and 2021 (n = 133, validation cohort). RESULTS: Among total 338 patients, 21% (n = 72) were infants; 49% (n = 167) had cirrhosis; 8% (n = 27) required continuous renal replacement therapy (CRRT); and 32% (n = 111) required management in hospital (MIH) before LT. Incidence of PMV post-LT was 20% (n = 69) and 3% (n = 12) required tracheostomy. Independent risk factors (OR [95% CI]) for PMV were cirrhosis (3.8 [1-14], p = .04); age <1-year (8.2 [2-30], p = .001); need for preoperative CRRT (6.3 [1.2-32], p = .02); and MIH before LT (12.4 [2.1-71], p = .004). PROVE-ALT score ≥8 [Range = 0-21] accurately predicted PMV in the validation cohort with 73% sensitivity and 80% specificity (AUC: 0.81; 95% CI: 0.71-0.91). CONCLUSION: PROVE-ALT can predict PMV after pediatric LT with a high degree of sensitivity and specificity. Once externally validated in other centers, PROVE-ALT will empower clinicians to plan patient-specific ventilation strategies, provide parental anticipatory guidance, and optimize hospital resources.

Gastric Linitis Plastica: Clinical Characteristics and Outcomes from the National Cancer Database.

BACKGROUND/AIM: Gastric linitis plastica (LP) is a rare type of gastric tumor with limited data. We sought to investigate the clinical characteristics, treatment modalities, and outcomes utilizing a national database. PATIENTS AND METHODS: The National Cancer Database (NCDB) was reviewed for LP of the stomach from 2004 to 2017. Kaplan-Meier analysis and Cox proportional hazards model were utilized to evaluate overall survival and predictors of survival. RESULTS: Out of 222,488 gastric cancer cases, 896 patients with histologically confirmed primary gastric LP were included. Patients were predominantly white (78.5%), female (51.9%) and presented at advanced stage disease (stage 4=69%). A total of 369 (41.2%) patients underwent surgical resection, 520 (58.0%) received chemotherapy and 158 (17.6%) received radiation therapy. The mean OS (overall survival) of the entire cohort was 16.9 months with 1-year and 5-year OS rates of 33% and 5%, respectively. Mean OS for the patients receiving surgery with chemotherapy and/or radiation, surgery alone, chemotherapy and/or radiotherapy alone, and no treatment was 28.4, 17.1, 12.3, and, 8.1 months, respectively (p<0.001). On multivariate cox-regression analysis, advanced-stage disease (stage IV) (p<0.001), no surgical resection (p<0.001), and no receipt of chemotherapy (p<0.001) were associated with increased hazards of death. Over time, the proportion of patients receiving surgical resection (30.7% from 48.3%) and radiation therapy decreased (13.8% from 20.6%) and the use of chemotherapy increased (63.9% from 54.8%). CONCLUSION: Gastric LP is associated with a poor prognosis. Multimodal therapy including surgical resection and systemic therapy in the neoadjuvant setting seems to provide the best long-term outcomes.

The use of tracheostomy to support critically ill children receiving orthotopic liver transplantation: a single-center experience.

BACKGROUND: Children with end-stage liver disease and multi-organ failure, previously considered as poor surgical candidates, can now benefit from liver transplantation (LT). They often need prolonged mechanical ventilation (MV) post-LT and may need tracheostomy to advance care. Data on tracheostomy after pediatric LT are lacking. METHOD: Retrospective chart review of children who required tracheostomy in the peri-LT period in a large, freestanding quaternary children's hospital from 2014 to 2019. RESULTS: Out of 205 total orthotopic LTs performed in 200 children, 18 (9%) required tracheostomy in the peri-transplant period: 4 (2%) pre-LT and 14 (7%) post-LT. Among those 14 needing tracheostomy post-LT, median age was 9 months [IQR = 7, 14] at LT and 10 months [9, 17] at tracheostomy. Nine (64%) were infants and 12 (85%) were cirrhotic at the time of LT. Seven (50%) were intubated before LT. Median MV days prior to LT was 23 [7, 36]. Eight (57%) patients received perioperative continuous renal replacement therapy (CRRT). The median MV days from LT to tracheostomy was 46 [33, 56]; total MV days from initial intubation to tracheostomy was 57 [37, 66]. Four (28%) children died, of which 3 (21%) died within 1 year of transplant. Total ICU and hospital length of stay were 92 days [I72, 126] and 177 days [115, 212] respectively. Among survivors, 3/10 (30%) required MV at home and 8/10 (80%) were successfully decannulated at 400 median days [283, 584]. CONCLUSION: Tracheostomy though rare after LT remains a feasible option to support and rehabilitate critically ill children who need prolonged MV in the peri-LT period.

Complete gastroesophageal junction avulsion after near drowning: A case report and review of literature.

INTRODUCTION: Gastroesophageal (GE) junction injuries are rare in the pediatric population. A complete GE junction separation in a child secondary to trauma has not reported in the literature yet. PRESENTATION OF CASE: A 14-year-old boy presented with a complete GE junction avulsion after a near-drowning experience. He underwent immediate damage control surgery and delayed gastric pull-up esophageal reconstruction in 3-months. At the most recent clinic visit 5 months from the reconstruction, he can tolerate a regular diet without difficulty and is gaining weight and recovering well. CONCLUSION: Complete GE junction injuries and avulsions are rare with limited data to guide management. These injuries are associated with mortality rates from 25% to 33%, therefore, high index of suspicion, prompt recognition and careful surgical planning is needed for favorable outcomes.

Urinary NT-proBNP as a potential noninvasive biomarker for screening of pulmonary hypertension in preterm infants: a pilot study.

OBJECTIVE: This pilot study aimed to determine the feasibility of urinary NT-proBNP (NT-proBNP) as a potential noninvasive screening marker for pulmonary hypertension (PH). STUDY DESIGN: A prospective cross-sectional study was conducted. Preterm infants (PI) (birthweight <1500 gm and <30 weeks gestational age (GA)) were enrolled. Serial urinary NT-proBNP measurements and echocardiograms (ECHO) were performed at 28, 32, and 36 weeks. RESULTS: Thirty-six patients were included in the final analysis (BPD-PH group = 6, BPD group = 20, control = 10). Urinary NT-proBNP levels were higher in the BPD-PH group compared with BPD and control groups at all study intervals. A urine NT-proBNP cutoff level of 2345 pg/ml at 28 weeks of GA had a sensitivity and specificity of 83.3% and 84.2%, respectively, for detection of BPD-PH (AUC 0.816, p = 0.022). CONCLUSION: Urinary NT-proBNP measurement is feasible in preterm infants and appears to be a good noninvasive screening tool for PH.

Primary Pediatric Non-Hodgkin Lymphomas of the Gastrointestinal Tract: A Population-based Analysis.

BACKGROUND/AIM: The aim of this study was to present the clinical characteristics, natural history and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in the pediatric population. PATIENTS AND METHODS: Surveillance, Epidemiology, and End Results (SEER) database was queried for patients aged 0 to 19 years with PGINHL between 1973 and 2014. RESULTS: A total of 452 cases were identified [mean age 11.0 (±5.1)] years, whites 84.1%, males (76.5%). The majority of tumors were noted in the small bowel (SB) (47.6%), followed by large bowel (LB) (28.5%) and the stomach (10.0%). Overall, the most common histological subtype was Burkitt lymphoma (51.8%), followed by diffuse large B-cell lymphoma (DLBCL) (26.1%). Mean overall survival (OS) of the entire cohort was 33,33 years with a 5-yr, 10-yr and 30-yr survival rate of 86%, 86% and 79%, respectively. Large bowel tumors had the best long-term survival rates whereas; gastric tumors had the worst with 30-yr survival rate 84% and 74%, respectively. Overall, 328 (72.6%) patients received surgery. No significant survival difference was noted between patients who underwent surgery and those who did not. CONCLUSION: This study presents the largest dataset of pediatric PGINHL and describes the clinical features and outcomes of these patients in addition to summarizing the literature.

De novo descending aorta thrombus in a patient with metastatic colorectal cancer: A case report.

Thromboembolic events in patients with cancer are frequently reported in literature and usually involve the venous circulation. De novo thrombus formation in the arterial system without any underlying atherosclerosis, dissection or aneurysm is extremely rare. We report the case of a patient with metastatic colorectal carcinoma who developed a thrombus in the descending aorta without any intervention or risk factors. The patient presented with non-specific gastrointestinal symptoms. A CT scan revealed an aortic thrombus obstructing 70% of the lumen. She opted for no treatment of her thrombus and was later complicated by extensive lower limb emboli. Patients with aortic thrombosis usually present with subtle symptoms which may be underestimated by both the patient and the physician. A high index of suspicion is required for early recognition and prevention of complications.

Knowledge and Perception Regarding Autism among Primary School Teachers: A Cross-sectional Survey from Pakistan, South Asia.

BACKGROUND: Early detection and intervention seem to improve development in autistic children, and teachers form an important part of their early social environment. OBJECTIVES: The objective of this study was to assess baseline knowledge and misconceptions regarding autism among school teachers and evaluate factors influencing their knowledge. MATERIALS AND METHODS: This is a cross-sectional survey enrolling primary school teachers using a self-administered questionnaire. RESULTS: Seventy-three teachers (mean age of 34 years, 66% females) responded. Gaps in awareness and knowledge were found. About 52 (71.2%) teachers identified themselves as having some knowledge about autism, with 23 (44.2%) among this group understanding autism as a neurological/mental disorder. The majority (73.1%) believe that special education is a helpful intervention. The only significant factor that influenced knowledge among teachers was attendance of behavioral classes (P = 0.01). CONCLUSION: Results suggest that teachers have an inadequate understanding of autism due to several misconceptions. This calls for increased education of teachers with regard to autism and other childhood disorders.

Proton pump inhibitors and other disease-based factors in the recurrence of adverse cardiovascular events following percutaneous coronary angiography: A long-term cohort.

BACKGROUND: Dual aspirin-clopidogrel antiplatelet therapy (DAPT) has been shown to decrease the risk of adverse cardiac events after percutaneous coronary intervention (PCI). Proton pump inhibitors (PPIs) are used in these patients to decrease the risk of gastrointestinal bleeding and several studies have reported potential interaction and conflicting clinical outcomes with their use. We aim to assess the effect of different PPIs and other factors on the recurrence of cardiovascular (CV) events in patients following PCI. METHODS: We performed a retrospective cohort on patients who underwent PCI in the last 5 years and were discharged with or without PPIs. Strict inclusion criteria were adopted, outcome measures were defined, and patient follow up up to 2 years was collected. RESULTS: Out of 740 patients, 453 (61.2 %) had received PPIs and 287 (38.8 %) were discharged without PPIs. Ninety-five (12.8 %) patients were readmitted due to adverse CV events. Statistically, there was no significant difference in the recurrence of CV events with the use of different PPIs (p = 0.384) and PPI use had an overall protective effect (p = 0.009, HR 0.58 (CI 0.39-0.88). Patients with history of diabetes mellitus (p = 0.048) had an increased risk of adverse CV events. CONCLUSION: We conclude that pharmacokinetic interaction between PPIs and antiplatelet therapy is not associated with adverse CV events. A comprehensive, multicenter, open-label trial including all PPI subclasses and patient and disease-based factors is warranted for a fair evaluation.

Primary Hepatic Carcinoid Tumor with Poor Outcome.

Primary Hepatic Carcinoid Tumor (PHCT) represents an extremely rare clinical entity with only a few cases reported to date. These tumors are rarely associated with metastasis and surgical resection is usually curative. Herein, we report two cases of PHCT associated with poor outcomes due to late diagnosis. Both cases presented late with non-specific symptoms. One patient presented after a 2-week history of symptoms and the second case had a longstanding two years symptomatic interval during which he remained undiagnosed and not properly worked up. Both these cases were diagnosed with hepatic carcinoid tumor, which originates from neuroendocrine cells. Case 1 opted for palliative care and expired in one month’s time. Surgical resection was advised to the second case, but he left against medical advice.