bluuebunny/crossref_metadata_2025 huggingface dataset:
Weight Gain After Heart Transplantation in Adults: Systematic Review and Meta-Analysis
Authors: Miura Kyoko , Yu Regina , Sivapalan Krishnaa , Liyanage Upekha E. , Entwistle Timothy , McKenzie Scott C. , Green Adèle C.
Published: September 2021

Gain in weight is common after heart transplantation but the magnitude of usual weight gain and whether this varies by country is unknown. We systematically reviewed all relevant studies to quantify weight change among heart transplant recipients (HTRs) in the years after transplantation and assess variation with geographic location. We searched PubMed, Cumulative Index to Nursing and Allied Health Literature, and Excerpta Medica Database databases to September 2020. Eligible studies reported adult HTRs’ mean/median weight and/or body mass index (BMI) up to time of transplantation (baseline) and posttransplantation in any language. Weighted mean differences (WMDs) (95% confidence intervals [CIs]) of weight/BMI from baseline to posttransplantation were estimated using a random-effects model. Ten studies met the inclusion criteria. Pooled analysis showed weight gain of 7.1 kg (95% CI, 4.4–9.8 kg) in HTRs 12 months posttransplant, with corresponding BMI increase of 1.69 kg/m2 (95% CI, 0.83–2.55 kg/m2). Greatest weight gain at 12 months posttransplant occurred in US HTRs (WMD weight 10.42 kg, BMI 3.25 kg/m2) and least, in European HTRs (WMD weight 3.10 kg, BMI 0.78 kg/m2). In conclusion, HTRs gain substantial weight in the years after transplantation, but varying widely by geographic location.
Is Foot Drop an Underreported Sequela of Extracorporeal Membrane Oxygenation?
Authors: Bergeron Amanda , Nathan Sriram , de Armas Ismael Salas , Smith Kischa , Gilley Christa , Janowiak Lisa , Kutilek Kayla , Kar Biswajit , Gregoric Igor D.
Published: September 2021

Foot drop in the absence of limb ischemia is a chronic complication in peripheral venoarterial extracorporeal membrane oxygenation (V-A ECMO) survivors; however, there is little published regarding the incidence and functional outcomes of this condition. Common peroneal nerve is the most common cause of foot drop, a condition that leads to significant debility and requires extensive physical therapy and rehabilitation, thereby affecting the patient’s quality of life. We completed a retrospective review of 153 patients who received femoral cannulation for V-A ECMO support for greater than 1 hour. The incidence of foot drop in our V-A ECMO population was 7.8% (12/153). Importantly, only two patients with foot drop were discharged home. The majority (10/12) of patients with foot drop required in-patient rehabilitation; five patients were nonambulatory, and five patients required an ankle foot orthosis assistive device. This study identifies foot drop as a relevant complication in peripherally cannulated V-A ECMO survivors and provides an initial incidence rate. By raising awareness of this complication in the V-A ECMO population, an early diagnosis is possible, which can enable appropriate rehabilitation. Prospective trials are needed to identify possible risk factors of foot drop and methods to reduce this complication of V-A ECMO.
Venoarterial Extracorporeal Membrane Oxygenation in Severe Drug Intoxication: A Retrospective Comparison of Survivors and Nonsurvivors
Authors: Duburcq Thibault , Goutay Julien , Preau Sebastien , Mugnier Agnes , Rousse Natacha , Moussa Mouhamed D. , Vincentelli André , Cuny Jerome , Parmentier-Decrucq Erika , Poissy Julien
Published: September 2021

Selecting patients most likely to benefit from venoarterial extracorporeal membrane oxygenation (V-A ECMO) to treat refractory drug-induced cardiovascular shock remains a difficult challenge for physicians. This study reported short-term survival outcomes and factors associated with mortality in V-A ECMO-treated patients for poisoning. Twenty-two patients placed on V-A ECMO after drug intoxication from January 2014 to December 2020 were retrospectively analyzed. The primary endpoint of this study was survival at hospital discharge. Univariate descriptive analysis was performed to compare survivors and nonsurvivors during hospitalization. The overall survival at hospital discharge was 45.4% (n = 10/22). Survival rate tended to be higher in patients treated for refractory shock (n = 7/10) compared with those treated for refractory cardiac arrest (n = 3/12, p = 0.08). Low-flow duration and time from admission to ECMO cannulation were shorter in survivors (p = 0.02 and p = 0.03, respectively). Baseline characteristics before ECMO, including the class of drugs involved in the poisoning, between survivors and nonsurvivors were not statistically different except pH, bicarbonate, serum lactate, Sequential Organ Failure Assessment, and Survival After Veno-arterial-ECMO (SAVE) score. All patients with SAVE-score risk classes II/III survived whereas 85.7% (n = 12/14) of those with SAVE-score risk classes IV/V died. A lactic acid >9 mmol/L predicts mortality with a sensitivity/specificity ratio of 83.3%/100%. V-A ECMO for severe drug intoxication should be reserved for highly selected poisoned patients who do not respond to conventional therapies. Shortening the timing of V-A ECMO initiation should be a key priority in improving outcomes. Low-flow time >60min, lactic acid >9mmol/L, and SAVE-score may be good indicators of a worse prognosis.
Effect of remote ischemic preconditioning on fibrin formation and metabolism in patients undergoing hip fracture surgery: a randomized clinical trial
Authors: Wahlstrøm Kirsten L. , Ekeloef Sarah , Sidelmann Johannes J. , Gögenur Ismail , Münster Anna-Marie B.
Published: September 2021

Remote ischemic preconditioning (RIPC) prior to surgery has recently been shown to reduce the risk of myocardial injury and myocardial infarction after hip fracture surgery. This study investigated whether RIPC initiated antithrombotic mechanisms in patients undergoing hip fracture surgery. This trial was a predefined sub-study of a multicentre randomized clinical trial. Adult patients with cardiovascular risk factors undergoing hip fracture surgery between September 2015 and September 2017 were randomized 1 : 1 to RIPC or control. RIPC was initiated before surgery with a tourniquet applied to the upper arm and it consisted of four cycles of 5 min of forearm ischemia followed by five minutes of reperfusion. The outcomes such as surgery-induced changes in thrombin generation, fibrinogen/fibrin turnover, tissue plasminogen activator, plasminogen activator inhibitor-1 and fibrin structure measurements were determined preoperatively (prior to RIPC) and 2 h postoperatively. One hundred and thirty-seven patients were randomized to RIPC (n = 65) or control (n = 72). There were no significant changes in thrombin generation, fibrinogen/fibrin turnover or fibrin structure measurements determined pre and postoperatively between patients in the RIPC and control groups. Subgroup analyses on patients not on anticoagulant therapy (n = 103), patients receiving warfarin (n = 17) and patients receiving direct oral anticoagulant therapy (n = 18) showed no significant changes between the RIPC-patients and controls. RIPC did not affect changes in thrombin generation, fibrin turnover or fibrin structure in adult patients undergoing hip fracture surgery suggesting that the cardiovascular effect of RIPC in hip fracture surgery is not related to alterations in fibrinogen/fibrin metabolism.
Patient Blood Management in intensive care patients
Authors: Hof Lotta , Choorapoikayil Suma , Meybohm Patrick , Zacharowski Kai
Published: September 2021

Purpose of review

Patients admitted to ICUs are a heterogeneous group, displaying multiple anaemia risk factors and comorbidities. Clinicians should therefore take all possible measures to identify modifiable risks. Patient Blood Management (PBM) is an approach promoting the timely application of evidence-based interventions designed to maintain patients own blood mass.

Recent findings

Within ICU-patients, anaemia is highly prevalent. Generally, anaemia is associated with impaired outcome and need of blood transfusion. Currently, with ICUs working at full capacity and the global blood reserves exhausted, the SARS-CoV-2 pandemic reinforces the need for PBM implementation. For instance, implementation of a comprehensive coagulation management and measures to avoid iatrogenic blood loss may prevent bleeding-associated complications and adherence to blood transfusion guidelines may reduce adverse events associated with transfusion.

Critically ill patients display various morbidities often requiring individualized treatment. PBM offers patient-centred measures to improve outcome any time during hospital stay.
Controversies in paediatric acute kidney injury and continuous renal replacement therapy: can paediatric care lead the way to precision acute kidney injury medicine?
Authors: Stanski Natalja L. , Fuhrman Dana , Basu Rajit K.
Published: September 2021

Purpose of review

Paediatric patients represent a unique challenge for providers managing acute kidney injury (AKI). Critical care for these children requires a precise approach to assessment, diagnostics and management.

Recent findings

Primarily based on observational data, large epidemiologic datasets have demonstrated a strong association between AKI prevalence (one in four critically ill children) and poor patient outcome. Drivers of AKI itself are multifactorial and the causal links between AKI and host injury remain incompletely defined, creating a management paradigm primarily supportive in nature. The previous decades of research have focused primarily on elucidating the population-level epidemiologic signal of AKI and use of renal replacement therapy (RRT), but in order to reverse the course of the AKI ‘epidemic’, future decades will require more attention to the individual patient. A patient-level approach to AKI in children will require sophisticated approaches to risk stratification, diagnostics and targeted utilization of therapies (both supportive and targeted towards drivers of injury).

In this review, we will summarize the past, present and future of AKI care in children, discussing the ongoing work and future goals of a personalized approach to AKI medicine.
Colon hypoganglionosis in Beckwith-Wiedemann syndrome: a new rare comorbidity?
Authors: Cazzaniga Laura , Parma Barbara , Licini Lisa , Dalla Rosa Davide , Cheli Maurizio , Selicorni Angelo
Published: September 2021

We describe the case of a patient with a clinical and molecular diagnosis of Beckwith-Wiedemann Syndrome (BWS) and a clinical, radiologic and histologic diagnosis of colon isolated hypoganglionosis. BWS is a genetic multisystem disorder characterized by generalized and lateralized overgrowth, macroglossia, abdominal wall defects, neonatal hypoglycemia and predisposition to embryonal tumors (Brioude et al., Nat Rev Endocrinol 1998; 14:229–249). Hypoganglionosis of the colon is a condition that clinically resembles Hirschsprung’s disease and it is part of a group of numerous and heterogeneous conditions that are defined ‘Variants of Hirschsprung’s disease’ (Friedmacher and Puri, Pediatr Surg Int 2013; 29:855–872). To the best of our knowledge, the association of BWS with Hirschsprung’s disease has been observed only in one patient, an infant with hypoglycemia (Shah et al., BMJ Case Rep 2020; 13:e235121). We suppose that dysganglionosis could be rare comorbidity of BWS. We suggest to put particular attention to patients affected by BWS who develop early severe constipation taking into account the possibility to study them at radiologic and histologic levels to show the possible evidence of Hirschsprung’s disease variants.
Pathophysiology of Pediatric Gastroesophageal Reflux Disease
Authors: Nikaki Kornilia , Sifrim Daniel
Published: September 2021

Gastroesophageal reflux (GOR) is defined as “the passage of gastric contents into the esophagus with or without regurgitation and vomiting” and gastroesophageal reflux disease (GORD) is defined “when GOR leads to troublesome symptoms affecting the daily functioning and/or complications.” This definition was first developed in 2006 by the Montreal consensus group (1) and later on adopted by pediatric gastroenterology societies such as ESPGHAN and NASPGHAN in 2009 (2). The definition of gastroesophageal reflux reveals little about its pathophysiology and is focused on symptomatology. In this way, it acts as an umbrella term for the multifactorial causes of the disease and the various phenotypes encountered; from functional heartburn to hypersensitive esophagus and nonerosive reflux disease, to erosive esophagitis and Barrett’s esophagus. This article is devoted to the pathophysiology of pediatric GORD in comparison to adult GORD and is divided in 2 parts. In the first part, we will systematically describe the different mechanisms for the generation and clearance of reflux events, while on the second part we will discuss the mechanisms involved in symptoms generation. Finally, we will discuss the similarities and differences between pediatric and adult GORD.
Outcomes of early versus delayed endoscopy in cirrhotic patients with acute variceal bleeding: a systematic review with meta-analysis
Authors: Bai Zhaohui , Wang Ran , Cheng Gang , Ma Dapeng , Ibrahim Mostafa , Chawla Saurabh , Qi Xingshun
Published: September 2021

Objectives

Endoscopy is the mainstay treatment option for acute variceal bleeding (AVB) in liver cirrhosis. However, the optimal timing of endoscopy in such patients remains unclear.

Methods

PubMed, EMBASE and Cochrane Library databases were searched. We compared the mortality, incidence of rebleeding, length of stay, endoscopic hemostasis, need for salvage and units of transfusion between cirrhotic patients with AVB who underwent early and delayed endoscopy. Meta-analyses were performed by using a random-effect model. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated. Subgroup analysis was performed in studies where early endoscopy was defined as <12 h.

Results

Nine retrospective studies involving 2824 patients were included. The early endoscopy group had a significantly lower overall mortality than the delayed endoscopy group in overall analysis (OR = 0.56, 95% CI, 0.33–0.95, P = 0.03), but the difference between them was NS in subgroup analysis (OR = 0.72, 95% CI, 0.38–1.38, P = 0.33). In-hospital (OR = 0.77, 95% CI, 0.26–2.32, P = 0.65) and 6-week (OR = 0.78, 95% CI, 0.42–1.47, P = 0.45) mortality were not significantly different between them. Overall rebleeding was not significantly different between early and delayed endoscopy groups in both overall (OR = 0.88, 95% CI, 0.51–1.51, P = 0.63) and subgroup (OR = 1.04, 95% CI, 0.55–1.95, P = 0.90) analyses. In-hospital (OR = 1.41, 95% CI, 0.67–2.96, P = 0.37) and 6-week (OR = 0.93, 95% CI, 0.40–2.17, P = 0.86) rebleeding remained not significantly different between them. Additionally, the length of stay, endoscopic hemostasis, need for salvage and units of transfusion were not significantly different between them.

Conclusions

Early endoscopy may improve the survival of cirrhotic patients with AVB, but has no remarkable benefit on the prevention of rebleeding. These findings should be further validated by high-quality studies.
No evidence of “weekend effect” in upper gastrointestinal bleeding in Italy: data from a nationwide prospective registry
Authors: Soncini Marco , Occhipinti Vincenzo , Zullo Angelo , Marmo Riccardo ,   on behalf of GISED - Gruppo Italiano per lo Studio dell’Emorragia Digestiva
Published: September 2021

Objectives

The literature offers conflicting information about ‘weekend effect’ in acute upper gastrointestinal bleeding (AUGIB). Aim of our study was to compare clinical outcomes of patients admitted for AUGIB during the weekend or on weekdays in Italy.

Methods

We analyzed data from a prospective registry of AUGIB (either nonvariceal, NV-AUGIB or variceal, V-AUGIB) from 50 Italian hospitals from January 2014 to December 2015. Mortality, rebleeding, need for salvage procedures and length of hospitalization were compared among patients admitted during the weekend or on weekdays.

Results

In total 2599 patients (mean age 67.4 ± 15.0 years, 69.2% males) were included, 2119 (81.5%) with NV-AUGIB and 480 (18.5%) with V-AUGIB. Totally 494 patients with NV-AUGIB (23.3%) and 129 patients with V-AUGIB (20.7%) were admitted during the weekend. The two study groups were similar in terms of physical status (American Society of Anesthesiologists score, comorbidities) and bleeding-specific prognostic scores. We did not find differences in terms of mortality (5.6 vs. 4.9%; P = 0.48), rebleeding (5.9 vs. 5.1%; P = 0.39), need for salvage procedures (4.0 vs. 3.6%; P = 0.67) or duration of hospitalization (8.5 ± 6.9 vs. 8.3 ± 7.2 days; P = 0.58) between patients admitted during weekend or weekdays. Considering separately NV-AUGIB and V-AUGIB, the only difference found in clinical outcomes was a higher rebleeding risk in patients with V-AUGIB admitted during the weekend (13.2 vs. 7.4%; P = 0.05).

Conclusions

Data from our large, prospective multicenter registry shows that in Italy there is no significant ‘weekend effect’ for either NV- or V-AUGIB. Our results show that the Italian hospital network is efficient and able to provide adequate care and an effective therapeutic endoscopy even during the weekend.
The performance of COBRA, a decision rule to predict the need for intensive care interventions in intentional drug overdose
Authors: Wiersma Tim , van den Oever Huub L.A. , van Hout Freek J.H.A. , Twijnstra Minke J. , Mauritz Gert-Jan , van ’t Riet Esther , Jansman Frank G.A.
Published: September 2021

Background

COBRA was developed as a decision rule to predict which patients visiting the emergency department (ED) following intentional drug overdose will not require intensive care unit (ICU) interventions. COBRA uses parameters from five vital systems (cardiac conduction, oxygenation, blood pressure, respiration, and awareness) that are readily available in the ED. COBRA recommends against ICU admission when all these parameters are normal.

Objective

The primary aim of this study was to determine the negative predictive value (NPV) of COBRA in predicting ICU interventions. Secondary outcomes were the sensitivity, specificity and positive predictive value (PPV), and the observation time required for a reliable prediction.

Design

Observational cohort study.

Settings and participants

Patients with a reported intentional overdose with drugs having potential acute effects on neurological, circulatory or ventilatory function were included, and data necessary to complete the decision rule was collected. The attending physician in the ED made the actual admission decision, on the basis of clinical judgement. COBRA was measured 0, 3 and 6 h after arrival at the ED.

Outcome measures

Need for ICU interventions (treatment of convulsion; defibrillation; mechanical or noninvasive ventilation; intravenous administration of vasopressive agents, antiarrhythmics, atropine, calcium, magnesium or sedation; continuous hemofiltration or administration of antagonist/antidote and fluid resuscitation).

Main results

Of 230 new cases (144 unique patients), 59 were immediately referred to the psychiatric services and/or sent home by the attending physician, 27 went to a regular ward, and 144 were admitted to the ICU. Of these 144 cases, 40 required one or more ICU interventions. By the time the first parameters were collected, the NPV of COBRA was 95.6%. After 3 h of observation, NPV was 100%, while sensitivity, specificity and PPV were 100, 61.1 and 35.1%, respectively. None of these values improved by prolonging the observation time to 6 h.

Conclusion

In patients with a reported intentional overdose with drugs having potential acute effects on neurological, circulatory or ventilatory function, the COBRA decision rule showed good performances in predicting the need for intensive care interventions, with a NPV of 100% after 3 h of observation.
Breastfeeding and the developmental origins of mucosal immunity: how human milk shapes the innate and adaptive mucosal immune systems
Authors: Dawod Bassel , Marshall Jean S. , Azad Meghan B.
Published: September 2021

Purpose of review

Breastfeeding provides passive immunity while the neonatal immune system matures, and may also protect against chronic immune-mediated conditions long after weaning. This review summarizes current knowledge and new discoveries about human milk and mucosal immunity.

Recent findings

New data suggest that certain microbes in maternal milk may seed and shape the infant gut microbiota, which play a key role in regulating gut barrier integrity and training the developing immune system. Human milk oligosaccharides, best known for their prebiotic functions, have now been shown to directly modulate gene expression in mast and goblet cells in the gastrointestinal tract. Epidemiologic data show a reduced risk of peanut sensitization among infants breastfed by peanut-consuming mothers, suggesting a role for milk-borne food antigens in tolerance development. Cross-fostering experiments in mice suggest the soluble Toll-like receptor 2, found in human milk, may be critical in this process. Finally, interest in human milk antibodies surged during the pandemic with the identification of neutralizing severe acute respiratory syndrome coronavirus 2 antibodies in maternal milk following both natural infection and vaccination.

Human milk provides critical immune protection and stimulation to breastfed infants. Understanding the underlying mechanisms could identify new therapeutic targets and strategies for disease prevention across the lifespan.
Pharmacogenomics insights into precision pediatric oncology
Authors: Ramos Kristie N. , Gregornik David , Ramos Kenneth S.
Published: September 2021

Purpose of review

Pharmacogenomic insights provide an opportunity to optimize medication dosing regimens and patient outcomes. However, the potential for interindividual genomic variability to guide medication dosing and toxicity monitoring is not yet standard of care. In this review, we present advances for the thiopurines, anthracyclines and vincristine and provide perspectives on the actionability of pharmacogenomic guidance in the future.

Recent findings

The current guideline on thiopurines recommends that those with normal predicted thiopurine methyltransferase and NUDT15 expression receive standard-of-care dosing, while ‘poor metabolizer’ haplotypes receive a decreased 6-mercaptopurine starting dose to avoid bone marrow toxicity. Emerging evidence established significant polygenic contributions that predispose to anthracycline-induced cardiotoxicity and suggest this knowledge be used to identify those at higher risk of complications. In the case of vincristine, children who express CYP3A5 have a significantly reduced risk of peripheral neuropathy compared with those expressing an inactive form or the CYP3A4 isoform.

The need for adequately powered pediatric clinical trials, coupled with the study of epigenetic mechanisms and their influence on phenotypic variation and the integration of precision survivorship into precision approaches are featured as important areas for focused investments in the future.
Effects of obesity and weight-loss surgery shift the microbiome and impact alloimmune responses
Authors: Zhou Hao , Tullius Stefan G.
Published: September 2021

Purpose of review

Obesity is a worldwide health problem with increasing rates in both children and adults. Bariatric surgery (BS) represents the only effective long-term treatment. Beneficial effects of BS may be mediated through shifts of the gut microbiome. Here, we introduce data linking the microbiome to alloimmune responses.

Recent findings

The rapid development of microbiome sequencing technologies in addition to the availability of gnotobiotic facilities have enabled mechanistic investigations on modulations of alloimmune responses through microbiomes. BS has been shown to improve comorbidities and chronic inflammation caused by obesity. Changes in microbiota and microbiota-related metabolites may play a role. Patients either listed or having received a transplant have undergone weight loss surgery, thus allowing to dissect mechanisms of microbial shifts to alloimmunity.

Weight loss and BS have the potential to improve transplant outcomes by ameliorating alloimmune responses. Those effects may be carried out through alterations of the gut microbiome.
The Cutting Edge of Gastroenteritis
Authors: Arostegui Dalia , Wallach Thomas
Published: September 2021

In recent years, multiple advances have been made in the care, diagnosis, and mechanistic understanding of acute gastroenteritis (AGE). In this review, we discuss the current state of the art of diagnosis and management, as well as how changes in practice can improve care and decrease costs. We will discuss present study demonstrating the effect of AGE on the microbiome and how that may be linked to secondary effects or long‐term changes. We will explore the use of novel technologies to further our capacity to understand how gastrointestinal infections occur and promulgate. Finally, will discuss advances in our understanding of how gastrointestinal infections capacitate other changes such as post‐viral motility or other post viral intestinal dysfunction.
Multidisciplinary Care for Refractory Pediatric Functional Abdominal Pain Decreases Emergency and Inpatient Utilization
Authors: Chu Andrew S. , Torres Laura , Kao Grace , Gilbert Caroyl , Monico Evelyn Caro , Chumpitazi Bruno P.
Published: September 2021

Children with refractory functional abdominal pain (FAP) experience functional disability and may utilize emergency department (ED) and/or inpatient services. Whether multidisciplinary programs which help care for children with refractory FAP affect acute healthcare utilization is unknown. A retrospective chart review of children initially evaluated by the outpatient Multidisciplinary Abdominal Pain Program (MAPP) from October 2016 to May 2019 was completed. Patient characteristics and number of ED visits and hospitalizations for abdominal pain in the year prior to versus year after MAPP evaluation were captured. Thirty‐eight children (ages 9–17 years [median 13 years]) were included. The median number of ED visits/patient/year decreased from 1 (range: 0–7) to 0 (range: 0–3) (P < 0.0001). Seven (18%) children had been hospitalized and, in these children, the median number of hospitalizations/patient/year decreased from 1 (range: 1–5) to 0 (range: 0–1) (P < 0.05). These data suggest multidisciplinary outpatient intervention for refractory FAP is associated with significant decreases in acute healthcare utilization.
Social and Educational Outcomes in Patients With Biliary Atresia
Authors: Alexander Emma C. , Greaves William , Vaidya Hrisheekesh J. , Burford Charlotte , Jain Vandana , Samyn Marianne
Published: September 2021

Objectives:

To systematically review the social outcomes of patients with biliary atresia (BA), including educational, employment and family outcomes.

Methods:

We conducted a systematic review of Medline, EMBASE, Global Health, Maternity and Infant Care Database, supplemented by reference searching. National Heart, Lung and Blood Institute scoring was conducted for quality assessment. The PROSPERO registration ID was CRD42020178846.

Results:

Fifty‐one studies were included (41 cohort, 10 cross‐sectional), including 4631 participants across 16 countries. Cohorts were BA post‐liver transplant (LT) (18 studies), native liver survivors (NLS) (16 studies), mixed (13 studies) and four other cohorts. Outcomes covered; education (n = 35), employment (n = 16), family outcomes (n = 22), and social functioning (n = 22). BA patients had lower school functioning scores than controls, with no difference between NLS versus post‐LT. Between 2% and 48% of children required additional educational support. Between 60% and 100% of adult patients with BA were employed. Pregnancies were described in 17 studies, with small samples, and some noted complications. Social functioning scores were similar to healthy controls in 8 of 11 comparisons.

Conclusions:

Despite BA being the primary indication for liver transplantation in childhood, social outcomes for children and adolescents are predominantly reported in non‐controlled, single‐centre survey‐based studies. School functioning is lower compared to peer groups, with no evidence of a difference for those having a liver transplant. We recommend routine psychosocial assessment of these patients during follow‐up, alongside multi‐centre collaborations, to maximise the quality of evidence for future patients.
Effect of Added Free Water to Enteral Tube Feeds in Children Receiving Commercial Blends
Authors: Hirsch Suzanna , Solari Toni , Rosen Rachel
Published: September 2021

Objectives:

To determine the impact of free water administration on clinical outcomes in medically complex patients, based on the hypothesis that patients receiving more thin liquids have worse outcomes related to increased risk of gastroesophageal reflux.

Methods:

This was a retrospective chart review of children initiated on commercial blenderized tube feedings from 2010 to 2019. The percentage of gastrostomy intake that was a thin liquid was determined, with thin liquids including free water or thin formula based on the International Dysphagia Diet Standardization Initiative (IDDSI) framework. Patients were categorized into those receiving low volumes of thin liquids (“percent thin” ⩽20%) and those receiving higher volumes (“percent thin” >20%). Emergency room visits, hospital admissions, and chest X‐rays during the year after starting the blend were compared.

Results:

Forty‐five patients age 12 months to 18.7 years (median 3.7 years) were included. Twenty patients (44%) were receiving a blend with an IDDSI‐categorized thin consistency and 25 patients (56%) were receiving a thicker blend. In addition to the blend, patients received a median of 320 mL per day of water (range 0–1000 mL). Patients receiving <20% thin liquids were less likely to undergo chest X‐rays during follow‐up than patients receiving larger amounts of thin liquids (10% in the minimal thin group versus 48% in the greater thin group, P = 0.03). In a multivariable logistic regression, this relationship remained significant after controlling for underlying pulmonary disease, aspiration, method of feed administration (bolus or continuous feeds), fundoplication status, and oral intake status.

Conclusions:

Our study demonstrates that patients on blenderized tube feeds receive widely variable amounts of added thin liquids, like water. The addition of water to tube feeds, while typically given for hydration, can modify feed viscosity and clinical outcomes, such as chest X‐ray performance.
Pulmonary Blastomycosis: A Rare Cause of Acute Chest Syndrome and Prolonged Fevers in a Pediatric Patient With Sickle Cell Disease
Authors: Halyko Michael , Thompson Karen , Ruiz Juan P. , Scott J. Paul
Published: September 2021

Blastomyces is a fungus found in the soil of regions of North America including the Mississippi and Ohio River Valleys. It can be inhaled into the lungs and cause pneumonia and disseminated disease. Although blastomycosis is not widely reported in the sickle cell literature, sickle cell patients may be at increased risk of complications from blastomycosis pneumonia due to their immune compromise and risk of developing acute chest syndrome. We describe the case of a 13-year-old female with homozygous sickle cell disease who presented with pneumonia and acute chest syndrome and was found to have pulmonary blastomycosis.
Clinical Features and Prognostic Factors of Early Outcome in Pediatric Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 227 Cases
Authors: Zhou Yong-Hai , Han Xin-Ru , Xia Fang-Qing , Poonit Neha-Devi , Liu Li
Published: September 2021

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening clinical syndrome in children, and the knowledge of it is still limited. Two hundred twenty-seven children with HLH in our hospital were retrospectively analyzed from January 2001 to December 2018. The age of the patients on admission ranged from 1 day to 14 years old. The 3 most common clinical manifestations include fever (98.7%), hepatomegaly (95.6%), and splenomegaly (92.1%). The decrease of high-density lipoprotein cholesterol (99.1%) is very common in children with HLH. Albumin<25 g/L, activated partial thromboplastin time >65 s, and lactose dehydrogenase >1000 U/L were independent risk factors for poor early prognosis in children with HLH, and their odds ratio values were 2.515, 3.094, and 2.378, respectively, while age >28 months was identified as a protective factor (odds ratio=0.295). Of the 227 children, 67 (29.52%) died within 30 days of onset. The mortality rate in 2013 to 2018 was significantly lower than that in 2001 to 2012 (16.35% vs. 40.65%, P=0.000). The shortening of the time from onset to admission and the reduction of time from admission to definite diagnosis could be some of the reasons for the decrease of HLH mortality in 2013 to 2018 (P<0.05, respectively). Our study suggests that early identification of risk factors for HLH, timely diagnosis and treatment are important measures to improve the short-term prognosis of HLH in children.
Efficacy and Safety of Iron Chelation Therapy After Allogeneic Hematopoietic Stem Cell Transplantation in Pediatric Thalassemia Patients: A Retrospective Observational Study
Authors: Kupesiz Funda T. , Sivrice Cigdem , Akinel Aysenur , Kintrup Gulen T. , Guler Elif , Kupesiz Alphan
Published: September 2021

Background:

Studies on the increased body iron load in patients with thalassemia major have thoroughly demonstrated the problems caused by iron overload. In patients who undergo hematopoietic stem cell transplantation (HSCT) as curative therapy, iron overload continues long after transplantation. There are few pediatric studies on chelation therapy in the posttransplant period. In this study, we present the outcomes of our patients who received posttransplant oral chelation therapy.

Patients and Methods:

This retrospective observational study evaluated the outcomes of pediatric patients with thalassemia major who used oral chelation therapy after allogeneic HSCT at the Akdeniz University Pediatric Bone Marrow Unit between January 2008 and October 2019.

Results:

Deferasirox therapy was initiated in 58 pediatric patients who underwent HSCT for thalassemia. Pretreatment mean serum ferritin was 2166±1038 ng/mL. Treatment was initiated at a mean of 12±6.7 months after transplantation and continued for a mean of 15.7±11.5 months. At treatment discontinuation, the mean serum ferritin was 693±405 ng/mL and the mean reduction was −1472.75±1121.09 ng/mL (P<0.001 vs. posttreatment). Serum ferritin was below 500 ng/mL in 52% of the patients at treatment discontinuation. Manageable side effects such as nausea, vomiting, liver enzyme elevation, and proteinuria were observed in 17% of the patients, while one patient developed ototoxicity.

Conclusions:

Deferasirox therapy effectively reduces iron overload in the posttransplant period. Studies evaluating the effects of early treatment on the graft may help to establish guidelines for posttransplant chelation therapy. Clear guidelines are needed regarding when to initiate and discontinue treatment.
Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern
Authors: Mrad Chaima , Audry Georges , Tabone Marie-Dominique , Le Pointe Hubert Ducou , Coulomb Aurore , Irtan Sabine
Published: September 2021

Botryoid growth pattern (BGP) is a polypoid mass growing into the renal pelvis, rarely seen in bilateral Wilms tumors where it represents a surgical challenge. We report our experience of nephron sparing surgery in 3 patients with BGP in bilateral Wilms tumor. Surgical en bloc removal was performed after calyx opening with no complications. The histology of the BGP was Intralobar Nephrogenic Rest in all cases while all Wilms tumors were of intermediate risk. One patient early recurred. At a follow-up of 9 months, 22 and 23 years, all patients were alive with a moderate renal insufficiency and hypertension.
Barriers to Care and Outcomes of Pediatric Acute Lymphoblastic Leukemia Treatment in the Gaza Strip
Authors: Mills David , Abushanab Salah , Elhaloul Awad , El Nuweiry Heiam , Shbair Mahmoud , Qaddoumi Ibrahim , Salman Zeena
Published: September 2021

Background:

Childhood acute lymphoblastic leukemia (ALL) is the most common pediatric cancer worldwide. Although children in high-income countries enjoy survival rates of ~90%, children in countries with limited resources suffer from survival rates of <35%. No published data on pediatric cancer incidence, management, or outcomes in the Gaza Strip are available.

Methods:

A retrospective cohort study was undertaken for pediatric (below 12 y of age) ALL diagnoses admitted to the only pediatric cancer ward in the Gaza Strip between 2010 and 2015. Outcomes included event-free survival (EFS) and overall survival (OS) calculated by Kaplan-Meier estimates. Events were defined as induction failure, relapse, and death.

Results:

The 3-year EFS estimate was 80% (95% confidence interval [CI], 66%-89%). The EFS at 1 and 3 years for high-risk ALL was 55% (95% CI, 27%-76%) and 23% (95% CI, 4%-51%), respectively. The 3-year OS was 93% (95% CI, 82%-97%). The 3-year OS for high-risk ALL was 69% (95% CI, 30%-90%). All 84 (100%) patients required referral to an outside hospital for definitive ALL diagnoses and induction therapy. Forty-four (52%) patients required at least one additional referral.

Conclusions:

The overall outcomes demonstrated relatively high survival rates at 3 years which may be artificially elevated due to exclusion of adolescents, limited follow up, and deceased patient charts unavailable. Structural determinants of health in Gaza lead to limited diagnostic and treatment capabilities, limited access to advanced medical training, and reliance on out-of-territory transfers for care. These barriers impact the access to comprehensive pediatric care within the Gaza Strip.
Stability of Symptom Clusters in Patients With Gynecologic Cancer Receiving Chemotherapy
Authors: Pozzar Rachel A. , Hammer Marilyn J. , Cooper Bruce A. , Kober Kord M. , Chen Lee-May , Paul Steven M. , Conley Yvette P. , Cartwright Frances , Wright Fay , Levine Jon D. , Miaskowski Christine
Published: September 2021

Background

Patients with gynecologic cancer undergoing chemotherapy experience multiple co-occurring symptoms. Understanding how symptom clusters change over time is essential to the development of interventions that target multiple co-occurring symptoms.

Objective

The aim of this study was to assess the relative stability of symptom clusters across a chemotherapy cycle in patients with gynecologic cancer.

Methods

This is a longitudinal, descriptive study. Eligible patients (n = 232) were English-speaking adults (≥18 years old) with gynecologic cancer. Data were collected in the week before patients' second or third cycle of chemotherapy (T1) and at 1 (T2) and 2 (T3) weeks after chemotherapy. Three dimensions of the symptom experience (occurrence, severity, and distress) were assessed using a modified version of the Memorial Symptom Assessment Scale. Symptom clusters for each dimension and time point were identified through exploratory factor analysis.

Results

A 5-factor solution was selected for each exploratory factor analysis. Hormonal, respiratory, and weight change clusters were identified across all dimensions and time points. A psychological symptom cluster was identified at T1 for occurrence and severity and at T2 and T3 for all 3 dimensions. A gastrointestinal symptom cluster was identified at T1 for occurrence and at T2 and T3 for all 3 dimensions. The hormonal, respiratory, psychological, and weight change symptom clusters exhibited common symptoms across dimensions and time points.

Conclusions

Hormonal, respiratory, weight change, and psychological symptom clusters are relatively stable across a cycle of chemotherapy in patients with gynecologic cancer.

Implications for Practice

Clinicians need to assess patients for multiple co-occurring symptoms and initiate multimodal interventions.
Factors Correlated With Fear of Cancer Recurrence in Cancer Survivors
Authors: Zhang Xu , Sun Di , Qin Nan , Liu Meishuo , Jiang Nan , Li Xiaohan
Published: September 2021

Background

Fear of cancer recurrence (FCR) is one of the most common and aversive psychological phenomena among cancer survivors. Understanding the independent and potentially modifiable risk factors that contribute toward FCR seen in cancer survivors would inform future interventional trials aimed at reducing this risk.

Objective

The aim of this study was to provide an evidence synthesis of factors correlated with FCR to inform the development of preventive interventions.

Methods

A literature search was performed of the PsycINFO, EMBASE, and MEDLINE (PubMed) databases. Reports published from inception to 2020 focusing on the correlates of FCR with physical, psychological, and social factors were identified. Authors assessed the studies’ risk of bias in accordance with the Standard Quality Assessment Criteria for Quantitative Studies (QualSyst criteria) and followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. After extracting data and assessing the quality of the included studies, meta-analysis was conducted using RevMan 5.3 software.

Results

Thirty-four articles were included in this study with a total sample of more than 13 000 cancer patients. Fear of cancer recurrence was positively correlated with anxiety, depression, avoidance, chemotherapy, distress, intrusive thoughts, fatigue, rumination, and neuroticism. It was negatively correlated with optimism, age, social support, quality of life, time since diagnosis, well-being, and self-efficacy.

Conclusions

We identified 16 factors that are correlated with FCR, 9 positively correlated with FCR and 7 negatively correlated with FCR.

Implications for Practice

The findings of this study provide direction for the development of precise interventions for FCR in cancer survivors and lay the foundation for the further construction of an FCR-related nursing theoretical framework.
If We Make It, Will They Take It? Attitudes Toward the Acceptability of Chlamydia Point-of-Care Testing Among Adolescents and Young Adults
Authors: Pastolero Peter Coronel , Suss Amy , Cambridge Rhonda , Hammerschlag Margaret R.
Published: September 2021

Background

Adolescent women, 15 to 19 years of age, have the highest rate of Chlamydia trachomatis infection in the United States. The objective of this study was to ascertain knowledge and experience of C. trachomatis and acceptance of C. trachomatis point-of-care testing (POCT) if made available over-the-counter (OTC). Currently, there are no tests for C. trachomatis available OTC for purchase.

Methods

Patients attending adolescent clinics at University Hospital of Brooklyn and Kings County Hospital received an anonymous 12-item questionnaire. Both clinics serve predominantly African and Caribbean American urban populations. Questions included demographics, sexual orientation, chlamydia knowledge, testing history, prior infection, partner notification, and acceptance of OTC POCT for C. trachomatis.

Results

Surveys from 151 patients (116 women, 35 men) aged 12 to 21 years (mean age, 17.6 years) were analyzed. Only 34 of the 151 (22.5%) respondents understood C. trachomatis transmission; 31 (20.5%) knew its complications. Sixty-seven (44.4%) would purchase an OTC test but 101 (66.8%) would pay no more than $20. All 151 patients reported that they would follow-up with doctor if positive; 143 (94.7%) would notify partners, although 5 of the 31 (16.1%) women with prior infection did not notify partners.

Conclusions

Nearly half (44%) of adolescents in our population would be interested in using a potential OTC test for C. trachomatis. Cost was a major disincentive. Knowledge of infection remains cursory. However, those with a history of C. trachomatis infection and familiar with its complications were more interested in purchasing a home test. Although 100% of the respondents reported that they would follow-up with their physician if they tested positive, past behavior suggests that partner notification might be suboptimal.
Reforms, Errors, and Dermatopathology Malpractice: Then and Now: A Comprehensive Retrospective
Authors: Mahalingam Meera
Published: September 2021

Medical malpractice occurs when a hospital, doctor, or other health care professional, through a negligent act or omission, causes an injury to a patient. The negligence might be the result of errors in diagnosis, treatment, aftercare, or health management. To be considered medical malpractice under the law, the claim must violate the standard of care, the injury must be caused by the negligence and, last but most certainly not least, the injury must result in significant damages. This review is an overview of medicolegal issues specific to the practice of Dermatopathology with the caveat that most are likely pertinent to other specialties of pathology as well. The safety of patients remains the priority in pathology as it does in any medical undertaking, and this is no different in the practice of Dermatopathology. The review is broadly divided in 2 parts—we begin with an overview of tort reforms, advocated by physicians to reduce costs associated with malpractice defense. In the second part we address practical issues specific to the practice of pathology and dermatopathology. These include among others, errors-related to the biopsy type, inadequacy of clinical information regarding the lesion that is biopsied, role of interstate dermatopathology as well as examples of select entities commonly misdiagnosed in dermatopathology. In the last decade, artificial intelligence (AI) has moved to the forefront of technology. While research into the uses of AI in pathology is promising, the use of AI in diagnostic practice is still somewhat uncommon. Given that AI is not fully integrated routinely as a diagnostic adjunct, its’ impact on pathology-specific medicolegal issues cannot, as yet at least, be defined. Restriction of medical malpractice is of particular relevance in the COVID-19 era, a period that is anything but normal. The response of states with specific pandemic-related guidelines is addressed with the caveat that this particular issue is only covered in select states. Furthermore, given that the COVID pandemic is only a year old, while it does not appear to have had an immediate impact on pathology-specific medicolegal matters, it is possible that the role of COVID on this issue, if any at all, will and can only be fully defined a few years down the line.