The Impact of a Prior Norwood Procedure on Cardiac Transplantation in Failed Fontan Physiology

Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a prior Norwood(FF).Methods:A single-institution retrospective review of all patients with Fontan failure who under-went cardiac transplantation from 2003–2021 was completed-22 underwent prior Norwood(FFN)and 11 did not(FF).Descriptive and inferential statistics were calculated for operative course and patient outcomes.Results:The operative course of the FFN cohort appeared to be more complex(not statistically significant,but clinically relevant)-this group exclusively experienced sternal re-entry events(3 of 22 patients)and concomitant neo-aor-tic reconstruction(6 patients),had a longer duration of surgery(median of 682 min vs.575.5 min),more time on circulatory arrest(median of 25.5 min vs.12.5 min),and more frequent use of open sternal management[50%of patients(11/22)vs.27.3%of patients(3/11)].Postoperatively,these patients underwent more mediastinal explora-tions[other than sternal closure;40.9%of patients(9/22)vs.18.2%of patients(2/11)],spent more time on mechanical ventilation(median of 5 days vs.2 days),had a longer length of stay(median of 30 days vs.19 days),and required more catheter-based re-interventions[22.7%of patients(5/22)vs.9.1%of patients(1/11)].Conclu-sion:Although underpowered,our results suggest that the operative course of FFN patients is more challenging,based mostly on neo-aortic arch issues.In turn,this likely leads to a more complex postoperative course.We are currently collaborating with other institutions to increase the cohort size and power of the study.

A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975.

The Effects of Preoperative Imaging on Surgical Outcome for Pediatric Appendicitis Patients Special Issue: Pediatric Surgery

Computed tomography (CT) has been the major imaging modality for the diagnosis of appendicitis because of its excellent imaging resolution and the ease of interpretation, but a concern has been raised regarding radiation exposure. We have shifted from CT to ultrasonography (US) as the primary imaging modality because of this concern. A retrospective review is conducted to determine whether a combined US/CT strategy can reduce radiation exposure while maintaining the same surgical outcome. Five hundred eighty-six patients between 10 and 18 years of age who underwent an appendectomy from 2007-2011wereincluded in our study. The patients were classified as Early (prior to 7/1/2009) or Late (after 7/1/2009) with the knowledge that a conscientious institutional effort was made to start utilizing US as the primary imaging modality, limiting CT use to selective cases in 2009. The diagnostic imaging studies performed (CT, US or no imaging) were recorded. Operative findings, pathology reports and clinical course were reviewed. The rates of acute appendicitis (AA), negative appendectomies (NA) and perforated appendicitis (PA) were calculated. During the Early period, 49.3% of patients (n = 150) had undergone CT scans prior to their appendectomy whereas only 37.9% (n = 107) had CT scans during the Late period. Of the CT scans obtained during the Late period, only 18.4% of patients (n = 52) had a CT scan first, and 55 CT scans were done after an initial US. Patient disease patterns were similar between Early and Late years. While no difference in NA rates for boys was found between the Early and Late periods, a significantly higher incidence of NA rates was found in girls in the Early period as opposed to the Late period. We conclude that a shift in practice patterns to reduce radiation exposure for pediatric patients with appendicitis has not resulted in inferior surgical outcome.

Remission of primary low-grade gastric lymphomas of the mucosa-associated lymphoid tissue type in immunocompromised pediatric patients

We report the remission of primary gastric lymphoma of the mucosa-associated lymphoid tissue （MALT） type in two immunocompromised pediatric patients. Patient 1, a 14-year-old boy in an immunocompromised state of unknown cause, complained of repeated abdominal pain. Examinations revealed gastric MALT with local invasion and lymph node involvement. Serum anti-Helicobacter pylori （H pylon） antibody was positive. H pylori eradication was abandoned due to its adverse effects. The MALT lesion spontaneously regressed over the next 24 months without any treatment for lymphoma. Patient 2, a 6-year-old boy, underwent cord blood transplantation for the treatment of adrenoleukodystrophy. He was administered immunosuppressants for graft-versus-host disease after transplantation. Nausea and hematochezia appeared and further examinations revealed gastric MALT with H pylori gastritis. Treatment consisting of medication for the Hpylori infection alone eradicated the Hpylori and completely resolved the patient＇s MALT lesion, as well. Patients i and 2 were followed up over periods of 10 years and 3 years, respectively, without any signs of relapse. In conclusion, gastric lymphoma of the MALT type can be cured by conservative treatment even in immunocompromised pediatric patients.

Assessment of pathogens and risk factors associated with bloodstream infection in the year after pediatric liver transplantation

BACKGROUND Bloodstream infection(BSI)is one of the most significantly adverse events that can occur after liver transplantation(LT)in children.AIM To analyze the profile of BSI according to the postoperative periods and assess the risk factors after pediatric LT.METHODS Clinical data,collected from medical charts of children(n=378)who underwent primary LT,were retrospectively reviewed.The primary outcome considered was BSI in the first year after LT.Univariate and multivariate analyses were performed to identify risk factors for BSI and respective odds ratios(ORs).RESULTS Of the examined patients,106(28%)experienced 162 episodes of pathogen-confirmed BSI during the first year after LT.There were 1.53±0.95 episodes per children(mean±SD)among BSIcomplicated patients with a median onset of 0.4 mo post-LT.The most common pathogenic organisms identified were Coagulase-negative staphylococci,followed by Enterococcus spp.and Streptococcus spp.About half(53%)of the BSIs were of unknown origin.Multivariate analysis demonstrated that young age(≤1.3 year;OR=2.1,P=0.011),growth failure(OR=2.1,P=0.045),liver support system(OR=4.2,P=0.008),and hospital stay of>44 d(OR=2.3,P=0.002)were independently associated with BSI in the year after LT.CONCLUSION BSI was frequently observed in patients after pediatric LT,affecting survival outcomes.The profile of BSI may inform clinical treatment and management in high-risk children after LT.

Characteristics and trends of pediatric firepit burns: insights for prevention and safety

Introduction As fire pits grow in popularity,so do the associated burn injuries.Our study examines pediatric fire pit burns characteristics to raise awareness and promote safety precautions.Methods We conducted a retrospective review of pediatric patients(<21 years)with firepit burns at a tertiary care hospital from 2016 to 2021.Results Eighty-four patients were identified,of whom 70.2%were male,with a median age of 62 months.The median percent total body surface area burned was 2%(interquartile range(IQR)=1-4).Thirty-five(41.7%)patients were admitted and 7(8.3%)underwent grafting.Neck and trunk burns had the highest grafting rates(66%and 33%,respectively).The hands(41.7%)and the lower extremities(27.4%)were the most frequently burned body areas.The leading causes of burns were ashes/hot coals(34.5%),flames(31.0%),and direct contact(25.0%),often resulting from falling into the fire(59.5%)or running or playing in activities near it(26.2%).Thirty-five(41.7%)were admitted for inpatient management,while 49(58.3%)were treated as outpatient.Eleven(13.2%)underwent at least one reconstructive surgery,7(8.4%)had at least one rehabilitation visit,and 65(77.4%)had follow-up clinic visits.The median length of stay was 2 days(IQR=1.0-3.5).The peak months for burns were from August through October(n=40,46.0%),with an increase observed from 10 cases in 2016to 20 cases in 2020.Conclusions Given the significant proportion of firepit burns resulting from unsafe fire behaviors,it is crucial that caretakers are aware of proper firepit safety precautions.Level ofevidence Il.

Predictors of the outcomes of acute-on-chronic hepatitis B liver failure

AIM： To identify the risk factors in predicting the out- come of acute-on-chronic hepatitis B liver failure pa- tients. METHODS： We retrospectively divided 113 patients with acute-on-chronic liver failure-hepatitis B virus （ACLF-HBV） and without concurrent hepatitis C or D virus infection and hepatocellular carcinoma into two groups according to their outcomes after anti-HBV therapy. Their demographic, clinical, and biochemical data on the day of diagnosis and after the first week of treatment were analyzed using the Mann-Whitney U test, Fisher＇s exact test, and a multiple logistic regres- sion analysis. RESULTS： The study included 113 patients （87 men and 26 women） with a mean age of 49.84 years. Fifty- two patients survived, and 61 patients died. Liver failure （85.2%）, sepsis （34.4%）, and multiple organ failure （39.3%） were the main causes of death. Mul- tivariate analyses showed that Acute Physiology and Chronic Health Evaluation （APACHE） Ⅱ scores ≥ 12 [odds ratio （OR） = 7.160, 95% CI： 2.834-18.092, P 〈 0.001] and positive blood culture （OR = 13.520, 95% CI： 2.740-66.721, P = 0.001） on the day of diagnosis and model for end-stage liver disease （MELD） scores 28 （OR = 8.182, 95% CI： 1.884-35.527, P = 0.005） after the first week of treatment were independent predictors of mortality. CONCLUSION： APACHE II scores on the day of diag- nosis and MELD scores after the first week of anti-HBV therapy are feasible predictors of outcome in ACLF- HBV patients.

Pediatric inflammatory bowel disease

Inflammatory bowel disease is an important cause of gastrointestinal pathology in children and adolescents. The incidence of pediatric inflammatory bowel disease is increasing; therefore, it is important for the clinician to be aware of the presentation of this disease in the pediatric population. Laboratory tests, radiology studies, and endoscopic procedures are helpful in diagnosing inflammatory bowel disease and differentiating between Crohn＇s disease and ulcerative colitis. Once diagnosed, the goal of medical management is to induce remission of disease while minimizing the side effects of the medication. Specific attention needs to be paid to achieving normal growth in this susceptible population. Surgical management is usually indicated for failure of medical management, complication, or malignancy. Algorithms for diagnostic evaluation and treatment of pediatric inflammatory bowel disease are presented. The specific psychosocial issues facing these patients are also discussed in this review as are the future goals of research in the complex problem of pediatric inflammatory bowel disease.

The impact of cleft lip and palate repair on maxillofacial growth

Surgical correction is central to current team-approached cleft treatment. Cleft surgeons are always concerned about the impact of their surgical maneuver on the growth of the maxilla. Hypoplastic maxilla, concaved mid-face and deformed dental arch have constantly been reported after cleft treatments. It is very hard to completely circumvent these postoperative complications by current surgical protocols. In this paper, we discussed the factors that inhibit the maxillofacial growth on cleft patients. These factors included pre-surgical intervention, the timing of cleft palate and alveolae repair, surgical design and treatment protocol. Also, we made a review about the influence on the maxillary growth in un-operated cleft patients. On the basis of previous researches, we can conclude that most of scholars express identity of views in these aspects： early palatoplasty lead to maxilla growth inhibition in all dimensions; secondary alveolar bone graft had no influence on maxilla sagittal growth; cleft lip repair inhibited maxilla sagittal length in patients with cleft lip and palate; Veau＇s pushback palatoplasty and Langenbeck＇s palatoplasty with relaxing incisions were most detrimental to growth; Furlow palatoplasty showed little detrimental effect on maxilla growth; timing of hard palate closure, instead of the sequence of hard or soft palate repair, determined the postoperative growth. Stilt, scholars hold controversial viewpoints in some issues, for example, un-operated clefts have normal growth potential or not, pre-surgical intervention and pharyngoplasty inhibited maxillofacial growth or not.

Western view of the management of gastroesophageal foreign bodies

The best modality for foreign body removal has beenthe subject of much controversy over the years.We have read with great interest the recent article by Souza Aguiar Municipal Hospital,Rio de Janeiro,Brazil,describing their experience with the management of esophageal foreign bodies in children.Non-endoscopic methods of removing foreign bodies(such as a Foley catheter guided or not by fluoroscopy)have been successfully used at this center.These methods could be an attractive option because of the following advantages:Shorter hospitalization time;easy to perform;no need for anesthesia;avoids esophagoscopy;and lower costs.However,the complications of these procedures can be severe and potentially fatal if not performed correctly,such as bronchoaspiration,perforation,and acute airway obstruction.In addition,it has some disadvantages,such as the inability to directly view the esophagus and the inability to always retrieve foreign bodies.Therefore,in Western countries clinical practice usually recommends endoscopic removal of foreign bodies under direct vision and with airway protection whenever possible.

Accurate diagnosis of prenatal cleft lip/palate by understanding the embryology

Cleft lip with or without cleft palate(CP) is one of the most common congenital malformations. Ultrasonographers involved in the routine 20-wk ultrasound screening could encounter these malformations. The face and palate develop in a very characteristic way. For ultrasonographers involved in screening these patients it is crucial to have a thorough understanding of the embryology of the face. This could help them to make a more accurate diagnosis and save time during the ultrasound. Subsequently, the current postnatal classification will be discussed to facilitate the communication with the CP teams.

Rare case of Helicobacter pylori-positive multiorgan Ig G4-related disease and gastric cancer

A 61-year-old male from Northeast China presented with a 2-mo history of abdominal distension,pruritus and jaundice.Laboratory testing revealed an elevated serum Ig G4 level.A computed tomography scan showed a typical feature of autoimmune pancreatitis(AIP) and cholecystocholangitis.Early gastric cancer was incidentally discovered when endoscopic untrasound-guided fine needle aspiration(EUSFNA) of the pancreas was carried out.The patient underwent radical subtotal gastrectomy for gastric cancer combined with cholecystectomy.Helicobacter pylori(H.pylori) and Ig G4-positive plasmacytes were detected in gastric cancer tissue,pancreatic EUSFNA sample and resected gallbladder specimen by immunohistochemistry.The patient was diagnosed with H.pylori-positive Ig G4-related AIP and sclerosing cholecystocholangitis as well as H.pylori-positive gastric cancer.He responded well to steroid therapy and remains healthy with no signs of recurrence at one year follow-up.We speculate that H.pylori might act as a trigger via direct or indirect action in the initiation of onset of gastric cancer and multiorgan Ig G4-related disease.

Ponseti method compared with soft-tissue release for the management of clubfoot:A meta-analysis study

AIM: To compare the functional outcomes of patients who underwent open surgery vs Ponseti method for the management of idiopathic clubfoot and to determine whether correlations exist between functional outcome and radiographic measurements.METHODS: A meta-analysis of the literature was conducted for studies concerning primary treatment of patients with idiopathic clubfoot. We searched PubM ed Medline, EMBASE, and the Cochrane Library databases from January 1950 to October 2011. Meta-analyses were performed on outcomes from 12 studies. Pooled means, SDs, and sample sizes were either identified in the results or calculated based on the results of each study.RESULTS: Overall, 835 treated idiopathic clubfeet in 516 patients were reviewed. The average follow-up was 15.7 years. Patients managed with Ponseti method did have a higher rate of excellent or good outcome than patients treated with open surgery(0.76 and 0.62, respectively), but not quite to the point of statistical significance(Q = 3.73, P = 0.053). Age at surgery wasnot correlated with the functional outcome for the surgically treated patients(r =-0.32, P = 0.68). A larger anteroposterior talocalcaneal angle was correlated with a higher rate of excellent or good outcomes(r = 0.80, P = 0.006). There were no other significant correlations between the functional and radiographic outcomes.CONCLUSION: The Ponseti method should be considered the initial treatment of idiopathic clubfeet, and open surgery should be reserved for clubfeet that cannot be completely corrected.

CD4+Foxp3+CD25+/-Tregs characterize liver tissue specimens of patients suffering from drug-induced autoimmune hepatitis:A clinical-pathological study

Background: The diagnosis of drug-induced autoimmune hepatitis(DIAIH) and its differentiation from idiopathic autoimmune hepatitis(AIH) is challenging. This study aimed to differentiate DIAIH from AIH by comparing the biochemical changes, histological features, and frequencies of CD4~+Foxp3~+CD25+/-regulatory T cells(Tregs) in liver tissues or peripheral blood lymphocytes.Methods: A total of 15 DIAIH patients and 24 AIH patients who underwent liver biopsies at initial presentation were enrolled in this study. The liver histological changes were assessed by HE staining. The phenotypic recognition and distribution of CD4~+Foxp3~+CD25+/-Tregs in liver tissues were evaluated by single/double immunostains in serial sections. The CD4~+Foxp3~+CD25+/-Tregs in peripheral blood were analyzed by flow cytometry.Results: The median values of ALT and AST were 404.50 U/L and 454.10 U/L in DIAIH patients and309.50 U/L and 315.00 U/L in AIH patients, respectively. More importantly, for the first time we found that patients with DIAIH had higher levels of serum ALT and AST, more severe degree of lobular inflammation,higher frequencies of zone 3 necrosis and higher number of lobular CD4~+Foxp3~+CD25~-Tregs compared with AIH(P < 0.05). Furthermore, there were positive correlations in DIAIH between the degree of lobular inflammation and either the AST/ALT level or the number of lobular CD4~+Foxp3~+CD25~-Tregs(P < 0.05).However, the frequency of peripheral blood CD4~+Foxp3~+CD25+/-Tregs were not significantly different between DIAIH and AIH.Conclusions: The differences of ALT, AST and the number of lobular CD4~+Foxp3~+CD25~-Tregs between patients with DIAIH and those with AIH are clinically helpful in differentiating these two diseases in their early stage.

Novel mutation in the ligand-binding domain of the androgen receptor gene （1790p） associated with complete androgen insensitivity syndrome

Mutations in the X-linked androgen receptor （AR） gene cause androgen insensitivity syndrome （AIS）, resulting in an impaired embryonic sex differentiation in 46,XY genetic men. Complete androgen insensitivity （CAIS） produces a female external phenotype, whereas cases with partial androgen insensitivity （PALS） have various ambiguities of the genitalia. Mild androgen insensitivity （MAIS） is characterized by undermasculinization and gynecomastia. Here we describe a 2-month-old 46,XY female patient, with all of the characteristics of CAIS. Defects in testosterone （T） and dihydrotestosterone （DHT） synthesis were excluded. Sequencing of the AR gene showed the presence in exon 6 of a T to C transition in the second base of codon 790, nucleotide position 2369, causing a novel missense Leu790Pro mutation in the ligand-binding domain of the AR protein. The identification of a novel AR mutation in a girl with CAIS provides significant information due to the importance of missense mutations in the ligand-binding domain of the AR, which are able to induce functional abnormalities in the androgen binding capability, stabilization of active conformation, or interaction with coactivators.

Clinical significance of connective tissue growth factor in hepatitis B virus-induced hepatic fibrosis

AIM:To determine the utility of connective tissue growth factor(CCN2/CTGF) for assessing hepatic fibrosis in hepatitis B virus(HBV)-induced chronic liver diseases(CLD-B).METHODS:Enzyme-linked immunosorbent assay was used to measure CCN2 in sera from 107 patients with chronic hepatitis B(CHB) and 39 patients with HBVinduced active liver cirrhosis and 30 healthy individuals.Liver samples from 31 patients with CHB,8 patients with HBV-induced liver cirrhosis and 8 HBV carriers with normal liver histology were examined for transforming growth factor β-1(TGF-β1) or CCN2 mRNA levels by in situ hybridization,and computer image analysis was performed to measure integrated optimal density(IOD) of CCN2 mRNA-positive cells in liver tissues.Histological inflammation grading and fibrosis staging were evaluated by H and E staining and Van Gieson's method.RESULTS:Serum CCN2 concentrations were,respectively,4.0-or 4.9-fold higher in patients with CHB or active liver cirrhosis as compared to healthy individuals(P < 0.01).There was good consistency between the levels of CCN2 in sera and CCN2 mRNA expression in liver tissues(r = 0.87,P < 0.01).The levels of CCN2 in sera were increased with the enhancement of histological fibrosis staging in patients with CLD-B(r = 0.85,P < 0.01).Serum CCN2 was a reliable marker for the assessment of liver fibrosis,with areas under the receiver operating characteristic(ROC) curves(AUC) of 0.94 or 0.85 for,respectively,distinguishing normal liver controls from patients with F1 stage liver fibrosis or discriminating between mild and significant fibrosis.CONCLUSION:Detection of serum CCN2 in patients with CLD-B may have clinical significance for assessment of severity of hepatic fibrosis.

Endoscopic treatment of a large colonic polyp as a cause of colocolonic intussusception in a child

Colocolonic intussusception is an uncommon cause of intestinal obstruction in children.The most common type is idiopathic ileocolic intussusception.However,pathologic lead points occur approximately in 5%of cases.In pediatric patients,Meckel’s diverticulum is the most common lead point,followed by polyps and duplication.We present a case of recurrent colocolonic intussusception which caused colonic obstruction in a 10-year-old boy.A barium enema revealed a large polypoid mass at the transverse colon.Colonoscopy showed a colonic polyp,3.5 centimeters in diameter,which was successfully removed by endoscopic polypectomy.

A novel fibroblast growth factor 15-dependent and bile acid-independent promotion of liver regeneration in mice

Objective:The role of intestine-derived factors in promoting liver regeneration after partial hepatectomy(PHx)are not entirely known,but bile acids(BAs)and fibroblast growth factor 15(Fgf15)that are highly expressed in the mouse ileum could promote hepatocyte proliferation.Fgf15 strongly suppresses the synthesis of BAs,and emerging evidence indicates that Fgf15 is important for liver regeneration.

Liver transplantation with simultaneous splenectomy increases risk of cancer development and mortality in hepatocellular carcinoma patients

BACKGROUND Splenectomy has previously been found to increase the risk of cancer development,including lung,non-melanoma skin cancer,leukemia,lymphoma,Hodgkin’s lymphoma,and ovarian cancer.The risk of cancer development in liver transplantation(LT)with simultaneous splenectomy remains unclear.AIM To compare hepatocellular carcinoma(HCC)recurrence and de novo malignancy between patients undergoing LT with and without simultaneous splenectomy.METHODS We retrospectively analyzed the outcomes of 120 patients with HCC within the University of California San Francisco criteria who received LT with(n=35)and without(n=85)simultaneous splenectomy in the Tri-Service General Hospital.Univariate and multivariate Cox regression analyses for cancer-free survival and mortality were established.The comparison of the group survival status and group cancer-free status was done by generating Kaplan–Meier survival curves and log-rank tests.RESULTS The splenectomy group had more hepatitis C virus infection,lower platelet count,higher-fetoprotein level,and longer operating time.Splenectomy and age were both positive independent factors for prediction of cancer development[hazard ratio(HR):2.560 and 1.057,respectively,P<0.05].Splenectomy and hypertension were positive independent factors for prediction of mortality.(HR:2.791 and 2.813 respectively,P<0.05).The splenectomy group had a significantly worse cancer-free survival(CFS)and overall survival(OS)curve compared to the non-splenectomy group(5-year CFS rates:53.4%vs 76.5%,P=0.003;5-year OS rate:68.1 vs 89.3,P=0.002).CONCLUSION Our study suggests that simultaneous splenectomy should be avoided as much as possible in HCC patients who have undergone LT.

Persistent bowel dysfunction after surgery for Hirschsprung’s disease:A neuropathological perspective

Hirschsprung’s disease(HD)is a congenital disorder,characterized by aganglionosis in the distal part of the gastrointestinal tract.Despite complete surgical resection of the aganglionic segment,both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options.There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD,which may play a role in persistent bowel dysfunction.These abnormalities include:(1)Histopathological abnormalities of enteric neural cells;(2)Imbalanced expression of neurotransmitters and neuroproteins;(3)Abnormal expression of enteric pacemaker cells;(4)Abnormalities of smooth muscle cells;and(5)Abnormalities within the extracellular matrix.Hence,a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction.In the long term,further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.