Extended Application of the Hybrid Procedure in Neonates with Left-Sided Obstructive Lesions in an Evolving Cardiac Program

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1–29 days) and median weight of 3.4 kg (range 2.4–4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1–74 days). Median hospital stay post-procedure was 47 days (range 15–270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1–4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.     

Hybrid Palliation for Ductal-Dependent Systemic Circulation

We reviewed our hybrid palliation experience for 91 neonates, with ductal-dependent systemic circulation, born between August 2007 and October 2015. For analysis, we stratified the 91 patients by a risk factor (RF) score and divided them into three groups: (1) high-risk two-functional ventricles (2V) median RF score of 3 (N = 20); (2) low-risk one-functional ventricle (1V) RF score 0–1 (N = 32); and (3) high-risk 1V RF score ≥2 (N = 39). Midterm survival (median 4 years) by group was: (1) 95 %, (2) 91 %, and (3) 15 %, (p = 0.001). In conclusion, hybrid palliation was associated with excellent midterm results for high-risk 2V and low-risk 1V patients with ductal-dependent systemic circulation. In contrast, high-risk 1V patients had significantly worse outcomes.     

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.     

Feasibility and Efficacy of Defatted Human Milk in the Treatment for Chylothorax After Cardiac Surgery in Infants

Chylothorax is a well-described complication after cardiothoracic surgery in children. Medical nutritional therapy for chylothorax includes medium-chain triglyceride (MCT) formulas and reduction in enteral long-chain triglyceride intake to reduce chyle production. Human milk is usually eliminated from the diet of infants with chylothorax because of its high long-chain triglyceride content. However, given the immunologic properties of human milk, young infants with chylothorax may benefit from using human milk over human milk substitutes. We performed a retrospective cohort study to describe the feasibility and efficacy of defatted human milk (DHM) for the treatment for chylothorax in infants after cardiac surgery and to compare growth outcomes between infants treated with DHM (n = 14) versus MCT formula (n = 21). There were no differences in mortality or length of hospital stay between the DHM and MCT formula treatment groups. The DHM treatment group had a significantly higher weight-for-age z-score at hospital discharge compared to the MCT formula group with median z-scores of ?1 (?2 to 0.5) and ?1.5 (?2 to 0), respectively (p = 0.02). In infants with chylothorax after cardiac surgery, DHM is a safe and feasible medical nutritional treatment and may have potential benefits for improved nutrition and growth.     

Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy

To date limited data are available to predict the progression to end-stage heart failure (HF) with subsequent death (non-SCD), need for heart transplantation, or sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). We aimed to determine predictors of long-term outcome in children with HCM. A total of 112 children (median 14.1, IQR 7.8–16.6 years) were followed up for the median of 6.5 years for the development of morbidity and mortality, including arrhythmic and HF-related secondary end points. HF end point included HF-related death or heart transplant, and arrhythmic end point included resuscitated cardiac arrest, appropriate ICD discharge, or SCD. Overall, 23 (21 %) patients reached the pre-defined composite primary end point. At 10-year follow-up, the event-free survival rate was 76 %. Thirteen patients (12 %) reached the secondary arrhythmic end point, and 10 patients (9 %) reached the secondary HF end point. In multivariate model, prior cardiac arrest (r = 0.658), QTc dispersion (r = 0.262), and NSVT (r = 0.217) were independent predictors of the arrhythmic secondary end point, while HF (r = 0.440), LV posterior wall thickness (r = 0.258), LA size (r = 0.389), and decreased early transmitral flow velocity (r = 0.202) were all independent predictors of the secondary HF end point. There are differences in the risk factors for SCD and for HF-related death in childhood HCM. Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years. LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.     

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

Objectives

To report data on Idiopathic inflammatory myopathies (IIM) from eastern India.

Methods

All IIM patients diagnosed over the last 5 y (2011–2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata.

Results

Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]. The overall sex ratio was M: F = 1: 2.6. The mean age at diagnosis was 6.94 y for JDM and 7 y for OM. The mean interval from onset to diagnosis was 5.2 mo. All patients had heliotrope rash and proximal myopathy (n = 11,100%). Other findings included Gottron papule (n = 7; 64%), arthritis (n = 6; 54%), malar rash (n = 5; 45%), dysphagia (n = 4; 36%), nasal intonation (n = 3; 27%), subcutaneous nodule (n = 2; 18%), cutaneous sinus (n = 1; 9%), calcinosis universalis (n = 1; 9%), GI bleed (n = 1; 9%). All patients had raised erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), lactate dehydrogenase (LDH) while 10 had raised creatine kinase (CK). Three were anti-nuclear-antibody (ANA) positive. Electromyography (EMG) showed proximal myopathy in most cases (n = 9; 82%). All patients received corticosteroid. Intravenous immunoglobulin (IVIG) was given to 2 patients. Two received hydroxychloroquine. Cyclophosphamide and azathioprine were given in one each.

Conclusions

This study, first reported profile of IIM from eastern India, showed JDM as the commonest form of IIM with a female preponderance. Five children had complete and 2 had partial remission. Two patients of JDM and 1 of OM died. Increased awareness, early referral, prompt diagnosis and treatment might improve the outcome and survival.

     

Progression of Aortic Regurgitation After Different Repair Techniques for Congenital Aortic Valve Stenosis

We sought to characterize the incidence of AR progression and determine risk factors for AR progression in a consecutive series of infants and children after surgical correction of congenital aortic valvular and supravalvular stenosis. N = 30 patients underwent repair of the aortic valve for isolated congenital aortic valve stenosis (n = 14, 47 %) or combined with aortic regurgitation (AR, n = 16, 53 %). N = 27 (90 %) had a valvular and n = 3 patients (10 %) presented with supravalvular pathology of their aortic valve. In n = 16 patients (53 %) a bicuspid and in n = 2 (6 %) patients, a unicuspid valve was present. Comparative survival was analyzed using the Cox model and log-rank calculations. Log-rank calculations were performed for variables reaching statistical significance in order to identify differences in survival between groups. Commissurotomy was performed in n = 20 patients, patch implantation in n = 4, cusp shaving in n = 8, cusp prolapse correction in n = 3, and cusp augmentation in n = 4 patients. In patients with combined dysfunction and preoperative AR, AR was successfully reduced by the initial procedure, and postoperatively the overall median AR grade was 1+ (range 0–2.5+, p = 0.001, for AR reduction among patients with any grade of preoperative AR). By the time of follow-up echocardiography, the median AR grade had significantly progressed toward 1.5+ (p = 0.004). At the time of mid-term follow-up at 3.2 years, none of the patients had moderate or severe AR grades >2.5+. Patients with a monocuspid aortic valve and patients who had some kind of patch implantation into their cusps or commissures or shaving of thickened cusps were more likely to present with progression of aortic regurgitation. Monocuspid aortic valve and patch implantation, as well as cusp shaving, are probably linked to AR progression. The standard procedure of commissurotomy results in an absolute rate of AR progression of 40 % over a medium-term follow-up period.     

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1–480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35–10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30–3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.     

Feeding Problems and Nutrient Intake in Children with and without Autism: A Comparative Study

Objective

To compare parent reported feeding difficulties and nutritional adequacy of children with Autism Spectrum Disorders (ASD) to an age and socio-economically matched group of typically developing children.

Methods

The scores on Children’s Eating Behavior Inventory (CEBI), three-day food records, anthropometric measures and adequacy of micro- and macro- nutrients were compared for 63 children diagnosed with ASD and 50 typically developing children enrolled from the department of pediatrics of a tertiary care teaching hospital from North India.

Results

The majority (79%) of the parents of ASD children reported some concern regarding their feeding behavior as compared to 64% of the parents of typically developing children. As compared to controls, ASD children had significantly higher CEBI scores (97.28 vs. 89.48, t = 3.15, P = 0.002) and more feeding problems (6.42 vs. 2.70, t = 3.74, P = 0.001). Relative to controls, ASD children consumed fewer number of food items (P = 0.022), particularly fruits (P = 0.004), vegetables (P = 0.011), and proteins (P = 0.015); had significantly lower daily intake of potassium (P = 0.001), copper (P = 0.007), and folate (P = 0.001). Although children with autism did not differ significantly from controls on intake of calories, height, weight, or body mass index, significantly greater proportion of ASD children failed to meet the estimated average requirement of thiamine (P = 0.039), vitamin C (P = 0.013), and copper (P = 0.005).

Conclusions

The findings underscore the need for comprehensive assessment and empirically-supported interventions for eating problems and dietary deficiencies found in ASD children.

     

Clinical Factors Associated with Dose of Loop Diuretics After Pediatric Cardiac Surgery: Post Hoc Analysis

A post hoc analysis of a randomized controlled trial comparing the clinical effects of furosemide and ethacrynic acid was conducted. Infants undergoing cardiac surgery with cardiopulmonary bypass were included in order to explore which clinical factors are associated with diuretic dose in infants with congenital heart disease. Overall, 67 patients with median (interquartile range) age of 48 (13–139) days were enrolled. Median diuretic dose was 0.34 (0.25–0.4) mg/kg/h at the end of postoperative day (POD) 0 and it significantly decreased (p = 0.04) over the following PODs; during this period, the ratio between urine output and diuretic dose increased significantly (p = 0.04). Age (r ?0.26, p = 0.02), weight (r ?0.28, p = 0.01), cross-clamp time (r 0.27, p = 0.03), administration of ethacrynic acid (OR 0.01, p = 0.03), and, at the end of POD0, creatinine levels (r 0.3, p = 0.009), renal near-infrared spectroscopy saturation (?0.44, p = 0.008), whole-blood neutrophil gelatinase-associated lipocalin levels (r 0.30, p = 0.01), pH (r ?0.26, p = 0.02), urinary volume (r ?0.2755, p = 0.03), and fluid balance (r 0.2577, p = 0.0266) showed a significant association with diuretic dose. At multivariable logistic regression cross-clamp time (OR 1.007, p = 0.04), use of ethacrynic acid (OR 0.2, p = 0.01) and blood pH at the end of POD0 (OR 0.0001, p = 0.03) was independently associated with diuretic dose. Early resistance to loop diuretics continuous infusion is evident in post-cardiac surgery infants: Higher doses are administered to patients with lower urinary output. Independently associated variables with diuretic dose in our population appeared to be cross-clamping time, the administration of ethacrynic acid, and blood pH.     

Hematopoietic stem cell transplantation for children with <Emphasis Type="Italic">β</Emphasis>-thalassemia major: multicenter experience in China

Background

β-Thalassemia major (β-TM) has become a public health problem in mainland China. Hematopoietic stem cell transplantation (HSCT) has remained the only cure for β-TM in mainland China since 1998.

Methods

This multicenter retrospective study provides a comprehensive review of the outcomes of 50 pediatric patients with β-TM who received HSCT between 1998 and 2009 at five centers in mainland China. Both related (n = 35) and unrelated donors (n = 15) with complete human leukocyte antigen matches were included. The stem cell sources included bone marrow (BM), peripheral blood stem cells, umbilical cord blood (UCB) and a combination of BM and UCB or a combination of BM and peripheral blood stem cells from a single sibling donor.

Results

The probabilities of 5-year overall survival (OS) and thalassemia-free survival (TFS) after the first HSCT were 83.1 and 67.3%, respectively. Graft failure (GF) occurred in 17 patients. Univariate analyses showed that umbilical cord blood transplantation (UCBT) was one of the potential risk factors for decreased OS (P = 0.051), and that UCBT (P = 0.002) was potentially related to TFS. GF incidence was distinct between the UCBT and non-UCBT groups (P = 0.004). Four cases of UCB-BM combined transplantation led to decreased risks of mortality and recurrence. In the UCBT group, related donor transplantation produced more favorable results than unrelated donor transplantation in OS (P = 0.009) but not in TFS (P = 0.217).

Conclusions

GF was the primary cause of UCBT failure. Though UCBT from related donors was not favorable, the combined transplantation of UCB and BM could improve the prognosis of UCBT.

     

Quality improvement program reduces venous thromboembolism in infants and children with long-gap esophageal atresia (LGEA)

Purpose

Patients with long-gap esophageal atresia (LGEA) treated with the Foker process are at increased risk of venous thromboembolism (VTE). An institutional quality improvement program to decrease VTE risk factor exposure and utilize prophylactic anticoagulation was implemented. We aim to evaluate the efficacy and safety of a VTE risk-reduction program in patients with LGEA.

Methods

Implementation and evaluation of a VTE risk-reduction program in patients with LGEA from 2012 to 2015 was performed. Symptomatic VTE with radiographic confirmation were defined as events. Post-program characteristics were evaluated and compared to a historical cohort.

Results

Sixty-seven patients were identified. Two developed VTE (7 %) post-program implementation; compared to 13/40 (33 %) VTE incidence in the historical cohort (p = 0.018). Baseline demographics were similar, including age, esophageal atresia type and gap length. Post-protocol patients had fewer paralysis episodes (p = 0.004), paralysis days (p = 0.003), central venous catheters (p = 0.003), thoracotomies (p < 0.001), ventilator hours (p = 0.02), and decreased hospital (p < 0.001) and ICU stay (p < 0.001). All patients in the VTE risk-reduction program were exposed to prophylactic anticoagulation. No bleeding complications and/or thrombosis-related mortality occurred.

Conclusion

VTE risk-reduction program implementation decreased symptomatic VTE incidence with associated decreases in ICU and hospital length of stay. Prophylactic anticoagulation can be utilized safely in a complicated pediatric surgical population.

     

Pancreatic surgery for tumors in children and adolescents

Purpose

Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies.

Methods

We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015.

Results

Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5–18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7–10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma.

Conclusions

Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.

     

Unplanned Repeat Echocardiography with Sedation in Children: Patient Risk Factors

Patient selection criteria for echocardiography with sedation in children are not well defined. We attempted to identify predictors of unplanned repeat echocardiography with sedation. This was a single-center, case–control study of echocardiograms performed in children aged 1–36 months. Cases underwent unplanned repeat examinations with sedation, while controls did not. Patient variables and study indications were compared. Logistic regression identified the most significant predictors. Cases (n = 104, median time to repeat echocardiogram 17 days, median age 12.9 months) were older than controls (n = 212, median age 5.0 months, P < 0.001). Significantly more cases than controls had structural cardiac disease (64 vs. 23 %) and anatomic complexity ≥moderate (38 vs. 5 %, P < 0.001 for both). Cases more often had Kawasaki disease (11 vs. 2 %), and controls more often had murmur (56 vs. 11 %, P < 0.001 for both). Logistic regression identified age 6 months to <2 years (OR 3.26, 95 % CI 1.70–6.28, P < 0.001), Kawasaki disease (OR 5.20, 95 % CI 1.46–18.50, P = 0.01), and known pre-echocardiogram anatomic complexity ≥moderate (OR 3.99, 95 % CI 1.64–9.66, P = 0.002) as significant risk factors. An indication for murmur was protective (OR 0.32, 95 % CI 0.13–0.76, P = 0.01). We identified several risk factors for unplanned repeat echocardiography with sedation in children, including age 6 months to <2 years, higher anatomic complexity, and Kawasaki disease. Murmur was a protective factor. These results may help pediatric echocardiography laboratories establish criteria for sedation.     

Delayed diagnosis of anorectal malformations (ARM): causes and consequences in a resource-constrained environment

Purpose

Delay in diagnosis or referral of patients with an anorectal malformation (ARM) is associated with significant morbidity and mortality. We describe the frequency and consequences of delay in recognition or referral of patients with ARM. Our study aims to determine whether the source healthcare facility affects the timing of diagnosis and to identify modifiable factors that may expedite diagnosis and referral.

Methods

Retrospective study of patients referred to a pediatric surgical service with a newly diagnosed ARM between July 2002 and December 2010. Data retrieved included patient demographics, perinatal history, time of diagnosis, clinical findings, management, and morbidity and mortality. Delay was defined as diagnosis after 24 h of birth or transfer to the tertiary referral centre more than 24 h after diagnosis.

Results

273 patients (186 males, 87 females) were referred during the study period. Delays were seen in 158 (57.9 %) patients with morbidity in 58 (36.7 %, p < 0.001) and mortality in 9 (5.7 %, p = 0.008). Delay was more frequent amongst babies born in community health centres (CHCs) than in hospitals (90.5 vs. 55.1 %, p = 0.002). The median age at diagnosis was 3 days (range 2 days to 5 years).

Conclusion

There is a need for staff education, particularly in CHCs, concerning routine neonatal examination and normal perineal anatomy. Girls, in whom a decompressive fistula is often present, are at particular risk of diagnostic delay. Delay causes increased morbidity and mortality.

     

Citation classics in pediatrics: a bibliometric analysis

Background

Citation analysis provides insights into the history and developmental trajectory of scientific fields. Our objective was to perform an analysis of citation classics in the journals of pediatric specialty and to examine their characteristics.

Methods

Initially, all the journals listed under the category of pediatrics (n = 120) were identified using Journal Citation Reports. Web of science database was then searched (1950–2016) to select the top-100 cited articles in the above identified pediatric journals. The top-100 cited article were categorized according the study design, sub-specialty, country, institutional affiliation, and language.

Results

The top-100 articles were published in 18 different journals, with Pediatrics having the highest numbers (n = 40), followed by The Journal of Pediatrics (n = 17). The majority (n = 62) of classics were published after 1990. The most cited article had citation count of 3516 and the least cited had a citation count of 593. The USA (n = 71) was the most commonly represented country, and 60 institutions contributed to 100 articles. Fifteen authors contributed to more than one classic as first or second author. Observational study (n = 55) was the commonest study design across all decades, followed by reviews (n = 12), scale development studies (n = 11), and guidelines (n = 11). Among the pediatric sub-specialties, growth and development articles were highly cited (n = 24), followed by pediatric psychiatry and behavior (n = 21), endocrinology (n = 15), and neonatology (n = 12).

Conclusions

The top-100 cited articles in pediatrics identify the impactful authors, journals, institutes, and countries. Observational study design was predominant—implying that inclusion among citation classics is not related to soundness of study design.

     

The impact of surgical strategies on outcomes for pediatric chronic pancreatitis

Purpose

To review our institutional experience in the surgical treatment of pediatric chronic pancreatitis (CP) and evaluate predictors of long-term pain relief.

Methods

Outcomes of patients ≤21 years surgically treated for CP in a single institution from 1995 to 2014 were evaluated.

Results

Twenty patients underwent surgery for CP at a median of 16.6 years (IQR 10.7–20.6 years). The most common etiology was pancreas divisum (n = 7; 35%). Therapeutic endoscopy was the first-line treatment in 17 cases (85%). Surgical procedures included: longitudinal pancreaticojejunostomy (n = 4, 20%), pancreatectomy (n = 9, 45%), total pancreatectomy with islet autotransplantation (n = 2; 10%), sphincteroplasty (n = 2, 10%) and pseudocyst drainage (n = 3, 15%). At a median follow-up of 5.3 years (IQR 4.2–5.3), twelve patients (63.2%) were pain free and five (26.3%) were insulin dependent. In univariate analysis, previous surgical procedure or >5 endoscopic treatments were associated with a lower likelihood of pain relief (OR 0.06; 95% CI 0.006–0.57; OR 0.07; 95%, CI 0.01–0.89). However, these associations were not present in multivariate analysis.

Conclusion

In children with CP, the step-up practice including a limited trial of endoscopic interventions followed by surgery tailored to anatomical abnormalities and gene mutation status is effective in ensuring long-term pain relief and preserving pancreatic function.

     

Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major

Objectives

To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.

Methods

A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit.

Results

β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among β-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio.

Conclusions

Serum hepcidin is elevated in children with β-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.

     

Catheter Ablation of Pediatric Focal Atrial Tachycardia: Ten-Year Experience Using Modern Mapping Systems

Experience of catheter ablation of pediatric focal atrial tachycardia (FAT) is still limited. There are data which were gathered prior to the introduction of modern 3D mapping and navigation systems into the clinical routine. Accordingly, procedures were associated with significant fluoroscopy and low success rates. The aim of this study was to present clinical and electrophysiological details of catheter ablation of pediatric FAT using modern mapping systems. Since March 2003, 17 consecutive patients <20 years underwent electrophysiological study (EPS) for FAT using the NavX^® system (n = 7), the non-contact mapping system (n = 6) or the LocaLisa^® system (n = 4), respectively. Radiofrequency was the primary energy source; cryoablation was performed in selected patients with a focus close to the AV node. In 16 patients, a total number of 19 atrial foci (right-sided n = 13, left-sided n = 6) could be targeted. In the remaining patient, FAT was not present/inducible during EPS. On an intention-to-treat basis, acute success was achieved in 14/16 patients (87.5 %) with a median number of 11 (1–31) energy applications. Ablation was unsuccessful in two patients due to an epicardial location of a right atrial focus (n = 1) and a focus close to the His bundle (n = 1), respectively. Median procedure time was 210 (84–332) min, and median fluoroscopy time was 13.1 (4.5–22.5) min. In pediatric patients with FAT, 3D mapping and catheter ablation provided improved clinical quality of care. Catheter ablation may be considered early in the course of treatment of this tachyarrhythmia in symptomatic patients.