Right Heart Function of Fetuses and Infants with Large Ventricular Septal Defect: A Longitudinal Case–Control Study

The objective of this study was to detect the effect of a large ventricular septal defect (VSD) on right ventricular function before and after birth. All consecutive children with large VSD who were born in our hospital between January 2013–February 2016 and followed up throughout early infancy, and who lacked malformations or chromosomal abnormalities, were identified by a retrospective review of the medical records and included in this retrospective longitudinal case–control study (n = 30). Thirty normal control cases with an equivalent gestational age and gender served as controls. Tricuspid annular plane systolic excursion (TAPSE), right ventricle (RV) Tei index, and tricuspid E/E _m were measured in the fetal, neonatal (day 1–28), and infant (day 29–70) periods. In all periods, the VSD and control groups did not differ in TAPSE values, but VSD associated with higher Tei indices and tricuspid E/E _m values (in the fetal period: VSD group RV Tei was 0.48 ± 0.12 and E/E _m was 11.84 ± 1.53, control group RV Tei was 0.42 ± 0.16 and E/E _m was 10.16 ± 1.61; in neonatal period: VSD group RV Tei was 0.41 ± 0.17 and E/E _m was 12.21 ± 1.59, control group RV Tei was 0.30 ± 0.13 and E/E _m was 7.20 ± 1.28; in the infant period: VSD group RV Tei was 0.39 ± 0.09 and E/E _m was 11.89 ± 2.80, control group RV Tei was 0.28 ± 0.12 and E/E _m was 5.26 ± 1.90, all p < 0.05). In the fetal and neonatal periods, TAPSE correlated negatively with Tei index and tricuspid E/E _m in both groups. However, in the infant period, only the control group exhibited correlations between TAPSE and Tei index or tricuspid E/E _m. Tei index correlated positively with tricuspid E/E _m in both groups in all three periods. The VSD group had smaller correlation coefficients than the control group. Large VSD may already start to impair RV diastolic and global function before birth. This impairment continued and increased after birth. These changes did not associate with obvious RV longitudinal systolic function impairment. Large VSD mainly affected RV function by decreasing diastolic function and myocardial performance.     

Early Ventricular Dysfunction After Anthracycline Chemotherapy in Children

The aim of this study was to determine the effect of induction dose of anthracycline chemotherapy on the biventricular function among children with acute hematological malignancies (AHM) using tissue Doppler imaging (TDI) and 2D speckle tracking echocardiography (2D-STE). Thirty pediatric patients with AHM and a mean age of 9.24 ± 4.14 years performed conventional echocardiography, TDI and 2D-STE. After induction chemotherapy, the RV showed mainly a diastolic alteration in its function manifested in significant reduction in the tricuspid TDI-derived E′ and E′/A′ ratio compared with the baseline (20.40 ± 3.81 vs. 17.47 ± 3.87 cm/s, p = 0.001, 1.29 ± 0.27 vs. 1.03 ± 0.37, p < 0.01, respectively), while the TDI-derived RV MPI and isovolumetric relaxation time of RV were significantly increased (0.32 ± 0.06 vs. 0.36 ± 0.08, p < 0.01, 24.73 ± 8.62 vs. 28.47 ± 11.51 ms, p < 0.05, respectively). The LV showed post-chemotherapy mainly an alteration in its longitudinal systolic function in the form of a reduction in MAPSE (13.61 ± 2.00 vs. 11.95 ± 1.75 mm; p < 0.001), TDI-derived systolic velocity of lateral mitral annulus (10.98 ± 2.34 vs. 10.03 ± 1.83 cm/s, p < 0.05), 2D-STE-derived global longitudinal strain (?21.58 ± 2.54 vs. ?19.18 ± 3.59 %, p = 0.001) and 2D-STE-derived global longitudinal strain rate (?1.76 ± 0.22 vs. 1.55 ± 0.29 1/s, p < 0.05), with preservation of LV diastolic function when compared to baseline. TDI and 2D-STE could be used for early detection of anthracycline-induced cardiotoxicity in the pediatric age group. Early after induction chemotherapy, the RV develops mainly diastolic dysfunction, while the LV showed a relative longitudinal systolic impairment.     

Implications of non-accidental trauma on resource utilization and outcomes

Purpose

The purpose was to compare the resource utilization and outcomes between patients with suspected (SUSP) and confirmed (CONF) non-accidental trauma (NAT).

Methods

The institutional trauma registry was reviewed for patients aged 0–18 years presenting from 2007 to 2012 with a diagnosis of suspicion for NAT. Patients with suspected and confirmed NAT were compared.

Results

There were 281 patients included. CONF presented with a higher heart rate (142?±?27 vs 128?±?23 bpm, p?<?0.01), lower systolic blood pressure (100?±?18 vs 105?±?16 mm Hg, p?=?0.03), and higher Injury Severity Score (15?±?11 vs 9?±?5, p?<?0.01). SUSP received fewer consultations (1.6?±?0.7 vs 2.4?±?1.1, 95% CI ? 0.58 to ? 0.09, p?<?0.01) and had a shorter length of stay (1.6?±?1.3 vs 7.8?±?9.8 days, 95% CI ? 4.58 to ? 0.72, p?<?0.01). SUSP were more often discharged home (OR 94.22, 95% CI: 21.26–417.476, p?<?0.01). CONF had a higher mortality rate (8.2 vs 0%, p?<?0.01).

Conclusions

Patients with confirmed NAT present with more severe injuries and require more hospital resources compared to patients in whom NAT is suspected and ruled out.

     

Tricuspid Annular Plane Systolic Excursion Does Not Correlate With Right Ventricular Ejection Fraction in Patients With Hypoplastic Left Heart Syndrome After Fontan Palliation

Tricuspid annular plane systolic excursion (TAPSE) reflects longitudinal myocardial shortening, the main component of right ventricular (RV) contraction in normal hearts. To date, TAPSE has not been extensively studied in patients with hypoplastic left heart syndrome (HLHS) and systemic RVs after Fontan palliation. This retrospective study investigated HLHS patients after Fontan with cardiac magnetic resonance (CMR) performed between 1 January 2010 and 1 August 2012 and transthoracic echocardiogram (TTE) performed within 6 months of CMR. The maximal apical displacement of the lateral tricuspid valve annulus was measured on CMR (using four-chamber cine images) and on TTE (using two-dimensional apical views). To create TTE–TAPSE z-scores, published reference data were used. Intra- and interobserver variability was tested with analysis of variance. Inter-technique agreement of TTE and CMR was tested with Bland–Altman analysis. In this study, 30 CMRs and TTEs from 29 patients were analyzed. The age at CMR was 14.1 ± 7.1 years, performed 11.9 ± 7.8 years after Fontan. For CMR–TAPSE, the intraclass correlation coefficients for inter- and intraobserver variability were 0.89 and 0.91, respectively. The TAPSE measurements were 0.57 ± 0.2 cm on CMR and 0.70 ± 0.2 cm on TTE (TTE–TAPSE z score, ?8.7 ± 1.0). The mean difference in TAPSE between CMR and TTE was ?0.13 cm [95 % confidence interval (CI) ?0.21 to ?0.05], with 95 % limits of agreement (?0.55 to 0.29 cm). The study showed no association between CMR–TAPSE and RVEF (R = 0.08; p = 0.67). In patients with HLHS after Fontan, TAPSE is reproducible on CMR and TTE, with good agreement between the two imaging methods. Diminished TAPSE suggests impaired longitudinal shortening in the systemic RV. However, TAPSE is not a surrogate for RVEF in this study population.     

Clinical and pathological features of patients with biliary atresia who survived for more than 5 years with native liver

Purpose

The objective of this study was to determine the predictive index for prognosis in patients with biliary atresia (BA).

Methods

A total of 71 patients were divided into two groups. Group A included 39 postoperative BA patients who survived for more than 5 years with normal liver function and did not present cirrhosis, and group B included 32 patients who died from liver failure within 1 year after surgery. The clinical data of the two study groups were compared, and liver pathology was evaluated using a scoring system.

Results

The average age and weight were similar in the two groups (64.1 ± 16.8 days vs. 60.7 ± 19.3 days, p > 0.05; 4.9 ± 0.9 kg vs. 4.7 ± 0.8 kg, p > 0.05). There were no significant intergroup differences in preoperative total bilirubin (TB), direct bilirubin (DB), alanine transaminase, aspartate transaminase, and international normalized ratio. The preoperative levels of gamma-glutamyl transpeptidase (γ-GT) and albumin in group A were significantly higher than those in group B (γ-GT: 956.8 ± 503.8 IU/L vs. 620.2 ± 437.1 IU/L, p = 0.00; ALB: 40.8 ± 2.5 g/L vs. 36.8 ± 3.6 g/L, p = 0.04), whereas alkaline phosphatase was significantly lower in group A compared to group B (512.2 ± 224.6 IU/L vs. 631.7 ± 254.7 IU/L, p = 0.02). The postoperative TB and DB after 2 weeks of the Kasai procedure decreased significantly more in group A than in group B (TB: 53.9 vs. 21.4%, p = 0.00; DB: 51.0 vs. 22.7%, p = 0.00), whereas γ-GT increased significantly less in group A than in group B (48.3 vs. 142.1%, p = 0.00). Cystic structures were observed at the porta hepatis on ultrasound in more patients from group A (28.2 vs. 3.2%, p < 0.00). There was no significant difference in the total pathological score between the two groups (p = 0.38) whereas the score of bile plugs was significantly higher in group A (0.95 vs. 0.38, p = 0.03).

Conclusion

The cystic structures observed at the porta hepatis on ultrasound preoperatively and the rapid decrease in TB and DB within 2 weeks postoperatively predict good long-term prognosis, whereas a significant increase in γ-GT with a lower preoperative level predicts poor long-term prognosis. The development of bile plugs may be an indicator of favorable prognosis.

     

The Impact of a Designated Cardiology Team Involving Telemedicine Home Monitoring on the Care of Children with Single-Ventricle Physiology After Norwood Palliation

We evaluated the effect of an interdisciplinary single-ventricle task force (SVTF) that utilizes a family-driven, telemedicine home monitoring program on clinical outcomes of stage II admissions and its acceptance by parents and cardiologists. Study population was divided into two cohorts, one with Norwood surgery dates before the SVTF (pre-SVTF) and one interventional (post-SVTF). Post-SVTF data also included surveys of parents and cardiologists on the efficacy of the SVTF. Comparative and multivariate statistical testing was performed. Compared to the pre-SVTF group, the post-SVTF group had lower complications after stage II (18.4 vs. 34.1 %, p = 0.02), higher weight-for-age z scores at stage II (?1.5 ± 0.97 vs. ?1.58 ± 1.34, p = 0.02) and were less likely to have a stage II weight-for-age z score below ?2 (26.5 vs. 31.7 %, p = 0.03). A multivariate regression analysis showed providing a written red-flag action plan to parents at discharge was independently associated with higher weight at stage II (β = 0.42, p = 0.04) and higher weight-for-age z score (β = 0.48, p = 0.02). Parents’ satisfaction with SVTF (α = 0.97) was 4.34 ± 0.62; (95 % CI 4.01–4.67) and cardiologists’ acceptance (α = 0.93) was 4.1 ± 0.7 (95 % CI 3.79–4.42). Development of SVTF was associated with a reduction in complications post-stage II and improved weight status at stage II. A written red-flag action plan provided to parents at the time of Norwood discharge was associated with higher weight status at stage II. Parents and cardiologists expressed satisfaction with the utility of SVTF and encouraged expansion to cover all children with congenital heart disease.     

Role of nasogastric tube in children undergoing elective distal bowel surgery

Background

Nasogastric tubes are being routinely used in children and adults undergoing elective abdominal surgery without much scientific evidence supporting their true usefulness. The aim of our study was to assess the role of nasogastric tube in children undergoing elective distal bowel surgery.

Materials and methods

All pediatric patients undergoing elective distal bowel surgery were enrolled and randomized into two groups: those with nasogastric tube (NG group) or without nasogastric tube (NNG group). Outcome parameters such as resumption of bowel function, enteral feed tolerance, postoperative complications, hospital stay and patient with their parent satisfaction were compared between the groups.

Results

A total of 60 patients were included with equal distribution in the NG and NNG groups. Patient variables were comparable in both the groups. Patients in NNG group progressed to full oral feeds significantly earlier (57 ± 18 vs. 106.07 ± 18.35 h, p < 0.001) and had shorter duration of hospital stay (91.93 ± 26.03 vs. 114.67 ± 18.83 h, p < 0.001) as compared to the NG group. Significant number of patients with nasogastric tube reported sore throat (9 vs. 1 p = 0.03) and nausea (5 vs. 0 p = 0.010). There was no significant difference in return of bowel function (39.43 h ± 15.92 vs. 43.60 h ± 17.77, p = 0.171), hiccups, sleep disturbance, complications and nasogastric tube reinsertion rate between the two groups.

Conclusion

Routine use of nasogastric tube after elective distal bowel surgery in children is not necessary.

     

Impact of Interatrial Communication on Left Ventricle Performance in Patients with Significant Post-tricuspid Shunt

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E′- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.     

Myocardial Layers Specific Strain Analysis for the Acute Phase of Infant Kawasaki Disease

Recently, the prevalence of infant Kawasaki disease (KD) has increased. However, the myocardial functional analysis of infant KD can be difficult and rarely reported. The purpose of this study was to investigate layer specific myocardial strain analysis for better assessment of the acute period in infant KD. The study retrospectively reviewed the echocardiographic data of 25 infant patients with KD at the acute phase. With advanced imaging, pulsed tissue Doppler velocity data, myocardial strain with three layers specific analysis was performed. Then the data were compared with 25 age-matched healthy control infants. The measures of longitudinal strain and radial strain were decreased in infant KD compared to healthy controls. The circumferential strain was significantly decreased in infant KD at all three myocardial layers, especially in the endocardial layer (KD: ?20.5 ± 6.4 % vs. control: ?25.6 ± 7.6 %, endocardium, p = 0.00001; ?14.6 ± 4.4 % vs. ?18.1 ± 4.0 %; middle myocardium, p = 0.01; ?9.7 ± 3.3 % vs. ?11.4 ± 3.8 %; epicardium, p = 0.04). The acute phase of infant KD demonstrated decreased myocardial strain measurement. Circumferential strain was the lowest in the endocardial layer. Further continuous long-term follow up for myocardial assessment should be recommended even after recovery with appropriate treatment.     

Mesenchymal expression of the <Emphasis Type="Italic">FRAS1/FREM2</Emphasis> gene unit is decreased in the developing fetal diaphragm of nitrofen-induced congenital diaphragmatic hernia

Purpose

Developmental mutations that inhibit normal formation of extracellular matrix (ECM) in fetal diaphragms have been identified in congenital diaphragmatic hernia (CDH). FRAS1 and FRAS1-related extracellular matrix 2 (FREM2), which encode important ECM proteins, are secreted by mesenchymal cells during diaphragmatic development. The FRAS1/FREM2 gene unit has been shown to form a ternary complex with FREM1, which plays a crucial role during formation of human and rodent diaphragms. Furthermore, it has been demonstrated that the diaphragmatic expression of FREM1 is decreased in the nitrofen-induced CDH model. We hypothesized that FRAS1 and FREM2 expression is decreased in the developing diaphragms of fetal rats with nitrofen-induced CDH.

Methods

Pregnant rats were exposed to either nitrofen or vehicle on gestational day 9 (D9), and fetuses were harvested on D13, D15 and D18. Microdissected diaphragms were divided into nitrofen-exposed/CDH and control samples (n = 12 per time-point and experimental group, respectively). Diaphragmatic gene expression levels of FRAS1 and FREM2 were analyzed by qRT-PCR. Immunofluorescence double staining for FRAS1 and FREM2 was combined with the mesenchymal marker GATA4 in order to evaluate protein expression and localization in pleuroperitoneal folds (PPFs) and fetal diaphragmatic tissue.

Results

Relative mRNA expression of FRAS1 and FREM2 were significantly reduced in PPFs of nitrofen-exposed fetuses on D13 (1.76 ± 0.86 vs. 3.09 ± 1.15; p < 0.05 and 0.47 ± 0.26 vs. 0.82 ± 0.36; p < 0.05), developing diaphragms of nitrofen-exposed fetuses on D15 (1.45 ± 0.80 vs. 2.63 ± 0.84; p < 0.05 and 0.41 ± 0.16 vs. 1.02 ± 0.49; p < 0.05) and fully muscularized diaphragms of CDH fetuses on D18 (1.35 ± 0.75 vs. 2.32 ± 0.92; p < 0.05 and 0.37 ± 0.24 vs. 0.70 ± 0.32; p < 0.05) compared to controls. Confocal laser scanning microscopy revealed markedly diminished FRAS1 and FREM2 immunofluorescence in diaphragmatic mesenchyme, which was associated with reduced proliferation of mesenchymal cells in nitrofen-exposed PPFs and fetal CDH diaphragms on D13, D15 and D18 compared to controls.

Conclusion

Decreased mesenchymal expression of FRAS1 and FREM2 in the nitrofen-induced CDH model may cause failure of the FRAS1/FREM2 gene unit to activate FREM1 signaling, disturbing the formation of diaphragmatic ECM and thus contributing to the development of diaphragmatic defects in CDH.

     

Echocardiographic Assessment of Right Atrial Pressure in a Pediatric and Young Adult Population

Right atrial pressure (RAP) reflects right-sided cardiac hemodynamics and is useful in management of patients with cardiac and systemic disease. Studies in older adults demonstrated that inferior vena cava (IVC) diameter, IVC collapsibility index, hepatic vein systolic filling fraction (SFF), and right atrial volume (RAV) correlated with mean RAP at catheterization. This study aimed to assess the utility of echocardiographic parameters for assessment of RAP in children and young adults. Patients with pulmonary hypertension or heart transplantation undergoing right heart catheterization were recruited for this prospective observational pilot study. Transthoracic echocardiographic assessment of RAP was performed simultaneously with catheterization. For each parameter, three consecutive cardiac cycles were recorded. Long- and short-axis images of the IVC were obtained. RAV was assessed by area–length and biplane methods. IVC diameters and RAV were indexed to body surface area (BSA)^0.5 and (BSA)^1.4, respectively. Relationships between echocardiographic parameters and mean RAP were correlated using “Pearson’s r.” Fifty subjects aged 0.3–23 years (median 13, mean 12.3 ± 7 years) were enrolled. Mean RAP correlated modestly with RAV (r = 0.51, p < 0.001). Long-axis IVC_max (r = 0.30, p < 0.05) and tricuspid E wave velocity (r = 0.36, p < 0.01) also correlated with mean RAP. RV free wall tissue Doppler velocities, IVC collapsibility index, and hepatic vein SFF had no relation to mean RAP. In a pediatric and young adult population with pulmonary hypertension or heart transplantation, echocardiographic assessment of RAV and long-axis IVC_max provided a reasonable estimate of mean RAP. IVC collapsibility index and hepatic vein SFF demonstrated no association with mean RAP.     

Correlations Between Echocardiographic Systolic and Diastolic Function with Cardiac Catheterization in Biventricular Congenital Heart Patients

Newer echocardiographic techniques may allow for more accurate assessment of left ventricular (LV) function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the pediatric congenital heart population. Purpose of this study was to evaluate which echocardiographic measurements correlated best with LV systolic and diastolic catheterization parameters. Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterization were included. Images were obtained in the four-chamber view. LV systolic echocardiographic data included ejection fraction, displacement, tissue Doppler imaging (TDI) s′ wave, global longitudinal strain, and strain rate (SR) s′ wave. Diastolic echocardiographic data included mitral E and A waves, TDI e′ and a′ waves, and SRe′ and SRa′ waves. E/TDI e′, TDI e′/TDI a′, E/SRe′, and SRe′/SRa′ ratios were also calculated. Catheterization dP/dt was used as a marker for systolic function, and LV end-diastolic pressure (EDP) was used as a marker for diastolic function. Correlations of the echocardiographic and catheterization values were performed using Pearson correlation. Twenty-nine patients were included (14 females, 15 males). Median age at catheterization was 3.4 years (0.04–17.4 years). dP/dt was 1258 ± 353 mmHg/s, and LVEDP was 10.8 ± 2.4 mmHg. There were no significant correlations between catheterization dP/dt and systolic echocardiographic parameters. LVEDP correlated significantly with SRe′ (r = ?0.4, p = 0.03), SRa′ (r = ?0.4, p = 0.03), and E/SRe′ (r = 0.5, p = 0.004). In pediatric congenital heart patients, catheterization dP/dt did not correlate with echocardiographic measurements of LV systolic function. Further studies are needed to determine which echocardiographic parameter best describes LV systolic function in this population. Strain rate analysis significantly correlated with LVEDP. Strain rate analysis should be considered as an alternative method to estimate LVEDP in this patient population.     

Results of a Feeding Protocol in Patients Undergoing the Hybrid Procedure

Neonates with single-ventricle physiology are at increased risk of developing gastrointestinal morbidities. Feeding protocols in this patient population have been shown to decrease feeding complications after the Norwood procedure, but no data exist to determine the effectiveness of a feeding protocol in patients undergoing the hybrid procedure. Goal of this study was to examine the impact of a standardized feeding protocol on the incidence of overall postoperative gastrointestinal morbidity after the hybrid procedure. Retrospective chart review was performed on neonates undergoing the hybrid procedure. Neonates were divided into two groups, pre-feeding protocol (pre-FP), which encompassed the years 2002–2008, and post-feeding protocol (post-FP), which encompassed the years 2011–2014. Preoperative, operative, and postoperative data were collected. T test or Fisher’s exact test was used for analysis. p < 0.05 was considered significant. Seventy-three neonates were in the pre-FP and 52 neonates were in the post-FP. There were no significant differences between the pre-FP and the post-FP in cardiac diagnosis (62 HLHS, 11 other vs. 39 HLHS, 13 other, respectively). Pre-FP underwent hybrid procedure later than the post-FP (9.1 ± 5.8 vs. 5.7 ± 3.4 days, respectively, p < 0.01) and achieved full enteral feeds earlier than the post-FP (3.2 + 2.9 vs. 7.8 + 3.9 days, respectively, p < 0.01). The incidence of necrotizing enterocolitis was higher in the pre-FP versus post-FP [11.0 % (8/65) vs. 5.8 % (3/49), respectively, p = 0.36]. Though not significant, the incidence of necrotizing enterocolitis decreased by almost 50 % after initiating a feeding protocol in patients undergoing the hybrid procedure. This is consistent with previous studies showing beneficial results of a feeding protocol in this complex patient population.     

<Emphasis Type="Italic">Bordetella Pertussis</Emphasis> Infection in Hospitalized Infants with Acute Bronchiolitis

Objective

To assess the frequency of B. pertussis infection among young infants hospitalized with acute bronchiolitis and to determine whether B. pertussis infection affects the clinical course of acute bronchiolitis.

Methods

A total of 172 infants <6 months of age hospitalized with acute bronchiolitis were tested for B. pertussis and respiratory viruses with real-time PCR. Cases were divided into 2 groups according to B. pertussis positive or negative. Clinical parameters, clinical severity scores and laboratory characteristics of the pertussis-positive and pertussis-negative cases were compared.

Results

Bordetella pertussis infection was detected in 44 (25.6%) of the 172 infants hospitalized for acute bronchiolitis, and as co-infection with respiratory viral agents in 27 (61.4%) infants. Of the 44 pertussis-positive infants, only 17 (38.6%) experienced a paroxysmal cough, 13 (29.5%) had whooping and 15 (34.1%) had post-tussive vomiting. There was no significant difference between pertussis-positive and pertussis-negative infants according to Wang clinical score at admission (4.9 ± 1.5 vs. 5.2 ± 2.5; p = 0.689). The overall disease severity score was also similar between the two groups (6.5 ± 1.4 vs. 6.9 ± 1.6; p = 0.095).

Conclusions

Bordetella pertussis infection is common in young infants hospitalized for acute bronchiolitis, mostly as co-infection with respiratory viruses. The clinical features of pertussis in the infants are not characteristic. Viral bronchiolitis and pertussis cases could not be differentiated by clinical findings. Co-infection with pertussis did not affect the clinical outcome in infants hospitalized with acute bronchiolitis.

     

Single-incision laparoscopic surgery (SILS) for children with Crohn’s disease

Purpose

Single-incision laparoscopic surgery (SILS) has been described in adults with Crohn’s disease, but its use in pediatric Crohn’s patients has been limited. The purpose of this study was to review our experience with SILS in pediatric patients with Crohn’s disease.

Methods

A retrospective review was performed for patients diagnosed with Crohn’s disease who underwent small bowel resection or ileocecectomy at a freestanding children’s hospital from 2006 to 2014. Data collected included demographic data, interval from diagnosis to surgery, operative time, length of stay, and postoperative outcomes.

Results

Analysis identified 19 patients who underwent open surgery (OS) and 41 patients who underwent SILS. One patient (2.4 %) within the SILS group required conversion to OS. Demographic characteristics were similar between the 2 cohorts. The most common indication for surgery was stricture/obstruction (SILS 70.7 % vs. OS 68.4 %, p = 0.86), and ileocecectomy was the most common primary procedure performed (SILS 90.2 % vs. OS 100 % OS). Operative times were longer for SILS (135 ± 50 vs. 105 ± 37 min, p = 0.02). However, when the last 20 SILS cases were compared to all OS cases, the difference was no longer statistically significant (SILS 123.3 ± 34.2 vs. OS 105 ± 36.5, p = 0.12). No difference was noted in postoperative length of stay (SILS 6.5 ± 2.2 days vs. OS 7.4 ± 2.2 days, p = 0.16) or overall complication rate (SILS 24.4 % vs. OS 26.3 %, p = 0.16).

Conclusion

SILS ileocecectomy is feasible in pediatric patients with Crohn’s disease, achieving outcomes similar to OS. As experience increased, operative times also became comparable.

     

Fundoplication with gastrostomy vs gastrostomy alone: a systematic review and meta-analysis of outcomes and complications

Purpose

We compare the outcomes of fundoplication with gastrostomy vs gastrostomy alone and review the need for subsequent fundoplication after the initial gastrostomy alone.

Methods

We searched studies published from 1969 to 2016 for comparative outcomes of concomitant fundoplication with gastrostomy (FGT) vs gastrostomy insertion alone (GT) in children. Gastrostomy methods included open, laparoscopic, and endoscopic procedures. Primary aims were minor and major complications. Secondary aims included post-operative reflux-related complications, fundoplication specific complications, and need for subsequent fundoplication after GT.

Results

We reviewed 447 studies; 6 observational studies were included for meta-analysis, encompassing 2730 children undergoing GT (n = 1745) or FGT (n = 985). FGT was associated with more minor complications [19.9 vs 11.4%, OR 2.02, 95% confidence interval (CI) 1.43–2.87, p ≤ 0.0001, I ² = 0%], minor complications requiring revision (6.8 vs 3.0%, OR 2.27, 95% CI 1.28–4.05, p = 0.005, I ² = 0%), and more overall complications (21.3 vs 12.0%, OR 1.99, 95% CI 1.43–2.78, p < 0.0001, I ² = 0%). Incidence of major complications (1.8 vs 2.0%, OR 1.39, 95% CI 0.62–3.11, p = 0.42, I ² = 5%) and reflux-related complications (8.8 vs 10.3%, OR 0.75, 95% CI 0.35–1.68, p = 0.46, I ² = 0%) in both groups was similar. Incidence of subsequent fundoplication in GT patients was 8.6% (mean).

Conclusions

Gastrostomy alone is associated with fewer minor and overall complications. Concomitant fundoplication does not significantly reduce reflux-related complications. As few patients require fundoplication after gastrostomy, current evidence does not support concomitant fundoplication.

     

Decreased Desmin expression in the developing diaphragm of the nitrofen-induced congenital diaphragmatic hernia rat model

Purpose

Congenital diaphragmatic hernia (CDH) is presumed to originate from defects in the primordial diaphragmatic mesenchyme, mainly comprising of muscle connective tissue (MCT). Thus, normal diaphragmatic morphogenesis depends on the structural integrity of the underlying MCT. Developmental mutations that inhibit normal formation of diaphragmatic MCT have been shown to result in CDH. Desmin (DES) is a major filament protein in the MCT, which is essential for the tensile strength of the developing diaphragm muscle. DES ^?/? knockout mice exhibit significant reductions in stiffness and elasticity of the developing diaphragmatic muscle tissue. Furthermore, sequence changes in the DES gene have recently been identified in human cases of CDH, suggesting that alterations in DES expression may lead to diaphragmatic defects. This study was designed to investigate the hypothesis that diaphragmatic DES expression is decreased in fetal rats with nitrofen-induced CDH.

Methods

Time-mated Sprague–Dawley rats were exposed to either nitrofen or vehicle on gestational day 9 (D9). Fetuses were harvested on selected time-points D13, D15 and D18, and dissected diaphragms (n = 72) were divided into control and nitrofen-exposed specimens (n = 12 per time-point and experimental group, respectively). Laser-capture microdissection was used to obtain diaphragmatic tissue elements. Diaphragmatic gene expression of DES was analyzed by quantitative real-time polymerase chain reaction. Immunofluorescence double staining for DES was combined with the mesenchymal marker GATA4 to evaluate protein expression and localization in developing fetal diaphragms.

Results

Relative mRNA expression levels of DES were significantly decreased in pleuroperitoneal folds on D13 (1.49 ± 1.79 vs. 3.47 ± 2.32; p < 0.05), developing diaphragms on D15 (1.49 ± 1.41 vs. 3.94 ± 3.06; p < 0.05) and fully muscularized diaphragms on D18 (2.45 ± 1.47 vs. 5.12 ± 3.37; p < 0.05) of nitrofen-exposed fetuses compared to controls. Confocal laser scanning microscopy demonstrated markedly diminished immunofluorescence of DES mainly in diaphragmatic MCT, which was associated with a reduction of proliferating mesenchymal cells in nitrofen-exposed fetuses on D13, D15 and D18 compared to controls.

Conclusion

Decreased expression of DES in the fetal diaphragm may disturb the basic integrity of myofibrils and the cytoskeletal network during myogenesis, causing malformed MCT and leading to diaphragmatic defects in the nitrofen-induced CDH model.

     

A comparison of the characteristics and precision of needle driving for right-handed pediatric surgeons between right and left driving using a model of infant laparoscopic diaphragmatic hernia repair

Purpose

We compared the characteristics and precision of right and left needle driving for right-handed pediatric surgeons using a laparoscopic diaphragmatic repair model.

Methods

Eighteen right-handed pediatric surgeons performed three needle driving maneuvers using both hands. We evaluated the required time and conducted an image analysis. The total path length, velocity, and acceleration of the needle driving were also evaluated.

Results

Obtained results show the findings for the required time (s, Rt 310.78 ± 148.93 vs. Lt 308.61 ± 122.53, p = 0.93), sum of needle driving balances (mm, Rt 5.23 ± 2.44 vs. Lt 5.05 ± 3.17, p = 0.83), the gap of the needle driving interval (Rt 1.2 ± 0.93 vs. Lt 2.17 ± 1.67, p = 0.04), total path length (mm, Rt 594.03 ± 205.29 vs. Lt 1641.07 ± 670.68, p < 0.01), and average velocity (mm/s, Rt 1.92 ± 0.54 vs. Lt 5.3 ± 1.39, p < 0.01).

Conclusion

For right-handed pediatric surgeons, left needle driving showed almost same quality of right needle driving as regarding the precision. But left needle driving also showed too fast but not economical movement unfortunately, implying rough and risky forceps manipulation. Non-dominant hand training is necessary to avoid organ injury.

     

Assessment of cardiac function in infants with transposition of the great arteries after surgery: comparison of two methods

Background

Assessment of cardiac function is crucial in pediatric patients undergoing cardiovascular surgery, monitoring cardiac output and changing hemodynamic conditions during surgery accordingly is important to improve post-surgical outcome. We aimed to measure cardiac index (CI) and maximal rate of the increase of left ventricular pressure dp/dt(max) with the pressure recording analytic method (PRAM, MostCare^®) and compared it with transthoracic echocardiographic cardiac index estimation in infants with transposition of the great arteries (TGA) undergoing surgical correction.

Methods

We enrolled 74 infants with TGA consecutively into this study. CI and dp/dt(max) were measured with PRAM and echocardiography at 0, 4, 8, 12, 24 and 48 h postoperatively. Blood brain natriuretic peptide (BNP) and blood lactate (Lac) were measured at baseline and after operation.

Results

The median age at surgery was 13 days (range 1–25 days) with an average weight of 3.24 kg (range 2.31–4.17 kg). CI estimated by PRAM was 1.11 ± 0.12 L/min/m² (range 0.69–1.36) and by Doppler echocardiography was 1.13 ± 0.13 L/min/m² (range 0.76–1.40). dp/dt(max) estimated by PRAM was 1.31 ± 0.03 mmHg/s (range 1.23–1.43) and by Doppler echocardiography was 1.31 ± 0.04 L/min/m² (range 1.25–1.47). CI (r = 0.817, P < 0.001) and dp/dt(max) (r?=?0.794, P?<?0.001) measured by two methods were highly correlated with a linear relation. Blood BNP and lactate increased to the highest level at 8–12 h post-operatively.

Conclusions

In the early post-operative period, PRAM provides reliable estimates of cardiac index and dp/dt(max) value compared with echocardiographic measurements. PRAM through mostcare^® is a reliable continuous monitoring method for peri-operative management in children with congenital heart disease.

     

Is maintenance of the ileocecal valve important to the intestinal adaptation mechanisms in a weaning rat model of short bowel?

Purpose

To evaluate the role of maintenance of the ileocecal valve (ICV) in intestinal adaptation mechanisms, in a weaning rat experimental model of short bowel.

Methods

Forty animals were operated on to produce short bowel syndrome. They were divided into five groups: maintenance (MV) or resection of ICV (RV), kill after 4 days (MV4 and RV4) or 21 days (MV21 and RV21), and a control group (21-day-old rats). Body weights, small bowel and colon lengths and diameters, villus heights, crypt depths, lamina propria and muscle layer thickness, as well as the apoptosis index of villi and crypts and expression of pro- and anti-apoptotic genes, were studied.

Results

Preservation of the ICV promoted increased weight gain (p?=?0.0001) and intestinal villus height after 21 days; crypt depth was higher in comparison to controls. It was verified a higher expression of Ki-67 in bowel villi and crypts (p?=?0.018 and p?=?0.015, respectively) in RV4 group and a higher expression in bowel villi of MV4 group animals (p?=?0.03). The maintenance of ICV promoted late increased expression of the anti-apoptotic gene Bcl-XL in the colon (p?=?0.043, p?=?0.002, p?=?0.01).

Conclusion

The maintenance of the ICV led to positive changes in this model.