Effects of <Emphasis Type="Italic">SEMA3</Emphasis> polymorphisms in Hirschsprung disease patients

Purpose

Recently, genetic markers within a locus on 7q21.11 containing the SEMA3A, SEMA3C, and SEMA3D genes were reported to be associated with Hirschsprung disease (HSCR). Here, we investigated three polymorphisms, rs1583147, rs12707682, and rs11766001, at this locus to determine their potential contributions to the susceptibility of Indonesian HSCR patients.

Methods

Three variants were analyzed in 60 non-syndromic HSCR patients and 118 ethnicity-matched controls for association studies by genotyping.

Results

The risk allele frequencies of SEMA3 rs12707682 (allele C) and rs1583147 (allele T) is higher in cases, 53 and 23 %, than in controls, at 42 and 13 %, respectively. However, these frequency differences were not statistically significant with p value of 0.06 and 0.023, respectively. These findings were consistent with transmission disequilibrium test results with p values of 0.041 and 0.11 for rs12707682 and rs1583147, respectively. Furthermore, the frequencies of SEMA3 rs11766001 risk allele in HSCR cases and controls were 1.7 and 0.8 %, respectively.

Conclusions

SEMA3 rs12707682 and rs1583147 variants are not common risk factors for HSCR in Indonesia. The rarity of the SEMA3 rs11766001 polymorphism in Indonesian population might be due to a founder effect.

     

Effects of <Emphasis Type="Italic">CYP3A5</Emphasis> polymorphisms on tacrolimus pharmacokinetics in pediatric kidney transplantation: a systematic review and meta-analysis of observational studies

Background

CYP3A5 genetic polymorphisms have been reported to be strongly associated with the tacrolimus pharmacokinetics in adult kidney transplantation. However, there is no published meta-analysis in the influence of CYP3A5 variants on the requirements of the tacrolimus dose in pediatric renal-transplant recipients (RTRs). We wished to determine the effects of CYP3A5 polymorphisms on tacrolimus pharmacokinetics in pediatric RTRs.

Methods

A literature search was conducted to include relevant articles by searching PubMed, EMBASE and the Cochrane Central Register of Controlled Trials. Pharmacokinetic-associated parameters such as dose administration, as well as concentrations and dose-adjusted concentrations of tacrolimus were extracted and the meta-analysis undertaken.

Results

The meta-analysis involved four studies and one study series involving 268 pediatric RTRs. A significant difference was observed in the mean trough concentration/dose of tacrolimus between recipients carrying CYP3A5* 3/*3 variants (referred to as "non-expressers") and those carrying CYP3A5*1 (referred to as "expressers") [standard mean difference (SMD)=-1.09, 95% confidence interval (CI): -1.92 to -0.25, P=0.011]. Moreover, significance was observed in the mean daily dose of tacrolimus between non-expressers and expressers in pediatric RTRs (SMD=0.44, 95% CI: 0.20 to 0.68, P<0.001).

Conclusion

Our meta-analysis identified a positive correlation between CYP3A5 genotypes and tacrolimus pharmacokinetics in pediatric RTRs.

     

Nasal Continuous Positive Airway Pressure in Bronchiolitis: <Emphasis Type="Italic">A Randomized Controlled Trial</Emphasis>

Objective

To evaluate the efficacy of nasal continuous positive airway pressure (nCPAP) in decreasing respiratory distress in bronchiolitis.

Design

Randomized controlled trial.

Setting

Tertiary-care hospital in New Delhi, India.

Participants

72 infants (age < 1y) hospitalized with a clinical diagnosis of bronchiolitis were randomized to receive standard care, or nCPAP in addition to standard care, in the first hour after admission. 23 parents refused to give consent for participation. 2 infants did not tolerate nCPAP.

Intervention

The outcome was assessed after 60 minutes. If nCPAP was not tolerated or the distress increased, the infant was switched to standard care. Analysis was done on intention-to-treat basis.

Main outcome measures

Change in respiratory rate, Silverman-Anderson score and a Modified Pediatric Society of New Zealand Severity Score.

Results

14 out of 32 in nCPAP group and 5 out of 35 in standard care group had change in respiratory rate ≥10 (P=0.008). The mean (SD) change in respiratory rate[8.0 (5.8) vs 5.1 (4.0), P=0.02] in Silverman-Anderson score [0.78 (0.87) vs 0.39 (0.73), P=0.029] and in Modified Pediatric Society of New Zealand Severity Score [2.5 (3.01) vs. 1.08 (1.3), P=0.012] were significantly different in the nCPAP and standard care groups, respectively.

Conclusion

nCPAP helped reduce respiratory distress significantly compared to standard care.

     

Carbapenem-resistant <Emphasis Type="Italic">Enterobacteriaceae</Emphasis> in pediatric bloodstream infections in rural Southern India

Objective

To measure the frequency of antimicrobial resistance in pediatric blood culture isolates of Escherichia coli and Klebsiella spp. with focus on carbapenem resistance.

Methods

Over a period of three years, pediatric blood culture isolates were tested for antimicrobial susceptibility, including molecular investigations for carbapenem resistance.

Results

Amikacin, carbapenems, colistin and tigecycline had an antimicrobial efficacy of >70% (n=140). 7 of the 15 randomly selected isolates were positive for carbapenemase gene; among them, five were New Delhi Metallo β-lactamase (NDM).

Conclusion

There was a high prevalence of Klebsiella spp. in pediatric bacteremia and dissemination of NDMmediated carbapenem resistance in pediatric wards.

     

Polymorphisms in the promoter region of <Emphasis Type="Italic">IL10</Emphasis> gene are associated with virus etiology of infant bronchiolitis

Background

Bronchiolitis is the most common infection leading to hospitalization in infancy. Interleukin-10 (IL-10) is an anti-inflammatory cytokine, and in our previous study, IL10 gene rs1800896 (??1082A/G) polymorphism was associated with viral etiology of infant bronchiolitis. The objective of this study was to evaluate the associations between IL10 single nucleotide polymorphisms (SNPs) at rs1800890 (??3575A/T), rs1800871 (??819C/T) or rs1800872 (??592C/A) either alone or combined with the SNP at rs1800896 (??1082G/A), and the etiology and severity of infant bronchiolitis.

Methods

Data on four IL10 SNPs were available from 135 full-term infants, hospitalized for bronchiolitis at age less than 6 months, and from 378 to 400 controls. Viral etiology was studied, and oxygen support, feeding support and the length of stay in hospital were recorded during bronchiolitis hospitalization.

Results

Infants with rhinovirus bronchiolitis had the IL10 rs1800890 variant AT or TT genotype less often (18.2%) than controls (63.3%, P?=?0.03), and likewise, had the IL10 rs1800896 variant AG or GG genotype less often (27.3%) than controls (65.5%, P?=?0.009). Twenty-eight infants with bronchiolitis had the variant–variant G_rs1800896T_rs1800890 haplotype, and none of them had rhinovirus infection. The IL10 rs1800871 or rs1800872 genotypes showed no associations with viruses. No association was found between any genotypes and bronchiolitis severity measures.

Conclusion

IL10 rs1800890 and rs1800896 polymorphisms differed between infants with rhinovirus bronchiolitis and controls, but not between infants with respiratory syncytial virus bronchiolitis and controls.

     

Central venous catheter salvage in children with <Emphasis Type="Italic">Staphylococcus aureus</Emphasis> central line-associated bloodstream infection

Background

Prompt central venous catheter (CVC) removal is currently recommended in children with Staphylococcus aureus central line-associated bloodstream infection (CLABSI). Our objective was to examine the outcome of attempted line salvage in children with S. aureus CLABSI and assess predictors of success.

Methods

A single-institution, retrospective cohort study was performed of all children with S. aureus CLABSI between 2012 and 2015. Patients with and without immediate CVC removal (≤?2 days after first positive culture) were compared. The primary outcome was failed CVC salvage (removal after 3+ days).

Results

Seventy-seven children met criteria for S. aureus CLABSI. Immediate CVC removal was performed in 27.3% of patients. Among the 72.7% patients in whom CVC salvage was attempted, 78.6% were successful and 21.4% required delayed CVC removal. Malignancy, short gut syndrome, neutropenia, methicillin-resistant S. aureus, and line type were not associated with salvage failure. No associated morbidity or mortality occurred in patients with a failed salvage attempt. New or recurrent bacteremia occurred in five patients, but three were successfully salvaged a second time.

Conclusions

CVC salvage was feasible in the majority of children with S. aureus CLABSI and was not associated with significant complications or attributable mortality as reported in adults.

     

<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.

     

Vitamin D supplementation for treatment and prevention of pneumonia in under-five children: <Emphasis Type="Italic">A randomized double-blind placebo controlled trial</Emphasis>

Objective

To evaluate the efficacy of single oral mega-dose of Vitamin D3 for treatment and prevention of pneumonia in underfive children.

Design

Randomized, double blind, placebo-controlled trial.

Setting

Tertiary-care hospital.

Participants

324 children (of 980 assessed) between 6 mo-5 y age (median (IQR): 12 (7,19.8) mo) with WHO-defined severe pneumonia. Of these, 126 (39%) were vitamin D deficient (serum 25(OH)D <12 ng/mL).

Intervention

100,000 IU of oral cholecalciferol (n= 162) or placebo (n= 162) in single dose, administered at enrolment.

Outcome variables

Primary: Time to resolution of severe pneumonia and proportion of children having recurrence of pneumonia in next 6 months; Secondary: Change in serum levels of 25(OH)D; immunoglobulins IgA, IgG, IgM, and cathelicidin 2 weeks following supplementation; and time taken for overall resolution of illness.

Results

Median (95% CI) time for resolution of severe pneumonia was 30 (29, 31) h in the vitamin D group as compared to 31 (29,33) h in the placebo group [adjusted hazard ratio (95% CI): 1·39 (1·11, 1·76); P=0·005]. The risk of recurrence of pneumonia in next 6 months was comparable in the two groups [placebo: 36/158 (22·8%); vitamin D: 39/156 (25%); RR (95% CI): 1·13 (0·67,1·90); P=0·69]. Proportion of vitamin D deficient children declined from 38% to 4% in the supplementation group, and from 41% to 33% in the placebo group, two weeks after supplementation. There was no significant effect of vitamin D supplementation on serum levels of cathelicidin, IgA and IgG. The time taken for complete recovery from pneumonia, duration of hospitalization, and fever clearance time were comparable for the two groups. No adverse event was noted related to the intervention.

Conclusion

There is no robust evidence of a definite biological benefit, either for therapy or prevention, to suggest a routine megadose supplement of vitamin D3 for under-five children with severe pneumonia.

     

Hereditary neuropathy with liability to pressure palsy (HNPP): report of a family with a new point mutation in <Emphasis Type="Italic">PMP22</Emphasis> gene

Background

Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder most commonly presenting with acute-onset, non-painful focal sensory and motor mononeuropathy. Approximately 80% of patients carry a 1.5 Mb deletion of chromosome 17p11.2 involving the peripheral myelin protein 22 gene (PMP22), the same duplicated in Charcot-Marie-Tooth 1A patients. In a small proportion of patients the disease is caused by PMP22 point mutations.

Case presentation

We report on a familial case harbouring a new point mutation in the PMP22 gene.The proband is a 4-years-old girl with acute onset of focal numbness and weakness in her right hand. Electroneurography demonstrated transient sensory and motor radial nerves involvement. In her father, reporting chronic symptoms (cramps and exercise-induced myalgia), we uncovered mild atrophy and areflexia on clinical examination and a mixed (predominantly demyelinating) polyneuropathy with sensory-motor involvement on electrophysiological study.Both carried a nucleotidic substitution c.178 + 2 T > C on intron 3 of the PMP22 gene, involving the splicing donor site, not reported on databases but predicted to be likely pathogenic.

Conclusions

We described a previously unreported point mutation in PMP22 gene, which led to the development of a HNPP phenotype in a child and her father. In children evaluated for a sensory and motor transient episode, HNPP disorder due to PMP22 mutations should be suspected. Clinical and electrophysiological studies should be extended to all family members even in the absence of previous episodes suggestive for HNPP.

     

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.

     

Need for Different Cutoff Values for Reading Mantoux Test with <Emphasis>2TU</Emphasis> and <Emphasis>5TU PPD</Emphasis>

Objective

To compare the tuberculin reaction of 2 tuberculin unit (TU) with 5TU purified protein derivative (PPD) (both calibrated against RT 23) in healthy children.

Methods

This was a cross sectional study done in the pediatric outpatient department of a tertiary care teaching hospital. Seventy healthy siblings of the children attending pediatric outpatient department in the age group of 1 to 12 y were enrolled. The exclusion criteria included previously diagnosed tuberculosis patients, malnutrition diagnosed according to the WHO classification, history of drug intake like steroids, recent history of measles, any skin lesions over forearm, history of fever, contact with tuberculosis and previous mantoux testing. The study was conducted wherein each child was subjected to simultaneous testing with 2TU and 5TU by standard technique. The reactions to both the tests was read at 48–72 h. Children with induration ≥10 mm were evaluated for tuberculosis by taking chest x-ray, gastric lavage or sputum smear examination for acid fast bacilli (AFB).

Results

Considering ≥10 mm induration as positive, subjects positive with 5TU were 7 (10%) and 2TU was 1(p value = 0.031); thus, there is no agreement between the two methods (McNemar’s test). Comparing the mean diameter of induration of 2TU and 5TU (p < 0.001, Wilcoxon test), signified no agreement between the two strengths. Bland-Altman plot and kappa statistic showed no agreement between the two strengths.

Conclusions

Cutaneous hypersensitivity to 2TU PPD is not comparable to that of 5TU PPD.

     

Effect of long-term <Emphasis Type="Italic">Helicobacter pylori</Emphasis> infection on growth of children: a cohort study

Background

The effects of Helicobacter pylori (H. pylori) infection on growth are a controversial issue. We investigated the effects of long-term H. pylori infection on height and weight in children.

Methods

A total of 200 children of 7-18 years old suffering from dyspeptic complaints were classified into two groups: H. pylori positive and negative groups, respectively. Whether the infection was impoved was followed up while performing urea breath test, and according to exposure time to the infection, the children were further divided into group 1 (≤1.5 months), group 2 (>1.5-≤6 months) and group 3 (>6 months). Antropometric measurements were obtained and repeated every six months.

Results

Mean growth velocity scores in the H. pylori positive and negative groups were 0.49±3.85 [95% confidence interval (CI): -0.21-1.18] and 1.98±4.42 (95% CI: 1-2.96), respectively. The difference between both groups was statistically significant (P=0.012). Mean growth velocity scores in groups 1, 2 and 3 were 0.96±3.84, 0.16±4.51 and -0.85±3.09, respectively. Mean growth velocity scores of group 3 were significantly lower than those of groups 0 and 1 (P=0.005 and P=0.041). The mean weight scores in group 3 were similar to those in group 2, but the scores in group 3 were significantly lower than those in group 1 (-1.75±1.05, -1.21±1.37 and -0.88±1.49, respectively).

Conclusion

As the duration of exposure is prolonged in children with H. pylori infection, the negative effect of the infection on both height and weight is evident.

     

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.

     

Two novel mutations in the <Emphasis Type="Italic">GAN</Emphasis> gene causing giant axonal neuropathy

Background

Giant axonal neuropathy (GAN) is a rare neurodegenerative disease transmitted in an autosomal recessive mode. This disorder presents motor and sensitive symptoms with an onset in early childhood. Progressive neurodegeneration makes the patients wheelchair dependent by the end of the second decade of life. Affected individuals do not survive beyond the third decade of life. Molecular analysis has identified mutations in the gene GAN in patients with this disorder. This gene produces a protein called gigaxonin which is presumably involved in protein degradation via the ubiquitin–proteasome system. However, the underlying molecular mechanism is not clearly understood yet.

Methods

Here we present the first patient from Mexico with clinical data suggesting GAN. Sequencing of the GAN gene was carried out. Changes in the nucleotide sequence were investigated for their possible impact on protein function and structure using the publicly available prediction tools PolyPhen-2 and PANTHER.

Results

The patient is a compound heterozygous carrying two novel mutations in the GAN gene. The sequence analysis revealed two missense mutations in the Kelch repeats domain. In one allele, a C>T transition was found in exon 9 at the nucleotide position 55393 (g.55393C>T). In the other allele, a transversion G>T in exon 11 at the nucleotide position 67471 (g.67471G>T) was observed. Both of the bioinformatic tools predicted that these amino acid substitutions would have a negative impact on gigaxonin’s function.

Conclusion

This work provides useful information for health professionals and expands the spectrum of disease-causing mutations in the GAN gene and it is the first documented case in Mexican 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.

     

Improved outcomes after technical modifications in tubularized incised plate urethroplasty for mid-shaft and proximal hypospadias

Purpose

To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique.

Methods

We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra.

Results

There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture.

Conclusions

Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.

     

Second Dose of Scorpion Antivenom in Children with Indian Red Scorpion (<Emphasis Type="Italic">Mesobuthus tamulus</Emphasis>) Sting Envenomation

Objective

To study the effect of the second dose of scorpion antivenom in children with nonresolving or worsening scorpion sting envenomation.

Methods

72 children aged ≤12 years with scorpion sting envenomation grade 2 and above were enrolled. 61 received the first dose of three vials of antivenom at admission (group A). Children with persistent/worsening envenomation within 6 hours received the second dose (group B). The time required for resolution of autonomic symptoms, myocardial dysfunction, predictors of the second dose and side effects were studied.

Results

The mean time taken for resolution of autonomic symptoms were comparable in Group A and B (4.1 vs. 5.3 h, P=0.4), and of myocardial dysfunction was shorter in Group A (10.8 vs. 37.6 h, P=0.02). On regression analysis, abnormal echocardiography at admission was found to be a significant predictor of the second dose (OR=27.6, 95% CI, 4.7–162.5; P=<0.001).

Conclusion

Children with severe scorpion sting envenomation with abnormal echocardiography may require a higher dose of scorpion antivenom.

Trial registration

CTRI/2015/03/005652.

     

Image-guided placement of percutaneous de novo low-profile gastrojejunostomy tubes in the pediatric population: a study of feasibility and efficacy

Background

De novo low-profile gastrojejunostomy tubes in pediatric patients offer less external catheter bulk and decreased propensity for dislodgement as children become more mobile. While small cohort studies have evaluated de novo placement of coaxial, adjustable-length, percutaneous gastrojejunostomy (GJ) tubes in children, placement of de novo low-profile GJ tubes in pediatric patients has not been analyzed.

Objective

This study evaluates technical feasibility, safety and clinical efficacy of percutaneous, retrograde placement of de novo low-profile GJ tubes in infants and children.

Materials and methods

Following institutional review board approval, all de novo low-profile GJ tube placements in patients were retrospectively reviewed between May 2014 and May 2017. Technical parameters of fluoroscopy time, tube size, T-fasteners and complications were recorded. Clinical data, including age, indication, weight gain and complications, were analyzed.

Results

Thirty-four de novo low-profile GJ tubes were placed in 34 patients (median age: 9.4 months, range: 2 months-11.8 years; median pre-procedural weight: 7.5 kg, range: 2.9-31.6 kg). Twenty-one 14-Fr and 13 16-Fr GJ tubes were placed with technical success rate of 100%. Average weight gain 3 months’ post procedure was 1.1 kg (range: 0.3-4.8 kg) and average weight percentile for age increase was 9.6% (range: -48.9% to 53.5%). One major complication occurred following balloon inflation within the tract causing pain requiring urgent replacement of the GJ tube. Minor complications occurred in 11 patients (32%): accidental dislodgement (n=9), skin irritation (n=4), tube dysfunction (n=2), leakage (n=2) and tube migration into the esophagus (n=1).

Conclusion

Percutaneous, antegrade, image-guided placement of de novo low-profile GJ tubes is technically feasible, safe and clinically efficacious in appropriately selected pediatric patients.

     

Laparoscopic <Emphasis Type="Italic">versus</Emphasis> open appendectomy for acute appendicitis in children

Objective

To compare the efficacy and safety of laparoscopic appendectomy and open appendectomy for acute appendicitis in children.

Methods

This study was conducted as a retrospective comparison of hospital records for postoperative complications, duration of operation, and postoperative length of stay between children (aged <18y) who underwent laparoscopic (n=190) or open (n=199) appendectomy over a six-year period. Quality of life was evaluated immediately and 1 month postoperatively.

Results

The major complication rate after surgery in laparoscopic group was significantly lower than that of open appendectomy group (13% vs 27%, P<0.05). The mean (SD) postoperative hospital stay was also shorter in laparoscopic group (2.4 (0.6) days vs 3.7 (1.1) days, P<0.05). The postoperative minor complication rate and hospital expenses were not significantly different between the two groups. The duration of surgery was longer in laparoscopic group (P<0.05). Children in laparoscopic group had less postoperative pain and higher quality of life after one month than those in open appendectomy group.

Conclusions

Laparoscopic technique seems to be safer than open appendectomy for acute appendicitis in children.

     

Single nucleotide polymorphisms within Adducin 3 and Adducin 3 antisense RNA1 genes are associated with biliary atresia in Thai infants

Background

A genome-wide association study in East Asians suggested a genetic association between biliary atresia (BA) and a cluster of variants within the Adducin 3 (ADD3) and ADD3 antisense RNA1 (ADD3-AS1) genes. Another study in Thai neonates reported an association between BA and rs17095355. To validate those findings, this study aimed to analyze the BA association with single nucleotide polymorphisms (SNPs) and the additive influence of ADD3 and ADD3-AS1 in Thai neonates.

Methods

DNAs from 56 BA cases and 166 controls were genotyped for rs2501577, rs11194981, rs12268910 (ADD3) and rs17095355 (ADD3-AS1), using TaqMan PCR. Genotype distributions were compared between the groups, and SNP–SNP interactions were analyzed by combination of allelotypes.

Results

The risk allele frequencies of rs2501577, rs11194981, and rs17095355 in the BA group were significantly higher than in the controls. Univariate analysis showed that recessive variants in the three SNPs were associated with BA risk at ORs of 1.81 (95% CI 1.32–2.50), 1.58 (95% CI 1.14–2.20) and 1.92 (95% CI 1.39–2.66), respectively. SNP–SNP interaction analysis showed that the SNP combination of the two genes rs17095355 and rs2501577 provided an additive increase in BA risk.

Conclusion

ADD3 and ADD3-AS1 variants increased susceptibility to BA, suggesting that these genes may play an additive role in the pathogenesis of the disease. In addition, these interactions may give a clue to the overexpression of the ADD3 protein in the liver of BA patients.