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1.

Purpose

The aim of this study was to examine parental stress among parents of children with congenital diaphragmatic hernia (CDH).

Methods

Between 2005 and 2009, a total of 51 children with CDH were treated at Astrid Lindgren Children’s Hospital. The survival rate at discharge was 86% and long-term survival rate 80%. One parent each of the long-term survivors (41 children) was included in the present study, and 34 parents (83%) agreed to participate. Participants received the Swedish Parenthood Stress Questionnaire (SPSQ). The questionnaire was supplemented by data from case records.

Results

Parents of children with CDH, who had been supported by ECMO or had a long hospital stay, showed significantly higher overall parental stress. Mothers scored an overall higher parental stress compared with fathers. A prenatal diagnosis of CDH or lower parental educational level resulted in significantly higher parental stress in some of the factors.

Conclusions

Parental stress in parents of children with CDH seems to increase with the severity of the child’s malformation. Mothers tend to score higher parental stress than fathers.
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2.

Purpose

The aim of this long-term follow-up study was to investigate the current self assessed physical health in a CDH birth cohort at a single center.

Methods

Between 1990 and 2009, 195 children born with CDH were treated at Astrid Lindgren Children’s Hospital. The primary survival rate was 85 %, and in 2010, 78 % were still alive. Data from medical records were supplemented by a questionnaire consisting of questions about perceived physical function. Patients were divided into groups according to time for intubation and need for extracorporeal membrane oxygenation.

Results

Children born with CDH reported themselves to be having greater problems with asthma, developmental delay, seizure disorder, poor vision, and scoliosis in comparison with normal Swedish children. They also described a sense of having less strength and becoming breathless more often than healthy friends. Symptoms of gastroesophageal reflux and abdominal pain were also reported.

Conclusions

The majority of the children perceived their physical health as being overall good, but there was an increase of reported symptoms correlating with the severity of the malformation.
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3.

Purpose

The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH).

Methods

Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children’s hospital in Stockholm. In 2012, long-term survivors (n?=?77) were asked to participate in the present study, which resulted in a 46% (n?=?35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed.

Results

The study participants did not differ from the non-participants in terms of prenatal diagnosis, gender, side of lesion, method of surgical repair, time to intubation, need for ECMO support, or way of discharge from the hospital. Children born with CDH considered themselves to have a good HRQoL, as good as healthy Swedish children. There were only a few significant HRQoL differences within the group of children with CDH, although several median scores in ECMO-treated patients were somewhat lower. Correlations between child and parent scores on HRQoL were low.

Conclusions

Health-related quality of life in children born with CDH is good overall, however, a correlation between the severity of the malformation and HRQoL cannot be excluded.
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4.

Objective

To compare the incidence of meconium aspiration syndrome and feed intolerance in infants born through meconium stained amniotic fluid with or without gastric lavage performed at birth.

Setting

Neonatal unit of a teaching hospital in New Delhi, India.

Design

Parallel group unmasked randomized controlled trial.

Participants

700 vigorous infants of gestational age ≥34 weeks from through meconium stained amniotic fluid.

Intervention

Gastric lavage in the labor room with normal saline at 10 mL per kg body weight (n=350) or no gastric lavage (n=350). Meconiumcrit was measured and expressed as ≤30% and >30%.

Outcome Measures

Meconium aspiration syndrome, feed intolerance and procedure-related complications during 72 h of observation.

Results

5 (1.4%) infants in lavage group and 8 (2.2%) in no lavage group developed meconium aspiration syndrome (RR 0.63, 95% CI 0.21, 1.89). Feed intolerance was observed in 37 (10.5%) and 53 infants (15.1%) in lavage and no lavage groups, respectively (RR 0.70, 95% CI 0.47, 1.03). None of the infants in either group developed apnea, bradycardia or cyanosis during the procedure.

Conclusion

Gastric lavage performed in the labor room does not seem to reduce either meconium aspiration syndrome or feed intolerance in vigorous infants born through meconium stained amniotic fluid.
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5.

Objective

To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normalweight and overweight schoolchildren.

Design

Cross-sectional study.

Setting

Thirteen private schools in urban Faridabad, Haryana.

Participants

961 school children aged 5-10 years.

Methods

Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing.

Outcome measures

Prevalence of fatty liver on ultrasound, and NAFLD and its association with biochemical abnormalities and demographic risk factors.

Results

On ultrasound, 215 (22.4%) children had fatty liver; 18.9% in normal-weight and 45.6% in overweight category. Presence and severity of fatty liver disease increased with body mass index (BMI) and age. Among the children with NAFLD, elevated SGOT and SGPT was observed in 21.5% and 10.4% children, respectively. Liver enzyme derangement was significantly higher in overweight children (27% vs 19.4% in normal-weight) and severity of fatty liver (28% vs 20% in mild fatty liver cases). Eleven (8.1%) children with NAFLD had metabolic syndrome. Higher BMI (OR 35.9), severe fatty liver disease (OR 1.7) and female sex (OR 1.9) had strong association with metabolic syndrome.

Conclusions

22.4% of normal-weight and overweight children aged 5-10 years had fatty liver. A high proportion (18.9%) of normal-weight children with fatty liver on ultrasound indicates the silent burden in the population.
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6.

Objective

To evaluate intergenerational change in anthropometric indices of children and their predictors.

Design

Prospective cohort.

Participants

New Delhi Birth Cohort participants (F1), born between 1969 and 1972, were followed-up for anthropometry at birth and 6-monthly intervals until 21 years. Their children (F2) below 10 years were evaluated anthropometrically.

Outcome measure

Intergenerational change (F2-F1) in height, weight and body mass index (BMI) of children in comparison to their parents at corresponding ages.

Results

432 F2-F1 pairs were analyzed in age-groups of 0-5 (26.9%) and 5-10 (73.1%) years. Children were considerably taller (0-5 years 0.99 SD; 5-10 years 1.17 SD) and heavier (0-5 years 0.77 SD; 5-10 years 1.52 SD) while only those aged 5-10 years were broader (had a higher BMI; 1.03 SD), than their parents. These increases for 0-5 and 5-10 years, respectively corresponded to 3.9 and 6.4 cm for height, 1.3 and 5.4 kg for weight and 0.2 and 1.9 kg/m2 for BMI. Lower parents’ anthropometric indices and poor water supply and sanitation facilities; higher age of parents at child birth and of children when measured (for height and weight); and more parental education (for weight and BMI), were associated with greater intergenerational gains in children.

Conclusion

Over one generation in an urban middle-class population, whose general living conditions had improved, underfive children have become considerably taller and heavier, and 5- 10 year old children have additionally become broader, than their parents at corresponding ages. Child populations probably ‘grow up’ before ‘growing out’.
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7.

Purpose

Review current practices and expert opinions on contraindications to extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH) and contraindications to repair of CDH following initiation of ECMO.

Methods

Modified Delphi method was employed to achieve consensus among members of the American Pediatric Surgical Association Critical Care Committee (APSA-CCC).

Results

Overall response rate was 81% including current and former members of the APSA-CCC. An average of 5–15 CDH repairs were reported annually per institution; 26–50% of patients required ECMO. 100% of respondents would not offer ECMO to a patient with a complex or unrepairable cardiac defects or lethal chromosomal abnormality; 94.1% would not in the setting of severe intracranial hemorrhage (ICH). 76.5% and 72.2% of respondents would not offer CDH repair to patients on ECMO with grade III–IV ICH or new diagnosis of lethal genetic or metabolic abnormalities, respectively. There was significant variability in whether or not to repair CDH if unable to wean from ECMO at 4–5 weeks.

Conclusions

Significant variability in practice pattern and opinions exist regarding contraindications to ECMO and when to offer repair of CDH for patients on ECMO. Ongoing work to evaluate outcomes is needed to standardize management and minimize potentially futile interventions.

Level of evidence

V (expert opinion).
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8.

Background

Nephrotic syndrome is a rare but severe feature of IgA nephropathy.

Case characteristics

Nine Japanese children with severe IgA nephropathy with nephrotic syndrome.

Intervention

All received low-dose intravenous methylprednisolone (IVMP) within five weeks after the disease onset. Eight out of nine patients achieved resolution of proteinuria without severe adverse events.

Message

Early low-dose intravenous methylprednisolone may be safe and effective for children with severe IgA nephropathy with nephrotic syndrome.
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9.

Background

The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure.

Case characteristics

Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis.

Outcome

All cases were successfully managed with steroid pulse therapy.

Message

Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.
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10.

Objective

To improve the rates of first hour initiation of breastfeeding in neonates born through cesarean section from 0 to 80% over 3 months through a quality improvement (QI) process.

Design

Quality improvement study.

Setting

Labor Room-Operation Theatre of a tertiary care hospital.

Participants

Stable newborns ≥35 weeks of gestation born by cesarean section under spinal anesthesia.

Procedure

A team of nurses, pediatricians, obstetricians and anesthetists analyzed possible reasons for delayed initiation of breastfeeding by Process flow mapping and Fish bone analysis. Various change ideas were tested through sequential Plan-Do- Study-Act (PDSA) cycles.

Outcome measure

Proportion of eligible babies breast fed within 1 hour of delivery.

Results

The rate of first-hour initiation of breastfeeding increased from 0% to 93% over the study period. The result was sustained even after the last PDSA cycle, without any additional resources.

Conclusions

A QI approach was able to accomplish sustained improvement in first-hour breastfeeding rates in cesarean deliveries.
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11.

Background

Associated anomalies in omphalocele are common, but to which extent these anomalies are diagnosed before or after birth is less well documented.

Aim

To investigate the different types of associated anomalies, long-term survival and the extent whether these are diagnosed pre- or postnatally in children with a prenatal diagnosis of omphalocele at a single institution.

Materials and methods

Retrospective review of all pregnancies with omphalocele managed and/or born at our institution between 2006 and 2016.

Results

A total of 42 cases with prenatally diagnosed omphalocele were identified. Of those 14 (31%) decided to terminate the pregnancy (TOP). Of the remaining 28 that continued, 12 were giant omphaloceles. The overall mortality rate was 18, 25% for giant and 12% for non-giant omphaloceles. 64% had associated anomalies. Only 1/3 of these anomalies is diagnosed prenatally.

Conclusion

The rate of associated malformations that are diagnosed postnatally is high, but the majority was malformations with a minor clinical significance or impact on future health. Beckwith–Wiedemann syndrome was present only in cases of non-giant omphalocele in our cohort.
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12.

Objective

To share experience of over 15 years in hematopoietic stem cell transplantation in children with primary immunodeficiency disorders.

Design

Medical record review.

Setting

A referral center for pediatric hemato-oncological disorders.

Participants

Children (<18 y) diagnosed to have primary immune deficiencies who underwent hematopoietic stem cell transplantation between 2002 and August 2017.

Main outcome measures

Disease-free survival, morbidity and mortality.

Results

85 primary immunodeficiency disorder transplants were performed with engraftment noted in 80 (94%) transplants and an overall survival of 67%. The conditioning regimen was individualized based on the underlying immune defect. Mixed chimerism was noted in 20% children with 56% (9/16) remaining disease-free. Graft versus host disease was noted in 33 (39.2%) children with most seen in children with chronic granulomatous disease. Severe combined immune deficiency transplants were mainly complicated by infections. Immune cytopenias complicated Wiskott Aldrich syndrome and Hemophagocytic lymphohistiocytosis transplants. 29.4% (25/85) children underwent haploidentical transplant in our cohort with a survival of 70% in this group. Infectious complications were the most common cause of death.

Conclusion

Primary immunodeficiency disorders are curable in India when transplanted in centers with experienced and trained pediatric transplant physicians and intensivists.
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13.

Objective

To describe the nutritional outcomes of children with severe acute malnutrition (SAM) in a village-level intervention.

Methods

This observational longitudinal study on 179 children aged <3 years was conducted in seven tribal blocks of Central and Eastern India with SAM managed in a comprehensive day care program.

Results

76% children with SAM showed improvement over a 4-6 months period, with 37% shifting to normal anthrometric status. There was a significant shift in Z scores.

Conclusions

This community-based intervention showed fair results for management of children with SAM at village level.
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14.

Background

Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by arterial or venous thrombosis in children.

Case characteristics

11-year-old child with pneumococcal meningitis also had cerebral sinus vein thrombosis and pulmonary artery segmental thrombosis.

Observation

Pro-thrombotic evaluation showed positive lupus anticoagulant at baseline and after 12 weeks. Investigations for lupus were negative at admission and after one year of follow-up.

Message

Antiphospholipid syndrome is a possibility even in thrombosis occurring in the setting of meningitis.
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15.

Background

Intractable obstructive apneas requiring multiple intubations are rare in newborns.

Case characteristics

We report a pair of twins born at 29 weeks gestation who had severe obstructive apneas due to Paradoxical Vocal Cord Motion (PVCM).

Outcome

The symptoms resolved promptly with ipratropium nebulization. Follow-up at 12 months of age revealed normal development.

Message

PVCM should be considered in the differential diagnosis of intractable obstructive apneas in very low birth weight preterm infants.
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16.

Objective

To assess prevalence of behavioral problems in children with epilepsy.

Methods

This was a cross-sectional study of children with epilepsy, and normal controls enrolled between July 2013 to June 2015. Child Behavior Checklist (CBCL) was used as a tool to assess the behavior based on parents reported observation.

Results

There were 60 children with epilepsy in 2-5 years and 80 in 6-14 years age groups, and 74 and 83 unaffected controls, respectively. Mean CBCL scores for most of the domains in children of both age groups were significantly higher than controls. Clinical range abnormalities were mainly detected in externalizing domain (23.3%) in 2-5 years, and in both internalizing (21.2%) and externalizing (45%) domains in children of 6-14 years. Younger age of onset, frequency of seizures and duration of disease had significant correlation with behavioral problems in both the age groups. Antiepileptic drug polytherapy was significantly associated with internalizing problems in older children.

Conclusion

Age at onset, frequency of seizures and duration of disease were found to be significantly associated with occurrence of behavioral problems.
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17.

Objective

To study the early markers of Metabolic syndrome in a cohort of low birth weight (LBW) children followed up since birth, at the age of 22 years.

Design

Prospective cohort study.

Setting

Tertiary-care hospital

Participants

Neonates weighing less than 2000 g discharged from a neonatal special care unit were followed up prospectively; 153 cases and 77 controls were assessed at 22 years of age.

Methods

Fasting, 30 minute and 120 minute glucose and insulin after a bolus of 75g of glucose was determined. Insulin resistance was calculated. A lipid profile was also done. Anthropometric measurements were taken and abdominal fat was determined by magnetic resonance imaging.

Main outcome

Prevalence of the five components of Metabolic Syndrome as described by the International Diabetic Federation (IDF).

Results

65.1% of the cohort was born small for gestational age. All three components of Metabolic syndrome were present in only three cases and none of the controls. However, two components were present in 25 (16.4%) cases and 5 (6%) controls (P=0.039). Cases in the lowest quartile of birthweight who became big at 22 years had significantly higher fasting insulin (P=0.001), Homeostatic Model Assessment–Insulin Resistance (Homa-IR) (P=0.001) and higher systolic blood pressure. Sum of skinfold thickness at 4 sites correlated significantly with fasting insulin and HOMA-IR, and was a stronger correlate compared to BMI, waist circumference and MRI fat. There was no difference in the biochemical parameters between appropriate for gestational age and small for gestational age infants.

Conclusion

Prevelence of three or more components of Metabolic syndrome was low in LBW children at 22 years, but of two components was high. Those ‘Small at birth and big at 22 years’ had high insulin resistance.
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18.

Background

Obstructive sleep apnea in children is frequently due to tonsil and adenoid hypertrophy. This study aimed to investigate the relationship between ambulatory clinical parameters and sleep respiratory events in obese children.

Methods

We carried out a prospective respiratory sleep study between 2013 and 2015. Nails obstruction, tonsils enlargement and palate position were subjectively measured. Italian attention deficit hyperactivity disorder (ADHD) rating scale for parents was also performed. The polygraph study was performed using a portable ambulatory device.

Results

Forty-four obese children were consecutively recruited into this study. Mild sleep respiratory disturbance was showed in 31.8 % of patients; 18.2% previously had an adeno (tonsillectomy). In 50% of these obese children, both apnea-hypopnea index and oxygen desaturation index showed polygraph abnormal results. ADHD rating scale for parents scores were positive in 9.1% of patients.

Conclusions

We found a high rate of mild sleep respiratory disturbance and ADHD-like symptoms referred by parents. The respiratory disturbance was not totally cured by surgery. Finally, otorhinolaryngology variables were not able to explain mild sleep respiratory disturbance.
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19.

Background

Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia, cardiac dysfunction and pulmonary hypertension. Inhaled nitric oxide (iNO) and milrinone are commonly used pulmonary vasodilators in CDH. We studied the hemodynamic effects of iNO and milrinone in infants with CDH.

Methods

A retrospective chart review was performed of all CDH infants admitted to two regional perinatal centers and infants classified into three groups: No-iNO group; iNO-responders and iNO-nonresponders. Oxygenation and hemodynamic effects of iNO and milrinone were assessed by blood gases and echocardiography.

Results

Fifty-four percent (39/72) of infants with CDH received iNO and 31% of these infants (12/39) had complete oxygenation response to iNO. Oxygenation response to iNO was not associated with a decrease in right ventricular pressures (RVP) or ECMO use. Four infants (33%) in the iNO-responder group and eight infants (30%) in the iNO-nonresponder group received milrinone. Milrinone lowered RVP and improved ejection fraction (EF). Response to iNO was associated with improved oxygenation to milrinone and increased survival following ECMO (67 vs. 20% among nonresponders).

Conclusions

Response to inhaled nitric oxide in combination with milrinone may be associated with improved oxygenation and better survival after ECMO in infants with CDH.
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20.

Objective

To investigate and compare the efficiency of general and disease-specific life quality scales in children with asthma.

Methods

Children with asthma, and their parents completed the Childhood Asthma Control Test (C-ACT), Pediatric Asthma Quality of Life Questionnaire (PAQLQ), Pediatric Quality of Life Inventory (PedsQL), and also underwent spirometry.

Results

82 children (55 males) with a median (IQR) age of 10.1 (8.9-10.5) years were included. C-ACT, PAQLQ and PedsQL child scores were significantly higher in children with controlled asthma.

Conclusions

Quality of life in children, assessed using diseasespecific quality of life measures, is better for children with good asthma control.
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