Retained capsule endoscope

Background

Capsule endoscopy was invented to visualize the entire small intestine in a non-invasive manner in adults.

Case characteristics

1 y, 9 mo-old boy presented with generalized edema for last 3 months. His routine investigations, including the upper gastrointestinal endoscopy, colonoscopy, and contrast enhanced computed tomography scan (CECT) were normal. In view of clinical suspicion of protein losing enteropathy, we planned capsule endoscopy.

Observation

The capsule was not passed even after 3 weeks. Laparoscopy revealed impacted capsule in a dilated intestinal loop proximal to an ileal stricuture.

Message

Capsule endoscopy should be used judiciously in children.

     

Emergency surgery for refractory status epilepticus

Background

Management of refractory status epilepticus in children is extremely challenging.

Case characteristics

Two children with medically refractory status epilepticus, both of whom had lesional pathology on MRI and concordant data on EEG and PET scan.

Intervention

Emergency hemispherotomy performed in both patients. A complete, sustained seizure freedom obtained postoperatively.

Message

Emergency surgery is a treatment option in selected cases of drug refractory status epilepticus with lesional pathology and concordant data.

     

Association of allergic rhinitis and sinusitis with childhood asthma

Objective

To study the point prevalence of allergic rhinitis and sinusitis in childhood asthma and to examine the relationship among them.

Methods

In 250 children (age <13 y) with mild-to-moderte asthma, allergic rhinitis was diagnosed by clinical plus nasal eosinophilia criteria, and sinusitis was diagnosed clinically plus confirmation by computerized tomography scan.

Results

The point prevalence of allergic rhinitis was 13.6%, and of sinusitis was 2%. On multivariate analysis, allergic rhinitis, sinusitis, and family history were significantly associated with asthma severity.

Conclusions

Allergic rhinitis is common in childhood asthama, but sinusitis is rare.

     

Steroid pulse therapy for Kawasaki disease complicated with myocarditis

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.

     

Prevalence of nonalcoholic fatty liver disease in normal-weight and overweight preadolescent children in Haryana,India

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.

     

Effect of recorded maternal voice on child’s cooperation during cardiac catheterization – A randomized controlled trial

Objective

To assess the effect of recorded maternal voice on child’s cooperation during cardiac catheterization.

Design

Randomized placebo controlled trial.

Setting

Cardiac catheterization laboratory at a tertiary care hospital.

Participants

90 children with congenital heart disease scheduled for cardiac catheterization between July 2014 and Dec 2014 randomized to maternal voice group and control group.

Intervention

During cardiac catheterization, children in maternal voice group listened to a 3-min audio-recording of their mother’s voice, played in loop, using head-phones. Children in the other group wore headphones without auditory stimuli.

Main outcome measures

Child’s cooperation during cardiac catheterization as assessed by Child Emotional Manifestation Scale.

Results

Children in the maternal voice group showed lower mean (SD) distress scores [13.2 (4.6) vs. 16 (5.6), P=0.01]. The requirement of sedative agents during the procedure was not different (P=0.09).

Conclusion

Allowing children to listen to recorded voice of their mother is an effective strategy to improve cooperation during cardiac catheterization.

     

Hematopoietic Stem Cell Transplantation for Primary Immunodeficiency Disorders: Experience from a Referral Center in India

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.

     

Etiology and Outcome of Cholelithiasis in Turkish Children

Objective

The aim of this study was to examine the etiology of gallstones in children and responses to ursodeoxycholic acid (UDCA) treatment.

Methods

74 children with cholelithiasis were recruited, and underwent ultrasonography to detect gallstones. All relevant clinical information was recorded in a structured proforma.

Results

The commonest risk factor was a family history of gallstones. Most children responded to UDCA treatment in the first six months; children with hemolytic diseases showed no response to UDCA.

Conclusion

UDCA treatment may be useful before surgery in asymptomatic patients of cholelithiasis without hemolytic diseases.

     

Encephalopathy in Henoch-Schönlein purpura

Background

Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP.

Case characteristics

Three children with features of HSP presented with seizures and CNS dysfunction.

Observation

All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage.

Message

HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment.

     

Kyphoscolitic type of Ehlers-Danlos syndrome with prenatal stroke

Background

The kyphoscoliotic type of Ehlers-Danlos syndrome (EDS type VIA) is an autosomal recessive disorder characterized by connective tissue dysplasia.

Case characteristics

We report two children with perinatal stroke; accompanied by neonatal joint hypermobility, hypotonia; and early development of kyphoscoliosis.

Outcome

Molecular analysis revealed a PLOD1 gene mutation. Our definitive diagnosis was a EDS VIA.

Message

Prenatal brain stroke is a rare clinical feature of EDSVIA.

     

Vaccine associated paralytic poliomyelitis unmasking common variable immunodeficiency

Background

Oral polio vaccine can rarely lead to Vaccine-associated paralytic poliomyelitis (VAPP).

Case characteristics

A 2-year-old child with asymmetric paralysis of lower limbs following first booster of oral polio vaccine; type 2 Vaccine-derived poliovirus (VDPV) isolated. Subsequently, the child was diagnosed to have common variable immunodeficiency.

Outcome

Paralysis gradually improved on follow-up; monthly intravenous immunoglobulin therapy started for primary immunodeficiency.

Message

We need to evaluate children with VAPP for underlying immunodeficiency.

     

Antiphospholipid Syndrome Complicating Pneumococcal Meningitis

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.

     

Empyema Due to Thoracic Migrating Appendicolith

Background

Retained appendicolith following appendicectomy, and can cause recurrent abscess in the abdomen and retroperitoneum.

Case characteristics

11-yr-old boy who presented with subpulmonic abscess and pneumonia following appendicectomy for perforated appendicitis.

Observations

Thoracotomy revealed a thick walled subpulmonic abscess surrounding an appendicolith along with a rent in the posterolateral aspect of the diaphragm.

Message

In children presenting with pus collections and a history of recent appendicectomy, the possibility of a migrating appendicolith should be considered.

     

MRI in Thoracic Tuberculosis of Children

Objective

To explore the utility of lung magnetic resonance imaging (MRI) in children with thoracic tuberculosis (TB).

Methods

This prospective study of forty children (age range of 5 to 15 y) with thoracic TB was approved by the institutional ethics committee. Chest radiograph, lung MRI and computed tomography (CT) scan were performed in all children. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) of MRI were calculated and kappa test of agreement was used to determine the strength of agreement between the MRI and CT findings.

Results

MRI performed equivalent to CT scan in detection of mediastinal and hilar lymphadenopathy, pleural effusion and lung cavitation (considered typical for TB) with sensitivity and specificity of 100%. MRI also yielded a sensitivity of 88.2% and specificity of 95.7% for nodules, with sensitivity of 100% and specificity of 92.9% for consolidation. Kappa agreement between CT and MRI in detection of each finding was almost perfect (k: 0.8–1).

Conclusions

Although CT scan is still superior to MRI in detection of smaller nodules, MRI demonstrates a very high degree of correlation and agreement with CT scan for detecting lung and mediastinal pathology related to TB, in children with thoracic TB and can be particularly useful in select population and follow-up of these children to avoid repeated radiation exposures.

     

Study of Family Clustering and <Emphasis Type="Italic">PNPLA3</Emphasis> Gene Polymorphism in Pediatric Non Alcoholic Fatty Liver Disease

Objectives

To find association of pediatric NAFLD with metabolic risk factors, and Patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene polymorphism.

Design

Cross-sectional study

Setting

Pediatric Hepatology unit of a tertiary care hospital

Participants

Overweight/obese children (<18 years) with (69 patients) or without (30 patients) NAFLD (ultrasonography based), and their parents.

Intervention

Metabolic screening, PNPLA3 gene polymorphism, and transient elastography

Outcome measure

Association of pediatric NAFLD with parental metabolic risk factors and PNPLA3 gene polymorphism.

Results

In the NAFLD group, there was high parental incidence of metabolic diseases, fatty liver (80%) and low high-density lipoproteins levels (84%). Family history of NAFLD (in any parent), higher alanine aminotransferase levels and higher total cholesterol levels in the child independently predicted possibility of NAFLD, but similar results could not be replicated for PNPLA3 gene polymorphism. Controlled attenuation parameter measurement (by transient elastography) had high sensitivity and specificity to diagnose steatosis.

Conclusion

There is high familial incidence of metabolic diseases in children with NAFLD. Controlled attenuation parameter can be useful as a non-invasive modality to screen fatty liver in children.

     

Endoscopic Management of Vesicoureteral Reflux and Long-term Follow-up

Objectives

To report our experience with endoscopic management of vesicoureteral reflux (VUR) by injection of a tissue bulking substance–Dextranomer/ hyaluronic acid copolymer at vesicoureteric junction.

Design

Retrospective analyses of case records.

Setting

Pediatric Surgery department in a tertiary care government Institute.

Participants

500 children (767 renal units) consecutively referred to the out-patient department with vesicoureteral reflux noted on micturating cysto-urethrogram (MCU) over a period of 13 years (2004-2016).

Intervention

Preoperative VUR grading and renal scars on radionuclide scans were documented. Dextranomer hyaluronic acid copolymer was injected through a cystoscope at the vesicoureteral junction as a day care procedure under short anesthesia. Patients were followed (average duration 27.3 mo) with clinical assessment, periodic urine cultures and renal scans.

Main outcome measure

Cessation of VUR and symptomatic relief / clinical success postoperatively at 3 months.

Results

Complete symptomatic relief was obtained in 482 (96.4%) patients. In 681 units where MCU was available, 614 (90%) units showed resolution of VUR.

Conclusion

Endoscopic injection of tissue bulking substances at vesicoureteric junction to stop VUR seems to be an effective intervention

     

Predictors and Outcome of Acute Kidney Injury in Children with Diabetic Ketoacidosis

Objective

To identify predictors and outcome of acute kidney injury (AKI) in children with diabetic ketoacidosis (DKA) admitted to a Pediatric Intensive Care Unit (PICU).

Methods

Retrospective case review of 79 children with DKA admitted between 2011-2014.

Results

28 children developed AKI during the hospital stay; 20 (71.4%) recovered with hydration alone. Serum chloride at 24 hours was independently associated with AKI. Children with AKI had prolonged acidosis, longer PICU stay, and higher mortality.

Conclusions

Majority of children with AKI and DKA recover with hydration. Hyperchloremia at 24 hours had independent association with AKI, although cause-effect relation could not be ascertained.

     

Transbronchial Lung Cryobiopsy for Diagnosis of Pediatric Interstitial Lung Disease

Background

Tissue diagnosis of Childhood interstitial lung diseases is of paramount importance to outline management.

Case characteristics

A 10-year-old boy with prolonged cough, and computed tomography of thorax with features suggestive of primary Langerhans’s cell histiocytosis.

Intervention

Transbronchial cryobiopsy of lung using flexible cryoprobe, revealed a final diagnosis of Surfactant protein C/ABCA3 deficiency.

Message

Transbronchial cryobiopsy of the lung can provide adequate lung tissue for a categorical diagnosis of interstitial lung diseases in children.

     

Outcome of antenatally presenting posterior urethral valves (PUV) in children

Objective

To analyze the outcome of children with posterior urethral valves who presented with antenatal hydronephrosis.

Methods

A 10-year retrospective review of records of 70 children with posterior urethral valves.

Results

The mean (SD) gestational age at diagnosis was 34 (4.48) weeks, and age at intervention was 130.5 (170.9) days. The nadir creatinine was significantly raised (>1.2 mg/dl) in children with oligohydramnios and diversion.

Conclusion

All boys with antenatally detected hydronephrosis need postnatal evaluation to rule out posterior urethral valves. Short term outcome is improved with postnatal treatments, and longer follow-up is needed to ensure a favourable outcome.

     

Repeated administrations of rituximab along with steroids and immunosuppressive agents in refractory steroid-resistant nephrotic syndrome

Background

A recent randomized control trial in children with steroid-resistant nephrotic syndrome revealed that two doses of rituximab did not reduce proteinuria.

Case characteristics

A 14-month-old boy developed refractory steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis.

Observation

The patient achieved complete remission 11 months after disease onset following eight doses of rituximab combined with steroids and cyclosporine.

Message

Long-lasting B cell depletion with repeated rituximab administrations may be required to achieve complete remission in patients with steroid-resistant nephrotic syndrome and massive proteinuria.