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1.

Background

Brucella has been known to cause pyrexia of unknown origin.

Case Characteristics

9-year-old boy with fever and abdominal pain; multiple abscesses within the liver on ultrasonography.

Observations

IgM Antibodies against Brucella were raised in his serum sample, and Brucella serum agglutination test was positive. Immunological work-up suggested selective IgA deficiency. Reduction in size following treatment with trimethoprim-sulphamethoxazole, amikacin and doxycycline.

Message

Brucellosis should be considered as an etiology of liver abscess in patients not responding to conventional antibiotics.
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2.

Background

Appendicolith can cause appendiceal obstruction and acute appendicitis. Its high prevalence may be related to the high perforation rate in pediatric appendicitis. This study assessed the characteristics of appendicolith and its clinical significance in pediatric appendicitis.

Methods

A retrospective study was performed among children and adolescents younger than 17 years who were preoperatively diagnosed with appendicitis in the pediatric emergency department (ED). A total of 269 patients with a mean age of 9.98 ± 3.37 years were enrolled. Clinical features and contrast-enhanced computed tomography findings were analyzed.

Results

Among the 269 patients, 147 (54.6%) had appendicoliths, with a mean maximal diameter of 5.21 ± 2.34 mm. Compared to the no appendicolith group, the appendicolith group demonstrated more prolonged abdominal pain (≥ 48 hours) before the ED visit (23.1% vs. 11.5%; P = 0.013), clinical features of severe appendicitis (presence of fever, vomiting, positive urine ketone, and increased C-reactive protein), and higher rate of perforation (43.5% vs. 9.8%; P < 0.001). Multivariate risk factor analysis for perforated appendicitis in the appendicolith group revealed that maximal diameter of 5 mm or more in the appendicolith (adjusted odds ratio [aOR] 2.919; 95% CI 1.325–6.428, P = 0.008) and proximal collapse adjacent to the appendicolith (aOR 2.943; 95% CI 1.344–6.443, P = 0.007) were significant.

Conclusions

Pediatric appendicitis with appendicolith often presents with prolonged abdominal pain and severe clinical conditions with a high risk of perforation.
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3.

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.
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4.

Background

Atrial flutter not responding to medications could be secondary to structural malformations of heart.

Case characteristics

A 5-year-old child with resistant arrhythmia, with onset in neonatal period.

Outcome

Multiple right atrial diverticuli were detected on CT angiography and cardiac catheterization. Patient reverted to sinus rhythm following surgical excision of diverticuli.

Message

In cases of intractable supraventricular tachycardia, structural anomalies of atrium should be suspected.
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5.

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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6.

Background

Synovial arteriovenous malformation is rare.

Case characteristics

We present three children with recurrent monoarthritis secondary to synovial arteriovenous malformation.

Outcome

Two children underwent excision of arteriovenous malformation. Another child had diffuse arteriovenous malformation, which was inoperable.

Message

Synovial arteriovenous malformations should be considered in the differential diagnosis of monoarthritis, especially of the knee.
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7.

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.
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8.

Background

Foreign bodies in the airway can be a diagnostic and therapeutic challenge.

Case characteristics

30-month-old girl with complaints of noisy and fast breathing following fall over a pile of sand. Sand was suctioned out by direct bronchoscopy. The child improved initially but condition worsened in next four days with marked stridor and wheeze.

Observation

Imaging revealed elongated sharp radiodense opacity in the cervical region, suggestive of foreign body. At repeat bronchoscopy, paint material was removed from the airway, leading to recovery

Message

Paint material mixed in the sand can adhere to the walls of the airway, and cause persistent symptoms of obstruction.
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9.

Background

Schimmelpenning syndrome is a multisystem disorder.

Case characteristics

A term female neonate with sebaceous nevi of the face had choroid osteoma of the right eye.

Observation

At one month of age, the infant was observed to have choroidal neovascularization that was successfully treated with laser photocoagulation and anti-VEGF.

Message

Choroid osteoma and neovascularization are rare associations of Schimmelpenning syndrome, and should be screened for and managed early.
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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

Inflammatory myofibroblastic tumors of the trachea are rare childhood quasi-neoplastic lesions.

Case characteristics

7-year-old boy with recurrent episodes of cough, breathing difficulty and wheeze, initially treated as asthma.

Intervention

CT chest and flexible bronchoscopy revealed a mass lesion of the trachea, which was excised by diode laser through the ventilating bronchoscope. Histopathology confirmed it as the inflammatory myofibroblastic tumor.

Message

Use of laser ensured complete endotracheal excision of the tumor.
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12.

Background

Blue rubber bleb nevus syndrome is a rare disease involving venous malformations.

Case characteristics

We present a 6-year-old female with the syndrome, and consumptive coagulopathy.

Intervention/Outcome

After management with sirolimus, symptoms resolved.

Message

Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.
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13.

Background

Neuromelioidosis is a rare conduction, which is difficult to diagnose and treat.

Case characteristics

Preadolescent girl presenting with prolonged fever, acute ascending paralysis and encephalopathy.

Outcome

Neuromelioidiosis was confirmed on brain biopsy culture. Patient improved with an intensive antibiotic regimen.

Message

Neuromelioidosis can mimic acute demyelinating encephalomyelitis clinically and radiologically.
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14.

Background

Neuroschistosomiasis is an uncommonly reported disease.

Case characteristics

An adolescent Indian boy residing in Kenya presented with headache, visual symptoms and seizures, with MRI showing space-occupying lesions in the occipital lobe and cerebellum.

Observation

Brain biopsy was diagnostic of neuro-schistosomiasis; complete recovery was seen with praziquantel and corticosteroid therapy.

Message

This case highlights the importance of considering epidemiology in differential diagnosis and establishing definitive diagnosis even if it is by invasive methods.
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15.

Background

Congenital tracheobiliary fistula is a rare developmental anomaly with a persistent communication between the biliary system and the trachea.

Characteristics

A 7-day-old baby with severe respiratory distress and aspiration pneumonia.

Outcome

Tracheobilliary fistula identified on bronchoscopy. Open surgical excision of fistula was followed by improvement.

Message

This condition should be considered in the differential diagnosis of intractable aspiration pneumonia.
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16.

Background

Allogeneic stem cell transplant is the only curative treatment for Wiskott-Aldrich syndrome.

Case characteristics

18-months-old boy with no sibling, cord blood or matched unrelated donor transplant options.

Outcome

Doing well 7 years after haploidentical stem cell transplantation using unmanipulated bone marrow as the stem cell source.

Message

Father as a haplo-identical donor is a feasible option.
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17.

Background

Immunodeficient children are more prone for invasive cryptococcal infections.

Case characteristics

A 2-year-old boy with disseminated cryptococcosis was evaluated for underlying immunodeficiency without success.

Intervention/outcome

Child was managed successfully.

Message

Immunocompetent children with disseminated cryptococcosis can present diagnostic or therapeutic challenge in resource-limited settings.
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18.

Background

Tyrosine Hydroxylase deficiency is a rare neurotransmitter disorder.

Case Characteristics

An Indian family with the disorder.

Observation

Phenotypic variation, elevated serum prolactin, genetic confirmation, and partial treatment-responsiveness.

Messages

Tyrosine Hydroxylase deficiency is a treatable inborn error of metabolism and serum prolactin assists in diagnosis.
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19.

Objective

To develop nomogram of Transcutaneous Bilirubin among healthy term and late-preterm neonates during first 96 hours of age.

Design

Longitudinal observational study.

Setting

Neonatal unit of a tertiary care Hospital of Central Gujarat, India.

Participants

1075 healthy term and late preterm neonates (≥35weeks).

Intervention

Six-hourly transcutaneous bilirubin was obtained from birth to 96 hour of life using Drager JM 103 Transcutaneous Bilirubinometer.

Main outcome measures

Nomogram of Transcutaneous Bilirubin with percentile values was obtained, rate of rise of bilirubin was calculated and predictive ability of normative data was analyzed for subsequent need of phototherapy.

Results

The age-specific percentile curves and nomogram were developed from the transcutaneous bilirubin readings of 1,010 neonates. Rate of rise in first 12 hour was 0.2 mg/dL and was 0.17 mg/dL in 12 to 24 hour of life which decreased on second day of life. Neonates who required phototherapy had consistently higher readings of transcutaneous bilirubin and also higher rate of rise in first 48 hrs.

Conclusion

Neonates whose transcutaneous bilirubin is above the 50th percentile should be monitored for the development of significant hyperbilirubinemia.
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20.

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
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