Open versus Closed Suctioning Among Mechanically Ventilated Pediatric Patients: A Randomised Control Trial.

OBJECTIVES: To compare the difference in efficacy of closed tracheal suction system (CTSS) to open tracheal suction system (OTSS) in reducing incidence of ventilator associated pneumonia (VAP). Also to evaluate their efficacy in stabilizing cardio-respiratory parameters, reducing mortality and duration of intubation. METHODS: This study was a single centre, parallel group, open label, randomized controlled study with an equal allocation (1:1) in pediatric patients requiring mechanical ventilation. A specific suction system of CTSS or OTSS was assigned to the two groups based on randomization. All the demographic, clinical, laboratory parameters and treatment outcomes were noted in the preformed sheet. RESULTS: Total 116 eligible pediatric ventilated patients were studied. Total incidence of VAP was 9 (7.75%) of which 3 occurred in open and 6 in closed suction group. Rate of VAP was similar among both the groups with RR 2.11 (95% CI 0.50-8.9). However, significant number of infection-related ventilator associated condition (IVAC) were found in CTSS (17) compared to OTSS (6) group with RR 3.5 (95% CI 1.3-9.9). SpO2 was better maintained in the CTSS group post-suction (p = 0.001). Incidence of mortality and intubation days were similar between both groups. CONCLUSIONS: Incidence of VAP was similar between open and closed suction groups.

Late Onset Subacute Profound Biotinidase Deficiency Caused by a Novel Homozygous Variant c.466-3T>G in the BTD Gene.

Biotinidase deficiency (BD) is an autosomal recessive disorder caused by bi-allelic mutation in the BTD gene. Clinical manifestations in BD mainly depends on residual biotinidase enzyme activity, although there are some exceptions. Broadly BD disorders are classified as profound BD and partial BD. Further profound BD can be early onset, late onset, and sometimes may be asymptomatic. Clinically late-onset profound BD can present with spectrum of manifestations ranging from single organ to multiple organ involvement, typically affecting function of brain, eye, ear, and skin. Here, a first-born child to consanguineous parents with late-onset profound BD presenting with hyperventilation secondary to lactic acidosis, hypotonia, evolving spasticity, and abnormal neuroimaging findings caused by novel homozygous variant, c.466-3T>G in the BTD gene is reported.

Mechanical Thrombectomy Using Retrievable Stents in Pediatric Acute Ischemic Stroke.

OBJECTIVE: To report efficacy and outcome of mechanical thrombectomy for treatment of pediatric acute ischemic stroke with large vessel occlusion using stent retrievers. METHODS: Retrospective record review of institutional database for patients <18 years of age. RESULTS: Five boys aged between 6 to 17 years received reperfusion therapy using mechanical thrombectomy for acute ischemic stroke with large vessel occlusion (2 basilar, 2 middle cerebral and 1 internal carotid artery). Pediatric National Institute of Health Stroke Scale (PedNIHSS) at onset ranged from 12 to 21. Complete recanalization as defined by the modified Treatment In Cerebral Infarction scale (mTICI 3 or 2b) was achieved in all, using stent retreivers. Favorable outcome as per the modified Rankin scale (mRS 0-1) was achieved in all with no peri-procedural complications. CONCLUSION: Mechanical thrombectomy using retrievable stents is a safe and effective therapy for pediatric ischemic stroke due to large vessel occlusion, and may be offered in carefully selected patients.

Respiratory Morbidity Following Pediatric Orthotopic Liver Transplantation.

We evaluated the pulmonary complications following orthotopic liver transplantation in 45 children (age <18 y). 22 patients (49%) developed respiratory complications. Pediatric end-stage liver disease (PELD) score >25 and positive fluid balance were independent risk factors. Patients with respiratory complication had significantly higher mortality and intensive care unit stay.

Familial 7q11.23 duplication with variable phenotype.

Chromosomal microdeletions and microduplications are known to cause variable clinical features ranging from apparently normal phenotype to intellectual disability, multiple congenital anomalies, and/or other variable clinical features. 7q11.23 region deletion is the cause for Williams-Beuren syndrome and duplication of same region 7q11.23 causes distinguishable clinical phenotype. Familial inheritance is known for both microdeletion and microduplication of 7q11.23 region. Here, we report a patient of paternally inherited 7q11.23 microduplication with developmental delay, macrocephaly, and structural brain malformations.