IntroductionRespiratory failure is a life threatening complication of Guillain Barré syndrome (GBS). There is no consensus on the specific treatment for this subset of children with GBS.MethodsThis was a prospective randomized study to compare the outcome of intravenous immunoglobulin (IVIG) and plasma exchange (PE) treatment in children with GBS requiring mechanical ventilation. Forty-one children with GBS requiring endotracheal mechanical ventilation (MV) within 14 days from disease onset were included. The ages of the children ranged from 49 to 143 months.Randomly, 20 children received a five-day course of IVIG (0.4 g/kg/day) and 21 children received a five-day course of one volume PE daily. Lumbar puncture (LP) was performed in 36 patients (18 in each group).ResultsBoth groups had comparable age (p = 0.764), weight (p = 0.764), duration of illness prior to MV (p = 0.854), preceding diarrhea (p = 0.751), cranial nerve involvement (p = 0.756), muscle power using Medical Research Council (MRC) sum score (p = 0.266) and cerebrospinal fluid (CSF) protein (p = 0.606).Children in the PE group had a shorter period of MV (median 11 days, IQR 11.0 to 13.0) compared to IVIG group (median 13 days, IQR 11.3 to 14.5) with p = 0.037.Those in the PE group had a tendency for a shorter Pediatric Intensive Care Unit (PICU) stay (p = 0.094).A total of 20/21 (95.2%) and 18/20 (90%) children in the PE and IVIG groups respectively could walk unaided within four weeks after PICU discharge (p = 0.606).There was a negative correlation between CSF protein and duration of mechanical ventilation in the PE group (p = 0.037), but not in the IVIG group (p = 0.132).ConclusionsIn children with GBS requiring MV, PE is superior to IVIG regarding the duration of MV but not PICU stay or the short term neurological outcome.The negative correlation between CSF protein values and duration of MV in PE group requires further evaluation of its clinical usefulness.Trial RegistrationClinicaltrials.gov Identifier NCT01306578

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract, and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of JDM, generalized edema has rarely been reported. Here, we report a 3.5-year-old boy with JDM presenting with generalized edema. The diagnostic criteria of JDM rely on typical clinical manifestations that include: severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum skeletal muscle enzymes, and myopathic electromyographic pattern. Our patient initially received methylprednisolone and intravenous immunoglobulin (IVIG) without significant improvement, so he was given azathioprine and a prolonged course of oral prednisolone. We conclude that JDM should be suspected in patients presenting with anasarca in the absence of laboratory parameters of other causes of generalized edema and an appearance of heliotrope rash with muscle weakness. Also, we suggest that muscle magnetic resonance imaging (MRI) should be considered among the diagnostic tools of JDM.

This study was designed to evaluate the effect of once-daily montelukast therapy on the clinical progress and the cytokine profile of patients with acute viral bronchiolitis. A randomized, double-blind, placebo-controlled trial included 85 patients (mean age, 3.5 +/- 2.35 months), clinically diagnosed as first-episode acute bronchiolitis in addition to 10 healthy controls of matched age and sex. Patients were randomly assigned to receive either montelukast (4-mg sachets; n = 47) or placebo (n = 38) daily from the time of admission until discharge. The primary outcome measure was the length of hospital stay (LOS), and clinical severity scores (CSs) and changes in plasma levels of interferon gamma and interleukin-4 were secondary outcomes. LOS for the montelukast group was found to be significantly lower than that of the placebo group (p < 0.05). This effect was also found at nonsignificant levels among patients with a positive family history of asthma or allergy. Moreover, cases receiving montelukast showed lower CSs all through the hospital stay that were significant in the first 24 hours (p < 0.05). Montelukast is probably of benefit as an episodic modifier in infants with acute viral bronchiolitis.

In children with DR-HD, NS is a safe rehydration fluid with a satisfactory rate of Na(+) drop and relatively low incidence of morbidity and mortality. Judicious use of IVE should be exerted and closer monitoring should be guaranteed for children requiring large volumes for IVE and for those showing rapid initial drop of serum Na(+) to avoid neurological complications and poor outcome.

No abstract