Pulmonary Involvement in Neuromuscular Diseases: A Review.

Neuromuscular diseases (NMDs) affect muscle function directly or indirectly by affecting nerves or neuromuscular junctions. One of the leading causes of death in patients with NMD is respiratory muscle weakness (RMW). Respiratory involvement in patients with NMD can manifest widely, from mild failure that may initially affect only sleep to severe failure that can be life-threatening. Care approaches include arranged and precise clinical follow-ups of signs of sleep-disordered breathing, daytime hypoventilation, coughing, and swallowing disturbances. This manuscript will review the mechanisms and abnormalities of respiratory function in patients with NMD and help optimize NMD management.

Iron deficiency and iron deficiency anemia in children with first attack of seizure and on healthy control group: a comparative study.

OBJECTIVE: Seizures are the most common pediatric neurologic problem. Research of the association between iron deficiency and seizures has shown conflicting results. This study evaluates iron status of children with a first seizure attack (febrile seizure (FS) or first unprovoked afebrile seizure (FUS) and healthy control group. MATERIALS & METHODS: In a cross sectional case control study, iron status of 6-60 month year old admitted children with first seizure to Shahid Sadoughi Hospital from August 2011-December 2012 were evaluated and compared with healthy control children that were referred to primary health care center of Azadshar, Yazd, Iran. RESULTS: 150 children were compared in three equal (FS, afebrile seizure, and control) groups. Hemoglobin levels in FUS (11.39 ± 1.07 g/dl) and FS (11.46 ± 1.18 g/dl) were lower than the control group (11.9 ± 0.89 g/dl) group. Serum iron levels in FS (38.52 ± 11.38 µg/dL) and FUS (42.68 ± 14.76 µg/dL) were lower than the control group (54.32 ± 13.46 µg/dL). Serum ferritin level in FUS (46.21 ± 27.63 ng/mL) and FS (48.91 ± 22.96 ng/ mL) was lower than the control group (75.13 ± 35.57 ng/mL). Iron deficiency (48% in FS, 44% in FUS and 28% in control group) and iron deficiency anemia (26% in FUS, 22% in FS, and 10% in healthy children) was more frequent in children with seizures. CONCLUSION: Iron status should be evaluated in children with a first attack of febrile or afebrile seizures.

Efficacy of chloral hydrate-hydroxyzine and chloral hydrate-midazolam in pediatric magnetic resonance imaging sedation.

OBJECTIVE: Magnetic resonance imaging (MRI) is a useful diagnostic tool for the evaluation of congenital or acquired brain lesions. But, in all of less than 8-year-old children, pharmacological agents and procedural sedation should be used to induce motionless conditions for imaging studies. The purpose of this study was to compare the efficacy and safety of combination of chloral hydrate-hydroxyzine (CH+H) and chloral hydrate-midazolam (CH+M) in pediatric MRI sedation. MATERIALS & METHODS: In a parallel single-blinded randomized clinical trial, sixty 1-7-year-old children who underwent brain MRI, were randomly assigned to receive chloral hydrate in a minimum dosage of 40 mg/kg in combination with either 2 mg/kg of hydroxyzine or 0.5 mg/kg of midazolam. The primary outcomes were efficacy of adequate sedation (Ramsay sedation score of five) and completion of MRI examination. The secondary outcome was clinical side-effects. RESULTS: Twenty-eight girls (46.7%) and 32 boys (53.3%) with the mean age of 2.72±1.58 years were studied. Adequate sedation and completion of MRI were achieved in 76.7% of CH+H group. Mild and transient clinical side-effects, such as vomiting of one child in each group and agitation in 2 (6.6 %) children of CH+M group, were also seen. The adverse events were more frequent in CH+M group. CONCLUSION: Combinations of chloral hydrate-hydroxyzine and chloral hydrate-midazolam were effective in pediatric MRI sedation; however, chloral hydrate-hydroxyzine was safer.