Journal of child neurology
-
Propionic acidemia is a hereditary metabolic disease caused by a deficiency of enzyme propionyl-CoA carboxylase, which is involved in the catabolism of ramified amino acids, odd-chain fatty acids, and other metabolites; the deficiency of this enzyme leads to an accumulation of toxic substances in the body. There are various forms of clinical presentation (severe neonatal, chronic intermittent, or slow and gradual). ⋯ After initiating treatment involving a diet that was low in proteins, carnitine, and biotin, and an open-formula diet of ramified amino acids, the patient made progress, showing signs of improved hypotonia and increased weight gain. His vomiting stopped, and ketoacidosis was corrected.
-
Admissions from January 1, 1985, to December 31, 2005, with the diagnosis of infant botulism were reviewed to describe the clinical presentation, course, outcome, and changes related to the availability of botulism immune globulin treatment. Botulism diagnoses were confirmed by the finding of toxin or Clostridium botulinum organisms in stool samples (type A, 14; type B, 25; type not noted, 5). Twenty-four patients were admitted from 1985-1994 and 20 from 1995-2004. ⋯ Length of stay was shorter in infants treated with botulism immune globulin (13.5 vs 23 days, P = .009), with a trend toward reduced need for nasogastric feeding and in shorter duration of tracheal intubation. All patients recovered fully. Even with the availability of botulism immune globulin, meticulous supportive care remained essential for recovery.
-
Semantics matter. It is all about precision in communication. Specific terms should not be degraded to general terms by using them interchangeably with others of different meaning or nuance. ⋯ Unfamiliar and excessive abbreviations should be avoided in manuscripts, titles, and PowerPoint presentations. Writers should be aware that computer programs may automatically alter spellings of technical terms, rendering them incorrect. One should respect correct grammar and orthography in both English and other languages, including accent marks in foreign words and names.
-
Randomized Controlled Trial Comparative Study
Intravenous sodium valproate versus diazepam infusion for the control of refractory status epilepticus in children: a randomized controlled trial.
An open-label, randomized controlled study was conducted at a tertiary care teaching hospital to compare efficacy and safety of intravenous sodium valproate versus diazepam infusion for control of refractory status epilepticus. Forty children with refractory status epilepticus were randomized to receive either intravenous sodium valproate or diazepam infusion. Refractory status epilepticus was controlled in 80% of the valproate and 85% of the diazepam patients. ⋯ None of the patients in the valproate group required ventilation or developed hypotension, whereas in the diazepam group 60% required ventilation and 50% developed hypotension after starting diazepam infusion. No adverse effects on liver functions were seen with valproate. It is concluded that intravenous sodium valproate is an effective alternative to diazepam infusion in controlling refractory status epilepticus in children and is free of respiratory depression and hypotension.
-
Many physicians consider examining the nervous system as one of the most difficult parts of the physical examination. Difficult and poorly cooperative children remain the most challenging group to examine accurately and completely. In this situation, the physician becomes less confident about the neurological findings and clinical evaluation. ⋯ In this review, some practical tips and techniques are presented that can be used to improve the likelihood of obtaining accurate information about the neurological status of young and difficult children. Certainly, repeated examinations and experience play an important role; however, solid knowledge, strong communication skills, accurate observational skills, and use of proper techniques are crucial for eliciting and interpreting neurological signs in difficult children. Finally, a patient and empathetic physician and supportive guiding parents are needed for a successful neurological assessment.