For childhood gastroenteritis, enteral rehydration is as effective if not better than IV rehydration. Enteral rehydration by the oral or nasogastric route is associated with significantly fewer major adverse events and a shorter hospital stay compared with IV therapy and is successful in most children.
We describe two patients with Pallister-Hall syndrome (PHS), both with evidence of a generalized skeletal dysplasia as typified by upper and lower acromesomelic limb shortening and the previously unreported fibular hypoplasia, radio-ulnar bowing, and proximal epiphyseal hypoplasia. Genomic DNA was only available for sequencing analysis in patient 2 and the mutation, c.3386_3387delTT was detected in exon 14 of the GL13 gene. It is also possible that the findings in patient 1 represent the phenotypic expression of a novel GLI3 mutation. This report further expands the PHS phenotype and raises the possibility of specific GLI3 mutations resulting in more severe skeletal features. It also suggests that PHS should be included in the differential diagnosis of antenatally ascertained acromesomelic limb shortening and bowing with fibular hypoplasia particularly in the presence of polysyndactyly.
Aim
To determine if the management of paediatric status epilepticus (SE) follows accepted clinical practice guidelines.
Methods
Retrospective, consecutive series of patients with SE who attended the emergency departments from two NSW sites over a 12‐month period. SE was defined as a convulsive seizure, 5 min or more in duration. Time to presentation to the ED, time to first‐ and second‐line treatment, number of benzodiazepine (BZD) doses given prior to intubation and adherence to guidelines were evaluated. The outcomes included seizure duration, need for respiratory support, admission to intensive care, morbidity and mortality.
Results
The time from onset of seizure to ED presentation was a median (p25–p75) time of 22 (15–40) min. Forty‐eight of 59 presentations received pre‐hospital midazolam. The median (p25–p75) time to first‐line treatment was 15 (8–25) min and to second‐line treatment was 43.5 (35–59) min. There was no significant difference in the results in the two hospitals. The total number of BZD doses ranged from 1 to 7 (median 3). There was non‐adherence to the clinical practice guidelines in 55 (93.2%) of 59 presentations.
Conclusions
We found excessive benzodiazepine use and delay in both definitive treatment of status epilepticus and in escalation from first‐ to second‐line anticonvulsant treatment. This raises the need for rapid escalation of treatment. We propose a ‘status epilepticus code’ for emergency departments.
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