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Plastic bronchitis, a condition associated with widespread mucous plugging of the tracheobronchial tree, is an increasingly recognised bronchoscopic finding in acute chest syndrome of sickle cell disease. Removal of casts by bronchoscopy is technically challenging. We describe a child with acute chest syndrome where bronchoscopic removal of extensive(More)
Mutations in the αB-crystallin (CRYAB) gene, encoding a small heat shock protein with chaperone function, are a rare cause of myofibrillar myopathy with autosomal-dominant inheritance, late-onset and moderate severity. We report a female infant presenting from 4 months with profound muscle stiffness, persistent creatine kinase elevation and electromyography(More)
Base deficit is a parameter often used to guide further treatment in acidotic children and is taken as a measure of how "sick" they are. Five children with septic shock are presented who had persisting base deficit after large volume resuscitation with 0.9% saline. Stewart's strong ion theory of acid-base balance is able to quantify the causes of metabolic(More)
OBJECTIVES Once-daily gentamicin therapy is becoming increasingly common in pediatric practice; however, little is known about pharmacokinetics in critical illness. Gentamicin exhibits concentration dependant killing; thus, peak serum concentrations at least eight times higher than minimum inhibition concentration of the target organism have been(More)
OBJECTIVE Stewart's strong ion theory quantifies unmeasured tissue acids produced following hypoxia or hypoperfusion, by calculation of the strong ion gap. Our study objectives were as follows: a) to determine the 24-hr profile of the strong ion gap following cardiopulmonary bypass surgery; and b) to compare the prognostic value in terms of intensive care(More)
OBJECTIVE Stewart's physicochemical approach to acid-base balance defines the aetiology of a metabolic acidosis by quantifying anions of tissue acids (TA), which consist of unmeasured anions (UMA) and/or lactate. We hypothesised that an increase in TA during metabolic acidosis would lead to a compensatory fall in the plasma chloride (Cl) relative to sodium(More)