The lower extremity in Morquio Syndrome (MPS-IV A)
Morquio Syndrome (or mucopolysaccharidosis IV) is a relatively rare autosomal recessive, usually inherited, mucopolysaccaride storage disease which involves the accumulation of keratin sulfate. While appearing normal at birth, they frequently present with spinal deformities, limb abnormalities and short stature (plus other abnormalities) at 2-3 years of age.
A retrospective chart and x ray review of 23 MPA-IV A children who presented at 6.8 years and who were followed for 6.7 years, found progressive hip subluxation, genu valgum and ankle valgus in all patients. On average, each patient required eight operative procedures.
Source: Journal of Pediatric Orthopaedics
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Efficacy of "Bag" vs. "Catheter" collected urine for suspected urinary tract infection (UTI)
192 non-toilet trained children less than 3 years of age, all of whom were suspected of having a UTI following a positive urine culture obtained from a urine "bag", had bladder catheter urine obtained and the specimens compared.
7.5% of "bag" obtained specimens had a false positive result and 29% of them with negative results, were falsely negative. Overall, "bag" obtained urine specimens were inaccurate in 40% of cases.
Every "Bag" -obtained positive urine culture should be confirmed by a more reliable method.
Source: The Journal of Pediatrics
Can specific sucking and swallowing exercises accelerate independent feeding in VLBW infants?
Sucking exercises (on a pacifier) do not improve time to full oral feeding in VLBW infants, while swallowing exercises consisting of placing a milk bolus (0.05-0.2ml) on the posterior part of the tongue, do.
Source: Acta Paediatrica
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