Hurler's syndrome, also known as mucopolysaccharidosis I (MPS I-H), is a rare condition inherited as an autosomal recessive trait. It is caused by a deficiency in alpha-L-iduronidase, an enzyme that participates in the degradation of the glycosaminoglycans (GAGs) heparin sulphate and dermatan sulphate. Children with Hurler's syndrome appear nearly normal at birth but, left untreated, show a progressive mental and physical deterioration caused by a build-up of GAGs in all organs of the body. Death is often caused by cardiac or respiratory failure and usually occurs before the second decade of life. In recent years, bone marrow transplantation (BMT) has been employed in the management of patients with Hurler's syndrome. However, the dental findings observed in these cases have not previously been reported in the dental literature. Here we report a patient aged 11 years and 6 months, presented to a Specialist Paediatric Dentistry Unit, who was successfully treated by BMT at 18 months of age.

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Aim An exploration of the threshold that dentists, doctors and nurses recognise for dental and child protection (CP) actions in sample clinical cases, and any differences between these professional groups.Method We present a cross-sectional survey of dentists, doctors and nurses (50 each), who regularly examine children, utilised five fictitious vignettes, combining an oral examination image and clinical history reflecting dental and CP issues. Demographics were collected, and each participant gave their likely action for the cases presented.Results Dentists were significantly better at answering the dental element than the doctors and nurses, (P <0.0001) with no significant difference between these two; only 8% of the latter had undergone any training in assessment of dental health. Although 90.6% of all professionals had undergone CP training, dentists were significantly less accurate at identifying the CP component than doctors and nurses, (P <0.0001) between whom there were no significant differences. Those with higher levels of CP training were most accurate at identifying correct CP actions.Conclusions CP training is effective at improving recognition of child maltreatment, although there remains a worrying lack of knowledge about thresholds for action among dentists. Doctors and nurses have minimal training in, or knowledge of, dental health in children, thus precluding appropriate onward referrals.

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