Management of oral manifestations of a child with Heimler Syndrome-2.

Heimler Syndrome 2 (HS-2) is a rare, autosomal recessive mild form of a peroxisomal biogenesis disorder. Though knowledge regarding the disorder is limited, emerging research has found that sensorineural hearing loss, occasional or late onset pigmentation, amelogenesis imperfecta and nail abnormalities are clinical characteristics representative of HS-2.A school-aged male presented to the dental department with a chief complaint of a lack of enamel on multiple teeth. The patient's medical history was significant for patent ductus arteriosus, bilateral sensorineural hearing loss and biallelic mutation of the PEX6 gene. The clinical exam revealed dental crowding, hypoplasia, hypo-calcification of multiple teeth and enlarged pulp chambers of maxillary molars. This case report details the clinical findings associated with HS-2, the comprehensive dental treatment to be rendered to the patient, and critical information to paediatric dentists and general dentists so that they can make proper referrals to medical specialties.

Hypophosphatemic Rickets and Pre-eruptive Spontaneous Dental Abscess.

The purpose of this report is to describe the diagnosis and dental treatment of a medically complex 14-year-old boy with X-linked hypophosphatemic rickets who presented with a pre-eruptive abscess on a mandibular molar and facial swelling.

Influence of smell loss on taste function.

The influence of damage to one sensory system on the functioning of other sensory systems has been of scientific interest for over a century. Recently it has been suggested that damage to the olfactory system (CN I) decreases the ability to taste; that is, to perceive sweet, sour, bitter and salty sensations via CN VII, IX, and X. If true, this would be in contrast to most other sensory systems, where compromise typically leads to either cross-modal enhancement of function or to no effects at all. In the present study, data from both regional and whole-mouth taste tests of 581 patients presenting to a smell and taste center with varying degrees of olfactory function were evaluated to address whether olfactory loss is associated with taste loss. No significant influence of olfactory dysfunction on any measure of taste perception was found when the effects of sex, age, and etiology were controlled. These data suggest that smell loss, per se, has no meaningful influence on taste function, and that clinical associations between smell and taste dysfunction, when observed, likely reflect comorbid influences.