Unilateral submandibular suppurative sialadenitis in a premature infant.

UNLABELLED: Isolated submandibular suppurative sialadenitis is extremely rare in newborn infants and is associated with prematurity and prolonged gavage feeding. This report describes a premature infant who developed a life-threatening airway obstruction due to suppurative submandibular sialadenitis. The diagnosis was made on clinical grounds and confirmed by ultrasonography. Staphylococcus aureus was grown from the pus expressed from the Wharton's duct orifice. Upper airway obstruction and respiratory failure were managed with intubation and mechanical ventilation, and the sialadenitis resolved quickly and completely with flucloxacillin treatment. Possible causes of sialadenitis include dehydration, decreased saliva flow and stasis during gavage feeding, duct obstruction by stones and direct bacterial inoculation. Ultrasonography is the diagnostic imaging of choice to exclude congenital tumours, lymphadenitis, congenital malformations of the Wharton's duct or the gland itself, and subcutaneous fat necrosis. CONCLUSION: Early diagnosis and antibiotic treatment of suppurative submandibular sialadenitis may prevent complications such as abscess formation, septicaemia and respiratory failure.

Founder effect at PGL1 in hereditary head and neck paraganglioma families from the Netherlands.

PGL1, a gene responsible for hereditary paragangliomas of the head and neck, recently was mapped to a 2-cM interval on chromosome 11q22-q23, by linkage and haplotype-sharing analysis of a large multibranch Dutch family. We determined the disease-linked haplotype, as defined by 13 markers encompassing a large interval on 11q21-q23, in 10 additional families ascertained from the same geographical locale. Alleles were identical for six contiguous markers, spanning a genetic distance of 6 cM and containing PGL1. Despite this strong indication of a common ancestor, no kinships between the families could be demonstrated through genealogical surveys going back to 1800 a.d. We conclude that a single ancestral mutation is responsible for most, if not all, hereditary paragangliomas, in this region of The Netherlands, and that strong founder effects may exist at the PGL1 locus.