Case report: hypophosphatemic rickets and aggressive periodontitis: a review of the role of dentine matrix protein 1 in the pathogenesis.

BACKGROUND: The association between hypophosphatemic rickets (HR) and excessive periodontal breakdown was reported in mice models of HR. In humans, this is the first report of a possible association between HR and periodontal breakdown. CASE REPORT: The following presents a report of a case of a 15 yearold child diagnosed with HR at age 9 years, with atypical premature spontaneous loss of teeth due to periodontal defects in the absence of dental abscesses, dental caries, or trauma. The case is discussed in the context of relevant literature; the possible role of dentine matrix protein 1 in the aetiology of such periodontal defects in patients with HR is also discussed. CONCLUSION: Spontaneous loss of teeth in the absence of abscess formation is not one of the reported features of HR, however, this report may alert clinicians of the possibility of such association especially in the autosomal recessive type. Further case reports and more elaborate genetic and molecular testing is needed to verify this especially in late diagnosis cases.

Craniofacial morphology in patients with hypophosphataemic vitamin-D-resistant rickets: a cephalometric study.

Hypophosphataemic vitamin-D-resistant rickets (HVDRR) is a hereditary disease mainly transmitted as an X-linked dominant trait and characterized by certain general clinical signs (Filho HM, de Castro LC, Damiani D. Arq Bras Endocrinol Metab. 2006;50:802). In literature, only one study had been published in 1965 on the cephalometric findings in patients with HVDRR (Marks SC, Lindahl RL, Bawden JW. J Dent Child. 1965;32:259). This is the first detailed study on craniofacial characteristics of patients with HVDRR in the dental literature. The aim of this study was to determine the effect of HVDRR on the parameters of the craniofacial skeleton of young Jordanian patients using cephalometric analysis. Lateral cephalometric radiographs were made for 22 Jordanian children (aged 2-16 years) diagnosed with HVDRR. The cephalometeric parameters of HVDRR group were compared with those of normal control group matched for gender and chronological age using paired t-test. The HVDRR group had a significant increase in the SNBa angle (P < 0.01); as well as reduced anterior cranial base length (P = 0.01), reduced maxillary length, corpus mandibular length and mandibular height (P = 0.01, 0.04 and 0.008 respectively). The cranial base and gonial angles were significantly increased in diseased individual, but the SNA and ANB angles were significantly reduced (P = 0.018 and 0.000 respectively). The angulation of the lower incisor to mandibular plane was also significantly reduced in the diseased group compared with Jordanian norm (P = 0.004). Patients with HVDRR have deficiency in the anterior cranial base length, ramus height and cranial base angle. Patients with HVDRR also have class III skeletal relationship.

Incidence of major infection in sickle cell pediatric patients at Qatif Central Hospital.

During the two-year period, 20 Jumada I 1408 (January 9, 1989) to 20 Jumada I 1410 (December 18, 1989), a total of 450 pediatric patients with sickle-cell anemia were admitted to the pediatric ward of Qatif Central Hospital. Thirty-nine patients had a major infection (septicemia, meningitis, or osteomyelitis), with an incidence of 8.6%. During the same period, 3700 non-sickle pediatric patients were admitted and 38 of them had a major infection, with an incidence of 1% (P<0.001). Three patients died in the sickle cell groups, for a case fatality rate of 7.6%, while only one patient in the control group died, for a case fatality rate of 2.6% (P<0.002). Salmonella species were responsible for 12 cases of septicemia and three episodes of osteomyelitis, and Streptococcus pneumoniae was the source in eight cases of septicemia and one case of meningitis in sicklers. Meningitis was caused by different organisms, such as Streptococcus pneumoniae, Salmonella, and Meningococcus. Antibiotics that cover both Salmonella and Streptococcus pneumoniae must be considered in all children with sickle cell anemia who have a febrile illness.