PEIR defects occur at a significant prevalence rate in unerupted teeth. Increased awareness and careful radiographic examination of unerupted teeth may improve early detection and treatment of PEIR defects in children.

While thalassaemia major may not be associated with the occurrence of either vascular canals or prominent antegonial notch, spiky-shaped and short roots, taurodonts, attenuated lamina dura, enlarged bone marrow spaces, small maxillary sinuses, absence of inferior alveolar canal and thin cortex of the mandible were found to be reliable diagnostic criteria for thalassaemia major.

Aim The aim of this study was to evaluate the dental development of patients with â-thalassemia major and to compare it with unaffected children. Methods and Materials Panoramic radiographs of 44 thalassemic patients taken before the age of 16 years were examined. The subjects consisted of 29 males and 15 females ranging in age from 4.9 to 15.7 (mean = 10.8±2.9) years and 44 controls matched for age and sex. The seven left mandibular permanent teeth, second molar to central incisor, were rated on an eight stage scale using the methods described by Demirjian et al.10 The stage of each tooth was converted to the corresponding numeric value and then all values were added to obtain a dental maturity score which corresponded to a dental age. Dental and chronologic ages were compared using a paired t-test. The relationship between the chronologic age and the amount of delay was also determined. Results Thirty-nine patients had a delay in the development of their dentition. The mean developmental dental delay was found to be 1.01 years (p<0.05). The range in delay was from 0.1 to 2.9 years. There was no significant difference between the mean chronologic and dental age of the control group (p> 0.05). The amount of delay in dental development increases as the patient.s age increased (p<0.05). Males were found to have a greater delay (mean 1.16 years) than females (mean delay 0.73 years), but this difference was not statistically significant. Conclusions The delay in dental development in β-thalassemia major varied according to the patient.s age. This positive correlation parallels the general growth of thalassemic children. Citation Hazza.a AM, Al-Jamal G. Dental Development in Subjects with Thalassemia Major. J Contemp Dent Pract 2006 September;(7)4:063-070.

No abstract

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.

CLP patients should be considered to have unfavorable C/R ratios, which could be the result of short root lengths for some teeth.

Objectives: To evaluate the accuracy of Demirjian method in estimating the chronological age of male and female Jordanian children and to establish a new dental age curve if the Demirjian method was not found to be accurate. Methods: Orthopantomograms (OPTs) of 1374 Caucasian Jordanian children (684 females and 690 males) aged 4 to16 years were selected and the dental age was determined by Demirjian method. The chronological ages of the children were obtained by subtracting their birthdates from the date of taking the radiograph. The OPTs were obtained from Archives of Dental Teaching Clinics /XXX and other private orthodontic practices in Irbid and Amman. Results: Demirjian method overestimated chronological age in female and male subjects aged 4 to 8 years. Afterwards, the method underestimated chronological age in females aged 9-11 years and 14-16 years. In male subjects, chronological age was underestimated in subjects aged 9-12 years and 15-16 years. New the dental age curves for Jordanian females and males were constructed. The constants for the quadratic model for the new curves were (b0=-25.341, b1=17.557, b2=-0.623) for females and (b0=-29.809, b1=17.396, b2=-0.595) for males. Conclusion: Demirjian method overestimated the chronological age of Jordanians below the age of 8 years and underestimated the age of Jordanians above 8 years. A new DA standard for Jordanian children was developed and tested for accuracy.

In MDS, the bone marrow produces abnormal, immature blood cells called blast cells. Imprecise, in half of pediatric MDS, blast count is normal. A retrospective observational study was conducted to review the outcome of our HCT in pediatric patients with MDS. Record of 35 MDS patients after BMT, 1993-2016, were reviewed. Median age at transplant was 4 yrs (0.8-14.8) and median time to transplant from diagnosis 8.1 (2.3-102.5) months. TRM was 17.1% (6); [low risk (LR) = 5 (19.2%) and high risk (HR) = 1 (11.1%)] MDS group succumbed within first 100 days. The rest were fully engrafted; [low risk = 21 (72.4%) and high risk = 8 (27.6%)]. Primary and secondary graft failure was observed in one patient each (2.9%). VOD was seen in 2 patients (5.7%) and 5 (14.3%) had hemorrhagic cystitis. With a median follow-up of 112.4 months and 12 events of mortality, 3-years OS was 68.1% ± 8.0%. No significant risk factor including age, time to transplant, disease risk group, gender, conditioning regimen, source of stem cells, or a GvHD through uni- or multi-variable analyses were found to be associated with OS. Bu/Cy/±ATG conditioning regimen showed a trend of superiority for OS and EFS in our small series. The relapse incidence in our cohort was 11.5% in LR MDS.