Aplastic Anemia in Triple X Syndrome.

Triple X syndrome is the most common sex chromosome aneuploidies (SCA) in females. Still, it is underdiagnosed because patients are usually without clear dysmorphism, and the syndrome is not associated with any significant congenital anomalies. We are reporting a case of a 5-year-old girl who presented with aplastic anemia, confirmed by a bone marrow aspiration and biopsy. Her complete workup showed that she has three copies of chromosome X, which, given the diagnosis of triple X syndrome, requires a supportive treatment but not a bone marrow transplant. Few cases of aplastic anemia with sex chromosome abnormalities have been reported. We are reviewing the triple X syndrome in different aspects of the presentation.

Oral microbiota analyses of Saudi sickle cell anemics with dental caries.

OBJECTIVES: The objectives of this study were to identify the composition of oral microbiota in a cohort of patients with sickle cell anemia (SCA) and a high mean number of decayed, missing, and filled permanent teeth (DMFT) and compare it to a cohort of patients with SCA and a low number of DMFT and elucidate the effect of fetal haemoglobin levels on the oral microbiota composition. METHODS: Patients who had been diagnosed with SCA, who were homozygous for sickling ß-globin mutation (ßS/ßS), who had Arab-Indian haplotype, and who ranged in age from 5 to 12 years were included in this study. Oral saliva from each participant (n = 100) was collected in GeneFiX™ Saliva DNA Microbiome Collection tube and DNA was extracted using GeneFiX™ DNA Isolation Kits. The composition of oral 16S rRNA from patients with SCA and high dental caries (n = 27, DMFT ≥5) and low dental caries (n = 73, DMFT ≤4) was analysed. Sequencing was performed on an Ion Personal Genome Machine using, Ion PGM Hi-Q view Sequencing 400-bp kit. RESULTS: We observed an overall increase in abundance of Proteobacteria, Chloroflexi, and Bacteroidetes in the high DMFT index group compared to those with a low DMFT index. In addition, there was an overall increased abundance of microbiota from Proteobacteria, Fusobacteria, Firmicutes, and Bacteroidetes in the patients with SCA with low fetal haemoglobin compared to those with high fetal haemoglobin (P < .05). Enterobacteriaceae species were the most significant abundant species of bacteria found in both the high DMFT index group and low fetal haemoglobin cohort (P < .05). CONCLUSIONS: Our data indicate that SCA in Saudi patients with high DMFT have a higher predominance of pathogenic bacteria compared to those with low DMFT. Furthermore, SCA in Saudi patients with low fetal haemoglobin have a higher predominance of pathogenic bacteria compared to those with higher fetal haemoglobin.

Prevalence of Dental Fear and Anxiety and Its Triggering Factors in the Dental Office among School-going Children in Al Ahsa.

AIMS AND OBJECTIVES: To estimate the prevalence of dental fear and anxiety (DFA) among school-going children in Al Ahsa.To identify the factors that trigger DFA in the dental office. MATERIALS AND METHODS: A cross-sectional questionnaire-based study was conducted. Eight hundred and sixteen, 7-12-year-old children studying in government and private schools spread across the city of Al-Ahsa, Saudi Arabia, participated in the study. A self-reported questionnaire with 15 close-ended questions pertaining to DFA in logical order was distributed to all the participants. Each question had three options as responses with images. The children were asked to choose the option that best fitted their response. RESULTS AND CONCLUSION: The prevalence of DFA among boys and girls was found to be 50.4 and 71.28%, respectively. The majority of the boys studying in government schools reported DFA when informed about the treatment in advance, while most of those studying in private schools reported DFA at the sight of the dental chair and dental injections, while walking into the clinic, in the waiting room, when informed about the treatment in advance and their tooth being drilled. On the contrary, the responses were very similar among girls irrespective of their school. Most of the boys and girls did not experience any DFA when their parents were allowed to accompany them. Hence, a tailor-made approach for the management of the child in the dental office is warranted. CLINICAL SIGNIFICANCE: A tailor-made approach for the management of DFA in the dental office plays a significant role in successful treatment. HOW TO CITE THIS ARTICLE: Alshuaibi AF, Aldarwish M, Almulhim AN, et a l. Prevalence of Dental Fear and Anxiety and Its Triggering Factors in the Dental Office among School-going Children in Al Ahsa. Int J Clin Pediatr Dent 2021;14(2):286-292.