METHODS: A sample of 158 preschool children with ECC awaiting dental treatment under GA was recruited over an 8-month period. Parents self-completed the Malay-ECOHIS before and 4 weeks after their child's dental treatment. At 4 weeks follow-up, parents also responded to a global health transition judgement item. Data were analysed using independent and paired samples t tests, ANOVA and Pearson correlation coefficients.
RESULTS: The response rate was 87.3%. The final sample comprised 76 male (55.1%) and 62 female (44.9%) preschool children with mean age of 4.5 (SD = 1.0) years. Following treatment, there were significant reductions in mean scores for total Malay-ECOHIS, child impact section (CIS), family impact section (FIS) and all domains, respectively (P child's oral condition to be "a little improved" while 37.7% reported it to be "much improved" following treatment, with Malay-ECOHIS mean change scores of 6.7 (ES = +1.1) and 9.6 (ES = +1.2), respectively. There was an observed gradient in the Malay-ECOHIS change scores and ES in relation to parents' perception of their child's oral health improvement after treatment, supporting the responsiveness of the measure. The Malay-ECOHIS MID was found to be 7 scale points.
CONCLUSION: The Malay-ECOHIS is empirically shown to be sensitive and responsiveness to dental treatment of ECC under GA.
DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key terms "Gianotti-Crosti syndrome" OR "papular acrodermatitis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results.
RESULTS: The eruption of Gianotti-Crosti syndrome is found predominantly on the cheeks, extensor surfaces of the extremities, and buttocks. There is a sparing of antecubital and popliteal fossae as well as palms, soles, and mucosal surfaces. Although often asymptomatic, the lesions may be mildly to moderately pruritic. Gianotti-Crosti syndrome is most common in children between 1 and 6 years of age. The Epstein-Barr virus and the hepatitis B virus are the most common pathogens associated with Gianotti-Crosti syndrome. No treatment for Gianotti-Crosti syndrome is necessary because it is self-limited. In an era of vaccine hesitancy and refusal, Gianotti-Crosti syndrome may be important to mention to parents, because it can occur and trigger alarmism.
CONCLUSIONS: Gianotti-Crosti syndrome is mainly a disease of early childhood, characterized by an acute onset of a papular or papulovesicular eruption with a symmetrical distribution. With the advent of more universal vaccination against hepatitis B virus, Epstein-Barr virus has become the most common etiologic agent of Gianotti-Crosti syndrome. Few cases of post-vaccination Gianotti-Crosti syndrome have been reported. Currently, the emphasis should be placed on its self-limiting attribution.
METHODS: The study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account.
RESULTS: We excluded 88 (7%) patients because of missing data, protocol violation, or alternative diagnosis. Patients from Bangladesh (n = 189, 15%) were described separately because 83% were not treated. IV immunoglobulin (IVIg), plasma exchange (PE), or other immunotherapy was provided in 941 (92%) of the remaining 1,023 patients, including patients with severe GBS (724/743, 97%), mild GBS (126/168, 75%), Miller Fisher syndrome (53/70, 76%), and other variants (33/40, 83%). Of 235 (32%) patients who did not improve after their initial treatment, 82 (35%) received a second immune modulatory treatment. A treatment-related fluctuation was observed in 53 (5%) of 1,023 patients, of whom 36 (68%) were re-treated with IVIg or PE.
CONCLUSIONS: In current practice, patients with mild and variant forms of GBS, or with treatment-related fluctuations and treatment failures, are frequently treated, even in absence of trial data to support this choice. The variability in treatment practice can be explained in part by the lack of evidence and guidelines for effective treatment in these situations.
METHODS: A cross-sectional HRQoL survey of Malaysian children with TDT was conducted using the PedsQL™ 4.0 Generic Core Scales. Patients with non-transfusion dependent thalassemia and other haemoglobinopathies were excluded. Parent-proxy and self-reported HRQoL scores were obtained using a multi-stage convenient sampling. The relationship between HRQoL scores and demographic factors were tested using association, correlation and regression analysis.
RESULTS: A total of 368 patients were recruited. The mean (SD) Total Summary Score (TSS) was 80.12(13.87). Predictors for a lower TSS was an increasing age group and the use of dual chelating agents (R2 = 0.057, F (4, 359) = 5.40, p =
METHODS: A group of 33 healthy children, aged from 5 years 9 months-12 years 4 months (mean ± SD = 8.83 ± 1.92 years), was recruited. Their otolith saccular function was assessed using 750 Hz tone burst for cVEMPs (with ER3A insert phone), while their utricular function was assessed using Brüel & Kjaer Mini-shaker Type 4810 (Naerum, Denmark) for oVEMPs.
RESULTS: For cVEMPs, the mean value of P13 latency, N23 latency, P13-N23 interamplitude and asymmetry ratio were 12.62 ± 1.38 ms, 19.85 ± 1.95 ms, 92.47 ± 50.35 μV and 14.03 ± 9.75%, respectively. For oVEMPs, the mean value of N10 latency, P15 latency, N10-P15 interamplitude and asymmetry ratio were 9.23 ± 1.07 ms, 14.41 ± 1.04 ms, 10.32 ± 5.65 μV and 15.84 ± 11.49%, respectively. Two-way ANOVA analysis found that ear laterality and gender had no significant effect on all cVEMPs and oVEMPs parameters. No significant correlation was found between age and all VEMPs parameters.
CONCLUSIONS: The normative data for cVEMPs and oVEMPs obtained in this study can be used as a guide by health professionals to assess saccular and utricular functions among children age from 5 to 12 years of age.
METHODS: A total of 590 preschoolers, comprising 317 boys and 273 girls were included. Pre-pilot parental questionnaires were used to assess diet, physical activity (PA) and sedentary behaviour practices and anthropometry was assessed in preschoolers and their parents.
RESULTS: Multiple logistic regression analyses showed that preschoolers with more frequent weekly intake of snacks [OR 2.7; 95% CI, 1.6-4.4; p Preschoolers with higher daily fruit and vegetable intake had lower SSB intake [OR 0.4; 95% CI, 0.2-0.8; p = 0.011]. A positive association of higher weekly vigorous PA [OR 2.0; 95% CI, 1.1-3.7; p = 0.030] and daily screen-based practices [OR 2.0; 95% CI, 1.2-3.6; p preschoolers. Continued effort is required to encourage healthier beverage choices, as well as healthy diet and active lifestyle practices among children during the critical early years of growth and development.
METHODS: Twenty-five typically developing children and 25 children with Down syndrome aged between 12 and 36 months were involved in this study. They were recruited from an early intervention center and various kindergartens from the West Coast of Peninsular Malaysia. Their play skills were assessed using the Symbolic Play Test Second Edition, and information about their vocabulary was obtained through the MacArthur Bates Communicative Development Inventories that was filled out by their parents.
RESULTS: There was a significant difference in the vocabulary and symbolic play scores of children with Down syndrome compared with typically developing children. There was also a positive correlation between symbolic play scores and receptive and expressive vocabulary scores for both groups of children.
CONCLUSION: When providing intervention, speech-language pathologists need to promote the development of symbolic play in addition to language, given the association between the two. They should also look into introducing an augmentative and alternative communication system to the children who demonstrate age-appropriate symbolic play skills but have trouble with symbolic language production.
Materials and Methods: The data were obtained from the Kuwait National Primary Immunodeficiency Disorders Registry during the period of 2004-2020.
Results: A total of 313 pediatric cases of IEI, 71% diagnosed at molecular level, were registered with a cumulative follow-up period of 29,734 months. Skin manifestations were seen in 40.3% of the patients, and they were among the presenting manifestations in 33%. Patients with skin manifestations were older at both onset and diagnosis ages of IEI symptoms, but this was statistically significant for the latter only. The diagnosis delay was significantly longer in patients with skin manifestations. There was a statistically significant association between having skin manifestations and IEI category, being more common in patients with complement deficiencies, combined immunodeficiencies, and diseases of immune dysregulation. There was no statistically significant association between having skin manifestations and both gender and survival. Skin infections were the most frequent manifestations followed by eczema and autoimmune associations. Among IEI with more than 10 cases, skin lesions were a consistent finding in dedicator of cytokinesis 8 (DOCK8) deficiency, hyper IgE syndrome, ataxia-telangiectasia, and recombination activation gene (RAG)1 deficiency.
Conclusions: Skin manifestations are common in IEI patients, and they had significant diagnosis delay and referral to specialists. Improvement of awareness about IEI is needed among pediatricians and dermatologists.
CASE PRESENTATION: A 5-year-old Somalian boy with no known medical illness presented with progressive nasal blockage associated with clear nasal discharge and intermittent spontaneous epistaxis for three months. CT paranasal sinus and neck region revealed poorly enhancing expansile mass in the right maxillary sinus with areas of necrosis within. Initial radiological differential diagnoses were lymphoma and rhabdomyosarcoma. The mass was biopsied and histologically showed diffuse sheets of small round blue cells that was positive to CD99, NSE and vimentin. The muscle and lymphoid markers were negative. Fluorescence in-situ hybridisation (FISH) study revealed the presence of EWSR1 gene rearrangement thus diagnosis of ES was rendered.
CONCLUSIONS: ES of sinonasal tract is a rare entity and its pathological features significantly overlap with others small round blue cells tumour. Demonstration of EWSR1 gene translocation is recommended for the diagnosis of ES particularly at uncommon sites.