Vaccination practices among physicians and their children

The purpose of this study was to identify vaccination patterns of both general pediatricians and subspecialists with regards to their own children and projected progeny. A 14 question survey was sent randomly to 1000 members of the Academy of Pediatrics in 2009. Two categories of questions included 1) how physicians with children vaccinated them in the past, and 2) how all respondents would vaccinate a child in 2009. A comparison was made between the answers of general and specialty pediatricians. 582 valid questionnaires were received (58.2% response rate) of which 431 were general pediatricians and 151 subspecialists. No statistical difference was found between general and specialty pediatricians on how they vaccinated their children up until 2009 (95% vs 93%). When asked about vaccinating a future child, a significant proportion of respondents would deviate from CDC guidelines, specialists more than general pediatricians (21% vs 9%). Generalists were more likely to give a future child Hepatitis A (OR: 3.6;95% CI 1.3 - 10.4), Rotavirus (OR: 2.2;95% CI 1.1 - 4.4), Meningococcal (OR: 9.9;95% CI 3.3-29.9), and influenza (OR: 5.4;95% CI 1.1 - 26.7) vaccines. Specialists were more likely to postpone MMR vaccinetion (OR: 4.4 95% CI 2.3 - 8.6). Safety was listed by both groups as the most common reason for altering the recommended immunization schedule. Until 2009, general pediatricians and pediatric specialists have largely adhered to ACIP recommendations, but due to vaccine safety and other concerns, both groups, albeit a higher percentage of specialists, reported greater numbers willing to diverge from these recommendations.

Neuroplastin in Ca^(2+)signal regulation and plasticity of glutamatergic synapses

The main function of neurons is information transmission in the form of action potentials.To fulfill this duty,neurons are connected functionally with each other via synapses,the microscopic structures where specialized molecular machinery is strategically placed to release and receive neurotransmitters and to generate and extinguish calcium(Ca^(2+))signals.These synaptic molecular components are highly dynamic and they influence each other to confer structural and functional adaptability(plasticity)to neuronal communication(Biederer et al.,2017).

Registration of the Seated Condylar Position (SCP/CR): Part II: Technique: The Two-Piece Wax Bite Registration

The purpose of this paper is to present the technique for registration of the Seated Condylar Position (SCP)/Centric Relation (CR) position of the condyles: a two-piece wax bite registration with deprogramming and no mandibular manipulation.

Registration of the Seated Condylar Position (SCP/CR): Part I: Rationale

The purpose of this paper is to present the rationale for registration of the Seated Condylar Position (SCP)/Centric Relation (CR) position of the condyles.

Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management

Achalasia is an esophageal motility disorder character-ized by failure of lower esophageal sphincter(LES) relaxation and is rare in children. The most common symptoms are vomiting, dysphagia, regurgitation, and weight loss. Definitive diagnosis is made with barium swallow study and esophageal manometry. In adults, endoscopic biopsy is recommended to exclude malig-nancy however; it is not as often indicated in children. Medical management often fails resulting in recurrent symptoms and the ultimate definitive treatment is sur-gical. Laparoscopic Heller myotomy with or without an anti-reflux procedure is the treatment of choice and has become standard of care for children with achala-sia. Peroral endoscopic myotomy is a novel therapy uti-lized with increasing frequency for achalasia treatment in adults. More experience is needed to determine the safety, efficacy, and feasibility of peroral endoscopic myotomy in children.

Buddy Study: Partners for better health in adolescents with type 2 diabetes

AIM: To investigate whether assigning young, healthy and motivated lay volunteer partners("buddies") to adolescents with type 2 diabetes improves hemoglobin A1c(HbA 1c). METHODS: Adolescents with type 2 diabetes were randomized to partnering with a "buddy" or to conventional treatment. During the initial screening visit, which coincided with a routine outpatient diabetes clinic visit, patients with type 2 diabetes underwent a physical examination, detailed medical history, laboratory measurement of HbA 1c, and completed two questionnaires(Pediatric Quality of Life Inventory and Children's Depression Inventory) to assess their overall quality of life and the presence of depressive symptoms. Patients were then randomized to the intervention(the buddy system) or conventional treatment(standard care). All patients were scheduled to return for followup at 3- and 6-mo after their initial visit. Hb A1 c was determined at all visits(i.e., at screening and at the 3- and 6-mo follow-up visits) and quality of life and depressive symptoms were evaluated at the screening visit and were reassessed at the 6-mo visit. RESULTS: Ten adolescents, recruited from a pool of approximately 200 adolescents, enrolled over a twoyear time period, leading to premature termination of the study. In contrast, we easily recruited motivated lay volunteers. We found no change in HbA 1c from the initial to the 6-mo visit in either group, yet our small sample size limited systematic assessment of this outcome. Participants repeatedly missed clinic appointments, failed to conduct self-glucose-monitoring and rarely brought their glucometers to clinic visits. Total quality of life scores(72.6 ± 6.06) at screening were similar to previously reported scores in adolescents with type 2 diabetes(75.7 ± 15.0) and lower than scores reported in normal-weight(81.2 ± 0.9), overweight(83.5 ± 1.8), and obese youths without diabetes(78.5 ± 1.8) or in adolescents with type 1 diabetes(80.5 ± 13.1). Among adolescents who returned for their 6-mo visit, there were no differences in total quality of life scores(70.2 ± 9.18) between screening and follow-up.CONCLUSION: Our approach, effective in adults with type 2 diabetes, was unsuccessful among adolescents and emphasizes the need for innovative strategies for diabetes treatment in adolescent patients.

Conotruncal heart malformations in Egypt: An epidemiological study

Conotruncal malformations of the heart are a major category of birth defects. No previous epidemiological studies on these malformations have been carried out in Egypt;therefore our study aimed to describe associations with maternal and infant factors in order to identify possible risk factors. Cases (N = 255) were children up to two years of age whose heart malformations were confirmed by echocardiography;they were enrolled at the Cardiology Department of the Pediatric Hospital of Cairo University. Controls (N = 155) were free of congenital heart malformations, matched to the cases by age, and recruited from outpatients of the same hospital. Mothers of cases (97%) and controls (89%) provided consent to participate in an interview about their medical, familial, and occupational histories. Odds ratios (OR) and exact 95% confidence intervals (CI) assessed the magnitude and statistical significance of case-control differences. Cases were divided into two groups based on the presence of d-transposition of the great arteries (TGA): there were 139 with TGA and 116 with normally-related great arteries (NGA). Maternal diabetes prevalence was elevated in both the TGA (OR = 3.4) and NGA (OR = 5.5) subgroups. Several agricultural factors were associated with increased risk: raising animals (for TGA: OR = 2.4, 95% CI 1.2-4.6), raising poultry (for NGA: OR = 1.8, 95% CI 1.1-3.2), and using chemical rodenticides (for all conotruncal cases: OR = 3.2, 95% CI 1.1-13.2). These results are consistent with previous studies of associations of maternal diabetes and pesticide exposure with conotruncal malformations. Further research is warranted to explore such associations and determine avenues for prevention.

Pott puffy tumor in an unusual location

Pott puffy tumor is almost always associated with an outward extension of suppurative acute frontal sinusitis. Pathogenesis includes osteomyelitis of the frontal bone with local spread of the infection to the outer cortex and termination of the suppurative process in a frontal subgaleal abscess. An unusual ectopic location of Pott puffy tumor is the tempo-parietal subgaleal space, located exterior to the squamous portion of the temporal bone. In this location, Pott puffy tumor is considered a suppurative complication of otomastoiditis. Reported here is a 4-year-old boy who developed amoxicillin-resistant acute otitis media, silent mastoiditis, temporal bone subperiosteal abscess, subgaleal abscess, and an epidural abscess. He had several fenestra of the tegmen mastoideum as a result of osteiitis of the temporal bone, which we believe permitted spread of the oto-mastoiditis to temporal bone, subgaleal space, and epidural space. Surgical specimens obtained from the middle ear and subgaleal abscess after 24-hours of intravenously-administered combination antibiotic therapy did not reveal any aerobic or anaerobic bacterial pathogens. This is only the second such pediatric case that we found during a careful search of the literature.

Profound Hypothermia Secondary to Clobazam Use in Epilepsy: A Novel Association

Clobazam, a 1-5 benzodiazepine, was introduced in the 1970s for the treatment of anxiety and agitation. Antiepileptic properties were recognized, and efficacy in a number of epilepsy syndromes was demonstrated in humans, with good tolerance. Recent reviews are generally favorable, with a relative minimum of medication-related side effects. However, a number of benzodiazepines have been associated with causing hypothermia. To date, this side effect has not been reported with clobazam. We report two cases of profound hypothermia associated with the use of this medication for the treatment of epilepsy. Both children had significant cerebral dysgenesis and were developmentally impaired, but neither had experienced hypothermia before. Temperature dysregulation was resolved with medication withdrawal after an extensive work-up for alternative causes. Hypothermia should be considered as a possible side effect of clobazam, although the exact mechanism is unknown. Appropriate monitoring of temperature is appropriate, and precautions should be offered by caregivers.

Anesthesia for ambulatory surgery in a child with hyposensitivity to pain

Congenital hyposensitivity to pain is a condition with predisposition to injury. In these patients, knowledge regarding anesthetic requirements and complications derives from individual case reports, or small case series. Different categories have been described. In patients with hyposensitivity to pain, preventing and treating anxiety as well as insuring immobilization, avoidance of triggering of autonomic reflexes, and sedation are integral aspects for a safe and adequate anesthetic management.

Siglec genes confer resistance to systemic lupus erythematosus in humans and mice

A recent meta-analysis revealed the contribution of the SIGLEC6 locus to the risk of developing systemic lupus erythematosus(SLE).However,no specific Siglec(sialic acid-binding immunoglobulin-like lectin)genes(Siglecs)have been implicated in the pathogenesis of SLE.Here,we performed in silico analysis of the function of three major protective alleles in the locus and found that these alleles were expression quantitative trait loci that enhanced expression of the adjacent SIGLEC12 gene.These data suggest that SIGLEC12 may protect against the development of SLE in Asian populations.Consistent with human genetic data,we identified two missense mutations in lupus-prone B6.NZMSle1/Sle2/Sle3(Sle1–3)mice in Siglece,which is the murine Siglec with the greatest homology to human SIGLEC12.Since the mutations resulted in reduced binding of Siglec E to splenic cells,we evaluated whether Siglece−/−mice had SLE phenotypes.We found that Siglece−/−mice showed increased autoantibody production,glomerular immune complex deposition and severe renal pathology reminiscent of human SLE nephropathy.Our data demonstrate that the Siglec genes confer resistance to SLE in mice and humans.

Trap1a is an X-linked and cell-intrinsic regulator of thymocyte development

The X-linked Trap1a gene encodes the tumor rejection antigen P1A,which is expressed in fetal tissues and multiple lineages of tumor cells.The function of this gene remains unknown.Using chimeric mice with wild-type(WT)and Trap1a^(−/y)bone marrow,we show that Trap1a^(−/y)donor cells are capable of generating most lineages of hematopoietic cells,with the notable exception of T cells.Deletion of Trap1a selectively arrests T-cell development at double-negative stage 1(DN1,with a CD4^(−)CD8^(−)CD25^(−)CD44^(+)phenotype).Because Trap1a is expressed in Lin^(−)Sca-1^(+)c-Kit^(+)and common lymphoid progenitors but not in immature thymocytes(DN1-DN4),Trap1a mutations affect the differentiation potential of progenitor cells without directly acting on T cells.Despite a similarity in the blockade of DN1 to DN2 transition,the Trap1a^(−/y)DN1 cells have normal expression of c-Kit,in contrast to what was reported in the Notch1^(−/−)DN1.Complementary DNA profiling of Trap1a^(−/y)and WT embryonic stem cells shows that Trap1a does not regulate the Notch pathway.Our data reveal that Trap1a is an X-linked regulator that affects the differentiation potential of progenitor cells into T cells through a Notch-independent mechanism and identify an important function for the Trap1a gene.