Vaccination Status in Children with Chronic Diseases: Are They Up-to-Date for Mandatory and Specific Vaccines?

OBJECTIVE: The aim of this study is to investigate the immunization coverage for mandatory and specific vaccines in a group of children with chronic diseases. MATERIALS AND METHODS: Children with chronic diseases aged 6 months to 18 years who were followed up by outpatient subspecialty clinics of a tertiary hospital were enrolled. Children who were up-to-date and who were under-vaccinated were compared with respect to demographic characteristics, parental educational status, healthcare providers' attitudes toward vaccination, age at the time of diagnosis, and duration of follow-up. RESULTS: A total of 366 patients with variable chronic diseases were enrolled. Of these, 84.7% were up-to-date for the mandatory vaccines. This rate was 99.5% for the primary series of diphtheria-tetanus-acellular pertussis and 98.9% for the first dose of measles-mumps-rubella vaccines. Vaccination coverage for specific vaccines was low (13.9% for influenza and 55% for conjugated pneumococcal vaccine). Being older at the time of diagnosis increased the likelihood of being up-to-date for mandatory vaccines by 1.1 times, while being followed up from multiple subspecialty outpatient clinics and attendance to private doctors' clinics for vaccination increased the likelihood of being up-to-date for specific vaccines by 19.1 and 6.4 times, respectively. CONCLUSION: In this study, immunization coverage for mandatory vaccines was comparable to that of the general population. However, vaccination coverage was low for specific vaccines. Therefore, efforts of prioritization of pediatric immunizations and raising awareness among healthcare providers about the impact of medical recommendations for specific vaccines among children with chronic diseases can help to improve vaccination rates.

The Effect of Vitamin D Prophylaxis on 25-OH Vitamin D Levels in Children.

BACKGROUND: Vitamin D deficiency is a major public health problem. The aim of our study was to determine serum 25-hydroxyvitamin D levels among healthy children aged 3-36 months in a setting where vitamin D prophylaxis is a national policy for infants during the first year of life and among pregnant women. METHODS: A total of 190 healthy children with a mean age of 15.9 ± 10.4 months were prospectively enrolled. RESULTS: The mean 25-hydroxyvitamin D level of children was 38.1 ± 16.2 ng/mL. 25 Hydroxyvitamin D level was ≥20 ng/mL in 87.4% of children while it was between 12 and 19 ng/mL in 10.5% and <12 ng/mL in 2.1% of the children. Children who were on vitamin D prophylaxis were found to have significantly higher 25-hydroxyvitamin D levels than those who were not on prophylaxis (41.6 ± 17.6 vs 33.6 ± 13.1 ng/mL; P = .001). None of the children >1 year of age who were on prophylaxis had 25 hydroxyvitamin D levels <20 ng/mL. No significant difference in 25-hydroxyvitamin D levels was found between children who were receiving different vitamin D doses (400 IU vs >400 IU). Analysis of covariance revealed that vitamin D prophylaxis and vitamin D supplementation of the mother during lactation had significant effects on 25-hydroxyvitamin D levels (P = .034 and P = .009, respectively). CONCLUSION: Although vitamin D prophylaxis at a dose of 400 IU seems to be sufficient to prevent vitamin D deficiency, we suggest that continuing vitamin D supplementation beyond 1 year of age with supplementation of pregnant and especially lactating mothers could have an impact on a replete vitamin D status among infants.

Comparison of Different Iron Preparations in the Prophylaxis of Iron-deficiency Anemia.

We compared the efficacy of ferrous sulfate (divalent) and ferric polymaltose (trivalent) compounds for the prophylaxis of iron-deficiency anemia (IDA). Study infants included exclusively breast milk-fed term infants. Subjects were divided randomly into 2 groups at 4 months of age and group 1 (n=56) received divalent and group 2 (n=56) received trivalent iron (Fe) preparation at a dose of 2 mg/kg/d for 5 months. At 9 months of age, after a 5-month prophylaxis, a significant increase was observed in hemoglobin (Hb), hematocrit, serum Fe levels, and transferrin saturation in both groups. However, group 1 had significantly higher Hb, hematocrit, mean corpuscular volume, Fe, and transferrin saturation than group 2 (11.7±0.6 g/dL, 34.6%±1.7%, 76.2±2.9 fL, 55.5±1.8 mcg, 20.8±3.9 g/L, respectively in group 1 vs. 11.3±0.5 g/dL, 33.5%±1.5%, 74.7±3.2 fL, 42.5±1.8 mcg, 14.1±7.5 g/L, respectively in group 2). No significant difference was found in ferritin values between the groups. Fe deficiency was found in 17 (30.3%) of the subjects in group 1, and 23 (41%) of subjects in group 2 whereas 5 (8.9%) subjects had IDA in group 1 and 12 (12%) in group 2 which were insignificant between groups. No significant difference was found with regard to side effects between 2 Fe preparations. Although divalent Fe therapy led to a higher Hb and serum Fe level, both ferrous and ferric Fe preparations were effective for prophylactic use in the prevention of Fe deficiency and IDA with comparable side effects.

Regression of Cardiac Rhabdomyomas in a Neonate after Everolimus Treatment.

Cardiac rhabdomyoma often shows spontaneous regression and usually requires only close follow-up. However, patients with symptomatic inoperable rhabdomyomas may be candidates for everolimus treatment. Our patient had multiple inoperable cardiac rhabdomyomas causing serious left ventricle outflow-tract obstruction that showed a dramatic reduction in the size after everolimus therapy, a mammalian target of rapamycin (mTOR) inhibitor. After discontinuation of therapy, an increase in the diameter of masses occurred and everolimus was restarted. After 6 months of treatment, rhabdomyomas decreased in size and therapy was stopped. In conclusion, everolimus could be a possible novel therapy for neonates with clinically significant rhabdomyomas.

Spondyloepimetaphyseal dysplasia with joint laxity, leptodactylic type: longitudinal observation of radiographic findings in a child heterozygous for a KIF22 mutation.

Spondyloepimetaphyseal dysplasia with joint laxity, leptodactylic type (SEMDJL2), is a rare disorder due to a KIF22 gene mutation and characterized by postnatal short stature, midface hypoplasia and generalized ligamentous laxity. Radiologic hallmark includes severe involvement of the epiphyses and the slender appearance of the metacarpals and phalanges. The aim of the study was to evaluate radiologic findings of SEMDJL2 in a child followed from age 2 years 9 months to 11 years. Using whole-exome sequencing, we identified a single nucleotide de novo p.Pro148Leu mutation in the KIF22 gene. The child had midface hypoplasia, short stature, hip dislocation and generalized laxity of the joints in the first examination. Knee subluxation and bilateral severe genu valgum became prominent after 3.5 years of age. Short stature became evident gradually with increasing age, and height was 3.6 standard deviations below the mean for age. Small epiphyses with delayed maturation and metaphyseal vertical striations at the distal metaphysis of the femur were observed on initial radiographs. However, the slender metacarpals and proximal phalanges and progressive epiphyseal dysplasia with small and flattened epiphyses on both wrists and knees became more prominent after 7 years of age. In conclusion, we observed that typical radiologic findings became apparent after early childhood.

Tetanus and diphtheria immunity among term and preterm infant-mother pairs in Turkey, a country where maternal and neonatal tetanus have recently been eliminated.

UNLABELLED: The aim of our study was to investigate the anti-tetanus and anti-diphtheria antibody titres and the placental transfer of these antibodies in a group of vaccinated and unvaccinated mothers and their term or preterm offsprings. Anti-tetanus and anti-diphtheria toxoid IgG antibodies were measured quantitatively by ELISA in 91 infant-mother pairs. Protective concentrations of anti-tetanus and anti-diphtheria were found in 58.3 and 50% of mothers in the unvaccinated group and 94.5 and 85.5% of the mothers in the vaccinated group. Protective concentrations were found in 63.9 and 50% of cord samples, respectively, in the unvaccinated group and in 96.4 and 85.5% of cord samples, respectively, in the vaccinated group (p = 0.0001). There were no differences in the maternal and cord geometric mean concentrations (GMCs) of anti-toxoid antibodies between those who received two doses or one dose of Td. The GMCs of maternal and cord anti-tetanus and anti-diphtheria were statistically similar between preterm and term groups. Placental transfer ratios (TR) for anti-tetanus and anti-diphtheria were 175 and 150%, respectively, in the preterm group and 213 and 178%, respectively, in the term group. There was a strong correlation between maternal and cord anti-toxoid antibody levels. Maternal vaccination was the only predictor of having protective concentrations of anti-toxoid antibodies in cord blood. CONCLUSIONS: Vaccinating pregnant women with at least one dose of Td would confer protection for both the term and preterm infant-mother pairs. Therefore, health personnel caring for pregnant women have the responsibility to emphasize the importance of Td vaccination to avoid missed immunization opportunities.

Primary hypertrophic osteoarthropathy caused by homozygous deletion in HPGD gene in a family: changing clinical and radiological findings with long-term follow-up.

Autosomal recessive primary hypertrophic osteoarthropathy1 (PHOAR1) is characterized by delayed closure of the fontanels, digital clubbing, arthropathy and periostosis. Homozygous mutations in hydroxyprostaglandin dehydrogenase (HPGD) gene are the underlying pathology of PHOAR1. The aim of this study was to analyze the HPGD gene and the changing clinical and radiological findings with advancing age of two siblings with the diagnosis of PHOAR1. A novel 2-bp homozygous deletion was found in exon 3 (c.310-311delCT) of HPGD gene in the patients. Clinical and radiological findings of the siblings were evaluated between 4 months and 8 years and 6 months of age. The painful swelling and sweating of the hands and feet, cranial ossification defect and expanded diaphyses were evident at infantile period and gradually showed improvement until 4 years of age. After the age of 4 years, digital clubbing and swelling of knees persisted, palmoplantar hyperkeratosis developed and acro-osteolysis became evident on hand radiographs. In conclusion, we suggest that the clinical findings of the patients with PHOAR1 should be classified in two periods as early and late childhood. We also observed that there was intrafamilial variability of clinical findings.

The utility of serial plasma sE-selectin measurements in the prediction of retinopathy of prematurity in premature infants.

BACKGROUND: sE-selectin has recently been suggested as a surrogate marker for prediction of ROP development. AIMS: The possible role of serial plasma sE-selectin measurements in early prediction and diagnosis of ROP was evaluated. STUDY DESIGN: Prospective observational study SUBJECTS: Forty six preterm infants aged <34weeks of gestation and weighing <1500 g were enrolled. Of these, 26 constituted the ROP group and 20 constituted the no-ROP group. sE-selectin levels were measured serially in blood samples on the 1st day and on 14th and 28th postnatal days. OUTCOME MEASURES: The primary outcome measure was to evaluate the role of sE-selectin concentrations in prediction of ROP. RESULTS: The mean gestational age and birth weight were significantly lower in the ROP group. The mean sE-selectin concentrations in ROP group were significantly greater than those in no-ROP group at each time point (1st, 14th and 28th days of postnatal life). A receiver operating characteristic (ROC) analysis showed that at a plasma concentration of ≥86ng/mL on the 1st postnatal day, sE-selectin had a sensitivity of 100% and a specificity of 94.1% with a positive predictive value of 96.3% and a negative predictive value of 100%. Plasma sE-selectin concentrations were significantly greater in infants who developed ROP in three different time points. CONCLUSIONS: This study shows for the first time that measurement of plasma sE-selectin concentrations as early as the first day of life might help identify preterm infants at risk of ROP.

Prevalence of Prader-Willi syndrome among infants with hypotonia.

OBJECTIVE: To investigate the prevalence of Prader-Willi syndrome (PWS) in infants with hypotonia between the ages of 0 and 2 years. STUDY DESIGN: Karyotyping studies were performed in all infants with hypotonia. The study group was composed of infants with hypotonia for whom the karyotyping was found to be normal. Fluorescence in situ hybridization and methylation analysis were performed simultaneously in the study group. Molecular studies for uniparental disomy were undertaken in the patients without deletions with an abnormal methylation pattern. RESULTS: Sixty-five infants with hypotonia with a mean age of 8 months were enrolled. A deletion was detected in 6 patients by fluorescence in situ hybridization analysis. Only 1 patient had no deletion but had an abnormal methylation pattern. A maternal uniparental disomy was observed in this patient. PWS was diagnosed in 10.7 % (7/65) of the infants with hypotonia. CONCLUSION: The prevalence of PWS syndrome is high among infants with hypotonia. PWS should be considered by pediatricians and neonatologists in the differential diagnosis of all newborns with hypotonia. Early diagnosis of PWS is important for the management of these patients.

Hepatitis B vaccination in juvenile systemic lupus erythematosus.

OBJECTIVES: In this study, we examined the antibody responses after recombinant hepatitis B vaccine in juvenile SLE patients and whether antibody levels were affected by immunosuppressive therapy. METHODS: This study consisted of 64 juvenile SLE patients and 24 healthy controls. We evaluated HBsAg, Anti-HBs and Anti-HbcIgG titers in SLE patients. 24 patients (37%) were non-immunised, 39 patients were immunised (61%) and 1 patient (1.5%) was chronic hepatitis B carrier. Of the 24 non-immunised patients, 3 had active disease (SLEDAI>10) and 1 was being treated for tuberculosis infection so they were not included in the vaccination program. Twenty non-immunised SLE patients were given 3 dose recombinant hepatitis B vaccine doses at 0,1,6 months. AntiHBs antibody titer >10 IU/ml one month after the last dose of vaccine was accepted as seroconversion. RESULTS: After 3 doses of vaccination, 16 (80%) of SLE patients and all of the healthy controls had seroconversion. Since two patients had SLEDAI score >10 after the first 2 doses of vaccine and one patient had SLEDAI score >10 after the first dose, these patients were given only two doses of hepatitis B vaccine. These patients had already seroconverted. One patient had exacerbation of the disease one month after the third dose of the vaccine. Protective antibody responses were statistically insignificant between the two groups (p=0.49). Geometric mean antibody titers of SLE patients were lower than those of the healthy controls. Adequate antibody response was not affected by immunosuppressive treatment as prednisone, azathioprine, and hydroxychloroquine. CONCLUSIONS: Juvenile SLE patients could reach an adequate antibody response after recombinant hepatitis B vaccination and this response is not affected by immunosuppressive treatment.