Catch-up vaccination and enhanced immunization against hepatitis B, hepatitis A, measles, mumps, rubella and varicella in children with idiopathic thrombocytopenic purpura.

BACKGROUND: Limited data exist about the vaccination of children with idiopathic thrombocytopenic purpura (ITP) against vaccine preventable diseases. This study identified the vaccination status of children with ITP against hepatitis A, hepatitis B, measles, mumps, rubella and varicella, completed the immunization of children with inadequate immunization, re-evaluated post-vaccination antibody titers and identified probable vaccination-related complications. PATIENTS AND METHODS: All of 46 children had chronic ITP were included. Seroconversion of hepatitis A, hepatitis B, varicella, measles, rubella and mumps vaccines was screened. All children with seronegative antibodies against vaccine preventable disease were given a vaccination appointment. Antibody levels were re-measured during a period ranging from 1 to 6 months. Potential complications were detected. RESULTS: There were 46 children with a mean age of 12.25 years. All children had chronic ITP and received intravenous immunoglobulin at least once previously. Considering the vaccination status, 50% (23 children) had vaccinations appropriate for their age, 47.8% (22 children) did not know their vaccination status and 2.2% (1 patient) did not have vaccinations. Seven children (15.2%) were seropositive for all antibody types and the remaining 39 children were scheduled for vaccination. Post-vaccination antibody titers confirmed that all children became seropositive for each disease. There was no complication in any patient. CONCLUSION: Immunization against hepatitis B, hepatitis A, measles, mumps, rubella and varicella is insufficient in a considerable number of children with ITP, Hepatitis B Virus (HBV) and Hepatitis A Virus (HAV) immunization being the most frequently inadequate. After immunization, adequate seroconversion levels were achievable without complications.

Vaccination in children with inherited bleeding disorders: Does the use of plasma an d factor affect the response to the vaccine: An institutional registry.

INTRODUCTION: We aimed to evaluate the vaccine seroconversion of paediatric patients with factor deficiency and to complete the missing vaccines. We also emphasize the importance of variables such as recombinant factor and plasma intake frequency. MATERIAL AND METHODS: This prospective observational study includes children between 2 and 17 age diagnosed with inhered factor deficiency. Seroconversion of hepatitis A, hepatitis B, varicella, measles, rubella and mumps vaccines were screened according to Ministry of Health Vaccination Schedule, it was completed if there were seronegative vaccines and the vaccine seroconversion was examined after vaccination approximately 1-6 months. RESULTS: A total of 61 children were included in the study [mean age 11.2±5.1 years (2-18)], 49 (80.3%) were males. Factor VIII deficiency constituted 60.7%, factor XI 14.8%, factor VII 13.1 %, factor IX 9.8%, factor X 1.6% of the cases. None of the children had clinically important injection site complications such as uncontrollable bleeding or gross hematoma and vaccination does not induce the development of inhibitor. Measles antibody positivity was significantly lower in those who received factor 3 or 4 days times a week compared to those who received it when necessary (p = .049). DISCUSSION AND CONCLUSION: This is the first study evaluating the vaccine conversion patients with inherited factor deficiency receiving plasma and plasma derived factor concentrates. It should be considered that children with haemophilia and their families may have vaccination hesitancy; therefore, follow-up of these patients should include a specified vaccination program to ensure adequate immunization of these patients.

Hemophagocytic Lymphohistiocytosis Associated With Hemolytic Uremic Syndrome in a Child: A Case Report and Systematic Literature Review.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder resulting from excessive activation and nonmalignant proliferation of T-lymphocytes and macrophages. Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Reports pertaining to the association between HLH and HUS are rarely published; however, we report on a 4-year-old boy who was diagnosed with both conditions and treated successfully with high-dose steroid and intravenous immunoglobulin. Differentiating HUS from HLH can be challenging because of their clinical similarities. Therefore, prompt diagnosis and immunosuppressive treatment are essential and life-saving to these patients.

The relation between increased carotid intima-media thickness with fibrin monomers in obese children.

AIM: This study aimed to investigate the association between carotid intima-media changes that play a part in the atherosclerotic process in childhood obesity and fibrin monomers as an important indicator of fibrin plaque. METHODS: This is a cross-sectional study of obese children and non-obese healthy control subjects. Height, weight, body mass index, waist/hip ratio, systolic/diastolic blood pressures were recorded, in addition, biochemistry, haemogram, fibrin monomers and d-dimer were measured in both groups. Right and left common carotid intima-media thicknesses were measured by ultrasonography and mean carotid intima-media thickness was calculated. RESULTS: Obese children (n = 89, 46.1% girls, median age: 12.6 ± 2.3 years) and healthy control group (n = 40, 52.5% girls, median age: 13.2 ± 2.2 years) were comparable in terms of gender, age and puberty stage. Mean carotid intima-media thickness was higher in obese children than the healthy control group (P = .002). There was no difference between the two groups in terms of fibrin monomers and d-dimer levels. In obese children, there was a weak negative correlation between mean carotid intima-media thickness and fibrin monomers (P = .030, r = -0.233). CONCLUSION: In obese children, mean carotid intima-media thickness was determined higher, as an early indicator of atherosclerosis. We want to emphasise that obese children are at risk for cardiovascular disease and should be evaluated in terms of atherosclerosis. This study investigates the relation between increased carotid intima-media thickness and fibrin monomers, in children, the first time in Literature.

Comparison of the Sleep Problems Among Adolescents Who Attempted Suicide and Healthy Adolescents.

The aim of this study was to compare sleep problems among adolescents who attempted suicide and healthy adolescents who never attempted suicide. Adolescents who attempted suicide (study group, n = 103) and healthy adolescents (control group, n = 59) completed a questionnaire prepared by researchers including demographic factors. In addition, the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS) were administered to both groups. The median age was 16 years and 73% were girls, in both groups. The study group had lower rate of attending to school (88.3% vs. 100%; p = 0.001), academic achievement (45.7% vs. 83.1%; p = 0.001), higher rate of smoking (37.9% vs. 13.8%; p = 0.001), socializing problems (31.1% vs. 3.4%; p = 0.001), and appetite changes (57.3% vs. 39.7%; p = 0.032) than controls. The rate of those with PSQI scores 6 or higher was 53.4% in the attempted suicide group and 37.3% in the control group (p = 0.048). Adolescents, classified as sleepy according to the ESS, did not differ significantly between the groups (p = 0.214). Adolescents who attempted suicide had poor sleep quality. It is crucial to examine the kinds of sleep problems adolescents who have attempted suicide have experienced. Among adolescents attending outpatient clinics with poor sleep quality, PSQI can be a useful screening tool. Those with high scores should be evaluated for suicide risk.

Evaluation of Platelet Functions in Patients with Hashimoto's Thyroiditis Versus Healthy Controls: a Cross-Sectional Analysis.

BACKGROUND: To evaluate platelet functions in patients with Hashimoto's thyroiditis (HT) versus healthy controls. METHODS: Seventy-five patients with HT and 29 healthy controls were included in this study. Age, serum levels of thyroid stimulating hormone (TSH), free triiodothyronine (FT3), free thyroxine (FT4), anti-thyroglobulin (anti-Tg) antibody and anti-thyroid peroxidase (anti-TPO) antibody, platelet count, in vitro platelet aggregation and ATP release reaction tests were recorded and compared between HT and control groups. RESULTS: Median (IQR) serum levels for TSH (p = 0.001), anti-TPO (p = 0.001), and anti-Tg (p = 0.001) antibodies were significantly higher, while FT4 levels (p = 0.005) were significantly lower in patients with HT than in controls. Patients had lower levels of ADP-induced platelet aggregation (p = 0.05) and lower ristocetin-induced ATP release activity (p = 0.05) compared to controls. Platelet count was positively correlated with serum FT4 levels (r = 0.27, p < 0.05). Conclusions: We found decreased ADP-induced platelet aggregation and ristocetin-induced platelet release activity as well as a positive correlation of platelet count with FT4 levels in patients with HT. Our findings support the role of thyroid hormone status and autoimmunity in the association between HT and platelet aggregation and secretion functions.

Decreased antioxidant status in migraine patients with brain white matter hyperintensities.

Migraine patients have an increased risk to develop deep white matter hyperintensities (WMH) than the general population. Oxidative stress is believed to play a role in the pathogenesis of migraine. The present study was undertaken to assess oxidant/antioxidant balance of migraineurs with and without WMH. We hypothesized that increased oxidative stress and decreased antioxidant response may play a role in the pathophysiology of WMH in migraineurs. The study included 32 patients in the migraine group and 17 age- and sex-matched healthy subjects without headache in the control group. The migraine group comprised 18 with WMH and 14 without WMH. We evaluated oxidative status with malondialdehyde (MDA) and to determine the activities of antioxidant enzymes: superoxide dismutase, glutathione peroxidase and catalase (CAT) in serum of migraineurs and controls. Comparison of the patient and control groups for oxidative parameters revealed significantly lower level of CAT and higher level of MDA in the patient group. Two-way comparison for CAT and MDA of the migraine with and without WMH and the controls revealed that CAT serum level significantly decreased in migraine patients with WMH than migraine patients without WMH and controls. In this preliminary study, we demonstrated that the levels of CAT were decreased in migraine patients with WMH compared to patients without WMH and controls. These findings suggest that decreased antioxidant response may play a role in the pathophysiology of WMH in migraineurs. Besides, our results encourage the new treatment and follow-up options based on antioxidant systems.

Increased serum hepcidin and ghrelin levels in children treated for iron deficiency anemia.

BACKGROUND: The aim of this study was to determine the relation between iron deficiency anemia (IDA) and serum leptin, hepcidin, and ghrelin levels. METHODS: Thirty children with IDA and 28 healthy children between the ages of 6 months and 6 years admitted to our hospital were evaluated prospectively. IDA was diagnosed based on clinical and laboratory findings. All children with IDA were treated with iron II-glycine-sulphate complex for 3 months. Complete blood count; iron metabolism parameters; and serum leptin, hepcidin, and ghrelin levels were studied in all healthy children and in children with IDA before and after treatment. RESULTS: In children with IDA, the decrease seen in serum leptin levels after the iron treatment was not statistically significant. However, the increase seen in serum hepcidin levels after the iron treatment was statistically significant (P = 0.038). Hepcidin levels were significantly higher in children with IDA who received iron treatment compared to healthy children (P = 0.008). After the iron treatment, serum ghrelin levels in children with IDA were also significantly higher compared to the levels before treatment and healthy children (P = 0.019 and 0.000, respectively). CONCLUSION: Serum ghrelin and hepcidin levels increase with iron treatment in children with IDA. In view of the higher serum ghrelin and hepcidin levels after iron treatment when compared to pretreatment levels and the healthy children, we suggest that the iron treatment has an important role in serum hepcidin and ghrelin synthesis.

In-vitro platelet hyperaggregation and hypersecretion associated with the use of fish oil in healthy children.

OBJECTIVE: The aim of this study is to evaluate how fish oil supplementation in children affects platelet function tests in vitro. MATERIALS AND METHODS: The study included 62 children (20 healthy children without any medications and 42 healthy children who volunteered to take fish oil supplementation) aged between 2 and 12 years. In the group of children taking fish oil supplementation, the baseline, fourth week, and eighth week values for platelet function tests were obtained. RESULTS: In the platelet aggregation tests induced by high dose of ADP after 8 weeks, the values were significantly higher compared with the values measured before the use of fish oil. The fish oil-supplemented group's values showed an increase in the fourth-week measurements compared with the control group and the baseline measurements in terms of platelet secretion test induced by collagen, standard dose of thrombin, and high-dose thrombin. Platelet secretion tests induced by standard dose of ADP at the end of the eighth week showed an increase compared with baseline test values. CONCLUSION: This study was done in in-vitro conditions wherein the platelet function in the pediatric age group was analyzed and it was found that eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) present in fish oil especially exhibit in-vitro hyperaggregation and increase the secretion of platelets. As a result of this, we consider that it is necessary to be careful while using fish oil supplementation in children as an antithrombotic agent and for a variety of other indications.

Iron deficiency anemia in infants: does it really affect the myocardial functions?

The aim was to assess the myocardial functions in infants with iron deficiency anemia (IDA). The findings of 22 infants with IDA and 16 healthy infants were compared. Complete blood count in all infants, serum iron level, total iron binding capacity (TIBC), and serum ferritin level in patients were evaluated. Echocardiography with tissue Doppler imaging (TDI) was performed in all infants. The mean hemoglobin was 8.5 versus 11 g/dL, hematocrit was 26.7 versus 33.1% and the mean corpuscular volume (MCV) was 59.8 versus 72.2 fL in patients and controls, respectively. The median serum iron level was 14.5 µg/dL, TIBC was 444 µg/dL, and serum ferritin level was 4.9 ng/mL in patients. There were significant differences between patients and controls for the myocardial performance indexes of the left ventricle (LV) (0.57 vs. 0.45, P < .05), interventricular septum (IVS) (0.56 vs. 0.48, P < .05), and the right ventricle (RV) (0.56 vs. 0.41, P < .05). The similar significances were also present for the ejection times (ET) of the LV (173.1 vs. 212.1 ms, P < .05), IVS (173.7 vs. 195.6 ms, P < .05), and RV (169.9 vs. 213.3 ms, P < .05). In spite of negatively significant correlations between hemoglobin, hematocrit, MCV and MPI, the correlations between these parameters and ET were positively significant. However, there were no correlations between serum iron level, TIBC, serum ferritin level, and the TDI parameters of patients. According to these findings, we could say that, the IDA affects the myocardial functions in a negative manner.

Infantile malignant osteopetrosis: a rare cause of neonatal hypocalcemia.

Infantile malignant osteopetrosis (IMO; OMIM 259700) is a rare inherited bone disease characterized by reduced or dysregulated activity of osteoclasts, resulting in generalized osteosclerosis. The disease usually presents within the first few months of life with anemia, hepatosplenomegaly, frontal bossing, nystagmus, blindness, deafness, and bone fractures. Children with IMO are at risk of developing hypocalcemia, with attendant tetanic seizures. We report the case of a baby boy who presented with neonatal hypocalcemia. Skeletal radiographs demonstrated sclerotic bones and a dense base of the skull with typical "space alien" face confirming the diagnosis of IMO. Pancytopenia developed at 2 months of age. Visual evoked potential showed severe bilateral optic nerve damage. Genetic mutation study revealed a new mutation in exon 13 of the TCIRG1 gene. Neonatal hypocalcemia can occur as result of IMO, which is easily missed out by clinicians. This causes delay in establishing the diagnosis and starting necessary treatment. Therefore, osteopetrosis should be kept in mind as a rare cause of neonatal hypocalcemia.

Increased coagulation in childhood obesity.

OBJECTIVE: This study aims to explore the relation between childhood obesity and procoagulant and anticoagulant systems. METHOD: Fifty-one obese children and 32 normal-weighted children with similar age and gender distribution and between ages of 5 and 16 years were recruited to the study. Antropometric measures of all subjects, existence of any accompanying disease, and medication histories had been recorded. Full blood count, procoagulant, and anticoagulant coagulation tests were run for all subjects. RESULTS: When hematologic variables of obese children were compared with those of healthy controls, it was found that average erythrocyte hemoglobin concentration, erythrocyte distribution width, and platelet count of obese children are significantly higher than healthy control group. It was also found that fibrinogen, thrombin time, factor (F) VIII, FIX, FX, and von Willebrand factor levels of obese children are higher than healthy control group. By contrast, antithrombin levels of obese children are found to be lower. CONCLUSION: In our study, we found that there is a procoagulant increase in the coagulation system activity of obese children compared to non-obese healthy children, whereas there is a significant decrease in anticoagulant system. These changes occurred in obese patients, especially higher levels of plasma procoagulant factors such as fibrinogen, FVIII, FIX, and von Willebrand factor, lead us to think that there is an activity in these patients at endothelial level. Further studies are needed on endothelial activity of obese children.

A Delayed Presentation of Arginase Deficiency Presenting with Status Epilepticus.

Arginase 1(ARG1) deficiency is a rare disorder of the urea cycle. The presentation is usually late, leading to loss of intellectual milestones, spasticity and liver involvement. Hyperammonemic crises are rarely encountered. We herein present a case of a 16-year immigrant girl of Syrian origin who was evaluated for acute onset of fever, vomiting, and seizures. Laboratory analyses showed slightly elevated lactate, creatine kinase, and coagulation parameters. Ammonium levels were also moderately increased. On 5th day of admission, she went into an encephalopathic state. Blood amino acid analysis showed highly elevated arginine levels. An increased level of orotic acid was found in urine organic acid analysis. Molecular genetic analysis of ARG1 gene showed a novel homozygous mutation. Although the presentation of ARG1 deficiency is usually chronic in the majority of patients, an acute crisis of encephalopathy due to hyperammonemia may occur and delayed diagnosis may lead to irreversible neurological damage. Key Words: Urea cycle disorder, Hyperammonemia, Argininemia, Encephalopathy.

Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study.

BACKGROUND: Experimental studies have addressed the role of oxidant stress in the pathogenesis of Hemophilia A. This study aimed to determine whether dynamic thiol-disulfide exchange, a recently recognized cellular defense system against oxidative stress, is disturbed in children with hemophilia A. METHODS: This prospective case control study included male children with hemophilia A (n=62) and randomly selected healthy age and sex-matched controls (n=62). Serum native thiol, total thiol and disulfide levels were analyzed with a novel spectrophotometric method. Ratios of disulfide/total thiol, disulfide/native thiol, and native/total thiol were calculated. Statistical comparisons were made using the independent samples t-test or the Mann-Whitney U test, according to whether the data were normally distributed or not. RESULTS: Serum native thiol (385.0 ± 35.9 versus 418.0 ± 44.3, respectively; p < 0.001) and total thiol (424.2 ± 38.7 versus 458.0 ± 46.3, respectively; p > 0.001) levels were significantly lower in children with Hemophilia A compared to controls. Children with hemophilia A had significantly lower serum native thiol to total thiol ratio than controls (p=0.024). Serum disulfide levels of children with hemophilia A were close to controls (19.2 [17.6- 22.1] versus 19.8 [17.8- 21.2]), respectively; p=0.879) whereas disulfide to native thiol ratio (p=0.024) and disulfide to total thiol ratio (p=0.024) were significantly higher. CONCLUSIONS: Decreased antioxidant capacity with levels of serum native thiol and total thiol in children with hemophilia A might be regarded as evidence for the disturbance of thiol/disulfide balance. Antioxidant treatment can be a future target of therapy in children with hemophilia A.

Life-threatening intracranial bleeding in a newborn with congenital cytomegalovirus infection: late-onset neonatal hemorrhagic disease.

The authors present a case of a 36-day-old infant with intracranial and intramuscular hemorrhage due to vitamin K deficiency bleeding, who received intramuscular vitamin K prophylaxis at birth. In this case, laboratory tests showed anemia, liver dysfunction with cholestasis, and coagulopathy, consistent with vitamin K deficiency abnormality. Serological analyses showed that cytomegalovirus immunoglobulin (Ig)M and IgG avidity were both positive. The infant was treated successfully with intravenous ganciclovir and blood products. This case suggests that it is imperative to meticulously investigate the etiology in neonates with late-onset hemorrhagic disease of the newborn. Cholestatic liver disease caused by congenital cytomegalovirus infection should be in mind in term infants who presented with late-onset hemorrhagic disease.

An experience of oseltamivir phosphate (tamiflu™) in a pediatric patient with chronic idiopathic thrombocytopenic purpura: a case report.

We report a pediatric patient with chronic idiopathic thrombocytopenic purpura who suffered from an influenza A (H1N1) virus infection. A 13-year-old girl presented with fever, coughing, and generalized petechiae. The influenza A antigen was positive in her pharyngeal aspirate. She was successfully treated with neuraminidase inhibitor oseltamivir phosphate. Her platelet counts progressively increased with oseltamivir phosphate without another blood product or agent. We suggest that oseltamivir phosphate can be used in patients with immune thrombocytopenic purpura who present with influenza virus A infection. We also suggest that oseltamivir phosphate can be effective in patients with immune thrombocytopenic purpura. Large study groups are needed to confirm the relationship between idiopathic thrombocytopenic purpura and neurominidase inhibitors.

Posterior reversible leukoencephalopathy syndrome in children with hematologic disorders.

OBJECTIVE: Posterior reversible leukoencephalopathy syndrome (PRES) is characterized by headache, altered mental status, cortical blindness, and seizures associated with neuroradiological findings. It involves predominantly white matter of the parieto-occipital lobes. Several medications and disorders play a role in the etiology of PRES. In this study, we aimed to show how the prognosis of PRES in hematological diseases of childhood might be according to the etiological factors. METHODS: Here, we report PRES in six patients, aged 4 to 14 years, with diagnoses of leukemia and aplastic anemia. RESULTS: Suggested causes in our patients were chemotherapeutics, hypertension, infection and antimicrobial drug administration, tumor lysis syndrome, acute renal failure and hemodialysis, immunosuppressive drug administration, and hypomagnesemia. One of the patients died of sepsis, renal failure and pulmonary hemorrhage and another died of relapse after total recovery from PRES. The other four patients are under follow-up without problems. CONCLUSION: We suggest that PRES can recover fully with early diagnosis and treatment whereas it can show poor prognosis depending on the etiology.

A rare presentation of central nervous system in a pediatric patient with Hodgkin disease: cavernous sinus syndrome.

Intracranial involvement by Hodgkin disease is rare. We report a pediatric patient with Hodgkin disease who had intracranial disease at presentation. The patient was referred to our hospital with a suspicion of central nervous system tumor. Although the most common presenting feature of intracranial Hodgkin disease is cranial nerve palsy with brain parenchyma being the most common intracranial site of involvement, to our best knowledge no pediatric case of Hodgkin disease presented with isolated cavernous sinus syndrome reported. We report this rare case because of its unusual presentation, in which Hodgkin disease presented with cavernous sinus syndrome. Physicians should consider the probability of Hodgkin disease in children of all ages who present with cavernous sinus syndrome.

Bone fracture: an unusual presentation of acute megakaryoblastic leukemia.

Some clinical manifestations of acute leukemia in children can mimic orthopedic conditions, and t is variable presentation often makes diagnosis difficult. Bone changes in leukemia are well documented, but there are only a few accounts of children with acute leukemia who present with bone fractures. This report describes a case of this rare combination in a very young boy who presented with fractures of both proximal humerus and left proximal femur and massive periosteal reactions of both humerus and femur and also cystic lesions of proximal femur and iliac bone accompanying aggressive acute megakaryoblastic leukemia.

Incidence of and risk factors for childhood thrombosis: a single-center experience in Ankara, Turkey.

This study was conducted to analyze the incidence of and risk for thrombosis in thrombotic children monitored in the Department of Pediatric Hematology of our hospital at the time of diagnosis, in addition to the clinical characteristics of those patients. The clinical and laboratory findings of 122 patients diagnosed with thrombosis from 1997 to 2006 were retrospectively analyzed. The incidence of thrombosis was 88.6/10,000 hospital admissions. The authors found that 31.1% of the patients studied had a thrombosis in more than 1 region. The incidence of thrombosis by anatomic site was as follows: 42 thromboses in the peripheral arterial system, 39 in an intracardiac region, 38 in the abdominal venous system, 36 in the deep peripheral venous system, and 28 in the cerebral vascular system. The mean age of the patients at the time of diagnosis was 4.9 years. Of the patients studied, 10.7% were neonates, 35.3% were infants younger than 1 year, and 48.4% were younger than 2 years. Most of the patients had a congenital cardiac disease and spontaneous thrombosis, and 66.1% had at least 1 acquired risk factor, the most common of which were having undergone surgery (42%) or wearing a central venous catheter (39%). A hereditary factor for the development of thrombosis was present in 54% of the patients. The most frequently observed hereditary risk factor was the MTHFR 677C-T mutation, and the second most common was the factor V Leiden mutation. Thrombosis should be considered a systemic disorder, and thrombotic patients should be evaluated with appropriate methods. Acquired and hereditary risk factors should be analyzed systematically in thrombotic patients.