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.

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.

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.

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.

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.

A rare chromosomal disorder in a newborn: Trisomy 3q.

Kahvecioglu D, Tatar-Aksoy H, Yildiz E, Bakir A, Alioglu B. A rare chromosomal disorder in a newborn: Trisomy 3q. Turk J Pediatr 2019; 61: 271-274. Trisomy 3q is a rare chromosomal disorder that leads to multiple congenital abnormalities. We hereby present a patient with chromosomal karyotype 46, XY, dup (3)(q23-29), which can be classified as pure 3q duplication and has thin sclera and iris dysgenesis, anterior and posterior segment dysgenesis besides the previously identified specific facial features. To the best of our knowledge only 12 cases have been reported with pure duplication in the literature. Our case is the 13th one reported and has noval findings concerning eye involvement. The ocular manifestations of the 3q duplication syndrome provide additional evidence of the involvement of genes which are responsible for eye development in this chromosomal region.

Ophthalmologic Findings in Children with Leukemia: A Single-Center Study.

OBJECTIVES: Ophthalmologic disease in patients with acute leukemia occurs due to primary leukemic infiltration (involvement), or secondary to the disease and its treatment. In recent years the life expectancy of acute leukemia patients has increased with the advent of modern therapies. The present study aimed to determine the incidence of ocular manifestations in children with acute leukemia. MATERIALS AND METHODS: The study included 120 patients diagnosed with acute leukemia at Baskent University Hospital, Pediatric Hematology Department between 1995 and 2010. All the patients were examined by an ophthalmologist via direct and indirect ophthalmoscopy. RESULTS: Among the patients, 83 (69.2%) were diagnosed with acute lymphoblastic leukemia, 35 (29.1%) with acute myeloblastic leukemia, and 2 (1.7%) with mixed-lineage leukemia. In all, 58 ophthalmic manifestations were noted in 41 patients (34.2%). In our patients, 12 ophthalmologic involvements were present at admission and 46 ocular findings occurred during follow-up. The incidence of these manifestations increased with age. CONCLUSION: Ophthalmologic manifestations were not correlated with gender, hematological parameters at disease onset, type of leukemia, or the frequency of relapse and survival. To more clearly determine the effect of ophthalmologic manifestations on the prognosis of leukemia, larger scale and multi-center studies are needed.

Is There a Tendency for Thrombosis in Gestational Diabetes Mellitus?

CONTEXT: Impact of gestational diabetes mellitus (GDM) on the coagulation system, dynamics involved at a pathophysiological level and the exact mechanism remain unclear. AIMS: To evaluate the association between diabetes-related parameters and hemostatic factors to search for a tendency of thrombosis in GDM. SETTINGS AND DESIGN: Nineteen pregnant women who had GDM, 16 healthy pregnant and 13 healthy nonpregnant controls admitted to the Endocrinology outpatient clinics were enrolled in the study. SUBJECTS AND METHODS: Fasting and postprandial glucose, hemoglobin A1c and insulin levels, and insulin resistance; fructosamine, thrombin activatable fibrinolysis inhibitor (TAFI), tissue factor pathway inhibitor (TFPI), plasminogen activator inhibitor Type-1 (PAI-1), tissue-type plasminogen activator (t-PA), fibrinogen, plasminogen and hemoglobin levels, platelet counts, prothrombin time (PT), and activated partial thromboplastin time (aPTT) were studied. STATISTICAL ANALYSIS USED: One-way analysis of variance, Kruskal-Wallis, and post hoc Tukey honestly significant difference or Conover's nonparametric multiple comparison tests for comparison of the study groups. RESULTS: PT and aPTT were significantly lower in GDM patients compared to controls (P < 0.05), whereas fibrinogen and plasminogen levels were significantly higher in this group compared to both nonpregnant and healthy pregnant controls (P < 0.05 for each). TAFI, TFPI, PAI-1, and tissue t-PA levels were not significantly different among groups. CONCLUSIONS: Our findings indicate tendency to develop thrombosis in GDM similar to diabetes mellitus; but more comprehensive studies with larger sample size are needed to determine the relationship between GDM and hemostasis.

Effects of neonatal sepsis on thrombocyte tests.

OBJECTIVE: Hemostatic disorders are common complications in sepsis, and coagulation abnormalities occur in almost all the septic patients. Thrombocytes have a key role in the pathogenesis of coagulation abnormalities in sepsis. This study aimed to investigate thrombocyte function disorders as a likely cause of hemorrhagic diathesis in patients with neonatal sepsis. MATERIAL AND METHODS: The study included 70 septic newborns (sepsis group) and 59 healthy newborns (control group). Blood samples were collected from the patients within the first 24 h of hospitalization. Thrombocyte aggregation and secretion tests were performed by optical aggregometry and lumi-aggregometry, respectively. Collagen (2 µg/mL), epinephrine (5 µM), standard (5 µM) and high (10 µM) doses of adenosine diphosphate (ADP), standard (1 unit) and high (4 units) doses of thrombin, ristocetin (1.25 mg/mL) and arachidonic acid (0.5 mM) were used as the agonists. RESULTS: The mean age of the septic newborns was significantly higher than that of the controls (6.78 ± 14.47 days versus 1.25 ± 1.17, p < 0.001). There was no difference between the groups regarding gender- and birth-related characteristics. No difference was observed between the groups regarding platelet count (293.37 ± 144.48 × 10(9)/L in the sepsis group and 254.22 ± 65.26 × 10(9)/L in the control group, p = 0.195). Platelet secretion induced by collagen, epinephrine, standard and high (10 µM) doses of ADP, or arachidonic acid and platelet aggregation induced by collagen, high-dose ADP, arachidonic acid, ristocetin or thrombin (1 unit) were significantly higher in the sepsis group than in the control group. CONCLUSION: Based on the results, we concluded that large-scale studies with recurrent tests performed in different periods of sepsis are needed.

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.

Platelet aggregation, secretion, and coagulation changes in children with asthma.

The chronic inflammation in asthma evolves by cells including eosinophils, mast cells and lymphocytes. Despite their principal function in hemostasis, platelets contribute to pathogenesis of asthma that activation of platelets occurs following antigen provocation and during asthma attack. Our aim was to evaluate the platelet functions and other hemostatic features of children with asthma, both during symptom-free period and asthma attack. We enrolled patients with asthma attack (n = 33), mild intermittent asthma (n = 18), mild persistent asthma (n = 15) and healthy children (n = 20). Demographic characteristics and disease-related features were noted. Platelet aggregation and secretion tests (expressed as ATP release) were performed by lumiaggregometer method by stimulation with collagen, epinephrine, ADP, thrombin, ristocetin and arachidonic acid. Plasma levels of D-dimer, factor VIII (FVIII) and von Willebrand factor (vWF) were assessed. There were no differences in platelet aggregation induced by agonists between study groups. ATP release from platelets of patients with asthma exacerbation induced by ADP was lower compared with mild intermittent asthma (P < 0.001). Epinephrine-stimulated ATP secretion was also lower in patients with asthma attack than mild intermittent (P = 0.039) and mild persistent asthma (P = 0.011) and controls (P = 0.018). vWF measurements were higher in children with asthma attack than other study groups (P = 0.001). However, FVIII was increased in patients with severe asthma attack. Asthma is a disease in which many immune cells play a role, one of which is the platelet. Distinctions in platelet secretion profiles and plasma levels of vWF and FVIII provide evidence that coagulation mechanisms might be critical for asthma pathogenesis.

A comparison of intravenous immunoglobulin (2 g/kg totally) and single doses of anti-D immunoglobulin at 50 µg/kg, 75 µg/kg in newly diagnosed children with idiopathic thrombocytopenic purpura: Ankara hospital experience.

We conducted this prospective randomized trial of intravenous immunoglobulin (IVIG) treatment in children with newly diagnosed immune thrombocytopenic purpura (ITP) to compare the efficacy of IVIG to standard and higher doses of anti-D IVIG. Seventy-eight patients who were previously untreated and between the age of 1 and 18 years with newly diagnosed acute ITP and a platelet concentration less than 20×10/l were eligible for enrollment. In this study IVIG treatment was compared with two different doses of anti-D. Study patients were randomized to receive treatment according to one of the two single anti-D IVIG doses [50 µg/kg (n=19) or 75 µg/kg (n=20)] or 2 g/kg (400 mg/kg per day, 5 day) total dose of IVIG (n=39). There is a significant increase of 24th hour, 48th hour, 72nd hour, 7th day and 30th day platelet counts in IVIG (2 g/kg, total dose) group compared to anti-D IVIG 50 µg/kg and anti-D IVIG 75 µg/kg groups. However, there were no difference between 24th hour, 48th hour, 72nd hour, 7th day and 30th day platelet counts across anti-D IVIG 50 µg/kg and anti-D IVIG 75 µg/kg groups. In conclusion, this study suggests that IVIG is well tolerated and significantly more effective than standard and high-dose anti-D IVIG for the treatment of newly diagnosed ITP in children. Apart from this, we believe that IVIG might be the first-line treatment of these patients. Regarding this issue further prospective studies comparing different IVIG treatment regimens with anti-D IVIG treatment regimens are needed.

A technical complication of radiosynovectomy in the ankle: leakage to distal tibiofibular syndesmosis.

Chronic hemophilic synovitis due to intra-articular bleeding in hemophilia A (a congenital coagulation defect) is a common manifestation. Radiosynovectomy is used for the ablation of chronic hemophilic synovitis. Complications due to radiosynovectomy may be seen, such as leakage of radioactivity to the lymph nodes, liver, and spleen via the lymphatic system. We report herein 2 cases that developed leakage to the distal tibiofibular syndesmosis, lymph nodes, liver, and spleen after radiosynovectomy to the ankles. In order to prevent leakage, low-volume injection is recommended.

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.

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.

Successful use of recombinant factor VIIa in a child with Schoenlein-Henoch purpura presenting with compartment syndrome and severe factor XIII deficiency.

In this article, we present a 7-year-old boy with Schoenlein-Henoch purpura (HSP) presented with compartment syndrome and factor XIII deficiency and treated with recombinant factor VIIa and fasciotomy. Treatment decisions for patients with HSP presenting with compartment syndrome should be made on a case-by-case basis. Factor XIII deficiency should be in mind in these patients. The use of recombinant factor VIIa might be effective and well tolerated for treating hemorrhage in patients with HSP and compartment syndrome. Surgical treatment should be preferred in patients with compartment syndrome. However, in patients who have a coagulation defect, the first priority is to correct the clotting deficiency. The use of recombinant factor VIIa is a treatment option for children who develop compartment syndrome due to a coagulation defect.