Evaluation of nocturnal blood pressure changes and urinary electrolyte excretion in children with enuresis.

BACKGROUND: Monosymptomatic nocturnal enuresis (MNE) is defined as involuntary nighttime urination of children over five years of age without any congenital or acquired defect in the central nervous system. Many factors, mainly nocturnal polyuria, sleep disorders, decreased bladder capacity, and bladder dysfunctions play a role in the etiology of MNE. METHODS: Eighty-three children diagnosed with MNE were included in the study. Complete blood cell count, blood biochemistry, renin, and aldosterone levels of all children were obtained. Twenty-four-hour urine samples were collected separately daytime and nighttime and urinary electrolytes were evaluated. Also, 24-hour ambulatory blood pressure monitoring (ABPM) was performed for each patient. The results were evaluated by comparing both enuretic children vs. control group and enuretic children with polyuria vs. without polyuria. RESULTS: When we compared the enuretic children and the control group in terms of urinary electrolytes, the fractional excretion of sodium (FENa) and fractional excretion of potassium (FEK) values of the enuretic group were higher than the control. The evaluation of the 24-hour ABPM findings revealed no significant difference in terms of the mean arterial pressure (MAP) and diastolic blood pressure (DBP) during the daytime and nighttime measurements. The daytime systolic blood pressure (SBP), however, was significantly lower in the enuretic group. When enuretic children with and without polyuria and the control group were compared, the nighttime, FENa, FEK, as well as nighttime urinary excretion of calcium and protein were significantly higher in enuretic children with polyuria. No difference was detected on the MAP, SBP, or DBP values. CONCLUSIONS: In conclusion, the nighttime urinary solute excretion of enuretic children was found to be higher and this condition may especially be associated with pathogenesis of nighttime polyuria. In enuretic children, nighttime blood pressure changes were not influential in the etiopathogenesis in all patient groups and multiple mechanisms may play a role in the pathogenesis of enuresis.

Primary cavitary tuberculosis in an infant.

In infants, tuberculosis usually progresses as hilar lymphadenopathy and parenchyma changes in lungs; associating cavitary lesions are rare. A six-month-old infant was admitted to our hospital with fever. Physical examination revealed decreased breathe sounds in the right lung. Chest radiograph showed pneumonic infiltration in the right middle lobe. The patient was hospitalized with a diagnosis of lobar pneumonia and antibiotic treatment was started. On the sixth day, because no clinical improvement was observed in the patient, computerized thorax tomography was performed. Tomography revealed multiple lymphadenopathies in the right hilar pretracheal and subcarinal region. The patient's tuberculin and acid-resistant bacteria tests were negative; however, the quantiferon test was positive. Family screening revealed active tuberculosis in the mother. Tuberculosis in infants may present with unusual clinical and radiologic findings, and primary cavitary tuberculosis can also be seen in this age group.

The Serum Concentrations of Trace Elements and Vitamin A in Turkish Six-Month-Old Infants with Different Feeding Practices.

Breast feeding is the first and most important step of a healthy diet. Breast milk contains important vitamins and trace elements such as iron, zinc, copper, and vitamin A. The aim of our study was to evaluate the levels of hemoglobin, hematocrit, mean corpuscular volume, serum iron, iron binding capacity, ferritin, serum zinc, copper and vitamin A in three groups of infants, which were determined based on feeding practices. The infants in all groups were not given prophylactic iron in the first 6 mo. Two hundred fifty-nine infants were included in the study. One hundred fifty-one (58.3%) were fed with breast milk, 91 (35.1%) were fed with breast milk+formula, and 17 (6.6%) were fed with formula only. Serum copper and vitamin A levels were found to be low in formula-only fed infants compared to other groups with a statistically significant difference (p=0.017, p=0.022 respectively). The serum zinc level was found to be low in 15.9% of the breast fed infants, 17.6% of the breast milk+formula fed infants, and 23.5% of the formula-only fed infants. Although the formula-only fed infants had lower values, the difference was not statistically significant among groups (p=0.716). We think that formula fed infants potentially have low levels of copper and vitamin A in the first 6 mo and may be offered supplements. Alternatively, formula mineral and vitamin contents could be enriched. We think that further studies on this subject are needed.

Right middle lobe atelectasis in children with asthma and prognostic factors.

BACKGROUND: Although right middle lobe (RML)-atelectasis of the lungs is a common complication of asthma, the relevant data is limited. The aim of this study is to define the characteristics of RML atelectasis in asthma during childhood. METHODS: Children with asthma who had recently developed RML atelectasis were included; anti-inflammatory medications, clarithromycin, and inhaled salbutamol were prescribed, chest-physiotherapy (starting on the sixth day) was applied. Patients were reevaluated on the sixth, fourteenth, thirtieth, and ninetieth days, chest X-rays were taken if the atelectasis had not resolved at the time of the previous visit. RESULTS: Twenty-seven patients (6.8 (4.8-8.3) years, 48.1% male) with RML atelectasis were included. Symptoms started 15 (7-30) days before admission. The thickness of the atelectasis was 11.8 ± 5.8 mm; FEV1% was 75.9 ± 14.2 and Childhood Asthma Control Test scores were 11.8 ± 5.6 at the time of admission. The atelectasis had been resolved by the sixth (n = 3), fourteenth (n = 9), thirtieth (n = 10), and ninetieth days (n = 3). The treatment response of the patients whose atelectasis resolved in fourteen days was better on the sixth-day (atelectasis thickness: 4.7 ± 1.7 vs. 11.9 ± 7.3 mm, p = 0.021) compared to those whose atelectasis resolved later. Nearly half (54.5%) of the patients whose atelectasis had resolved by fourteen days were using controller medications at the time of admission. However, only two patients (13.3%) were on controller treatment in the latter group (p = 0.032). Regression analysis didn't reveal any prognostic factors for the early resolution of atelectasis. CONCLUSIONS: Early diagnosis and treatment of RML atelectasis prevents complications. Patients who had early resolution of atelectasis had already been on anti-inflammatory medications, and responded better to aggressive treatment within the first week.

Lactate Level Is More Significant Than Carboxihemoglobin Level in Determining Prognosis of Carbon Monoxide Intoxication of Childhood.

OBJECTIVES: To evaluate the demographics, risk factors, correlation between carbon monoxide (CO) level and clinical findings, and laboratory findings determining the prognosis and ischemic myocardial injury due to CO intoxication in patients admitted to pediatric emergency department. MATERIALS AND METHODS: Six hundred seventy-four patients were admitted with CO intoxication between May 2007 and October 2009, 288 patients who required hospitalization were enrolled into the study prospectively. RESULTS: Incidentally, 144 (50%) of the patients were evenly distributed as girls and boys. Their age ranged between 7 months and 17 years; mean age was 8.6 years. The mean CO level was 26.8. The high levels were detected regarding lactate in 199 (90.1%) patients, creatine kinase (CK)-MB in 130 (45.1%) patients, CK in 80 (27.8%) patients, cardiac Troponin I in 35 (17.2%) patients, and lactate dehydrogenase in 34 (15.7%) patients. There was a significant positive correlation when symptoms like syncope, loss of consciousness, and convulsion were compared with carboxyhemoglobin, lactate, CK, CK-MB, and lactate dehydrogenase levels (P < 0.05), whereas there was no correlation when compared with cardiac Troponin I (P > 0.05). To determine the accuracy of predicting severe CO intoxication, sensitivity of 52.6% and specificity of 85.7% were found in receiver operating characteristic analysis when the lactate level was 3.85 mmol/L, whereas sensitivity of 70.5% and specificity of 59.6% were found when the carboxyhemoglobin level was 27.1%. One hundred forty-six (%50.8) of the patients had normal electrocardiographic findings, whereas 135 (46.8%) had sinus tachycardia, 6 (2%) had right branch block, and 1 (0.34%) had atrioventricular block. In 34 patients who had high CK-MB and Troponin I levels, only sinus tachycardia was detected in electrocardiography, and there were no ST changes. Hyperbaric oxygen was necessary in 2 patients admitted with coma. CONCLUSIONS: In children admitted because of CO intoxication, the blood lactate levels may give more accurate information in terms of loss of consciousness and convulsion, lactate level could be taken as a measure of severe poisoning and may help to decide for hyperbaric oxygen treatment.

Characteristics of Children with Acute Carbon Monoxide Poisoning in Ankara: A Single Centre Experience.

The purpose of the study was to define characteristics of children with acute carbon monoxide poisoning. Eighty children hospitalized with acute carbon monoxide poisoning were recruited prospectively over a period of 12 months. Sociodemographic features, complaints and laboratory data were recorded. When the patient was discharged, necessary preventive measures to be taken were explained to parents. One month later, the parents were questioned during a control examination regarding the precautions that they took. The ages of the cases were between one month and 16 yr. Education levels were low in 86.2% of mothers and 52.6% of fathers. All families had low income and 48.8% did not have formal housing. The source of the acute carbon monoxide poisoning was stoves in 71.2% of cases and hot-water heaters in 28.8% of cases. Three or more people were poisoned at home in 85.1% of the cases. The most frequent symptoms of poisoning were headache and vertigo (58.8%). Median carboxyhemoglobin levels at admission to the hospital and discharge were measured as 19.5% and 1.1% (P < 0.001). When families were called for re-evaluation, it was determined that most of them had taken the necessary precautions after the poisoning incident (86.3%). This study determined that children with acute childhood carbon monoxide poisoning are usually from families with low socioeconomic and education levels. Education about prevention should be provided to all people who are at risk of carbon monoxide poisoning before a poisoning incident occurs.

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.

What a pandemic teaches us about vaccination attitudes of parents of children with asthma.

BACKGROUND: During the recent pandemic, Influenza A/H1N1 vaccine uptake remained far below the targeted rates. Associated factors regarding vaccine refusal in the general population have been reported in many studies, however the reasons behind refusals for asthmatic children have not yet been identified. We aimed to investigate Influenza A/H1N1 virus vaccine acceptance for children with asthma, to determine the attitudes and beliefs of parents concerning Influenza A/H1N1 disease and vaccine and to identify the association of asthma control parameters with vaccination. METHODS: The parents of asthmatic children aged 6-18 years participated in a cross-sectional survey study in three pediatric allergy outpatient clinics. The survey measured demographic factors, asthma control parameters, vaccination rates, and beliefs and attitudes regarding Influenza A/H1N1 vaccine. RESULTS: Of the 625 asthmatic children, 16.8% (n=105) were immunized with Influenza A/H1N1 and 45.7% (n=286) with seasonal influenza vaccine. Educational background of parents (p<0.001 and p=0.002, for father's and mother's educational level, respectively), previous vaccination with seasonal influenza (p<0.001), and having a family member vaccinated against Influenza A/H1N1 (p<0.001) had a significant influence on vaccine acceptance, while fear of side effects (88.6%) was the major parental reason for refusing the vaccine. Asthma control parameters had no influence on uptake of the vaccine. Physician recommendation (84.8%) was important in the decision-making process for immunization. The statement "Children with asthma should receive swine flu vaccine" increased the likelihood of being vaccinated [OR: 2.160, (95%CI 1.135-4.111), p=0.019]. CONCLUSION: Although asthmatic children are considered to be a high-priority group for Influenza A/H1N1 vaccination, we found low uptake of vaccine among our patients. Beliefs and attitudes rather than asthma control parameters influenced parental decisions for immunization. Understanding the underlying determinants for refusing the vaccine will help to improve vaccine campaigns in advance of a future outbreak.

Long-term outcomes of the shaken baby syndrome prevention program: Turkey's experience.

AIM: Shaken baby syndrome (SBS) is a condition which may cause to serious health problems in the baby. SBS may be prevented by increasing awareness with giving education to parents especially in the early postnatal period. In shaken baby prevention programs, education is recommended to be given before the 2-4(th) month during which the frequency of crying is increased. It is important that education given in the early period is permanent until the period during which the frequency of crying is increased. The aim of this study was to evaluate the persistency of the benefit of the SBS prevention program until the 2-4(th) month during which crying is intensified. MATERIAL AND METHODS: This study is an interventional study. When the babies became 2-4 months old, a questionnaire which questioned the usefulness of education and the experiences with babies was applied to a group selected randomly among the mothers who received SBS prevention education during pregnancy or in the first 7 postnatal days (group A). The same questionnaire was applied to 143 mothers whose babies completed their first 2 months, who presented to the hospital for vaccination and who did not receive education about SBS as the control group (group B). The data were evaluated using the Statistical Program for Social Sciences (SPSS) 15.0 statistical analysis package program. Ethical approval was obtained from the local ethics committee (30.12.2009, 2785). RESULTS: The rate of the mothers who stated "yes" to the sentence "babies occasionally cry" which was one of the main messages of the education was statistically significantly higher in group A compared to group B (p=0.001). The rate of the mothers who stated "I agree" to the sentence "battering is harmful for babies" was statistically significantly higher in group A compared to group B (p=0.001). CONCLUSIONS: Conclusively, it was found that SBS prevention program education was permanent until the 2-4(th) month.

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.

Dysfunctional voiding in children with asthma.

A prospective study was performed to evaluate the prevalence of suspected dysfunctional voiding (DV) and associated risk factors in children with asthma. The DV is defined as any voiding symptoms and/or urinary incontinence. Children (4-10-year-old) with asthma (n=178) and healthy subjects (n=197) were enrolled. DV and Incontinence Scoring System were administrated. Demographic features and asthma related characteristics were recorded. Suspected DV was noted in 19 (27.9%) of patients with asthma and 5 (6.6%) of healthy subjects in children younger than 6 years of age (p=0.001). In this patient group, asthma increased the risk of suspected DV (OR=5.7 (95% CI 1.988 to 16.344)). Children with asthma older than 6 years of age had similar prevalence of suspected DV but they had higher frequency of voiding and urgency. Asthma is associated with higher DV symptom scores in younger children who have already completed toilet training and with some of DV symptoms such as frequency and urgency in older children.

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.

Prevalence of obesity and associated risk factors among adolescents in Ankara, Turkey.

OBJECTIVE: The purpose of this study was to investigate the prevalence of and the risk factors associated with obesity among adolescents in Ankara, Turkey. METHODS: The study was conducted in 26 schools in Ankara during the time period from September 2010 to March 2011. A total of 8848 adolescents aged 11-18 years were chosen using a population-based stratified cluster sampling method. Body mass index (BMI) of the participants was compared with the BMI references for Turkish children and adolescents to estimate the prevalence of overweight and obesity. A standardized questionnaire aiming to determine the sociodemographic characteristics, computer use, television (TV) watching, physical activity, and presence of obesity in the family was applied to the study group. RESULTS: The results showed that the overall prevalence of obesity among adolescents was 7.7% (8.4 % for females and 7.0% for males). It was observed that BMI increased as computer use increased. A greater proportion of the overweight and obese adolescents watched TV and use computer for more than 2 hours/day as compared to their normal-weight counterparts. The normal-weight subjects were found to show a higher participation in regular physical activity. Obesity prevalence among the families of obese adolescents was 56.5%. CONCLUSIONS: The prevalence of adolescent obesity in Ankara, Turkey is lower as compared to many European countries and to the United States. Computer use, watching TV, physical activity and family factors are important risk factors for obesity.

Increased thrombin-activatable fibrinolysis inhibitor and decreased tissue factor pathway inhibitor and thrombomodulin levels in children with hypothyroidism.

OBJECTIVE: We determined the profile of coagulation/fibrinolytic and vascular endothelial cell function parameters including plasminogen activator inhibitor (PAI) and thrombin-activatable fibrinolysis inhibitor (TAFI), tissue factor pathway inhibitor (TFPI), thrombomodulin (TM), and tissue plasminogen activator (tPA) levels in children with hypothyroidism. METHODS: Forty children with hypothyroidism aged 0-16 months who presented for the first time to our hospital and 29 age-and sex-matched healthy controls were enrolled in the study. All coagulation tests were performed with ELISA method. One year after Na-L-thyroxine treatment, the study parameters were re-evaluated in 25 euthyroid children out of the 40 patients diagnosed with hypothyroidism. RESULTS: Although no significant effect was detected regarding PAI antigen (Ag) and tPA Ag, the levels of TAFI, TM, and TFPI were consistent with subclinical hypercoagulability and hypofibrinolysis. There was a significant increase in TAFI Ag levels and a significant decrease in TFPI Ag and TM Ag levels in hypothyroid patients compared to healthy controls. As a result of correlation tests, the largest impact of hypothyroidism on coagulation system was on TFPI. In accordance with these findings, TAFI Ag levels decreased and TFPI Ag and TM Ag levels increased with hormonal replacement therapy. CONCLUSIONS: Increased TAFI and decreased TFPI and TM in patients with hypothyroidism may indicate a potential hypercoagulable and hypofibrinolytic state as well as possible endothelial dysfunction, which may increase the risk of atherosclerotic and atherothrombotic complications. Thyroid hormone levels should also be checked in patients with a predisposition to coagulation, and thyroid replacement therapy should be initiated.

Procoagulant and anticoagulant factors in childhood hypothyroidism.

The aim of this study was to investigate the effects of thyroid hormone deficiencies in childhood on the elements of coagulation proteins. Consecutive 54 children with hypothyroidism and 55 healthy controls aged 1 month-16 years were enrolled. One year after Na-L-thyroxine treatment, the study parameters were reevaluated. Thyroid function tests, procoagulant and anticoagulant proteins were performed for children with hypothyroidism and healthy controls. Significant decreased results were found in children with hypothyroidism in terms of fibrinogen, TT, and anticoagulant proteins including AT, PC, PS, and fPS. Significant increases were found with respect to APTT, fibrinogen, and TT. In the evaluation of posttreatment changes a statistically significant increase was found in vWF, FVIII, AT, PC, PS, and fPS. A positive correlation was found between fT4 and vWF, FVIII, PC, and PS. We would like to emphasize that the coagulation system especially vWF and FVIII, and particularly the anticoagulant system, should be monitored closely in patients followed up for hypothyroidism. Thyroid hormones should be examined and, if necessary, hormone replacement therapy should be administered in patients followed up for a predisposition to coagulation. Additionally, further studies with larger series are needed to investigate the effects of hypothyroidism on the coagulation system.

The effect of an anaphylaxis guideline presentation on the knowledge level of residents.

OBJECTIVE: To determine the effects of an anaphylaxis guideline presentation in residency training, which is an important period for having skilled and knowledgable doctors in the future and see how the residents' level of knowledge changes after presentation. The study is the first in Turkey to identify ways to integrate clinical practice guidelines (CPGs) in residency training. METHODS: In this interventional study to evaluate a continuous medical education (CME) intervention without a comparison group, a clinical practice guidelines on diagnosis and management of anaphylaxis was presented to the Family Medicine and paediatrics residents at the Ankara Training and Research Hospital, by a specialist in paediatric allergy. The presentation was done within routine training sessions. A test was applied before and 10 weeks after the presentation. Electronic sources and hard copies were used for dissemination. The results were analysed with SPSS 15.0. The categorical data was analysed with Fisher's Exact test and the Mann-Whitney U test was used to compare the groups. The threshold for statistical significance was set at P < 0.05. RESULTS: The post-test scores were 58.2/100 for family physicians and 71.7/100 for paediatricians. For both pre- and post-test, paediatricians had significantly higher scores than the family physicians (p < 0.05). On the other hand, the family physicians had significantly higher post-test scores than their own pre-test scores (p < 0.05), while there was no significant increase in the scores of the paediatricians (p > 0.05). CONCLUSION: The study adds to limited information on the effects of clinical practice guidelines in Turkey. It shows us that the awareness of CPGs is low among physicians, and further research is needed to determine the potential role of clinical practice guidelines in continuous medical education. Physicians need a better training about how to manage anaphylaxis, and the best methods to identify their training needs must be determined.

A novel pathogenic DNA variation in the OCRL1 gene in Lowe syndrome.

The oculocerebrorenal syndrome of Lowe (OCRL) is an X-linked disorder characterized by congenital cataracts, renal tubular dysfunction, cognitive problems and maladaptive behavior. The syndrome is caused by pathogenic DNA variations in the X-linked OCRL1 gene. A 24-month-old boy was referred to our hospital with delayed motor milestones, hypotonia, involuntary purposeless movements of hands and feet, congenital cataract, severe feeding difficulties, and failure to thrive. Physical examination at the age of 24 months revealed a body weight of 7350 g (-5.1 SDS). Length was 71 cm (-5.1 SDS) and head circumference 45 cm (-3.9 SDS). He had deep-set small eyes, frontal bossing, flat occiput, parietal prominence, bilateral congenital cataract, cryptorchid left testis, joint hypermobility, decreased muscle tone, and hyporeflexia. Biochemical analysis revealed the characteristic findings of renal Fanconi syndrome. Genetic analysis showed a novel pathogenic DNA variation (c.1528C>T) in exon 15 of the OCRL1 gene. Clinical findings and genetic analysis confirmed the diagnosis of OCRL syndrome.