Association of Attention Deficit Hyperactivity Disorder and Autism Spectrum Disorders with Mean Platelet Volume and Vitamin D.

BACKGROUND The purpose of this study was to assess the values of the mean platelet volume (MPV) in children with attention deficit hyperactivity disorder (ADHD) and with autism spectrum disorders (ASDs) to determine the risk of cardiovascular disease in these 2 disorder groups. MATERIAL AND METHODS The study included a total of 79 patients with ADHD or ASDs and controls in the Van region of Turkey. The control group included subjects of matching age and sex with no ADHD, ASDs, or chronic disease and taking no vitamins. The hematological parameters of the patients, including MPV, vitamin B12, and vitamin D, were assessed. RESULTS The study included a total of 79 children and adolescents aged 2-18 years (32 females and 47 males). Of the patients, 36 were in the ADHD group, 18 in the ASDs group, and 25 in the control group. There was no statistically significant difference in hematological parameters between the groups, but there were significant differences in terms of vitamin D and vitamin B12. The patient groups showed lower levels of vitamin B12 and vitamin D. In the ADHD group, there was a negative correlation between both vitamins and MPV (p<0.05). Partial correlation analysis of the ADHD group showed that MPV in particular was negatively correlated to vitamin D, and not to vitamin B12 (p: 0.03). CONCLUSIONS Both ADHD and ASDs may accompany increased risk for cardiovascular disease due to the presence of vitamin B12 and D deficiency and their own characteristics. Therefore, these disorders should be closely followed up.

Initial Lymphocyte Count as Prognostic Indicator for Childhood Immune Thrombocytopenia.

Acute ITP is a benign, self-limiting disease. Chronic ITP is diagnosed when thrombocytopenia persists beyond 12 months. The main objective of the present study was to examine whether absolute lymphocyte counts at diagnosis has predictive value with chronic ITP. A total of 601 patients diagnosed as ITP between 1995 and 2014 were retrospectively evaluated. CBCs with differential counts were performed at presentation for 601 patients. Absolute lymphocyte counts at presentation were independently predictive of disease duration. The male to female ratio was almost 1:1 and 25.9 % (156/601) of the patients had chronic ITP. We determined that age >6.75 year, platelet counts >6.950/mm3 and absolute lymphocyte counts ≤2.050/mm3 was associated with a significant risk for developing chronic ITP. Absolute lymphocyte counts at the time of diagnosis were predictive variables for the development of chronic ITP. Further researches are needed to confirm the current finding and to assess the underlying pathophysiology with the course of the ITP in observational studies.

An analysis of children with brucellosis associated with haemophagocytic lymphohistiocytosis.

This retrospective study included seven paediatric cases aged from 4 to 14 (10.2±3.4) years with pathologically proved haemophagocytic lymphohistiocytosis from a single institution during 2009 and 2013. Over this time period, 496 patients with brucellosis were diagnosed. None of the patients (3 boys and 4 girls) had a history of any haematologic disorder. All patients had an anamnesis for recently consumed unpasteurised homemade dairy products or had a contact history with sheep and/or cows. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titres were 1: 1280 in seven patients. Blood culture was positive for Brucella melitensis in three patients (42%). Bone marrow cultures were positive for B. melitensis in four patients (57%). Fever was present in all patients (100%) with haemophagocytic lymphohistiocytosis. The other most common symptoms were malaise, myalgia, anorexia, sweating and weight loss. In addition, sweating was observed in five patients, and lymphadenopathy, petechiae, and weight loss were observed in one patient. Hepatomegaly, splenomegaly, and hepatosplenomegaly were found in four (57%), six (85%) and four (57%), patients, respectively. Haemophagocytosis was documented in bone marrow examinations of all children except in two cases. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 4 weeks after antibiotic treatment of brucellosis.

Evaluation of the Hemostatic Disorders in Adolescent Girls with Menorrhagia: Experiences from a Tertiary Referral Hospital.

Bleeding disorders are a common cause of menorrhagia in the adolescent age group. We aimed to evaluate the incidence of hemostatic disorders, using clinical and laboratory findings of bleeding disorders in adolescent girls with menorrhagia. A retrospective chart review used to evaluate adolescent girls with menorrhagia who were referred to Yuzuncu Yil University Pediatric Hematology clinic between January 2010 and December 2014. Out of 52 patients referred for investigation, 50 patients were included in the study. The mean age and mean menarche age were 14.8 ± 1.42 (range: 12-17) and 12.47 ± 0.55, respectively. In 42 % (n = 21) of patients, anemia was detected. In 22 % (n = 11) of patients, a bleeding disorder was detected: five cases with von Willebrand disease, two cases with acute immune thrombocytopenic purpura, one case with Bernard-Soulier syndrome, one case with Glanzmann thrombasthenia, one case with aplastic anemia and one case with factor X deficiency. The remaining 39 out of the 50 patients were finally diagnosed with dysfunctional uterine bleeding. When compared the patients with bleeding disorders and without bleeding disorders, bleeding from other sites, including gingival bleeding or epistaxis, low platelet counts and prolonged activated partial thromboplastin time were found statistically more frequent in patients with bleeding disorders (p < 0.05). Menorrhagia in adolescents is frequently associated with underlying bleeding disorders. Adolescents with heavy menstrual bleeding and a history of nose or gingival bleeding should be evaluated for congenital bleeding disorders.

Hematologic Findings in Children With Brucellosis: Experiences of 622 Patients in Eastern Turkey.

The aim of the present study was to present the hematologic findings of 622 cases diagnosed with acute brucellosis. Medical records of children with brucellosis, admitted to Yuzuncu Yil University Hospital over a 6-year period, were analyzed retrospectively. Three hundred ninety-seven (63.8%) patients were male and 225 (36.2%) were female. The mean age was 11 years (range, 1 to 16 y). The mean hematologic values were as follows: hemoglobin 9.4 (3.4 to 17) g/dL, hematocrite 28% (12% to 50%), leukopenia 2.8 (1.1 to 33)×10/L, leukocytosis 13.7 (1.1 to 33)×10/L, and platelets 79 (3 to 972)×10/L. An overall 28.6% of patients were found to have anemia, alone or in combination with leukopenia or thrombocytopenia. Sixteen percent of patients had thrombocytopenia, 13.9% had leukopenia, 7.7% had pancytopenia, and 4% had both leukopenia and thrombocytopenia. Hematologic complications such as anemia, thrombocytopenia, and leukopenia are more frequently seen in acute brucellosis cases. However, acute brucellosis should also be considered in the differential diagnosis in the presence of other hematologic abnormalities such as severe thrombocytopenia, bicytopenia, and pancytopenia.

Voriconazole-associated visual disturbances and hallucinations.

Voriconazole is a second-generation azole widely used for the prevention and treatment of fungal infection in leukemia patients. Voriconazole is considered the primary antifungal agent for invasive aspergillosis. We report a case of 16-year-old girl who developed visual disturbance and visual and auditory hallucinations after intravenous voriconazole treatment for invasive pulmonary aspergillosis. Due to the visual hallucinations and visual disturbance began acutely and shortly after the initiation of voriconazole, and no other cause could be determined, the symptoms were considered to be the side effects of voriconazole. Simultaneous development of visual side effects and hallucinations rarely have been reported before.

Eosinophilic leukemoid reaction in a male adolescent with Löeffler syndrome.

The Löeffler syndrome is characterized by pulmonary infiltrates on a chest x-ray accompanied with peripheral eosinophilia. In this article, we have highlighted the Löeffler syndrome complicated with a eosinophilic leukemoid reaction in a previously healthy boy. The patient was treated with albendazole for five days, with a successful result. In countries where parasitic diseases are endemic, the Löeffler syndrome must always be considered in patients who present with a eosinophilic leukemoid reaction.

Diagnostic evaluation of our patients with hemophilia A: 17-year experience.

AIM: Hemophilia A is a rare inherited bleeding disorder resulting from factor VIII deficiency and is a group of diseases characterized by intra-articular and intramuscular bleeding. In this study, we aimed to retrospectively evaluate the treatment outcomes, demographic and clinical characteristics of our patients who were treated and followed up for last 17 years in our pediatric hematology unit with a diagnosis of Hemophilia A. MATERIAL AND METHODS: The medical records of 83 patients who were diagnosed with Hemophilia A and followed up between 1997 and 2014 in our hospital's pediatric hematology clinic were reviewed retrospectively. The demographic data, prophylaxis state, development of inhibitors and clinical characteristics of the patients were evaluated. RESULTS: When the complaints at presentation were examined, it was found that 27 (32%) patients had hemarthrosis, 24 (29%) patients had ecchymosis and hematoma, 13 (16%) patients had prolonged bleeding after trauma or cut, 10 (12%) patients had gingival, mouth or nose bleeding, 4 (5%) patients had prolonged bleeding after circumcision, 4 (5%) patients had gastrointestinal bleeding, 1 (1%) patient had hematuria. Fifty (60%) patients were considered severe hemophilia A, 20 (24%) patients were considered moderate hemophilia A and 13 (16%) patients were considered mild hemophilia A according to factor activity. Among severe hemophilia A patients, primary prophylaxis was being administered in 2 (2%) patients and secondary prophylaxis was being administered in 40 (48%) patients. Inhibitor positivity was found in 8 (10%) of these patients. It is found that hemophilic artropathy developed in 17 patients and 8 of these 17 patients had undergone radioisotope synovectomy. CONCLUSIONS: Treatment of severe bleeding in hemophilia A patients should be performed in hospital and the presence of inhibitor must be investigated in cases of uncontrolled bleeding where adequate doses of factor concentrates have been administered for treatment. In order to decrease the development of inhibitor, prophlaxis should be suggested to patients rather than repetetive treatment when bleeding occurs. The radioactive synovectomy should not be overlooked in countries like ours in which factors can not be used adequately.

Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report.

Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.

Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report / Anestesia em paciente com anemia de Fanconi e displasia do desenvolvimento do quadril: relato de caso / Anestesia en paciente con anemia de Fanconi y displasia del desarrollo de la cadera: caso clínico

Fanconi anemia is a rare autosomal recessive inherited bone marrow failure syndrome with congenital and hematological abnormalities. Literature regarding the anesthetic management in these patients is limited. A management of a developmental dislocation of the hip was described in a patient with fanconi anemia. Because of the heterogeneous nature, a patient with fanconi anemia should be established thorough preoperative evaluation in order to diagnose on clinical features. In conclusion, we preferred caudal anesthesia in this patient with fanconi anemia without thrombocytopenia, because of avoiding from N2O, reducing amount of anesthetic, existing microcephaly, hypothyroidism and elevated liver enzymes, providing postoperative analgesia, and reducing amount of analgesic used postoperatively.

A anemia de Fanconi é uma síndrome hereditária autossômica recessiva rara, caracterizada por deficiência da medula óssea e anomalias congênitas e hematológicas. A literatura sobre o manejo anestésico dos pacientes é limitada. O manejo de uma displasia do desenvolvimento do quadril foi descrito em um paciente com anemia de Fanconi. Por causa da natureza heterogênea, um paciente com anemia de Fanconi deve ser submetido à avaliação pré-operatória para diagnosticar as características clínicas. Em conclusão, o bloqueio caudal foi a nossa escolha para esse paciente com anemia de Fanconi, sem trombocitopenia, para evitar o N2O, reduzir a quantidade de anestésico, a microcefalia existente, o hipotireoidismo e o aumento das enzimas hepáticas, proporcionar analgesia pós-operatória e reduzir a quantidade de analgésico usada no pós-operatório.

La anemia de Fanconi es un síndrome hereditario autosómico recesivo raro, caracterizado por deficiencia de la médula ósea y por anomalías congénitas y hematológicas. La literatura sobre el manejo anestésico de esos pacientes es limitada. El manejo de una displasia del desarrollo de la cadera fue descrito en un paciente con anemia de Fanconi. Debido a la naturaleza heterogénea, un paciente con anemia de Fanconi debe ser sometido a la evaluación preoperatoria para diagnosticar las características clínicas. En conclusión, el bloqueo caudal fue nuestra elección para ese paciente con anemia de Fanconi sin trombocitopenia para evitar el N2O, reducir la cantidad de anestésico, microcefalia existente, hipotiroidismo y aumento de las enzimas hepáticas, proporcionar analgesia postoperatoria y reducir la cantidad de analgésico usado en el postoperatorio.

Ocular findings in children with thalassemia major in Eastern Mediterranean.

AIM: To investigate ophthalmologic findings in children with thalassemia major (TM) and compare the findings with healthy controls. METHODS: In a cross-sectional study, 43 children with thalassemia major from pediatric hematology outpatient clinics from two university hospitals and age/sex matched 47 healthy children were included in the study. After a complete ophthalmic examination, tear function tests including the Schirmer test, fluorescein tear break-up time (BUT), ultrasound pachymetry, and axial length measurement were performed. Obtained data was recorded for statistical analysis and the values of right eyes were compared between groups. RESULTS: The mean best corrected visual acuity was 1.34±0.75 in TM and 1.08±0.28 in controls. It was found lower than 0.1 logMAR unit in 10 (23.2%) children with TM and 2 (4.2%) in controls, and the difference was statistically significant (P<0.05). The mean central corneal thickness was 540±26.95 in children with TM and 536.98±20.45µm in controls (P>0.05). The mean axial length was 22.53±0.50 in TM and 22.57±0.43mm in the control group. The mean Schirmer test score was 19.94±6.91 in TM and 24.22±3.95mm in the control group (P<0.01). The mean BUT score was 9.62±1.28 in TM and 9.73±0.6s in the control group (P>0.05). CONCLUSION: In TM, while corneal thickness, axial length, and BUT are close to controls, the Schirmer scores are less than normal. The study revealed that TM may be affected by the tear function and visual acuity.

Retinal fiber layer thickness in children with thalessemia major and iron deficiency anemia.

PURPOSE: We aimed to measure peripapillary retinal nerve fiber layer (RNFL) thickness in children with thalassemia major (tha-major), children with iron deficiency anemia (IDA), and children in a healthy control group. MATERIALS AND METHODS: A total of 47 children with tha-major and 22 children with IDA were selected from two pediatric hematology outpatient clinics as our experimental groups, while 35 healthy children were randomly selected from a primary school to act as a control group. After a complete eye examination was conducted and intraocular pressure measurements were obtained, RNFL measurements were performed using optical coherence tomography, and the information was recorded for statistical analysis. RESULTS: The mean age of the participants was 9.65 ± 4.13 years in the tha-major group, 9.14 ± 2.53 years in the IDA group, and 9.13 ± 3.29 years in the control group, respectively, with no statistically significant difference among the three groups (p > 0.05). Mean peripapillary RNFL thickness was 119.38 ± 35.49 microns in the tha-major group, 184.00 ± 31.14 microns in the IDA group, and 187.73 ± 27.36 microns in the control group. It was significantly thinner in all quadrants in the tha-major group vs. the other two groups (p < 0.01), and in only the inferior quadrant in the IDA group (p < 0.05). Average RNLF thickness correlated positively with mean hemoglobin value (r = 0.488; p < 0.001) and negatively with mean ferritin level (r = -0.544; p < 0.001), but no correlations with mean number of transfusions and mean visual acuity were observed (p > 0.05). CONCLUSION: The study revealed that peripapillary RNFL is thinner in tha-major in all quadrants and in only the inferior quadrant in IDA. Thinning of the RNLF correlated with hemoglobin value and ferritin level, but not with number of transfusions and visual acuity.

Successful treatment with enoxaparin of glans ischemia due to local anesthesia after circumcision.

The severe ischemia or necrosis of the glans penis is a rarely seen complication after circumcision. We report the case of a 7-year-old boy with severe glans penis ischemia occurring 24 hours after circumcision with local anesthesia (0.1% xylocaine containing ephedrine). His hemogram and levels of proteins C and S and fibrinogen were normal, but the D-dimer level was higher than normal (2.57 mg/L; normal level 0-0.5 mg/L). Normal blood flow was indicated on color Doppler ultrasonography. The severe ischemia in the glans penis was successfully treated with subcutaneous injection of enoxaparin (low-molecular-weight heparin) for 5 days, and the appearance of the glans was close to normal on the fifth day. The patient did not require any surgical intervention and was discharged without sequelae. As a result, we consider that ischemia of the glans penis may initially be managed with enoxaparin, especially in cases in which the D-dimer level is higher than normal.

Infantile hemangiomas, complications and follow-up.

OBJECTIVE: To study the risk factors for hemangioma-related complications, treatment indications and analyze the outcome of patients with infantile hemangioma. DESIGN: Retrospective. SETTING: University hospital. PATIENTS: Fifty-five patients (1-69 months; median: 12 months) with infantile hemangioma with mean follow-up 19 months. The eligibility was based on the criteria of the International Society for the Study of Vascular Anomalies (ISSVA). INTERVENTION: The surgical treatment included total excision whereas medical treatment was carried out by interferon and /or corticosteroids. MAIN OUTCOME MEASURES: Data was collected including sex, age, prematurity, age at onset, number, anatomic location and size of hemangioma, age at treatment, cause of treatment decision, family history, presence of extra malformations, involvement of internal organs, presence of life altering or life threatening complications, response to treatment, dose and duration of medications, complications associated with treatment, follow-up period, and final outcome. RESULTS: Thirty-four (62%) patients were followed-up without treatment, whereas 21 others underwent treatment including steroids, interferon, and surgery. The size of hemangioma was a major factor that predicted hemangioma-related complications (P=0.002). Patients with hemangioma related complications had bigger lesions (size >40 cm² or the longest size on a single plane >5 cm). Nineteen patients (34%) had complications, but only 8 (14.5%) out of them had life or function-threatening complications. CONCLUSION: Although dosing and treatment protocol is still debatable, steroids and interferon are good options for hemangioma treatment. The management strategy should be individualized for each case.

Decreased vitamin b(12) levels in children with nocturnal enuresis.

Objectives. Nocturnal enuresis is a common pediatric problem, the etiology of which is unclear. In the present study, vitamin B(12) and folate levels were measured in children with nocturnal enuresis and compared with those in healthy control group children to investigate whether there was any relation with enuresis and neurogenic maturation as a first time in the literature. Methods. In this cross-sectional study, we included thirty children (16 girls, 14 boys) who had presented with primary nocturnal enuresis (PNE) complaints in the study group and 31 children (13 girls, 18 boys) in the control group. Body weight and height measurements were obtained and complete blood counts and vitamin B(12) and folate levels were measured in all children. Results. No difference was found in age, height, and weight between study and control groups. Also the mean levels of the hemoglobin, hematocrit, and mean corpuscular volume (MCV) were not different between the two groups. Significantly lower mean vitamin B(12) and folate levels were found in the enuresis group compared with the control group. Conclusions. Further studies are needed to clarify B(12) and folate deficiency in larger series so that these tests can be included in routine investigations of enuretic children.

Hand grip and pinch strength in patients with nocturnal enuresis: is there a role of muscle strength in pathogenesis of enuresis?

AIMS: To examine the association of muscle strength with nocturnal enuresis. SUBJECTS AND METHODS: One hundred sixty-three patients with monosymptomatic nocturnal enuresis (8-14 years old) were recruited from outpatient clinics. Two hundred eight healthy students served as the control group (8-14 years old) from two primary schools. Physical examinations and the age, gender, height, weight, dominant hand, and body mass of these children index were recorded. A calibrated, Jamar dynamometer was used to assess grip strength at the first two settings. A pinch gauge was used to assess the key pinch of the right and left hands of these children. Two measurements of each grip and pinch were obtained at 15 sec intervals and mean values were analyzed. RESULTS: The hand grip strength test and pinch was significantly lower in the enuretic group compared with the control group. These low results of the muscle strength in the enuretic group showed that whole body muscle weakness may contribute to the pathogenesis of enuresis as a possible etiological factor. CONCLUSIONS: In this first study on the association of muscle strength with nocturnal enuresis, it was found that neuromuscular function is abnormal in enuretic patients. In this neuromuscular abnormality, the motor activity is affected.