Mecnun Çetin

Assessment of pulmonary artery pressure and right ventricular function in children with adenotonsillar hypertrophy using different parameters

OBJECTIVE Our aim was comparison of preoperative and postoperative right ventricular functions of children with adenotonsillar hypertrophy (ATH) who have findings of upper airway obstruction, using new echocardiographic parameters. METHODS Forty-one children who have admitted to our hospital with symptoms suggestive of upper airway obstruction, whose history and physical examination findings suggest upper airway obstruction and who have undergone adenoidectomy/adenotonsillectomy and 40 healthy children, all of whom between 2 and 12 years of age, were included in the study. Patient group was evaluated by pulsed wave tissue Doppler echocardiography as well as with conventional echocardiography before the operation and 6 months after the operation. RESULTS Of 41 children in study group, 26 (63.4%) had adenotonsillectomy and 15 (36.6%) had adenoidectomy. Tricuspid annular plane systolic excursion (TAPSE) was significantly lower in preoperative group compared to control group (18.46±1.67, 19.77±1.62; p=0.000, respectively). Myocardial performance index (MPI) was significantly higher in preoperative group than postoperative and control group (0.40±0.07, 0.36±0.06, 0.35±0.07; p=0.032, respectively). Tricuspid isovolumic acceleration (TIVA) was significantly lower in preoperative group than preoperative and control group (2.97±0.8, 3.43±0.7, 3.43±0.9; p=0.020, respectively). Disappearance of this difference was found between postoperative and control groups (p=0.984). Pulmonary acceleration time (PAcT) was found to be significantly lower in preoperative group compared to postoperative and control group (109.68±18.03, 118.93±17.46, 120.0±14.07; p=0.010, respectively). Mean pulmonary artery pressure (mPAP) was significantly higher in preoperative group than control group (29.64±8.11, 24.95±6.33; p=0.010, respectively). In postoperative group mPAP was found to be similar to control group (25.48±7.85, 24.95±6.33; p=0.740, respectively). CONCLUSIONS TAPSE, PAcT, MPI and TIVA are useful markers for evaluation of preoperative and postoperative ventricular function in children with ATH who have findings of upper airway obstruction. We think that using these practical and easy-to perform parameters may be relevant for evaluation and postoperative follow-up of patients with ATH who have findings of upper airway obstruction. Besides adenotonsillectomy is a beneficial treatment option for these patients.

Obez çocuklarda sol ventrikül fonksiyonlarının değerlendirilmesinde doku doppler ekokardiyografi

Objective: The aim of this study was to investigate the impact of childhood obesity on ventricular functions using tissue Doppler echocardiography. Materials and methods: The mitral tissue Doppler signals were recorded in the apical four-chamber view, with the sample volume placed at the lateral walls of mitral annulus. The mitral flow was examined with the sample Pulse Dop pler positioned parallely to flow just at the orifice of the mi tral leaflets. Twenty obese (mean age, 161.5±25.8 months) and 20 healthy children (mean age, 151.2±33.5 months) were included to this study. Results: Body mass index (BMI) was significantly higher in obese group (30.92±6.87). Isovolumic relaxation time (IVRT) which is one of the left ventricular (LV) diastolic function parameters was 66.10±7.30 ms in obese group, and 58.70±9.06 ms in the control group. IVRT was significantly higher in the obese group (p=0.007). In obese group, the mitral annulus tissue Doppler E velocity was decreased, flow velocity was increased and decrement in Em/Am ratio was significant. We found significant negative correlation between BMI and LV Em/Am ratio. Myocardial performance index (MPI) in obese group and control group was 0.50±0.07 and 0.41±0.04, respectively (p<0.001). Between MPI and LV Em/Am ratio there was a significant negative correlation while MPI showed positive correlation with BMI. In obese group septal and LV posterior wall thickness was shown to be significantly increased compared to the control group (p<0.001). Conclusion: Our findings, may be important for determina tion of the relationship between obesity and cardiovascular risk factors in children. Tissue Doppler echocardiographic imaging may be useful in revealing this relationship.

Partial ileus associated with vincristine: A case report

Vinkristin; lenfoma ve lösemi gibi bir çok malignitenin kemoterapi protokolünde sıklıkla kullanılan hücre siklusuna oldukça spesifik bir vinka alkaloididir. Vinkristin nörotoksisitesi; periferal nöropati, otonomik nöropati, kranial sinir paralizileri ve ensefalopati olmak üzere dört farklı şekilde karşımıza çıkabilmektedir. Bu yan etkiler vinkristin kullanımında sınırlayıcı faktörler olarak rol oynar (1). Hodgkin lenfoma tanısıyla vinkristin tedavisi sonrası parsiyel intestinal obstrüksiyon gelişen ve medikal olarak tedavi edilen 13 yaşındaki bir kız olguyu sunuyoruz.

Chediak–Higashi syndrome presented with hemophagocytic lymphohistiocytosis

© 2018 Indian Journal of Medical and Paediatric Oncology | Published by Wolters Kluwer Medknow Sir, Herein, we report a case of Chediak–Higashi syndrome (CHS) presented with hemophagocytic lymphohistiocytosis (HLH). A 3‐year‐old girl presented with recurrent infections, fever, and icterus. Physical examination revealed hepatosplenomegaly, bilateral cervical and inguinal lymphadenopathy, silvery gray hair, strabismus, and bilateral nystagmus. Giant granules in lymphocytes and monocytes were seen on blood smear. Bone marrow aspirate exhibited erythrophagocytosis and numerous giant granules of predominantly myeloid lineage [Figure 1a]. Examination of the hair showed an irregular distribution of large and small pigment clumps [Figure 1b]. Magnetic resonance imaging of the brain showed diffuse cerebral atrophic [Figure 1c]. The girl’s parents were consanguineous. On laboratory examination, urinary analysis was unremarkable. Hemoglobin, white blood cell count, and thrombocyte count were 7, 6 g/dL, 2300/mm3, and 76.000/mm3, respectively. Sedimentation rate was 2 mm/h (N: 0–20 mm/h), and serum triglyceride 390 mg/dL (N: 32–99 mg/dL), fibrinogen 67 ng/dL (N: 200–400 ng/dL), and ferritin was 129.000 ng/mL (N: 7–140 ng/mL) [Table 1]. Prothrombin time was 21 s (N: 11–15 s), and activated partial thromboplastin time was 62 s (N: 25–35 s).

Evaluation of cardiac autonomic functions in Children with Thalassemia Trait, Iron Deficiency Anemia and Iron Deficiency

Iron deficiency (ID) is at the most encountered nourishment deficiency in the world and 15-20% of the whole population has iron deficiency anemia (IDA). The most dramatic health consequence of severe anemia, which is well documented, is increased risk of child mortality. ID leads to the neuromotor and cognitive deterioration, developmental delay even in the absence of anemia (1-3). In thalassemia trait (thalassemia minor), which is another prevalent hypochromic microcytic anemia, blood values are similar to those in IDA. These patients are often wrongly diagnosed as IDA and treated for ID in vain. In thalassemia trait (TT), unlike IDA, red cell distribution width (RDW), free erythrocyte protoporphyrin, ferritin, serum iron and total iron binding capacity (TIBC) is normal. The final diagnosis is established by determining the increased HbA2 in hemoglobin electrophoresis (4). Heart rate variability (HRV) corresponds to the change in interval of heart beat and it is a physiological condition. HRV is accepted as one of the principal predictive marker of autonomic nervous function of the heart and it has been examined for the last twenty years. The HRV measurement which is a non-invasive and easy technique to apply has been extensively studied. The variation of HRV with regards to the time and frequency has been shown to be comprehensively related with the autonomic nervous function of the heart. It is a well-known and described data that HRV is related with the cardiovascular disorders and mortality (5-7).

Asymmetric dimethylarginine levels and diabetes duration: Relationship with measures of subclinical atherosclerosis and cardiac function in children and adolescents with Type 1 diabetes:

Aims: We aimed to evaluate asymmetric dimethylarginine levels in young patients with Type 1 diabetes mellitus according to diabetes duration and to examine the relationship between these levels and measures of atherosclerosis and myocardial function. Materials and methods: In total, 83 patients (8.5–22 years) with Type 1 diabetes mellitus were stratified by diabetes duration: 12–60 months (Group 1, n = 27), >60–120 months (Group 2, n = 29) and >120 months (Group 3, n = 27). Asymmetric dimethylarginine levels were assessed. Carotid intima–media thickness was measured. Myocardial function was assessed by M-mode, conventional Doppler and tissue Doppler echocardiography. Results: Asymmetric dimethylarginine level was significantly higher in Group 1, while carotid intima–media thickness was significantly greater in Group 3 (p < 0.05). Tissue Doppler echocardiography showed the ratio of peak early to peak late diastolic myocardial annular velocity decreased significantly in Groups 2 and 3 with a negative correlation with duration (r: −0.310, p = 0.004) and HBA1c levels (r = −0.391, p < 0.001). Myocardial performance index in all groups and isovolumic relaxation time in Group 3 increased significantly. Asymmetric dimethylarginine levels were negatively correlated with carotid intima–media thickness and isovolumic relaxation time (p < 0.05). Conclusion: In contrast to adult diabetics, asymmetric dimethylarginine concentration decreases as diabetes duration increases in young Type 1 diabetic patients and is associated with worsening measures of cardiovascular risk and poorer diastolic function.

A Rare Case of Asymptomatic Giant Right Atrial Aneurysm: Case Report

Right atrial aneurysm is a rare abnormality of the heart. This defect is seen in all age groups and is usually diagnosed incidentally. The aneurysm can be asymptomatic or present with symptoms related to supraventricular arrhythmias or thromboembolic phenomenon. We present the case report of a 74-year-old male with right atrial aneurysm which was incidentally detected during echocardiographic evaluation.

Wiskott–Aldrich syndrome: Two case reports with a novel mutation

ABSTRACT Background: The Wiskott–Aldrich syndrome (WAS) is X-linked recessive disorder associated with microplatelet thrombocytopenia, eczema, infections, and an increased risk of autoimmunity and lymphoid neoplasia. The originally described features of WAS include susceptibility to infections, microthrombocytopenia, and eczema. Aim: In this case report, we present our experience about two cases diagnosed with a new mutation. Methods: We report phenotypical and laboratory description of two cases with WAS. Results: We, for the first time, detected a new hemizygote mutation of WAS gene (NM_000377.2 p.M393lfs*102 (c.1178dupT)) in two patients. The first case was an 11-month-old boy presenting with complaints of recurrent soft tissue infection, ear infection, anemia, and thrombocytopenia with a low platelet volume. The second case was a 2-month-old boy presenting with thrombocytopenia and a low platelet volume. Both cases were the first-degree relatives: they were cousins and their mothers were sisters. Conclusion: Herein, we report two cases of WAS and a new gene mutation which would disrupt the WAS protein function within the Polyproline (PPP) domain. This report adds to the growing number of mutations which cause complex clinical manifestations associated with WAS.

Hydatid cyst involvement of both pulmonary arteries in a 14-year-old girl

Abstract Hydatid disease is the most common mediterranean parasitic infection; it commonly affects the liver and lungs and rarely involves multiple organs. A 14-year-old girl presented with a 1-year history of dyspnoea and fatigue. She was found to have pulmonary hypertension owing to hydatid cysts in the right ventricle and both pulmonary arteries. After administration of albendazole she underwent pulmonary endarterectomy and a calcified hydatid cyst was removed from the right ventricle. She made a complete recovery and remained on albendazole for 6 months.

Idiopathic Chest Pain in Children: Is it Gastroesophageal Reflflux Disease?

ABS TRACT Objective: Causes of chest pain in children, rarely due to cardiac disease and usually described as idiopathic, account for 20% to 45% of all cases. In this study, we investigated the frequency of gastroesophageal reflux in children with noncardiac chest pain and the characteristics of the pain. Material and Methods: Children with at least two episodes per month of chest pain for a maximum of six months were enrolled in the study. After diagnostic evaluation excluded a cardiac source of chest pain, all patients underwent 24-hour pH monitoring. Characteristics of chest pain and other symptoms were recorded daily by the patients or their parents using the symptom diary. Lansoprazole treatment was administered to patients diagnosed as having gastroesophageal reflux disease. Then, the results of the lansoprazole treatment were evaluated on the basis of the presence of chest pain and other symptoms at the second month after the completion of therapy. Results: Thirty-one children (17 boys and 14 girls) aged between eight and 18 years (11.6±2.4 years) with the primary complaint of chest pain were studied. Based on the 24-hour pH monitoring test results, eight patients (25.8%) for distal sensor and six patients (19.4%) for proximal sensor were defined as Gastroesophageal Reflux Disease (GERD)-positive. No significant difference was noted for characteristics of chest pain and other symptoms between the GERD-positive and GERD-negative groups. All GERD-positive children became symptom-free after the two-month lansoprazole therapy. Conclusion: The symptoms of GERD-induced chest pain are often non-specific, and can be determined with certainty only by 24-hour pH monitoring, so 24-hour pH monitoring may proposed to all children with idiopathic chest pain.