Takotsubo (stress) cardiomyopathy in childhood.

Takotsubo cardiomyopathy (TC) is a significant cause of acute heart failure in adults. However, triggering factors, ECG and echocardiographic findings, and prognosis of TC have not been well studied in children. Therefore, this study aimed to evaluate the demographic characteristics, signs and symptoms, laboratory findings, and prognosis of children with TC. We analyzed demographic characteristics of childhood TC cases (aged < 18 years) treated at our university hospital from 2014 to 2019. The triggering factors of TC; outcomes of the disease; and laboratory, ECG, and echocardiographic findings at presentation were also examined. A total of 17 patients (mean age 9.71 ± 5.03 years, 52.9% female) were included in the study. Chest pain, palpitation, and dyspnea were the most common presenting symptoms. The most common triggering factor was emotional stress (47%) in our study population. Ten patients (58.8%) had a classical form of TC, with apical left ventricular wall motion abnormalities. None of the patients died, and 15 (88.2%) had complete recovery of left ventricular systolic function. Only two patients (11.8%) had a left ventricular ejection fraction < 50% at the end of follow-up.Conclusion: This is the first retrospective data on childhood TC. The majority of patients with TC experienced an emotional stress as a trigger of the disease, and nearly 90% of the patients completely recovered.What is Known? • The occurrence of acute heart failure following an emotional or physical stress is called Takotsubo cardiomyopathy (TC). • Takotsubo cardiomyopathy is rarely reported in childrenWhat is New? • The present study is the first case series of children with TC. • In contrast to the female predominance in adult population, TC was almost equally distributed between boys and girls in the pediatric age group.

Assessment of anxiety-depression levels and perceptions of quality of life in adolescents with dysmenorrhea.

BACKGROUND: This study aimed to assess the anxiety-depression levels and the perceptions of quality of life, as well as the factors affecting these variables, in adolescents with dysmenorrhea. METHODS: The participants included 60 adolescents with dysmenorrhea and 41 healthy adolescents between the ages of 12 and 18. This study used the Pediatric Quality of Life Inventory (PedsQL) for assessing the perceptions of quality of life, the State-Trait Anxiety Inventory (STAI) for measuring anxiety levels, and the Children's Depression Inventory (CDI) for measuring depression levels. RESULTS: It was determined that compared to healthy controls, the depression and anxiety scores were higher and the quality of life was impaired in adolescents with dysmenorrhea. In addition, it was shown that the depression and anxiety levels increased and the psychosocial health subscale scores of quality of life decreased with increasing dysmenorrhea severity. However, the likelihood of dysmenorrhea was found to be higher with increasing depression scores, while the anxiety levels had no effect on dysmenorrhea. CONCLUSION: In dysmenorrhea management, it is important to enhance awareness among pediatric clinicians and gynecologists regarding the associations between dysmenorrhea and mental problems.

The Prevalence of Hemoglobinopathies in Young Adolescents in the Province of Mugla in Turkey: Results of a Screening Program.

Thalassemia is an autosomal recessive inherited blood disorder. It is prevalent in Mediterranean countries such as Sardinia, Greece, Cyprus, Turkey, Lebanon and also Southeast Asia. Our aim was to investigate the carrier prevalence of thalassemia and other hemoglobinopathies in adolescents who live in Mugla Province, Turkey. We analyzed retrospectively the surveys conducted at primary schools between 1997 and 2013. Complete blood count (CBC) and high performance liquid chromatography (HPLC) were used to screen for thalassemia and hemoglobinopathies. Patients were diagnosed as having thalassemia trait if the mean corpuscular volume (MCV) was ≤ 80.0 fL, mean corpuscular hemoglobin (Hb) was ≤ 27.0 pg and Hb A2 levels were ≥ 3.5%. A total of 164,814 students were analyzed. The median age of the students was 13.5 years (minimum 13.0, maximum 14.0). The total number of students with abnormal HPLC results was 5861 (3.8%). There was a significant decrease in the newborn of new thalassemia patients found with screening programs for hemoglobinopathies in Mugla Province from 1997 to 2013. The number of students with abnormal HPLC results for thalassemia, sickle cell disease and other Hb traits were 3.2, 0.15 and 0.4%, respectively. It is important to recognize that including Hb, MCV, red blood cell (RBC) count and HPLC tests for carrier screening are necessary to find hemoglobinopathies. Our study supported that the number of new patients significantly decreased using these screening programs from 1997 to 2013.

Anaphylaxis related with positively charged white-cell reduction filters.

Allergic reactions related to blood transfusion frequently occur and most of them are mild reactions such as urticaria, erythema, pruritus and flushing. More severe and life threatening allergic reactions such as anaphylactic shock rarely occur. Application of white cell reduction filters during transfusions may prevent alloimmunization, febrile nonhemolytic reactions and transmission of intracellular infectious agents. Despite their beneficial effects, white-cell reduction filters may cause allergic reactions. In this article we present three patients who had anaphylactic reactions during blood transfusion with positively charged leucocyte filters.

Prevalences of Allergic Disorders in Children with Terra Firma-Forme Dermatosis.

BACKGROUND: Terra firma-forme dermatosis (TFFD) is a clinical condition that may be defined as a dirty appearance of skin. Although it has been defined for many years, its clinical value is not well known. OBJECTIVE: We aimed to determine the prevalence of allergic disorders (asthma, allergic rhinitis, and eczema) to investigate if this clinical condition is associated with allergic disorders in children with TFFD. MATERIALS AND METHODS: A questionnaire descriptive of allergic disorders [International Study of Asthma and Allergies in Children (ISAAC)] was applied to all children diagnosed with TFFD at the pediatric clinics during a 6-month period specified for the study. The results were compared with the two ISAAC studies that have been previously conducted in our region. RESULTS: The prevalence of TFFD among 1695 children examined at our outpatient clinic was found to be 3.18% (n = 54). The youngest of the children was 6 months old and the oldest 13 years, with an average age of 6.08 ± 2.69 years. Our study group had significantly greater rates and intensities of lifetime wheezing, wheezing in the last 12 months (current wheezing), lifetime allergic rhinitis, allergic rhinitis in the last 12 months; and the rate of physician-diagnosed allergic rhinitis compared to the comparator groups (P < 0.005). CONCLUSIONS: The results support the view that TFFD may be a sign of associated asthma and allergic rhinitis.

Olfactory Dysfunction in ß Thalassemia Major Patients Treated With Iron-Chelating Agents.

Ocular and ophthalmological adverse effects may be seen in ß-thalassemia major (BTM) patients treated with regular blood transfusions and iron-chelating agents. We hypothesized that olfactory dysfunction may be present in this population. In this study, we aimed to investigate olfactory dysfunction in patients with BTM and determine the etiological factors. A total of 43 patients with BTM were included in the study. Forty-three patients without any nasal complaints, history of facial trauma, or nasal surgery were included as the controls. All participants had nasal endoscopy. The iron-chelating agents used, their duration of use, as well as hemoglobin and ferritin levels of the BTM patients were recorded. Sniffin' Sticks test (SST) was used to assess olfactory functions, and BTM and control groups were compared for the results. The correlations of SST scores with the other study parameters were analyzed. Eight (18.6%) of 43 patients in the BTM group had hyposmia while none of the patients in the control group had hyposmia (P < .001). Older age, low-hemoglobin level, and longer use of deferoxamine were found to be correlated with olfactory dysfunction. Olfactory dysfunction may be seen in BTM patients treated with iron-chelating agents. The results of this study suggest that screening for olfactory function may be needed in routine follow-up of BTM patients.

Olfactory dysfunction in ß-thalassemia major patients treated with iron-chelating agents.

Ocular and ophthalmologic adverse effects may occur in patients with ß-thalassemia major (BTM) treated regularly with blood transfusions and iron-chelating agents. We hypothesized that olfactory dysfunction may be present in this patient population. We aimed to investigate olfactory dysfunction in patients with BTM and to determine etiologic factors. A total of 43 patients with BTM were included in the study. Forty-three subjects without nasal complaints, history of facial trauma, or nasal surgery were included as the controls. All participants had nasal endoscopy. The use of iron-chelating agents by patients with BTM and their duration of use were recorded, as well as hemoglobin and ferritin levels. The Sniffin' Sticks test (SST) was used to assess olfactory function, comparing results between the BTM and control groups. The correlations of SST scores with the other study parameters were analyzed. Eight (18.6%) of 43 patients in the BTM group and none of the subjects in the control group had hyposmia (p < 0.001). Older age, low hemoglobin level, and longer use of deferoxamine were found to be correlated with olfactory dysfunction. Olfactory dysfunction can occur in patients with BTM treated with iron-chelating agents. The results suggest that screening for olfactory function should be part of the routine follow-up of patients with BTM.

The Incidence of Ototoxicity in Patients Using Iron Chelators.

OBJECTIVE: In this study, we aimed to detect the incidences of ototoxicity in patients with hemoglobinopathies taking deferoxamine (DFO), deferiprone, and deferasirox using the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events (CTCAE) scale to obtain more objective data. MATERIALS AND METHODS: Fifty-five transfusion-dependent patients were evaluated in this study. The NCI CTCAE scale was used to assess ototoxicity levels. The average ferritin and hemoglobin levels, the type of iron chelator, and the duration of therapy of all the patients were recorded. RESULTS: Ototoxicity was observed in 15 patients (31.9 %), all of whom were taking DFO. The median age was 19.5 (6-43) in patients without ototoxicity and 29 (16-50) in those with ototoxicity; this difference was statistically significant (p<0.05). The median ferritin and pre-tx Hb levels were 1391 ng/mL and 9.06 mg/dL, respectively, in patients with ototoxicity and 986.7 ng/mL and 9.24 mg/dL, respectively, in those without ototoxicity; these differences were not significant (p>0.05). Ototoxicity was not observed in the eight patients who used only deferasirox and deferiprone. CONCLUSION: The ototoxicity incidence with DFO at doses below 50 mg/kg/day was 27.3%. Deferiprone and deferasirox were not associated with ototoxic effects in patients taking these drugs.

The Place of Complementary Medicine in the Treatment of Autistic Children.

OBJECTIVES: The purpose of this study is to achieve a vision for autistic children and their parents aimed at generating interest in ideas such as "Sanitas Per Aquam" (SPA), massage and music therapy, which has begun to have widespread use and to attract attention. METHODS: This cross-sectional, descriptive study was carried out with autistic children and their parents from February to April 2015 in Mugla, Turkey. The study was began by interviewing experts in the field and by developing a suitable assessment questionnaire. In order to direct the flow of conversation between the researchers and the autisitc children and their parents, the researchers conducted semi-structured face to face interviews in a form that had been determined by using reports in the literature and the opinions of experts in the field. RESULTS: Forty two boys (84%) and eight girls (16%) with autism participated in our study. Children in the 0 ─ 7 age group spent long time in the bathroom (P = 0.001). Boys liked to be hugged more than girls (P = 0.01). Children ages 0 ─ 7 years liked bright lighting while those 15 years of age and older liked gloomy lighting (P = 0.009). Except for these statistically significant sex- and age-related differences, no other statistically significant differences were noted in the parameters of this study. Although the result was not statistically significant, more children with mild autism disorder obeyed commands like inhale or exhale (P = 0.051). CONCLUSION: Treatment for autism spectrum disorders is not yet fully possible, so many studies are being done to alleviate some symptoms and to improve the quality of life for individuals with autism and their families. As a result of our study, whether touching the areas the children want touched and listening to their favorite music are required to stimulate the brain remain as questions in our minds.

Silibinin attenuates methotrexate-induced pulmonary injury by targeting oxidative stress.

The aim of the present study was to assess the protective effect of silibinin against methotrexate (MTX)-induced pulmonary toxicity. Rats were divided into four groups (MTX, MTX + silibinin, silibinin and control. MTX was injected intraperitoneally (i.p) into female Wistar rats (10 mg/kg/day for 3 days), which resulted in significant increases in the serum levels of alanine aminotransferase, aspartate aminotransferase and oxidant enzymes, including nitric oxide and myeloperoxidase. Furthermore, significant reductions were detected in the serum activity levels of the antioxidative enzymes, glutathione peroxidase and superoxide dismutase, when compared with the control group. However, administration of silibinin (100 mg/kg/day for 10 days, i.p.) was shown to ameliorate the MTX-induced pulmonary toxicity, as indicated by the normalization of the oxidative stress parameters. Furthermore, silibinin treatment was demonstrated to reduce the histopathological changes associated with MTX. In conclusion, silibinin exhibited protective effects against MTX-induced pulmonary toxicity, which may be attributed to its antioxidant activity.

Assessment of Myocardial Performance Index and Aortic Elasticity in Patients With Beta-Thalassemia Major.

BACKGROUND: This study aimed to assess myocardial performance index (MPI) and arterial elasticity indices in asymptomatic patients with beta-thalassemia major without known heart disease and to determine relationship between these indices and parameters indicating iron load of body. METHODS: The study included 55 asymptomatic beta-thalassemia patients (median age: 20 years (10 - 48 years)) without known history of heart disease and 40 age- and sex-matched healthy controls. MPI and arterial elasticity indices were determined by using standard two-dimensional and Doppler echocardiography. Data were analyzed by SPSS for Windows version 20.0 (SPSS Inc., Chicago, IL, USA). RESULTS: Left ventricular mass index (83.917 (50.62 - 144) and 68.37 (41.9 - 113.3)) and MPI (0.464 (0.33 - 0.68) and 0.431 (0.31 - 0.51)) were significantly higher in patients with beta-thalassemia when compared to control group (P < 0.001 and P = 0.006). Aortic elasticity indices were significantly higher while aortic strain and distensibility values were significantly lower in patients with beta-thalassemia compared to controls (all P values < 0.001). Positive correlations were detected between aortic stiffness index and platelet (r = 0.235; P = 0.019) and ferritin values (r = 0.328; P = 0.008). Presence of thalassemia (ß = -0.729; P = 0.041) and higher platelet value (ß = 0.235; P = 0.019) were significant determinants for increased aortic stiffness in linear regression analysis. CONCLUSION: Arterial elasticity indices and MPI are impaired in patients with beta-thalassemia major and these parameters may be used to predict cardiovascular complications in asymptomatic patients with beta-thalassemia major.

Evaluation of the protective effect of silibinin in rats with liver damage caused by itraconazole.

Itraconazole (ITZ) belongs to the triazole group of antifungals with potent keratinophilic and lipophilic features. Hepatotoxicity is one of its most remarkable features. Silibinin (SIL) is a plant used worldwide which is used in the treatment of many liver diseases and it is especially very well known for its hepatoprotective-cytoprotective effect. The aim of our study was to research the protective effect of SIL in ITZ-induced hepatotoxicity using biochemical and pathological tests. Liver enzymes and antioxidant enzyme activities were measured spectrophotometrically by using commercial kits. ALT and AST levels in ITZ group were significantly increased compared to the group, while the activities of GSH-Px and SOD had decreased (p < 0.05). When ITZ group was compared to ITZ + SIL group, AST, ALT, and levels of NO and MPO were significantly decreased, while the activities of GSH-Px and SOD were increased (p < 0.05). Histopathological evaluation showed that SIL significantly decreased periportal inflammation and parenchymal hepatocyte apoptosis in ITZ and ITZ + SIL groups (p < 0.05). Eventhough not statistically significant, partial improvement with the use of SIL has been detected (p > 0.05) in hepatocyte degeneration and multinuclear giant cell formation. According to the evaluation performed with comet assay method, ITZ leads to DNA damage, and the use of SIL significantly decreases DNA damage (p < 0.05). We have detected that the use of ITZ increases oxidative stress (MPO, NO), decreases antioxidant activity (SOD and GSH-Px), and leads to DNA damage and histopathological liver damage, whereas the use of SIL has a cytoprotective effect on the liver by increasing the antioxidant effect (SOD, GSH-Px) and by decreasing the oxidative stress (NO, MPO). ITZ causes the generation of ROS and leads to DNA damage and liver damage. SIL has a cytoprotective effect on the liver by increasing antioxidant enzyme activities, preventing the formation of ROS.

Evaluation of the protective effect of silibinin against diazinon induced hepatotoxicity and free-radical damage in rat liver.

BACKGROUND: Diazinon (0,0-Diethyl 0-(1-6-methyl-2-isoprophyl 4 pyrimidinyl) phosphorothioate) (DI) is a very effective organophosphate pesticide, used widely in agriculture. Consequently, data on poisoning cases secondary to DI exposure are important. The DI may affect a variety of tissues, including liver. Silibinin is a pharmacologically active constitute of Silybum marianum, with documented antioxidant activity. OBJECTIVES: The aim of our study was to evaluate both histopathologically and biochemically whether silibinin is protective in DI induced liver damage. MATERIALS AND METHODS: Thirty two Wistar albino rats were divided into four groups, as follows: 1) control group - oral corn oil was given; 2) DI group - rats were administered orally 335 mg/kg in the corn oil solution; 3) Silibinin group - 100 mg/kg/day silibinin was given alone orally, every 24 hours for 7 days; 4) Silibinin + DI group - DI plus silibinin was given. All rats were sacrificed at the end of experiment. Superoxide dismutases (SOD), glutathione peroxidase (GPX), nitric oxide (NO) and myeloperoxidase (MPO) were investigated in serum and liver tissue. In addition, serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) enzyme activities were evaluated. The liver tissue was evaluated histopathologically with Hematoxilin & Eosin dye. RESULTS: Biochemically, ALT, AST, NO, MPO in serum and NO, MPO in liver tissue were found to be significantly higher in DI group, compared to control group (P < 0.001). In Group Silibinin + DI, serum AST, ALT, NO, MPO levels were significantly lower (P < 0.01), and both serum and tissue SOD activities were significantly higher, compared to DI group (P < 0.001). Diazinon induced histopathological changes in liver tissue were: severe sinusoidal dilatation, moderate disruption of the radial alignment of hepatocytes around the central vein, severe vacuolization in the hepatocyte cytoplasm, inflammation around central vein and portal region. In rats receiving both DI and silibinin, the DI induced changes accounted for less sinusoidal dilatation, vacuolization in the hepatocyte cytoplasm and the inflammation around central vein and portal region (P < 0.05). CONCLUSIONS: The DI was found to induce liver damage by oxidative stress mechanisms. Silibinin reduced the oxidative stress by inducing antioxidant mechanisms, thereby showing protective effect against DI induced liver damage. Further studies with silibinin should be performed regarding DI toxicity.

A thalassaemia intermedia case with concomitant left atrial thrombus.

Although marked improvements have been observed in the life expectancy of patients with thalassaemia by regular blood transfusion and strict iron chelation therapies in recent years, these patients still have to deal with several complications, mainly cardiovascular. One of the life-threatening complications is the chronic hypercoagulable state and thromboembolic events which develop due to haemostatic alterations in patients with thalassaemia, although they are more frequently seen in those with thalassaemia intermedia. Many thromboembolic complications, mainly deep venous thrombosis and cerebral thrombosis, have been reported in thalassaemia. However, intracardiac thrombosis is rarely seen. In this manuscript, we presented a case that underwent splenectomy 6 years ago but not regularly attended the control visits and presented to our clinic with gradually increasing exercise dyspnoea for 2 months.

Toxic methemoglobinemia treated with ascorbic Acid: case report.

Methemoglobinemia is a disorder characterized by the presence of a higher than normal level of methemoglobin. Prilocaine which is one of the oxidizing local anaesthetics is widely used in many local procedures. The first choice of treatment of complications due to the use of these local anaesthetics is methylene blue, while ascorbic acid is the alternative choice. The side effects of metilen blue restrict its usage in some special conditions. Ascorbic acid is a good alternative drug with limited experience in methemoglobinemia. We present a case of a methemoglobinemia treated with ascorbic acid successfully to emphasize the use of ascorbic acid as an alternative method.

Evaluation of shigellosis in a Turkish children's hospital.

BACKGROUND: The aim of the present study was to evaluate cases of Shigella and determine the pattern of antimicrobial resistance of shigella species in central Turkey. METHODS AND RESULTS: One hundred and ninety-eight patients with shigella gastroenteritis presenting to Sami Ulus Children's Hospital from June 2002 to December 2002 were included in the study. The clinical and laboratory data of the patients with shigellosis were collected by chart review. Male/female ratio was 1.35 and mean age was 4.83 +/- 3.2 years (range: 1-16 years). Shigella sonnei (83.3%) was the most common serogroup. This was followed by S. flexneri (10.1%), then S. dysenteria (5.1%), and S. boydii (1.5%). The antibiotic susceptibility of the shigella strains was determined. Most strains of Shigella species were resistant to trimethoprim-sulfamethoxazole (90.4%). All strains were found to be sensitive to ciprofloxacin (100%). Ampicillin susceptibility was 86.4% and cefotaxim (and/or ceftriaxon) susceptibility was 98%. CONCLUSION: Ampicillin is the drug of choice in the treatment of shigella infection in this region of Turkey. Cefotaxim or ceftriaxon was the second choice of antibiotics. Thus, Shigella is an important etiologic agent of diarrhea in Turkey. To determine the antibiotic susceptibility of the Shigella strains and appropriate empiric antibiotic treatment in Turkey, further studies will be needed.

Is it a new syndrome or a clinical variability in cerebro-oculo-nasal syndrome?

We present a male infant 2.5-months old with asymmetric skull, anophthalmia, apparent hypertelorism, abnormal nares, unilateral cleft lip and palate, and structural abnormalities of the central nervous system. These findings are similar to cerebro-oculo-nasal syndrome except for the appearance of nose. This case is either a clinical variability in cerebro-oculo-nasal syndrome or a new entity.