Assessment of cardiac functions in children with sickle cell anemia: doppler tissue imaging study.

BACKGROUND AND AIMS: The extent in which sickle cell anemia (SCA) impacts myocardial function in children is unclear. Doppler tissue imaging (DTI) was introduced as a new non-invasive echocardiographic method for assessment of ventricular systolic and diastolic functions. We undertook this study to assess subclinical impact of SCA on global myocardial performance in affected children using DTI and to correlate it with mean hemoglobin concentration. METHODS: Eighty five children with SCA (mean age 11.82 ± 3.7 years) was included as the study group and 55 age- and sex-matched healthy children as the control group. Conventional two-dimensional echocardiography was performed in both groups and DTI was used to determine right ventricular (RV) and left ventricular (LV) Tei indexes. Mean Hb concentration was correlated to the cardiac functions of SCA children. RESULTS: RV and LV Tei indexes were significantly higher in SCA group (mean ± SD: 0.54 ± 0.19 vs. 0.27 ± 0.01, p <0.0001 and 0.47 ± 0.09 vs. 0.30 ± 0.07, p <0.0001, respectively). Also, mean Hb concentration was correlated negatively with both LV Tei index (r = -0.611, p <0.0001) and with RV Tei index (r = -0.894, p <0.0001). On the contrary, fractional shortening (FS) did not correlate with mean Hb concentration (r = -0.044, p = 0.681). CONCLUSIONS: DTI technique appears to be more sensitive than conventional echocardiography in the early detection of myocardial dysfunction in children with SCA. This provides insights into the value of early screening and the potential for preventive therapy in children to avert cardiac morbidity and mortality in adults with SCA.

Type 1/type 2 cytokine serum levels and role of interleukin-18 in children with steroid-sensitive nephrotic syndrome.

INTRODUCTION: In view of the conflicting evidence of helper T cell type 1 (Th1) or type 2 (Th2) pattern of cytokine synthesis in steroid sensitive nephrotic syndrome (SSNS), this study aimed to assess type-1/type-2 cytokines level in different stages of SSNS and to evaluate the role of IL-18. METHODS: We prospectively studied thirty children with SSNS, aged 2-12 years. The children were evaluated in the active stage before treatment initiation and re-evaluated again during remission while still on steroid treatment. A subgroup of children (21/30) was also evaluated during remission after steroid withdrawal. The control group included 30 healthy age- and sex-matched siblings. Serum levels of IL-2, IFN-γ, ΙL-4, IL-13 and IL-18 were measured by ELISA. RESULTS: IL-2 levels were not significantly different between children in different stages of SSNS and controls (p>0.05). Levels of IL-4, IL-13 and IL-18 were significantly higher during the active stage of SSNS compared to remission and controls (p<0.05). Serum IFN-γ was significantly lower in children with active disease compared to remission stages and controls (p<0.05). In children with SSNS, serum levels of IL-18 correlated significantly with both IL-4 and IL-13 during all stages (r=0.72 and p<0.0001, r=0.82 and p<0.0001, respectively). CONCLUSION: Children with active SSNS seem to have a shift to type-2 cytokine production, and IL-18 expression is significantly correlated with this type-2 immune response.

Endocrinopathies in beta-thalassemia major. Prevalence, risk factors, and age at diagnosis in Northwest Saudi Arabia.

OBJECTIVE: To define the prevalence, risk factors, and age at diagnosis of endocrinopathies in beta-thalassemia major (BTM) in Northwest Saudi Arabia. METHODS: This retrospective cross-sectional study included patients with BTM attending a combined endocrine-hematology clinic in Al-Madinah, Kingdom of Saudi Arabia from March 2009 to December 2010. Clinical and biochemical data from the initial clinic visits were used to define the prevalence and age of diagnosis of endocrinopathies. Demographic and laboratory variables were analyzed to identify significant risk factors. RESULTS: Eighty-one patients (42 males), aged 2-28 years were screened. Thirty-eight of them (46.9%) had at least one endocrinopathy. Of these, 28.9% (11/38) were aged less than 10 years. Hypogonadism was the most common complication detected in 52.7% (19/36) of patients of pubertal age group and 23.4% (19/81), of all cohort followed by short stature in 20.9% (17/81), subclinical hypothyroidism in 14.8% (12/81) and hypoparathyroidism in 11.1% (9/81). Patients with endocrinopathies were older (p=0.001), had longer duration of transfusion (p=0.001), and were started at a late age on chelation than those without endocrinopathies (p=0.07). Recent serum ferritin was poorly correlated to endocrinopathies (p=0.15). CONCLUSION: Endocrinopathies are common in our BTM cohort, and patients with this condition benefit from regular endocrine screening within the first 10 years of life. Although endocrinopathies were more prevalent in older patients; further, longitudinal studies are needed to define the exact age of onset and independent risk factors for these complications.