Imaging of Pulmonary Atresia With Ventricular Septal Defect.

The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important for complete anatomical delineation and planning for treatment. Preoperative imaging used for assessment of the main pulmonary artery (its size, valve, and confluence), aortopulmonary collaterals (its origin, insertion, course, and size), presence of patent ductus arteriosus, other sources of collaterals as bronchial and coronary arteries, and pattern of pulmonary arborization. Imaging can detect associated aortic, pulmonary venous and coronary anomalies, and other congenital heart disease. Postoperative imaging after unifocalization and stent is for assessment of patency, stenosis, and occlusion of stent or perivascular lesions as seroma.

Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins.

We aimed to review computed tomography and magnetic resonance angiography of congenital anomalies of pulmonary veins. Total anomalous pulmonary venous return shows all pulmonary veins drain abnormally in another site rather than left atrium. Imaging can detect anomalous veins either supracardiac, infracardiac, or mixed. Partial anomalous pulmonary venous return shows some pulmonary vein have abnormal drainage that well delineated with computed tomography angiography. Scimitar syndrome is a type of partial anomalous pulmonary venous return where the pulmonary veins of the right lung drain infracardiac and is associated with right lung hypoplasia and dextrocardia. Pseudoscimitar show anomalous vein that takes a tortuous course and drains into the left atrium producing a false-positive scimitar sign. Cor triatriatum shows septum divide left atrium with proximal chamber receives blood flow from the pulmonary veins. Levoatriocardinal vein is an anomalous connection between the left atrium and anomalous vein from systemic venous system that is embryo logically derived from the cardinal veins. Computed tomography angiography can detect pulmonary vein stenosis, atresia, hypoplasia, and varix. Imaging is important for intimal diagnosis and detects the anomalous vessels and its connection, presence of stenosis, and associated other congenital cardiac anomalies. Also, it is a great role in assessment of patients after surgery.

Comparison of contrast enhanced magnetic resonance angiography with invasive cardiac catheterization for evaluation of children with pulmonary atresia.

Complete assessment of the source of pulmonary blood supply and delineation of the anatomy of pulmonary arteries are essential for the management and prognostic evaluation of pulmonary atresia (PA) patients. Invasive cardiac catheterization is considered the gold standard imaging modality to achieve this. We investigated the role of contrast enhanced magnetic resonance angiography (MRA) to evaluate the pulmonary blood supply and the anatomy of the pulmonary arteries and compared this with cardiac catheterization in children with PA. We studied 20 children with PA. Median age was 2.5 years (range 6 months-13 years). All patients were examined with cardiac catheterization and contrast enhanced MRA, and the results of both modalities were compared. There was a complete agreement between both modalities in the detection of the main pulmonary artery morphology and determination of the confluence state of the central pulmonary arteries. There was an 88% agreement for patency of the ductus arteriosus and 66% for patency of the surgically placed shunt. There was a complete agreement between both techniques on determining the presence of collaterals more than 2.5 mm. Twenty-eight collaterals of less than 2.5 mm were detected only by contrast enhanced MRA. There was a strong correlation between both modalities in measuring the pulmonary arteries and collaterals diameter (P<0.001). Contrast enhanced MRA is a safe and accurate non-invasive technique to evaluate the pulmonary artery morphology and the sources of pulmonary blood supply in children with PA.

Low bone mass in children with malignant lymphoma.

The aim of this work was to study the effect of disease process on bone mass and calcium homeo-stasis in children with malignant lymphoma at diagnosis, 3 months after starting chemotherapy, and after 1 year. Evaluation of lumber vertebrae (L2-L4) bone mineral density using dual-energy X-ray absorptiometry and calcium homeostasis parameters and bone turnover biochemical markers (serum osteocalcin and urinary deoxypyridinoline) had been assayed in twenty lymphoma patients at presentation and after treatment. Low bone mass for chronological age was observed in 4 patients (20%) at diagnosis and persisted after 3 months and 1 year. Parathyroid hormone level demonstrated no differences between children with lymphoma at different stages of therapy and controls, while 25(OH) D(3) was significantly lower in lymphoma patients at different stages of therapy as compared to controls (p < .001). Osteocalcin was significantly lower in lymphoma patients at different stages of therapy. Deoxypyridinoline showed only significant higher values after 3 months of therapy compared to controls (p = .01). In conclusion, low bone mass was observed in children with lymphoma and is related to decreased osteoblastic activity and decreased mineralization of bone.

Impact of long-term oral iron supplementation in breast-fed infants.

OBJECTIVE: To weigh benefits of oral iron supplements on infant's growth against its potential hazards. METHODS: 248 exclusively breast-fed infants aged 4-6 months were consecutively enrolled and divided into treatment group given iron containing multivitamin (TG = 198) and control group (placebo, PG = 50) given the same multivitamin but without is subdivided according to clinical assessment into group A (well nourished) and group B (malnourished); both were further stratified according to basal blood iron status. Assessment was done after 6 and 12 months with concurrent collection of morbidity parameters (diarrhea and fever). Data were normalized and analyzed using SPSS and Eurogrowth softwares. RESULTS: After 6 months treatment, weight and length gain was better in TG compared to placebo especially evident in anemic malnourished infants (P 0.05). Morbidity risk was linked to immunologic background of infant; odds ratio for diarrhea and fever was higher in malnourished compared to well nourished (P 0.05) or iron therapy (P for well-nourished non-anemic treatment vs PG > 0.05). CONCLUSION: Oral iron supplementation resulted in better effects on growth velocity of breast fed infants especially those who were initially malnourished and anemic or at least iron depleted, with less marked morbidity than in iron replete infants.

Acute painful crises of sickle cell disease in Egyptian children: predictors of severity for a preventive strategy.

The objective of this study was to predict which infants with sickle cell disease (SCD) are prone to develop severe painful crises. In a mixed hospital - and community-based population (76 cases), demographic data, SCD diagnostic parameters, and basal blood counts were correlated with 2 indices of SCD severity: pain rate (average number of days of painful episodes per year of follow-up) and serious life-threatening complications, such as hyperhemolytic crises. Data were analyzed blind to these indices. The Student t test, analysis of variance, and Pearson correlation were used to determine association with pain rate. Discriminant analysis was used for the prediction of SCD severity. Pain rate was significantly high in hemoglobin SS patients, especially in those with an early onset of dactylitis. There were statistically significant negative correlations of pain rate with basal hemoglobin level, hematocrit, percent hemoglobin F, and arterial oxygen saturation (P <.01 for all correlations). The top 3 predictors of SCD severity were (in descending order) genotype, basal hemoglobin level, and early dactylitis. Severe forms of SCD could be predicted in early infancy with 100% accuracy by using the basal diagnostic parameters for the disease. These infants should be closely monitored with special attention to ventilation status, even before the development of dactylitis.

Excitatory amino acids and magnesium sulfate in neonatal asphyxia.

OBJECTIVE: The excitatory amino acids (EAA); glutamate and aspartate are released into the cerebrospinal fluids (CSF) of asphyxiated newborns. The objectives of this study were: (a) to examine the relation of the concentration of EAA in the CSF with the degree of brain injury, (b) To determine the time of the release of these EAA into the CSF, and (c) to detect the effect of magnesium sulfate (MgSO(4)) on their levels. DESIGNS AND METHODS. A randomized controlled trial was conducted on 47 full term asphyxiated newborns. Twenty three infants received an intravenous 10% solution of MgSO(4) at a dose of 250 mg/kg within the first 24h of life while the other 24 newborns received isotonic saline (0.9%) of an equal volume. Levels of glutamate and aspartate were measured before and 72 h after giving the trial solution. Results. In the study population (n=47) both glutamate and aspartate were significantly elevated in infants with higher grades of HIE compared to those with lower grades (P=0.013 and 0.031, respectively). Compared to baseline level, glutamate decreased significantly over time in placebo group (-8.28+/-14.26, P=0.025) and in MgSO(4) group (-14.39+/-18.72, P=0.005). Glutamate concentration did not differ between groups when measured at baseline (29.26+/-16.31 vs. 31.27+/-22.62, P=0.82) and at 72 h (19.28+/-15.63 vs. 19.6+/-16.54, P=0.87). The change in aspartate concentration over time was not significant in placebo group (-0.45+/-1.96, P=0.34) or in MgSO(4) group (-0.7+/-3.19, P=0.37). Aspartate did not differ between groups when measured at baseline (3.52+/-2.4 vs. 3.92+/-2.59, P=0.49) or at 72 h (2.79+/-1.24 vs. 3.05+/-2.48, P=0.92). Conclusions. The EAA; glutamate and aspartate are released in the CSF of asphyxiated newborns immediately after birth and declined by 72 h. Their initial concentrations correlated with the severity of HIE. Postnatal administration of MgSO(4) did not alter the levels of these 2 EAA.

Low turnover bone disease in Egyptian children with acute leukemia.

The aim of this work was to study bone turnover markers, calcium homeostasis and bone mineral density (BMD) in children with acute leukemia at diagnosis, after induction chemotherapy, and during maintenance therapy to delineate abnormalities present. After evaluation of L2-L4 BMD using dual-energy X-ray absorptiometry in patients with acute myeloid and lymphoid leukemia at presentation and after treatment, the results were compared to 352 healthy age- and sex-matched Egyptian controls. Calcium homeostasis parameters and bone turnover biochemical markers (serum osteocalcin and urinary deoxypyridinoline) were also assayed and the results were compared to 12 healthy age- and sex-matched controls. Osteopenia was observed at diagnosis and during treatment in patients with acute leukemia. At diagnosis osteopenia was observed in 27 patients (62.8%): 10 (23.3%) had non severe osteopenia and 17 (39.5%) had severe osteopenia. This low BMD persisted in those who were followed up. Parathyroid hormone (PTH) (pg/ml) levels demonstrated non significant differences between children with acute leukemia at different stages of therapy and controls, while, 25 (OH) D3 (ng/ml) was significantly lower in acute leukemia patients at different stages of therapy compared to controls (p<0.001). Osteocalcin (ng/ml) is significantly lower in patients at different stages of the disease compared to controls (p<0.001) but there was no significant difference between patients at different stages of therapy. Deoxy-pyridoline cross links showed non-significant difference between the different types of acute leukemia and with controls. Osteopenia is a significant problem in children with acute leukemia at presentation and after chemotherapy. Osteopenia in acute leukemia appears to be of the low turnover type (decreased osteoblastic activity and decreased bone mineralization).