Clinical and histopathological relationship of sildenafil and bosentan treatments in rats with monocrotaline induced pulmonary hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a challenging disorder characterized by increasing pulmonary artery pressure, which is hard to treat. OBJECTIVE: This study was aimed to investigate the effects of bosentan, sildenafil and their combination. METHODS: Saline or MCT were applied to Wistar rats. By the development of PAH (4th week), MCT-given rats were treated orally with bosentan, sildenafil and combination of sildenafil and bosentan or placebo. ECHO examinations were performed. Tissues obtained from all of the rats were evaluated under an electron microscope. RESULTS: Left ventricular end diastolic diameter significantly increased in sildenafil and combined groups. Sildenafil group revealed a significant decrease in RV pressure and wall thickness. Examination of lung revealed a significant amount of connective tissue formation and increase in inflammatory cells in all the groups except controls in the interalveolar septum. Examination of PA revealed an increase in connective tissue volume, hypertrophic changes and expansions in granular endoplasmic reticulum cisternaes in smooth muscle cells in active groups rather than in the controls. Unlike the controls, the examination of the RV revealed an enlargement of the sarcoplasmic reticulum cisternaes in some cells, due to the calcium increase. CONCLUSION: Sildenafil and the combined therapy demonstrated to have more impact on pressure and the RV parameters in rats, with lower inflammatory findings in lung tissue (Fig. 6, Ref. 31).

Therapeutic hypothermia and myocardium in perinatal asphyxia: a microvolt T-wave alternans and Doppler echocardiography study.

OBJECTIVES: This is the first study evaluating the predictive value of myocardial performance on arrhythmia and mortality via tissue-Doppler and microvolt T-wave alternans in infants with hypoxic-ischemic encephalopathy treated with therapeutic hypothermia-rewarming. METHODS: The study included 23 term newborns having criteria for hypoxic-ischemic encephalopathy, and 12 controls. Tissue-Doppler imaging and T-wave alternans were performed in the first six hours after birth in patients from both groups and after hypothermia-rewarming treatment on the fifth day. RESULTS: The basal T-wave alternans values were higher in patients in lead aVF(p < 0.001) which also correlated with existing acidemia (r = 0.517; p = 0.012). Basal T-wave alternans and post­treatment values of patients were compared in leads V1 (p < 0.001) and aVF (p < 0.001); a significant decrease was found on the fifth day. Moreover, right ventricle diastolic diameter and estimated systolic pulmonary artery pressure of patients in the first 6 hours were higher (p = 0.03, p < 0.001, respectively). Although, the ejection fraction of patients did not decrease, basal values of left and right ventricular systolic and diastolic functions were lower initially, and increased significantly after treatment. CONCLUSION: The global cardiac functions and myocardial performance of newborns with hypoxic-ischemia might be improved with therapeutic hypothermia which can be determined by using T-wave alternans and tissue-Doppler measurements. However, further studies are needed to assess whether these measurements are prognostic in determining the myocardial dysfunction and arrhythmias (Tab. 2, Fig. 3, Ref. 26).

Prenatal Diagnosis and Postnatal Follow-up of congenitally corrected transposition of the great arteries and recurrent supraventricular tachycardia.

The prenatal sonographic diagnosis of congenitally corrected transposition of the great arteries (ccTGA), a rare form of congenital heart disease is very difficult. A24-year-old woman was referred to our center at 28 weeks' gestationbecause of suspected fetal cardiac anomalies. We report a case of ccTGA with supraventricular tachycardia, ventricular septal defect, pulmonary valve stenosis, tricuspid valvar displacement and moderate tricuspid regurgitation during her pregnancy. The combined presence of SVT and ccTGA are occasionally present in the literature.

Gated-SPECT de Perfusión Miocárdica en Niños con Anemia Falciforme: correlación con ecocardiografía / Gated myocardial perfusion scintigraphy in children with sickle cell anemia: correlation with echocardiography

Objetivo. El corazón es uno de los órganos afectados por la anemia de células falciforme (SCA). El objetivo de este estudio prospectivo es evaluar la perfusión miocárdica y la función del ventrículo izquierdo (LV) en niños con SCA por la gammagrafía de perfusión miocárdica (G-MPS) y comparar los resultados con los hallazgos ecocardiográficos. Métodos. Se evaluaron cuarenta y tres pacientes con SCA mediante estudios de G-MPS y ecocardiografía. La perfusión miocárdica y la función ventricular con la valoración de la motilidad y del engrosamiento se analizaron tanto de forma visual como cuantitativa. Se calcularon el EDV, ESV, EF, y el índice pulmón /corazón (L/H). Resultados. Ninguno de los pacientes presentó deterioro de la perfusión post-estrés en el G-MPS. Se observó dilatación del LV en 15 pacientes, que también estuvo presente en el ecocardio. Los valores de EF se encontraban dentro de los límites normales. La correlación entre los valores de EF, mediante ambos métodos no fueron estadísticamente significativas. Sin embargo la dilatación del ventrículo izquierdo detectados por ambos métodos y los valores de EDV-ESV en la G-MPS se correlacionaron con los diámetros sistólico y diastólico final calculados por la ecocardiografía (p<0,05). La ecocardiografía en modo-M mostró mayor índice de rendimiento miocárdico (LV-MPI) en pacientes con dilatación del LV. También hubo una relación significativa entre la dilatación del ventrículo izquierdo y la frecuencia de transfusiones sanguíneas (> 5/año) y el síndrome torácico agudo (p <0,05). Conclusión. El deterioro de la perfusión miocárdica en los niños con SCA no se observa con frecuencia. Debe considerarse el realizar la gammagrafía sólo en pacientes con síntomas cardíacos. Puesto que los valores de EF de los niños con SCA no se deterioran en las primeras etapas, el LV-MPI y la dilatación del ventrículo izquierdo deberían considerarse como un parámetro significativo(AU)

Objectives. The heart is one of the organs affected by sickle cell anemia (SCA). This prospective study has aimed to evaluate myocardial perfusion and left ventricle (LV) function in children with SCA by gated myocardial perfusion scintigraphy (G-MPS) and to compare the results with echocardiographic parameters. Methods. Forty-three patients with SCA were evaluated by G-MPS and echocardiography. Myocardial perfusion and motion with thickening function were analyzed both visually and quantitatively. End-diastolic (EDV), end-systolic volumes (ESV), ejection fraction (EF), lung-to-heart (L/H) ratio were also calculated. Results. None of the patients showed stress perfusion impairment in G-MPS. LV dilatation in 15 patients was observed both by G-MPS and echocardiography. EF values were within normal limits. Correlation between EF values calculated by two methods was not statistically significant. However, LV dilatation detected by both methods and EDV-ESV values in G-MPS were correlated to end-diastolic and end-systolic diameters calculated in echocardiography (p<0.05). M-Mode echocardiography revealed higher myocardial performance index (LV-MPI) in patients with LV dilatation. There was also a significant relationship between LV dilatation and frequent blood transfusions (>5/years) and acute chest syndrome (p<0.05). Conclusion. Myocardial perfusion impairment in children with SCA is not frequently observed. Thus, performing the scintigraphy only in patients with cardiac symptoms should be considered. Since EF values of the children with SCA are not deteriorated in early stages, LV-MPI and LV dilatation should be considered as a significant parameter other than EF or perfusion data(AU)

Gated myocardial perfusion scintigraphy in children with sickle cell anemia: correlation with echocardiography.

OBJECTIVES: The heart is one of the organs affected by sickle cell anemia (SCA). This prospective study has aimed to evaluate myocardial perfusion and left ventricle (LV) function in children with SCA by gated myocardial perfusion scintigraphy (G-MPS) and to compare the results with echocardiographic parameters. METHODS: Forty-three patients with SCA were evaluated by G-MPS and echocardiography. Myocardial perfusion and motion with thickening function were analyzed both visually and quantitatively. End-diastolic (EDV), end-systolic volumes (ESV), ejection fraction (EF), lung-to-heart (L/H) ratio were also calculated. RESULTS: None of the patients showed stress perfusion impairment in G-MPS. LV dilatation in 15 patients was observed both by G-MPS and echocardiography. EF values were within normal limits. Correlation between EF values calculated by two methods was not statistically significant. However, LV dilatation detected by both methods and EDV-ESV values in G-MPS were correlated to end-diastolic and end-systolic diameters calculated in echocardiography (p<0.05). M-Mode echocardiography revealed higher myocardial performance index (LV-MPI) in patients with LV dilatation. There was also a significant relationship between LV dilatation and frequent blood transfusions (>5/years) and acute chest syndrome (p<0.05). CONCLUSION: Myocardial perfusion impairment in children with SCA is not frequently observed. Thus, performing the scintigraphy only in patients with cardiac symptoms should be considered. Since EF values of the children with SCA are not deteriorated in early stages, LV-MPI and LV dilatation should be considered as a significant parameter other than EF or perfusion data.

Analysis of the SRY gene in a girl with 45,X/46,XY genotype.

Analysis of the SRY gene in a girl with 45,X/46,XY genotype: We present a Turner syndrome patient with a 45, X/46, XY mosaicism. A seven-year-old patient was admitted to our laboratory for evaluation of Turner Syndrome. On physical examination, short stature, a webbed neck, a broad chest with widely spaced nipples, syndactyly on toes, horse shoe kidney and typical findings of aortic coarctation were present. The chromosome karyotype of the patient was 45 X/46 XY. Molecular analysis of the blood lymphocytes confirmed the presence of the SRY gene of the patient. We report here the clinical and cytogenetic findings in a patient with an unusual sex chromosome mosaicism.

DRB1, DQA1, DQB1 genes in Turkish children with rheumatic fever.

OBJECTIVES: Several studies have suggested that genetic susceptibility to rheumatic fever (RF) may be linked to HLA Class II alleles. The purpose of this study was to examine the association between HLA Class II genes and RF in Turkish children. METHODS: DNA typing HLA Class II genes (DRB1, DQA1, DQB1) were performed in 55 children with RF and 50 healthy unrelated controls using sequence specific primers (SSP). RESULTS: The frequency of the HLA DQA1*03 (OR: 0.462, p < 0.05) allele was significantly decreased in the patient group. Also, the frequency of the combination of DRB1*04 and DQA1*03 allele (OR: 0.42, p < 0.01) was more significantly decreased in the patient group. Differences in frequencies of the DRB1 and DQB1 alleles between groups were not significant. CONCLUSIONS: Our data indicate that the HLA DQA1*03 allele may be a protecting factor in Turkish children with RF. Our results also suggest that the combination of the DRB1*04 and DQA1*03 alleles may be a stronger protective factor than the DQA1*03 allele alone.

Unusual extraction of an active fixation ventricular pacing lead with outer coil fracture in a child.

AIM: In this report we describe an asymptomatic paced child with outer coil fracture of the bipolar screw-in steroid eluting ventricular lead without insulation defect, loss of ventricular capture and unexpected increase in lead impedance in the bipolar VVIR pace configuration. METHOD AND RESULT: A previously unpublished method was performed to retract the helix and the lead. As a result, the fractured lead was successfully retrieved. CONCLUSION: We suggest that, this unusual extraction method can be tried as an alternative approach in the removal of an active fixation ventricular pacing lead with impaired mechanical function possibly due to fracture.

Evaluation of cerebral maturation by visual and quantitative analysis of resting electroencephalography in children with primary nocturnal enuresis.

This study was undertaken to evaluate resting electroencephalographic (EEG) changes and their relations to cerebral maturation in children with primary nocturnal enuresis. Cerebral maturation is known to be important in the pathogenesis of this disorder. Twenty-five right-handed patients with primary nocturnal enuresis, aged 6 to 14 years, and 23 age- and sex-matched healthy children were included in this cross-sectional case-control study. The abnormalities detected using such techniques as hemispheral asymmetry, regional differences, and hyperventilation response in addition to visual and quantitative EEG analysis were examined statistically by multivariate analysis. A decrease in alpha activity in the left (dominant hemisphere) temporal lobe and in the frontal lobes bilaterally and an increase in delta activity in the right temporal region were observed. We concluded that insufficient cerebral maturation is an important factor in the pathogenesis of primary nocturnal enuresis, and EEG, as a noninvasive and inexpensive method, could be used in evaluating cerebral maturation.

Lack of association between plasma leptin levels and appetite in children with iron deficiency.

A negative correlation between leptin and appetite or food intake has been shown in healthy individuals. However, the role of leptin in clinical conditions characterized by anorexia has not been established. One of the well-known clinical features of iron-deficiency anemia is poor appetite. We examined the changes in plasma leptin levels in relation to expected improvement in appetite with iron treatment in children with iron deficiency. In 24 infants and small children (mean age +/- standard deviation = 19.6 +/- 7.7 months) with iron deficiency, we studied plasma leptin levels before and after iron therapy. After 15.0 +/- 2.4 wk of iron treatment, serum ferritin levels improved significantly, with accompanying increases in their subjective appetite scores and food intakes. However, as their mean age and plasma leptin levels adjusted their body mass indexes were unchanged. Serum ferritin correlated significantly with appetite score (r = 0.680, P < 0.001) and food intake (r = 0.480, P < 0.01). Leptin correlated only with body mass index (r = 0.405, P < 0.01). Lack of association between plasma leptin levels and degree of appetite in iron-deficient children treated with iron suggests a leptin-independent mechanism for the observed increase in appetite.

Denver developmental screening test II for early identification of the infants who will develop major neurological deficit as a sequalea of hypoxic-ischemic encephalopathy.

BACKGROUND: The primary aim of this study was to find widely available, inexpensive, and non-invasive parameters for early identification or prediction of the infants with hypoxic-ischemic encephalopathy (HIE) who will have a severe adverse outcome (classified as death or a major neurological deficit). METHODS: Fifty-seven full-term or near-term newborn infants with a diagnosis of HIE were consecutively admitted to the neonatal intensive care unit and studied. Occurrence of seizures during the first 24 h, cranial ultrasonography (US) findings within the first 5 days of life, and Denver developmental screening test II (DDST II) at 6 months of age, were analyzed in relation to mortality and neurological status at 2 years of age. RESULTS: Of the 57 infants, 10 were lost to follow-up. Twenty of the remaining 47 infants had a severe adverse outcome. Among the predictors of severe adverse outcome, occurrence of seizures was found to have a poor predictive accuracy. Cranial US had 100% sensitivity, however with a rather low specificity (55%). However, DDST II at 6 months of age, yielded a very high predictive accuracy (sensitivity=100%, specificity=95%). CONCLUSION: We conclude that DDST II at 6 months of age could be used in predicting severe neurological outcome in infants with HIE.

Effect of gestational age on plasma fibronectin concentrations in the neonate.

BACKGROUND: Fibronectin (FN) is known to have important roles in host defense against infection. The risk for neonatal sepsis increases with the degree of immaturity of the host. Conflicting results have been reported on the relationship between plasma FN levels and gestational age (GA) in the preterm neonate. METHODS: In the present study, we determined plasma FN concentrations with an immunodiffusion method in 40 newborns of various gestational ages, ranging from 30 to 42 weeks. RESULTS: We found a strong direct correlation between plasma FN levels and GA (r = 0.86; P < 0.001). CONCLUSIONS: We speculate that the increased risk for neonatal sepsis with degree of prematurity may, in part, be explained by impaired defense with decreasing plasma FN levels seen with lessening GA.

Scurvy. A case report.

An 18-month-old girl presented with irritability, epistaxis, spongy appearance of the gums perifollicular papules with follicular hyperkeratosis, ecchymosis, painful swollen knees and scorbutic rosary. Her diet consisted mainly of wheat flour. X-ray of the knees showed findings compatible with scurvy. Ascorbic acid level was below 0.003 g/L. Ascorbic acid therapy resulted in a dramatic clinical improvement. Scurvy is an uncommon disease in our society today. It is important to recognize the signs and symptoms of scurvy because it is easily treated with vitamin C replacement.