Disproportionate septal hypertrophy associated with erythroblastosis fetalis

Article Abstract:

Erythroblastosis fetalis is a blood disease of the newborn, and is characterized by anemia, jaundice, enlargement of the liver and spleen, and generalized edema (accumulation of fluids throughout the body). The clinical characteristics and results of echocardiography, the visualization of the heart using sound waves, were assessed in 10 infants with erythroblastosis fetalis. Echocardiography was performed on the infants in the first 48 hours of life. Disproportionate septal hypertrophy, the enlargement of the septum, or wall of tissue separating the right and left heart ventricles, was detected in five of the 10 infants. These infants were born between 1984 and 1988 and required neonatal intensive care and double-volume exchange transfusion, in which small amounts of blood are transfused and withdrawn until the blood volume is almost entirely exchanged. There was no relationship between the development of disproportionate septal hypertrophy and levels of glucose or bilirubin (the pigment in bile) or the hematocrit, a measure of the red blood cell content of the blood. Infants who did not undergo transfusions while in utero (before birth) had higher measures of hypertrophy than infants who received transfusions in utero. However, there was no correlation between the number of transfusions and the measure of hypertrophy. These findings show a significant prevalence of disproportionate septal hypertrophy in infants with erythroblastosis fetalis, which can be reduced by transfusions given in utero. (Consumer Summary produced by Reliance Medical Information, Inc.)

Abnormalities, Diseases, Infants (Newborn), Neonatal diseases, Erythroblastosis fetalis, Heart septum

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Atrioventricular canal in Down syndrome: prevalence of associated cardiac malformations compared with patients without Down syndrome

Article Abstract:

Children with Down's syndrome, a congenital disorder which results in mental retardation, frequently have heart defects. Atrioventricular canal (AVC) is one of the most common cardiac complications among those with Down's syndrome. This heart defect results from an embryonic malformation in which the common atrioventricular canal fails to divide; this causes septal defect and valve incompetence. Both Down's syndrome and AVC have been studied, but AVC as it typically occurs in Down's syndrome patients has not been fully explored. To further investigate this, the medical records of 220 patients with AVC were reviewed. Approximately half (105 children) also had Down's syndrome, and the other half (115 children) did not. Records of echocardiograms and angiocardiograms, diagnostic imaging techniques, were a part of the clinical review which assessed the anatomic types that were associated with each group. The children who also had Down's syndrome more frequently had a complete form of AVC, which was often associated with Fallot's tetralogy (another congenital condition, which is characterized by a defective interventricular septum, narrowing of the pulmonary artery, misplaced aorta, and an enlarged right ventricle). Patients without Down's syndrome more frequently had left-sided abnormalities and a partial atrioventricular canal. These results support other reports which indicate that AVC that is associated with Down's syndrome usually has predictable and distinct characteristics. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Genetic aspects, Congenital heart defects, Congenital heart disease in children, Atrioventricular canal, Common

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Correction of atrioventricular septal defect: results influenced by Down syndrome?

Article Abstract:

Atrioventricular septal defect (AVSD) is an abnormal opening between the upper and lower portions of the heart chambers. It is a common defect of children born with the genetic disease Down's syndrome and can be repaired surgically. However, many surgeons do not perform surgical repair of AVSD in cases of Down's syndrome. To determine whether the presence of Down's syndrome affects the outcome of AVSD repair, 85 patients were evaluated. Complete AVSD was present in 36 Down's syndrome patients and 13 patients without the disorder. Thirty patients survived surgery, of whom seven were among those without Down's syndrome. A partial AVSD was present in 36 patients. All five of these who had Down's syndrome survived. Of the 31 patients without Down's syndrome, 30 survived. A low body weight for age was associated with a poor outcome. The many other malformations that were present in the children without Down's syndrome may have contributed to their poor surgical outcome. Down's syndrome does not appear to complicate the surgical repair of AVSD. Therefore, a conservative approach to treatment of AVSD in patients with Down's syndrome is not justified. (Consumer Summary produced by Reliance Medical Information, Inc.)

Surgery, Ventricular septal defects, Atrial septal defects in children

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