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Biomedical subjects

J J Nora

Publications and source records attributed to J J Nora.

At least 19 recordsLinked to original sources
PubMed1978

Exogenous progestogen and estrogen implicated in birth defects.

A five-year study of possible teratogenicity of exogenous female sex hormones included three case-control studies and one cohort study. The first case-control study disclosed an estimated relative risk of 8.41 and a highly significant difference in maternal hormonal exposure (P less than .001) between controls and infants with three major anomalies of the VACTERL group (V, vertebral; A, anal; C, cardiac; T, tracheal; E, esophageal; R, renal; and L,limb). Relative risk (RR) estimates of 5.58 (P = .017) and 3.35 (P less than .001) were found in two case-control studies involving maternal hormonal exposure and patients with congenital heart lesions without other malformations. A controlled, single-blind prospective study disclosed an excess of patients with major malformations (RR = 2.75), congenital heart anomalies (RR = 6), and neurological and neural tube disorders preponderant in the presence of a precipitously declining exposure rate during a three-year period in our referral area.

Abnormalities, Drug-Induced↗
PubMed1978

The silent ductus arteriosus.

Preterm infants at risk of developing a patent ductus arteriosus were followed sequentially by physical examination, echocardiographic determinations of the LA/AO ratio, and chest roentgenograms. The results show that a significant number of infants who have no clinical signs or symptoms of a PDA have large left-to-right shunts. The presence of this shunt was suggested by acute increase in left atrial size by ECHO determination and confirmed by retrograde single-film aortography. Clinical signs and symptoms often developed several days after documentation of the left-to-right shunt.

Aortography↗
PubMed1977

Pulmonary artery banding for ventricular septal defect with pulmonary hypertension.

Sixty children in this series underwent pulmonary artery banding (PAB); isolated ventricular septal defect (VSD) was present in 24, and 20 were corrected seven months to ten years and eight months after PAB. Other anomalies were present in 37, and 15 were eventually totally corrected. Survival after PAB was 90% (95.8% with isolated VSD), and 94.3% (95% with isolated VSD) after total correction. Mean age at banding was 12.9 months, and 52.6 months at debanding. All patients underwent catheterization before PAB; 39 underwent catheterization after PAB, and ten after total correction. Pulmonary artery blood pressure was reduced from 53.5 mm Hg to 25.3 mm Hg by banding. Most patients had only moderately increased pulmonary blood flow. Banding at the altitude of Denver (1,600 m) appears to be an effective means to convert high-risk infants into lower-risk children for total correction. The cumulative mortality for two-stage treatment of VSD was 8.3%.

Altitude↗
PubMed1977

Familial congenital complete heart block and maternal systemic lupus erythematosis.

A family is reported in which two siblings had congenital complete heart block with resultant congestive heart failure, the father and paternal grandfather show adult-onset conduction defects, and the mother has systemic lupus erythematosis. The interaction of heredity and environment is discussed in this context. A review of the literature on familial complete heart block suggests that so-called pure congenital-onset familial heart block, originally felt to be genetic, may in fact have an important enivronmental component, specifically related to ongoing maternal factors such as systemic lupus erythematosis.

Adult↗
PubMed1977

Atypical radiographic findings in neonates with absent pulmonary valve and tetralogy of Fallot.

At birth, three infants had typical to-and-fro murmurs and laboratory features suggesting tetralogy of Fallot with absent pulmonary valve. Initial posteroanterior chest x-ray films were atypical for the condition because of the apparent absence of dilation of the proximal pulmonary arterial tree. Lateral chest x-ray films invariably demonstrated a large hilar mass. Radiographic findings on the posteroanterior chest x-ray film considered pathognomonic for the complex lesion may not evolve until later during the first year of life.

Cardiomegaly↗
PubMed1976

Fusion of two apparently intact human X chromosomes.

Cytological studies have been presented from a 15-year-old girl with short stature and failure of puberty. Buccal mucosa preparations revealed X-chromatin mass approximately double in size of that of a normal female. Leukocyte metaphases suggested a two cell line composition of the patient. One population of cells conformed with 45,X chromosome distribution. The chromosome complement of her other cell line had a modal number of 46. In this cell line a "C" chromosome was replaced by an exceptionally large submetacentric chromosome. This abnormal element exhibited late DNA replicating pattern. G-banding study revealed that the abnormal chromosome was produced as a result of fusion involving telomeric ends of long arms of 2 intact X chromosomes. This translocation X was bearing 2 C-banded areas; one around the centromere and the other at the distal end of the long arm. The distal C-band area did not show any evidence for centromeric function. It appears that a centromere becomes latent in the presence of another centromere in a translocation bearing 2 total chromosomes. Such a change of state in the additional centromere is vital for the stability of the translocation chromosome.

Adolescent↗
PubMed1976

Recurrence risks in children having one parent with a congenital heart disease.

The risk of recurrence of a congenital cardiovascular malformation in a child having one parent with congenital heart disease has been determined for each of the seven most common anomalies presently compatible with survival to reproductive age. The range of risk is 2.5% to 4.3% depending on the lesion. This is within the range of expectation for the model of multifactorial inheritance previously used to predict recurrence in other first-degree relatives of probands (siblings and parents) with congenital heart disease. The cardiovascular abnormality occurring in the child was most often the same as in the parent or was a closely related variant of it.

Adult↗
PubMed1976

Echocardiography in Marfan's syndrome.

Echocardiographic examinations were performed in 26 patients with clinical evidence of Marfan's syndrome. Twelve patients were demonstrated to have isolated dysfunction of the mitral valvular apparatus of varying severity. Four patients demonstrated involvement of the aortic root as well as mitral valvular abnormalities, and six patients had problems involving the aortic root only. Four patients had no demonstrable cardiac abnormalities. Therapeutic decisions which could previously have been made with confidence only on the basis of cardiac catheterization with angiocardiographic studies were made by ultrasonic evaluation.

Adolescent↗