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Biomedical subjects

R Russo

Publications and source records attributed to R Russo.

At least 253 records · Page 14Linked to original sources

Heterocellular hereditary persistence of fetal hemoglobin (HPFH). Molecular mechanisms of abnormal gamma-gene expression in association with beta thalassemia and linkage relationship with the beta-globin gene cluster.

We report a study of four families of Italian origin in which heterocellular HPFH is inherited linked to beta thalassemia over two or three generations. The HPFH + beta thalassemia carriers showed thalassemic blood pictures and elevated HbF and F-cell number without increase in the HbF/F-cell content. Association of this gene complex with a second beta thalassemia trait gives rise to a mild clinical picture characterized by 9-12 g/dl of mainly HbF in peripheral blood and no transfusion requirement. In two families, independent segregation of the HPFH or beta-thal trait was observed, and in one case the study of the DNA polymorphisms within the gamma delta beta gene cluster indicated that the HPFH mutation lies outside that DNA region. In one family the coexistence of a polymorphic variant of the A gamma chain (the A gamma T chain) allowed us to demonstrate that the increased gamma chain synthesis caused by the heterocellular HPFH determinant is directed by both chromosomes.

Adolescent↗

Dehiscence of aortic valve prostheses: analysis of a ten-year experience.

Up to the end of 1982, reoperation for dehiscence of an aortic prosthesis was necessary in 5% of patients operated on for primary aortic valve replacement in the previous decade at the University of Padova Cardiac Surgery Center. This complication occurred early (median time to diagnosis 4 months) and was associated with an elevated 30-day operative mortality (27%, 70% CL 19-37%). This is probably (P = 0.09) related to preoperative heart failure. The follow-up of the traced surviving patients (92%) indicates a continuing poor prognosis with a 6-year survival rate of only 13.5% (70% CL 5.0-30%). All the events were cardiac related and directly or indirectly connected with the persistence or recurrence of dehiscence that was observed in 72% of the cases. In the face of these results, a retrospective study has been performed to identify, on the basis of the available data, the subsets of patients more prone to develop this complication. Our results suggest that a significant increased risk (P less than 0.001) can be identified in patients presenting with bacterial endocarditis (12.2% rate), in patients with concomitant aneurysm of the ascending aorta (10.9%) and in patients with degenerative regurgitation or severe calcifications of their native valve, with rates of 7.0 and 6.0 respectively. In these situations particular care is required to avoid undue stress on the annular tissue. We also suggest the use of buttressed interrupted sutures.

Adult↗

Patterns of local-regional recurrence and results in Stages I and II breast cancer treated by irradiation following limited surgery. An update.

One hundred forty-six women with Stage I and Stage II breast cancer received radical radiotherapy after having excisional biopsy ( lumpectomy ) at Massachusetts General Hospital between 1956-1978. They were grouped according to age: those younger than 49 years and those older than 50 years. The 5-year survival rates were 93 and 73% for patients with Stage I and Stage II cancer, respectively; the corresponding 5-year relapse survival rates were 75 and 56%. The local recurrence rate was 8% in patients with Stage I disease and 17% in those with Stage II disease. Survival was not significantly affected by patients' age, by the presence or absence of blood vessel or lymphatic involvement, or by the addition of adjuvant chemotherapy. No major complications occurred. Modification in radiation dose and technique resulted in improved overall survival and local control. Limited surgery followed by radical radiation therapy offers a therapeutically effective, cosmetically acceptable alternative to radical surgery for early stage breast cancer.

Adult↗

The intrahepatic and extrahepatic bile ducts in surgical jaundice: radiological evaluation and therapeutic implications.

Many invasive and noninvasive imaging modalities are available to image the biliary tract in suspected surgical jaundice. Technical advances in existing techniques and development of new techniques allow biliary obstruction to be detected in its earliest stages. Criteria for the presence of biliary obstruction include anatomic visualization and measurement of both the intrahepatic and extrahepatic portions of the biliary tree. These criteria will be reviewed and potential diagnostic pitfalls discussed. Anicteric dilatation, disparate dilatation, and segmental obstruction of the biliary tract will be discussed as these entities pose a diagnostic as well as therapeutic challenge. The newer endoscopic and percutaneous interventional techniques will be considered and placed in perspective.

Carcinoma↗

Mitral valve prosthesis dehiscence necessitating reoperation. An analysis of the risk factors involved.

Between January 1970 and December 1981, a total of 21 reoperations for periprosthetic leak were performed on 20 patients out of 999 with previously implanted prosthetic mitral valves. In most of them reoperations were performed within the first year, since the initial procedure and the leading indications were intractable congestive heart failure or infection of the mitral prosthesis. The mortality rate was 30% and was related to the preoperative cardiac functional status. The preoperative variables significantly related to an increased incidence of dehiscence of the mitral prosthesis necessitating reoperation were a degenerative disease (P = 0.016) or an infective endocarditis (P = 0.0006) of the native valve, both causing mitral regurgitation. Rheumatic disease, type of prosthesis, supra- or subannular insertion, age of the patient, and operative year, were not significant, neither were calcifications that are probably neutralized by the routine use of special surgical techniques. It is suggested that the use of techniques specifically designed to eliminate periprosthetic leak in patients affected by mitral regurgitation due to degenerative or infective disease of the native valve, might lead to a further reduction of reoperations for this complication.

Adolescent↗

[Usefulness of the 12-lead standard electrocardiogram in assessing myocardial infarct size].

The value of the electrocardiogram in assessing infarct size was studied using serial estimates of serum creatine phosphokinase (CPK) in serum and serial 12-lead electrocardiograms in patients admitted to a coronary care unit with acute myocardial infarction. Sum of the ST segment deviations from the isoelectric line (sigma ST12) and sum of the conventional scores of Q waves amplitude (sigma Q12) were obtained from each electrocardiogram, and then the time-course of these parameters was considered. The correlation between maximum sigma Q12 and CPK-peak in anterior infarcts, was highly significant (r = 0.733; P less than 0.001). Maximum sigma ST12, measured upon admission or immediately thereafter in patients hospitalized within 4 hours from the onset of chest pain, was found to correlate significantly with CPK-peak (r = 0.675, P less than 0.001 for the whole group; r = 0.758, P less than 0.01 for patients with inferior infarcts). Time-course of sigma ST12 and CPK showed 4 different patterns. Among them, type 1 ("rapid necrosis") showed the most significant correlations (maximum sigma ST12 within 4 hours from symptoms versus CPK-peak: r = 0.909, P less than 0.001; maximum sigma Q12 versus CPK-peak in anterior infarcts: r = 0.782, P less than 0,05; and maximum sigma ST12 within 4 hours from the onset of pain versus sigma Q12 in patients with anterior infarcts: r = 0.863, P less than 0,05). There was no correlation between sigma ST12 at any other time and CPK-peak: this observation is in accordance with the presence of a rapid decrease in the mean sigma ST12 after the first 3-4 hours from the beginning of symptoms. This study shows that the analysis of ST segment deviations and of Q waves development in the standard electrocardiogram provides useful information on the size of acute myocardial infarction as reflected by the peak value of serum CPK.

Adult↗

Pregnancy in patients with a porcine valve bioprosthesis.

Seven patients who became pregnant after valve replacement with a Hancock bioprosthesis were followed up during 8 pregnancies. Six had undergone isolated mitral valve replacement, and 1 had mitral and aortic valve replacement. Their age at the time of operation ranged from 14 to 31 years (average 24); delivery occurred 21 to 88 months (average 51.3) after valve replacement. All women were in sinus rhythm at the time of gestation, and administration of oral anticoagulants was avoided in all. No embolic episodes occurred either after operation or during pregnancy, labor, or puerperium. The only major complication during pregnancy was cardiac failure in 1 patient, associated with onset of atrial fibrillation. Four women had vaginal delivery and 3 required cesarean section. All but 1 delivered a normal, healthy baby. One premature infant died soon after birth because of respiratory distress. No maternal or fetal hemorrhagic complications were observed. One patient died 3 months after delivery in severe heart failure caused by diffuse calcification of both mitral and aortic xenografts. Another women underwent successful reoperation soon after the second pregnancy because of calcific stenosis of the mitral porcine valve. It is concluded that (1) bioprosthetic valves can be considered the most suitable devices employed in women of childbearing age because anticoagulants can be avoided, therefore eliminating the risks related to inappropriate administration of oral anticoagulants as well as the hazards associated with the potential teratogenic effect of coumarin drugs; and (2) pregnancy might favor calcification of porcine heterografts, leading to bioprosthetic failure. Until further data are available to support this suspicion, close clinical and echocardiographic follow-up study of these patients is recommended after pregnancy.

Adolescent↗

[Evaluation of left ventricular enlargement in isolated aortic insufficiency. Importance of the total cardiac volume calculated on the standard chest film].

A radiographic and hemodynamic investigation has been performed in 53 patients with pure isolated aortic regurgitation, in order to evaluate the most reliable method to detect left ventricular enlargement in this condition. Twelve plain chest film measurements have been compared to the end diastolic volume of the left ventricle calculated by left ventricular angiography. The most sensitive detector of left ventricular enlargement was the plain film total heart volume ( r = 0.754); the cardiothoracic ratio, the cardiac perimeter and the frontal area were less sensitive measurements, but still statistically significant (p less than 0.001). The results of our investigation support the conclusion that plain film total heart volume is the most reliable single measurement for evaluating the left ventricular enlargement in patients with isolated pure aortic incompetence.

Adolescent↗

Pregnancy in a patient after mitral valve replacement with a porcine bioprosthesis.

The course of two pregnancies in a woman, who had previously undergone mitral valve replacement with a porcine bioprosthesis, is reported. The present case suggests that porcine heterografts are to be considered as the most suitable cardiac valve substitutes in females of childbearing age, since anticoagulants are not needed, avoiding therefore the risks related to both an incorrect anticoagulation and to the recognized teratogenic effect of coumarin drugs.

Adult↗

[Fetal malformations caused by oral anticoagulants during pregnancy. Report of a case].

The case of a newborn infant girl with a partial agenesis of the corpus callosum and hypoplasia of the nasal bones is reported. She was the product of a full-term pregnancy of a woman who had previously undergone mitral valve replacement with a Björk-Shiley prosthesis and had been maintained on oral anticoagulants (sodium warfarin) until the 38th week of gestation. This case underlines: 1) The need for maintaining an adequate anticoagulation in pregnant women with a mechanical prosthesis. 2) The potential risks of fetal malformations when the oral anticoagulants are given during the first trimester of pregnancy and of fetal hemorrhagic complications when these drugs are administered during the last two trimesters of gestation. 3) The lack of effective pharmacological alternatives to coumarin drugs, which can allow an adequate anticoagulation in the mother with minimal hazards for the fetus.

Abnormalities, Drug-Induced↗