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Biomedical subjects

L Tuuteri

Publications and source records attributed to L Tuuteri.

At least 19 recordsLinked to original sources
PubMed1981

Primary cardiac tumours in infancy and childhood. A review and six case reports.

Primary cardiac tumours are rarities in infants and children. We have had 6 patients, aged 17 days to 10 years, with primary cardiac tumours: 1 had a fibroma, 1 had a myxoma, 2 had an haemangioma and 2 had a rhabdomyoma. Correct diagnosis was missed pre-operatively in 3 cases and finally established at autopsy. Three of the patients underwent cardiac operation-one of them unsuccessfully. At surgery the main difficulties were with tumour infiltration and the multifocal nature of some tumours. We conclude that the echo- and angiocardiography are essential tools in the diagnosis of primary cardiac tumours. Large tumours or conduction tissue infiltration means an extremely poor prognosis. Multifocal cardiac tumours can be successfully removed, even in newborn babies, and the outcome for the patients is also affected by other important factors, e.g. tuberous sclerosis in rhabdomyomas.

Child↗
PubMed1979

Gyrate atrophy of the choroid and retina with hyperornithinemia: tubular aggregates and type 2 fiber atrophy in muscle.

We studied 21 patients with gyrate atrophy of the choroid and retina and hyperornithinemia. Although the patients were not weak, type 2 muscle fibers were almost universally atrophic and had tubular aggregates. Gyrate atrophy is the first disease in which females are shown to have tubular aggregates; the sexes were affected equally. In gyrate atrophy the number of type 2 fibers decreases with age. The muscle and eye changes are probably related to abnormal creatine synthesis, caused, in gyrate atrophy, by the increased body pool of ornithine; muscle abnormalities may also be present in other tapetoretinal dystrophies.

Adolescent↗
PubMed1976

Atrial myxoma in a family.

A family is described in which the mother and three of seven children had atrial myxoma. The mother had biatrial myxoma; surgical treatment resulted in massive intraoperative embolization and death. Surgery was sucessful in two sons with left atrial myxoma and systemic arterial embolization. A third son had calcified right atrial myxoma with destruction of the tricuspid valve and episodes of syncope and pulmonary embolism; surgery including valve replacement, was successful. The mother's father and a brother had died suddenly without a definite diagnosis. The family data are consistent with dominant transmission. The possibility of finding affected relatives should be borne in mind when studying patients with atrial myxoma.

Adolescent↗
PubMed1975

Causes of death in transposition of the great arteris. A clinical and autopsy study of 140 cases.

The causes of death were assessed in 140 infants and children with transposition of the great arteries studied clinically and post mortem. Half of the children were under one month old. Death occurred during the first year of life in 118 cases. Balloon atrial septostomy and/or cardiac surgery were performed in 37 cases, and 7 patients died following operations for extracardiac malformations. Congestive heart failure was the most common single cause of death, occurring in 109 cases. All but 7 patients were cyanotic. Extracardiac malformations were encountered in 39 patients and were considered a main cause of death in 22 of these. Various infections, mostly pneumonia, occurred in half of the cases. Forty-one patients had vascular accidents in various organs. These complications were more common in operated than in nonoperated cases. Miscellaneous causes of death, including hyaline membrane disease and/or pulmonary atelectases, occurred in 30 patients. The study illustrated the complex symptomatology and therapeutic problems presented by critically ill infants with transposition of the great arteries.

Child↗
PubMed1975

Mulibrey nanism: review of 23 cases of a new autosomal recessive syndrome.

Mulibrey (muscle, liver, brain, eye) nanism is probably an autosomal recessive condition characterized by progressive growth failure of prenatal onset, triangular face with hydrocephaloid skull, general thinness and muscular hypotonicity, peculiar voice, venous congestion caused by pericardial constriction, and pigment dispersion and yellowish dots in ocular fundi. Two thirds of the patients had cutaneous nevi flammei and one third cystic fibrous dysplasia of the tibia. Probably a substantial portion of the affected are lost by early abortion and others by infantile death. The physical capacity and life expectancy seem to vary depending on the degree of the cardiac affection.

Adolescent↗