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Biomedical subjects

J Seger

Publications and source records attributed to J Seger.

At least 37 records · Page 2Linked to original sources
PubMed1988

Restrictive cardiomyopathy with kappa light chain deposits in myocardium as a complication of multiple myeloma. Histochemical and electron microscopic observations.

kappa Light chain deposits occurring in myocardium as a complication of multiple myeloma were identified ultrastructurally and immunohistochemically in a right ventricular endomyocardial biopsy specimen from a patient who presented with clinical and hemodynamic findings of restrictive cardiomyopathy. These deposits were not evident on routine histopathologic examination; they were Congo red-negative and gave a positive immunoperoxidase reaction for kappa light chains and a negative reaction for lambda chains. They consisted of amorphous, electron-dense granules that formed discontinuous layers adjacent to the plasma membranes of cardiac myocytes, arteriolar endothelial and smooth-muscle cells, and neural elements. These observations underscore the need for critical study of endomyocardial biopsy specimens, using electron microscopy and immunohistochemical reagents, for the precise identification of protein components in tissue deposits in patients suspected of having cardiac amyloidosis or related disorders.

Aged↗
PubMed1986

Procainamide-induced incessant supraventricular tachycardia in the Wolff-Parkinson-White syndrome.

A patient with the Wolff-Parkinson-White syndrome presented with incessant orthodromic atrioventricular tachycardia following initiation of procainamide therapy. This finding was repeatedly documented both clinically as well as during electrophysiologic testing. Escape atrial complexes, which occurred following junctional premature complexes, failed to initiate tachycardia in the control state but tachycardia was always reinitiated by an identical escape sequence after procainamide. In addition, the tachycardia persisted and was repeatedly spontaneously reinitiated for prolonged periods after procainamide. The pro-arrhythmic effects of procainamide may be explained on the basis of both its vagolytic action on the atrioventricular node as well as by prolongation of refractoriness in the accessory pathway. These observations add to the literature on pro-arrhythmic effects of commonly used antiarrhythmic drugs.

Electrophysiology↗
PubMed1986

Polymorphous ventricular tachycardia: clinical characterization, therapy, and the QT interval.

Forty-five consecutive patients with polymorphous ventricular tachycardia (PVT) were studied. The arrhythmia proved to be of a drug-related cause in 27 and due to an electrolyte disorder in four patients. Coexistent cardiac diseases without metabolic or drug-related abnormalities included ischemic heart disease in three, cardiomyopathy in three, and mitral valve prolapse in two. PVT was exercise-induced in four and associated with bradyarrhythmias in two. A prolonged QT or corrected QT interval was inconsistently related to the occurrence of PVT. In patients in whom PVT was induced by certain type I drugs, other type I antiarrhythmic drugs were usually either ineffective or resulted in aggravation of arrhythmia. For the group as a whole, treatment with lidocaine resulted in inconsistent beneficial effects, while cardiac pacing was almost universally effective for those with drug-induced PVT, regardless of the length of the QT interval. Long-term amiodarone therapy proved safe and effective for 12 of the 24 patients with drug-induced PVT who required long-term therapy for their original arrhythmia. We conclude that identification of PVT is the key clinical issue and that the QT interval is not necessarily the prime abnormality nor the variable to be considered in predicting success of therapy. Temporary cardiac pacing appears to be very effective in the short-term management of these patients. Use of type I antiarrhythmic agents in patients with drug-induced PVT generally resulted in aggravation of arrhythmia. In contrast, long-term amiodarone therapy for control of the original arrhythmia appears to be a promising approach for those with PVT associated with type I agents.

Adolescent↗
PubMed1986

[Absence of a connection between Alzheimer's disease and complement markers].

The hypothesis of a linkage between Alzheimer's disease (AD) (presenile dementia) and the complement components C2, C4, Bf linked to the Major Histocompatibility Complex has been investigated in a large family originating from Calabria, in which AD is transmitted as an autosomal dominant monogenic mendelian trait. The analysis of complotypes of 33 members of a pedigree, from which 10 individuals are affected, allowed to demonstrate that there wasn't any linkage between AD and those markers. Furthermore, no complete or partial deficiency in the C2, C4, Bf components has been observed in affected patients.

Alzheimer Disease↗
PubMed1985

Implementation of periodic acid-thiosemicarbazide-silver proteinate staining for ultrastructural assessment of muscle glycogen utilization during exercise.

Distribution of glycogen particles in semithin and ultrathin sections of biopsy samples from human muscles subjected to either short- or long-term running were investigated using PAS and Periodic Acid-ThioSemiCarbazide-Silver Proteinate (PA-TSC-SP) staining methods. Glycogen particles were predominantly found immediately under the sarcolemma or aligned along the myofibrillar I-band. After long-term exhaustive exercise type-1 fibers with a few or no glycogen particles in the core of the fibers were frequently observed. The subsarcolemmal glycogen stores of these "depleted" type-1 fibers were about three times as large as after exhaustive short-time exercise. Another indication of utilization of subsarcolemmal glycogen stores during anaerobic exercise was that many particles displayed a pale, rudimentary shape. This observation suggests fragmental metabolization of glycogen. Thus, depending on type of exercise and type of fiber differential and sequential glycogen utilization patterns can be observed.

Adolescent↗
PubMed1985

Long-term complete autologous reconstitution following cyclophosphamide and allogeneic marrow infusion in a case of severe aplastic anemia.

A 23 year old man with severe idiopathic aplasia, who had previously been unsuccessfully treated with oxymetholone for a period of 6 months, received two intravenous (IV) infusions of bone marrow cells from his HLA identical brother. He was given 200 mg/kg anti-lymphocyte globulins (ALG) IV, followed by a first infusion of 13 X 10(9) nucleated cells IV, but no improvement was observed. Three months later, he was treated with cyclophosphamide (25 mg/kg over 4 days) followed by a second infusion of 18 X 10(9) nucleated cells IV of the same donor. Within a four month period there was evidence of complete marrow reconstitution, and the patient has experienced no relapse of the disease during the subsequent 13 years follow up period. The mechanism of the recovery is unclear: no evidence of graft acceptance could be established. The respective roles of ALG, cyclophosphamide and/or bone marrow infusions are discussed.

Adult↗
PubMed1984

Two patients with interstitial del (14q), one with features of Holt-Oram syndrome. Exclusion mapping of PI (alpha-1-antitrypsin).

Patient no 1, a boy, was carrier of a de novo del (14) (pter- greater than q23::q32- greater than qter). Patient no 2, a boy, had a de novo del (14) (pter- greater than q23::q24.2- greater than qter). Common dysmorphisms included bushy eyebrows, frontal bossing, and micrognathia. Patient no 2 had features of Holt-Oram syndrome, i.e. congenital heart defect and severe ulnar defect. Patient no 1 had congenital heart defect but no typical osseous disorders. The association of Holt-Oram syndrome and del 14q24.1 is stressed. Patient no 1 was heterozygous for Pl (alpha-1-antitrypsin) phenotypes. The gene locus could thus be excluded from q24 and q31, and tentatively assigned to q32.1.

Abnormalities, Multiple↗
PubMed1983

Red cell enzyme polymorphism in a Breton population, the Bigoudens.

Data are presented on the red cell enzymes of the Bigoudens. Deviations fro the expected Hardy-Weinberg distribution are interpreted as the result of a recent mixing of previously separated groups, in addition to the effect of silent alleles and inbreeding. Special reference to the historical background.

Acid Phosphatase↗
PubMed1983

Adaptive response in human skeletal muscle subjected to prolonged eccentric training.

The peripheral adaptation process associated with repeating eccentric training over a longer period of time was studied in m. vastus lateralis of eleven healthy males aged 24 +/- 4 years. The maximal dynamic concentric muscle strength was only slightly improved after 8 weeks of training. However, eccentric work capacity was dramatically increased (375%). A maximal eccentric stint immediately after fulfilled 8 weeks of training caused a selective glycogen depletion from the type 28 fibers. An increased number of type 2C fibers was observed. The ultrastructure analysis showed an essentially well-preserved fine structure. Volume density of mitochondria was somewhat higher in all fiber types after training. Z-band widths were not affected by eccentric training. It is concluded that skeletal musculature adapts itself in a functional manner to the extreme tension demands put on them. Improved coordination and reorganization of the contractile apparatus of muscle fibers are the determining mechanisms of this adaptation.

Adaptation, Physiological↗
PubMed1983

Plasma protein polymorphisms in a puzzling Breton community, the Bigoudens.

A total of 386 and 393 members of a Breton community, in France, the Bigoudens, have been tested for polymorphisms of C3 and haptoglobin (Hp) plus Transferrin (Tf) respectively. An interesting observation was a gap between the observed and expected phenotype C3 frequencies. The Hp and Tf gene frequencies were not found to be closer to the other Celtic peoples than to the French and English populations.

Alleles↗
PubMed1982

Atypical transmission of ABO blood groups in a French family.

A group AB mother (Mrs P.D.) gave birth to a group O female baby (C.D.). Extensive study of the blood group genetic markers in both the parents and the child, carried out on several occasions, showed nothing unusual outside the ABO system. Mrs P.D. then, gave birth to a second female baby who was also group O. Mrs P.D. had normal amounts of A, B, H and Lewis antigens in her saliva. The H, A and B agglutinability of her red cells was in the range of normal A2B group. This A2B blood group was characterized by very low A gene-specified glycosyltransferase activity in serum. Moreover this activity was undetectable in red blood cell membranes. These results are discussed in the light of various hypotheses in order to explain this unusual transmission of ABO blood group.

ABO Blood-Group System↗
PubMed1981

Human transferrin (Tf) and group-specific component (Gc) subtypes in Tunisia.

Simultaneous subtyping of two genetic markers--group-specific component (Gc) and transferrin (Tf)--by electrofocusing enabled us to compute the following gene frequencies for the Tunisian population: Gc1S, 0.525; Gc1F, 0.260; Gc2, 0.215; TfC1, 0.770; TfC2, 0.215; TfD1, 0.015. The frequencies of TfD, TfC2, and Gc1 are higher than those found in Caucasoid populations and can be explained by Negroid contribution. A selective advantage related to the metabolic role of this vitamin D-binding protein does not seem very likely for any particular Gc type or subtype. It is postulated that the differences in the frequencies of the Gc alleles might be related to selective advantage for genes belonging to other genetic systems originally closely linked to either Gc1 or to Gc2 alleles.

Blood Proteins↗
PubMed1981

Sex limited ahaptoglobinaemia.

Hypohaptoglobinaemia and ahaptoglobinaemia occurred in three generations, mainly to male members of a family. Also small amounts of haptoglobin were detected in most of the female relatives. Haemolytic anaemia seemed likely and the glucose 6 phosphate dehydrogenase (G.6.P.D.) activity was normal. The probable genotype of these apparently healthy individuals was Hp2/Hp2. These preliminary data might suggest a defect in control of gene expression by steroid hormones.

Alleles↗