Hereditary deficiency of the third component of complement in a child with fever, skin rash, and arthralgias: Response to transfusion of whole blood

1977 ◽  
Vol 90 (2) ◽  
pp. 180-186 ◽  
Author(s):  
Stephen G. Osofsky ◽  
Barry H. Thompson ◽  
Thomas F. Lint ◽  
Henry Gewurz
1971 ◽  
Vol 25 (02) ◽  
pp. 354-378 ◽  
Author(s):  
R Gottlob ◽  
L Stockinger ◽  
U Pötting ◽  
G Schattenmann

SummaryIn vitro whole blood clots of various ages, experimental thrombi produced in the jugular vein of rabbits and human thrombi from arteries and veins were examined in semi-thin sections and by means of electron microscopy.In all types of clots examined a typical course of retraction was found. Retraction starts with a dense excentrical focus which grows into a densification ring. After 24 hours the entire clot becomes almost homogeneously dense; later a secondary swelling sets in.Shortly after coagulation the erythrocytes on the rim of the clot are bi-concave discs. They then assume the shape of crenate spheres, turn into smooth spheres and finally become indented ghosts which have lost the largest part of their contents. In the inner zone, which makes up the bulk of the clot, we observed bi-concave discs prior to retraction. After retraction we see no crenations but irregularly shaped erythrocytes. Once the secondary swelling sets in, the cross-section becomes polygonal and later spherical. After extensive hemolysis we observe the “retiform thrombus” made up of ghosts.Experimental and clinical thrombi present the same morphology but are differentiated from in vitro clots by: earlier hemolysis, immigration of leukocytes, formation of a rim layer consisting of fibrin and thrombocytes, and the symptoms of organization. Such symptoms of organization which definitely will prevent lysis with streptokinase were found relatively late in experimental and clinical thrombi. Capillary buds and capillary loops were never found in clinical thrombi prior to the third month.The morphological findings agree with earlier physical and enzymatic investigations. The observation that phenomena of reorganization occur relatively late and frequently only in the rim areas of large thrombi explains why lytic therapy is possible in some of the chronic obliterations.


1988 ◽  
Vol 263 (24) ◽  
pp. 12147-12150 ◽  
Author(s):  
J D Lambris ◽  
D Avila ◽  
J D Becherer ◽  
H J Müller-Eberhard

1974 ◽  
Vol 8 (4) ◽  
pp. 421-421
Author(s):  
Jerry A Winkelstein ◽  
Mary Ruth Smith ◽  
Hyun S Shin ◽  
David H Carver

1962 ◽  
Vol 8 (4) ◽  
pp. 491-499 ◽  
Author(s):  
June M. Stephens

The blood of normal wax moth larvae is not bactericidal for Pseudomonas aeruginosa. The blood becomes moderately bactericidal when larvae are actively immunized against P. aeruginosa. This activity was measured by a modification of Nagington's technique for the estimation of typhoid antibody. Bactericidal activity appears to be the only measurable type of antibody response against P. aeruginosa. Cell-free blood was as active as whole blood; 0.02 ml of immune serum kills about 1000 organisms. The blood of normal wax moth larvae is bactericidal for Shigella dysenteriae but the blood of insects immunized against either this organism or P. aeruginosa shows no increase in activity against S. dysenteriae. A number of non-specific agents, both protein and non-protein, did not stimulate bactericidal activity in serum after their injection into normal larvae. Immune sera prepared against some strains of P. aeruginosa were not active against other strains. Storage at 37 °C or absorption with zymosan both result in blackening of immune blood and loss of bactericidal activity. Bactericidal activity is evident only during the immune state of the insect, i.e. from about 18 hours until the third day after vaccination; it develops at the same time that inhibition of melanization was observed in the blood from vaccinated larvae.


1979 ◽  
Vol 13 (10) ◽  
pp. 1093-1096 ◽  
Author(s):  
Jerry A Winkelstein ◽  
Lawrence E Kurlandsky ◽  
Andrea J Swift

1972 ◽  
Vol 15 (1) ◽  
pp. 49-56 ◽  
Author(s):  
Arthur Weinstein ◽  
Keith Peters ◽  
David Brown ◽  
Rodney Bluestone

PEDIATRICS ◽  
1983 ◽  
Vol 71 (1) ◽  
pp. 81-87
Author(s):  
John J. Roord ◽  
Mohamed Daha ◽  
Wietse Kuis ◽  
Henri A. Verbrugh ◽  
Jan Verhoef ◽  
...  

A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.


PEDIATRICS ◽  
1972 ◽  
Vol 50 (5) ◽  
pp. 688-692
Author(s):  
Anatole S. Dekaban ◽  
Kenton R. Holden ◽  
George Constantopoulos

Repeated fresh plasma or whole blood transfusions were given to five patients with either Hurler, Hunter, or Sanfilippo types of mucopolysaccharidosis. Clinical observations and total 24-hour urinary AMPS and their composition and molecular weight distribution were determined before, during, and after transfusions. The two patients who received plasma transfusions showed no noticeable change in the amount of AMPS excreted; of the three patients who received whole blood transfusions, two had slightly less excretion of AMPS while the third showed no difference. The AMPS in the CSF were measured in one patient before and after blood transfusions and found to be unchanged; likewise, the determination of molecular weights in the isolated AMPS was virtually identical. In the patients studied, the transfusions caused no demonstrable difference in the patients' clinical condition.


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