close

Вход

Забыли?

вход по аккаунту

?

HEREDITARY DEFICIENCY OF THE THIRD COMPONENT OF COMPLEMENT IN TWO SISTERS WITH SYSTEMIC LUPUS ERYTHEMATOSUS-LIKE SYMPTOMS.

код для вставкиСкачать
1255
HEREDITARY DEFICIENCY OF THE THIRD
COMPONENT OF COMPLEMENT IN TWO SISTERS
WITH SYSTEMIC LUPUS ERYTHEMATOSUS-LIKE
SYMPTOMS
YUJI SANO, HIROAKI NISHIMUKAI, HAJIME KITAMURA, KAZUYOSHI NAGAKI, SHINYA INAI,
YASUTERU HAMASAKI, IKURO MARUYAMA, and AKIHIRO IGATA
We observed two sisters with lupus-like syndrome with homozygous C3 deficiencies. A 19-year-old
woman and her 15-year-old sister developed malar rash,
arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The
older sister experienced recurrent bronchitis in her
childhood, but the younger sister had no recurrent
infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and
C3 hemolytic activity were extremely low.
In recent years, the development of techniques
for the measurement of individual components of the
complement system has led to the identification of
deficiencies of each component. Now, inherited complement deficiencies have been described for all components (Cl-C9) (1,2). In regard to association with
__
-
-
From Third Department of Internal Medicine, Faculty of
Medicine, Kagoshima University, Kagoshima 890; Department of
Legal Medicine, Kyoto Prefectural University of Medicine, Kamikyo-Ku, Kyoto 602; The Division of Immunology, The Center for
Adult Diseases, Higashinari-Ku, Osaka 537; Department of Clinical
Pathology, Osaka Medical College, Takatsuki 569; and First Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Kagoshima 890, Japan.
Yuji Sano, lkuro Maruyama, and Akihiro Igata: Third
Department of Internal Medicine, Faculty of Medicine, Kagoshima
University; Hiroaki Nishimukai: Department of Legal Medicine,
Kyoto Prefectural University of Medicine; Hajime Kitamura and
Kazuyoshi Nagaki: The Division of Immunology, The Center for
Adult Diseases, Osaka; Shinya Inai: Department of Clinical Pathology, Osaka Medical College; Yasuteru Hamasaki: First Department
of Internal Medicine, Faculty of Medicine, Kagoshima University.
Address reprint requests to Yuji Sano, MD, Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, 1208-1 Usuki-cho, Kagoshima 890,Japan.
Submitted for publication January 9, 1981; accepted in
revised form April 29, 1981.
Arthritis and Rheumatism, Vol. 24, No. 10 (October 1981)
diseases, complement deficiencies are generally divided into three groups (1): 1) deficiencies of the earlyacting complement components (Cl, C4, and C2) are
frequently associated with systemic lupus erythematosus (SLE) or other rheumatic diseases (3); 2) deficiencies of the late-acting complement components (CSC8) are associated with increased susceptibility to
infections, especially to Neisseria infections (43); and
3) in the cases of C3 deficiencies, recurrent bacterial
infections frequently occur (6-9). We found no previous report on C3 deficiency associated with SLE or
lupus-like syndrome.
In this article, C3 deficiencies are reported in 2
sisters with lupus-like syndrome without severe recurrent infections.
CASE REPORT
Patient 1. A 19-year-old woman was referred to
the Kagoshima University Hospital with a diagnosis of
SLE. She had been born after an uneventful pregnancy and delivery. In early childhood, she experienced
recurrent episodes of bronchitis, but she enjoyed good
health since age 10. At age 16, she noticed a spotty
erythematous rash over her chest and abdomen which
disappeared within several months. During the next
year, she had fever, generalized arthralgia, a malar
rash, and photosensitivity. She was given prednisone
(30 mg daily) with some improvement of facial rash,
but intermittent arthralgia continued. There was no
history of proteinuria or hypertension. One notable
physical finding was a patchy, slightly scarred rash
with erythematous border on the face. There was no
lymphadenopathy, and liver and spleen were not palpable.
SANO ET AL
1256
Table 1. Complement component profiles of C3-deficient individuals
Hemolytic activities
Total complement
(CHSO/ml)
c1
c4
c2
c3
c5
C6
c7
C8
c9
Protein concentrations
Clqt
c4
c3
c5
B
CI-INA
C3bINA
Patient 1
Patient 2
Pooled normal
human serum
6.3
5.8
40.0
90,400 (87.9%)*
26.400 (39.8%)
430 (43.9%)
320 (0.7%)
229.000 ( I 10.1%)
7,900 (148.0%)
22,000 (183.3%)
22,400 (81.8%)
29,600 (197.3%)
141
11.7
N DS
110
24.9
1 I5
100
100,000 (97.3%)*
26,400 (39.8%)
380 (38.8%)
270 (0.6%)
229,000 (ll0.1%)
7,200 (134.8%)
19,800 (165.0%)
21,800 (79.6%)
30.000 (200.0%)
102,800
66,400
980
47.000
208,000
5,340
12,Ooo
27.400
15.000
141
11.7
NDS
I30
25.9
I19
I10
* Site forming unitdml. Numbers in parentheses are % of NHS.
t Normal ranges: C l q = 60-130 %NHS; C4 = 20-50 mgldl; C3 = 60-110 mg/dl; C5
%NHS; factor B = 13-23 mg/dl; CI-INA = 75-145 %NHS; C3h-1NA = 60-140 %NHS.
i ND = not detected.
Laboratory findings were as follows: hematocrit 33%; hemoglobin 10.5 gm/dl; white blood cell
count 5,WO/mm3; platelets normal; erythrocyte sedimentation rate 23 mm/hour; urinalysis normal; serum
glutamic oxaloacetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), alkaline phosphatase, and bilirubin all within normal range; total protein 7.8 gmldl with 19.4% y-globulin; IgA 363 mg/dl,
IgM 492 mg/dl, IgG 2,320 mg/dl; and blood urea
nitrogen (BUN) and creatinine normal. Repeated tests
for antinuclear antibodies (ANA) and L E cell preparations gave negative results. Total complement activity
(CHSO) was not detectable.
Patient 2. A 14-year-old girl (younger sister of
patient 1) had been well until age 10, when afacial rash
and arthralgias developed. Soon thereafter she noted
loss of hair. Oral prednisone therapy (15 mg/day)
provided partial relief. At age 13, she had an episode of
skin infection after an intramuscular injection of BCG.
During the next year, after exposure to the sun, she
developed a facial rash and arthralgia which disappeared (except for a mild malar rash) without treatment.
Laboratory findings were as follows: hematocrit 35%; hemoglobin 12.2 gm/dl; white blood cell
count 5,500/mm3; platelets normal; erythrocyte sedi-
=
85-125
mentation rate 52 mm/hour; microscopic hematuria
negative; 24-hour urinary protein was 1 gm; SGOT,
SGPT, alkaline phosphatase, bilirubin, BUN, and serum creatinine all within normal range: total serum
protein 7.5 mg/dl; yglobulin 23.8%; and IgA 238 mg/
dl, IgM 405 mg/dl, IgG 2,560 mg/dl. Repeated tests for
ANA and L E cell preparations gave negative results.
CH5O was not detectable.
Figure 1. Double immunodiffusion analysis of serum of patient 1.
The precipitin line was observed between normal human serum and
antLC3, but not between patient’s serum and anti-C3. In the
younger sister’s (patient 2) serum, the same result was obtained.
HEREDITARY DEFICIENCY OF C3
Table 4. Phagocytic and chemotactic study of patients' sera
Table 2. Reconstitution of CH50 by purified C3
Patient 1
Patient 2
1257
Patient I
Patient 2
NHS
Phagocytosis*
Serum
Serum + C3t
2
40
4
NTS
64
NTS
Chemotactic index5
Serum
Serum + C3t
1.19
I .76
1.09
I .65
1.70
NTS
NHS*
~~
6.3
31.3
Before addition of C3
After addition of C3
5.8
28.2
40
Not tested
~ _ _ _ _ _
* NHS
=
normal human serum
MATERIALS AND METHODS
Sera. Serum samples were obtained from venous
blood clotted at room temperature. They were frozen and
stored at -80°C until use. Sera obtained from 16 healthy
donors were pooled and used as normal control serum
(NHS).
Complement assay. CH5O was determined by the
method of Mayer (10). C3 hemolytic activity was measured
as described previously (2). Diluted samples were incubated
with EAC14oxy2 and excess amount of C5 in gelatin glucose
veronal buffer. After 15 minutes, C6-9 reagent ( 1 1 ) was
added, followed by another incubation at 37°C for 60 minutes. Tubes were centrifuged and degrees of hemolysis were
measured. Hemolytic activities of other complement components were measured a s described previously (12-14). Each
hemolytic titer was expressed as site-forming unit (SFU)/ml.
Complement and complement-related proteins were also
assayed immunochemically. Concentrations of C Iq, C3, C4,
C5, factor B, and C1 inactivator (CI-INA) were measured
by single radial immunodiffusion method. Antisera of these
components were available from the Behringwerke Institute
(Marburg, West Germany). C3b inactivator ( C 3 b I N A ) was
assayed by the electroimmunodiffusion technique of Laurel1
(15), using a n t i - C 3 b l N A sera obtained from Kent Laboratones (Redmond, Washington). C3 was also analyzed by the
Ouchterlony double immunodiffusion method.
Reconstitution of CH50 by purified C3. Human C3
was purified by the method of Tack et al (16). Serum of the
proposita was assayed for CH50 before and after the addition of purified C3, to the amount found in NHS.
Assay for C3-cleaving factor. Vallota's method was
used for assay of C3-cleaving factor ( I 7). Briefly, mixtures of
100 p.1 of N H S and 25 p.1 of the patient's serum were
incubated at 37°C for 30 minutes, and the conversion of C3
and factor B were analyzed by immunoelectrophoresis.
Assay for immune complex. A modification of a
method as described by Ahlstedt et al (18) was used in the
assay for immune complex. We used 3.0 kg of C l q per tube
for absorption o n the inner surfaces of polystyrene tubes by
Table 3. Bactericidal activity of patients' sera
Serum
Serum
Serum
Serum
+ Ab*
+ C3t
+ Ab + C3t
Patient I
Patient 2
NHS
16
16
32
64
16
16
64
64
64
NTS
32
NTS
* The number of PMN that had ingested bacteria.
t Purified human C3 was added to the amount in NHS.
S NT =: not tested.
5 The number of cells responding to serum divided by the nuniher
of cells responding to control buffer.
Table 5. Total hemolytic activities and functional a n d
immunochemical C3 levels in the family members
C3 concentrations*
~Immunochemical
Functional
CH50 plml
mg/dlt
SFU/mlt
Subjects
111-2
4
5
IV-5
7
8
10
11
12
14
15
17
18
19
v-1
2
3
4
5
6
7
8
9
10
II
12
13
17
18
32.5
33.9
29.0
38.8
30.9
33.9
35.3
38.8
31.6
31.8
38.4
42.6
31.3
38.4
41.4
33.4
32.5
36.5
6.3
5.8
33.4
44.1
41.3
39.8
34.3
36.5
36.4
32.1
38.6
44.1
50 (58.1)
77 (89.5)
44 (5 I .2)
56 (65.1)
38 (44.2)
77 (89.5)
77 (89.5)
92 (107.0)
77 (89.5)
50 (58. I )
114 (132.6)
100 (1 16.3)
50 (58. I )
114 (132.6)
114 (132.6)
56 (65. I )
50 (58.1)
92 (107.0)
ND
ND
44 ( 5 I .2)
72 (83.7)
100 (1 16.3)
114 (132.6)
100 ( I 16.3)
92 (107.0)
86 (100.0)
86 (100.0)
72 (83.7)
100 (1 16.3)
NHS
40.0
86(100.0)
9
* Ah = lysozyme-free antibody against E coli B was added.
t Purified human C3 was added to the amount in NHS.
* ND
S NT
t % of N H S
=
not tested.
=
not detected. N T
=
not tested.
29,600 (63.0)
40,000 (85.1)
24,000 (51.1)
30.100 (63.8)
21,200 (44.7)
34,000 (72.4)
34.300 (73.0)
46.000 (97.9)
35,000 (75.0)
27,000 (57.4)
N.r
54.000 ( I 15.0)
20.300 (43.2)
54.000 (115.0)
51,000 (108.5)
22,500 (57.9)
21.000 (44.7)
42.700 (90.9)
320 (0.7)
270 (0.6)
27,000 (57.4)
40.900 (87.0)
52.500 ( I 11.7)
52.700 (112.1)
45.500 (96.8)
54,700 ( I 16.4)
43,500 (92.6)
38.000 (80.9)
43,000 (91 .5)
NT
47,000
SANO ET AL
1258
I
II
m
m
V
@
Proposed Homorygotes
0
Not tosted
Proposed Heterorygotes
00
NormalC3
Figure 2. Pedigree of the family with C3 deficiency. Males are indicated by squares, females by circles. Patient 1 is
indicated by the arrow.
incubation at 4°C for 17 hours. The excess of Clq was rinsed
away just before use, and diluted patient’s serum was added.
Incubation was performed at room temperature for 2 hours.
The tubes were rinsed again, and horseradish peroxidaselabeled rabbit anti-human IgG antibodies were added. The
tubes were incubated for 2 hours at room temperature, and
then they were rinsed again. The enzyme activity remaining
in the tubes was then measured by adding 4-aminoantipyrine
and hydroquinone-methyl-ether. The light absorption at 5 10
nm was registered after 90 minutes at 50°C. The concentration of immune complex was expressed as equivalents of
aggregated human IgG in CLg/ml.
Assay for bactericidal activity, phagocytosis, and chemotaxis. Immune bactericidal activity of the patient’s sera
was assayed using Escherichia coli B/SM strain 004 by the
microtiter plates as described by Inoue et al (19). To one
drop of serially diluted test serum in Tris-buffered saline
containing 1 .O mM MgClz and 0.15 mM CaCI2, the following
were added: one drop of antibody (lysozyme-free) against E
cofi diluted appropriately in the buffer or buffer alone, one
drop of purified human C3 or buffer alone, and another drop
of bacterial suspension. After incubation at 37°C for 1 hour,
one drop of 1 mg/ml streptomycin dissolved in tripticase soy
broth was added to the mixture, followed by further incubation at 37°C overnight. The viability of bacteria was observed macroscopically, and grades of growth were expressed as the titer from 0 (no growth) to 4. The bactericidal
activity of the test serum was expressed as the reciprocal of
the serum dilution that inhibited the bacterial growth to the
level of grade 2.
Bacterial uptake by polymorphonuclear leukocytes
(PMN) was quantitatively measured by a modification of a
method as described by Quie et al (20,21). Briefly, each
phagocytic mixture contained 2 x lo7 Streptococciis pyogenes type 12 organisms, 5 x lWPMN, and 0.04 ml test
serum in a total volume of 0.24 ml. The phagocytic mixtures
were incubated at 37°C for 15 minutes. The phagocytosis of
bacteria by PMN was determined by preparing smears of
phagocytic mixture, staining with Giemsa stain, and determining the number of 500 PMN that had ingested bacteria.
The procedure of Nelson et al (22) was employed to
measure chemotaxis. Quantitation of chemotaxis was done
by measuring the linear distance that the cells had migrated
from the margin of the well toward the chemotactic factor
(distance A: chemotaxis) and the distance the cells had
migrated from the margin of the well toward the control
medium (distance B: spontaneous migration). The chemotactic index was represented by N B .
Genetic typing of C3. Phenotypes of C3 of all family
members were determined by high voltage agarose gel
electrophoresis as described previously (23).
RESULTS
Complement study. Titers of the complement
components and complement-related proteins in sera
of patient 1 and patient 2 are presented in Table 1. The
complement profiles of these patients were similar.
CH50 and functional C 3 were found in only trace
amounts; C 2 and C4 activities were decreased, whereas C9 levels were increased. Other complement com-
HEREDITARY DEFICIENCY OF C3
ponents were within normal range. C3 protein was not
detected, and the amount of C4 protein was reduced.
However, Clq, C5, factor B, CI-INA, and C3b-INA
proteins were found in normal amounts.
The absence of C3 in sera of these patients was
confirmed by double immunodiffusion analysis (Figure
1). A precipitin line was not observed between their
sera and anti-C3.
Table 2 shows the reconstitution study. After
addition of purified C3, serum CH5O of patients 1 and 2
were restored from 6.3 to 31.3 units and from 5.8 to
28.2 units, respectively.
C3 and factor B conversion was not found in the
reaction mixtures of NHS and the serum of the patients. Furthermore, CH50 in these mixtures was not
reduced. By this result, the presence of C3-cleaving or
C3-inhibiting factor(s), which might be responsible for
C3 depression in their sera, was disproved.
Immune complex assay. Values of immune complex in sera of both patients were 44.2 &ml aggregated IgG and 9.7 pg/ml aggregated IgG. Normal human
serum gave values less than 3 pg/ml aggregated IgG.
Studies of complement-mediated functions. Table 3 represents the results of bactericidal study.
Serum bactericidal activities of patients were reduced
when compared with normal human serum. Normal
bactericidal activities were restored upon the addition
of purified C3. The phagocytic activities were significantly reduced in patients’ sera compared to control
sera, and chemotactic activities in their sera were also
reduced (Table 4).
Family study. Sera of family members were
assayed for CH5O and functional and immunochemical
C3. As shown in Table 5, CHSO was reduced, and C3
levels were approximately half of NHS in 9 of the
tested family members, suggesting that these 9 subjects are heterozygous for C3 deficiency. Twenty
other family members showed normal complement
profiles. The family tree is shown in Figure 2. C3
phenotype was determined by high voltage agarose gel
electrophoresis; the family members, except the proposita and her sister, had C3-S. No other types, such
as C3-F and C3-FS. could be detected.
DISCUSSION
Two sisters with lupus-like syndrome were
found to be homozygous C3 deficient. In both cases,
serum C3 was not detected immunochemically, and
their C3 activities were only 0.7 and 0.6% of NHS.
1259
Their serum CH50 was restored by addition of purified
C3. Their complement profiles (decreased C4 and C2,
trace C3, and increased C9) were similar to other C3
deficiencies reported previously (6,7,24). C3-cleaving
or C3-inhibiting factor, such as C3 nephritic factor (25)
and the endogenous C3-cleaving enzyme (26), were
not detected in their sera. In both sera, immune
complexes were detected, but C lq concentrations and
functional C 1 were normal. Therefore circulating immune complexes would not explain the decreased C2
and C4 or cause the absence of C3.
The C3 phenotypes in sera of the family members, except the two sisters, were C3-S. From the
results of the measurement of C3 protein and the
typing, it is probable that the genotype of the proposed
heterozygous deficiency is C3”/C3- and that of the
proposed homozygote is C3-/C3-. The mode of inheritance of C3 in the family would be consistent with an
autosomal codominant trait as reported in other C3
deficiencies (8,9,24,27).
The present patients were quite different from
others with C3 deficiencies described previously (69,24) in regard to the clinical manifestations of the
affected individual. The symptoms of the 2 sisters
were similar. They had erythematous malar rash,
intermittent arthralgia, fever, and photosensitivity.
Though these clinical manifestations were suggestive
of SLE, neither ANA nor LE cell was detected.
Complement component deficiencies are known to be
associated frequently with SLE or lupus-like syndromes. However, there have been no reports of a
case with SLE or lupus-like syndromes associated
with C3 deficiencies. The previously reported C3
deficiencies (6-9) were associated with recurrent, severe, and occasionally life-threatening infections with
onset in infancy or early childhood. In our patients,
the elder sister had experienced recurrent bronchitis
only in her childhood, and the younger sister had no
episodes of that kind of infection. However, bactericidal activities, phagocytosis, and chemotaxis were
reduced in both patients’ sera. Interpretation of this
dissociation is difficult. Recently, Pussel et a1 (28)
reported a family in which three C3 deficiencies were
associated with nephritis. In the present subjects,
however, no clinical evidence of nephritis was found.
Alloalbuminemia was found incidentally in both
sisters. The alloalbumin migrated slower than the
common t y p e on electrophoresis. Close linkage was
not found between C3 and albumin loci. Details of the
genetic features of the alloalbuminemia in the family
will be reported elsewhere.
SANO ET AL
1260
ACKNOWLEDGMENTS
We are indebted to Dr. Seigo Ono, Department of
Pediatrics, Faculty Medicine, Kagoshima University, for
phagocytosis and chemotaxis studies. We thank Dr. Ietoshi
Ohno for his contributions to family study.
REFERENCES
1. Agnello V: Complement deficiency states. Medicine
57: 1-23, 1978
2. Inai S, Kitamura H, Hiramatsu S, Nagaki K: Deficiency
of the ninth complement component in man. J Clin Lab
Immunol 2%-87, 1979
3. Agnello V: Association of systemic lupus erythematosus
and SLE-like syndromes with hereditary and acquired
complement deficiency states. Arthritis Rheum (suppl)
2 1 :S 146-S 152, 1978
4. Petersen BH, Lee TJ, Snyderman R, Brooks GF: Neisseria meningitidis and Neisseria gonorrhoeae bacteremia associated with C6, C7 or C8 deficiency. Ann
Intern Med 90:917-920, 1979
5. Haeney MR, Thompson RA, Faulkner J, Mackintosh P,
Ball AP: Recurrent bacterial meningitis in patients with
genetic defects of terminal complement components.
Clin Exp Immunol 40: 16-24, 1980
6. Alper CA, Colten HR, Rosen FS, Rabson AR, Macnab
GM, Geer JS: Homozygous deficiency of C3 in a patient
with repeated infections. Lancet 2: 1179-1 181, 1972
7. Ballow M, Shira JE, Harden L, Yang SY, Day NK:
Complete absence of the third component of complement in man. J Clin Invest 56:703-710, 1975
8. Grace HJ, Brereton-Stiles GG, Vos GH, Schonland M:
A family with partial and total deficiency of complement
C3. S Afr Med J 50: 139-140, 1976
9. Davis AE 111, Davis JS, Rabson AR, Osofsky SG,
Colten HR, Rosen FS, Alper CA: Homozygous C3
deficiency: detection of C3 by radioimmunoassay. Clin
Immunol Immunopathol 8:543-550, 1977
10. Mayer MM: Complement and complement fixation,
Experimental Immunochemistry. Second edition. Edited by EA Kabat, MM Mayer. Springfield, Illinois,
Charles C Thomas, 1961, pp 133-240
11. Cooper NR, Muller-Eberhard HJ: The reaction mechanism of human C5 in immune hemolysis. J Exp Med
132:775-793, I970
12. Hoffman LG, Lepow IH, Mayer MM, Muller-Eberhard
HJ: Complement, Methods in Immunology and Immunochemistry. Vol. IV. Edited by CA Williams, MW
Chase. New York, Academic Press, 1977, pp 127-274
13. Kitamura H , Inai S: Molecular analysis of the reaction
of C9 with EACI-8. J Immunol 113:1992-2003, 1974
14. Inai S, Nagaki K, Ebisu S, Kato K, Kotani S, Misaki A:
Activation of the alternative complement pathway by
water-insoluble glucans of streptococcus mutants: the
relation between their chemical structures and activating
potencies. J Immunol 117: 1256-1260, 1976
15. Laurell CB: Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal
Biochem 15:45-52, 1966
16. Tack BF, Prahl JW: Third component of human complement: purification from plasma and physicochemical
characterization. Biochemistry 15:45 13-4521, 1976
17. Vallota EA, Gotze 0, Spiegelberg HL, Fomstal J, West
CD, Muller-Eberhard HJ: A serum factor in chronic
hypocomplementemic nephritis distinct from immunoglobulins and activating the alternate pathway of complement. J Exp Med 139:1249-1261, 1974
18. Ahlstedt S, Hanson LA, Wadsworth C: A Clq immunosorbent assay compared with thin-layer gel filtration for
measuring IgG aggregates. Scand J Immunol5:293-298,
1976
19. Inoue K, Yano K. Amano T: Chemical studies on
damages of Escherichia cofi by the immune bactericidal
reaction. 11. Release of phosphatidyl-ethanolamine from
a phospholipase A-deficient mutant of E coli during the
immune bactericidal reaction. Biken J 17: 135-140, 1974
20. Quie PG, White JG, Holmes B. Good RA: In vitro
bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous
disease of childhood. J Clin Invest 46568-679, 1%7
21. Kim MH, Rodey GE, Good RA, Chilgren RA. Quie PG:
Defective candidacidal capacity of polymorphonuclear
leukocytes in chronic granulomatous disease of childhood. J Pediatr 75:300-303, 1969
22. Nelson RD, Quie PG, Simmons RL: Chemotaxis under
agarose: a new and simple method for measuring chemotaxis and spontaneous migration of human polymorphonuclear leukocytes and monocytes. J Immunol
115:1650-1656, 1975
23. Teisberg P: High voltage agarose gel electrophoresis in
the study of C3 polymorphism. Vox Sang 19:47-56, 1970
24. Osofsky SG, Thompson BH, Colonel L, Lint TF,
Gewurz H: Hereditary deficiency of the third component of complement in a child with fever, skin rash and
arthralgia: response to transfusion of whole blood. J
Pediatr 90:180-186, 1977
25. Spitzer RE, Vallota EH, Forristal J, Stitzel A, Davis
NC, West CD: Serum C3 lytic system in patients with
glomerulonephritis. Science 164:436-437, 1964
26. Alper CA, Blork KJ, Rosen FS: Increased susceptibility
to infection in a patient with type I1 essential hypercatabolism of C3. N Engl J Med 288:601-606, 1973
27. Alper CA, Colten HR, Gear JSS, Rabson AR, Rosen
FS: Homozygous human C3 deficiency: the role of C3 in
antibody production, C 1s-induced vasopermeability,
and cobra venom-induced passive hemolysis. J Clin
Invest 57:222-229, 1976
28. Pussell BA, Bourke E, Nayef M, Moms S, Peters DK:
Complement deficiency and nephritis: a report of a
family. Lancet I: 675-677, 1980
Документ
Категория
Без категории
Просмотров
1
Размер файла
514 Кб
Теги
like, two, lupus, complement, sister, deficiency, components, systemic, erythematosus, hereditary, symptom, third
1/--страниц
Пожаловаться на содержимое документа