Prevalence of hereditary properdin, C7 and C8 deficiencies in patients with meningococcal infections

Schlesinger, Menachem; Nave, Z.; Levy, Yael; Slater, Paul E.; Fishelson, Zvi

doi:10.1111/j.1365-2249.1990.tb05350.x

Cited by 60 publications

(10 citation statements)

References 28 publications

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“…Heat-inactivated NHS (HIS) was prepared by heating NHS for 30 min at 56°C. C8-deficient human serum (C8D) was prepared from a C8-deficient patient as described (33). Purified human C8, C9, and complement C9-depleted human serum (C9D-NHS) were purchased from Complement Technology Inc (Tyler, TX).…”

Section: Methodsmentioning

confidence: 99%

Caveolin-1 and Dynamin-2 Are Essential for Removal of the Complement C5b-9 Complex via Endocytosis

Moskovich

Herzog

Ehrlich

et al. 2012

Journal of Biological Chemistry

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Background: Cells resist complement-dependent cytotoxicity by elimination of the membranolytic C5b-9 complex from their surface. Results: Modulations of caveolin-1 and dynamin-2 expression and activity affect C5b-9 endocytosis and cell death. Conclusion: Elimintion of C5b-9 and complement resistance depend on caveolae formation, dynamin activity and lipid rafts. Significance: Targeting of key factors in the C5b-9 elimination pathway may enable us to regulate C5b-9 homeostasis and cell resistance to complement-dependent cytotoxicity.

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Section: Methodsmentioning

confidence: 99%

Caveolin-1 and Dynamin-2 Are Essential for Removal of the Complement C5b-9 Complex via Endocytosis

Moskovich

Herzog

Ehrlich

et al. 2012

Journal of Biological Chemistry

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“…A relatively high frequency of C6 deficiency has been detected in the western Cape population of South Africa (330,358). The association of complement deficiencies with invasive neisserial infections prompted several studies to estimate the frequency of complement deficiencies among patients with their first episode of systemic neisserial infection (24,80,94,109,111,114,134,192,247,313,321,351,363,393). Figueroa and Densen (128) plotted the incidence of complement deficiency detected in patients with meningococcal disease versus the incidence of meningococcal disease in the general population and showed that the rate of complement deficiencies detected dropped in areas where the incidence of disease was high.…”

Section: Frequency Of Hereditary Complement Deficiencies Among Patienmentioning

confidence: 99%

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

2010

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SUMMARY The complement system comprises several fluid-phase and membrane-associated proteins. Under physiological conditions, activation of the fluid-phase components of complement is maintained under tight control and complement activation occurs primarily on surfaces recognized as “nonself” in an attempt to minimize damage to bystander host cells. Membrane complement components act to limit complement activation on host cells or to facilitate uptake of antigens or microbes “tagged” with complement fragments. While this review focuses on the role of complement in infectious diseases, work over the past couple of decades has defined several important functions of complement distinct from that of combating infections. Activation of complement in the fluid phase can occur through the classical, lectin, or alternative pathway. Deficiencies of components of the classical pathway lead to the development of autoimmune disorders and predispose individuals to recurrent respiratory infections and infections caused by encapsulated organisms, including Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. While no individual with complete mannan-binding lectin (MBL) deficiency has been identified, low MBL levels have been linked to predisposition to, or severity of, several diseases. It appears that MBL may play an important role in children, who have a relatively immature adaptive immune response. C3 is the point at which all complement pathways converge, and complete deficiency of C3 invariably leads to severe infections, including those caused by meningococci and pneumococci. Deficiencies of the alternative and terminal complement pathways result in an almost exclusive predisposition to invasive meningococcal disease. The spleen plays an important role in antigen processing and the production of antibodies. Splenic macrophages are critical in clearing opsonized encapsulated bacteria (such as pneumococci, meningococci, and Escherichia coli) and intraerythrocytic parasites such as those causing malaria and babesiosis, which explains the fulminant nature of these infections in persons with anatomic or functional asplenia. Paramount to the management of patients with complement deficiencies and asplenia is educating patients about their predisposition to infection and the importance of preventive immunizations and seeking prompt medical attention.

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“…A study of 104 patients with N meningitidis infections showed that 10% of these individuals were completely deficient for at least one complement component (23). Subsequent studies revealed a complement deficiency of 30% in patients with recurrent episodes of meningococcal sepsis, whereas no defects were found among patients with a single episode of meningococcal infection (24).…”

Section: Discussionmentioning

confidence: 99%

Remitting fever of unknown origin in a 20‐year‐old man

Cinci

Kirschfink

Lorenz

et al. 2012

Arthritis Care & Research

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A 20-year-old man presented with a 12-month history of intermittent febrile episodes; remitting arthritis of the wrists, knees, and lower legs; and disabling myalgia during an attack. He also reported transient erythema on the arms and legs during an attack. Fever and erythema seemed to respond to oral antibiotic therapy and acetaminophen, but arthralgia and myalgia disappeared only slowly. The patient had no signs of oropharyngeal, gastrointestinal, bronchopulmonal, or urogenital infection. Further attacks occurred every 1 to 2 months with identical symptoms and course. The duration of each attack was 3 to 4 days and he was completely asymptomatic between the attacks. Because of the rapid rise and decline of the Creactive protein (CRP) level (Figure 1) and the uniform presentation of the attacks, we had doubts whether the observed partial response to oral antibiotics was really a causal relationship. An autoinflammatory syndrome was suspected and the patient took colchicine 0.5 mg twice a day to prevent further attacks. However, due to gastrointestinal side effects he had to discontinue colchicine after 1 day and we could not evaluate the efficacy of colchicine. A trial of anakinra was initiated during the following attack. Despite significant improvement of all symptoms, including the debilitating myalgia, the fever recurred after a few days and the patient was admitted to the hospital to reevaluate the diagnosis. Medical HistoryThe patient had atopic dermatitis during early childhood, which resolved after a few years. At age 10 years he experienced an episode with fever, reduced global health, distinct purpura on both legs and the lower trunk, severe headache, vomiting, and signs of meningitis. A lumbar puncture finally revealed a positive culture for Neisseria meningitidis. With antibiotic therapy, he recovered rapidly without any persisting symptoms. He did not report any other severe infections and he was not more frequently affected by common infections. Social and Family HistoryThe patient was born in Germany and his family originated from an Eastern European country. His mental development was unremarkable. After secondary school he began working as a storekeeper. The medical history of his 16-year-old sister, his parents, and other relatives was unremarkable. Physical ExaminationThe patient had an athletic stature (height 183 cm, weight 70 kg) and appeared uncomfortable and weak. He was febrile with a temperature of 40°C in the morning and he experienced increasing pain in his left foot for 2 weeks. The pain level reached 3 of 10 during rest and 7 of 10 during exercise. He did not have headaches. Ibuprofen 400 mg reduced the fever but not the pain. Mild swelling appeared at the tendons on the back of his left foot. The sole of the left foot was painful during walking, indicating tenosynovitis of the flexor and extensor tendons of the left foot. The muscles at the cervical spine appeared painful upon palpation. No signs of meningitis, arthritis, exanthema, purpura, serositis, or hepatosplenomegaly were ...

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Prevalence of hereditary properdin, C7 and C8 deficiencies in patients with meningococcal infections

Cited by 60 publications

References 28 publications

Caveolin-1 and Dynamin-2 Are Essential for Removal of the Complement C5b-9 Complex via Endocytosis

Caveolin-1 and Dynamin-2 Are Essential for Removal of the Complement C5b-9 Complex via Endocytosis

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

Remitting fever of unknown origin in a 20‐year‐old man

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