Meningococcemia

Meningococcemia

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Meningococcemia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Meningococcal Disease
  • Meningococcemia-Meningitis

Disorder Subdivisions

  • Fulminant Meningococcemia (Waterhouse-Friderichsen Syndrome)
  • Chronic Meningococcemia

General Discussion

Meningococcemia is a rare infectious disease characterized by upper respiratory tract infection, fever, skin rash and lesions, eye and ear problems, and possibly a sudden state of extreme physical depression (shock) which may be life-threatening without appropriate medical care. There are two forms of meningococcemia. Fluminant meningococcemia develops very rapidly and is more severe than chronic meningococcemia, which has a waxing and waning course.

Symptoms

Meningococcemia is characterized by sudden intense headache, nausea, fever, vomiting, and skin rash. The affected individual may first complain of an upper respiratory infection. Chills may develop, then skin rash on the arms or legs and the trunk. Diarrhea may also be present. Later the rash may become widespread or develop into bleeding spots under the skin (petechiae, ecchymoses, or purpura). There may be associated swelling, muscle pain, skin deterioration or gangrene in the arms and legs. Pneumonia may also develop along with the other symptoms if the affected individual has a suppressed immune system.



In cases where meningitis occurs along with meningococcemia, the affected individual may have the symptoms listed above along with the combination of headache, confusion, stiff neck, and muscle pain from irritation of membranes surrounding the brain and spinal cord (meningismus). (For more information on this disorder, choose "Meningitis" as your search term in the Rare Disease Database).



Fulminant Meningococcemia is also known as Waterhouse-Friderichsen Syndrome and is the most severe form of the disorder. The disease comes on very suddenly and the progression of the symptoms is very rapid. In less than a few hours the affected individual may experience very high fever, chills, weakness, vomiting and severe headache. A red rash appears on the arms and legs and spreads very quickly over the body including the eyes and nose. In addition, the affected individuals blood pressure may drop dangerously, the fever may drop dramatically, and they may go into shock. Without immediate medical treatment this disorder can be life-threatening.



Chronic Meningococcemia is a rarer form of the disease. It is characterized by fever that comes and goes over a period of weeks or months. Muscle and joint pain with headache as well as a skin rash may also come and go. This form of the disorder may also include an enlarged spleen.

Causes

Meningococcemia is caused by infection with the meningococci bacteria (Neisseria memingitidis) which are gram-negative diplococci bacteria. There are various groups of this bacteria that cause different forms of the disease and they are grouped by strains A, B, C, D, X, Y, Z, 29E and W135. These groups can be identified by testing the blood, scrapings of the skin rash and samples of the cerebrospinal fluid of the patient. Testing may take up to five days as the cultures are very slow growing.



Infection with the bacteria is usually caused by a carrier. The natural place for the bacteria to be located is in either the nose or throat of the carrier, and they can be spread the infection through airborne or close contact methods. The carrier may spread the infection for weeks or months if they are not diagnosed and treated.

Affected Populations

Meningococcemia affects males and females in equal numbers. However, most cases develop in persons twenty years of age or younger and half of these cases are in children under five years of age. In the United States 1.2 cases per 100,000 occur annually. Winter and spring are the most common seasons of the year when cases are reported. Epidemics can occur under crowded conditions and tend to occur at 20 to 30 year intervals. In other parts of the world epidemics are usually caused by the Group A strain of the bacteria. During epidemics, rates of 5 to 24 cases per 100,000 persons have occurred. In Sao Paulo, Brazil, during 1974 the epidemic rate was 370 per 100,000 persons infected with Meningococcemia. In the United States, the most prevalent Group strains of the bacteria are B,C,Y, and W-135.

Standard Therapies

Diagnosis

The diagnosis of Meningococcemia may be confirmed by a thorough clinical evaluation and specialized blood tests.



Treatment

Meningococcemia is usually treated with Penicillin or Ampicillin. In adults the method of treatment is often through intravenous Penicillin G. In children penicillin is still the treatment of choice, however, other organisms must be ruled out before treatment is begun. For persons who are unable to take penicillin, other antibiotics are used such as: cefuroxime, cefotaxime or ceftriaxone.



In persons who survive severe meningococcal septicemia there may be ongoing problems with veins and arteries. There are usually serious orthopedic problems. If gangrene occurs amputation may be necessary. These patients should have continuing medical evaluations as a precaution against other conditions that can arise in later years.



During times of epidemics, prophylaxis with other antibiotics (i.e., Rifampin, minocycline, and sulfadiazine) is used to protect persons exposed to or in close contact with infected patients.

Investigational Therapies

A new orphan product used for the prevention and treatment of purpura fulminans in Meningococcemia is being developed by Immuno Clinical Research Corp. of New York. The name of the new product is Protein C Concentrate (Protein C Concentrate (Human) Vapor Heated, Immuno).



Researchers are studying the safety and effectiveness of an experimental vaccine for Meningococcemia.



The drug recombinant bactericidal/permeability-increasing protein (Neuprex) has received an orphan drug designation for use in the treatment of Meningococcemia. More studies are needed to determine the long-term safety and effectiveness of this treatment of Meningococcemia. For more information, contact:



Xoma Corporation

2910 Seventh Street

Berkeley, CA 94710



Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Cecil Textbook of Medicine, 20th Ed.: J. Claude Bennett and Fred Plum, Editors; W.B. Saunders Co., 1996. Pp. 1619-21.



Harrison's Principles of Internal Medicine, 14th Ed.: Kurt J. Isselbacher, M.D. et al., Editors; McGraw-Hill, Inc., 1998. P. 912.



Internal Medicine, 4th Ed.: Jay H. Stein, Editor-In-Chief; Mosby-Year Book, Inc., 1994. P. 2114.



Infectious Diseases: Sherwood L. Gorbach, John G. Bartlett, and Neil R. Blacklow, Editors; W.B. Saunders Company, 1992. P. 1455.



Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases, 4th Ed.: Gerald L. Mandell, M.D. et al., Editors; Churchill Livingstone Inc., 1995. Pp. 1899-1906.



Clinical Dermatology, 2nd Ed.: Thomas P. Habif, Editor; The C.V. Mosby Company, 1990. Pp. 210-11.



JOURNAL ARTICLES

Chondro-Osseous Growth Abnormalities After Meningococcemia, A Clinical and Histopathological Study. D. P. Grogan et al.; J Bone Joint Surg (July 1989; 71(6)). Pp. 920-28.



Meropenem Pharmacokinetics in a Patient with Multiorgan Failure From Meningococcemia Undergoing Continuous Venovenous Hemodiaflitration. M. M. Meyer et al.; Am J Kidney Dis (Apr 1999; 33(4)). Pp. 790-95.

Resources

Centers for Disease Control and Prevention

1600 Clifton Road NE

Atlanta, GA 30333

Tel: (404)639-3534

Tel: (800)232-4636

TDD: (888)232-6348

Email: cdcinfo@cdc.gov

Internet: http://www.cdc.gov/



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



National Meningitis Association

P.O. Box 725165

Atlanta, GA 31139

Tel: (866)366-3662

Fax: (877)703-6096

Email: support@nmaus.org

Internet: http://www.nmaus.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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