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Toxic shock syndrome (TSS) is a condition caused by bacterial toxins. Symptoms may include
fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
,
rash A rash is a change of the human skin which affects its color, appearance, or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, c ...
,
skin peeling Desquamation occurs when the outermost layer of a tissue, such as the skin, is shed. The term is . Physiologic desquamation Keratinocytes are the predominant cells of the epidermis, the outermost layer of the skin. Living keratinocytes reside in ...
, and low blood pressure. There may also be symptoms related to the specific underlying infection such as mastitis,
osteomyelitis Osteomyelitis (OM) is an infection of bone. Symptoms may include pain in a specific bone with overlying redness, fever, and weakness. The long bones of the arms and legs are most commonly involved in children e.g. the femur and humerus, while the ...
,
necrotising fasciitis Necrotizing fasciitis (NF), also known as flesh-eating disease, is a bacterial infection that results in the death of parts of the body's soft tissue. It is a severe disease of sudden onset that spreads rapidly. Symptoms usually include red o ...
, or
pneumonia Pneumonia is an inflammatory condition of the lung primarily affecting the small air sacs known as alveoli. Symptoms typically include some combination of productive or dry cough, chest pain, fever, and difficulty breathing. The severit ...
. TSS is typically caused by
bacteria Bacteria (; singular: bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were am ...
of the ''
Streptococcus pyogenes ''Streptococcus pyogenes'' is a species of Gram-positive, aerotolerant bacteria in the genus '' Streptococcus''. These bacteria are extracellular, and made up of non-motile and non-sporing cocci (round cells) that tend to link in chains. They ...
'' or ''
Staphylococcus aureus ''Staphylococcus aureus'' is a Gram-positive spherically shaped bacterium, a member of the Bacillota, and is a usual member of the microbiota of the body, frequently found in the upper respiratory tract and on the skin. It is often posit ...
'' type, though others may also be involved. Streptococcal toxic shock syndrome is sometimes referred to as toxic-shock-like syndrome (TSLS). The underlying mechanism involves the production of superantigens during an invasive streptococcus infection or a localized staphylococcus infection. Risk factors for the staphylococcal type include the use of very absorbent tampons, skin lesions in young children characterized by fever, low blood pressure, rash, vomiting and/or diarrhea, and multiorgan failure. Diagnosis is typically based on symptoms. Treatment includes
intravenous fluids Intravenous therapy (abbreviated as IV therapy) is a medical technique that administers fluids, medications and nutrients directly into a person's vein. The intravenous route of administration is commonly used for rehydration or to provide nutri ...
,
antibiotics An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting bacterial infections, and antibiotic medications are widely used in the treatment and preventio ...
, incision and drainage of any
abscess An abscess is a collection of pus that has built up within the tissue of the body. Signs and symptoms of abscesses include redness, pain, warmth, and swelling. The swelling may feel fluid-filled when pressed. The area of redness often extends ...
es, and possibly
intravenous immunoglobulin Immunoglobulin therapy is the use of a mixture of antibodies (normal human immunoglobulin or NHIG) to treat several health conditions. These conditions include primary immunodeficiency, immune thrombocytopenic purpura, chronic inflammatory ...
. The need for rapid removal of infected tissue via surgery in those with a streptococcal cause, while commonly recommended, is poorly supported by the evidence. Some recommend delaying surgical debridement. The overall risk of death is about 50% in streptococcal disease, and 5% in staphylococcal disease. Death may occur within 2 days. In the United States, streptococcal TSS occurs in about 3 per 100,000 per year, and staphylococcal TSS in about 0.5 per 100,000 per year. The condition is more common in the
developing world A developing country is a sovereign state with a lesser developed industrial base and a lower Human Development Index (HDI) relative to other countries. However, this definition is not universally agreed upon. There is also no clear agreeme ...
. It was first described in 1927. Due to the association with very absorbent tampons, these products were removed from sale.


Signs and symptoms

Symptoms of toxic shock syndrome (TSS) vary depending on the underlying cause. TSS resulting from infection with the bacterium ''Staphylococcus aureus'' typically manifests in otherwise healthy individuals via signs and symptoms including high
fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
, accompanied by low
blood pressure Blood pressure (BP) is the pressure of circulating blood against the walls of blood vessels. Most of this pressure results from the heart pumping blood through the circulatory system. When used without qualification, the term "blood pressure ...
,
malaise As a medical term, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. The word has existed in French since at least the 12th century. The term is often used ...
and confusion, which can rapidly progress to
stupor Stupor is the lack of critical mental function and a level of consciousness, in which an affected person is almost entirely unresponsive and responds only to intense stimuli such as pain. The word derives from the Latin '' stupor'' ("numbness, inse ...
,
coma A coma is a deep state of prolonged unconsciousness in which a person cannot be awakened, fails to respond normally to painful stimuli, light, or sound, lacks a normal wake-sleep cycle and does not initiate voluntary actions. Coma patients exhi ...
, and multiple organ failure. The characteristic rash, often seen early in the course of illness, resembles a sunburn (conversely, streptococcal TSS will rarely involve a sunburn-like rash), and can involve any region of the body including the lips, mouth, eyes, palms and soles of the feet. In patients who survive, the rash desquamates (peels off) after 10–21 days. STSS caused by the bacterium ''
Streptococcus pyogenes ''Streptococcus pyogenes'' is a species of Gram-positive, aerotolerant bacteria in the genus '' Streptococcus''. These bacteria are extracellular, and made up of non-motile and non-sporing cocci (round cells) that tend to link in chains. They ...
'', or TSLS, typically presents in people with pre-existing skin infections with the bacteria. These individuals often experience severe pain at the site of the skin infection, followed by rapid progression of symptoms as described above for TSS.


Pathophysiology

In both TSS (caused by ''S. aureus'') and TSLS (caused by ''S. pyogenes''), disease progression stems from a
superantigen Superantigens (SAgs) are a class of antigens that result in excessive activation of the immune system. Specifically it causes non-specific activation of T-cells resulting in polyclonal T cell activation and massive cytokine release. SAgs are ...
toxin. The toxin in ''S. aureus'' infections is TSS Toxin-1, or TSST-1. The TSST-1 is secreted as a single polypeptide chain. The gene encoding toxic shock syndrome toxin is carried by a mobile genetic element of ''S. aureus'' in the SaPI family of
pathogenicity island Pathogenicity islands (PAIs), as termed in 1990, are a distinct class of genomic islands acquired by microorganisms through horizontal gene transfer. Pathogenicity islands are found in both animal and plant pathogens. Additionally, PAIs are found i ...
s. The toxin causes the non-specific binding of MHC II, on professional antigen presenting cells, with T-cell receptors, on T cells. In typical T-cell recognition, an antigen is taken up by an antigen-presenting cell, processed, expressed on the cell surface in complex with class II major histocompatibility complex (MHC) in a groove formed by the alpha and beta chains of class II MHC, and recognized by an antigen-specific T-cell receptor. This results in polyclonal T-cell activation. Superantigens do not require processing by antigen-presenting cells but instead, interact directly with the invariant region of the class II MHC molecule. In patients with TSS, up to 20% of the body's T-cells can be activated at one time. This polyclonal T-cell population causes a
cytokine storm A cytokine storm, also called hypercytokinemia, is a physiological reaction in humans and other animals in which the innate immune system causes an uncontrolled and excessive release of pro-inflammatory signaling molecules called cytokines. Norma ...
, followed by a multisystem disease.


Risk factors

A few possible causes of toxic shock syndrome are: * Cuts or open wounds on the skin * Surgical wounds * Viral infections (e.g. Chicken Pox) * Contraceptive sponges * History of a recent birth, miscarriage, or abortion * Using super-absorbent tampons * Previously having TSS


Diagnosis

For staphylococcal toxic shock syndrome, the diagnosis is based upon
CDC The Centers for Disease Control and Prevention (CDC) is the national public health agency of the United States. It is a United States federal agency, under the Department of Health and Human Services, and is headquartered in Atlanta, Georg ...
criteria defined in 2011, as follows: #
Body temperature Thermoregulation is the ability of an organism to keep its body temperature within certain boundaries, even when the surrounding temperature is very different. A thermoconforming organism, by contrast, simply adopts the surrounding temperatur ...
> 38.9 °C (102.02 °F) # Systolic
blood pressure Blood pressure (BP) is the pressure of circulating blood against the walls of blood vessels. Most of this pressure results from the heart pumping blood through the circulatory system. When used without qualification, the term "blood pressure ...
< 90 mmHg # Diffuse
macular A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this sy ...
erythroderma #
Desquamation Desquamation occurs when the outermost layer of a tissue, such as the skin, is shed. The term is . Physiologic desquamation Keratinocytes are the predominant cells of the epidermis, the outermost layer of the skin. Living keratinocytes reside in ...
(especially of the palms and soles) 1–2 weeks after onset # Involvement of three or more organ systems: #* Gastrointestinal (
vomiting Vomiting (also known as emesis and throwing up) is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenter ...
,
diarrhea Diarrhea, also spelled diarrhoea, is the condition of having at least three loose, liquid, or watery bowel movements each day. It often lasts for a few days and can result in dehydration due to fluid loss. Signs of dehydration often begin w ...
) #* Muscular: severe
myalgia Myalgia (also called muscle pain and muscle ache in layman's terms) is the medical term for muscle pain. Myalgia is a symptom of many diseases. The most common cause of acute myalgia is the overuse of a muscle or group of muscles; another li ...
or creatine phosphokinase level at least twice the upper limit of normal for laboratory #*
Mucous membrane A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It i ...
hyperemia (vaginal, oral, conjunctival) #*
Kidney failure Kidney failure, also known as end-stage kidney disease, is a medical condition in which the kidneys can no longer adequately filter waste products from the blood, functioning at less than 15% of normal levels. Kidney failure is classified as eit ...
( serum creatinine > 2 times normal) #*
Liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it i ...
inflammation (bilirubin, AST, or ALT > 2 times normal) #*
Low platelet count Thrombocytopenia is a condition characterized by abnormally low levels of platelets, also known as thrombocytes, in the blood. It is the most common coagulation disorder among intensive care patients and is seen in a fifth of medical patients and ...
(platelet count < 100,000 / mm3) #*
Central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all p ...
involvement (confusion without any focal neurological findings) # Negative results of: #* Blood, throat, and CSF cultures for other bacteria (besides ''S. aureus'') #* Negative serology for ''
Rickettsia ''Rickettsia'' is a genus of nonmotile, gram-negative, nonspore-forming, highly pleomorphic bacteria that may occur in the forms of cocci (0.1 μm in diameter), bacilli (1–4 μm long), or threads (up to about 10 μm long). The term "ricke ...
'' infection,
leptospirosis Leptospirosis is a blood infection caused by the bacteria '' Leptospira''. Signs and symptoms can range from none to mild (headaches, muscle pains, and fevers) to severe ( bleeding in the lungs or meningitis). Weil's disease, the acute, sever ...
, and
measles Measles is a highly contagious infectious disease caused by measles virus. Symptoms usually develop 10–12 days after exposure to an infected person and last 7–10 days. Initial symptoms typically include fever, often greater than , cough, ...
Cases are classified as confirmed or probable as follows: * Confirmed: All six of the criteria above are met (unless the patient dies before desquamation can occur) * Probable: Five of the six criteria above are met


Treatment

The severity of this disease frequently warrants hospitalization. Admission to the
intensive care unit 220px, Intensive care unit An intensive care unit (ICU), also known as an intensive therapy unit or intensive treatment unit (ITU) or critical care unit (CCU), is a special department of a hospital or health care facility that provides intensi ...
is often necessary for supportive care (for aggressive fluid management, ventilation, renal replacement therapy and
inotropic An inotrope is an agent that alters the force or energy of muscular contractions. Negatively inotropic agents weaken the force of muscular contractions. Positively inotropic agents increase the strength of muscular contraction. The term ''inotro ...
support), particularly in the case of multiple organ failure. Treatment includes removal or draining of the source of infection—often a tampon—and draining of abscesses. Outcomes are poorer in patients who do not have the source of infection removed. Antibiotic treatment should cover both ''S. pyogenes'' and ''S. aureus''. This may include a combination of
cephalosporin The cephalosporins (sg. ) are a class of β-lactam antibiotics originally derived from the fungus ''Acremonium'', which was previously known as ''Cephalosporium''. Together with cephamycins, they constitute a subgroup of β-lactam antibiotics ...
s,
penicillin Penicillins (P, PCN or PEN) are a group of β-lactam antibiotics originally obtained from '' Penicillium'' moulds, principally '' P. chrysogenum'' and '' P. rubens''. Most penicillins in clinical use are synthesised by P. chrysogenum usin ...
s or
vancomycin Vancomycin is a glycopeptide antibiotic medication used to treat a number of bacterial infections. It is recommended intravenously as a treatment for complicated skin infections, bloodstream infections, endocarditis, bone and joint infection ...
. The addition of
clindamycin Clindamycin is an antibiotic medication used for the treatment of a number of bacterial infections, including osteomyelitis (bone) or joint infections, pelvic inflammatory disease, strep throat, pneumonia, acute otitis media (middle ear in ...
or
gentamicin Gentamicin is an antibiotic used to treat several types of bacterial infections. This may include bone infections, endocarditis, pelvic inflammatory disease, meningitis, pneumonia, urinary tract infections, and sepsis among others. It is not ...
reduces toxin production and mortality. In some cases doctors will prescribe other treatments such as blood pressure medications (to stabilize blood pressure if it is too low), dialysis, oxygen mask (to stabilize oxygen levels), and sometimes a ventilator. These will sometimes be used to help treat side affects of contracting TSS.


Prognosis

With proper treatment, people usually recover in two to three weeks. The condition can, however, be fatal within hours. TSS has a mortality rate of 30%–70%. Children who are affected by TSS tend to recover easier than adults do.


Complications

* Amputation of fingers, toes, and sometimes limbs * Death * Liver or Kidney failure * Heart problems * Respiratory distress * Septic shock * Other abnormalities may occur depending on the case


Prevention

During menstruation: * Use pads at night instead of tampons * Try to keep up with changing a tampon every 4 to 8 hours * Use low absorbent tampons * Follow directions when using vaginal contraceptives (sponges or diaphragms) * Make sure to maintain good hygiene during a menstrual cycle For anyone: * Keep open wounds clean * Watch wounds and cuts for signs of infection (e.g. pus, redness, and warm to touch) * Keep personal items personal (e.g.towels, sheets, razors) * Wash clothing and bedding in hot water


Epidemiology

Staphylococcal toxic shock syndrome is rare and the number of reported cases has declined significantly since the 1980s. Patrick Schlievert, who published a study on it in 2004, determined incidence at three to four out of 100,000 tampon users per year; the information supplied by manufacturers of sanitary products such as Tampax and Stayfree puts it at one to 17 of every 100,000 menstruating females per year. first published as "Lingering Risk", ''
San Jose Mercury News ''The Mercury News'' (formerly ''San Jose Mercury News'', often locally known as ''The Merc'') is a morning daily newspaper published in San Jose, California, in the San Francisco Bay Area. It is published by the Bay Area News Group, a subsidia ...
'', 13 December 2004
TSS was considered a sporadic disease that occurred in immunocompromised people. It was not a more well-known disease until the 1980s, when high-absorbency tampons were in use by menstruating women. Due to the idea of the tampons having a high absorbency this led women to believe that they could leave a tampon in for several hours. Doing this allowed the bacteria to grow and infect women. This resulted in a spike of cases of TSS. Philip M. Tierno Jr. helped determine that tampons were behind TSS cases in the early 1980s. Tierno blames the introduction of higher-absorbency tampons in 1978. A study by Tierno also determined that all-cotton tampons were less likely to produce the conditions in which TSS can grow; this was done using a direct comparison of 20 brands of tampons including conventional cotton/rayon tampons and 100% organic cotton tampons from Natracare. In fact, Dr Tierno goes as far to state, "The bottom line is that you can get TSS with synthetic tampons, but not with an all-cotton tampon." A rise in reported cases occurred in the early 2000s: eight deaths from the syndrome in California in 2002 after three successive years of four deaths per year, and Schlievert's study found cases in part of Minnesota more than tripled from 2000 to 2003. Schlievert considers earlier onset of menstruation to be a cause of the rise; others, such as Philip M. Tierno and Bruce A. Hanna, blame new high-absorbency tampons introduced in 1999 and manufacturers discontinuing warnings not to leave tampons in overnight. TSS is more common during the winter and spring and occurs most often in the young and old. Toxic shock syndrome is commonly known to be an issue for females who menstruate. TSS although can occur in people of all ages and genders. Around fifty percent of Toxic Shock Syndrome cases are in non menstruating cases. TSS in these cases can be caused by skin wounds, surgical sites, nasal packing, and burns.


History


Initial description

The term "toxic shock syndrome" was first used in 1978 by a Denver pediatrician, James K. Todd, to describe the staphylococcal illness in three boys and four girls aged 8–17 years. Even though ''S. aureus'' was isolated from mucosal sites in the patients, bacteria could not be isolated from the blood, cerebrospinal fluid, or urine, raising suspicion that a toxin was involved. The authors of the study noted reports of similar staphylococcal illnesses had appeared occasionally as far back as 1927, but the authors at the time failed to consider the possibility of a connection between toxic shock syndrome and tampon use, as three of the girls who were menstruating when the illness developed were using tampons. Many cases of TSS occurred after tampons were left in after they should have been removed.


Rely tampons

Following controversial test marketing in
Rochester, New York Rochester () is a City (New York), city in the U.S. state of New York (state), New York, the county seat, seat of Monroe County, New York, Monroe County, and the fourth-most populous in the state after New York City, Buffalo, New York, Buffalo, ...
, and
Fort Wayne, Indiana Fort Wayne is a city in and the county seat of Allen County, Indiana, United States. Located in northeastern Indiana, the city is west of the Ohio border and south of the Michigan border. The city's population was 263,886 as of the 2020 Censu ...
, in August 1978,
Procter and Gamble The Procter & Gamble Company (P&G) is an American multinational consumer goods corporation headquartered in Cincinnati, Ohio, founded in 1837 by William Procter and James Gamble. It specializes in a wide range of personal health/consumer hea ...
introduced superabsorbent Rely tampons to the
United States The United States of America (U.S.A. or USA), commonly known as the United States (U.S. or US) or America, is a country Continental United States, primarily located in North America. It consists of 50 U.S. state, states, a Washington, D.C., ...
market in response to women's demands for tampons that could contain an entire menstrual flow without leaking or replacement. Rely used carboxymethylcellulose (CMC) and compressed beads of
polyester Polyester is a category of polymers that contain the ester functional group in every repeat unit of their main chain. As a specific material, it most commonly refers to a type called polyethylene terephthalate (PET). Polyesters include natura ...
for absorption. This tampon design could absorb nearly 20 times its own weight in fluid. Further, the tampon would "blossom" into a cup shape in the vagina to hold menstrual fluids without leakage. In January 1980, epidemiologists in
Wisconsin Wisconsin () is a state in the upper Midwestern United States. Wisconsin is the 25th-largest state by total area and the 20th-most populous. It is bordered by Minnesota to the west, Iowa to the southwest, Illinois to the south, Lake M ...
and
Minnesota Minnesota () is a state in the upper midwestern region of the United States. It is the 12th largest U.S. state in area and the 22nd most populous, with over 5.75 million residents. Minnesota is home to western prairies, now given over t ...
reported the appearance of TSS, mostly in those menstruating, to the
CDC The Centers for Disease Control and Prevention (CDC) is the national public health agency of the United States. It is a United States federal agency, under the Department of Health and Human Services, and is headquartered in Atlanta, Georg ...
. ''S. aureus'' was successfully cultured from most of the subjects. The Toxic Shock Syndrome Task Force was created and investigated the epidemic as the number of reported cases rose throughout the summer of 1980. In September 1980, CDC reported users of Rely were at increased risk for developing TSS. On 22 September 1980, Procter and Gamble recalled Rely following release of the CDC report. As part of the voluntary recall, Procter and Gamble entered into a consent agreement with the FDA "providing for a program for notification to consumers and retrieval of the product from the market". However, it was clear to other investigators that Rely was not the only culprit. Other regions of the United States saw increases in menstrual TSS before Rely was introduced. It was shown later that higher absorbency of tampons was associated with an increased risk for TSS, regardless of the chemical composition or the brand of the tampon. The sole exception was Rely, for which the risk for TSS was still higher when corrected for its absorbency. The ability of carboxymethylcellulose to filter the ''S. aureus'' toxin that causes TSS may account for the increased risk associated with Rely.


Notable cases

* Clive Barker, fully recovered, contracted the syndrome after visiting the dentist. * Lana Coc-Kroft, fully recovered, contracted the syndrome due to group A streptococcal infection. *
Jim Henson James Maury Henson (September 24, 1936 – May 16, 1990) was an American puppeteer, animator, cartoonist, actor, inventor, and filmmaker who achieved worldwide notice as the creator of The Muppets and '' Fraggle Rock'' (1983–1987) and ...
, d. 1990, contracted the syndrome due to group A streptococcal infection and subsequently died from it. * Nan C. Robertson, d. 2009, the 1983 winner of the
Pulitzer Prize for Feature Writing The Pulitzer Prize for Feature Writing is one of the fourteen American Pulitzer Prizes that are annually awarded for Journalism. It has been awarded since 1979 for a distinguished example of feature writing giving prime consideration to high lite ...
for her medically detailed account of her struggle with toxic shock syndrome, a cover story for ''The New York Times Magazine'' which at that time became the most widely syndicated article in ''Times'' history. * Barbara Robison, lead vocalist for the psychedelic rock band the Peanut Butter Conspiracy, was performing in
Butte, Montana Butte ( ) is a consolidated city-county and the county seat of Silver Bow County, Montana, United States. In 1977, the city and county governments consolidated to form the sole entity of Butte-Silver Bow. The city covers , and, according to t ...
on April 6, 1988; during the concert, she fell ill and was transported to a hospital. She did not recover, and died sixteen days later on April 22 from toxic shock poisoning at the age of 42. * Mike Von Erich, d. 1987, developed the syndrome after shoulder surgery: he made an apparent recovery but experienced brain damage and weight loss as a result of the condition; he died by suicide later.


References


External links

* * {{DEFAULTSORT:Toxic Shock Syndrome Bacterial diseases Bacterial toxins Bacterium-related cutaneous conditions Syndromes Wikipedia emergency medicine articles ready to translate Wikipedia medicine articles ready to translate