Carrion's Disease
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Carrion's disease is an
infectious disease An infection is the invasion of tissues by pathogens, their multiplication, and the reaction of host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmissible disease or communicable dise ...
produced by ''
Bartonella bacilliformis ''Bartonella bacilliformis'' is a bacterium, Gram negative aerobic, pleomorphic, flagellated, motile, coccobacillary, 2–3 μm long, 0.2–0.5 μm wide, and a facultative intracellular bacterium. History The bacterium was discovered by Peru ...
'' infection. It is named after
Daniel Alcides Carrión Daniel Alcides Carrión García (August 12, 1857 – October 5, 1885) was a Peruvian medical student after whom Carrion's disease is named. Fatal experiment Carrión described the disease in the course of what proved to be a fatal experiment u ...
.


Signs and symptoms

The clinical symptoms of bartonellosis are pleomorphic and some patients from endemic areas may be asymptomatic. The two classical clinical presentations are the acute phase and the chronic phase, corresponding to the two different host cell types invaded by the bacterium (red blood cells and endothelial cells). An individual can be affected by either or both phases.


Acute phase

It is also called the hematic phase. The most common findings are fever (usually sustained, but with temperature no greater than 102 °F (39 °C)), pale appearance, malaise, painless liver enlargement,
jaundice Jaundice, also known as icterus, is a yellowish or greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving abnormal heme meta ...
,
enlarged lymph nodes Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In cli ...
, and
enlarged spleen Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulating ...
. This phase is characterized by severe
hemolytic anemia Hemolytic anemia or haemolytic anaemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels (intravascular hemolysis) or elsewhere in the human body (extravascular). This most commonly ...
and transient immunosuppression. The case fatality ratios of untreated patients exceeded 40% but reach around 90% when
opportunistic infection An opportunistic infection is an infection caused by pathogens (bacteria, fungi, parasites or viruses) that take advantage of an opportunity not normally available. These opportunities can stem from a variety of sources, such as a weakened immune ...
with ''Salmonella'' spp. occurs. In a recent study, the attack rate was 13.8% (123 cases) and the case-fatality rate was 0.7%. Other symptoms include a
headache Headache is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches. Headaches can occur as a result ...
, muscle aches, and general abdominal pain. Some studies have suggested a link between Carrion's disease and heart murmurs due to the disease's impact on the circulatory system. In children, symptoms of anorexia, nausea, and vomiting have been investigated as possible symptoms of the disease. Most of the mortality of Carrion's disease occurs during the acute phase. Studies vary in their estimates of mortality. In one study, mortality has been estimated as low as just 1% in studies of hospitalized patients, to as high as 88% in untreated, unhospitalized patients. In developed countries, where the disease rarely occurs, it is recommended to seek the advice of a specialist in infectious disease when diagnosed. Mortality is often thought to be due to subsequent infections due to the weakened immune system and opportunistic pathogen invasion, or consequences of malnutrition due to weight loss in children. In a study focusing on pediatric and gestational effects of the disease, mortality rates for pregnant women with the acute phase were estimated at 40% and rates of spontaneous abortion in another 40%.


Chronic phase

It is also called the eruptive phase or tissue phase, in which the patients develop a cutaneous rash produced by a proliferation of endothelial cells and is known as "Peruvian warts" or "''verruga peruana''". Depending on the size and characteristics of the lesions, there are three types: miliary (1–4 mm), nodular or subdermic, and mular (>5mm). Miliary lesions are the most common. The lesions often ulcerate and bleed. The most common findings are bleeding of verrugas, fever, malaise, arthralgias (joint pain), anorexia, myalgias, pallor, lymphadenopathy, and liver and spleen enlargement. On microscopic examination, the chronic phase and its rash are produced by angioblastic hyperplasia, or the increased rates and volume of cell growth in the tissues that form blood vessels. This results in a loss of contact between cells and a loss of normal functioning. The chronic phase is the more common phase. Mortality during the chronic phase is very low.


Cause

Carrion's disease is caused by ''
Bartonella bacilliformis ''Bartonella bacilliformis'' is a bacterium, Gram negative aerobic, pleomorphic, flagellated, motile, coccobacillary, 2–3 μm long, 0.2–0.5 μm wide, and a facultative intracellular bacterium. History The bacterium was discovered by Peru ...
''. Recent investigations show that ''
Candidatus In prokaryote nomenclature, ''Candidatus'' (Latin for candidate of Roman office) is used to name prokaryotic phyla that are well characterized but yet-uncultured. Contemporary sequencing approaches, such as 16S sequencing or metagenomics, provide m ...
Bartonella ancashi'' may cause verruga peruana, although it may not meet all of
Koch's postulates Koch's postulates ( )"Koch"
''
Giemsa stain Giemsa stain (), named after German chemist and bacteriologist Gustav Giemsa, is a nucleic acid stain used in cytogenetics and for the histopathological diagnosis of malaria and other parasites. Uses It is specific for the phosphate groups of ...
, Columbia blood agar cultures,
immunoblot The western blot (sometimes called the protein immunoblot), or western blotting, is a widely used analytical technique in molecular biology and immunogenetics to detect specific proteins in a sample of tissue homogenate or extract. Besides detect ...
, indirect
immunofluorescence Immunofluorescence is a technique used for light microscopy with a fluorescence microscope and is used primarily on microbiological samples. This technique uses the specificity of antibodies to their antigen to target fluorescent dyes to specif ...
, and PCR. Diagnosis during the chronic phase can be made using a Warthin-Starry stain of wart biopsy, PCR, and immunoblot.


Treatment

Because Carrion's disease is often comorbid with ''
Salmonella ''Salmonella'' is a genus of rod-shaped (bacillus) Gram-negative bacteria of the family Enterobacteriaceae. The two species of ''Salmonella'' are ''Salmonella enterica'' and ''Salmonella bongori''. ''S. enterica'' is the type species and is fur ...
'' infections,
chloramphenicol Chloramphenicol is an antibiotic useful for the treatment of a number of bacterial infections. This includes use as an eye ointment to treat conjunctivitis. By mouth or by injection into a vein, it is used to treat meningitis, plague, cholera, a ...
has historically been the treatment of choice.
Fluoroquinolone A quinolone antibiotic is a member of a large group of broad-spectrum bacteriocidals that share a bicyclic core structure related to the substance 4-quinolone. They are used in human and veterinary medicine to treat bacterial infections, as wel ...
s (such as
ciprofloxacin Ciprofloxacin is a fluoroquinolone antibiotic used to treat a number of bacterial infections. This includes bone and joint infections, intra abdominal infections, certain types of infectious diarrhea, respiratory tract infections, skin infec ...
) or chloramphenicol in adults and chloramphenicol plus
beta-lactam A beta-lactam (β-lactam) ring is a four-membered lactam. A ''lactam'' is a cyclic amide, and ''beta''-lactams are named so because the nitrogen atom is attached to the β-carbon atom relative to the carbonyl. The simplest β-lactam possible is ...
s in children are the
antibiotic 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 prevention of ...
regimens of choice during the acute phase of Carrion's disease. Chloramphenicol-resistant ''B. bacilliformis'' has been observed. During the eruptive phase, in which chloramphenicol is not useful,
azithromycin Azithromycin, sold under the brand names Zithromax (in oral form) and Azasite (as an eye drop), is an antibiotic medication used for the treatment of a number of bacterial infections. This includes middle ear infections, strep throat, pneumon ...
,
erythromycin Erythromycin is an antibiotic used for the treatment of a number of bacterial infections. This includes respiratory tract infections, skin infections, chlamydia infections, pelvic inflammatory disease, and syphilis. It may also be used duri ...
, and ciprofloxacin have been used successfully for treatment.
Rifampin Rifampicin, also known as rifampin, is an ansamycin antibiotic used to treat several types of bacterial infections, including tuberculosis (TB), ''Mycobacterium avium'' complex, leprosy, and Legionnaires’ disease. It is almost always used tog ...
or
macrolide The Macrolides are a class of natural products that consist of a large macrocyclic lactone ring to which one or more deoxy sugars, usually cladinose and desosamine, may be attached. The lactone rings are usually 14-, 15-, or 16-membered. Macrol ...
s are also used to treat both adults and children. Because of the high rates of comorbid infections and conditions, multiple treatments are often required. These have included the use of corticosteroids for respiratory distress, red blood cell transfusions for
anemia Anemia or anaemia (British English) is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, or a reduction in the amount of hemoglobin. When anemia comes on slowly, th ...
, pericardiectomies for pericardial tamponades, and other standard treatments.


Pop Culture

The disease was featured in an episode of
The WB The WB 100+ Station Group (originally called The WeB from its developmental stages until March 1999) was a national programming service of The WB—owned by the Warner Bros. Entertainment division of Time Warner, the Tribune Company, and group fo ...
supernatural drama
Charmed ''Charmed'' is an American fantasy drama television series created by Constance M. Burge and produced by Aaron Spelling and his production company Spelling Television, with Brad Kern serving as showrunner. The series was originally broadcast ...
that aired on February 3rd, 2000. In the episode
Piper Halliwell Piper Halliwell is a fictional character from the American television series ''Charmed'', played by Holly Marie Combs from October 7, 1998 until May 21, 2006. The character was created by Constance M. Burge, who based Piper on her second-oldest s ...
becomes infected with the condition after importing a crate of
Kiwano ''Cucumis metuliferus'', commonly called the African horned cucumber, horned melon, spiked melon, jelly melon, kiwano, or cuke-a-saurus is an annual vine in the cucumber and melon family, ''Cucurbitaceae''. Its fruit has horn (anatomy), horn-li ...
for her club, P3. She is bitten by a
Sandfly Sandfly (or sand fly) is a colloquial name for any species or genus of flying, biting, blood-sucking dipteran (fly) encountered in sandy areas. In the United States, ''sandfly'' may refer to certain horse flies that are also known as "greenhea ...
that was alive in the crate infecting her. Piper slowly begins to die of the condition as her sisters Prue and Phoebe rush to find a magical way to save her.


References

{{Bacterial cutaneous infections Bacterial diseases Bacterium-related cutaneous conditions Insect-borne diseases Tropical diseases