Cystic fibrosis (CF) is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.
Caroli disease is a rare inherited disorder characterized by cystic dilatation of the bile ducts within the liver. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally widened bile ducts affecting an isolated portion of liver. The second form is more diffuse, and when associated with portal hypertension and congenital hepatic fibrosis, is often referred to as "Caroli syndrome". The underlying differences between the two types are not well understood. Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about one in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease.
Colistin, also known as polymyxin E, is an antibiotic medication used as a last-resort treatment for multidrug-resistant Gram-negative infections including pneumonia. These may involve bacteria such as Pseudomonas aeruginosa, Klebsiella pneumoniae, or Acinetobacter. It comes in two forms: colistimethate sodium can be injected into a vein, injected into a muscle, or inhaled, and colistin sulfate is mainly applied to the skin or taken by mouth. Colistimethate sodium is a prodrug; it is produced by the reaction of colistin with formaldehyde and sodium bisulfite, which leads to the addition of a sulfomethyl group to the primary amines of colistin. Colistimethate sodium is less toxic than colistin when administered parenterally. In aqueous solutions it undergoes hydrolysis to form a complex mixture of partially sulfomethylated derivatives, as well as colistin. Resistance to colistin began to appear as of 2015.
Burkholderia is a genus of Pseudomonadota whose pathogenic members include the Burkholderia cepacia complex, which attacks humans and Burkholderia mallei, responsible for glanders, a disease that occurs mostly in horses and related animals; Burkholderia pseudomallei, causative agent of melioidosis; and Burkholderia cepacia, an important pathogen of pulmonary infections in people with cystic fibrosis (CF). Burkholderia species is also found in marine environments. S.I. Paul et al. (2021) isolated and characterized Burkholderia cepacia from marine sponges of the Saint Martin's Island of the Bay of Bengal, Bangladesh.
Burkholderia cepacia complex (BCC), or simply Burkholderia cepacia, is a group of catalase-producing, lactose-nonfermenting, Gram-negative bacteria composed of at least 20 different species, including B. cepacia, B. multivorans, B. cenocepacia, B. vietnamiensis, B. stabilis, B. ambifaria, B. dolosa, B. anthina, B. pyrrocinia and B. ubonensis. B. cepacia is an opportunistic human pathogen that most often causes pneumonia in immunocompromised individuals with underlying lung disease. Patients with sickle-cell haemoglobinopathies are also at risk. The species complex also attacks young onion and tobacco plants, and displays a remarkable ability to digest oil. Burkholderia cepacia is also found in marine environments and some strains of Burkholderia cepacia can tolerate high salinity. S.I. Paul et al. (2021) isolated and biochemically characterized salt tolerant strains of Burkholderia cepacia from marine sponges of Saint Martin's Island of the Bay of Bengal, Bangladesh.
Stenotrophomonas maltophilia is an aerobic, nonfermentative, Gram-negative bacterium. It is an uncommon bacterium and human infection is difficult to treat. Initially classified as Bacterium bookeri, then renamed Pseudomonas maltophilia, S. maltophilia was also grouped in the genus Xanthomonas before eventually becoming the type species of the genus Stenotrophomonas in 1993.
Elizabethkingia meningoseptica is a Gram-negative, rod-shaped bacterium widely distributed in nature. It may be normally present in fish and frogs; it may be isolated from chronic infectious states, as in the sputum of cystic fibrosis patients. In 1959, American bacteriologist Elizabeth O. King was studying unclassified bacteria associated with pediatric meningitis at the Centers for Disease Control and Prevention in Atlanta, when she isolated an organism that she named Flavobacterium meningosepticum. In 1994, it was reclassified in the genus Chryseobacterium and renamed Chryseobacterium meningosepticum(chryseos = "golden" in Greek, so Chryseobacterium means a golden/yellow rod similar to Flavobacterium). In 2005, a 16S rRNA phylogenetic tree of Chryseobacteria showed that C. meningosepticum along with C. miricola were close to each other but outside the tree of the rest of the Chryseobacteria and were then placed in a new genus Elizabethkingia named after the original discoverer of F. meningosepticum.
Burkholderia cenocepacia is a Gram-negative, rod-shaped bacterium that is commonly found in soil and water environments and may also be associated with plants and animals, particularly as a human pathogen. It is one of over 20 species in the Burkholderia cepacia complex (Bcc) and is notable due to its virulence factors and inherent antibiotic resistance that render it a prominent opportunistic pathogen responsible for life-threatening, nosocomial infections in immunocompromised patients, such as those with cystic fibrosis or chronic granulomatous disease. The quorum sensing systems CepIR and CciIR regulate the formation of biofilms and the expression of virulence factors such as siderophores and proteases. Burkholderia cenocepacia may also cause disease in plants, such as in onions and bananas. Additionally, some strains serve as plant growth-promoting rhizobacteria.
Burkholderia gladioli is a species of aerobic gram-negative rod-shaped bacteria that causes disease in both humans and plants. It can also live in symbiosis with plants and fungi and is found in soil, water, the rhizosphere, and in many animals. It was formerly known as Pseudomonas marginata.
Burkholderia pyrrocinia is a Gram-negative bacterium which has been found in soil as well as in the sputum of patients with cystic fibrosis.
Burkholderia multivorans is a species in the phylum Pseudomonadota. The cells are rod-shaped. It is known to cause human disease, such as colonisation of the lung in cystic fibrosis.
Burkholderia vietnamiensis is a species of bacteria. It can be found as an opportunistic pathogen in patients with cystic fibrosis or other immunocompromising illnesses. It differs from most Burkholderia species in that it is often susceptible to aminoglycosides, but not polymyxin B. Many isolates have been found to be catalase positive. B. vietnamiensis is able to fix N
2.
Burkholderia lata is a bacterium from the genus of Burkholderia and the family of Burkholderiaceae which belongs to the Burkholderia cepacia complex.
Pandoraea pulmonicola is a Gram-negative, non-spore-forming, motile bacterium with a single polar flagellum, of the genus Pandoraea. P. pulmonicola has been isolated from respiratory samples of patients with cystic fibrosis and other respiratory diseases. P. pulmonicola is a part of the Burkholderia cepacia complex, which is a group of bacteria commonly associated with infections in individuals with compromised immune systems.
Inquilinus limosus is a bacterium first isolated from cystic fibrosis patients' lungs, and is rarely observed elsewhere, prompting extensive research into its biology.
Burkholderia pseudomultivorans is a bacterium from the genus Burkholderia and the family Burkholderiaceae which was isolated from samples of the human respiratory tract. Burkholderia pseudomultivorans belongs to the Burkholderia cepacia complex.
Claire E. Wainwright is a paediatric respiratory physician and professor of pediatrics, residing and working in Queensland. She commenced her medical training in London and completed her specialist training at the Royal Children's Hospital, Brisbane. She is now head of the Cystic Fibrosis Service at the Queensland Children's Hospital and a professor of pediatric medicine at the University of Queensland, Australia. Wainwright has published numerous academic papers focusing upon her main area of interest; the impacts of fungal infections upon children with cystic fibrosis. However, her interests also expand to include other airway complications within children.
Globicatella sanguinis is a bacterium from the family of Globicatella. Globicatella sanguinis can cause in rare cases acute meningitis and urosepsis.
Ornibactin is a siderophore, or small iron-binding compound secreted by bacteria to transport iron into the cell. Ornibactin is produced by Burkholderia cenocepacia under iron-deficient conditions. B. cenocepacia is known to opportunistically infect humans, specifically ones suffering from cystic fibrosis.
Cedecea davisae is a gram-negative, motile, rod-shaped, non-sporulating, lipase-positive bacteria.
