Primary congenital alactasia
Primary congenital alactasia, also called congenital lactase deficiency, is an extremely rare, autosomal recessive enzyme defect that prevents lactase expression from birth. People with congenital lactase deficiency cannot digest lactose from birth, so cannot digest breast milk. This genetic defect is characterized by a complete lack of lactase (alactasia). About 40 cases have been reported worldwide, mainly limited to Finland. Before the 20th century, babies born with congenital lactase deficiency often did not survive, but death rates decreased with soybean-derived infant formulas and manufactured lactose-free dairy products. Diagnosis
In order to assess lactose intolerance, intestinal function is challenged by ingesting more dairy products than can be readily digested. Clinical symptoms typically appear within 30 minutes, but may take up to two hours, depending on other foods and activities. Substantial variability in response (symptoms of nausea, cramping, bloating, diarrhea, and flatulence) is to be expected, as the extent and severity of lactose intolerance varies among individuals.The next step is to determine whether it is due to primary lactase deficiency or an underlying disease that causes secondary lactase deficiency. Physicians should investigate the presence of undiagnosed coeliac disease, Crohn disease, or other enteropathies when secondary lactase deficiency is suspected and infectious gastroenteritis has been ruled out.Lactose intolerance is distinct from milk allergy, an immune response to cows milk proteins. They may be distinguished in diagnosis by giving lactose-free milk, producing no symptoms in the case of lactose intolerance, but the same reaction as to normal milk in the presence of a milk allergy.
Serovars C and D are occupational hazards for slaughterhouse workers and for people in contact with birds. Serovar E isolates (known as Cal-10, MP, or MN) have been obtained from a variety of avian hosts worldwide, and although they were associated with the 1920s–1930s outbreak in humans, a specific reservoir for serovar E has not been identified. The M56 and WC serovars were isolated during outbreaks in mammals. Treatment
Treatment is usually with antibiotics, such as doxycycline or tetracycline, and can be administered through drops in the water or injections. Many strains of C. psittaci are susceptible to bacteriophages. Use as a biological weapon
Psittacosis was one of more than a dozen agents that the United States researched as potential biological weapons before the nation suspended its biological weapons program. Notable casualties
In 1930, during the 1929–1930 psittacosis pandemic, Lena Rose Pepperdine died of parrot fever. She was the first wife of George Pepperdine, the founder of Pepperdine University. References
The initial content for this article was adapted from sources available at https://www.cdc.gov. External links
Psittacosis on Birds n Ways
Ornithosis in Pigeons - Pigeonpedia