What Is Lysinuric Protein Intolerance?
Lysinuric protein intolerance (LPI) is a genetically inherited condition in which certain amino acids (namely ornithine, lysine, and arginine) are not adequately broken down in affected individuals. Due to their inability to break down these amino acids, these individuals are prone to experiencing nausea and vomiting soon after they eat protein-rich foods.
Even more problematic are the other symptoms that are frequently seen in those who are inflicted with LPI. These symptoms stem directly from the inability to digest protein and include:
- Shortness in height
Brittle and weak bones that can easily fracture (osteoporosis)
- Immune system problems
- Enlarged organs, particularly the spleen and liver
Another severely problematic symptom of LPI is pulmonary alveolar proteinosis. Pulmonary alveolar proteinosis is a condition in which excess protein concentrates in the lungs. Due to this excess build up, normal functions of the lungs are affected. In some cases, pulmonary alveolar proteinosis is life-threatening.
Excess protein also can build up in the kidneys of LPI sufferers and this often leads to a serious condition that is referred to as end-stage renal disease. In this condition, the kidneys become blocked and cannot adequately do one their most important jobs, which is to remove fluid and waste materials from the body.
Yet another harmful condition that can arise in individuals having LPI is an excess build up of ammonia in the blood. In the worst cases, this excess ammonia leads to severe cognitive problems and even coma.
Although it does not discriminate against any particular group, LPI is more common in the Japanese and Finnish populations than in other populations. Symptoms generally arise shortly after birth and, specifically, after affected infants begin eating solid foods that are rich in protein.