Hereditary coproporphyria (HCP) is a disorder of heme biosynthesis, classified as an acute hepatic porphyria. HCP is caused by a deficiency of the enzyme coproporphyrinogen oxidase, coded for by the CPOX gene, and is inherited in an autosomal dominant fashion, although homozygous individuals have been identified. Unlike acute intermittent porphyria, individuals with HCP can present with cutaneous findings similar to those found in porphyria cutanea tarda in addition to the acute attacks of abdominal pain, vomiting and neurological dysfunction characteristic of acute porphyrias. Like other porphyrias, attacks of HCP can be induced by certain drugs, environmental stressors or diet changes. Biochemical and molecular testing can be used to narrow down the diagnosis of a porphyria and identify the specific genetic defect. Overall, porphyrias are rare diseases. The combined incidence for all forms of the disease has been estimated at 1:20,000. The exact incidence of HCP is difficult to determine, due to its reduced penetrance. Signs and symptoms
Clinically, patients affected with HCP present similarly to those with other acute porphyrias, such as acute intermittent porphyria (AIP) and variegate porphyria (VP). Patients with HCP and VP can present with symptoms shared between the acute and cutaneous porphyrias. This includes the acute attacks of abdominal pain, nausea, vomiting, diarrhea, tachycardia, hypertension and seizures, as well as the cutaneous findings seen in porphyria cutanea tarda (PCT), namely increased skin fragility, bullous lesions after exposure to sunlight and increased scarring.Individuals with HCP may be asymptomatic in the absence of triggering factors.
Side-effects caused by dental crossbite can be increased recession on the buccal of lower incisors and higher chance of inflammation in the same area. Another term for an anterior crossbite due to dental interferences is Pseudo Class III Crossbite or Malocclusion. Single tooth crossbite
Single tooth crossbites can occur due to uneruption of a primary teeth in a timely manner which causes permanent tooth to erupt in a different eruption pattern which is lingual to the primary tooth. Single tooth crossbites are often fixed by using a finger-spring based appliances. This type of spring can be attached to a removable appliance which is used by patient every day to correct the tooth position. Skeletal crossbite
An anterior crossbite due to skeletal reasons will involve a deficient maxilla and a more hyperplastic or overgrown mandible. People with this type of crossbite will have dental compensation which involves proclined maxillary incisors and retroclined mandibular incisors. A proper diagnosis can be made by having a person bite into their centric relation will show mandibular incisors ahead of the maxillary incisors, which will show the skeletal discrepancy between the two jaws. Posterior crossbite
Bjork defined posterior crossbite as a malocclusion where the buccal cusps of canine, premolar and molar of upper teeth occlude lingually to the buccal cusps of canine, premolar and molar of lower teeth. Posterior crossbite is often correlated to a narrow maxilla and upper dental arch. A posterior crossbite can be unilateral, bilateral, single-tooth or entire segment crossbite. Posterior crossbite has been reported to occur between 7–23% of the population.