Ears, nose, cartilage turn blue
Introduction
Introduction The main symptoms of patients with brown-yellowing arthritis are brownish yellow in the skin, sclera, and corneal pigmentation. The ear, nose, and cartilage can turn blue, the tympanic membrane is grayish black, and hearing is often diminished. Due to the lack of homogentisate oxidase, the intermediate metabolite of phenylalanine and tyrosine (uric acid) can not be further oxidatively decomposed and accumulated in the body. Darkens the skin, sclera, and cartilage, while uric acid causes pigmentation of cartilage and other connective tissues, and degenerative arthritis of the spine and peripheral joints. On the other hand, urinary acid is excreted in the urine, and is alkalized and oxidized in the urine to darken the urine, so it is also called black uric acid (Alkaptonuria). The disease is a rare hereditary disease that is rare.
Cause
Cause
Brownish yellow disease is a rare genetic disorder of amino acid metabolism disorder. Usually the disease can have a family history, the incidence rate of men and women is different, about 2:1. The brownish-yellow arthritis is caused by pigmentation in the intervertebral disc or cartilage, resulting in degeneration of the intervertebral disc and joint lesions, that is, brown-yellow-like pigmentation. Deposition in the joint structure of the tissue causes brownish-induced arthritis.
Examine
an examination
Related inspection
Otolaryngology CT examination of ear, nose and throat swab bacterial culture
Laboratory inspection:
Uroic acid test: After the urine is left standing or after alkalization, it becomes black, and the spotting reagent turns brown.
Other auxiliary inspections:
X-ray inspection
(1) Spinal changes: the thoracic vertebrae are prominently protruding and the lumbar vertebrae are excessively curved. The spine is osteoporosis, and bone spurs are formed at the edge of the late vertebral body. The intervertebral disc has extensive degeneration, and there is layered calcification. There is a special double-transverse calcification with increased density of flat thin ellipse, which is parallel to each other and has a translucent layer in the middle. Intervertebral discs are widely affected, especially at the lumbar vertebrae. The intervertebral space is significantly narrower.
(2) Degenerative changes can occur in the shoulder and knee joints. The tendon ligaments may have calcification, the ankle joints become narrow, and the lower joints are hardened. There is also calcification in the cartilage of the pubic symphysis, and bone erosion under the cartilage. Calcification can also occur with tendons attached to the femoral head, acetabulum, large trochanter, small trochanter, ischial tuberosity, and soft tissue around the joint.
2. Special examination of uric acid
The uric acid can be measured by a spectrophotometer, and the content of urinary acid in blood and urine can also be determined by a special enzyme.
Diagnosis
Differential diagnosis
Because the peripheral joint disease of this disease occurs later in brown yellow disease, involving large joints, and there is no difference in radiological signs and osteoarthritis, so clinical attention should be paid to rheumatoid arthritis and ankylosing spine. Identification of inflammation and gout. For some patients with disc calcification of the disc or calcification of the heart valve, it is also necessary to identify the corresponding disease, and the positive urinary acid positive support the diagnosis of brown huang disease.
