Angled arc shadow

Introduction Angular arc shadow is one of the symptoms of nail-sacral syndrome damage to the nail. Nail-patella syndrome or hereditary bone-to-finger malnutrition is a hereditary disease characterized by dysplasia or loss of the tibia and nail nutrition. Obstruction, elbow dysplasia, sacral horn and renal failure. The syndrome is autosomal dominant, and the locus is linked to the adenylate cyclase and ABO blood group on chromosome 9.

Cause

The syndrome is autosomal dominant, and the locus is linked to the adenylate cyclase and ABO blood group on chromosome 9.

an examination

Related inspection

CT examination blood test

The main diagnosis of this disease is family history. The typical clinical manifestations are X-ray signs of bone and proteinuria. Renal biopsy is performed as necessary.

Clinically more common in adolescents, the main manifestations of kidney damage are proteinuria, microscopic hematuria, edema and hypertension, occasionally nephrotic syndrome, the course of the disease is relatively benign, only 10% of patients into the kidney failure. Extrarenal manifestations include nail dystrophies, absent bones on one or both sides, elbow deformities, angular pelvis, and other skeletal abnormalities. Most of the disease is caused by difficulty in walking due to lack of humerus. It can be diagnosed according to typical bone changes, and kidney damage can be diagnosed. Radiological examination showed that the humeral angle was a characteristic change and had a clear diagnostic significance.

It has been reported that a small number of patients have ultrastructural changes in the glomerular basement membrane without bones, skin, nails, and other typical manifestations of this syndrome. These patients are considered to be the frustration or single nephrotic variant of the syndrome. . However, the electron micrographs published by these institutes do not strongly support this view. Judging the renal biopsy specimens can not only use the glomerular basement membrane moth, and the fibrils must be identified by phosphotungstic acid staining. Because of its higher sensitivity, it is more valuable for diagnosis.

Differential diagnosis

Concave: The nail is concave, which means that there are some horizontal stripes, vertical stripes and spots on the nails.

Convex armor: The center of the nail surface is obviously raised above the circumference, and the end of the nail is drooping. Like a shell or an inverted spoon, there is a slight concave point on the surface of the light. The color of the nail is slightly white, and the color of the nail is white. Partial powder.

There is a straight line on the nail: a straight line on the nail means that the fingernail has a vertical line.

A malnutrition: A has a lot of mediastinum is the characteristics of this disease, no change under the armor and nail week. More common in children from 18 months to 18 years old, the adult subsided, so it began to be called child 20 malnutrition. However, this disease also occurs in young people and adults, so it is now called malnutrition.

Finger (toe) nail brittle soft atrophy: finger (toe) nail soft atrophy is a clinical manifestation of pseudohypoparathyroidism. Patients with pseudohypoparathyroidism often find rough skin, hyperpigmentation, hair loss, fingernails, soft and atrophy, and even shedding; cataracts can occur in the intraocular lens.

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