Calyces become blunted

Introduction In the renal sinus, 7 to 8 funnel-shaped renal sputum, renal pelvis wrapped around the kidney nipple. 2 to 3 kidney sputum synthesizes a kidney sputum. Two to three renal pelvis merge to form a flattened, funnel-shaped renal pelvis. The dullness of the renal pelvis refers to the flattening, funnel-shaped shape of the renal pelvis due to certain physical conditions. The dullness of the renal pelvis is one of the clinical manifestations of nail-sacral syndrome, and it is also one of the complications of nail-sacral syndrome. Nail-patella syndrome or hereditary bone-onyx dysplasia (hereditary osteo-onychodysplasia), a hereditary disease characterized by dysplasia or loss of the tibia A nutritional disorder, elbow dysplasia, sacral horn and renal failure. In 1897, Little described the disease for the first time. In the mid-1960s, Muth and Silverman described the lesions of the glomerular structure of the syndrome. In the late 1960s and early 1970s, Hoyer and Bennett began a comprehensive study of the ultrastructure and renal pathological features of the glomerular basement membrane of this syndrome. A modern theory of the structural basis of nail-sacral syndrome renal lesions was proposed and suggested that the syndrome may be due to biochemical defects in basement membrane collagen.

Cause

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

Pathogenesis:

Little is known about the pathogenesis of this syndrome. Some people think it is a collagen disease, which is abnormal during the synthesis, assembly or degradation of collagen. The cytological mechanisms of this disease have not been studied. The lack of non-glomerular basement membrane damage in pathological changes suggests that the various damages in this syndrome may be due to different mechanisms, and not all lesions are associated with basement membrane abnormalities. A small number of patients developed anti-glomerular basement membrane nephritis, supporting the hypothesis that glomerular basement membrane components are abnormal. Using a monoclonal antibody against the Goodpasture epitope, it was found that the glomerular basement membrane of 2/3 patients with renal biopsy specimens did not bind to the monoclonal antibody, suggesting a basement membrane for this syndrome. There is some degree of heterogeneity in the components, which also suggests the presence of Goodpsture antigen deletion or alteration. It is worth noting that it is not clear whether this is a primary or secondary change in this syndrome.

an examination

Related inspection

Renal angiography kidney MRI

The main diagnosis of nail-sacral syndrome 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 and hypertension, occasionally nephrotic syndrome, the course of disease is relatively benign, only 10% of patients enter the kidney failure late. Extrarenal manifestations include nail dystrophies, absent bones on one or both sides, elbow deformities, angular pelvis, and other skeletal abnormalities. Nail-sacral syndrome is mostly caused by difficulty in walking due to lack of humerus. It can be diagnosed according to typical skeletal changes, and can be diagnosed with kidney damage. 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 phagocytosis, it must be identified by phosphotungstic acid staining because of its higher sensitivity, it is more valuable for diagnosis.

The diagnosis of this disease is not difficult, most of them are caused by difficulty in walking due to lack of humerus. According to typical bone changes, diagnosis can be made, and kidney damage can be diagnosed. Radiological examination showed a humeral angle and was characterized by a characteristic diagnosis. 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. Renal biopsy specimens cannot be judged only by glomerular basement membrane moth phagocytosis, and fibrils must be identified by phosphotungstic acid staining.

Differential diagnosis

Need to be identified with the following symptoms:

Kidney stones: Stones are composed of inorganic salts or organic matter. There is a normal core in the stone, which consists of exfoliated epithelial cells, bacterial agglomerates, parasite eggs or worms, fecal blocks or foreign bodies. The inorganic salts or organic matter are deposited on the core layer. Due to the different organs involved, the composition, shape, texture, and influence on the body of the mechanism of stone formation are different. Small stones in the renal pelvis and renal pelvis can move with the body position. The shape of the larger stone is consistent with the shape of the cavity, which can be expressed as a typical staghorn or coral. Sometimes stones can fill the entire renal pelvis and renal pelvis and resemble the performance of pyelography. Lateral view, most of the kidney stones overlap with the spine.

Hydronephrosis: less common, with a transitional epithelium in the renal pelvis and cystic dilatation. Its occurrence also has congenital and acquired points. Different from the inflammatory obstruction mechanism of the diverticulum, there is no obvious cause of hydronephrosis. It is speculated that a functional obstruction may be formed due to the loss of the ring muscle at the entrance of the funnel. The most common symptoms are pain in the upper abdomen and sideways, occasionally touching a lump, sometimes causing hematuria. Hydronephrosis should be differentiated from multiple renal pelvis expansion caused by ureteral obstruction, pyelonephritis, medullary necrosis, and renal tuberculosis. In the hydronephrosis secondary to the outlet obstruction, it is feasible to perform an incision in the pelvis funnel to improve drainage. If the expansion is due to internal stones, the kidneys are partially removed as appropriate. At present, the surgical treatment of the percutaneous route can achieve satisfactory results.

Renal diverticulum: The renal diverticulum is the cystic cavity of the renal parenchyma that covers the transitional epithelial cells. It communicates with the renal pelvis or renal pelvis through a narrow passage. The diverticulum has no secretory function, but the urine can flow back into the sputum. The disease was first described by Rayer in 1841 and can be multiple, located anywhere in the kidney, but the renal pelvis is more susceptible.

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