Short longitudinal groove

Introduction Short longitudinal sulcus is a special appearance of Digolger syndrome patients after birth, which is caused by thymic hypoplasia, peripheral lymphoid tissue and lack of T lymphocyte or T cell dysfunction in the blood circulation. Digeorg syndrome, a congenital athymic or hypoplasia health search, is the result of developmental defects in the third and fourth pharyngeal sacs. It belongs to primary cellular immunodeficiency disease.

Cause

The cause of this disease is the congenital immune deficiency caused by the abnormalities of the first to sixth pairs of pharyngeal sacs in the early stage of the embryo. At the 6th to 10th week of the embryo, the thymus, parathyroid gland and part of the face, aortic arch and cardiac tuberculosis develop from the first to the sixth cell components of the pharyngeal sac. By the 12th week of pregnancy, the thymus migrated to the chest, which was previously caused by abnormal development of these embryonic tissues.

an examination

Related inspection

Chest CT examination chest external examination

1, laboratory inspection

(1) Lymphocytes decrease in peripheral blood, especially T cells decrease, and the percentage of B cells increases.

(2) Cellular immune function has been reduced to varying degrees.

(3) The humoral immune function is variable, and serum immunoglobulin is often not low.

(4) The blood calcium content is lowered.

(5) The level of parathyroid hormone is lowered.

2. X-ray examination shows that the thymus is shrinking or absent.

3. Lymph node biopsy showed a lack of lymphocytes in the paracortical area.

This disease should be distinguished from other causes of thymic dysfunction, the disease is a genetic disease, that is, thymic dysplasia, but not combined with parathyroid function insufficiency.

Differential diagnosis

This disease should be distinguished from other causes of thymic dysfunction, the disease is a genetic disease, that is, thymic dysplasia, but not combined with parathyroid function insufficiency.

1, laboratory inspection

(1) Lymphocytes decrease in peripheral blood, especially T cells decrease, and the percentage of B cells increases.

(2) Cellular immune function has been reduced to varying degrees.

(3) The humoral immune function is variable, and serum immunoglobulin is often not low.

(4) The blood calcium content is lowered.

(5) The level of parathyroid hormone is lowered.

2. X-ray examination shows that the thymus is shrinking or absent.

3. Lymph node biopsy showed a lack of lymphocytes in the paracortical area.

This disease should be distinguished from other causes of thymic dysfunction, the disease is a genetic disease, that is, thymic dysplasia, but not combined with parathyroid function insufficiency.

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