2014年 · 第94卷第42期

human leucocyte antigen G (HLA-G) is a non-classical major histocompatibility complex (MHC-I). In recent years, the research on HLA-G genes and molecules is increasing day by day. Since 1989, Geraghty first reported successful cloning of HLA-G^[1]In 1998, the 1st International Conference on HLA-G was held at the Institute of Immunology of St. Louis Hospital in France. At that time, there were 208 HLA-G related articles, and now there are 1 738 articles. In China, many scholars have also carried out research and reports on HLA-G.

Peri-prosthetic aortic valve leakage (PVL) is one of the most common serious complications after cardiac surgical prosthetic valve replacement. In the past, surgical thoracotomy was used to repair paravalvular leakage, or valve replacement again, but the trauma was large, the risk was high, and it was easy to repeat residual leakage. In recent years, transcatheter occlusion of PVL has become another important alternative treatment^[1,2,3]。 However, this method has no special occluder, the operation is relatively complicated, and there are complications such as aggravation of postoperative hemolysis. In this study, a new polyester-free second generation vascular plug occluder (AVP Ⅱ) was applied to aortic valve PVL, which is easier to operate and can effectively reduce complications.

Exhaled nitric oxide (FeNO) detection is a new non-invasive airway inflammation detection technique developed in recent years. It has the advantages of quantitative, non-invasive, simple and safe, and has high specificity and sensitivity^[1]。 The American Thoracic Society/European Society of Respiratory Diseases (ATS/ERS) published standard practice for testing FeNO in 2005^[2]and reaffirmed the value of FeNO as a marker of airway inflammation in 2009 and 2011^[3,4]。 Many countries have successively carried out testing of FeNO. In 2006, the Global Asthma Initiative (GINA) included FeNO in asthma management protocols^[5]In 2008, the Respiratory Branch of Chinese Medical Association listed it in the Guidelines for the Prevention and Treatment of Bronchial Asthma^[6]。 The application progress of FeNO in the diagnosis and treatment of respiratory diseases is summarized as follows.

A 56-year-old male was seen for "abdominal pain accompanied by fever for 15 d, cough and shortness of breath". The patient had multiple skin nodules for more than 30 years, showing a trend of gradual aggravation, and had a history of hypertension for more than 10 years. The patient had a son and a brother with multiple nodules in the skin and was diagnosed as neurofibroma type I. Physical examination: There were extensive tumor-like masses of varying sizes on the skin of the whole body, coffee-like spots widely distributed on the epidermis, ecchymosis under the skin, no optic nerve atrophy, no papilledema and iris hamartoma (Lish nodule) nodules were found on fundus examination; A mass about 10 cm ×15 cm in size can be accessed in the left upper abdomen, with poor boundary, and positive percussion pain in the left kidney area. The digital image shows: thoracic scoliosis deformity, and multiple nodules of different sizes are seen in the soft tissues of the chest wall before and after the nodes on both sides; CT examination showed that an oval cystic mass of 7.0 cm ×7.7 cm ×8.9 cm was seen in the left upper abdominal cavity. The mass was closely related to the body and tail of the pancreas, and the boundary was unclear. There was no enhancement in the mass, uneven thickness of the mass wall, obvious enhancement of the cyst wall, separation, partial wall and separation shadows were seen in the mass. The boundary between the mass and the adjacent gastric cavity, spleen, left kidney and adrenal gland was clear. The left kidney was slightly moved downward under compression, and the boundary between the splenic artery and left renal vein was clear (FIG. 1A, lb); No enlarged lymph nodes were seen retroperitoneally, and multiple soft tissue density nodules and masses were seen subcutaneously in the abdomen, with mild enhancement (Figure 1C)； Multiple soft tissue density nodules and masses were seen under the scalp, and small round low density shadows were seen in the skin, with no obvious enhancement (Figure 1D)； The patient underwent abdominal mass resection, small intestinal mass resection, and subcutaneous nodule resection of abdomen and trunk. Postoperative pathology: (1) Abdominal subcutaneous and trunk nodules: HE staining showed that the tumor was composed of loosely arranged spindle cells, with few and indifferent cytoplasm, slender and wavy nucleus, and the cells were embedded in fibrous matrix (Figure 2)； (2) Adrenal gland: 1 gray-red gray-yellow mass, 7.0 cm ×7.7 cm ×8.9 cm in size, with calcification of cyst wall; HE: The tumor tissue cells are organ-like arrangement, with abundant blood vessels, moderate cell abnormality, intact envelope, calcification, most of the tumors in the envelope are bleeding, necrosis, and cholesterol crystals (Figure 3)； Immunohistochemical staining results: S-100 (+ +), synaptophysin (+ +), chromaffin (–), neuron-specific enolase (–), cytokeratin 7 (–), carcinoembryonic antigen (–), cell proliferation marker Ki-67 (–), tumor suppressor gene P53 (–), cytokeratin 18 (–); (3) Tumor of small intestinal wall: a piece of gray-white nodular tissue with mucosa, the size is 1.3 cm ×1.3 cm ×1.0 cm, and the mucosa area is 1.6 cm ×0.4 cm; Immunohistochemical staining results: neural marker acid binding protein S – 100 (–), oncogene product CD117 (+ +), endothelial cell marker CD34 (+ +), smooth muscle actin (+ +), Ki – 67 (–) (Figure 4，Figure 5)。 The diagnosis was as follows: neurofibroma type I with giant pheochromocytoma and small intestinal stromal tumor.

A 4-year-old child came to the Department of Ophthalmology of Beijing Children's Hospital for 3 years of photophobia in both eyes, with blepharospasm, polydipsia and polyuria, and lagging growth and development. No one else in the family had similar symptoms. The child had a full-term cesarean section, growth retardation, irritability, irritability, daily water consumption 1 000~1 200 ml. Full body examination: height 78 cm, weight 9.1 kg, square skull, mild valgus of bilateral costal margins, bracelet sign (+), bracelet sign (+) (Figure 1), neurological pathology (–).