Del Rosario M A, Fitzgerald J F, Chong S K, Croffie J M, Gupta S K
Division of Gastroenterology-Hepatology-Nutrition, James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis 46202-5225, USA.
J Pediatr Gastroenterol Nutr. 1998 Aug;27(2):191-5. doi: 10.1097/00005176-199808000-00012.
The finding of characteristic small intestinal mucosal abnormalities on histologic examination of a biopsy specimen remains the first requirement for the diagnosis of celiac disease. A reliable and noninvasive test would be ideal for the patient's convenience and for reducing health-care costs. The sensitivity and specificity of anti-gliadin antibodies (AGA-immunoglobulin [Ig] G, AGA-IgA) have been variable; anti-endomysium IgA (EmA-IgA) is more helpful. In an earlier study conducted at the authors' institution, celiac disease was present in 19 patients examined from 1992 to 1995. Anti-endomysium titers were higher than normal in all 19 patients (100%). Total villous atrophy was seen in 14 of 17 biopsy specimens (82%) and subtotal atrophy in 3 (18%). The purpose of the current study was to evaluate further the accuracy of EmA-IgA in diagnosing celiac disease.
One hundred seven patients were screened for celiac disease between March 1996 and July 1997. The level of EmA-IgA was measured in all patients, and AGA-IgG and AGA-IgA were measured in 104 patients. Forty-six patients underwent endoscopic biopsy of the small bowel, with measurement of disaccharidase enzymes in 45 patients.
Five of 46 patients had celiac disease (three boys and two girls; mean age, 5.3 years; 2-9.5 years); one also had cystic fibrosis and another had insulin-dependent diabetes mellitus. All five had marked to complete villous atrophy with crypt hyperplasia and increased serum EmA-IgA (100% sensitivity). None of the remaining patients had increased EmA-IgA (100% specificity). Serum levels of AGA-IgG and AGA-IgA were increased in all four celiac disease patients (100% sensitivity), but they were also high in patients without celiac disease (38% and 92% specificity, respectively), which compromises their diagnostic value. None of the patients confirmed to have celiac disease had IgA deficiency. Abnormal disaccharidase enzyme activities were documented in all five celiac disease patients: severe generalized deficiency (n = 2), moderately severe generalized deficiency (n = 2), and alactasia with moderate deficiency of the alpha-glucosidases (n = 1).
This study confirmed the reliability and accuracy of EmA-IgA in the diagnosis of celiac disease. Small bowel biopsy may be unnecessary in EmA-positive patients in whom celiac disease is suspected.
在活检标本的组织学检查中发现特征性小肠黏膜异常仍是诊断乳糜泻的首要条件。对于患者的便利性及降低医疗成本而言,一种可靠且非侵入性的检测方法将是理想之选。抗麦醇溶蛋白抗体(抗麦醇溶蛋白免疫球蛋白[Ig]G、抗麦醇溶蛋白IgA)的敏感性和特异性一直存在差异;抗肌内膜IgA(EmA-IgA)更具诊断价值。在作者所在机构进行的一项早期研究中,1992年至1995年检查的19例患者患有乳糜泻。所有19例患者(100%)的抗肌内膜滴度均高于正常水平。17例活检标本中有14例(82%)出现完全绒毛萎缩,3例(18%)出现部分萎缩。本研究的目的是进一步评估EmA-IgA在诊断乳糜泻中的准确性。
1996年3月至1997年7月期间,对107例患者进行了乳糜泻筛查。检测了所有患者的EmA-IgA水平,并对104例患者检测了抗麦醇溶蛋白IgG和抗麦醇溶蛋白IgA。46例患者接受了小肠内镜活检,45例患者检测了双糖酶活性。
46例患者中有5例患有乳糜泻(3名男孩和2名女孩;平均年龄5.3岁;2至9.5岁);1例还患有囊性纤维化,另1例患有胰岛素依赖型糖尿病。所有5例均有明显至完全的绒毛萎缩伴隐窝增生,且血清EmA-IgA升高(敏感性100%)。其余患者中无一例EmA-IgA升高(特异性100%)。4例乳糜泻患者的血清抗麦醇溶蛋白IgG和抗麦醇溶蛋白IgA水平均升高(敏感性100%),但在无乳糜泻的患者中也较高(特异性分别为38%和92%),这降低了它们的诊断价值。确诊为乳糜泻的患者中无一例存在IgA缺乏。所有5例乳糜泻患者均记录到双糖酶活性异常:严重广泛性缺乏(2例)、中度严重广泛性缺乏(2例)和乳糖酶缺乏伴α-葡萄糖苷酶中度缺乏(1例)。
本研究证实了EmA-IgA在诊断乳糜泻中的可靠性和准确性。对于怀疑患有乳糜泻且EmA阳性的患者,可能无需进行小肠活检。