Fernandes M I, Troncon L E, Iazigi N, Collares E F
Departamento de Puericultura e Pediatria da Faculdade de Medicina de Ribeirão Preto.
Arq Gastroenterol. 1988 Apr-Jun;25(2):93-103.
Thirteen children carrying protein-losing gastroenteropathy confirmed by the excretion fecal 51Cr albumin are presented. The ages ranged from 3 months to 12 years old, and the mean age of onset of symptom was 6 years 11 months old. Symptoms initiated between 3 to 7 years old in 11 patients. First and second grade malnutrition were detected within 10 cases. The most frequent clinical observation was diarrhea, found in 11 patients, followed by peripheral edema (10), anorexia (10), paleness (10), muscular hypotrophy (9), abdominal distension (8), ascite (7), wasted (7) and vomiting (5). The serum albumin level was below 2.5 g/100 ml in 12 patients, with and average of 1.7 g/100 ml. The gammaglobulin fraction was below 1.0 g/100 ml in 8 patients and lymphocytopenia in 5. Tests have shown a compromised fat absorption, thus, 7 among 8 lipiodol's test were negatives. The etiologic diagnosis were: paracoccidioidomycoses (5 patients), primary enteric lymphangiectasia (1), stronglyoidiasis (1), constrictive pericarditis (1), rheumatic carditis with double mitral lesion (1), Ménétrier's disease (1), short gut malabsorption syndrome (1), celiac disease (1) and cow's milk allergy (1). The responsible mechanism for the great loss of proteins to the digestive tube as well as diagnose and therapeutic aspects of the patients are commented. A report of 4 cases was done, as a way to show the pathology more characteristically due to protein losing gastroenteropathy.
本文报告了13例经粪便51Cr白蛋白排泄证实患有蛋白丢失性胃肠病的儿童。年龄范围为3个月至12岁,症状开始出现的平均年龄为6岁11个月。11例患者症状起始于3至7岁。10例患者检测出一级和二级营养不良。最常见的临床观察结果是腹泻,11例患者出现腹泻,其次是外周水肿(10例)、厌食(10例)、面色苍白(10例)、肌肉萎缩(9例)、腹胀(8例)、腹水(7例)、消瘦(7例)和呕吐(5例)。12例患者血清白蛋白水平低于2.5g/100ml,平均为1.7g/100ml。8例患者γ球蛋白组分低于1.0g/100ml,5例患者淋巴细胞减少。检查显示脂肪吸收受损,因此,8例碘油试验中有7例为阴性。病因诊断为:副球孢子菌病(5例)、原发性肠淋巴管扩张症(1例)、粪类圆线虫病(1例)、缩窄性心包炎(1例)、风湿性心脏病伴二尖瓣双病变(1例)、门脉高压性胃病(1例)、短肠吸收不良综合征(1例)、乳糜泻(1例)和牛奶过敏(1例)。本文对蛋白质大量丢失至消化道的相关机制以及患者的诊断和治疗方面进行了讨论。还报告了4例病例,以更典型地展示蛋白丢失性胃肠病的病理情况。