Skopnik H, Heimann G
Abt. Kinderheilkunde, Rheinisch-Westfälischen-Technischen Hochschule Aachen.
Klin Padiatr. 1987 Nov-Dec;199(6):453-6. doi: 10.1055/s-2008-1026839.
The symptom complex--hypoproteinemia, edema and anemia--occurs in approximately 5 percent of CF-patients during the first 6 months of life. Since milk is the only nutritional source in this age group, a hypocaloric nutrition respectively marginaly low protein intake may contribute to these symptoms in case of an exocrine pancreatic insufficiency. Even if breast milk and soy bean formulas can advance the disease it must be mentioned that relapses of the symptom triad had never occurred under pancreatic enzyme supplementation independently of the source of milk being fed. A cow's milk protein intolerance and coincidental exocrine pancreatic insufficiency lead to the symptom complex in an infant in spite of an adequate caloric and protein intake. This case revealed again that sweat electrolyte tests are unreliable during the acute period of disease, sweat tests were positive when the presenting symptoms were resolved. The case demonstrates that repeated sweat chloride tests are mandatory during an asymptomatic phase of disease besides the exclusion of a renal or cardiac origin of the symptom complex. An early postnatal diagnosis of CF and immediate initiation of pancreatic enzyme and fat soluble vitamin supplementation could prevent such courses of disease.
症状复合体——低蛋白血症、水肿和贫血——在约5%的囊性纤维化(CF)患者出生后的前6个月出现。由于牛奶是这个年龄组唯一的营养来源,在存在外分泌性胰腺功能不全的情况下,热量摄入不足或蛋白质摄入量略低可能导致这些症状。即使母乳和大豆配方奶粉可能会使病情进展,但必须指出,无论喂养何种奶源,在补充胰酶的情况下,症状三联征从未复发。尽管热量和蛋白质摄入充足,但牛奶蛋白不耐受和同时存在的外分泌性胰腺功能不全会导致婴儿出现症状复合体。该病例再次表明,在疾病急性期,汗液电解质检测不可靠,当出现的症状缓解时,汗液检测呈阳性。该病例表明,除了排除症状复合体的肾脏或心脏病因外,在疾病无症状期必须反复进行汗液氯化物检测。CF的早期产后诊断以及立即开始补充胰酶和脂溶性维生素可以预防此类病程。