Brazilian Journal of Medical and Biological Research | |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy | |
T.m. Santos-machado1  L.m. Cristófani1  M.t.a. Almeida1  P.t. Maluf1  P.a. Costa1  M.a. Pereira1  J.l.b.c. Brito1  V. Odone-filho1  | |
[1] ,Universidade de São PauloSão Paulo SP ,Brasil | |
关键词: Langerhans' cell histiocytosis; histiocytosis X; gastrointestinal involvement; protein-losing enteropathy; hypoalbuminemia; Cr51-labeled albumin test; | |
DOI : 10.1590/S0100-879X1999000900007 | |
来源: SciELO | |
【 摘 要 】
Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.
【 授权许可】
CC BY
All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License
【 预 览 】
Files | Size | Format | View |
---|---|---|---|
RO202005130076383ZK.pdf | 116KB | download |