ABSTRACT
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease, the clinical manifestation is increased acute episodic capillary leakage (SCL), leading to protein-rich fluid leakage from the blood vessels to the interstitial tissue space, resulting in progressive systemic edema, hypoproteinemia, hypotension, blood concentration and other clinical symptoms. Because of ISCLS frequent recurrences, high mortality, pathogenesis unclear and no definite treatment plan, it is extremely important to fully understand the disease to carry out early intervention to save the patients' lives. This article reviews the progress of ISCLS, including its disease onset characteristics, clinical features, pathological mechanism, diagnosis and treatment of ISCLS.
ABSTRACT
Objective To investigate the changes of the difference between hematocrit (Hct) and plasma albumin (Alb) in the course of patients with systemic capillary leak syndrome (SCLS). Methods 281 case reports on human vascular leaking from the PubMed database from January 1st, 1996 to September 30th, 2015 were screened by systematic review method. Studies related to intracranial vascular leakage or intraocular vascular leakage were excluded. 213 articles related to SCLS were identified (164 in English, 16 in French, 8 in Japanese, 7 in German, 7 in Spanish, 4 in Italian, 2 in Chinese, 2 in Danish, 2 in Dutch, and 1 in Swedish). Due to the unavailable full text, 40 articles were excluded. A total of 173 articles related to SCLS were screened, of which 84 patients were enrolled. The data of Alb, Hct, age, gender, weight change, the length of hospital stay and 24-hour fluid infusion volume in SCLS patients were recorded, and the difference between Hct and plasma Alb (Hct-Alb) was calculated. According to the time when accurate Hct and Alb data were collected, they were divided into three groups: basic value group before onset, value group at onset and value group at recovery/discharge after onset. The levels of Hct and Alb and Hct-Alb at different time points in the course of the disease were compared. Pearson test was used to analyze the correlation between Hct-Alb and 24-hour fluid infusion volume. Results ① A total of 12 cases with both exact values of Alb and Hct [or hemoglobin (Hb)] at the time of onset and recovery after treatment were selected from 84 cases of SCLS. It was shown that the Hct-Alb at the time of onset was significantly higher than that after treatment (26.33±16.36 vs. 0.55±8.81, P < 0.001). ② A total of 17 cases with both the pre-onset baseline value and the exact values of Alb and Hct (or Hb) at the time of onset were selected from 84 cases of SCLS. It was shown that the Hct-Alb at the time of onset was significantly higher than that of the pre-onset basic value (15.83±11.37 vs. 1.82±7.97, P < 0.001). ③ A total of 14 cases with both exact values of Alb, Hct and 24-hour fluid infusion volume at the time of onset were selected from 84 cases of SCLS. It was shown that the Hct-Alb was 35.45±19.58 at the time of onset. The average 24-hour fluid infusion volume was (9.82±4.95) L, and the maximum volume of fluid infusion was 20 L. Pearson correlation analysis showed that the Hct-Alb at the time of onset was significantly positively correlated with 24-hour fluid infusion volume (r = 0.578, P < 0.05). Conclusions In the analysis of SCLS cases published with adequate data available from 1996 to 2015, it was revealed that: ① the difference in Hct-Alb levels at the onset of SCLS was 32.06±17.41. ② The greater the difference between Hct and plasma Alb, the more amount of fluid required to maintain normal blood pressure.