Abstract | INTRODUCTION: Idiopathic systemic capillary leak syndrome is a rare and fatal disease due to the unexplained episodic attacks of capillary leakage of plasma from the intravascular into the interstitial space. The attack consists of three phases, a prodromal phase, peripheral leak phase and recruitment phase. During the peripheral leak phase, generalized edema, mainly in the trunk and extremities, with hemoconcentration and hypoalbuminemia occurs, while usually the visceral organs like lungs, brain, heart and kidneys seem not to be involved. Treatment of the acute phase is supportive, focusing on adequate but not overzealous fluid resuscitation, because pulmonary edema usually occurs in the recruitment phase. CASE PRESENTATION: CONCLUSIONS:
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Authors | Yuri Hirosaki, Shunji Hayashidani, Sayako Ouchi, Tukasa Ohshima, Ryuji Nakano, Hideo Yamamoto |
Journal | Journal of medical case reports
(J Med Case Rep)
Vol. 9
Pg. 90
(Apr 28 2015)
ISSN: 1752-1947 [Electronic] England |
PMID | 25928289
(Publication Type: Case Reports, Journal Article)
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Topics |
- Aged
- Capillary Leak Syndrome
(complications, diagnosis, therapy)
- Disease Progression
- Edema
(etiology)
- Fatal Outcome
- Female
- Fluid Therapy
(methods)
- Humans
- Pulmonary Edema
(etiology)
- Shock
(therapy)
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