10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report

BMC Pediatrics

Yoshihiro Komada²  Hidemi Toyoda²  Hirofumi Sawada²  Mikihiro Inoue¹  Kohei Otake¹  Yuhki Koike¹  Kentaro Kihira²  Hiroyuki Ohashi²  Tadashi Iwasa² 

[1] Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan;Department of Pediatrics, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan

关键词: Terbutaline;    Theophylline;    Methylprednisolone pulse;    Vascular endothelial damage;    Idiopathic systemic capillary leak syndrome;   

Others  :  1138815
DOI  :  10.1186/1471-2431-14-137

 received in 2013-06-27, accepted in 2014-05-23,  发布年份 2014

【 摘 要 】

Background

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by episodic life-threatening hypotension, hypoalbuminemia, and hemoconcentration.

Case presentation

A 10-year-old girl presented with abdominal pain, vomiting, diarrhea, fever and developed generalized edema a day after admission. Clinical and laboratory findings were consistent with ISCLS. She received aggressive fluid replacement, methylprednisolone pulse (30 mg/kg/day), high-dose intravenous immunoglobulin (IVIG, 2 g/kg/day) and plasma exchange in acute phase. She received fasciotomy of bilateral lower extremities as she developed complications of compartment syndrome. Since there were two episodes of ISCLS attacks, theophylline and terbutaline were initiated for prevention of attacks and then the remission is currently maintained. Because of high fatality rate in ISCLS, prompt diagnosis and intervention are very important.

Conclusion

We describe here, a rare case of pediatric ISCLS. ISCLS should be considered as a differential diagnosis, when the patient presents with unexplained or sudden hypovolemic shock. Reports on pediatrics ISCLS are very few, and accumulation of similar case reports is needed.

【 授权许可】

   
2014 Iwasa et al.; licensee BioMed Central Ltd.

【 参考文献 】

• [1]Clarkson B, Thompson D, Horwith M, Luckey EH: Cyclical edema and shock due to increased capillary permeability. Am J Med 1960, 29:193-216.
• [2]Milner CS, Wagstaff MJ, Rose GK: Compartment syndrome of multiple limbs: an unusual presentation. J Plast Reconstr Aesthet Surg 2006, 59(11):1251-1252.
• [3]Foeldvari I, Waida E, Junker AK: Systemic capillary leak syndrome in a child. J Pediatr 1995, 127(5):739-741.
• [4]Karatzios C, Gauvin F, Egerszegi EP, Tapiero B, Buteau C, Rivard GE, Ovetchkine P: Systemic capillary leak syndrome presenting as recurrent shock. Pediatr Crit Care Med 2006, 7(4):377-379.
• [5]Onal H, Aktuglu-Zeybek C, Altun G, Ozyilmaz I, Alhaj S, Aydin A: Capillary leak syndrome in a 5-month-old infant associated with intractable diarrhoea. Ann Trop Paediatr 2007, 27(1):81-86.
• [6]Dowden AM, Rullo OJ, Aziz N, Fasano MB, Chatila T, Ballas ZK: Idiopathic systemic capillary leak syndrome: novel therapy for acute attacks. J Allergy Clin Immunol 2009, 124(5):1111-1113.
• [7]Sion-Sarid R, Lerman-Sagie T, Blumkin L, Ben-Ami D, Cohen I, Houri S: Neurologic involvement in a child with systemic capillary leak syndrome. Pediatrics 2010, 125(3):e687-e692.
• [8]Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, Papo T, Soubrier M, Ruivard M, Malizia G, Tieulie N, Riviere S, Ninet J, Hatron PY, Amoura Z: The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med 2011, 154(7):464-471.
• [9]Piastra M, Pietrini D, Conti G, De Rosa G, Rigante D: Sudden shock from capillary leak. Lancet 2012, 379(9819):976.
• [10]Milani GP, Dellepiane RM, Castellazzi ML, Mazzoni MB, Bianchetti MG, Fossali EF: Episodic idiopathic systemic capillary leak syndrome in a girl. Pediatr Int 2013, 55(4):e81-e82.
• [11]Perme T, Pokorn M, Markelj G, Avcin T, Battelino T, Ursic T, Vidmar I, Grosek S: Two episodes of systemic capillary leak syndrome in an 8-year-old boy, following influenza A virus infection. Pediatr Infect Dis J 2014, 33(2):222-224.
• [12]Xie Z, Ghosh CC, Patel R, Iwaki S, Gaskins D, Nelson C, Jones N, Greipp PR, Parikh SM, Druey KM: Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood 2012, 119(18):4321-4332.
• [13]Nagao Y, Harada H, Yamanaka H, Fukuda K: Possible mediators for systemic capillary leak syndrome. Am J Med 2011, 124(1):e7-e9.
• [14]Nakagawa N, Ota H, Tanabe Y, Kabara M, Matsuki M, Chinda J, Sakamoto N, Fujino T, Takehara N, Takeuchi T, Kawabe J, Sato N, Kawamura Y, Fukuhara T, Ikuta K, Kikuchi K, Hasebe N: A case of idiopathic systemic capillary leak syndrome with high serum levels of G-CSF on exacerbation. Intern Med 2011, 50(6):597-600.
• [15]Kinoshita Y, Kasaoka S, Fujita M, Oshima C, Kawamura Y, Tsuruta R, Maekawa T: Synchronized changes in serum vascular endothelial growth factor during the clinical course of chronic systemic capillary leak syndrome. Intern Med 2010, 49(8):791-794.
• [16]Yabe H, Yabe M, Koike T, Shimizu T, Morimoto T, Kato S: Rapid improvement of life-threatening capillary leak syndrome after stem cell transplantation by bevacizumab. Blood 2010, 115(13):2723-2724.
• [17]Zipponi M, Eugster R, Birrenbach T: High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome. BMJ Case Rep 2011. doi:10.1136/bcr.12.2010.3599
• [18]Tahirkheli NK, Greipp PR: Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series. Ann Intern Med 1999, 130(11):905-909.