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Case Reports
. 2009 Jul;18(3):87-93.
doi: 10.1297/cpe.18.87. Epub 2009 Aug 1.

Late-onset Lymphedema and Protein-losing Enteropathy with Noonan Syndrome

Kosei Hasegawa  1 , Yoshiharu Nagaoka, Hidehiko Maruyama, Kunihiko Aya, Hiroyuki Tanaka, Tsuneo Morishima
Affiliations
Case Reports

Late-onset Lymphedema and Protein-losing Enteropathy with Noonan Syndrome

Kosei Hasegawa et al. Clin Pediatr Endocrinol. 2009 Jul.

Abstract

Noonan syndrome is characterized by facial dysmorphology, congenital heart disease and growth failure. Although it is also accompanied by deranged lymph-vessel formation, protein-losing enteropathy (PLE) with Noonan syndrome is rarely reported. We report clinical information about a boy with Noonan syndrome and late-onset lymphedema and PLE after standing for long periods of time during athletic practice sessions. The boy recovered from lymphedema and PLE after administration of 2.5 g of albumin followed by resting and raising his legs. They did not recur after he began walking again. Standing for long periods of time congested the lymph stream at the abdominal lymph vessel, whose formation is frequently disturbed in Noonan syndrome, and the increased pressure caused lymphedema and PLE. PLE is one of the clinical manifestations of Noonan syndrome.

Keywords: Noonan syndrome; growth hormone; lymphedema; protein-losing enteropathy.

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Figures

Fig. 1
Fig. 1
Distinctive features of our patient. Edema of the lower abdomen and hydrocele testis were observed.
Fig. 2
Fig. 2
Growth charts of our patient. Growth hormone treatment was started at the age of 12 yr of age.
Fig. 3
Fig. 3
Clinical courses during 2006–2007. Hypoproteinemia worsen after athletic meets in 2006 and 2007.
Fig. 4
Fig. 4
Clinical courses during and around admission.
Fig. 5
Fig. 5
Results of 99mTc-human serum albumin scintigraphy on day 2 (A, 5 min after injection; B, 6 h after injection) and day 6 (C, 5 min after injection; D, 8 h after injection). White arrows indicate protein loss from the small intestine.
See this image and copyright information in PMC

References

    1. Noonan JA. Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease. Am J Dis Child 1968;116: 373–80 - PubMed
    1. Bloomfield FH, Hadden W, Gunn TR. Lymphatic dysplasia in a neonate with Noonan’s syndrome. Pediatr Radiol 1997;27: 321–3 - PubMed
    1. Ho WL, Wang JK, Li YW. Radiological features of late-onset lymphoedema in Noonan’s syndrome. Pediatr Radiol 2003;33: 200–2 - PubMed
    1. Lin WS, Hwang MS, Chung HT, Chu JJ, Lai MW, Yang JS, et al. Protein-losing enteropathy after the Fontan operation: clinical analysis of nine cases. Chang Gung Med J 2006;29: 505–12 - PubMed
    1. Meijers BK, Schalla S, Eerens F, Van Suylen RJ, Broers B, Cheriex EM, et al. Protein-losing enteropathy in association with constrictive pericarditis. Int J Cardiovasc Imaging 2006;22: 389–92 - PubMed

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