Literature DB >> 673526

Lymphatic abnormalities in Noonan's syndrome.

P Lanning, S Similä, I Suramo, T Paavilainen.   

Abstract

Five boys who had Noonan's syndrome and lymphatic abnormalities are reported. The youngest boy had clinical lymphoedema and the other four showed dermal backflow after interdigital injection of Patent Blue indicating impairment of flow along the superficial lymphatics. One boy had severe bilateral chylothorax. The lymphographic findings in four of these boys are reported. Patients with the Noonan syndrome frequently have oedema of the hands and feet at birth, which decreases during the first years of life [10]. It has been demonstrated by lymphography that similar peripheral oedema in patients with the Turner's syndrome is due to lymphatic hypoplasia [1, 3]. We report certain lymphatic abnormalities diagnosed by lymphography in four out of five patients with Noonan's syndrome.

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Year:  1978        PMID: 673526     DOI: 10.1007/BF00975679

Source DB:  PubMed          Journal:  Pediatr Radiol        ISSN: 0301-0449


  7 in total

1.  LYMPHANGIOGRAPHY AND CHROMOSOME STUDIES IN FEMALES WITH LYMPHOEDEMA AND POSSIBLE OVARIAN DYSGENESIS.

Authors:  P F BENSON; M H GOUGH; P E POLANI
Journal:  Arch Dis Child       Date:  1965-02       Impact factor: 3.791

2.  The Noonan syndrome with intestinal lymphangiectasia.

Authors:  D B Herzog; R Logan; J B Kooistra
Journal:  J Pediatr       Date:  1976-02       Impact factor: 4.406

3.  The Ullrich-Noonan syndrome (Turner phenotype).

Authors:  J J Nora; A H Nora; A K Sinha; R D Spangler; H A Lubs
Journal:  Am J Dis Child       Date:  1974-01

4.  Noonan syndrome with intestinal lymphangiectasis. A metabolic and anatomic study.

Authors:  H L Vallet; P G Holtzapple; W R Eberlein; W C Yakovac; T Moshang; A M Bongiovanni
Journal:  J Pediatr       Date:  1972-02       Impact factor: 4.406

5.  Lymphographic differential diagnosis of neoplastic diseases.

Authors:  M Wiljasalo
Journal:  Acta Radiol Diagn (Stockh)       Date:  1965

6.  Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan's syndrome.

Authors:  H A Baltaxe; J G Lee; K H Ehlers; M A Engle
Journal:  Radiology       Date:  1975-04       Impact factor: 11.105

7.  Lymph nodes and vessels in primary lymphoedema. Their relative importance in aetiology.

Authors:  J B Kinmonth; P W Eustace
Journal:  Ann R Coll Surg Engl       Date:  1976-07       Impact factor: 1.891

  7 in total
  9 in total

1.  Noonan syndrome: clinical aspects and molecular pathogenesis.

Authors:  M Tartaglia; G Zampino; B D Gelb
Journal:  Mol Syndromol       Date:  2010-01-15

2.  Coarctation of the aorta and renal hypoplasia in a boy with Turner/Noonan surface anomalies and a 46,XY karyotype: a clinical model for the possible impairment of a putative lymphogenic gene(s) for Turner somatic stigmata.

Authors:  T Hasegawa; T Ogata; Y Hasegawa; M Honda; T Nagai; Y Fukushima; Y Nakahori; N Matsuo
Journal:  Hum Genet       Date:  1996-05       Impact factor: 4.132

Review 3.  Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury.

Authors:  Chung-Dann Kan; Jieh-Neng Wang; Jing-Ming Wu; Yu-Jen Yang
Journal:  Tex Heart Inst J       Date:  2007

Review 4.  Systematic literature review of lymphatic imaging-guided procedural management of Noonan syndrome.

Authors:  Taylor Paul Cox; Christopher James Vance; Sarah K Daley; Cristobal Papendieck; Hugh McGregor; Philip Kuo; Marlys H Witte
Journal:  J Vasc Surg Venous Lymphat Disord       Date:  2022-05-10

5.  MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome.

Authors:  C C Pieper; J Wagenpfeil; A Henkel; S Geiger; T Köster; K Hoss; J A Luetkens; C Hart; U I Attenberger; A Müller
Journal:  Sci Rep       Date:  2022-07-01       Impact factor: 4.996

6.  The swollen leg and primary lymphoedema.

Authors:  N B Wright; H M Carty
Journal:  Arch Dis Child       Date:  1994-07       Impact factor: 3.791

Review 7.  Cell Fate Determination of Lymphatic Endothelial Cells.

Authors:  Young Jae Lee
Journal:  Int J Mol Sci       Date:  2020-07-06       Impact factor: 5.923

Review 8.  A PTPN11 mutation in a woman with Noonan syndrome and protein-losing enteropathy.

Authors:  Na Wang; Wen Shi; Yang Jiao
Journal:  BMC Gastroenterol       Date:  2020-02-13       Impact factor: 3.067

9.  Pathological MAPK activation-mediated lymphatic basement membrane disruption causes lymphangiectasia that is treatable with ravoxertinib.

Authors:  Harish P Janardhan; Karen Dresser; Lloyd Hutchinson; Chinmay M Trivedi
Journal:  JCI Insight       Date:  2022-09-08
  9 in total

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