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Lymphatic abnormalities in Noonan's syndrome.

作者信息

Lanning P, Similä S, Suramo I, Paavilainen T

出版信息

Pediatr Radiol. 1978 Jun 19;7(2):106-9. doi: 10.1007/BF00975679.

DOI:10.1007/BF00975679
PMID:673526
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.

摘要

相似文献

1
Lymphatic abnormalities in Noonan's syndrome.
Pediatr Radiol. 1978 Jun 19;7(2):106-9. doi: 10.1007/BF00975679.
2
Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan's syndrome.2例努南综合征患者经淋巴管造影显示有肺淋巴管扩张。
Radiology. 1975 Apr;115(1):149-53. doi: 10.1148/115.1.149.
3
Lymphatic dysplasia in a neonate with Noonan's syndrome.一名患有努南综合征的新生儿的淋巴发育异常。
Pediatr Radiol. 1997 Apr;27(4):321-3. doi: 10.1007/s002470050139.
4
Radiological features of late-onset lymphoedema in Noonan's syndrome.
Pediatr Radiol. 2003 Mar;33(3):200-2. doi: 10.1007/s00247-002-0768-6. Epub 2002 Jul 18.
5
Cardiovascular malformations in Turner's and Noonan's syndrome.特纳综合征和努南综合征中的心血管畸形。
Br Heart J. 1978 May;40(5):500-9. doi: 10.1136/hrt.40.5.500.
6
Cutaneous manifestations of Noonan's syndrome.
Arch Dermatol. 1978 Jun;114(6):929-30.
7
[Dysplasia of the lymphatic system in Noonan's syndrome (author's transl)].
Ann Radiol (Paris). 1979 Jan-Feb;22(1):53-5.
8
Chronic lymphangiectasis in Turner's syndrome.特纳综合征中的慢性淋巴管扩张症。
Br J Ophthalmol. 1986 May;70(5):396-9. doi: 10.1136/bjo.70.5.396.
9
[Turner's phenotype and diagnosis of Noonan's syndrome].
Pediatr Med Chir. 1982 Sep-Oct;4(5):535-8.
10
[Hypertrophic cardiomyopathy associated with Noonan's syndrome and membranous aortic subvalvular stenosis associated with Turner's syndrome. Report of 2 clinical cases].[与努南综合征相关的肥厚型心肌病及与特纳综合征相关的膜性主动脉瓣下狭窄。2例临床病例报告]
G Ital Cardiol. 1987 Sep;17(9):800-6.

引用本文的文献

1
Case report: Noonan syndrome with protein-losing enteropathy.病例报告:努南综合征合并蛋白丢失性肠病。
Front Genet. 2023 Sep 27;14:1237821. doi: 10.3389/fgene.2023.1237821. eCollection 2023.
2
Pathological MAPK activation-mediated lymphatic basement membrane disruption causes lymphangiectasia that is treatable with ravoxertinib.病理性 MAPK 激活介导的淋巴基底膜破坏导致淋巴管扩张,可通过 ravoxertinib 治疗。
JCI Insight. 2022 Sep 8;7(17):e153033. doi: 10.1172/jci.insight.153033.
3
MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome.

本文引用的文献

1
LYMPHANGIOGRAPHY AND CHROMOSOME STUDIES IN FEMALES WITH LYMPHOEDEMA AND POSSIBLE OVARIAN DYSGENESIS.患有淋巴水肿及可能存在卵巢发育不全的女性的淋巴管造影和染色体研究
Arch Dis Child. 1965 Feb;40(209):27-32. doi: 10.1136/adc.40.209.27.
2
Lymphographic differential diagnosis of neoplastic diseases.肿瘤性疾病的淋巴造影鉴别诊断
Acta Radiol Diagn (Stockh). 1965:Suppl 247:1+.
3
Noonan syndrome with intestinal lymphangiectasis. A metabolic and anatomic study.伴有肠道淋巴管扩张症的努南综合征。一项代谢与解剖学研究。
磁共振淋巴管成像在儿童和成人努南综合征中的淋巴异常诊断。
Sci Rep. 2022 Jul 1;12(1):11164. doi: 10.1038/s41598-022-13806-w.
4
Systematic literature review of lymphatic imaging-guided procedural management of Noonan syndrome.对努南综合征的淋巴成像引导程序管理的系统文献回顾。
J Vasc Surg Venous Lymphat Disord. 2022 Sep;10(5):1192-1196.e3. doi: 10.1016/j.jvsv.2022.03.017. Epub 2022 May 10.
5
Cell Fate Determination of Lymphatic Endothelial Cells.淋巴管内皮细胞的命运决定。
Int J Mol Sci. 2020 Jul 6;21(13):4790. doi: 10.3390/ijms21134790.
6
A PTPN11 mutation in a woman with Noonan syndrome and protein-losing enteropathy.一名患有努南综合征和蛋白丢失性肠病的女性存在PTPN11突变。
BMC Gastroenterol. 2020 Feb 13;20(1):34. doi: 10.1186/s12876-020-01187-1.
7
Noonan syndrome: clinical aspects and molecular pathogenesis.努南综合征:临床特征与分子发病机制
Mol Syndromol. 2010 Feb;1(1):2-26. doi: 10.1159/000276766. Epub 2010 Jan 15.
8
Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury.心包内手术后孤立性乳糜心包:右淋巴输出管意外损伤的可能作用
Tex Heart Inst J. 2007;34(1):82-7.
9
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.
Hum Genet. 1996 May;97(5):564-7. doi: 10.1007/BF02281861.
10
The swollen leg and primary lymphoedema.腿部肿胀与原发性淋巴水肿。
Arch Dis Child. 1994 Jul;71(1):44-9. doi: 10.1136/adc.71.1.44.
J Pediatr. 1972 Feb;80(2):269-74. doi: 10.1016/s0022-3476(72)80589-4.
4
The Ullrich-Noonan syndrome (Turner phenotype).乌尔里希-努南综合征(特纳表型)。
Am J Dis Child. 1974 Jan;127(1):48-55. doi: 10.1001/archpedi.1974.02110200050007.
5
The Noonan syndrome with intestinal lymphangiectasia.
J Pediatr. 1976 Feb;88(2):270-2. doi: 10.1016/s0022-3476(76)80998-5.
6
Pulmonary lymphangiectasia demonstrated by lymphangiography in 2 patients with Noonan's syndrome.2例努南综合征患者经淋巴管造影显示有肺淋巴管扩张。
Radiology. 1975 Apr;115(1):149-53. doi: 10.1148/115.1.149.
7
Lymph nodes and vessels in primary lymphoedema. Their relative importance in aetiology.原发性淋巴水肿中的淋巴结与淋巴管。它们在病因学中的相对重要性。
Ann R Coll Surg Engl. 1976 Jul;58(4):278-84.