Transnodal lymphangiography and post-CT for protein-losing enteropathy in Noonan syndrome. 2015

Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
Department of Radiology, Tokai University Hachioji Hospital, Tokai University School of Medicine , Tokyo , Japan.

Noonan syndrome, which is a multiple congenital disorder, may be associated with lymphatic abnormalities. Protein-losing enteropathy (PLE) developing in Noonan syndrome is rare. We performed transnodal lymphangiography by directly accessing bilateral inguinal nodes under ultrasound guidance in a 17-year-old female with PLE developing in Noonan syndrome to assess detailed anatomical findings regarding lymphatic vessels. There have been no reports on transnodal lymphangiography for Noonan syndrome. Post-lymphangiographic CT images revealed multiple lymphatic abnormalities and lipiodol extravasation into the duodenum and the proximal jejunum. Transnodal lymphangiography was easy and safe for PLE developing in Noonan syndrome, and post-lymphangiographic CT provided invaluable information.

UI MeSH Term Description Entries
D008220 Lymphography Radiographic study of the lymphatic system following injection of dye or contrast medium. Lymphangiography,Lymphangiographies,Lymphographies
D009634 Noonan Syndrome A genetically heterogeneous, multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, CRYPTORCHIDISM, multiple cardiac abnormalities (most commonly including PULMONARY VALVE STENOSIS), and some degree of INTELLECTUAL DISABILITY. The phenotype bears similarities to that of TURNER SYNDROME that occurs only in females and has its basis in a 45, X karyotype abnormality. Noonan syndrome occurs in both males and females with a normal karyotype (46,XX and 46,XY). Mutations in a several genes (PTPN11, KRAS, SOS1, NF1 and RAF1) have been associated the NS phenotype. Mutations in PTPN11 are the most common. LEOPARD SYNDROME, a disorder that has clinical features overlapping those of Noonan Syndrome, is also due to mutations in PTPN11. In addition, there is overlap with the syndrome called neurofibromatosis-Noonan syndrome due to mutations in NF1. Male Turner Syndrome,Turner Syndrome, Male,Familial Turner Syndrome,Female Pseudo-Turner Syndrome,Noonan Syndrome 1,Noonan-Ehmke Syndrome,Pseudo-Ullrich-Turner Syndrome,Turner Phenotype with Normal Karyotype,Turner's Phenotype, Karyotype Normal,Turner's Syndrome, Male,Turner-Like Syndrome,Ullrich-Noonan Syndrome,Female Pseudo Turner Syndrome,Male Turner's Syndrome,Noonan Ehmke Syndrome,Pseudo Ullrich Turner Syndrome,Pseudo-Turner Syndrome, Female,Turner Like Syndrome,Turner Syndrome, Familial,Ullrich Noonan Syndrome
D011504 Protein-Losing Enteropathies Pathological conditions in the INTESTINES that are characterized by the gastrointestinal loss of serum proteins, including SERUM ALBUMIN; IMMUNOGLOBULINS; and at times LYMPHOCYTES. Severe condition can result in HYPOGAMMAGLOBULINEMIA or LYMPHOPENIA. Protein-losing enteropathies are associated with a number of diseases including INTESTINAL LYMPHANGIECTASIS; WHIPPLE'S DISEASE; and NEOPLASMS of the SMALL INTESTINE. Enteropathy, Exudative,Idiopathic Hypercatabolic Hypoproteinemia,Enteropathy, Protein-Losing,Protein-Losing Enteropathy,Enteropathies, Exudative,Enteropathies, Protein-Losing,Exudative Enteropathies,Exudative Enteropathy,Hypercatabolic Hypoproteinemia, Idiopathic,Hypercatabolic Hypoproteinemias, Idiopathic,Hypoproteinemia, Idiopathic Hypercatabolic,Hypoproteinemias, Idiopathic Hypercatabolic,Idiopathic Hypercatabolic Hypoproteinemias,Protein Losing Enteropathies,Protein Losing Enteropathy
D011860 Radiography, Abdominal Radiographic visualization of the body between the thorax and the pelvis, i.e., within the peritoneal cavity. Abdominal Radiography,Abdominal Radiographies,Radiographies, Abdominal
D011877 Radionuclide Imaging The production of an image obtained by cameras that detect the radioactive emissions of an injected radionuclide as it has distributed differentially throughout tissues in the body. The image obtained from a moving detector is called a scan, while the image obtained from a stationary camera device is called a scintiphotograph. Gamma Camera Imaging,Radioisotope Scanning,Scanning, Radioisotope,Scintigraphy,Scintiphotography,Imaging, Gamma Camera,Imaging, Radionuclide
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D013668 Technetium Tc 99m Aggregated Albumin A gamma-emitting radionuclide imaging agent used for the diagnosis of diseases in many tissues, particularly in cardiovascular and cerebral circulation. Tc 99m-Albumin,Tc 99m-Albumin Colloid,99m-Tc-HSA,99mTc-Albures,99mTc-HSA,99mTc-Human Serum Albumin,Tc-99m-HAM,Tc-99m-MAA,Tc-99m-Microalbumin,Tc-HAMM,Tc99m-Albumin,Technetium-99m Albumin Colloid,99mTc Albures,99mTc Human Serum Albumin,99mTcAlbures,Albumin Colloid, Technetium-99m,Albumin, 99mTc-Human Serum,Colloid, Tc 99m-Albumin,Colloid, Technetium-99m Albumin,Serum Albumin, 99mTc-Human,Tc 99m Albumin,Tc 99m Albumin Colloid,Tc 99m HAM,Tc 99m MAA,Tc 99m Microalbumin,Tc HAMM,Tc99m Albumin,Technetium 99m Albumin Colloid
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography

Related Publications

Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
January 2023, Frontiers in genetics,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
July 2009, Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
April 2015, Pediatrics international : official journal of the Japan Pediatric Society,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
February 2020, BMC gastroenterology,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
October 1966, Vnitrni lekarstvi,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
July 2018, Indian journal of pediatrics,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
January 2000, European radiology,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
January 1997, Clinical and experimental rheumatology,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
January 1986, Journal of pediatric gastroenterology and nutrition,
Tomohiro Matsumoto, and Takahiro Kudo, and Jun Endo, and Kazunobu Hashida, and Nao Tachibana, and Takatsugu Murakoshi, and Terumitsu Hasebe
August 1986, Archives of disease in childhood,
Copied contents to your clipboard!