Results 31 to 40 of about 346 (122)

Investigation into the genetics of fetal congenital lymphatic anomalies

Prenatal Diagnosis, Volume 43, Issue 6, Page 703-716, June 2023., 2023
Abstract Objective Congenital lymphatic anomalies (LAs) arise due to defects in lymphatic development and often present in utero as pleural effusion, chylothorax, nuchal and soft tissue edema, ascites, or hydrops. Many LAs are caused by single nucleotide variants, which are not detected on routine prenatal testing.
Daniella Rogerson, Anna Alkelai, Jessica Giordano, Madhulatha Pantrangi, Meng‐Chang Hsiao, Chia‐Ling Nhan‐Chang, Joshua E. Motelow, Vimla Aggarwal, David Goldstein, Ron Wapner, Carrie J. Shawber   +10 more
wiley   +1 more source

Clinical differences in sirolimus treatment with low target levels between children and adults with vascular malformations – A nationwide trial

Clinical and Translational Science, Volume 16, Issue 5, Page 781-796, May 2023., 2023
Abstract The clinical presentation of patients with slow‐flow vascular malformations is very heterogeneous. High clinical burden and subsequent reduced health‐related quality of life is something they have in common. There is an unmet medical need for these patients for whom regular treatments like surgery and embolization are either insufficient or ...
Veroniek E. M. Harbers, Lilly G. J. M. Zwerink, Gerard A. Rongen, Willemijn M. Klein, Carine J. M. van der Vleuten, Ingrid M. P. van Rijnsoever, Lynda Gerdsen‐Drury, Uta E. Flucke, Bas H. Verhoeven, Peter C. J. de Laat, Chantal M. A. M. van der Horst, Leo J. Schultze Kool, D. Maroeska W. M. te Loo   +12 more
wiley   +1 more source

Kaposiform hemangioendothelioma with fatal income: Kasabach–Merritt phenomenon and hypercalcemia

Clinical Case Reports, Volume 10, Issue 2, February 2022., 2022
Kaposiform hemangioendothelioma, a rare borderline vascular tumor of infancy, can rarely present with consumptive thrombocytopenia and coagulation abnormalities (known as Kasabach–Merritt phenomenon). Associated hypercalcemia can also occur, which is possibly related to parathyroid hormone‐related protein produced by tumor.
Baklouti Massara, Rekik Mariem, Bahloul Emna, Triki Meriam, Safi Faiza, Boudaya Sonia, Amouri Meriem, Gargouri Lamia, Sellami Tahia, Turki Hamida   +9 more
wiley   +1 more source

An institutional audit of the use of novel drugs in pediatric oncology

Cancer Reports, Volume 4, Issue 6, December 2021., 2021
Abstract Background Significant challenges persist in treating children with rare, relapsed, or refractory malignancies. Novel molecularly targeted drugs promise improved outcomes for these children with reduced toxicity. However, there is often limited evidence to substantiate their clinical efficacy and guide their use.
Justin Lee, Lynn Gillam, Sarah Kouw, Maria C. McCarthy, Jordan R. Hansford   +4 more
wiley   +1 more source

Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

Diagnostic Pathology, 2021
Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes.
Qurratulain Chundriger, Muhammad Usman Tariq, Jamshid Abdul-Ghafar, Arsalan Ahmed, Nasir Ud Din   +4 more
doaj   +1 more source

mTOR Inhibitors in the complex treatment of high-risk vascular malformations in children

Хірургія дитячого віку, 2020
Recent experimental and clinical studies demonstrated that mTOR receptor inhibitors can stop the progression of vascular malformations and significantly improve patients’ quality of life.
I.M. Benzar, D.V. Zhumik
doaj   +1 more source

Japanese Clinical Practice Guidelines for Vascular Anomalies 2017

The Journal of Dermatology, Volume 47, Issue 5, Page e138-e183, May 2020., 2020
Abstract The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety and systematizing treatment, employing evidence‐based medicine techniques and aimed at improvement of the outcomes.
Hidefumi Mimura, Sadanori Akita, Akihiro Fujino, Masatoshi Jinnin, Mine Ozaki, Keigo Osuga, Hiroki Nakaoka, Eiichi Morii, Akira Kuramochi, Yoko Aoki, Yasunori Arai, Noriko Aramaki, Masanori Inoue, Yuki Iwashina, Tadashi Iwanaka, Shigeru Ueno, Akihiro Umezawa, Michio Ozeki, Junko Ochi, Yoshiaki Kinoshita, Masakazu Kurita, Shien Seike, Nobuyuki Takakura, Masataka Takahashi, Takao Tachibana, Kumiko Chuman, Shuji Nagata, Mitsunaga Narushima, Yasunari Niimi, Shunsuke Nosaka, Taiki Nozaki, Kazuki Hashimoto, Ayato Hayashi, Satoshi Hirakawa, Atsuko Fujikawa, Yumiko Hori, Kentaro Matsuoka, Hideki Mori, Yuki Yamamoto, Shunsuke Yuzuriha, Naoaki Rikihisa, Shoji Watanabe, Shinichi Watanabe, Tatsuo Kuroda, Shunsuke Sugawara, Kosuke Ishikawa, Satoru Sasaki   +46 more
wiley   +1 more source

Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report

World Journal of Surgical Oncology, 2011
Kaposiform hemangioendothelioma (KHE) is an uncommon vascular tumor of intermediate malignant potential, usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and Kasabach-Merritt ...
Kulkarni Suyash S, Sethi Shweta, Rekhi Bharat, Jambhekar Nirmala A   +3 more
doaj   +1 more source

How we use angiopoietin-2 in the diagnosis and management of vascular anomalies. [PDF]

Pediatr Blood Cancer
Abstract The diagnosis of vascular anomalies remains challenging due to significant clinical heterogeneity and uncertain etiology. Evaluation using biopsy and/or genetic testing for somatic variants is invasive, expensive, and prone to sampling error. There is great need for noninvasive and easily measured blood laboratory biomarkers that can aid not ...
Engel ER, Le Cras TD, Ricci KW.
europepmc   +2 more sources

The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies

Orphanet Journal of Rare Diseases, 2019
Background Lymphatic anomalies (LAs) include several disorders in which abnormal lymphatic tissue invades the neck, chest, and various organs. Progressive cases may result in lethal outcomes and have proven difficult to treat.
Michio Ozeki, Akifumi Nozawa, Shiho Yasue, Saori Endo, Ryuta Asada, Hiroya Hashimoto, Toshiyuki Fukao   +6 more
doaj   +1 more source