Results 11 to 20 of about 346 (122)

Detection of NRAS mutation in cell-free DNA biological fluids from patients with kaposiform lymphangiomatosis [PDF]

Orphanet Journal of Rare Diseases, 2019
Background Kaposiform lymphangiomatosis (KLA) has recently been distinguished as a novel subtype of generalized lymphatic anomaly (GLA) with foci of spindle endothelial cells.
Michio Ozeki, Yoko Aoki, Akifumi Nozawa, Shiho Yasue, Saori Endo, Yumiko Hori, Kentaro Matsuoka, Tetsuya Niihori, Ryo Funayama, Matsuyuki Shirota, Keiko Nakayama, Toshiyuki Fukao   +11 more
doaj   +2 more sources

Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature. [PDF]

BMJ Case Rep, 2022
Kaposiform lymphangiomatosis (KLA) is a rare clinicopathological entity among lymphatic anomalies. The main involved sites are the mediastinum and the lungs but the disease can also affect multiple extrathoracic organs. Little is known about the pathophysiology, the natural history, the treatment response and the long-term outcome of this disorder. KLA
Fedidat R, Elia A, Hershko A, Fridlender Z.   +3 more
europepmc   +3 more sources

Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. [PDF]

J Pediatr, 2014
To describe the clinical and imaging characteristics of a new lymphatic disorder with a unique histological pattern and poor prognosis.An observational, retrospective study identified and characterized 20 patients with distinct lymphatic histopathology referred to the Vascular Anomalies Center at Boston Children's Hospital between 1995 and 2011.The ...
Croteau SE, Kozakewich HP, Perez-Atayde AR, Fishman SJ, Alomari AI, Chaudry G, Mulliken JB, Trenor CC.   +7 more
europepmc   +4 more sources

Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis. [PDF]

Pediatr Radiol, 2021
Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection.
Soderlund KA, Mamlouk MD, Shah VN, Roland JL, Dillon WP.   +4 more
europepmc   +6 more sources

A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. [PDF]

Genet Med, 2019
Kaposiform lymphangiomatosis (KLA) is a rare, frequently aggressive, systemic disorder of the lymphatic vasculature, occurring primarily in children. Even with multimodal treatments, KLA has a poor prognosis and high mortality rate secondary to coagulopathy, effusions, and systemic involvement. We hypothesized that, as has recently been found for other
Barclay SF, Inman KW, Luks VL, McIntyre JB, Al-Ibraheemi A, Church AJ, Perez-Atayde AR, Mangray S, Jeng M, Kreimer SR, Walker L, Fishman SJ, Alomari AI, Chaudry G, Trenor Iii CC, Adams D, Kozakewich HPW, Kurek KC.   +17 more
europepmc   +4 more sources

Primary splenic involvement in kaposiform lymphangiomatosis: A case report. [PDF]

Int J Surg Case Rep
Kaposiform Lymphangiomatosis (KLA) is a rare, pathologically distinct lymphatic anomaly characterized by abnormal lymphatic channel malformation and a high mortality rate. Previous studies describe primary thoracic involvement.A previously healthy eighteen-month-old female presented to the pediatric emergency department with sudden onset of petechiae ...
Covil E, Gates R.
europepmc   +3 more sources

Signaling pathways and inhibitors of cells from patients with kaposiform lymphangiomatosis. [PDF]

Pediatr Blood Cancer, 2019
AbstractBackgroundKaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly with significant morbidity and mortality. KLA is characterized by diffuse multifocal lesions comprised of focal areas of “kaposiform” spindled cells accompanying malformed lymphatic channels.
Boscolo E, Pastura P, Glaser K, Goines J, Hammill AM, Adams DM, Dickie P, Hsi Dickie B, Le Cras TD.   +8 more
europepmc   +4 more sources

Case Report: Life-threatening Kasabach–Merritt phenomenon in a 2-month-old child [PDF]

Frontiers in Pediatrics
We present a rare case of a 2-month-old girl, born at term, who was admitted to the pediatric intensive care unit for fussiness, increasing abdominal distension, and intermittent diarrhea for one week. She was found to be hypertensive, coagulopathic, and
Ashley V. Wong Grossman, Juan Pablo Forero, Jeffrey S. Yu, Megan M. Gilbert, Kanwaljeet J. S. Anand   +4 more
doaj   +2 more sources

Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels. [PDF]

Pediatr Blood Cancer, 2020
AbstractKaposiform lymphangiomatosis (KLA) is a rare, life‐threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin‐2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status.
Crane J, Manfredo J, Boscolo E, Coyan M, Takemoto C, Itkin M, Adams DM, Le Cras TD.   +7 more
europepmc   +4 more sources

Trametinib normalizes angiopoietin-2 levels and successfully treats kaposiform lymphangiomatosis. [PDF]

J Vasc Anom (Phila)
Kaposiform lymphangiomatosis (KLA) is a complex lymphatic anomaly with high morbidity and mortality. Angiopoietin-2 (Ang-2) levels are elevated in the blood of patients with KLA and can be used to aid in diagnosis and monitor treatment response.
McDaniel CG, Merkle S, Lacuna S, Cooper S, Courtemanche R, Nguyen T, Le Cras TD.   +6 more
europepmc   +2 more sources