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Gastrointestinal system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
6.10. Chronic diarrhoea may be the presenting symptom ofcystic fibrosis.lactose intolerance.excessive consumption of fruit juices and cordials.intestinal lymphangiectasia.coeliac disease.
Miscellaneous procedures
Published in A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha, Clark’s Procedures in Diagnostic Imaging: A System-Based Approach, 2020
A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha
Findings in lymphoedema include: Delayed transit of activity relative to the normal side in unilateral oedema.Reduced activity non-visualisation of the lymphatic channels.Poorly visualised/reduced number of lymph nodes.Diversion of activity through the skin lymphatics, indicated by dermal backflow.Visualisation of collateral lymphatic vessels.The presence of abnormal deep collateral flow, e.g. visualisation of popliteal nodes.Presence of lymphocoeles/lymphangiectasia.
Confusion on terminology: Primary lymphedema and lymphangioma (lymphatic malformation)
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
The deep forms of lymphangioma/lymphatic malformations include two specific well-defined CVMs: cavernous lymphangiomas (=lymphatic malformations) and cystic lymphangiomas (=lymphatic malformations), which are also known as cystic hygromas.16 Superficial forms of lymphangioma/lymphatic malformations include lymphangioma circumscriptum and acquired lymphangioma/lymphatic malformation, which is also referred to in the literature as lymphangiectasia.14 Although both entities share similar clinical and histological features, the term lymphangioma circumscriptum describes lymphatic vessel dilation due to a LM; whereas, the term lymphangiectasia, or acquired lymphangioma, describes dilated lymphatic vessels of previously normal lymphatics that have become obstructed by an external cause.14, 17
The Histopathological Findings of Patients Who Underwent Blepharoplasty Due to Dermatochalasis
Published in Seminars in Ophthalmology, 2018
Ali Karnaz, Yasemin Aslan Katircioglu, Evin Singar Ozdemir, Pınar Celebli, Sema Hucumenoglu, Firdevs Ornek
Normal lymphatic vessels cannot be easily distinguished owing to their thin endothelial cells and narrow lumens in normally functioning skin.22 Lymphatic vessels that are easily discerned owing to dilation secondary to obstruction or impaired lymphatic flow exhibit certain histological findings such as thinned walls, sparse endothelial cells, and lymph valves.22,23 Therefore, lymphangiectasia is the morphological evidence of lymphostasis.23 We determined the number of lymphangiectatic vessels and maximal dilated lymphangiectatic vessel diameter to assess the presence and severity of lymphostasis. Agliano et al. reported an appareance characterized by lymphatic vessels with increased luminal diameter in the dermatochalasis group.15 They found a significantly greater mean lymphatic luminal diameter in the dermatochalasis group compared to the control group; however, although lymphatic vessel density was greater in the DC group, the difference between the DC and control groups did not reach statistical significance. Nagi et al. found significantly greater lymphatic vessel diameter and number of dilated lymphatic vessels compared to the control group.24 In our study, lymphatic vessel density and lympathic vessel diameter were greater in the DC group (p = 0.01, p = 0.02).
Fetal hydrops – a review and a clinical approach to identifying the cause
Published in Expert Opinion on Orphan Drugs, 2020
Esther Dempsey, Tessa Homfray, John M Simpson, Steve Jeffery, Sahar Mansour, Pia Ostergaard
Homozygous mutations in PIEZO1 cause generalized lymphatic dysplasia of Fotiou in postnatal life and can present prenatally as nonimmune fetal hydrops [113]. The PIEZO1 gene encodes a highly conserved, large transmembrane protein that is a mechanically activated cation channel [114]. Postnatally affected individuals, in addition to widespread lymphedema, may have systemic complications including pulmonary lymphangiectasia, pleural effusions chylothoraces and pericardial effusions [104]. Autosomal dominant gain-of-function mutations in PIEZO1 cause dehydrating hereditary stematocytosis which can also present with a severe fetal hydrops/perinatal edema phenotype [110]
Steroid-resistant protein-losing gastroenteropathy complicated with Sjögren’s syndrome successfully treated with mizoribine
Published in Modern Rheumatology, 2018
Yasumori Izumi, Kenjiro Nakaoka, Masatsugu Kamata, Nozomi Iwanaga, Shunsuke Imadachi, Hirokazu Kurohama, Masahiro Ito, Kiyoshi Migita
The pathogenic mechanism of PLGE associated with rheumatic disease remains unclear. PLGE associated with SLE is thought to be related to capillary hyperpermeability caused by inflammatory cell infiltration, with deposition of immunoglobulins and complement components in vascular endothelial cells of the intestinal mucosa [10]. Deposits of IgG, C3, C1q, and fibrinogen were detected on submucosal capillaries of biopsy samples without lymphangiectasia in patients with PLGE associated with SS [7]. The biopsy of the patient described in this report showed inflammatory cell infiltration without lymphangiectasia in the mucosa, suggesting that the condition was induced by increased capillary permeability.