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Clinical features and current management experience in Gorham-Stout disease: a systematic review.

BACKGROUND: Gorham-Stout disease (GSD) is a rare complex lymphatic malformation. Since its initial description in 1838, only approximately 400 patients have been documented. There is currently no consensus on the diagnostic criteria or treatment options for GSD. The objective of this study was to review the clinical characteristics of patients with GSD and determine the current diagnostic and treatment models. METHODS: A comprehensive search of the PubMed, Web of Science, Embase, and Cochrane Library databases was conducted to identify all relevant literature on GSD published over the decade

Yellow nail syndrome: a review.

Yellow nail syndrome (YNS; OMIM 153300, ORPHA662) is a very rare disorder that almost always occurs after 50 years of age but a juvenile or familial form has also been observed. YNS is diagnosed based on a triad associating yellow nail discoloration, pulmonary manifestations (chronic cough, bronchiectasia, pleural effusion) and lower limb lymphedema. Chronic sinusitis is frequently associated with the triad. YNS etiology remains unknown but a role of lymphatic impairment is usually evoked. YNS is more frequently isolated but may be associated in rare cases with autoimmune diseases, other clini

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… Four patients’ limb lymphangiograms showed lymphatic abnormalities, such as tortuous, dilated or hypoplastic vessels, which the authors considered suggestive of lymphatic dysfunction or defective lymph drainage being responsible for YNS. … Four YNS patients underwent lower limb direct lymphangiography, less used at present, but lymphatic abnormalities were noted only in the patient with severe lymphedema. …

Molecular events in the jaw vascular unit: A traditional review of the mechanisms involved in inflammatory jaw bone diseases.

Inflammatory jaw bone diseases are common in stomatology, including periodontitis, peri-implantitis, medication-related osteonecrosis of the jaw, radiation osteomyelitis of the jaw, age-related osteoporosis, and other specific infections. These diseases may lead to tooth loss and maxillofacial deformities, severely affecting patients' quality of life. Over the years, the reconstruction of jaw bone deficiency caused by inflammatory diseases has emerged as a medical and socioeconomic challenge. Therefore, exploring the pathogenesis of inflammatory diseases associated with jaw bones is crucial fo

Гистологическая оценка образцов гистерэктомии после эндометрической абляции в Новосере у пациентов с нетипичной эндомией гиперплазии или эндометрической карциномой.

ОКРУЖАЮЩАЯ СРЕДА: Для пациентов с тяжелым менструальным кровотечением (HMB) разрешается лечение с помощью эндомеальной абляции " Новасуре " без признаков злокачественных или предмалярных повреждений. В этом анализе рассматривается редкая, но клинически значимая ситуация, в которой эндометричная карцинома (ЭК) или атипичная гиперплазия (АЭГ) выявляется гистологически после эндомеальной абляции у доменопаузальных пациентов. ЦЕЛЬ: гистологическая оценка образцов гистерэктомии с корреляцией с клиническими параметрами у пациентов, страдающих гистерэктомией после случайного гистологическог

Diagnosis and treatment of cystic lung disease.

Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/ serologic markers, together with treatment modalities

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… Although the origin of LAM cells is unknown, the pelvis, especially the uterus, is presumed to be the source because axial lymphatic abnormalities in the pelvis are most common and those distant from the pelvis less frequent. …

Vascular endothelial growth factor signaling in health and disease: from molecular mechanisms to therapeutic perspectives.

Vascular endothelial growth factor (VEGF) signaling is a critical regulator of vasculogenesis, angiogenesis, and lymphangiogenesis, processes that are vital for the development of vascular and lymphatic systems, tissue repair, and the maintenance of homeostasis. VEGF ligands and their receptors orchestrate endothelial cell proliferation, migration, and survival, playing a pivotal role in dynamic vascular remodeling. Dysregulated VEGF signaling drives diverse pathological conditions, including tumor angiogenesis, cardiovascular diseases, and ocular disorders. Excessive VEGF activity promotes tu

Exploring the genetic alterations of Gorham-Stout disease.

The "vanishing bone disease" or Gorham-Stout disease (GSD) is a very rare disorder characterized by massive lymphatic and angiomatous proliferation accompanied by progressive osteolysis, without the deposition of new bone matrix. Because of its rare and complex clinical features, diagnosis is challenging and its etiopathogenesis is not completely known; the genetic basis of GSD has been hypothesized and different mutations have been reported in patients. Our review aims to describe all these genetic alterations found in GSD patients and their association with clinical features. The identificat

A new therapeutic approach in Gorham-Stout disease: a case report.

BackgroundGorham-Stout disease is a rare condition of unknown prevalence and unknown exact cause. Its pathogenesis is based on enhanced osteoclastic activity leading to bone resorption and bone replacement by distended lymphatic vessels. Because of its rarity and a various range of symptoms the disease may give, diagnosis is challenging and a strong index of suspicion is required. Although it is a benign condition, the prognosis may be unpredictable. The treatment options suggested so far are limited, and every case should be provided with the best individual approach. Herein, we present a cas

Иммунный код мозга: декодирование антигенной презентации на менингеальном-глимфатичном интерфейсе в неврологических расстройствах.

Классический взгляд на иммунную привилегию центральной нервной системы (СНС) эволюционировал от изоляции к регулируемому перекрестному разговору. Перенос церебральных цереброспинных флюидов и менингетических лимфатических сосудов (mLVs) переносят молекулы, полученные из головного мозга, на эмунизированные пограничные ткани и лимфатические узлы шейки матки, где антигенные клетки (APC) декодируют этот молекулярный дренаж в адаптивный иммунитет. Мы настраиваем эту последовательность как код мозга, в котором личность, время, маршрут и клеточный проводник экспортированных антигенов коллективно дикт

Immunodeficiencies in Adults: Key Considerations for Diagnosis and Management.

Immunodeficiencies in adults are increasingly recognized yet often remain underdiagnosed, leading to significant morbidity from recurrent infections, autoimmunity, and malignancy. Both primary immunodeficiencies (PIDs), now known as inborn errors of immunity (IEI), and secondary immunodeficiencies (SIDs) contribute to immune dysfunction in adults. Although SIDs are more common in adults due to factors like medications, malignancies, metabolic disorders, chronic conditions, and protein-losing conditions, IEI-particularly common variable immunodeficiency (CVID)-can also manifest in adulthood wit

Multiple cystic lung disease.

Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main dise

Tumor microenvironment targeted nano-drug delivery systems for multidrug resistant tumor therapy.

In recent years, nano-drug delivery systems (Nano-DDS) that target the tumor microenvironment (TME) to overcome multidrug resistance (MDR) have become a research hotspot in the field of cancer therapy. By precisely targeting the TME and regulating its unique pathological features, such as hypoxia, weakly acidic pH, and abnormally expressed proteins, etc., these Nano-DDS enable effective delivery of therapeutic agents and reversal of MDR. This scientific research community is increasing its investment in the development of diversified systems and exploring their anti-drug resistance potential.

Targeting endoplasmic reticulum stress-induced lymphatic dysfunction for mitigating bisphosphonate-related osteonecrosis.

BACKGROUND: Bisphosphonates (BPs) are the first-line treatment to stop bone resorption in diseases, including osteoporosis, Paget's disease, multiple myeloma and bone metastases of cancer. However, BPs-related osteonecrosis of the jaw (BRONJ), characterized by local inflammation and jawbone necrosis, is a severe intractable complication. The cumulative inflammatory burden often accompanies impaired lymphatic drainage, but its specific impact on BRONJ and the underlying mechanisms remain unclear. METHODS: The mouse BRONJ model was established to assess the integrity and drainage function of ly

Lenalidomide treatment of Japanese patients with myelodysplastic syndromes with 5q deletion: a post-marketing surveillance study.

Lenalidomide was approved in Japan for the treatment of patients with myelodysplastic syndromes associated with a 5q deletion (del 5q-MDS) in August 2010. A post-marketing surveillance (PMS) study enrolled 173 patients with del 5q-MDS who started lenalidomide treatment between August 2010 and September 2011 (mean ± standard deviation [SD] age 72.4 ± 9.0 years) and observed for up to 6 cycles or 6 months. Adverse drug reactions (ADRs) and serious ADRs were reported in 78.0% and 50.9% of patients. The most commonly observed ADRs were thrombocytopenia or platelet count decreased (46.2%), neutrope

A Rare Case of Papillary Thyroid Carcinoma Metastasizing to the Spine.

Papillary thyroid carcinoma (PTC) typically presents with an excellent prognosis. Distant metastasis, particularly to the spine, is rare and associated with poorer outcomes. This report highlights the importance of considering metastatic PTC in the differential diagnosis of persistent back pain in patients with thyroid abnormalities. A 61-year-old female presented with progressive lower back pain and an enlarging neck mass. Evaluation revealed a Thyroid Imaging Reporting and Data System, Category 4 (TIRADS-4) thyroid nodule and a lytic lesion in the T10 vertebra. Biopsy confirmed papillary thy

Доклад по делу: Выяснение роли генетических аномалий в миэломатном плевральном эффюляции: случай с несколькими миеломами типа IgG Lambda.

A 73-year-old Мужчина с многократной миеломой, с постепенно ухудшающейся болью в левом плече, которая не реагировала на консервативное управление. После проведения всеобъемлющих диагностических процедур, включая съемку и генетическое тестирование, у него был диагностирован множественный миэлома типа IgG Lambda, осложненный развитием миэломатного плеврального эффюля (MPE). Лечение пациента состояло из сочетания химиотерапии PDD, включая Бортезомиб, Доксорубицин и Дексаметазон, наряду с адъюнктивными терапиями, направленными на контроль плевральной эфлуции. Оценка после обработки выявила разреше

СРП 2013

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Impact of preoperative anemia on outcomes in patients undergoing curative resection for gastric cancer: a single-institution retrospective analysis of 2163 Chinese patients.

We sought to evaluate whether preoperative anemia was an important determinant of survival in gastric cancer (GC). A single institution cohort of 2163 GC patients who underwent curative resection were retrospectively analyzed. Anemia was defined as a preoperative hemoglobin level <120 g/L in males and <110 g/L in females. Overall survival (OS) was analyzed using the Kaplan-Meier method, and a multivariate Cox proportional hazards model was performed to identify the independent prognostic factor. Anemic patients had a poorer OS compared with nonanemic patients after resection for tumor-nodes-me

The regulatory networks and mechanisms of bone microenvironment in tumorigenesis and metastasis.

Bone tumors, encompassing heterogeneous primary and metastatic lesions, are driven in their initiation, progression, and metastasis by dysregulation of the bone microenvironment (BME). Clinically manifested by localized pain, pathological fractures, and neurological deficits, these malignancies substantially threaten patient survival. Radiographic patterns (osteolytic, osteoblastic, mixed) directly reflect pathogenic BME-tumor interactions, particularly involving osteoblast-osteoclast imbalance. The BME-a specialized niche of bone-resident cells (osteocytes, osteoblasts, osteoclasts), immune c

Multimodal imaging diagnosis of multiple myeloma with renal involvement: a case report.

BackgroundExtramedullary renal involvement secondary to multiple myeloma (MM) is extremely rare and prone to preoperative misdiagnosis. Its clinical and imaging manifestations have not been systematically summarized, leading to frequent preoperative misdiagnosis. To summarize the multimodal imaging characteristics and clinical diagnostic value of MM with renal involvement through a typical case report, so as to improve the understanding and early recognition rate of this disease.Case presentationA 67-year-old male patient with left lumbodorsal pain was examined by multimodal imaging. Ultrasoun