2,037 results match your criteria Lymphangiectasia

Pulmonary lymphangiectasia in myotubular myopathy: a novel unrecognized association?

Neuromuscul Disord 2022 May 2. Epub 2022 May 2.

Division of Neonatology, Department of Pediatrics, McGill University Health Centre, Montreal, Quebec, Canada.

Chylothorax has been reported in rare cases of X-linked myotubular myopathy, but the pathophysiology of this association is not fully understood. We report a case of a neonate presenting prenatally with hydrops and chylothorax. The patient died at 17 days of life due to respiratory failure secondary to severe pulmonary hypertension. Read More

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Hypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature.

BMC Gastroenterol 2022 May 15;22(1):246. Epub 2022 May 15.

Department of Gastroenterology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

Background: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.

Case Presentation: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Read More

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Malabsorption Spectrums in India.

J Assoc Physicians India 2022 Apr;70(4):11-12

Kakatiya Medical College, Kakatiya.

Among the causes of malabsorption, tropical sprue is one of the leading cause.Several reports indicating that celiac disease, now being recognised more frequently.

Material: 94 patients, aged more than 12 years, presenting with Chronic diarrhoea and malabsorption syndrome were analyzed by clinical presentation, endoscopic and histopathological examination. Read More

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Implications of Corneal Refractive Surgery in Patients with Fabry Disease.

Ophthalmol Ther 2022 Jun 16;11(3):925-929. Epub 2022 Apr 16.

Hoopes Vision Research Center, Hoopes Vision, 11820 S. State Street Suite #200, Draper, UT, 84020, USA.

Fabry disease is an X-linked lysosomal storage disorder involving abnormal metabolism of glycosphingolipids, resulting in a range of multisystem organ dysfunction and ocular manifestations. Reports of laser-assisted in situ keratomileuses (LASIK) and photorefractive keratectomy (PRK) are lacking in this patient population. Proceeding with corneal refractive surgery in patients with Fabry disease raises concerns regarding the pre-existing corneal manifestations, reduced mesopic visual acuity, the potential for conjunctival lymphangiectasia, and predisposition to dry eye syndrome. Read More

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Renal lymphangiectasia in pediatric population: case series and review of literature.

Ann Saudi Med 2022 Mar-Apr;42(2):139-144. Epub 2022 Apr 7.

From the Department of Surgery, Alfaisal University, Riyadh, Saudi Arabia.

Renal lymphangiectasia is a rare disorder where perirenal, parapelvic, and/or intra-renal lymphatics are dilated. The clinical presentation of renal lymphangiectasia can range from asymptomatic to renal failure. Ultrasound, computed tomography and magnetic resonance imaging have been used for the diagnosis. Read More

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Evaluation of the degree and distribution of lymphangiectasia in full-thickness canine small intestinal specimens diagnosed with lymphoplasmacytic enteritis and granulomatous lymphangitis.

J Vet Med Sci 2022 Apr 14;84(4):566-573. Epub 2022 Mar 14.

Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Tokyo, Japan.

Intestinal lymphangiectasia (IL) is often observed in dogs with chronic small intestinal diseases. Hypoplasia of the lymphatic vessel due to decreased lymphangiogenesis, which has been suggested in human idiopathic IL, may contribute to the pathogenesis of canine IL. This study aimed to evaluate the diameter and number of lymphatic vessels in full-thickness small intestinal specimens of dogs with IL. Read More

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Primary intestinal lymphangiectasia: a rare disease as a cause of protein-losing enteropathy.

Rev Esp Enferm Dig 2022 Mar 4. Epub 2022 Mar 4.

Aparato Digestivo, Complejo Asistencial Universitario de León, España.

Primary intestinal lymphangiectasia is a rare disorder associated with protein-losing enteropathy. The main manifestations are those resulting from hypoalbuminemia. Diagnosis requires the typical endoscopic image of intestinal lymphangiectasia and increased 24-hour fecal alpha-1-antitrypsin clearance. Read More

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CCBE1 in Cardiac Development and Disease.

Front Genet 2022 9;13:836694. Epub 2022 Feb 9.

Stem Cells and Development Laboratory, CEDOC, Chronic Diseases Research Centre, NOVA Medical School, Universidade Nova de Lisboa, Lisboa, Portugal.

The collagen- and calcium-binding EGF-like domains 1 (CCBE1) is a secreted protein extensively described as indispensable for lymphangiogenesis during development enhancing VEGF-C signaling. In human patients, mutations in have been found to cause Hennekam syndrome, an inherited disease characterized by malformation of the lymphatic system that presents a wide variety of symptoms such as primary lymphedema, lymphangiectasia, and heart defects. Importantly, over the last decade, an essential role for CCBE1 during heart development is being uncovered. Read More

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February 2022

A rare case of intestinal lymphangiectasia induced by pazopanib.

Int Cancer Conf J 2022 Jan 1;11(1):87-90. Epub 2021 Dec 1.

Department of Urology, Kyoto University Graduate School of Medicine, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto, 606-8507 Japan.

A 62-year-old man underwent left radical nephrectomy for left renal cell carcinoma at our hospital in 1999. At the age of 79 years, he was diagnosed with intra-abdominal disseminations, lung metastases, pancreas metastases, and bilateral femoral muscle metastases during a routine follow-up computed tomography scan. The patient began treatment with pazopanib. Read More

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January 2022

Protein-losing enteropathy caused by a jejunal ulcer after an internal hernia in Petersen's space: A case report.

World J Clin Cases 2022 Jan;10(1):323-330

Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo 113-8603, Japan.

Background: The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery. Of particular concern are internal hernias occurring in Petersen's space, a space that is surgically created after treatment for gastric cancer and obesity. These hernias cause devastating sequelae, such as massive intestinal necrosis, fatal Roux limb necrosis, and superior mesenteric vein thrombus. Read More

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January 2022

Acquired scrotal lymphangiectasia, a long-term sequela of penile carcinoma: diagnosis an enigma.

BMJ Case Rep 2022 Jan 12;15(1). Epub 2022 Jan 12.

Department of Urology, AIIMS Rishikesh, Rishikesh, Uttarakhand, India.

Acquired lymphangiectasia is an unique entity encountered by an urologist which is marked by occlusion of lymphatics associated with abnormal permanent dilation of cutaneous lymphatics. There is presence of thin-walled ectatic vessels in the superficial and mid dermis, which develops later in life. Trauma, infection, radiation or surgery are important causes of this rare aetiology. Read More

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January 2022

Duodenal Lymphangiectasia.

Am J Gastroenterol 2022 Apr;117(4):533

Department of Gastroenterology and Digestive Oncology, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.

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Long-term efficacy of lymphovenous anastomosis for the treatment of retroperitoneal lymphangiectasia: A case report and a review of literature.

Microsurgery 2022 Jan 11. Epub 2022 Jan 11.

Department of Plastic Surgery, Cleveland Clinic, Cleveland, Ohio, USA.

Lymphovenous anastomosis (LVA) represents an alternative treatment for retroperitoneal lymphangiectasia. In contrast to sclerotherapy or excision, which may risk lymphatic obstruction and subsequent lymphedema, LVA preserves existing lymphatic architecture and transit. This report shows long-term efficacy of LVA for functional decompression of a symptomatic pathologically dilatated retroperitoneal lymphatics. Read More

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January 2022

Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection.

Echocardiography 2022 02 6;39(2):375-380. Epub 2022 Jan 6.

Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan.

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. Read More

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February 2022

Conjunctival lymphangiectasia and retinal angiopathy in hereditary transthyretin amyloidosis.

Int J Retina Vitreous 2022 Jan 6;8(1). Epub 2022 Jan 6.

Department of Ophthalmology, Western University, London, ON, Canada.

Background: Hereditary transthyretin amyloidosis (ATTR amyloidosis) is a rare condition where a mutation in the transthyretin gene leads to systemic deposition of amyloid. The manifestations and prognosis of ATTR amyloidosis depends on the specific ATTR mutation, with over 100 mutations reported in the literature. The manifestations of many rare forms of ATTR amyloidosis have not been well described, particularly the late-onset ophthalmic findings. Read More

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January 2022

Role of enteroscopy in the diagnosis of whipple's disease.

Acta Gastroenterol Belg 2021 Oct-Dec;84(4):669-671

Department of Gastroenterology, Centro Hospitalar Universitário de São João, Porto, Portugal.

Whipple's disease is a rare chronic systemic infection caused by Tropheryma whipplei. The widespread infection by this rod is responsible for the protean clinical manifestations of the disease, although its classical form is notable for the prevalence of abdominal symptoms such as chronic diarrhea and abdominal pain. Whitish-yellow patches, suggestive of lymphangiectasia, are typically observed in the duodenum during upper endoscopy. Read More

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December 2021

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.

Medicina (Kaunas) 2021 Nov 29;57(12). Epub 2021 Nov 29.

Department of Pulmonology, Medical Academy, Lithuanian University of Health Sciences, 44307 Kaunas, Lithuania.

Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. Read More

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November 2021

Redefining WILD syndrome: a primary lymphatic dysplasia with congenital multisegmental lymphoedema, cutaneous lymphovascular malformation, CD4 lymphopaenia and warts.

J Med Genet 2021 Dec 16. Epub 2021 Dec 16.

Infection Care Group, St George's University Hospitals NHS Foundation Trust, London, UK.

Background: Primary lymphoedema (PL) syndromes are increasingly recognised as presentations of complex genetic disease, with at least 20 identified causative genes. Recognition of clinical patterns is key to diagnosis, research and therapeutics. The defining criteria for one such clinical syndrome, 'WILD syndrome' (arts, mmunodeficiency, ymphoedema and anogenital ysplasia), have previously depended on a single case report. Read More

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December 2021

A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma.

BMC Gastroenterol 2021 Dec 11;21(1):461. Epub 2021 Dec 11.

Department of Gastroenterology, Qingdao Municipal Hospital, Jiaozhou Road 1#, Qingdao, 266071, People's Republic of China.

Background: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma.

Case Presentation: A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Read More

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December 2021

Malignancy-associated acquired vulvar lymphangioma circumscriptum: A clinicopathologic study of 71 cases.

J Cutan Pathol 2022 May 21;49(5):426-433. Epub 2021 Dec 21.

Department of Pathology and Laboratory Medicine, University of Kansas Hospital, Kansas City, Kansas, USA.

Background: Acquired lymphangioma circumscriptum of the vulva is rare and can occur subsequent to malignancies of the anogenital and pelvic region. We sought to investigate the clinicopathologic characteristics of malignancy-associated acquired vulvar lymphangioma circumscriptum (AVLC).

Methods: We identified all cases of AVLC within our institution with history of prior malignancy between 2005 and 2021. Read More

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Histopathology of intestinal villi in neonatal and paediatric age: main features with clinical correlation - Part I.

Pathologica 2022 Feb 2;114(1):12-21. Epub 2021 Dec 2.

Institute of Pathology, Spedali Civili di Brescia, Brescia, Italy.

The neonatal and paediatric spectrum of small bowel disorders encompass a wide variety of conditions, ranging from food allergies to life-threatening surgical emergencies or life-long medical conditions and, as such, it comes with a whole set of diagnostic challenges for the non-paediatric pathologist. Histologic examination is a cornerstone of diagnosis in a large number of diseases and may still provide important diagnostic clues in the appropriate clinical context. In this review, divided in two sections, we aim to provide a comprehensive histopathological summary of paediatric small bowel alteration and their differential diagnoses with a reference to the main clinical aspects required for appropriate interpretation. Read More

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February 2022

Association between intestinal lymphangiectasia and expression of inducible nitric oxide synthase in dogs with lymphoplasmacytic enteritis.

J Vet Med Sci 2022 Jan 1;84(1):20-24. Epub 2021 Dec 1.

Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, Japan.

Intestinal lymphangiectasia (IL) is a common complication in dogs. Since nitric oxide (NO) is known to relax the lymphatic vessel, we evaluated inducible NO synthase (iNOS) expression using immunohistochemistry in 13 dogs with lymphoplasmacytic enteritis (LPE) with or without IL. The duodenal iNOS expressing cells were significantly increased in dogs with IL-negative or IL-positive LPE dogs (P=0. Read More

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January 2022

Chylothorax: A Stepwise Approach to Diagnosis and Treatment.

Neonatal Netw 2021 Nov;40(6):386-392

Chylothorax, a lymphatic flow disorder characterized by an abnormal circulation of lymph fluid into the pleural cavity, is the most common cause of pleural effusions during the neonatal period. This condition affects 1/15,000 neonates every year. Affected neonates often manifest with respiratory distress, electrolyte imbalances, sepsis, and even immunodeficiencies. Read More

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November 2021

Protein-losing enteropathy caused by disseminated Mycobacterium avium complex infection in a patient receiving antiretroviral therapy: an autopsy case report.

AIDS Res Ther 2021 11 29;18(1):90. Epub 2021 Nov 29.

Department of Infectious Diseases, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-ku, Osaka, Osaka, 534-0021, Japan.

Background: Disseminated Mycobacterium avium complex infection is an important indicator of acquired immunodeficiency syndrome (AIDS) in patients with advanced human immunodeficiency virus (HIV) infection. Effective antiretroviral therapy has dramatically reduced the incidence of and mortality due to HIV infection, although drug resistance and poor medication adherence continue to increase the risk of disseminated M. avium complex infection. Read More

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November 2021

Contribution of fetal magnetic resonance imaging in fetuses with congenital heart disease.

Pediatr Radiol 2022 Mar 29;52(3):513-526. Epub 2021 Nov 29.

Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Road, Phoenix, AZ, 85016, USA.

Background: Increasing evidence supports an association among congenital heart disease (CHD), structural brain lesions on neuroimaging, and increased risk of neurodevelopmental delay and other structural anomalies. Fetal MRI has been found to be effective in demonstrating fetal structural and developmental abnormalities.

Objective: To determine the contribution of fetal MRI to identifying cardiovascular and non-cardiovascular anomalies in fetuses with CHD compared to prenatal US and fetal echocardiography. Read More

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Efficacy of laser CO treatment for refractory lymphedema secondary to cancer treatments.

Lasers Surg Med 2022 03 27;54(3):337-341. Epub 2021 Nov 27.

Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Lymphedema is a frequent debilitating condition among cancer patients. Daily supportive treatment may be necessary without long-term improvement. We describe two cases with chronic refractory lymphedema treated with fractional 10,600 nm CO laser. Read More

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Papulovesicular lesions near the right breast.

JAAD Case Rep 2021 Dec 24;18:64-66. Epub 2021 Aug 24.

Department of Dermatology, University of Texas at Houston Health Science Center, Houston, Texas.

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December 2021

Bilateral Renal Lymphangiectasia with No Significant Morbidity for Over 25 Years: A Case Report.

Am J Case Rep 2021 Nov 8;22:e933934. Epub 2021 Nov 8.

Department of Urology, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.

BACKGROUND Renal lymphangiectasia is a rare benign disorder with unknown pathophysiology. Renal lymphangiectasia can affect both males and females with no known age predilection. Its diagnosis can be accomplished with radiological images and confirmed by aspiration of lymphatic fluid in certain cases. Read More

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November 2021

Congenital interstitial lung diseases: What the anesthesiologist needs to know.

Paediatr Anaesth 2022 Feb 11;32(2):138-147. Epub 2021 Nov 11.

Clinique d'Anesthésie pédiatrique, Hôpital Jeanne de Flandre, CHU de Lille, Lille, France.

Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Read More

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February 2022