Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Saturday, June 30, 2007

Is double-balloon enteroscopy an accurate method to diagnose small-bowel disorders?

Is double-balloon enteroscopy an accurate method to diagnose small-bowel disorders?

Surg Endosc. 2007 Jun 26

Safatle-Ribeiro AV, Kuga R, Ishida R, Furuya C, Ribeiro U, Cecconello I, Ishioka S, Sakai P. Gastroenterology, University of São Paulo, São Paulo, SP, Brazil,

Keywords: Enteroscopy - Double-balloon enteroscopy - Small intestine endoscopy

BACKGROUND AND STUDY AIMS: The aim of this study was to analyze the contribution of the double-balloon enteroscopy (DBE) for diagnosis of the small bowel disorders.

PATIENTS AND METHODS: Forty-four patients (20 women, 24 men; mean age 53.5 years-old, range 21-89 years) with chronic gastrointestinal bleeding, diarrhea, polyposis, weight-loss, Roux-en-Y surgery, and other indications underwent DBE.

RESULTS: Twenty patients had occult or obscure gastrointestinal bleeding. The source of bleeding was identified in 15/20 (75%): multiple angiodysplasias in four, arterial-venous malformation beyond the ligament of Treitz in two that could be treated with injection successfully. Other diagnoses included: duodenal adenocarcinoma, jejunal tuberculosis, erosions and ulcer of the jejunum. Of 24 patients with other indications, the diagnosis could be achieved in 18 of them (75%), including: two lymphomas, plasmocytoma, Gardner's syndrome, Peutz-Jeghers' syndrome, familial adenomatous polyposis, Behçet's disease, jejunal submucosal lesion, lymphangiectasia due to blastomycosis and unspecific chronic jejunitis. Of three cases with Roux-en-Y reconstruction, two underwent DBE in order to perform biopsies of the excluded duodenum. Additionally, two patients underwent DBE to exclude Crohn's disease and lymphoma of the small bowel. The mean length of small bowel examination was 240 +/- 50 cm during a single approach. The diagnostic yield was 75% (33/44 cases) and therapeutic yield was 63.6%. No major complications were observed, only minor complication such as sore throat in 4/44 (9.1%).

CONCLUSIONS: 1. DBE is a safe and and accurate method to diagnose small bowel disorders; 2. this method permits chromoscopy, biopsies and treatment of the lesions.

Springer Link

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Sunday, June 24, 2007

Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy.

Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy.

Neonatology. 2007

Akcakus M, Koklu E, Bilgin M, Kurtoglu S, Altunay L, Canpolat M, Budak N.
Division of Neonatology, Department of Paediatrics, School of Medicine, Erciyes University, Kayseri, Turkey.

Author Contacts
Esad Koklu, MDDivision of Neonatology, Department of PaediatricsSchool of Medicine, Erciyes UniversityTR-38039 Kayseri (Turkey)Tel. +90 352 437 4937, Fax +90 352 437 5825, E-Mail

Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. Treatments for lymphangiectasia have included corticosteroids for patients with primary inflammatory conditions, dietary modifications, surgical resection for isolated lesions, octreotide, antiplasmin therapy and fibrin glue pleurodesis. However, there is no experience with pleurodesis by autologous blood therapy in the literature. We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of life and improved with pleurodesis by autologous blood therapy.


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Monday, June 18, 2007

Acquired lymphangiectases and breast cancer

Acquired lymphangiectases and breast cancer
Actas Dermosifiliogr. 2007 Jun

Valdés F, Peteiro C, Toribio J.
Unidad de Dermatología. Hospital da Costa. Burela, Lugo. Spain.

Acquired lymphangiectases represent superficial lymphatic dilatations caused by a wide range of processes. Many cases reported in the literature develop in patients with upper limb lymphedema secondary to mastectomy, radiotherapy, keloids or scleroderma. Clinically they consist of traslucent vesicles in a chronic lymphedematous area. Histologically they are characterized by the presence of dilated spaces with flattened endotelial cells in the papillary dermis. All these cases have a good prognosis and there have not been any reports of malignant transformation. We describe a 67-year-old woman that showed multiple papules along her left upper limb associated with lymphedema. She had undergone a mastectomy followed by radiotherapy fourteen years before due to a breast cancer. Laboratory and radiological exams were within normal limits. The cutaneous lesions showed characteristic clinical and histological features of lymphangiectases and they progressively resolve in several weeks without any treatment.

Actos dermo-sifiliograficas

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Sunday, June 10, 2007

Chronic interstitial lung disease in children

Chronic interstitial lung disease in children
J Pediatr (Rio J). 2007 May/June

Paiva MA, Amaral SM.

Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil.

OBJECTIVES: To describe clinical and diagnostic features and the results of therapeutic conduct in a group of pediatric patients with chronic interstitial lung disease.

METHODS: A retrospective study of 25 immunocompetent patients, aged 2 months to 17 years, with chronic interstitial lung disease, admitted to the Pediatric Pulmonary Section, Department of Pediatrics, Hospital dos Servidores do Estado, over a 20-year period (1984-2004). A routine protocol for persistent chronic pneumonias was used and the patients with interstitial lung disease were selected. Clinical, laboratory and imaging data were analyzed.

RESULTS: Twenty-five patients were diagnosed with chronic interstitial lung disease, 13 were aged less than 2 years and 17 were male. Diagnoses were made based on history, physical examination and routine tests in one case, based on more complex tests in three cases and based on the results of invasive tests in 21 cases (20 by lung biopsy and one by bronchoalveolar lavage). Except for one patient with pulmonary lymphangiectasia, the long-term treatment (1 to 7 years) consisted of corticosteroid, in six cases associated with hydroxychloroquine. Four patients required home oxygen therapy. The authors followed the patients in the outpatient department (6 a 8 visits/year). Patient outcome was: good (15); regular, with mild sequelae (4); and poor, with severe sequelae (3). One patient was lost in the follow-up period and two died.

CONCLUSIONS: Chronic interstitial lung diseases in children are a group of rare pulmonary disorders, but a relevant one because of the possible progression to pulmonary fibrosis. Early diagnosis and a long-term, specialized treatment and follow-up are important for the patient outcome. Pediatricians should be aware of these diseases because in many cases diagnosis and treatment are overlooked.

PMID: 17551654 [
PubMed - as supplied by publisher]

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Saturday, June 02, 2007

Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis.

Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis.
Ann Allergy Asthma Immunol. 2007 May

Cole SL, Ledford DK, Lockey RF, Daas A, Kooper J.
Department of Medicine, Division of Allergy and Immunology, University of South Florida College of Medicine, James A. Haley Veterans' Hospital, Tampa, Florida 33612, USA.

BACKGROUND: Opportunistic infections commonly occur in immunocompromised patients; however, it is unusual for an adult to present with a combined cellular and humoral immunodeficiency. Cryptococcal meningitis is a fatal condition if untreated and is usually found in patients with cellular immunodeficiency.

OBJECTIVE: To report the case of an adult patient with cryptococcal meningitis secondary to intestinal lymphangiectasia.

METHODS: A 59-year-old man was admitted to the hospital for disseminated cryptococcal meningitis and osteomyelitis. Laboratory evaluation, computed tomography, esophagogastroduodenoscopy, and biopsy were performed.

RESULTS: Laboratory evaluation revealed a lymphopenia, hypoalbuminemia, hypogammaglobulinemia, and negative human immunodeficiency virus test results by enzyme-linked immunosorbent assay and polymerase chain reaction. The complete blood cell count, urinalysis, serum and urine protein electrophoresis, and functional antibody responses to protein and polysaccharide antigens were normal. Results of computed tomography of the chest, abdomen, and pelvis were unremarkable. Multiple lymphangiectasias were visualized with esophagogastroduodenoscopy and confirmed by biopsy. The patient was treated with intravenous amphotericin B and flucytosine, and the meningitis resolved.

CONCLUSIONS: Based on a computerized search of the medical literature, this is the first description of cryptococcal meningitis secondary to intestinal lymphangiectasias. The combination of lymphopenia, hypogammaglobulinemia, and hypoalbuminemia should alert the clinician to the possibility of intestinal lymphangiectasias and the potential for immune dysfunction.

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