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PubMed1991

[Current problems in the diagnosis and treatment of small intestinal diseases].

Based on the data obtained during clinical examination of 1,026 patients with small intestinal diseases the authors provide the portion of laboratory and instrumental methods employed in the diagnosis of different disease entities. The clinical picture of small intestinal diseases is mainly determined by the gravity of malabsorption. Histological examination of the small intestinal mucosa is a method of choice in the diagnosis of gluten enteropathy, Whipple's disease, primary lymphangiectasis and amyloidosis. Immunoassays play the key role in recognition of variable immunodeficiencies and disease of heavy alpha-chains. Meanwhile in differential diagnosis of Crohn's disease, small intestinal tumors, congenital abnormalities of rotation and in some others, the leading part is played by x-ray methods. The authors describe the treatment schedule based on the pathogenetic approach, that makes it possible to reach a stable clinical remission and recovery of the working capacity even in part of patients with stage III malabsorption.

Diagnosis, Differential↗
PubMedDate not supplied

[Generalized lymphatic dysplasia in a 6-month-old nursing infant].

G.L.D. is a rare syndrome characterized by chylothorax, chylous ascites and lymphedema associated to minor symptoms following from lymphangiectasis or to lymphangiomatosis. This syndrome is caused by congenital dysplasia of lymphatic vessels and has, generally, an extremely severe prognosis. In the present papers the authors describe one case of G.L.D. in a six months old infant with chylous ascites, chylothorax, lymphedema, hemangioma and a history of fetal ascites. The preservative (dietetic and evacuative) therapy was not resolutive, therefore an exploratory laparotomy was necessary, but no surgical solution was possible because of the absence of chylous vessels in the anatomical region of the principal lymphatic route. However, laparatomy led to a progressive recovery of ascites and chylothorax, probably due to thrombosis of dysplasic lymph vessels and to neoformation of collateral circulation.

Chylothorax↗
PubMed1985

Differential pathogenicity of Brugia malayi, B. patei and B. pahangi in immunodeficient nude mice.

Immunodeficient nude mice chronically parasitized by subperiodic Brugia malayi developed an elephantoid appearance with persistent lymphoedema of limbs and massive lymphangiectasis of subcutaneous vessels containing viable adult worms. Removal of worms reversed the process. The syndrome was not caused by B. patei or B. pahangi and was not correlated with the presence or absence of microfilaremia. Histologic examination of elephantoid mice revealed dilated and tortuous lymphatics containing small nonobstructive lymph thrombi composed of small mononuclear cells and multinucleate giant cells. Draining lymph nodes were not enlarged or congested and mast cells in oedematous tissue were not degranulated. Analysis of lymph aspirated from dilated lymphatics showed increased total protein content: bacterial sepsis was not detected. This work suggests that viable adult B. malayi exert direct pathologic effects upon lymphatics and that this parasite is more pathogenic than related Brugia spp.

Animals↗
PubMed1982

[Cystic lymphangioma of the adrenal gland causing arterial hypertension].

The authors report a new cases of cystic lymphangioma of the adrenal diagnosed at the time of investigation of systemic hypertension which became normal after the lesion was treated surgically. The place of these lymphangiectasis cysts amongst adrenal cystic lesions, their clinical manifestations, methods of diagnosis and treatment are envisaged.

Adrenal Gland Neoplasms↗
PubMed1981

A mycobacteriosis in a sheep resembling paratuberculosis (Johne's disease).

In a sheep which was euthanased because of severe emaciation and weakness, slight thickening of the ileum was seen grossly. Microscopically there was a granulomatous ileitis with obliterative lymphangitis and lymphangiectasis. Granulomatous lesions were also present in the liver and some mesenteric lymph nodes. Large numbers of acid-fast bacilli were present within epithelioid macrophages in the lamina propria of the ileum. Although the identity of the Mycobacterium spp. involved was not established, the possibility of paratuberculosis is discussed. The apparent rarity of this disease in sheep in South Africa is considered. Particular attention is drawn to the absence of diarrhoea in this case, to the slightness of the gross changes and to the importance of submitting material for mycobacterial culture.

Animals↗
PubMed1980

Prenatal ultrasonographic diagnosis of fetal cystic hygroma.

The terms cystic lymphangioma or hygroma have been used to describe large, single or multilocular, fluid-filled cavities. While such cavities are most often located on one side of the fetal neck, they occasionally are multiple and may be found in other locations, such as the axillary regions, mediastium, oral cavity and groin. Bilateral hygromas of the neck are not uncommonly observed in aborted fetuses, often in association with wide-spread lymphangiectasis. A case is presented of a fetus with cystic hygromas in the cervical region which were revealed by prenatal ultrasonographic examination.

Adult↗
PubMed1998

The role of magnetic resonance imaging in diagnosis of peripheral lymphatic disorders.

Magnetic resonance imaging (MRI) was used in 13 patients with peripheral lymphedema and 2 patients with extensive cavernous lymphangioma of the limb for the purpose of evaluating its role in diagnosis of lymphatic disorders. In chronic lymphedema, MRI showed deformity of lymphatics at different tissue levels. In the subcutis, MRI characteristically displayed diffuse edema or a honeycombed pattern consistent with reticular lymphangiectasis and "lakes" with a marked increase in signal intensity with T2-weighted imaging. In lymphedema hyperplasia and chylous reflux, MRI depicted dilated retroperitoneal lymphatic collectors and lumbar trunks. In cavernous lymphangiomatosis, MRI demonstrated a prominent lattice-like pattern which had lower signal intensity on T1-weighted imaging and higher intensity on T2-weighted imaging. The findings of MRI are valuable not only for accurate assessment of lymphatic dysplasia syndromes but also provide a blueprint for treatment options.

Adolescent↗
PubMedDate not supplied

[Exudative enteropathy in congenital lymphedema-lymphangiectasia syndrome].

BACKGROUND: Congenital peripheral elephantiasiformic alterations are very rare in paediatric patients. In a patient with lymphangiectasia-lymphedema syndrome we demonstrate over a 8-year follow-up that not only cosmetic and social indications for surgical treatments but also internal care become important during the course. PATIENT: We report on a boy with congenital lymphedemas of the extremities and the genital region, which were several times surgically treated. The patient became symptomatic firstly with tetanic cramps caused by malabsorption syndrome due to intestinal lymphangiectasia at the age of 6 years. Synopsis of clinical and laboratory findings and the patient's course are pointing to a mild Hennekam syndrome with still unknown aetiology. RESULTS: The boy developed adequately with permanent oral substitution of electrolytes and vitamins, protein-rich diet, supplementation of medium-chain fatty acids and compressing bandages. Infusions of human albumin to correct persistent hypalbuminemia as well as cytostatic treatment with cyclophosphamide as a formal trial were ineffective and are not advisable, therefore.

Child↗
PubMed2002

Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.

Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.

Adolescent↗