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PubMed1976

The distribution and enteric loss of 51Cr-labelled lymphocytes in normal subjects and in patients with coeliac disease and other disorders of the small intestine.

Peripherally harvested lymphocytes have been labelled with 51Cr, reinjected into human subjects and their distribution then studied. Evidence is presented which suggests faecal loss of 51Cr represents loss of T lymphocytes and that there is normally a pathway of lymphocyte removal into the gut of probable importance in lymphocyte migration streams. In 9 normal subjects, without structural intestinal disease, faecal loss of lymphocytes over 5 days was 0.20% (SEM +/- 0.06) whereas in 5 patients with untreated coeliac disease faecal loss was 1.13 +/- 0.34%, in 7 with Crohn's disease it was 1.01 +/- 0.21% and in 5 with intestinal lymphangiectasia loss was 0.61 +/- 0.10%. In 1 patient with acute tropical sprue, enteric loss was 0.97%. By contrast, faecal loss was normal in 3 coeliac patients in remission on a gluten-free diet. Measurements were also made using an external counter. In contrast to the normals, where count rates steadily diminished, an increasing activity was recorded over the umbilicus over 7 days after dose administration in all the disease categories studied with the exception of the treated coeliacs. The finding of an increased enteric loss of lymphocytes may explain many of the immunological abnormalities in the conditions studied.

Adult↗

PubMed1989

Primary disorders of the lymphatic vessels--a unified concept.

Congenital defects of lymphatics constitute a spectrum of disorders that may manifest with a variety of clinical presentations including lymphedema, chylous effusions, lymphangiomatous malformations with cystic masses and localized gigantism, and intestinal lymphangiectasia with malabsorption. These entities constitute a relatively rare group of disorders, the origin of which remains somewhat controversial, but in some it appears to be due to early lymphatic obstruction. Five cases are described, which demonstrate the anatomical pathology of these entities. A classification and description of the defects is also presented. An attempt is made to present a unified theory of origin for this seemingly diverse group of diseases. While these entities may be challenging from a diagnostic and therapeutic standpoint, a wide variety of imaging modalities, which includes lymphography, computed tomography scanning, and ultrasound, may be used to diagnose the extent and internal structural characteristics of the abnormalities.

Adolescent↗

PubMed1974

The relative importance of the factors involved in the absorption of vitamin E in children.

The vitamin E status and ease of repletion in groups of children with coeliac disease, intestinal lymphangiectasia, and abetalipoproteinaemia was studied and compared with earlier studies in cystic fibrosis and obstructive jaundice. Each group represents an experimental model in which one of the transport steps involved in the absorption of vitamin E is defective or absent and thus the relative importance of these factors could be determined. Chylomicron formation and an adequate intraluminal concentration of bile salts were found to be the most important factors for the efficient absorption of the vitamin. The results in the five groups of patients have therapeutic implications if it is considered desirable to correct vitamin E deficiency states.

Abetalipoproteinemia↗

PubMed1975

Intestinal lymphagiectasia: a reappraisal.

Intestinal lymphangiectasia (IL) may vary widely in its manifestations and severity. Fifteen children seen between 1960 and 1974 with histologically proven IL are analyzed by clinical, laboratory, radiologic, and histologic criteria. Remissions occurred in most patients and none died. Exacerbations occurred in five children. Diarrhea was present in 14 patients and in 13 appeared before the age of 3 years. Vomiting occurred in nine patients and growth retardation in seven. Four children had associated peripheral lymphedema and two of these had a family history of lymphedema, both had affected fathers and one had affected siblings and paternal cousins. Seven had hypoproteinemic edema, and of these, four suffered from hypocalcemic seizures. Chylous effusions were present in five. Hypoproteinemia was present in 12 although five had no hypoalbuminemic edema. Six had lymphopenia which was related to the severity of the disease and was the last abnormality to disappear after clinical remission. Lymphopenia may first appear years after the protein loss begins. Upper gastrointestinal tract series were performed in 13 children and had diagnostic supportive value in seven. Six children had two or more small-intestinal biopsies done. They all showed great variation from one examination to the other, ranging from a normal appearance to severe changes. Lymphatic block may occur at different sites-in the lamina propria only, generalized (lamina propria, submucosa, serosa, and mesentery), or conversely in the mesentery alone with minimal changes in the lamina propria. In three patients intravenous hyperalimentation was necessary. Specific treatment with a high-protein, low-fat diet with added medium-chain triglyceride (MCT) is valuable. Surgical resection was of benefit in one patient, and anastomosis of mesenteric to para-aortic lymph nodes in another.

Adolescent↗

PubMed1991

Intestinal lymphangiectasia masquerading as coeliac disease.

Intestinal lymphangiectasia (IL) usually presents with either non-specific general or gastro-intestinal symptoms. As IL may mimic other gastro-intestinal disorders, the diagnosis is often delayed. Intestinal lymphangiectasia was diagnosed in three children who were originally treated as cases of coeliac disease. Two were sisters who had been placed on a gluten-free diet, for 3 years in one and 10 years in the other, with no favourable response. The third patient had been tried on various formulae and underwent many investigations for failure to thrive, oedema, abdominal distension and recurrent chest infections. The diagnosis of IL was based on clinical history, physical examination and radiological and histological findings. The three patients were commenced on a medium-chain triglyceride-based diet and vitamins, with satisfactory results.

Adolescent↗

PubMed1990

Protein-losing enteropathy in systemic lupus erythematosus associated with intestinal lymphangiectasia.

This case report concerns a young woman with systemic lupus erythematosus who presented with a protein-losing enteropathy. Intestinal biopsy revealed lymphangiectasia. Mesenteric lymph nodes showed paracortical lymphoid depletion and multiple small sinusoids. Elevated cholesterol levels and normal lymphocyte counts characterized the peripheral blood samples. Lymph nodes, though enlarged, showed no evidence of obstructive pathology. No secondary cause of lymphangiectasia could be identified. The patient exhibited antinuclear antibodies and antibodies to dsDNA at the onset, and then, 5 yr later, the classic features of systemic lupus erythematosus (SLE). The lymphocytopenia and hypolipidemia that characterizes lymphangiectasia is not a feature of the SLE cases reported to date. Furthermore, the mechanism for the dilated lymphatics and villous edema is more likely immunological than mechanical disruption of lymphatics.

Adolescent↗

PubMed2003

Surgical resection of duodenal lymphangiectasia: a case report.

Intestinal lymphangiectasia, characterized by dilatation of intestinal lacteals, is rare. The major treatment for primary intestinal lymphangiectasia is dietary modification. Surgery to relieve symptoms and to clarify the etiology should be considered when medical treatment failed. This article reports a 49-year-old woman of solitary duodenal lymphangiectasia, who presented with epigastralgia and anemia. Her symptoms persisted with medical treatment. Surgery was finally performed to relieve the symptoms and to exclude the existence of underlying etiologies, with satisfactory effect. In conclusion, duodenal lymphangiectasia can present clinically as epigastralgia and chronic blood loss. Surgical resection may be resorted to relieve pain, control bleeding, and exclude underlying diseases in some patients.

Duodenal Diseases↗

PubMed1992

Cryptococcal disease presenting as cellulitis.

Three immunocompromised patients presented with cellulitis as the primary manifestation of cryptococcal disease. Two were recipients of cadaveric renal transplants who were receiving immunosuppressive drug therapy. The other patient had profound lymphopenia and severe hypoalbuminemia due to intestinal lymphangiectasia. All had failed to respond to empiric therapy for presumed bacterial cellulitis before results of skin biopsy or aspiration were available for the correct diagnosis to be made. With administration of systemic antifungal therapy, two patients survived. Although other forms of cryptococcal involvement of the skin are not rare, cellulitis is seldom considered to be a cutaneous manifestation of the disease. Our cases and a review of the English-language literature indicate that Cryptococcus neoformans must be included in the differential diagnosis of cellulitis in immunocompromised patients and that the presence of cryptococcal cellulitis suggests disseminated cryptococcal disease. Prompt diagnosis and treatment may dramatically reduce mortality.

Adult↗

PubMed1989

Intestinal lymphangiectasia markedly improved with antiplasmin therapy.

A 35-yr-old woman with intestinal lymphangiectasia was treated with trans-4-aminomethyl cyclohexane carboxylic acid. After 6 wk of antiplasmin therapy, her serum total protein increased to normal levels and 131I-polyvinyl pyrrolidone excretion was also normalized. With a daily administration of trans-4-aminomethyl cyclohexane carboxylic acid, the patient has manifested no symptoms for 8 yr up to the present. Throughout the clinical course, the values for euglobulin lysis time showed a close relationship to changes in serum total protein. It was then suggested that increased plasma fibrinolysis may enhance lymphatic permeability to plasma proteins. During this treatment, a decreased percentage of T lymphocytes became normalized together with serum immunoglobulin values. In addition, the therapy has resulted in the disappearance of duodenal lesions observed endoscopically.

Adult↗

PubMed2005

Enamel hypoplasia of the primary dentition in a 4-year-old with intestinal lymphangiectasia.

Intestinal lymphangiectasia (IL) is a rare disorder, and its incidence and prevalence is unknown for either Australia or world-wide. It is characterized by diarrhoea, mild steatorrhoea, oedema, enteric loss of protein (protein-losing enteropathy) and abnormal dilated lymphatic channels in the small intestine. Whilst oedema and diarrhoea are the predominant clinical features, other observed features include hypoalbuminemia, hypogammaglobulinemia, trace metal deficiency, hypocalcemia and chylous pleural effusions. While medical presentation of the condition has been reported widely, few descriptions of oral findings have been published. A search of Medline found two reports of dental findings in the permanent dentition in patients with IL. To date, there have been no reports on dental findings in the primary dentition. The primary dentition of a 4-year-old boy with IL had teeth with enamel defects which reflected the timing of enamel development and the period in which the disease was active. The present report highlights the need for early involvement of the dental team in the dental management of children with IL.

Child, Preschool↗

PubMed1998

Octreotide in the treatment of intestinal lymphangiectasia.

Primary intestinal lymphangiectasia is characterized by dilated small bowel lymphatics and loss of lymph into the bowel lumen resulting in hypoproteinaemia and oedema. Some patients have a more generalized lymphatic abnormality associated with lymphoedema of the limbs and chylous pleural effusions. There is no specific treatment although enteric protein loss may decrease with a low-fat diet. This report describes a patient with severe primary intestinal lymphangiectasia, associated with limb oedema and recurrent pleural effusions, who responded to treatment with octreotide. Before starting octreotide she required weekly intravenous albumin infusions to maintain the serum albumin above 20 g/l. Bilateral pleural effusions repeatedly reaccumulated despite pleurectomy and subsequently tetracycline pleurodesis. Treatment with octreotide, 200 microg twice daily, resulted in a reduction in enteric protein loss from 16 to 4.1% in 5 days (normal less than 1%) and the serum albumin was maintained between 22 and 26 g/l without the need for albumin infusion. Oedema in the arms resolved completely and the pleural effusions did not reaccumulate. The mechanism of action of octreotide in this condition appears to be due to a reduction in gut protein loss and another, as yet unidentified, action.

Adolescent↗

PubMed1991

[The diagnostic value of In-111 transferrin imaging in protein-losing gastroenteropathy].

The diagnostic value of In-111 transferrin abdominal imaging was studied in 17 patients with clinical suspicion of protein-losing gastroenteropathy, compared to that of alpha-1-antitrypsin fecal clearance test. The presence or absence of gastrointestinal protein-loss was finally decided based on the result of alpha-1-antitrypsin fecal clearance test (normal range, less than 13 ml/day) or of protein content measurement on the gastric juice (normal range, less than 71 mg/dl). In-111 transferrin was labeled in vitro. After intravenous administration, serial anterior abdominal images were obtained. All seven patients with a value equal to or more than 20 ml/day on the alpha-1-antitrypsin clearance test and two out of ten patients with a value less than 20 ml/day showed definite intestinal activity demonstrating protein-loss, and the loss-site was estimated by observing the movement of radiotracer within the bowel lumen. The patients with severe protein-loss showed the intestinal activity on the early image. In the patients with milder protein-loss, on the other hand, the activity was noted on the later image. All two patients with positive In-111 transferrin imaging and negative alpha-1-antitrypsin test were associated with protein-losing gastropathy, where a value of alpha-1-antitrypsin clearance was not increased because of denatured alpha-1-antitrypsin by acidic gastric juice and pepsin. These results suggest that In-111 transferrin abdominal imaging can be more useful for the diagnosis of protein-losing gastroenteropathy, because of its capabilities to evaluate the patients with gastropathy as well as to estimate the loss-site.

Adult↗

PubMed1976

Antibodies to nucleic acids in congenital immune deficiency states.

The presence of antibodies to native DNA, single-stranded DNA, and double-stranded RNA was determined for 37 patients with selective IgA deficiency, 11 patients with Wiskott-Aldrich syndrome, seven patients with common variable agammaglobulinemia, 14 patients with ataxia telangiectasia, six patients with intestinal lymphangiectasia, and one patient with Nezelof syndrome. Of 37 patients with selective IgA deficiency, 11 had antibodies to at least one nucleic acid; six had antibodies to native DNA, seven had antibodies to single-stranded DNA, and four had antibodies to double-stranded RNA. The only other congenital immune deficiency disease studied in which antibodies to nucleic acids were found was the Wiskott-Aldrich syndrome; in this group three of 11 patients had antibodies to native DNA. Retrospective analysis of our patients with SLE disclosed a 2.6% prevalence of IgA deficiency, a prevalence clearly higher than in the general population. These studies provide further evidence of the association between autoimmunity and abnormalities of IgA production and suggest a relationship between thymic-derived immune regulation and IgA production.

Adult↗

PubMed2004

Octreotide in intestinal lymphangiectasia: lack of a clinical response and failure to alter lymphatic function in a guinea pig model.

Intestinal lymphangiectasia, which can be classified as primary or secondary, is an unusual cause of protein-losing enteropathy. The main clinical features include edema, fat malabsorption, lymphopenia and hypoalbuminemia. Clinical management generally includes a low-fat diet and supplementation with medium chain triglycerides. A small number of recent reports advocate the use of octreotide in intestinal lymphangiectasia. It is unclear why octreotide was used in these studies; although octreotide can alter splanchnic blood flow and intestinal motility, its actions on lymphatic function has never been investigated. A case of a patient with intestinal lymphangiectasia who required a shunt procedure after failing medium chain triglycerides and octreotide therapy is presented. During the management of this case, all existing literature on intestinal lymphangiectasia and all the known actions of octreotide were reviewed. Because some of the case reports suggested that octreotide may improve the clinical course of intestinal lymphangiectasia by altering lymphatic function, a series of experiments were undertaken to assess this. In an established guinea pig model, the role of octreotide in lymphatic function was examined. In this model system, the mesenteric lymphatic vessels responded to 5-hydroxytryptamine with a decrease in constriction frequency, while histamine administration markedly increased lymphatic constriction frequency. Octreotide failed to produce any change in lymphatic function when a wide range of concentrations were applied to the mesenteric lymphatic vessel preparation. In conclusion, in this case, octreotide failed to induce a clinical response and laboratory studies showed that octreotide did not alter lymphatic function. Thus, the mechanisms by which octreotide induced clinical responses in the cases reported elsewhere in the literature remain unclear, but the present study suggests that it does not appear to act via increasing lymphatic pumping.

Adult↗

PubMed2001

Lymphangiectasia with persistent Müllerian derivatives: confirmation of autosomal recessive Urioste syndrome.

We report on a sibship with protein-losing enteropathy related to intestinal lymphangiectasia, a peculiar face, and genital anomalies. The parents are distantly related and from Dutch ancestry. The first patient was born with a protein-losing enteropathy, craniofacial anomalies, and renal defects. At 1 year of age, she died of severe complications of the protein-losing enteropathy and respiratory distress. Her brother was a cytogenetically normal male fetus identified by prenatal ultrasound at 19 weeks with similar anomalies. The pregnancy was terminated at 20 weeks. Autopsy showed müllerian duct remnants. These cases seem to confirm the Urioste syndrome [Urioste et al., 1993: Am J Med Genet 47:494-503]. Although it was previously only reported in 46,XY individuals, this report of a consanguineous family with an affected sibship of both sexes suggests it to be an autosomal recessive entity.

Abnormalities, Multiple↗

PubMed1994

Immunological study in primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia is a rare congenital condition associated with protein-losing enteropathy. Hypogammaglobulinemia and lymphopenia secondary to this condition are frequent but infectious complications are not. So far few immunological studies have been made in these patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease in serum gammaglobulins, especially IgG and IgA, and in peripheral blood lymphocytes, especially CD4 T helper cells. From a functional standpoint, the proliferative response to certain mitogens was reduced. A decrease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of duodenal biopsy specimens revealed a decreased number of intraepithelial lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in the terminal ileum, confirmed by endoscopic biopsy. The role of these abnormalities in the development of infectious complications and lymphoma is underscored.

Adolescent↗

PubMed2005

Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition.

Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.

Adolescent↗

PubMedDate not supplied

Cutaneous manifestations and massive genital involvement in Hennekam syndrome.

We describe a 16-year-old boy with intestinal lymphangiectasia, lymphedema of the limbs and genitalia, mild mental retardation, and facial anomalies (Hennekam syndrome) and cutaneous lesions. Severe edema in the genital area created a gigantic mass that included the scrotum and penis. Numerous grouped red-violaceous pseudo-vesicular lesions and plaques, as well as verrucous brown papules, were present on the penis and scrotum. The prepuce was hypertrophic, with severe phimosis. Histologic analysis revealed dilated lymphatic vessels lined by a discontinuous layer of flat endothelial cells in the papillary dermis and extending down to the reticular dermis. Dilated blood vessels were also present but no cellular abnormalities were identified. A diagnosis of superficial cutaneous lymphatic malformations was made. To the best of our knowledge, this is the first detailed cutaneous histologic investigation in a patient with Hennekam syndrome. We assume that the onset of cutaneous lesions in our patient was likely triggered by a generalized worsening of his lymphedema.

Adolescent↗