Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Lymphatic Abnormalities”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 109 records · Page 6Linked to original sources
PubMedDate not supplied

[Necrolytic migratory erythema in Waldmann's disease].

BACKGROUND: We report a case of necrolytic migratory erythema in a patient with Waldmann's disease. PATIENTS AND METHODS: A 55-year-old male patient with a history of Waldmann's disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient's cutaneous lesions. DISCUSSION: Waldmann's disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann's disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.

Dietary Fats↗
PubMed1997

Jejunal lymphangioma. An unusual cause of chronic gastrointestinal bleeding.

We discuss a case of lymphangioma of the small bowel that caused chronic anemia secondary to gastrointestinal blood loss. We believe that this is the first jejunal lymphangioma to be diagnosed by Sonde endoscopy. There are several other lymphatic abnormalities of the small intestine that are briefly discussed. In the Western literature, anemia or chronic gastrointestinal blood loss is an unusual presentation of lymphangioma of the small bowel.

Adult↗
PubMed2006

Nonimmune hydrops fetalis due to generalized lymphatic dysplasia in an infant with Robertsonian trisomy 21.

We report the first case of generalized lymphatic dysplasia and trisomy 21 presenting with nonimmune hydrops fetalis. This infant showed intractable chylothorax, chylous ascites, and periodic bouts of edema. A karyotype analysis revealed Robertsonian trisomy 21: 46,XY,t(14q21q)(q10;q10) +21. This patient died of multiple organ failure at 400 days of life, despite the management of chylous effusions. The lymphoscintigraphy and histopathological findings led to the final diagnosis of generalized lymphatic dysplasia, which might also contribute to the development of hydrops. Refractory chylothorax in trisomy 21 patients may emphasize the need for intensive scrutiny of lymphatic disorders.

Adult↗
PubMed1993

Progression of nonimmune hydrops in a fetus with Noonan syndrome.

Presented is a case of nonimmune hydrops caused by Noonan syndrome. This autosomal dominant disorder, with an incidence of 1 in 1000 births, is manifested by trunk and limb edema, lymphatic abnormalities, webbed neck, short stature, and cardiac and genital anomalies. In this case the initial presentation was polyhydramnios at 31 weeks' gestation, followed in 1 week by pleural effusions and a week later by skin edema. An 18-week ultrasound had been normal. Because of variable expressivity, only the most severe cases of the disease are usually diagnosed. Thus, the incidence may be higher than 1 in 1000, and Noonan syndrome may be underdiagnosed as a cause of nonimmune hydrops. Since lymphedema in this syndrome may regress spontaneously, conservative obstetric management is preferable unless the fetus develops distress.

Adult↗
PubMed2002

Mass treatment to eliminate filariasis in Papua New Guinea.

BACKGROUND: The global initiative to eradicate bancroftian filariasis currently relies on mass treatment with four to six annual doses of antifilarial drugs. The goal is to reduce the reservoir of microfilariae in the blood to a level that is insufficient to maintain transmission by the mosquito vector. METHODS: In nearly 2500 residents of Papua New Guinea, we prospectively assessed the effects of four annual treatments with a single dose of diethylcarbamazine plus ivermectin or diethylcarbamazine alone on the incidence of microfilariae-positive infections, the severity of lymphatic disease, and the rate of transmission of Wuchereria bancrofti by mosquitoes. Random assignment to treatment regimens was carried out according to the village of residence, and villages were categorized as having moderate or high rates of transmission. RESULTS: The four annual treatments with either drug regimen were taken by 77 to 86 percent of the members of the population who were at least five years old; treatments were well tolerated. The proportion with microfilariae-positive infections decreased by 86 to 98 percent, with a greater reduction in areas with a moderate rate of transmission than in those with a high rate. The respective aggregate frequencies of hydrocele and leg lymphedema were 15 percent and 5 percent before the trial began, and 5 percent (P<0.001) and 4 percent (P=0.04) after five years. Hydrocele and leg lymphedema were eliminated in 87 percent and 69 percent, respectively, of those who had these conditions at the outset. The rate of transmission by mosquitoes decreased substantially, and new microfilariae-positive infections in children were almost completely prevented over the five-year study period. CONCLUSIONS: Annual mass treatment with drugs such as diethylcarbamazine can virtually eliminate the reservoir of microfilariae and greatly reduce the frequency of clinical lymphatic abnormalities due to bancroftian filariasis. Eradication may be possible in areas with moderate rates of transmission, but longer periods of treatment or additional control measures may be necessary in areas with high rates of transmission.

Adolescent↗
PubMed2004

Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a multisystem disorder of women, characterized by cystic degeneration of the lungs, renal angiomyolipomas (AML), and lymphatic abnormalities. LAM lesions result from the proliferation of benign-appearing, smooth muscle-like LAM cells, which are characterized by loss of heterozygosity (LOH) of one of the tuberous sclerosis complex (TSC) genes. LAM cells are believed to migrate among the involved organs. Because of the apparently metastatic behavior of LAM, we tried to isolate LAM cells from body fluids. A cell fraction separated by density gradient centrifugation from blood had TSC2 LOH in 33 of 60 (55%) LAM patients. Cells with TSC2 LOH were also found in urine from 11 of 14 (79%) patients with AML and in chylous fluid from 1 of 3 (33%) patients. Identification of LAM cells with TSC2 LOH in body fluids was not correlated with severity of lung disease or extrapulmonary involvement and was found in one patient after double lung transplantation. These studies are compatible with a multisite origin for LAM cells. They establish the existence of disseminated, potentially metastatic LAM cells through a relatively simple, noninvasive procedure that should be valuable for molecular and genetic studies of somatic mutations in LAM and perhaps other metastatic diseases.

Body Fluids↗
PubMed1997

Yellow nail syndrome in infancy.

This report describes an infant with clinical features consistent with the yellow nail syndrome (YNS), a rare autosomal dominant disorder. He presented at birth with congenital lymphoedema and was referred at 6 months of age for investigation of recurrent cough and wheeze. He had clinical and radiological evidence of bilateral pleural effusions and a pericardial effusion. Following a lung biopsy and pericardial window these were shown to be manifestations of his lymphatic abnormality. He also had persisting middle ear effusions causing conductive deafness requiring hearing aids and secondary immunodeficiency requiring regular immunoglobulin infusions.

Humans↗
PubMed1965

RETROGRESSION IN TESTICULAR SEMINOMA WITH VIABLE METASTASES.

Stages in the retrogression of testicular seminoma are described. Eosinophilic necrosis fringed by palisaded histiocytes may be followed by fibrous replacement. Oxidation of unsaturated phospholipids in necrotic tumour may lead to deposition of lipofuscin around the lesion. Search for partially or completely scarred lesions is essential before contemplating a diagnosis of primary retroperitoneal seminoma. Retrogressed seminoma can often be distinguished from retrogressed teratoid tumours. Apparently paradoxical teratoid metastases in association with a testicular seminoma are explained on the basis of misinterpreted retrogressed teratoid tumours in association with the seminoma. Inguinal node metastases from testicular seminoma may be the result of abnormal lymphatic drainage following previous scrotal operations, testicular torsion etc.

Cicatrix↗
PubMed1987

Cystic hygromas in children: sonographic-pathologic correlation.

The sonographic findings in eight children with surgically proved cystic hygroma were reviewed and correlated with the pathologic specimens. Six tumors occurred in the neck, one occurred in the axilla, and one involved the soft tissues of the thigh, scrotum, and pelvis. A cystic hygroma characteristically appears as a multiloculated cystic mass with septa of variable thickness that contain solid components arising from the cyst wall or the septa. Correlation of the sonogram with the pathologic specimen demonstrated that the echogenic component corresponded to a cluster of abnormal lymphatic channels, too small to be resolved with ultrasound. Large lesions had ill-defined boundaries, with cystic components dissecting between normal tissue planes. Sonographically, one can usually differentiate these tumors from other cervical masses, especially soft-tissue hemangiomas. Sonography is also helpful in determining the extent of the lesion before surgery and in assessing postoperative complications and recurrences.

Axilla↗
PubMed1972

Impaired lymphocyte transformation in intestinal lymphangiectasia: evidence for at least two functionally distinct lymphocyte populations in man.

Intestinal lymphangiectasia is a disease characterized by hypoproteinemia and edema resulting from protein-losing gastroenteropathy secondary to abnormal intestinal lymphatics. Immunologic abnormalities associated with this disease include hypogammaglobulinemia, lymphocytopenia, skin anergy, and impaired allograft rejection. In the present study, the in vitro blastogenic transformation of lymphocytes from 12 patients with intestinal lymphangiectasia was assessed in order to gain insight into the mechanism of the cellular immune defect in this disease. Peripheral blood lymphocytes from patients with intestinal lymphagiectasia showed impaired in vitro transformation to nonspecific mitogens, specific antigens, and allogeneic cells when compared to equal numbers of cells from normal individuals. Patients with the most deficient in vitro reactivity tended to have the lowest serum albumin concentration and the lowest absolute lymphocyte count. Lymphocytes obtained from chylous effusions in each of the four patients studied transformed more vigorously than peripheral blood cells from the same patients. These results may be explained by the loss of recirculating, long-lived lymphocytes into the gastrointestinal tract, resulting in a relative depletion of the population of lymphocytes necessary for in vitro blast transformation. This disease thus represents a clinical analogue of animals with experimental thoracic duct drainage, and provides evidence for the existence, in man, of two functionally distinct lymphocyte populations. In addition, these findings establish a new mechanism of impaired delayed hypersensitivity and defective in vitro lymphocyte transformation, i.e. the gastrointestinal loss and consequent depletion of the long-lived, recirculating population of lymphocytes from the peripheral lymphocyte pool.

Adolescent↗
PubMedDate not supplied

Yellow nail syndrome in a 10-year-old girl.

A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with medium-chain triglycerides. Moderate improvement in the lymphedema of the lower extremities was observed. To our knowledge this is the first case of yellow nail syndrome to be treated with diet.

Bronchiectasis↗
PubMedDate not supplied

Vascular lesions of the gastrointestinal tract.

Vascular lesions of the gastrointestinal (GI) tract include arterio-venous malformations as angiodysplasia and Dieulafoy's lesion, venous ectasias (multiple phlebectasias and haemorroids), teleangiectasias which can be associated with hereditary hemorrhagic teleangiectasia (HHT), Turner's syndrome and systemic sclerosis, haemangioma's, angiosarcoma's and disorders of connective tissue affecting blood vessels as pseudoxanthoma elasticum and Ehlers-Danlos's disease. As a group, they are relatively rare lesions that however may be a major source of upper and lower gastrointestinal bleeding. Clinical presentation is variable, ranging from asymptomatic cases over iron deficiency anaemia to acute or recurrent bleeding that may be life-threatening. Furthermore, patients may present with other symptoms, e.g. pain, dysphagia, odynophagia, the presence of a palpable mass, intussusception, obstruction, haemodynamic problems resulting from high cardiac output, lymphatic abnormalities with protein loosing enteropathy and ascites, or dermatological and somatic features in syndromal cases. Diagnosis can usually be made using endoscopy, sometimes with additional biopsy. Barium radiography, angiography, intraoperative enteroscopy, tagged red blood cell scan, CT-scan and MRI-scan may offer additional information. Treatment can be symptomatic, including iron supplements and transfusion therapy or causal, including therapeutic endoscopy (laser, electrocautery, heater probe or injection sclerotherapy), therapeutic angiography and surgery. The mode of treatment is of course depending on the mode of presentation and other factors such as associated disorders. If endoscopic or angiographic therapy is impossible and surgical intervention not indicated, pharmacological therapy may be warranted. Good results have been reported with different drugs, albeit most of them have not been tested in large trials.

Connective Tissue Diseases↗
PubMed2003

[Two unusual complications of the Klippel-Trénaunay syndrome: carpal tunnel syndrome and cerebral venous thrombosis].

Most neurological complications of the Klippel-Trénaunay syndrome are due to the presence of cerebral or spinal arteriovenous malformations and their consequences. We report 2 cases of unusual complications of this syndrome. In the first case, where the anomalies were restricted to the left upper extremity, the occurrence of hand numbness led to the discovery of a carpal tunnel syndrome, probably due to the lymphatic abnormal proliferation within the carpal tunnel. In the second case the disease was much more widespread and affecting, among other localizations, the cephalic extremity. The patient presented with intractable left motor seizures that appeared to be due to an occlusion of the right transverse and sigmoid sinuses. Treatment with heparin and anticonvulsants led to rapid recovery.

Adolescent↗
PubMed2005

Diagnostic protocol for lymphoscintigraphy in newborns.

The purpose of this methods paper is to offer pediatricians and nuclear medicine physicians a diagnostic protocol for performing lymphoscintigraphy in newborns that may be useful for enhancing diagnosis and management of newborns with congenital lymphatic abnormalities. Indications for lymphoscintigraphy, choice of tracer, optimal dose, routes of administration, methods of data acquisition, timing, and interpretation of results for newborns are presented and discussed.

Diagnosis, Differential↗