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Results for “Lymphatic Abnormalities”

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PubMed1992

Pulmonary abnormalities in Klippel-Trenaunay syndrome. A histologic, ultrastructural, and immunocytochemical study.

Klippel-Trenaunay (KT) syndrome is a rare, sporadic, congenital vascular disease of unknown etiology. We describe pulmonary findings in an 18-year-old male patient followed up since birth with the KT syndrome. The patient developed pleural and pericardial serous effusions that led to an open lung biopsy. Previous pulmonary findings have been limited to thromboembolic phenomena and pulmonary vein varicosities. On the other hand, reports of lymphatic hyperplasia, aplasia, and hypoplasia in KT have been limited to the extremities. For the first time, we describe lymphatic involvement of the lung in KT. The plexiform hyperplasia of the lymphatic channels with smooth muscle hyperplasia leading to lymphatic obstruction, pleural and pericardial effusions are new findings. The lymphatic nature of the plexiform channels was confirmed by immunohistochemistry. Von Willebrand factor and QD-END/10 monoclonal antibodies either did not react or reacted poorly with lymphatic endothelium, features used to distinguish lymphatic and venous endothelium. Ultrastructurally, the absence of basement membrane continuity further substantiated the lymphatic nature of the channels. From our findings, the lymphatic abnormality in the syndrome appears to be more generalized than previously thought. This entity should be distinguished from lymphangioleiomyomatosis to which it bears a superficial morphologic appearance.

Adolescent↗
PubMed2005

Detection of lymph node involvement in hematologic malignancies using micromagnetic resonance lymphangiography with a gadolinum-labeled dendrimer nanoparticle.

Animal models of lymphoma should reflect their counterparts in humans; however, it can be difficult to ascertain whether an induced disease is intralymphatic or extralymphatic based on direct visualization. Current imaging methods are insufficient for identifying lymphatic and intralymphatic involvement. To differentiate intralymphatic from extralymphatic involvement, we have developed a magnetic resonance imaging-based lymphangiography method and tested it on two animal models of lymphoma. A gadolinium (Gd)-labeled dendrimer nanoparticle (generation-6; approximately 220 kDa/ approximately 10 nm) was injected interstitially into mice bearing hematologic malignancies to perform dynamic micromagnetic resonance lymphangiography (micro-MRL). Both a standard T1-weighted 3D fast spoiled gradient echo and a T2/T1-weighted 3D fast imaging employing steady-state acquisition (3D-FIESTA-C) were compared in an imaging study to differentiate intralymphatic from extralymphatic involvement of tumors. The lymphatics and lymph nodes were visualized with both methods in all cases. In addition, 3D-FIESTA-C depicted both the lymphatic system and the extralymphatic tumor. In an animal model, 3D-FIESTA-C demonstrated that the bulk of the tumor thought to be intralymphatic was actually extralymphatic. In conclusion, micro-MRL, using Gd-labeled dendrimer nanoparticles with the combined method, can define both the normal and abnormal lymphatics and can distinguish intralymphatic from extralymphatic diseases in mouse models of malignant lymphoma.

Animals↗
PubMed2000

Lymphatic malformation causing intractable chylorrhagia.

Lymphatic malformation is a developmental error usually noticed at birth or in early childhood. Lesions of the upper leg and lower trunk are the most difficult to remove, because they are often the largest ones encountered and also because they tend to extend proximally into the retroperitoneal tissues. Chyle reflux, usually associated with lymphedema of the extremity, has not been reported to be caused by lymphatic malformation. We report a case of intrapelvic retroperitoneal lymphatic malformation with an extension of gluteal-thigh soft-tissue involvement causing intractable chylorrhagia. The tumor was subtotally excised, and the defect was closed by a distally based, peninsular latissimus dorsi myocutaneous flap. The flap served both as a filling material and as a "bridge" between the residual tumor, including abnormal lymphatics, and normal lymph flow.

Adolescent↗
PubMed1993

[A case of yellow nail syndrome presenting as recurrent pneumonia].

We report a case of yellow nail syndrome (YNS) associated with recurrent episodes of pneumonia. The patient, a 31-year-old female, presented with yellow nails, chronic bronchitis and pleural effusion. The etiology of YNS remains unknown although it has been postulated to be associated with lymphatic abnormalities. YNS is a rare disease with only 22 cases reported in Japan. However, YNS is often associated with respiratory infections, and should thus be borne in mind.

Adult↗
PubMed1990

An easy method for diagnosis of lymphedema.

Lymphoscintigraphy has been very useful in determination of lymphatic abnormalities. However, the radioactive isotopes used have been investigational and difficult obtain. The purpose of this study was to examine patients with extremity edema by lymphoscintigraphy using a radioactive colloid readily available in our nuclear pharmacy, Technetium 99m sulfur minicolloid. Forty limbs in 20 patients were evaluated using Technetium 99m sulfur minicolloid lymphoscintigraphy. All patients had lower extremity edema initially attributed to a venous or lymphatic etiology. There were 12 patients with normal bilateral studies. Seven patients exhibited unilateral obstruction to lymphatic flow, and one had unilateral enhanced flow of lymph. Those with normal studies included five patients with nonspecific edema, four with varicosities, and one patient each with acute deep vein thrombosis, chylous ascites, and excision of the greater saphenous vein for arterial bypass grafting. Five patients with obstructed patterns had previous arterial bypass procedures, one had trauma to the extremity, and one had lymphedema tarda. The one enhanced lymphoscintigraphic pattern was seen in a patient with acute cellulitis. All patients had Doppler venous examinations and other studies included strain gauge phlethysmography, venograms, computed tomography, magnetic resonance imaging, and ultrasound. As with other scintigraphic imaging agents used to study lymphatic flow, Technetium 99m provides clinically useful information in evaluating the swollen extremity noninvasively.

Adult↗
PubMed2002

Prenatal diagnosis of vascular anomalies.

BACKGROUND/PURPOSE: Vascular anomalies are diagnosed prenatally with increasing frequency. The authors reviewed a group of children treated at their center who had an abnormal prenatal diagnosis to determine (1) fetal age at which the vascular anomaly was detected, (2) general diagnostic accuracy, and (3) impact on ante- and postnatal care. Their findings are compared with reported cases and series. The authors clarify appropriate terminology and underscore the need for interdisciplinary participation of specialists in the field of vascular anomalies. METHODS: Patients referred during prenatal life and children with a history of abnormal antenatal findings seen at our vascular anomalies center during a 1-year period (September 1999 through August 2000) were included in this study. The fetal age at diagnosis, pre- and postnatal diagnoses, antenatal course, and neonatal outcome were obtained from the parents, through chart reviews, and through telephone interviews with the treating obstetricians. RESULTS: Twenty-nine patients with vascular anomalies were identified: 17 had a correct prenatal diagnosis, and 12 had an incorrect diagnosis, an overall diagnostic accuracy of 59%. Capillary-lymphatic-venous malformations (CLVM) most often were correctly diagnosed (67%), followed by lymphatic malformation (LM, 62%) and hemangioma (59%). In the infants who received correct diagnoses in utero, there were no fetal deaths and there was no neonatal morbidity. Maternal steroids were administered for a fetus with an intrahepatic hemangioma and deteriorating cardiac function, with subsequent stabilization and successful delivery of a healthy neonate. Among infants with incorrect diagnoses, there was 1 postnatal death, 1 case of erroneous gender assignment, 1 case of unnecessary fetal surgical intervention, 1 unnecessary neonatal laparotomy, and 1 delay in diagnosis of a malignancy. Cesarean section was done for 65% of correctly diagnosed cases, (including 2 ex utero intrapartum [Exit] procedures) and for 33% of incorrectly diagnosed cases. Most diagnoses were made during the mid- to late second trimester and third trimester; only 4 cases (14%) were detected before 20 weeks. CONCLUSIONS: In this series, accurate diagnosis optimized antenatal care by providing an opportunity for planning deliveries, for pharmacologic fetal intervention in 1 case, and for appropriate parental counselling. Inaccurate diagnosis was associated with significantly increased morbidity and mortality. Finally, the intrauterine diagnosis of LM should be distinguished from posterior nuchal translucency, an obstetric term applied to fetal lymphatic abnormalities detected in the first and second trimesters that do not manifest as postnatal LM.

Arteriovenous Malformations↗
PubMed1977

Primary chylopericardium: the role of lymphangiography.

Primary chylopericardium is often a benign asymoptomatic disease, but patients with associated mediastinal lymphatic tumors may develop cardiac tamponade. Lymphangiography demonstrates mediastinal lymphatic abnormalities in these high risk patients on whom subtotal pericardiectomy and thoracic duct ligation should be performed. Since such tumors have been found in half of reported cases and because extrathoracic lymphangiomas may coexist, lymphangiography with skeletal survey is recommended in all cases. These conclusions are based on a review of 31 published cases and an additional case of our own. Lymphangiographic findings were available in 17 of these patients.

Adolescent↗
PubMed1985

Chylous ascites in infants and children: a case report and literature review.

Successful treatment of an infant with chylous ascites secondary to "congenital leaky lymphatics" is described, followed by a literature review of cases of pediatric chylous ascites. The infant was placed on home total parenteral nutrition for 10 weeks, during which time the chylous ascites resolved and did not recur with the introduction of a normal diet. Forty cases of pediatric chylous ascites have been reported since 1960, with an age range from birth to 18 years. The presenting signs and symptoms were secondary to abdominal distension in over 80% of cases. The chylous ascites was secondary to trauma, which included child abuse (19%), obstruction (27%), or lymphatic abnormalities (54%). Most studies described were not helpful in making the diagnosis, except for lymphangiography and exploratory laparotomy. Many patients were treated with a high-protein, low-fat diet, with or without medium-chain triglycerides, with variable success. Total parenteral nutrition (TPN) was the primary therapeutic modality in five patients, and in all but one of the cases there was complete resolution of the chylous ascites over 3 to 10 weeks with no significant side effects. Total parenteral nutrition, including home TPN, is a safe and effective therapeutic modality for some cases of chylous ascites.

Adolescent↗
PubMed2004

The lymphangiogenic vascular endothelial growth factors VEGF-C and -D are ligands for the integrin alpha9beta1.

Mice homozygous for a null mutation of the integrin alpha9 subunit die 6-12 days after birth from bilateral chylothoraces suggesting an underlying defect in lymphatic development. However, until now the mechanisms by which the integrin alpha9beta1 modulates lymphangiogenesis have not been described. In this study we show that adhesion to and migration on the lymphangiogenic vascular endothelial growth factors (VEGF-C and -D) are alpha9beta1-dependent. Mouse embryonic fibroblasts and human colon carcinoma cells (SW-480) transfected to express alpha9beta1 adhered and/or migrated on both growth factors in a concentration-dependent fashion, and both adhesion and migration were abrogated by anti-alpha9beta1 function-blocking antibody. In SW-480 cells, which lack cognate receptors for VEGF-C and -D, both growth factors induced alpha9beta1-dependent Erk and paxillin phosphorylation. Human microvascular endothelial cells, which express both alpha9beta1 and VEGF-R3, also adhered to and migrated on both growth factors, and both responses were blocked by anti-alpha9beta1 antibody. Furthermore, in a solid phase binding assay recombinant VEGF-C and -D bound to purified alpha9beta1 integrin in a dose- and cation-dependent fashion showing that VEGF-C and VEGF-D are ligands for the integrin alpha9beta1. The interaction between alpha9beta1 and VEGF-C and/or -D may begin to explain the abnormal lymphatic phenotype of the alpha9 knock-out mice.

Animals↗
PubMed1986

Nonimmune hydrops fetalis in Noonan's syndrome.

Nonimmune hydrops fetalis has been reported to be associated with congenital malformations. We describe two newborns with Noonan's syndrome who presented with nonimmune hydrops fetalis that was most likely secondary to a generalized lymphatic vessel dysplasia. Other manifestations of lymphatic abnormalities in Noonan's syndrome, such as pedal edema and pulmonary and intestinal lymphangiectasis, have been observed in children. Nonimmune hydrops represents one end of the spectrum of abnormalities seen in this syndrome.

Edema↗
PubMed2001

Does a 'notched' nuchal translucency indicate Down syndrome fetuses or other adverse pregnancy outcome?

The aim of the present study was to assess the sonographic contour of the increased nuchal translucency (NT) and to correlate this with pregnancy outcome. Fifty sonographic images of fetuses with increased NT [> 95th centile thickness of the normal range for crown-rump length (CRL) between 38 and 84 mm] were retrospectively assessed. In all the cases a complete pregnancy and even infancy follow-up (< 36 months) was available. The NT appearances were subdivided into two forms: a 'notched' or 'uniform' appearance. The images were correlated with karyotype results [trisomy 21 (DS) vs euploid cases] and pregnancy outcome. Complicated outcomes were classified as being either DS fetuses, miscarriage or termination of pregnancy because of structural anomaly. Thus 30/35 (86%) of the euploid fetuses had a 'uniformly' increased NT, whereas 8/13 DS cases (62%) had a 'notched' appearance (Fisher's exact test, p = 0.004). Additionally, 27/29 fetuses (93%) which had an uneventful pregnancy outcome had a 'uniform' increased NT, whereas 12/26 (57%) of the fetuses which had adverse pregnancy outcome had a 'notched' appearance of their NT (Fisher's exact test, p < 0.001). Although it was not possible to correlate the sonographic data with post-evacuation microdissection findings, it is possible that a uniformly shaped, increased NT may be more representative of a developmental delay in a normal fetus. Conversely, a 'notched' nuchal surface may represent abnormal lymphatic or cardiovascular development more commonly seen in DS fetuses.

Adult↗
PubMed2002

Radiological features of late-onset lymphoedema in Noonan's syndrome.

Noonan's syndrome is a multiple congenital anomaly syndrome with diverse manifestations. Lymphatic abnormalities occur in less than 20% of patients. We report a 14-year-old boy who presented with swollen lower limbs and dysmorphic features characteristic of Noonan's syndrome. The radiological features of this unusual case of late-onset lymphoedema in association with Noonan's syndrome are presented.

Adolescent↗
PubMed1991

Lymphoedema/hypercalcaemia syndrome mediated by parathyroid-hormone-related protein.

A 24-year-old woman presented with a cryptogenic, hypercalcaemic syndrome with metastatic pulmonary calcification and leg and massive breast enlargement and bilateral chylous pleural effusions compatible with a generalised lymphatic abnormality. The patient's serum parathyroid hormone-related protein concentration was very high at a time when she was significantly hypercalcaemic, implying that the hypercalcaemia was mediated by this protein.

Adult↗
PubMed2006

[The lymphatic malformations: clinical aspects and evolution].

Lymphatic malformations (LM) are the most frequent vascular malformations. There are three types of lesions involving lymphatic development that must be included in LM: vascular anomalies and knots (truncal malformations [TLM]); cystic anomalies, superficial or deep, uni- or multicystic (extratruncal malformations [ETLM]) and hemolymphatic anomalies which combine venous, arterial, or capillary malformations with LM. ETLM can be ubiquitously distributed but most are located in the cervical or axillary regions. Most ETLM are diagnosed at birth and in 80-90% of the cases before the age of 2. The clinical aspects are extremely variable: superficial ETLM (vesicular) and deep ETLM, localised or diffuse, mono- or multicystic. TLM are generally located on a lower limb with neonatal lymphatic oedema (often in a polymalformation context). All forms of the hemolymphatic combination can be identified. They are generally located on the limbs and are often unilateral. They are usually sporadic but can also be can be found in polymalformation syndromes (Klippel-Trénaunay, Parkes-Weber, Protée, Maffucci). ETLM generally tend to increase in volume and spread with age with stabilisation at puberty. They do not tend to spontaneously regress. Specific local complications can have serious consequences. They are linked to haemorrhaging, infections and compression phenomena. There can also be complications such as skeletal and soft tissue hypertrophy.

Age Factors↗
PubMed1997

Community respiratory viruses: organ transplant recipients.

Respiratory infections are common after solid organ transplantation, but the significance of community respiratory viral infections in this patient population has not been determined. Review of the literature indicates that infection of organ transplant recipients by community respiratory viruses can result in significant morbidity with some associated mortality. These viruses include respiratory syncytial virus (RSV), parainfluenza virus (PIV), influenza virus, and adenovirus. As in normal hosts, infection of organ transplant recipients by these viruses can result in limited upper respiratory tract symptoms, such as rhinorrhea, cough, and fever. Immunocompromised patients can also have lower respiratory tract infection, resulting in bronchiolitis, pneumonitis, respiratory failure, and death. The highest incidence of infection with these viruses is reported in lung transplant recipients, with an incidence up to 21%. In addition to the effects of the usual immunosuppressant regimen, lung transplant recipients have altered lung immunity due to impaired ciliary clearance, poor cough reflex, and abnormal lymphatic drainage, predisposing these patients to lower respiratory tract infections. Of additional importance to organ transplant recipients is the correlation of organ rejection to recent viral infections with these agents. Influenza A and B, PIV, and adenovirus have been reported to be associated with acute rejection in renal transplant recipients. Diagnosis of these infections is often made by positive respiratory cultures, often with a delay between symptom onset and diagnosis. Clinical trials of antiviral agents in this patient population have not been carried out, and treatment has often been limited to severe, life-threatening cases.

Adenoviridae↗
PubMedDate not supplied

[Ivermectin].

Ivermectin is a semi-synthetic derivative of a macrocyclic lactone. It causes paralysis in many nematodes and arthropodes because of its effect on ion-channels in cell membranes. Ivermectin was first used in veterinary medicine. In man, it was shown to be microfilaricid against Onchocerca volvulus. Most of the adverse reactions following treatment were mild, without the systemic and ocular side effects usually complicating diethylcarbamazine therapy. In endemic areas after repeated administration of ivermectin, a dramatic reduction in dermal microfilarial load was observed, resulting in a decrease in transmission. There was a significant decrease in the prevalence of anterior segment lesions in the eyes and acute onchocercal skin disease. Moreover, ivermectin also exhibited microfilaricidal activity against Wuchereria bancrofti and Brugia malayi. Annual mass treatment with a single dose of diethylcarbamazine alone, or associated with ivermectin, was initiated in endemic areas for lymphatic filariasis. The preliminary results showed a decrease in the reservoir of microfilariae and rate of transmission, a reduction in the frequency of clinical lymphatic abnormalities due to bancroftan filariasis. In Loa loa infections ivermectin decreases microfilaremia, but serious adverse events such as encephalopathy can be induced in patients with high rate of microfilaremia. Ivermectin appears to be the drug of choice in Strongyloides stercoralis infections, a single dose is highly effective with less frequent side effects than thiabendazole. Oral ivermectin is an alternative to topical scabicides, it appears as effective as local treatment for common scabies, but there are few comparative studies. The best indications for ivermectin in this ectoparasitic infection could be the outbreak in institutions and crusty scabies, but in association with topical treatment. The precise position of this agent in the treatment of scabies remains to be specified. Ivermectin is also affective in the treatment of ascariasis and cutaneous larva migrans. It could also be a promising treatment for head lice.

Antiparasitic Agents↗