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At least 271 records · Page 15Linked to original sources
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↗
PubMed1978

Nonspecific peritesticular fibrosis manifested as testicular enlargement.

Nine cases of peritesticular fibrosis initially appeared to be enlargement of the testes. Seven patients under went orchiectomy and two had biopsy specimens taken of the grossly thickened tunica albuginea. In six patients, a hydrocele was present with intramural fibrosis and minimal chronic inflammation. Active epididymitis and chronic interstitial orchitis were present in two patients, with some spread of the inflammation into the fibrotic peritesticular tissues. One case, without associated hydrocele or epididymitis, showed dense fibrosis of the peritesticular tissues with focal edema, lymphangiectasis, and minimal perivascular round cell infiltration. This clinicopathological entity of diffuse peritesticular fibrosis is an unrecognized cause of benign testicular enlargement. The hypothesis is advanced that the primary process is a congenital hypoplasia of the peritesticular lymphatics progressing at various speeds into a state of chronic lymphedema and end stage noninflammatory fibrosis.

Adult↗
PubMedDate not supplied

Heterotopic pancreatic tissue presenting as a solid and cystic lung lesion: a very unusual bronchopulmonary foregut malformation.

We describe the history and lung pathology of a premature female infant, who presented with respiratory distress immediately after birth. A thoracic computerized tomography scan showed abnormalities suggestive of congenital cystic adenomatoid malformation of the left lung. In addition, echocardiography revealed a tetralogy of Fallot. A left thoracotomy was performed and the lower lobe of the left lung was removed. Despite intensive supportive therapy, pulmonary hypoperfusion resulted in severe hypoxemia and death. Gross and microscopic analysis of the resected lobe revealed a partly cystic and solid lesion with multiple bronchus-derived cysts and an exuberant multifocal proliferation of glandular tissue, resembling bronchial glands, mixed with heterotopic cartilage surrounding ducts. Immunohistochemical analysis showed the presence of chromogranin A-reactive islet-like structures amidst exocrine tissue showing trypsin and chymotrypsin immunoreactivity, establishing the diagnosis of pulmonary pancreatic heterotopy. In the remaining pulmonary parenchyma, there were secondary changes consistent with partial obstruction and lymphangiectasis which was attributed to the presence of the cardiac malformation. To our knowledge, this is only the fourth reported case of heterotopic pancreatic tissue in the lung, and the first case where this bronchopulmonary foregut anomaly is not associated with a enteric duplication.

Abnormalities, Multiple↗
PubMed2004

Panniculitis in a patient with mixed connective tissue disease.

A 51-year-old woman who had been suffering from mixed connective tissue disease (MCTD) for 8 years developed an erythematous rash with pain and tenderness on her left leg. A skin biopsy revealed septal panniculitis with multiple lymphangiectasis. No vasculitis was observed. An increase in her prednisolone dose from 5 mg to 20 mg/day led to an improvement in these lesions. Panniculitis is very rare in MCTD. The clinical significance of panniculitis in MCTD is also discussed.

Journal Article↗
PubMed1979

Histologic diagnosis of diseases of malabsorption.

The diagnoses which may be arrived at by examination of peroral small bowel mucosal biopsy specimens are presented. Celiac sprue, unclassified sprue (refractory sprue), infectious gastroenterititis, stasis syndrome and kwashiorkor have a severe mucosal lesion. Other clinical conditions are required to establish the diagnosis in these diseases. A number of diseases have specific diagnostic features. Included are Whipple's disease, abetalipoproteinemia, collagenous sprue, primary intestinal lymphoma, eosinophilic gastroenteritis, giardiasis, coccidiosis, strongyloidiasis, lymphangiectasis and the intestinal immunodeficiency diseases. Mucosal abnormalities may be present in other diseases but the diagnoses are usually made on other criteria than small bowel biopsy. These include vitamin B12 or folic acid deficiency, Crohn's disease, gastrinoma, acrodermatitis enteropathica, amyloidosis, chronic granulomatous disease, lipid storage diseases, histoplasmosis, capillariasis, cytomegalovirus infection, schistosomiasis and macroglobulinemia.

Biopsy↗
PubMed1977

Functioning and nonfunctioning cysts of the adrenal cortex and medulla.

Adrenal cysts are rare clinical and pathologic entities. The vast majority are minute in size, unilateral, and found in females at autopsy. They come to the physician's attention when they produce symptoms or are identified on x-ray studies. When symptomatic, they generally produce lumbar discomfort and gastrointestinal symptoms, and occasionally are palpable by abdominal examination. There is no significant agreement as to cause and pathogenesis of the disease. The most common histologic types are lymphangiomatous endothelial cysts, secondary to lymphangiectasis, and the fibrous wall or hemorrhagic pseudocysts. In many patients preoperative diagnosis is now possible with high quality nephrotomography, ultrasonography, and adrenal arteriography. The presence of a suprarenal mass with peripheral or laminar calcification strongly suggests the presence of an adrenal pseudocyst. Selective biochemical studies are mandatory to rule out the presence of an occult cystic pheochromocytoma, and adrenal cortical tumor. Adrenal cysts must be differentiated from all space-occupying lesions of the upper abdomen. Surgical exploration is recommended in almost all patients for accurate diagnosis and to rule out malignant disease or occult pheochromocytoma. The choice of the surgical approach should be planned to provide for safe and adequate exposure, depending on the size and location of the lesion. Careful dissection with preservation of the adjacent kidney, liver, and pancreas should be performed.

Adrenal Cortex↗
PubMed1987

The pathology of lymphoreticular and genital tissues of silvered leaf monkeys (Presbytis cristata) experimentally infected with Wuchereria bancrofti.

The discovery of the silvered leaf monkey, Presbytis cristata, as a suitable experimental host for the human filarial parasite, Wuchereria bancrofti, opened the door for major advances in our understanding of the disease caused by this parasite. To study the pathogenesis of bancroftian filariasis in this model, 15 adult P. cristata which had been experimentally infected with 250 infective third-stage larvae of the parasite were examined. After inoculation with larvae, the monkeys were maintained under study for periods of 2 to 3 years, at which time all had achieved patent infections. At necropsy, Wuchereria-induced macroscopic lesions were not detected in experimentally infected monkeys. Microscopic findings included nematodiasis, microfilariasis, lymphadenitis, lymphangiectasis, perilymphangitis, splenitis, orchitis, periorchitis, epididymitis and funiculitis. Sections of normal adult worms were most often found in lymphatic vessels near lymph nodes, or in the lymphatics of the male genital system. These worms caused microfiliariasis in some regional lymph nodes. Inflammatory responses to filariae were most prominent in proximity to degenerated and dead worms, whereas intact, normal appearing adult worms elicited only minimal cellular response.

Animals↗
PubMed1988

Intracellular iron storage and the pathogenesis of paratuberculosis. Comparative studies with other mycobacterial, parasitic or infectious conditions of veterinary importance.

The distribution of iron and mycobacteria was examined in the intestinal tract of ruminants with naturally-occurring M. paratuberculosis infection and compared with mycobacterial infections in several species. This distribution was compared with that of iron in chronic lesions caused by other microbial or parasitic agents. In the clinical form of paratuberculosis in cattle, sheep and goats there was marked lymphangiectasis and a high proportion of the granulomatous lesions contained siderotic macrophages with a high mycobacterial content. In cattle with preclinical lesions of granulomatous enteropathy, the greatest number of acid-fast organisms was present in siderotic, non-differentiated, ileo-caecal macrophages; concurrent mast cell-associated allergic enteropathy was also apparent in the duodenum, proximal and mid-ileum of most animals. In paratuberculosis-affected herds, a high proportion of non-productive cows were without classical granulomatous change but had cultural or immunological evidence of M. paratuberculosis infection and similar allergic catarrhal enteropathy of the upper intestinal tract. Interstitial haemorrhage of the ileocaecal valve, with the accumulation of haemosiderin and ferritin in undifferentiated macrophages was observed in some of these cattle and also in others with experimentally-induced copper deficiency and acute ostertagiasis. Colonisation of the ileo-caecal or caecal glandular crypts by large, apparently saprophytic acid-fast organisms indicated regional tolerance to such organisms in all cattle. In other mycobacterioses such as bovine or avian tuberculosis, undifferentiated, siderotic macrophages containing mycobacteria were also seen in early granulomas, but epithelioid and giant cell differentiation invariably led to the disappearance of intracellular iron and a reduction in mycobacterial numbers. In possums in which epithelioid and giant cells did not occur in response to M. bovis infection, siderosis persisted in many macrophages and overwhelming mycobacterial multiplication occurred. These studies indicate that, in most infections with mycobacteria, differentiation of macrophages radically reverses their iron acquisitive properties, creating an intracellular environment unsuitable for mycobacterial multiplication. It seems likely that allergically mediated microvascular haemorrhage, local tolerance of commensal mycobacteria and attenuation of the macrophage siderosis reversal mechanism provide unique conditions for early, uninhibited, intracellular multiplication of M. paratuberculosis in the ileo-caecal valve of certain mature ruminants.

Animals↗
PubMed1993

Diffuse pulmonary lymphangiomatosis.

Angiomatous lesions of the lung are uncommon and not well characterized. We describe nine cases of a distinct lymphatic vascular lesion that we have termed "diffuse pulmonary lymphangiomatosis." Extrathoracic lymphangiomatous lesions were not identified. The patients comprised seven males and two females with a mean age at presentation of 10 years (age range, 1 month to 33 years). Six patients were younger than 10 years of age. Symptoms included "wheezing" or "asthma" (44%) and dyspnea (22%) present for 3 months to 20 years. Chest radiographs showed bilateral interstitial infiltrates, often greatest in the lower lobes. Pleural or pericardial effusions were present or developed in six patients. Pulmonary function tests showed in two patients and mixed obstruction and restriction in five patients. Open lung biopsies (nine patients) and autopsy (one patient) showed anastomosing endothelial lined spaces along pulmonary lymphatic routes (especially pleural and interlobular septal) accompanied by asymmetrically spaced bundles of spindle cells, which were prominent in six cases. Hemosiderin deposition often was present in the spindle cell areas and in the adjacent lung. The lining cells were positive for factor VIII-related antigen (eight cases) and Ulex europaeus I agglutinin (four cases). The spindle cells were reactive with antibodies to vimentin (nine cases), desmin (six cases), actin (seven cases), progesterone receptor (seven cases), and proliferating cell nuclear antigen (one case); they were negative for estrogen receptor (seven cases), keratin, (eight cases), and HMB-45 (eight cases). Diffuse pulmonary lymphangiomatosis was progressive in eight cases and was most aggressive in the youngest children. Two of the children have died, one of pulmonary hemorrhage and the other after heart-lung transplantation. Diffuse pulmonary lymphangiomatosis is distinct from lymphangiectasis, lymphangioleiomyomatosis, pulmonary capillary hemangiomatosis, Kaposi's sarcoma, and kaposiform hemangioendothelioma. Due to its distribution and histology, a lymphatic origin of the lesion is favored.

Adolescent↗
PubMed1992

Magnetic resonance imaging in human lymphedema: comparison with lymphangioscintigraphy.

Magnetic resonance (MR) imaging and isotope lymphography (lymphangioscintigraphy, LAS) was done in 32 patients with peripheral lymphedema (19 primary and 13 secondary). MRI characteristically showed diffuse dermal and subcutaneous edema, a nonedematous, occasionally hypertrophied skeletal muscle compartment, variability in regional lymph node size and appearance depending on the underlying clinical disorder, serpiginous "channels" or "lakes" consistent with dermal collateral lymphangiectasis and sequestered lymph, and increased subcutaneous fat. In contrast, LAS showed dermal diffusion ("backflow"), cross-over with retrograde tracer backflow (reflux), delayed tracer transport, and depending on the cause of lymphedema (i.e., primary or secondary), discrete or poorly defined lymph trunks (tracer "bands") and delayed or nonvisualization of regional lymph nodes. Although not a first-line clinical test, MR particularly in conjunction with LAS noninvasively provides accurate anatomical definition of the peripheral lymphatic system. In contradistinction to LAS, MR can visualize lymph trunks, nodes, and soft tissues proximal to sites of lymphatic obstruction. Together these imaging modalities may substitute for conventional oil contrast lymphography in the evaluation of the pathogenesis and evolution of most lymphologic disorders.

Adolescent↗
PubMed2005

Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature.

The authors present the case of a 48-year-old man with diffuse pulmonary lymphangiomatosis. This rare lymphatic disorder is characterized by proliferation of anastomosing lymphatic vessels varying in size. Clinical presentation and imaging findings are highly suggestive. Bronchoscopic examination of this patient showed, for the first time to our knowledge, vesicles disseminated throughout the bronchial tree. Histopathological examinations are necessary to differentiate lymphangiomatosis from lymphangiectasis. The diagnosis can be made by transbronchial biopsy without performing open lung biopsy which was, until now, considered necessary for diagnosis.

Biopsy, Needle↗
PubMed2006

Histopathological and immunohistochemical investigations of hepatic lesions associated with congenital portosystemic shunt in dogs.

Canine livers with congenital portosystemic shunt were investigated histopathologically and immunohistochemically before and 8-272 days after partial ligation of the shunt. Lesions included hypoplasia of portal veins, arteriolar and ductular proliferation, lymphangiectasis, mild to moderate fibrosis, fatty cysts, and mostly mild hepatocellular damage with frequent atrophy and steatosis, regardless of the location of the shunting vessel. Perisinusoidal hepatic stellate cells (HSCs) in normal canine liver expressed alpha-smooth muscle actin (alpha-SMA), but no desmin. In altered livers, however, raised expression of alpha-SMA was detected, together with expression of desmin, in varying numbers of HSCs. This was interpreted as a sign of cellular proliferation and transformation to myofibroblast-like cells. Additionally, there was an obvious perisinusoidal increase of several extracellular matrix components. Postoperative biopsy samples showed basically the same lesions as those of pre-operative samples, except that signs of resolution of hepatic changes were apparent.

Animals↗
PubMed1997

Impairment of left ventricular function by acute cardiac lymphatic obstruction.

OBJECTIVES: We performed the following study to define the effects of acute cardiac lymphatic obstruction on left ventricular (LV) systolic and diastolic function. METHODS: Cardiac lymphatic obstruction was created in 8 pentobarbital-anesthetized dogs by identifying (Evans blue) and ligating the right and left epicardial lymphatics, the afferent and efferent lymphatics associated with the pretrachael and cardiac lymph nodes, and the thoracic duct. Left ventricular function was assessed by analysis of micromanometer-conductance catheter-derived LV pressure-volume relationships. Contractility was assessed by preload recruitable stroke work (PRSW). The active and passive phases of LV relaxation were assessed by the time constant o isovolumic relaxation (tau) and the end-diastolic pressure-volume relationship (stiffness), respectively. RESULTS: PRSW decreased significantly and tau increased significantly from baseline at 1, 2, and 3 h after cardiac lymphatic obstruction (n = 8), but stiffness did not change. Cardiac lymphatic obstruction had similar effects on LV function in a group of autonomically blocked dogs (n = 5). Left ventricular function did not change in sham treated controls (n = 8). Cardiac lymphatic obstruction induced a significant increase in LV wet/dry weight ratios (3.58 +/- 0.01) when compared to the control group (3.53 +/- 0.02). Histopathology of the myocardium in the lymphatic obstruction groups revealed significant lymphangiectasis and increased interstitial spacing when compared to controls. CONCLUSIONS: Acute cardiac lymphatic obstruction depresses contractility and active relaxation and causes mild LV myocardial edema, but does not alter diastolic stiffness.

Acute Disease↗
PubMed2006

An approach for using full thickness skin grafts for complex penile surgeries in children.

PURPOSE: Many problems in pediatric urology derive from a paucity of penile skin resulting from prior surgical interventions. While hypospadias surgery is most often responsible for creating this problem, excessive circumcision also can leave a patient with too little skin to cover the penis. To our knowledge we describe the first series of pediatric patients in whom FTSGs were used in a variety of difficult circumstances where penile skin was lacking. MATERIALS AND METHODS: We retrospectively studied a cohort of 11 children 2 to 13 years old who underwent urethral repair and adjunctive skin grafting due to circumcision injuries (4 patients), traumatic urethral injury (1) or congenital lymphangiectasis (1), or for congenital hypospadias with previous failed surgery (5). In our patients available penile skin was used to reconstruct the urethra, while full thickness inguinal skin grafts were fashioned to resurface the denuded penis following reconstruction. RESULTS: All patients underwent successful reconstruction and grafting. There were no intraoperative complications. There was 100% take of the grafts. Average followup was 23 months (range 3 weeks to 8.6 years). One patient had slight chordee at 6 years postoperatively, and 1 had development of a urethrocutaneous fistula at 8.6 years. All patients reported normal caliber urinary streams. CONCLUSIONS: Use of full thickness inguinal skin grafts to resurface the penis provided patients with an esthetically acceptable result, and where necessary allowed penile skin to be used for urethroplasty. This technique is useful and justifies consideration in appropriately selected patients.

Adolescent↗
PubMed1981

Pulmonary fibrosis of low calcium diet fed guinea pigs induced by the administration of a soluble immune complex.

Feeding animals a low calcium diet leads to lymphangiectasis of peribronchial lymphatic vessels, sometimes followed by overflow of lymphatic fluid into the surrounding tissue. Adding a soluble immune complex (SIC) to the diet, the peculiar diffuse interstitial pneumonia and finally pulmonary fibrosis were noted. Small honey-combed structures and sometimes histologic accommodation of epithelial lining cells were observed. Sometimes accumulation of leucocytes within honey-combed structures was seen.

Animals↗
PubMedDate not supplied

[Recurrent chylothorax -- a fifteen years long history].

The authors describe the clinical case of a 15 year-old adolescent with recurrent bilateral chylothorax leading to multiple hospitalizations. As in other cases described the diagnosis was made on the basis of classical clinical features and characteristic CT scan. Most cases of pulmonary lymphangiectasis, described in the literature, are lethal in the neonatal period. In this case the girl has a reasonable life quality, in spite of multiple hospitalizations. Lower limb lymphedema, facial anomalies (flat face, palpebral edema, tooth anomalies and small mouth) and mild development retardation allow us to consider our case as the first portuguese case of Hennekam Syndrome. Molecular studies will allow, in the future to explain most of the clinical manifestations and to include other patients in the same clinical entity.

Adolescent↗
PubMedDate not supplied

A rare case of interstitial del(1)(p34.3p36.11) diagnosed prenatally.

We report a very rare constitutional interstitial deletion of chromosome 1p defined within 1p34.3-36.11 bands with an intact pter. The diagnosis was made by standard cytogenetics and fluorescence in situ hybridization (FISH) studies on a cordocentesis specimen obtained at 21 weeks gestation. Termination of pregnancy was performed at 22 weeks gestation due to the ultrasound diagnosis of congenital heart disease. Autopsy confirmed congenital heart disease (cardiomegaly, Ebstein's anomaly, secundum atrial septal defect, subendocardial fibroelastosis), pulmonary lymphangiectasis, a high arched palate, short neck, and bilateral long proximally implanted thumbs. To our knowledge, this is the first case of del(1) (p34.3p36.11) diagnosed prenatally.

Abnormalities, Multiple↗