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PubMed1979

Extralobar pulmonary sequestration: analysis of 15 cases.

Extralobar pulmonary sequestrations occurring in 15 patients were studied. Twelve of the lesions were discovered during the first day of life; all of these patients died. The lesions occurred more often in male patients (11 cases). Polyhydramnios was present in four infants, and these four plus an additional infant had localized or generalized edema. Associated congenital anomalies were present in ten patients, including three examples each of diaphragmatic hernia and bronchogenic cyst. In one case the extralobar pulmonary sequestration was composed entirely of a congenital cystic adenomatoid malformation. In one other case, congenital adenomatoid malformation was present in a nonsequestered portion of lung. Significant lymphatic dilatation was found in four cases, two of which resembled congenital lymphangiectasis. Hyaline membranes, present in the normal lungs of three preterm infants, were absent in extralobar pulmonary sequestrations.

Aorta, Thoracic↗
PubMed2001

Net-targeted mutant mice develop a vascular phenotype and up-regulate egr-1.

The ternary complex factors (TCFs) Net, Elk-1 and Sap-1 regulate immediate early genes through serum response elements (SREs) in vitro, but, surprisingly, their in vivo roles are unknown. Net is a repressor that is expressed in sites of vasculogenesis during mouse development. We have made gene-targeted mice that express a hypomorphic mutant of Net, Net delta, which lacks the Ets DNA-binding domain. Strikingly, homozygous mutant mice develop a vascular defect and up-regulate an immediate early gene implicated in vascular disease, egr-1. They die after birth due to respiratory failure, resulting from the accumulation of chyle in the thoracic cage (chylothorax). The mice have dilated lymphatic vessels (lymphangiectasis) as early as E16.5. Interestingly, they express more egr-1 in heart and pulmonary arteries at E18.5. Net negatively regulates the egr-1 promoter and binds specifically to SRE-5. Egr-1 has been associated with pathologies involving vascular stenosis (e.g. atherosclerosis), and here egr-1 dysfunction could possibly be associated with obstructions that ultimately affect the lymphatics. These results show that Net is involved in vascular biology and egr-1 regulation in vivo.

Animals↗
PubMed1996

Penetration of M cells and destruction of Peyer's patches by Yersinia enterocolitica: an ultrastructural and histological study.

Yersinia enterocolitica is enteropathogenic for man and rodents. Previous studies provided evidence that Y. enterocolitica invades the lymphoid follicles of the Peyer's patches (PP) of the small intestine. In this study Y. enterocolitica-induced tissue alterations of the follicle-associated epithelium (FAE) and the underlying PP tissue were analysed by scanning (SEM) and transmission electron microscopy (TEM) as well as by conventional histological examination. For this purpose, an experimental mouse infection model including orogastric infections as well as ileal loop experiments were used. A rapid and selective colonisation of the FAE after orogastric yersinia infection was observed by SEM. TEM studies confirmed that Y. enterocolitica adhered closely to the FAE including M cells and enterocytes. Histological studies and TEM revealed that Y. enterocolitica selectively invaded the PP via M cells but not via other cells of the FAE. One day after Y. enterocolitica infection the FAE was altered and small micro-abscesses comprising yersiniae expressing the major outer-membrane protein YadA were observed immediately beneath the FAE. Adjacent villi were dilated from lymphangiectasis and transmigrating polymorphonuclear leucocytes (PMNL) were found within the epithelium. At 5-7 days after infection the FAE and parts of PP were destroyed. Profound alterations of the cyto-architecture of the PP were due to the enormous recruitment of PMNL. By day 5 after infection, abscesses were found in the mesenteric lymph nodes. However, TEM studies revealed evidence that Y. enterocolitica may disseminate from the PP not only via the lymphatics but also by invasion of blood vessels. Taken together, the results of this study demonstrate that the FAE is the primary site of host-pathogen interaction in Y. enterocolitica infection and that this pathogen penetrates M cells and subsequently induces destruction of the PP.

Animals↗
PubMed1993

A case of acquired lymphangioma of the vulva.

A 46-year-old woman developed multiple papules on the right labium majus. A histological examination revealed acanthotic epidermis and dilated vessels in the papillary dermis. The clinical and histological features were compatible with those of lymphangiectasis or acquired lymphangioma of the vulva, which occurs after surgery or irradiation for cervical cancer. This patient, however, had no such past history. Acquired lymphangioma of the vulva arising without obvious causes seems to be unusual.

Endothelium↗
PubMed1979

Acute ureteral obstruction secondary to bullous cystitis of the trigone: report of 2 cases.

Two boys presented with acute bullous cystitis limited to the trigone and periureteral zone and producing marked but transient acute ureteral obstruction. One patient was anuric and likely would have died without intervention. Cystoscopy in both cases showed localized bullous edema of the bladder mucosa; biopsy showed acute inflammation, mucosal edema, and lymphangiectasis. Radiological findings included distal ureteral obstruction and numerous nodular filling defects in the bladder, caused by the edema. The etiology of the cystitis could not be determined in either case.

Acute Disease↗
PubMed1995

An adult case of lymphangiomatosis of the mediastinum, pulmonary interstitium and retroperitoneum complicated by chronic disseminated intravascular coagulation.

Pulmonary lymphangiomatosis is a rare clinical and pathological entity which is distinct from lymphangiomyomatosis and from pulmonary lymphangiectasis. We report a case of a 20 year old man with diffuse lymphangiomatosis involving the mediastinum, lungs and retroperitoneum. The patient's intrathoracic lymphangiomatosis produced restrictive and obstructive impairment of his pulmonary function, but did not result in chylothorax or chylopericardium, which are two common manifestations of the disease. The patient's clinical course was remarkable for the concomitant development of chronic disseminated intravascular coagulation (DIC). Lymphangiomatosis involving both the mediastinum and pulmonary parenchyma is rare, and DIC is a very unusual complication of lymphangiomatosis.

Adult↗
PubMed1981

Intrauterine fetal cystic hygromas: sonographic detection.

The sonographic characteristics of intrauterine cystic hygromas are described. A thin-walled, multiseptated cystic structure near the fetal head and neck and eccentrically situated with respect to the long axis of the fetus is characteristic. Another associated finding is intradermal lymphangiectasis which, when present, is though to be pathognomonic of this condition. The differential diagnosis includes neural tube defects, unusually situated umbilical cord, gastroschisis and omphalocele, an empty second gestational sac, and amniotic bands. Four cases of intrauterine fetal cystic hygroma are reported and their echographic findings illustrated.

Diagnosis, Differential↗
PubMed2001

A case of del(13)(q22) with multiple major congenital anomalies, imperforate anus and penoscrotal transposition.

"13q-"syndrome is known to have widely variable manifestations, including retinoblastoma, mental & growth retardation, malformation of brain & heart, anal atresia, and anomalies of the face and limbs. Here we report a case of del(13)(q22) with multiple major congenital anomalies for the first time in Korea. The patient was born at 36(+4) weeks of pregnancy by caesarian section. Birth weight was 1490g. On examination the following features were noted: - imperforate anus, ambiguous genitalia (bifid scrotum, penoscrotal transposition, hypospadia), syndactyly of toes, absence of thumbs, abnormal facies (dolichocephaly, telecanthus, large low set ears, saddle nose, high arched palate, micrognathia). Neurocranial ultrasonography showed atrophy of the corpus callosum and multiple calcifications. He died at 14 days. Post-mortem autopsy findings showed cholestasis and fatty metamorphosis of liver, abnormal lobulation (Rt:2, Lt:1) and lymphangiectasis of the lung, VSD, ASD, PDA of heart, and acute tubular necrosis of kidney. Cytogenetic studies was confirmed to 46,XY,del(13) (q22) by Giemsa banded chromosomes from peripheral blood lymphocytes.

Abnormalities, Multiple↗
PubMed2001

Lymphatic filariasis in children: adenopathy and its evolution in two young girls.

Lymphatic filariasis is a widespread infectious disease of children in endemic areas, but little is known about the early lymphatic damage in children and its evolution, either with or without treatment. Two girls (ages 6 and 12 years) from a Wuchereria bancrofti endemic region of Brazil presented with chronic inguinal adenopathy. Neither had microfilaremia. By ultrasound both were shown to have living adult worms in their enlarged inguinal nodes and had occult local lymphatic damage (lymphangiectasis). One girl spontaneously developed acute adenitis in the affected node prior to any intervention; this adenitis resolved within 10 days and was associated with the progressive disappearance over 45-90 days of all local abnormalities detectable by ultrasound. In the other child, after treatment with a single dose of diethylcarbamazine (DEC), the same clinical picture of transient adenitis and resolving abnormalities (detectable by ultrasound) occurred. These findings demonstrated filariasis as the cause of adenopathy in children, and also both spontaneous and treatment-induced worm-death, with subsequent reversal of lymphatic abnormalities.

Animals↗
PubMedDate not supplied

[Diagnostic value of jejunal biopsy].

Trying to find out the clinical elements that define the precise indication ofr a jejunal biopsy, 28 children with several pathological conditions were studied. They were 12 cases of kwashiorkor, 11 cases with chronic diarrhea, two cases of chronic pancreatitis, 2 cases with ferropenic anemia resistant to the oral treatment with iron and one case of chilous ascitis. It is concluded that only in those cases in which the biopsy is the precise medium for diagnosis is where it would be indicated, such as intestinal lymphangiectasis or in those cases with signs or evidence of malabsorption without diarrhea. The chronic diarrhea per se does not seem to be a formal indication for biopsy.

Adolescent↗
PubMed1999

[MRI features of lymphedema of the lower extremity: comparison with lymphangioscintigraphy].

OBJECTIVE: To investigate the features of chronic lymphedema of the extremity. METHODS: Magnetic resonance imaging (MRI) and lymphangioscintigraphy (LAS) examinations were performed on 12 patients with peripheral lymphedema. RESULTS: MRI characteristically showed diffusive subcutaneous edema, reticular lymphangiectasis and "channels" with sequestered lymph. MRI scan clearly displayed the proliferative and extended lymphatic vessels, trunks and chylocyst. LAS showed dermal diffusion (dermal backflow) or retention at the injection site of the tracer with poorly defined lymphatic trunks and delayed or no visualization of regional lymph nodes. CONCLUSION: MRI can visualise peripheral lymph trunks, lymph nodes and soft tissue. LAS is more helpful in ascertaining the condition of obstruction of lymphatic system. Using these two imaging modalities together is helpful for anatomical diagnosis and delineating the disarranged pattern of lymphedema.

Adolescent↗
PubMed2002

Antenatal diagnosis and obstetric management of cystic hygroma occurring in twin pregnancies. A case report.

The lymphatic system has an early development in the embryo. Usually, its development is complete by the 40th postconceptional day. If this connection fails to develop, lymphatic stasis and dilatation of the lymphatic channels may occur, causing a number of pathologies such as: lymphangiomas, lymphangiectasis and cystic hygromas. Prenatal diagnosis can be made during the first trimester of pregnancy by ultrasonographic examination. A case of a twin pregnancy associated with cystic hygroma and bilateral hypoplasia of lower and upper limbs of both foetuses without chromosomal abnormalities is reported.

Adult↗
PubMed2002

Primary Sjögren's syndrome with protein-losing gastroenteropathy: report of two cases.

Protein-losing gastroenteropathy is a rare complication in autoimmune diseases, especially in Sjögren's syndrome. We report two cases of primary Sjögren's syndrome, one in a 37-year-old female and another in a 50-year-old female, both of whom presented with peripheral edema. Protein-losing gastroenteropathies of the stomach and small intestine in the first patient and of the small intestine only in the second patient were demonstrated by abdominal Tc-99m labeled albumin abdominal scintigraphy and pathologic findings. Results of gastrointestinal tract biopsies from both patients showed chronic inflammatory cell infiltrations without lymphangiectasis or vasculitis. The patients were successfully treated with corticosteroids. Results of follow-up Tc-99m labeled albumin scintigraphy were well correlated with clinical improvement and the increase in serum albumin. Sjögren's syndrome should be considered as a cause of protein-losing enteropathy. Tc-99m labeled albumin abdominal scintigraphy is helpful in diagnosing the condition, locating the protein-losing sites, and monitoring the treatment outcome, especially in cases where protein loss occurs in the stomach.

Adult↗
PubMed1964

CURRENT CONCEPTS OF THE PATHOGENESIS AND PATHOLOGY OF INFLAMMATORY LESIONS OF THE INTESTINE.

The histopathologic lesions of regional enteritis and ulcerative colitis, particularly in their early stages, are distinct and distinguishable, irrespective of the sites that are involved. Regional enteritis is characterized by lymphangiectasis, lymphedema, lymphoid hyperplasia, and granulomatous inflammation of the submucosal and subserosal layers of intestine, whereas chronic ulcerative colitis is an exudative, ulcerative disorder of the mucosal layer that commences with "crypt abscesses" and only in its later stages progresses to deeper coats of the wall.Electron microscopy of a rectal biopsy from a juvenile patient with chronic ulcerative colitis for five years disclosed a labyrinthine system of clefts and compartments between columnar, mucosal epithelial cells. Regenerated colonic epithelial cells were of primitive, germinal type and featured a "vesicular" rather than a "goblet" pattern of mucus secretion. Clusters of small "clavate fimbriae" projected from the tips of microvilli. Each of these newly recognized substructures measured 30 to 60 mmu. in diameter, and was enclosed by a tri-laminar "unit membrane", derived from the surface plasma membrane of the cell.

Adolescent↗
PubMedDate not supplied

[Alpha 1-antitrypsin as an endogenous marker of protein-losing enteropathies].

A novelty of the present studies is the use of alpha 1-antitrypsin (A-1--AT) as an endogenous marker of enteric protein loss. Enteric clearance of alpha 1-antitrypsin was determined in 10 patients with the symptoms of PLE, and in 6 healthy individuals. Alpha 1-Antitrypsin concentration has been assayed in single, random samples of feces collected from 42 patients and 12 healthy individuals (normal values: 1.31 +/- 0.72 mg/g of feces). Markedly increased enteric clearance and A-1-AT concentrations in single, random samples of feces have been found in patients with enteric lymphangiectasis, Crohn's disease, ulcerative colitis, and constrictive pericarditis, slightly lower in coeliac, chronic diarrhoea, nonspecific hemorrhagic colitis, esophagitis, lambliasis, hypogammaglobulinemia, Wiskott-Aldrich syndrome, Rendu-Osler-Weber syndrome, hepatitis in newborn, and Gilbert's disease. Statistically significant positive clearance has been noted (r = 0.997; p less than .001). A single assay of A-1-AT in feces is simple, repeatable, and sensitive technique in the diagnosis and evaluation of these diseases in which the symptoms of enteric protein loss are seen.

Adolescent↗
PubMed1992

Perinatal manifestations of maternal yellow nail syndrome.

A term female firstborn infant had unexplained nonimmune fetal hydrops and recurrent left chylothorax at 4 weeks of age. A few months before conception, her mother had had acute dystrophic nail changes and is being treated for recurrent sinusitis, bronchiectasis, and a deficiency of serum IgG2. We suggest that they both suffer from a dominantly inherited congenital lymphedema syndrome known as 'yellow nail dystrophy.' Prenatal manifestation of this disorder has not been reported previously. The child's anthropometric and neurological development was normal at 1 year of age, whereas mild ankle edema and marbling of the skin of the limbs were salient clinical findings. Inherited lymphedema leading to nonimmune fetal hydrops also has been recognized in chromosomal disorders, Noonan's syndrome, multiple pterygium syndrome, pulmonary lymphangiectasis, and mixed-vessel lymphatic dysfunction. Indicators of parental lymphedema are not on record in those instances.

Chylothorax↗
PubMed2005

[CD11b-positive cells expression in rectal mucosa from ovalbumin sensitized and challenged rabbits].

Rabbit MAC-1 receptor, homologue to human CD11b is present in macrophages. The aim of the study was to determine quantitative and distributive modifications of CD11b-positive cells that participate in immune response at rectal mucosa, in an animal model of mucosal immunity. New Zealand rabbits were divided into three groups. G1: control; G2: ovalbumin (OVA) sensitized; G3: OVA-senstitized and rectal challenged. Animals were subcutaneously sensitized twice with 70 microg OVA and 30 ml aluminium hydroxide in 2 ml saline solution. Rectal challenge was developed with a solution of 50 mg OVA in 5 ml saline solution. Sensitized groups (G2 and G3) showed a positive PCA (Passive Cutaneous Anaphylaxis) at 1/160 fold dilutions. In G3 we observed a patchy mucosal edema, lymphangiectasis and eosinophil leucocyte infiltration. Cells were counted as the number of cells per high power field. G1: 9.64 (SE 0.22); G2: 18.10 (SE 0.09) and G3: 23.60 (SE 0.29). (G2 vs G1 p < 0.001; G3 vs G1 p < 0.001; G3 vs G2 p < 0.001). We conclude that there is a close relationship between the food antigen OVA penetration (after challenge) and the increase of CD11b positive cells in rectal mucosa. This fact could be due to the cellular influx to the inflammatory site by the action of chemotactic factors released after challenge.

Animals↗
PubMed1991

[A rare interstitial lung disease].

Pulmonary lymphangioleiomyomatosis (p.l.) is a rare disease of unknown etiology, and restricted to fertile women. It is characterized by a nodular proliferation of smooth muscle cells in the peribronchial, perivascular and perilymphatic lung tissue, accompanied by cystic dilations of the alveoles, rupture of the alveolar wall, lymphangiectasis, and septal collagen fiber deposition. Radiological-alterations range from enhanced interstitial shadowing to honey comb lung. Common clinical symptoms are progressive dyspnea, pneumothorax, chylous pleural effusion and hemoptysis. Here we present the case of a 43 years old woman, undergoing nephrectomy because of hamartoma of the left kidney, with recurrent pneumothorax and progressive dyspnea, verifying the diagnosis of p.l. by open lung biopsy. Pathogenesis of the disease, differential diagnosis and possible therapeutic approaches are discussed.

Adult↗