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PubMed1989

[The instrumental diagnosis of disseminated lesions of the lungs].

A study is presented of 52 patients with disseminated pulmonary lesions. Among them: histiocytosis X--17, alveolar microlithiasis--5, pulmonary phospholipoproteinosis--7, idiopathic hemosiderosis--14, primary pulmonary amyloidosis--1, primary diffuse lung tumours--8. All patients were subjected to complex bronchological examination including use of different biopsy transbronchial methods as well as open biopsy of the lung. The diagnostic value of the employed bronchial biopsies is discussed. A diagnostic algorithm of rarely met pulmonary disseminations has been developed. Recommendations on the use of different instrumental methods are formulated.

Adolescent↗
PubMed1989

[Familial occurrence of intracerebral cavernous angioma].

This article reported a familial occurrence of intracerebral cavernous angioma in four members of one generation diagnosed by X-ray CT, MRI or operative specimen. Case 1, a 34-year-old female, was examined just after an episode of sudden convulsive seizure. On examination, she had a cutaneous angioma without any neurological deficit. X-ray CT revealed a high density mass lesion in the left frontal lobe, and MRI demonstrated a mass lesion in the chronic stage with an old hematoma circumscribed by hypointensity ring indicating peripheral hemosiderosis. Complete excision was carried out and a diagnosis of cavernous angioma was made after histological examination. Case 2, the 37-year-old brother of Case 1, suddenly developed left hemiparesis and hypesthesia with severe headache. X-ray CT revealed a high density mass in the right parietal lobe and two other calcifications. The right parietal lesion was excised and a histopathological diagnosis of cavernous angioma with intracerebral hematoma was made. Case 3, the 49-year-old sister of Case 1, suddenly fell into a coma and was admitted immediately. X-ray CT revealed a large pontine hemorrhage. She died on the 4th day of hospitalization without operative treatment. Necropsy was not carried out. Case 4, the 39-year-old sister of Case 1, was asymptomatic, however, she was examined on the supposition of a familial occurrence of intracerebral cavernous angioma. On examination, it was found she had multiple cavernous angioma without any neurological deficit. X-ray CT revealed parietal intracerebral calcification. MRI demonstrated a mass lesion with peripheral hypointensity ring in the right parietal lobe, and another small lesion in the pons.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[Alveolar hemorrhage].

Alveolar hemorrhage (AH) is the result of diffuse bleeding into the acinar portion of the lung. Cardinal symptoms of AH include hemoptysis, dyspnea, alveolar filling opacities on chest roentgenogram, anemia and hypoxemia. However, AH is often misdiagnosed as pneumonia or pulmonary edema at the time of initial presentation. Isolated AH may occur but is more often seen in diffuse connective tissue diseases or in rapidly progressive glomerulonephritis. - At the Medical Clinic of the University Hospital of Zurich we have diagnosed AH in 18 patients (13 males, 5 females) over the last ten years (1978-1988). In 2 patients AH occurred as an isolated symptom: once due to occupational inhalation of fumes containing trimellitic anhydride, and once as so-called idiopathic pulmonary hemosiderosis. In 16 patients AH was associated with kidney disease, including the following disorders: vasculitis and collagen vascular disorders (9), rapidly progressive glomerulonephritis (4) and Goodpasture's syndrome (3). In 5 patients the presenting symptom was AH. 9 patients presented initially with renal symptoms and in 2 patients renal and pulmonary features occurred simultaneously. 7 patients died of the underlying disease or its complications. Both patients with isolated AH have survived. In the remaining 9 patients 1 required dialysis temporarily and 4 permanently. All patients except the one with AH due to inhalation of trimellitic anhydride were treated with immunosuppressive agents. - Since the pulmonary features are similar in each of the AH syndromes, diagnosis of the underlying disorder is heavily dependent upon pathologic evaluation of diseased extrapulmonary organs. Moreover, immunologic studies are essential. Failure to diagnose or treat AH syndromes in the early stages may have lethal consequences.

Adult↗
PubMed1989

[Iron chelation in children].

Iron-chelating treatment is indicated in all children on prolonged transfusion therapy (i.e., chiefly patients with thalassemia and Blackfan-Diamond anemia). The purpose of iron-chelating treatment is to prevent the development of manifestations of iron overload including cardiac hemosiderosis and insulin-dependent diabetes mellitus (which are two potentially fatal complications), hepatic cirrhosis, hypoparathyroidism, hypothyroidism, and delayed puberty. Deferoxamine is the only effective iron-chelating agent and should be given in a daily dose of 40 mg/kg at initiation of the transfusion program. Administration is by subcutaneous infusions from 8 to 10 hours per day. The goal of iron-chelating treatment is to maintain serum ferritin levels between 500 and 1,000 ng/ml. This long-term treatment is a significant burden for patients and it can be hoped that non-toxic iron-chelating agents, active by mouth, will become available.

Adolescent↗
PubMed1989

[Treatment of post-transfusion iron overload by deferoxamine].

Iron chelation therapy must be associated with the regular blood transfusions required for thalassaemia and other chronic anemias. We report here a study concerning 4 groups of patients, aged 6 to 28, regularly transfused at Necker Enfants-Malades hospital: a) 20 with thalassaemia major; b) 6 with thalassaemia intermedia; c) 2 with sickle cell disease and d) 2 with Blackfan-Diamond syndrome. The transfusion regimen consisting of monthly or quarterly transfusions varied as a function of the groups. Desferal was used in all patients. The dosage and the route of administration (IV, IM, SC) were adapted to the amount of iron transfused and to the nature of the disease. The serum ferritin level was considered as the indicator of the iron overload. Comparisons were established between the quantities of iron transfused, ferritin levels, and parameters such as dosage, route of administration and compliance to Desferal. During the period of study 3 patients died from cardiac failure due to transfusional hemosiderosis. Endocrine complications (diabetes 2 cases, hypocalcemia 3 cases, hypothyroidism 1 case and delayed puberty 7 cases) were observed. This high incidence of complications induced by post-transfusional iron overload has recently prompted us to improve the quality of chelation therapy through the use of the services of a specialized center where patients as well as their families can be trained more adequately in home care and self-treatment.

Adolescent↗
PubMed1989

[Echocardiographic study at rest and during effort in adult asymptomatic thalassemic patients].

An echocardiographic study was performed in 21 young patients with thalassemia major (TM, age 16-22 years), with no cardiac symptoms, and in 24 age-matched normals (N) at rest and during sitting bicycle exercise (EX). All TM were receiving blood transfusions regularly to maintain hemoglobin level above 11 g/dl and subcutaneous infusion of desferrioxamine (40 mg/Kg/day) to reduce hemosiderosis. At rest, in comparison to N, TM showed a significant increase in LV end-diastolic dimension (EDD), septal, posterior wall thickness and mass, whereas wall thickness to EDD ratio and LV fractional shortening (FS) were not different. Stress echocardiography was recorded with success in 90% of TM and 83% of N. During EX, FS increased progressively with increase of heart rate in both groups, though the increase of FS was lower in TM. Thus, asymptomatic young patients with thalassemia major, under well transfusion-chelation therapy, showed cardiac changes from volume overload. LV systolic function, normal at rest, was moderately impaired during exercise test.

Adolescent↗
PubMed1989

Caval syndrome in a Dirofilaria immitis-infected dog treated with dichlorvos.

In the dog of this report, lesions found were compatible with death attributable to acute circulatory collapse induced by caval syndrome and compounded by microfilarial death. Hepatic centrilobular hemorrhage and necrosis were suggestive of circulatory collapse, renal hemosiderosis was suggestive of disseminated intravascular coagulopathy, and multifocal subacute hepatic inflammation associated with microfilariae was similar to lesions attributed to microfilarial death. Treatment with dichlorvos may have precipitated these events. Previous descriptions of dichlorvos toxicity in microfilaremic dogs have suggested that death is attributable to shock, but pathogenic mechanisms have not been established. Although generalizations from a single case must be made with caution, lesions in this dog suggest that toxic effects may be mediated through effects on adult and larval heartworms.

Animals↗
PubMed1989

[The pathogenesis of trehalose dimycolate-induced hemorrhagic pneumonia induced in mice as animal model of human alveolar hemorrhagic syndrome].

Trehalose dimycolate (TDM) is a glycolipid contained in the cell walls of Mycobacteria, Nocardia and Corynebacteria. An intraperitoneal injection of TDM into mice has been known to produce hemorrhagic pneumonia without affecting any other organs. Thus, it provides a unique experimental model for studies of the mechanisms of alveolar hemorrhagic syndrome, including idiopathic pulmonary hemosiderosis. It has been reported that T lymphocytes are essential for the production of TDM-induced hemorrhagic pneumonia, however, the overall cellular mechanism is not yet clear. The purpose of this study is to re-examine and clarify the role of T lymphocytes in the pathogenesis of TDM-induced hemorrhagic pneumonia. To achieve it we considered 1) the dynamics of infiltrating lymphocytes to find out if there is a certain T lymphocyte subpopulation infiltrating predominantly into the lung, 2) the effect of in vivo depletion of T lymphocyte subpopulation by monoclonal antibodies, and 3) the effect of transfer of T lymphocytes into nude mice. The analysis of the dynamic change of the number of lymphocytes showed that the number of L3T4+ cells as well as Lyt2+ cells decreased on day 2 or 3 after TDM injection, thereafter increased, however, neither subpopulation infiltrated predominantly into the lung. Alveolar hemorrhages occurred in L3T4+ cell-depleted and/or Lyt2+ cell-depleted mice, and hemorrhages were enhanced in Lyt2+ cell-deplete mice. Alveolar hemorrhages occurred even in nude mice, and the intensity of hemorrhages or the cell numbers in the lung did not differ from those in T lymphocyte-reconstituted nude mice, however, hemorrhages were enhanced in L3T4+ cell-reconstituted nude mice. These results suggested that T lymphocytes are not primarily involved in the cellular mechanisms of the pathogenesis of TDM-induced hemorrhagic pneumonia, however, L3T4+ cells modify the process of the production of hemorrhagic pneumonia secondarily and enhance it.

Animals↗
PubMed1989

[Pulmonary hemorrhagic syndromes. Clinical and diagnostic approach].

The diagnosis of pulmonary hemorrhage syndromes (PHS) rests on a body of clinical and paraclinical evidence found in all etiologic forms. Usually, the combination of hemoptysis, iron deficiency anemia, and roentgenologic infiltrates is easy to recognize; in more atypical cases, alveolar bleeding can be demonstrated by determination of the Golde score on bronchoalveolar lavage specimens. Whereas clinical features are nearly invariable, etiologies cover a broad spectrum including some glomerular diseases, idiopathic pulmonary hemosiderosis, connective tissue disorders, pulmonary conditions related to compromised immunity, airborne pulmonary aggressions, and some apparently primary vascular disorders. Regardless of the severity of clinical features at presentation, pulmonary hemorrhage syndromes are always a diagnostic and therapeutic emergency.

Bronchoalveolar Lavage Fluid↗
PubMed1989

[Arterial hypertension and veno-occlusive disease. Connections with pulmonary hemorrhage].

Primary pulmonary hypertension, as defined by the World Health Organization, specifically includes three entities: primary plexogenic arteriopathy, pulmonary veno-occlusive disease, and silent recurrent thromboembolism. Pulmonary hemorrhage may occur in each of these syndromes but is not usually a prominent symptom. The hemorrhage is probably the result of a variety of causes and related to the specific morphologic characteristics of the pulmonary vasculature in these diseases. In primary plexogenic arteriopathy, hemoptysis and pulmonary hemosiderosis occur mainly in advanced disease with dilatation lesions and plexiform lesions. In veno-occlusive disease, pulmonary hemorrhage occurs chiefly in adult patients and is almost certainly capillary in origin. In recurrent thromboembolism, hemoptysis is considerably more common in the symptomatic form than in the silent form, probably because hemorrhagic infarction and anticoagulant therapy play a greater role in the former situation.

Hemorrhage↗
PubMed1986

[Enterotoxemia in newborn calves due to Cl. perfringens types A, C and D].

The clinical symptoms and the morphologic picture of calf enterotoxemia are described. Studied were a total of thirty-two dead and slaughtered animals. Bacteriologically, the disease was shown to be caused by types A, D, and C of Clostridium perfringens. Types C and D proved pathogenic for guinea pigs, while type A did not. Isolated was a strain of Clostridium perfringens, which had high toxigenicity. It was found that calves were fairly often affected with the disease. Most severe were the infections caused by type C of Cl. perfringens, with most pronounced morphologic lesions. There were differences in the changes caused by all three types of the organism in calves, which made it possible to distinguish them as causative agents. Type A induced slight icterus and slightly manifested hemosiderosis of the liver, kidneys, and spleen; type D was responsible for severe injury and hyalin dystrophy of the kidneys; and type C caused necrotic enteritis, pronounced hemorrhagic diathesis, degenerative changes in the ganglial cells, and demyelinization of the brain.

Animals↗
PubMed1987

The effects of metabolic diseases on the cardiovascular system.

Many metabolic diseases result in pathological changes within the cardiovascular system, often with the most severe effects on the function of the heart and great vessels. Metabolic disorders affecting the heart include disorders of amino acid metabolism, storage diseases, neuromuscular diseases, diseases of metal and pigment metabolism, carnitine deficiency, and connective tissue disorders. Several inborn errors of metabolism may involve the myocardium due to the accumulation of abnormal metabolites in the myocardial cells. In addition, the heart valves and coronary vessels may be involved. If the predominant effect is in the myocardial cell, it will be manifested clinically as a cardiomyopathy. Some disorders, in particular oxalosis, may involve the conduction system as a result of the deposition of oxalate crystals and result in conduction disturbances such as in alkaptonuria, primary oxalosis, and homocystinuria. Myocardial involvement may result in cardiomyopathy of the three functional types: (1) congestive, as in Fabry's disease, (2) hypertrophic, as in glycogen storage disease, type II, or (3) restrictive, as in Gaucher's disease. In the storage disease severe valvular as well as myocardial involvement occur predominantly in the glycogen storage diseases, types II-IV, mucolipidoses, sphingolipidoses, and neuronal ceroid lipofuscinosis. There are a variety of neuromuscular disorders that may be associated with cardiomyopathy, including the muscular dystrophies, Friedreich's ataxia, and Kugelberg-Welander syndrome. The pathological features of these conditions are not specific, but result usually in a congestive form of cardiomyopathy. Patients with metal and pigment metabolic disorders include iron storage disease, either hemochromatosis or transfusional hemosiderosis, Menkes' kinky hair syndrome, and Dubin-Johnson syndrome. Either a restrictive or a congestive form of cardiomyopathy may occur. The systemic form of carnitine deficiency is an autosomal recessive disorder and may present as a cardiomyopathy with congestive heart failure and lipid accumulation in the myocardial cells. Connective tissue disorders are generalized diseases that may involve the heart and valvular tissue, but also the blood vessels. These include Marfan's syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, and pseudo-xanthoma elasticum.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

Goiter in a cockatiel (Nymphicus hollandicus).

Goiter in a 2-year-old male cockatiel with respiratory distress was characterized by bilateral enlargement of the thyroid glands. Microscopic lesions included features of both hyperplastic goiter and colloid goiter. There also was localized hemosiderosis due to hemorrhage in subcapsular space and in thyroid follicle lumens.

Animals↗
PubMed1988

Clinicopathologic features of young and old sphha/sphha mice. Mutants with congenital hemolytic anemia.

A colony of mice with congenital hemolytic anemia, sphha/sphha, were evaluated over a 3-year period. Prominent findings included decreased survivability, reticulocytosis, increased peripheral blood leukocytes, extramedullary hematopoiesis in liver and spleen, lymphoid hyperplasia and membranoproliferative glomerulonephritis. Older (12 to 21 months) anemic animals had elevated serum levels of IgG1 and IgA. There was deposition of C3, IgG, IgM, and IgA in renal glomeruli of both control and anemic mice, but deposition of IgM and IgA was more prominent and widely distributed in anemic animals and correlated with mesangial expansion and the presence of electron dense deposits in the mesangium and in glomerular capillary walls. Prominent renal tubular hemosiderosis was noted in young and old anemic mice. The relation between the hemolytic anemia and glomerular disease is unclear but these mice may be an animal model useful for exploration of changes attendant with chronic hemolysis and evaluation of renal disease that accompanies hemolytic anemia.

Age Factors↗
PubMed1987

Transfusion and long-term hemodialysis.

We analyzed the transfusion practice at a large regional renal dialysis center. More than half of all long-term dialysis patients received at least one transfusion annually, and the average transfused patient received 10 U of blood. A review of data on 318 dialysis patients over one year showed there is a sizable group (15.0%) who require greater than ten transfusions of blood. This group of intensely transfused dialysis (ITD) patients account for a disproportionate 67% of all units transfused. The majority of these patients (73%) were women, which may have been related to the relatively small proportion (12.5%) of women undergoing dialysis who were treated with synthetic androgens. These ITD patients were older and underwent dialysis longer than others in the population studied. One year mortality in the ITD group was 27%; more than twice that of the entire group (12.8%). A retrospective examination of transfusion practice in the five years preceding the present study showed that the number of transfusion recipients was essentially a constant fraction of the dialysis population. However, there was a significant trend to less intense transfusion of each transfused patient. Most of the ITD group had significant iron overload, and attending physicians indicated that concern about transfusion related hemosiderosis outranked other reasons including acquired immunodeficiency syndrome, that would account for the more conservative transfusion practice of recent years.

Blood Transfusion↗
PubMed1987

Pathology of experimental Babesia microti infection in the Syrian hamster.

Pathologic changes produced after 4 weeks of infection by Babesia microti in Syrian hamsters are described and compared to babesiosis of humans. Following intraperitoneal inoculation, both intravascular and extravascular hemolysis developed. Up to 70% of red blood cells were parasitized. The principal morphologic abnormalities were an increase in extramedullary hematopoiesis and hyperplasia of the mononuclear phagocytic cells of the red pulp manifested grossly as splenomegaly, marked renal tubular hemosiderosis and hypertrophy of Kupffer cells. The disease was not fatal to any hamsters during the 4 week study. The clinical signs and lesions were less severe than fatal babesiosis of asplenic humans and similar to severe, but nonfatal disease in spleen intact humans. The hamster may serve as an animal model for the studying the pathophysiology of human babesiosis and for studying potential chemotherapeutic agents.

Animals↗
PubMed1986

Aortic dissection in a gorilla.

Autopsy findings in a 39-year-old male gorilla included aortic dissection, internal rupture of the aortic arch with axial direction of the tear, external rupture of the ascending aorta, cardiac tamponade, myocardial hypertrophy, cystic and basophilic degeneration of the aortic media, marked obesity, severe degenerative joint disease, focal glomerulonephritis, and widespread hemosiderosis.

Aortic Dissection↗
PubMed1987

The liver in acquired immune deficiency syndrome: emphasis on patients with intravenous drug abuse.

To assess the spectrum of hepatic abnormalities in acquired immune deficiency syndrome (AIDS), we reviewed clinical, biochemical, and pathological material in 32 patients with AIDS. Eight-four percent of AIDS cases had a history of intravenous drug abuse. Ninety percent of AIDS patients has some liver biochemical abnormality at the first presentation of illness. During the course of AIDS, significant (p less than 0.05, paired Student's t test) rises in alkaline phosphatase and bilirubin occurred, without rises in aminotransferases. Mean abnormalities were mild, reflecting approximately 2-fold increases over baseline. Liver failure was not believed to contribute to the death of any AIDS patient. Pathological findings in AIDS included specific infectious diagnosis in 26%, granulomas in 16%, hemosiderosis in 26%, nonspecific abnormalities in 39%, cirrhosis in 23%, and chronic active hepatitis in 3%. AIDS cases were also compared to 10 selected age, sex, and epidemiologically similar non-AIDS patients. Although granulomas or infections were not seen in our comparison group, only the incidence of chronic active hepatitis was significantly different between the groups. If only those with intravenous drug abuse were studied, then none of 24 AIDS patients versus four of eight non-AIDS cases (p less than 0.005) had chronic active hepatitis. AIDS patients with specific hepatic infections tended to have a higher alkaline phosphatase and aspartate aminotransferase (p less than 0.05) than noninfected cases. However, substantial overlap existed, and no difference in hepatomegaly was noted. Ninety percent of AIDS patients were ingesting at least one potentially hepatotoxic drug. We conclude that AIDS patients have a high incidence of underlying hepatic abnormalities. However, clinical and biochemical abnormalities are similar in our selected liver biopsy patients with intravenous drug abuse with or without AIDS. As expected, AIDS patients have a higher incidence of hepatic granulomas and infections, but these patients were not clearly distinguishable from other AIDS cases. Histological examination showed a wide array of changes by light microscopy, but no specific lesion of AIDS was noted. The low incidence of chronic active hepatitis in this AIDS population may imply that the altered T lymphocyte function in AIDS could influence the course of liver disease in these patients.

Acquired Immunodeficiency Syndrome↗