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At least 1,315 records · Page 73Linked to original sources
PubMed1995

Interstitial lung diseases in children: a review.

Interstitial lung diseases (ILD) are disorders of the lower respiratory tract, characterized by chronic inflammation of the lung parenchyma, varying degree of fibrosis, derangement of the alveolar walls and loss of the functional alveolar capillary units. ILD are relatively uncommon in children. Most of the interstitial lung diseases have no known etiology. In children, common diseases associated with ILD include viral respiratory tract infections (RSV, parainfluenza, etc.), gastroesophageal reflux, idiopathic pulmonary fibrosis, pulmonary hemosiderosis, eosinophilic pneumonia, pneumonitis associated with AIDS, etc. Chronic inflammation of the alveoli (alveolitis), the initial injury in ILD, and several mediators released from inflammatory cells (eosinophils, neutrophils and macrophages) can cause fibrosis and derangement of alveolar walls. Dyspnea and a non-productive cough are the cardinal symptoms of ILD. Other findings include chest pain, hemoptysis and weight loss. Clubbing of fingers occur in approximately 50 per cent of cases. Diagnosis is based on a combination of history, clinical findings, radiographic findings, pulmonary function tests and histologic findings. Open lung biopsy has been very helpful in providing information regarding the extent and nature of the damage, prognosis and response to therapy. There are 3 main aspects in the treatment of ILD. The most important step is to identify and eliminate the cause. The second is suppression of the inflammation. The third is supportive and symptomatic treatment. Corticosteroids are the drugs commonly used for suppression of inflammation. Immunosuppressive drugs (azathioprine, cyclophosphamide) have also been tried. Lung transplantation and heart transplantation have been successfully achieved in selected patients. The results of therapy should be regularly monitored by clinical symptoms, chest radiographs and serial pulmonary function studies.

Age of Onset↗
PubMed1993

A unique rodent model for both the cardiotoxic and hepatotoxic effects of prolonged iron overload.

BACKGROUND: Hemochromatosis is a disease of excessive iron storage leading to tissue damage and fibrosis. Both genetic hemochromatosis, which can affect 1 in 500 of some populations, and the form of this disease which occurs as a secondary consequence of the hemoglobinopathy, homozygous beta-thalassemia, with 40 million carriers worldwide, have a common pathology. The cardiotoxicity and hepatotoxicity, which occurs with this disease, have never been produced experimentally in other species. EXPERIMENTAL DESIGN: Using a regimen of iron dextran administered subcutaneously to gerbils on a weekly basis for 7 weeks, we have produced severe hemosiderosis, especially of the liver and heart. By examining gerbils at 1, 2 and 3 months after the final iron injections we followed the subsequent development of hemochromatosis in the hearts and livers of iron overloaded animals. RESULTS: Hemochromatosis of the liver was evident as a scarring fibrosis in all cases between 1 and 3 months after iron dextran administration to gerbils. The iron burden in the cardiac myocytes of gerbils gradually increased between 1 and 3 months, resulting in hemochromatosis of the heart 2 and 3 months after the final iron dextran injections. CONCLUSIONS: Repeated parenteral injections of iron dextran to gerbils resulted in hemochromatosis affecting the liver and heart with a pathology which is the same as occurs in the end-stage disease in man. This model will allow the detailed study of the mechanism of iron induced, free radical tissue damage, which is though to be the cause of these lesions and will also be useful in the evaluation of iron chelating therapies to determine whether the hepatic and cardiac pathology of iron overload can be modulated over a long period.

Animals↗
PubMedDate not supplied

Histoenzymological and ultrastructural investigations regarding the role of the endogenous peroxidase at the pulmonary level in cardiogenic pulmonary hypertension.

We found optically and ultrastructurally endogenous peroxidase in 90 intraoperatory pulmonary biopsies taken from patients with valvular cardiopathies and different degrees of pulmonary hypertension. The enzymatic activity increases proportionally to the pulmonary hypertension and it reaches its maximum in severe cases of hemosiderosis. Ultrastructurally we found two reaction types namely: 1) in peroxisomes of circulating monocytes turning into tissue ones, in medium or slight pulmonary hypertension (PHT) and 2) (as granular reactions on the nuclear membrane and endoplasmic reticle in alveolar macrophages, granular pneumocytes in aggravating and severe medium PHT. The presence of both these two types of reaction in the same cell demonstrates the passing stages from circulating macrophages to tissue ones attesting a transitional process of the phagocytic cells.

Heart Valve Diseases↗
PubMed1994

Secondary diabetes in children with thalassaemia major (homozygous thalassaemia).

Life expectancy of patients suffering from homozygous beta-thalassaemia has been improved due to the modern treatment of this disease. This has allowed development of late hemosiderosis-related complications and disturbances of the endocrine and exocrine functions of the pancreas. Carbohydrate metabolism of 16 patients with thalassaemia major was studied. Three of them presented with a pronounced clinical picture and biochemical constellations of a severe diabetes mellitus. The remainder had no clinical symptoms of carbohydrate metabolism disorders. The pancreatic beta-cell function of the patients was assessed by measuring the serum concentrations of immunoreactive insulin and by a glucose tolerance test. Most patients showed very low basal insulin levels while glucose tolerance was reduced in only one of them. In this patient we also established delayed insulin response after an intravenous glucose load. We concluded that the disturbed insulin secretion found in the children studied is most likely the earliest manifestation of the pancreatic beta-cell insufficiency which precedes the changes in the glucose tolerance.

Adolescent↗
PubMed1994

Veno-occlusive disease of the liver induced by low-dose cyclophosphamide.

A 2-yr-old child with idiopathic pulmonary hemosiderosis and autoimmune thrombocytopenia developed hepatic veno-occlusive disease after being treated with long-term, low-dose cyclophosphamide. The authors believe that the injury was induced by cyclophosphamide. Although transplant dosages of cyclophosphamide alone or in conjunction with other drugs have been shown to induce hepatic venocclusive disease, this is the first case of hepatic venocclusive disease associated with nontransplant dosages of cyclophosphamide. Histopathologically, the injury was characterized by occlusive lesions of intrahepatic veins, both the terminal hepatic venules and portal veins branches, together with necrosis (predominately involving Zones 3 and 2) and hemorrhages. An added risk factor could have been release of platelet-derived growth factor that in turn activated and promoted proliferation of fibroblasts and smooth muscle cells in the vessels. Previously reported cases of cyclophosphamide-related injury are reviewed.

Child, Preschool↗
PubMed1994

Hepatic dysfunction in patients with carcinoma who are severely thrombocytopenic and immunosuppressed.

BACKGROUND: The use of intensive cytotoxic drug therapy for malignant disorders often results in hepatic dysfunction. It is important to determine the cause of hepatic injury to institute appropriate therapy; however, neutropenia and thrombocytopenia may prevent performance of hepatic biopsy to establish a cause. STUDY DESIGN: We prospectively evaluated the cause of hepatic dysfunction using laparoscopic biopsy of the liver in 20 consecutive patients who were receiving intensive cytotoxic therapy, with or without bone marrow transplantation, or who were being treated for severe aplastic anemia. One to three direct-vision laparoscopic biopsies were performed in each patient during general anesthesia and bleeding was controlled with spatula electrocautery. Platelet concentrate transfusions were given before, during, and immediately after the biopsy. RESULTS: Platelet and leukocyte counts at the time of hepatic biopsy ranged from 1,000 to 83,000 per microL (median of 23,500 per microL) and zero to 14,300 per microL (median of 2,200 per microL), respectively. Nineteen of 20 patients had platelet counts of less than 68,000 per microL. Bleeding at biopsy site was controlled during the procedure without evidence of bleeding or complications after biopsy. Biopsy specimens revealed graft-versus-host disease (n = 2), hepatic veno-occlusive disease (n = 1), steatosis (n = 5), cholestasis (n = 19), hemosiderosis (n = 19), and granuloma (n = 1). CONCLUSIONS: In several patients, the knowledge derived from hepatic biopsy results altered the therapeutic strategy. The use of laparoscopic hepatic biopsy to assess the cause of hepatic dysfunction should be encouraged because it is a safe procedure, even in patients who are severely thrombocytopenic and immunocompromised.

Adolescent↗
PubMedDate not supplied

[Pathomorphologic changes in experimental siderosis].

Experimental hypersiderosis was induced in rabbits by intravenous injection of iron-containing preparation Ferrum Leck. The resultant pronounced hemosiderosis was found in the liver, kidneys, heart, lungs and spleen. Dystrophic and necrotic processes arose in the liver, kidneys, heart. Accumulation of hemosiderin in the vascular walls led to their higher permeability, edema, wall thickness, proliferative changes, especially in the coronary vessels. Intravascular circulation impairment was evident from compression of microvessels by siderophages, red cells aggregation, formation of microthrombi.

Animals↗
PubMed1975

Intravitreal injection of autologous blood in primates.

Thirteen owl monkeys received intravitreal injections of 0.3 ml of autologous blood in single or multiple injections. Blood was antiocoagulated either with heparin to inhabit clot formation or with citrate to allow clot production within the vitreous body. Clinical observations showed that approximately 60% of the heparinized eyes cleared, whereas only about 5% of the citrated eyes did. Thus, clotting appears to have a significant effect on the clearance of blood from the vitreous chamber. In addition to the abscence of hemosiderosis, histological and ultrastructural studies of retinas showed no retinal damage.

Animals↗
PubMed1994

[Lung biopsies with light microscopic and ultrastructural observations in 34 cases of diffuse pulmonary diseases].

Lung biopsies with light microscopic and ultrastructural observations were performed in 34 cases of diffuse pulmonary diseases. Open lung biopsy 2 cases, transfiberoptic bronchoscopy lung biopsy 32 cases. There were 19 (56%) idiopathic interstitial fibrosis, 3 (9%) pulmonary alveolar proteinosis, 3 (9%) pulmonary sarcoidosis, 1 (3%) idiopathic pulmonary hemosiderosis, 2 (6%) pulmonary mycosis, 1 (3%) alveolar cell carcinoma, 2 (6%) pulmonary tuberculosis and 3 (9%) pulmonary collagen diseases. Diagnosis was based on clinical data and pathologic manifestation. Lung biopsy with light and electronic microscopic examination is useful in etiologic diagnosis and differential diagnosis for diffuse pulmonary diseases.

Adolescent↗
PubMed1994

[A case of crescentic glomerulonephritis associated with anti-myeloperoxidase antibody presenting as alveolar hemorrhage].

A 77-year-old man was admitted because of hemoptysis. Chest roentgenograms initially showed progressive infiltrative shadows, which improved spontaneously in 3 months. Transbronchial lung biopsy specimens obtained during the first admission revealed alveolar hemorrhage with neither granuloma nor vasculitis. Alveolar hemorrhage associated with renal dysfunction recurred 9 months later. Serum creatinine level was elevated to 3.5 mg/dl. No other organ than lungs or kidneys was involved. Renal biopsy was performed to confirm the pathological diagnosis of crescentic glomerulonephritis. Anti-basement-membrane antibody was negative, whereas anti-neutrophil-cytoplasmic antibody was positive for perinuclear pattern (P-ANCA) by indirect immunofluorescent (IF) method. He was diagnosed as having idiopathic crescentic glomerulonephritis complicated with alveolar hemorrhage, and the presence of anti-myeloperoxidase (MPO) antibody in serum was anticipated. Anti-MPO antibody level in his serum evaluated by ELISA was markedly elevated. Although myeloperoxidase has been considered as a common antigen to P-ANCA and anti-MPO antibody, the determination of P-ANCA has been clinically unreliable because of equivocal results. In contrast, the presence of anti-MPO antibody is highly specific for idiopathic crescentic glomerulonephritis complicated with alveolar hemorrhage or its incomplete variant case. Also, it is a better index of disease activity. Therefore, there is a possibility that those patients diagnosed as having idiopathic pulmonary hemosiderosis or pulmonary-renal syndrome may be categorized into the one disease, anti-MPO antibody-associated disease, and the measurement of anti-MPO antibody may lead to prompt treatment prior to the histological diagnosis.

Aged↗
PubMed1994

[The evolutionary effects of therapy on the skeletal lesions in beta-thalassemia].

Up to the mid-1960s, beta-thalassemia was treated with blood transfusions as frequent as needed to keep symptoms under control and to prevent transfusional hemosiderosis. In the following years, high transfusion regimens and iron chelation therapy with desferrioxamine were used. Because of these different treatment modalities, skeletal findings in thalassemia have markedly changed. In the past, thalassemic patients treated with a low transfusion regimen and without chelation therapy developed osteopenia--with widened medullary spaces, cortical thinning and trabecular atrophy--secondary to chronic expansion of red marrow, due to increased erythropoietin response to chronic anemic hypoxia. Typical radiographic patterns in the skull included widened diploic space, atrophic-especially outer--tables and, in some patients, the "hair-on-end" pattern. As for the face, obliteration of the paranasal sinuses and the typical "rodent facies" were observed. In the ribs, bulbous expansion of the posterior and anterior segments and the "rib within a rib" patterns were observed. As for the spine, coarse trabecular arrangement was seen. The "cobweb" pattern was seen in the pelvis and finally the lack of the normal concave outline was observed in the long bones. In the patients treated with high transfusion regimens and iron chelation therapy over the last 30 years, both skull anomalies and disfigurement are less frequent. The skull is almost normal, with the exception of osteopenia and thickened diploic space in the frontal bone only; the paranasal sinuses are usually not obliterated. The hands and rib are normal, just like long bones, pelvis, scapulae and vertebral bodies. Nevertheless, in some adequately treated patients new skeletal features have been recently observed in the long bones, which are similar to those occurring in rickets and/or scurvy, and in the vertebral bodies, resembling platyspondylia. These abnormal features might be caused by several factors--i.e., marrow expansion, transfusion regimens, direct/indirect effects of desferrioxamine, iron load, endocrine abnormalities, deficiency of some minerals and finally dysvitaminoses. Nevertheless, osteopenia remains the main negative factor of thalassemia.

Adolescent↗
PubMed1976

The female genital tract of the owl monkey (Aotus trivirgatus) with special reference to the ovary.

The female reproduction tracts and ovaries of 30 owl monkeys, Aotus trivirgatus, were examined morphologically using the light and electron microscope. The animals ranged in age from a third trimester stillborn fetus to those of mature old age. The epithelia of the generative tracts were inactive or resting; evidence of prior menstruation was occasionally evident from hemosiderosis in endometrial stroma. Cyclicity of estral or menstrual type, however, was not established. One pregnant uterus (11-12 days gestation) was in the series. Prior pregnancy was deduced by the presence of perivascular fibrosis of myometrial vessels. The immature ovary contained an occasional developing or involving Graafian follicle, but no interstitial tissue. The mature ovary developed large, multilobulated masses of luteinized interstitial tissue which occupied the medulla, crowded the hilum, and thinned the cortex. Small dark intracortical cells, derived from cortical stroma, became foamy, lipoid-laden, contained hemofuscin, and formed the peripheral zone of the interstitial cell masses. The theca interna of involuting follicles appeared to be a significant source of the inner cells of the interstitial masses. Ultrastructure of the outer pigmented cells of luteal interstitial masses suggested steroidal activity; the function of the inner cells was not morphologically evident. Corpora lutea could not be identified with certainly, either by form, stigmata, or histology; nor could they be differentiated from interstitial masses. The latter appeared, therefore, to act in concert as a single, massive bilateral corpus luteum.

Animals↗
PubMed1993

Studies on familial hypotransferrinemia: unique clinical course and molecular pathology.

Some unsolved problems--late onset of anemia and growth retardation (at age 7 years), healthy siblings showing very low transferrin (TF) level, and unexplained mode of inheritance--were found in family members of a congenital atransferrinemia already reported in 1972. The long-term clinical, laboratory, and developmental observations revealed that after 5 years of apo-TF supplementary therapy the patient's anemia gradually disappeared, and he started to grow again without further therapy. Immunoelectrophoretic study disclosed a severe deficiency of both TF and haptoglobin in the patient. The recovery from his anemia and the resumption of his physical development were dependent only on his TF level: that is, from a negligible level it increased to 10-20 mg/dl (normal, 205-370 mg/dl), a level similar to that of his TF-deficient siblings, who had been in good health since birth. The TF analysis of the patient and his family suggests that the minimum TF requisite in this family may be close to 10-20 mg/dl; subjects with more than 20 mg/dl are apparently healthy; with less than 10 mg/dl they may develop severe growth retardation and anemia, and extreme deficiency may be lethal. Also, coexisting haptoglobin deficiency might alleviate hemosiderosis. Further, the isoelectric focusing study disclosed that there was only a small amount of TF variant in these siblings including the patient. The study of the family confirmed that this variant was produced by an allelic gene derived from their father. So, the original diagnosis of congenital atransferrinemia should be revised as familial hypotransferrinemia transmitted with autosomal recessive mode, and the subjects with a recessive character may be compound heterozygotes of the "variant" allele and the "null" allele.

Adolescent↗
PubMed1993

The 5q- syndrome: a single-institution study of 43 consecutive patients.

A favorable prognosis and a low rate of leukemic transformation has been attributed to the 5q- syndrome, a myelodysplastic syndrome (MDS) characterized by macrocytic anemia, hypolobulated micromegakaryocytic hyperplasia, and an interstitial deletion of chromosome 5. We examined the characteristics and outcome of 43 consecutive patients in our institution strictly defined by morphologic criteria and a solitary 5q- cytogenetic defect. The median age at diagnosis was 68 years, with a clear female predominance (7:3). Eighty percent of the patients were red blood cell transfusion-dependent at diagnosis and all untransfused patients had macrocytic indexes. In contrast, significant neutropenia or thrombocytopenia was rare. The French-American-British (FAB) class distributions were RA (72%), RARS (7%), RAEB (16%), and RAEB-IT (5%). At a median follow-up of 31 months, 56% of the patients survive, with a projected median survival of 63 months. The incidence of acute leukemia was 16% and was uniformly fatal. Clinical hemosiderosis occurred in 28% of the patients, resulting in two deaths. Neither survival nor the risk of leukemic transformation was predictable from initial clinical parameters, including FAB classification, Bournemouth score, and degree of aneuploidy. The lack of significant neutropenia and thrombocytopenia seemed to account for a very low incidence of infection and bleeding resulting in a prognosis equal or superior to historical patients with MDS. Therapeutic endeavors, including the use of corticosteroids, androgens, cis-retinoic acid, pyridoxine, and danazol, were largely unsuccessful.

Acute Disease↗
PubMed1993

[Diffuse pulmonary hemorrhage: review of nine cases].

Nine cases of diffuse pulmonary hemorrhage (DPH) diagnosed in our hospital during the past eight years are reviewed. We assess the clinical, diagnostic, etiologic and evolutive characteristics of all these cases and, thus, of such entity in our environment. The nine patients had anemia, hemoptysis and transient pulmonary infiltrations. Renal affectation was observed in seven patients. Based on clinical and laboratory data, supplementary explorations and immunological and histological studies, the following etiologic diagnosis were established: idiopathic extracapillary glomerulonephritis (three cases), idiopathic pulmonary hemosiderosis (two cases), Wegener's granulomatosis (one case), unclassifiable systemic necrosant vasculitis or overlapping syndrome (two cases). In one patient, it was not possible to establish the etiology. Global mortality was 44.4% (four patients); two of them died during the course of the initial massive hemoptysis (not controlled, as in the other patients, with steroids) and two other patients died due to late complications. In this series, none of the patients developed a disease associated to antibodies against the basal membrane. The application of a severe diagnostic systematic has been a key factor for the management of these patients.

Adolescent↗
PubMed1995

[Clinical and echocardiographic evaluation of thalassemic cardiomyopathy].

To evaluate the reliability of the echocardiographic examination in assessment of adult patient with thalassemia major, in comparison with clinical, electrocardiographic and/or chest x ray exams, 103 patients with thalassemia major, mean age 20 years (range 14 to 30 years), were studied and compared with 30 age matched normal subjects. All patients were receiving transfusions regularly to maintain hemoglobin levels above 11 g/dl and subcutaneous infusions of desferrioxamine (about 40 mg/kg/day) to reduce hemosiderosis. The patients were divided into three groups according to their cardiac impairment, deduced by clinical history, electrocardiography (ECG) and/or chest x ray. Group I (36 patients) showed no signs or symptoms of cardiac impairment. Group II (38 patients) had only signs of cardiac impairment by ECG and/or chest x ray. Group III (29 patients) had both symptoms and signs of cardiac failure. In comparison to normal controls, Group I showed an increase in left ventricular (LV) dimension (EDD) and mass (p < 0.001), Group II and III showed a decrease in LV fractional shortening (FS; p < 0.001) too. In comparison to Group I, Group II showed a decrease in LV FS (p < 0.05), Group III showed an increase in LV EDD and mass (p < 0.001) too. In comparison to Group II, Group III showed an increase in LV EDD and mass (p < 0.001), and a decrease in LV FS (p < 0.001). In conclusion, echocardiographic examination appears a tool more reliable than clinical, electrocardiographic and/or chest x ray examination in assessment of adult patient with thalassemia major.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1996

[Essential trace element and skin diseases].

We have described herein various skin diseases which are caused by essential trace element deposition, deficiency, allergy, etc. Pigmentation of hemochromatosis and hemosiderosis are recognized by hemosiderin deposition in the dermis. Acrodermatitis enteropathica is caused by a deficiency of Zn and is classified as either a hereditary type or as an acquired type. The former is autosomal recessive and the latter is caused by a low intake of Zn. Wilson's disease and Menkes' kinky hair syndrome, which are caused by abnormal Cu metabolism, elicit hyperpigmentation and morphological changes of the hair, respectively. It appears that kinky hair formation results from low activity of sulfhydryl oxidase which is a Cu enzyme. Bowen disease, which is carcinoma in situ, is caused by As toxicosis. Some cases, such as palmo-plantar-pustulosis, lichen planus and oral lichen planus are caused by allergies to metals used in dental surgery, especially Ni, Co, Cr and Sn.

Bowen's Disease↗
PubMedDate not supplied

Audit of referral and explant diagnoses in lung transplantation: a pathologic study of lungs removed for parenchymal disease.

BACKGROUND: Lung transplantation is performed for an increasing range of pulmonary conditions in which the diagnosis is often clinical or based on limited biopsy material. Diagnosis may be made late in the course of the disease where specific features are no longer present. Posttransplantation complications and disease recurrence may relate to the primary disease, and accurate diagnosis is therefore essential. METHODS AND RESULTS: A pathologic review of 183 explanted lungs over a 10-year period (heart-lung = 109, single lung = 65, double lung = 9) showed 29 significant discrepancies or additional features likely to effect outcome. The final pathologic diagnosis was cystic fibrosis (n = 66), emphysema (59), bronchiectasis (17), pulmonary fibrosis (19), sarcoidosis (10), Langerhans cell histiocytosis (3), pulmonary veno-occlusive disease (3), posttransplantation obliterative bronchiolitis (2), primary hemosiderosis (1), rheumatoid obliterative bronchiolitis (1), extrinsic allergic alveolitis (1), pneumoconiosis (1). Unsuspected diagnoses included tuberculosis (8) (four cases of which were active and in single lung recipients requiring antituberculous chemotherapy), sarcoidosis (9), (of which, six were unsuspected primary diagnoses and three were additional diagnoses), veno-occlusive disease (3), carcinoma (1), pneumoconiosis (1), and pulmonary fibrosis (2). Aspergillus infection (2) and bronchocentric granulomatosis (3) were found in patients with cystic fibrosis. One active tuberculosis case also showed an aspergilloma. Unsuspected infections requiring therapy in immunosuppressed patients and previously unsuspected sarcoidosis, which is known to recur in the graft, were the major novel diagnoses. Discrepancy rate was 12 of 65 in single lungs (19%) and 17 of 109 in heart-lungs (16%). CONCLUSIONS: These results emphasize the need for accurate preoperative diagnosis especially when the similarly diseased native lung remains in situ.

Adult↗