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PubMed1999

Aspergillosis and other causes of mortality in the stitchbird in New Zealand.

Necropsy findings from natural deaths in free living and captive stitchbirds (Notiomystis cincta) were examined over a 3 yr period (November 1991-94) to establish whether disease was an important factor in translocation failures and captive breeding programs undertaken by the New Zealand Department of Conservation. Fresh and fixed material from seven free-living birds and 11 captive birds were examined and were compared with those of a retrospective study of archival material from captive and wild birds collected over a 13 yr period (1979-91). The causes of death in both the present and retrospective study showed a similar pattern with aspergillosis and aspiration pneumonia being the most significant cause of mortality in captive birds. Aspergillosis was diagnosed as the cause of death in 11 of 31 stitchbirds from Mt Bruce; eight of these deaths occurred in the winter months (June-August). The other causes of death in captive birds included trauma, coccidiosis, and sporadic bacterial infections. Hemosiderosis and airsacculitis were common histological findings in most of the wild and captive stitchbirds examined.

Animals↗
PubMed1999

[Transbronchial biopsy in diffuse infiltrative lung diseases].

224 transbronchial biopsies were made between 1990 and 1997 in 208 patients suffering diffuse, bilateral, disseminated pulmonary diseases with unidentified origin. The examinations were carried out by flexible bronchoscope with X-ray control after local anaesthesia. The obtained patterns yielded mucous membrane in 15, intact alveoli in 19 percent. In 25 percent of biopsies (57 cases) definitive diagnosis were verifiable. The histological examinations verified the diagnosis of sarcoidosis, hemosiderosis, TB, malignancies, eosinophil pneumonitis etc. In 71 biopsies (62 cases) according to histological opinion were pulmonary fibrosis or some synonyms of these (alveolitis, interstitial fibrosis). From these latter cases the documentation of 18 patients were insufficient, but the data of 44 cases were available. 7/44 carcinosis, 3/44 inactive TB, 8/44 regressive X-ray patterns were verifiable in this group. The idiopathic pulmonary fibrosis as final diagnosis only in 18/44 (41%) cases were established. Further diagnostic procedures are needed if the clinical data, the results of HRCT and histological examinations are not in correlation.

Biopsy↗
PubMed1999

[Acute lung injury as initial manifestation of diffuse alveolar hemorrhage].

OBJECTIVE: To determine clinical features and outcome in hospitalized patients with acute lung injury secondary to diffuse pulmonary hemorrhage (DPH). PATIENTS AND METHOD: Eight adult patients suffering DPH and acute lung injury were undergone to diagnosis procedure and following. RESULTS: Two cases of idiopathic pulmonary hemosiderosis, two cases of systemic lupus erythematous, two cases of Goodpasture's syndrome, one case of idiopathic rapidly progressive glomerulonephritis and one of periarteritis nodosa were diagnosed. Treatment was methylprednisolone in all cases; besides, three cases required ventilatory support, and one immunosuppressive therapy, plasmapheresis and hemodialysis. One patient died and three of them suffered relapse with satisfactory outcome. CONCLUSION: We remark the presence of acute lung injury as an initial feature of DPH in our experience and the importance of a prompt diagnosis of the underlying disease.

Acute Disease↗
PubMed1975

Pulmonary lymphangiomyomatosis. A review.

Anatomic and clinical observations of 28 cases, including 23 previously unpublished, of pulmonary lymphangiomyomatosis are recorded and discussed. This brings the total reported to 57. All patients were women in the reproductive age group with the major complaint of breathlessness. This was usually progressive, and death from pulmonary insufficiency resulted within 10 years. Functional changes were obstructive or restrictive, or both. Pneumothorax, chylous effusions and hemoptysis were frequent complications. Radiographically the lesions initially appear as fine, linear and nodular, predominantly basal densities, and progress to a pattern of bullous change, or honeycombing, involving all portions of the lungs not sparing the region of the costophrenic sinuses as is typical of eosinophilic granuloma. There may be associated pleural effusions. A progressively increasing lung volume is characteristic. The lesions consist of an irregular, nodular or laminar "irrational" proliferation of smooth muscle within all portions of the lung, with loss of parenchyma leading to honeycombing. Proliferated muscle can obstruct bronchioles (with air trapping and formation of bullae often complicated by pneumothorax), venules (with pulmonary hemorrhage and hemosiderosis accompanied clinically by hemoptysis) and lymphatics (with chylothorax or chyloperitoneum). Both thoracic and abdominal lymph nodes and the thoracic duct can also be involved in the myoproliferative process with formation of subsidiary minute channels and obstruction. Renal or perirenal angiomyolipomas can also occur, as exemplified by 2 patients in the present series. Identical pulmonary lesions occasionally occur in tuberous sclerosis. Especially since these patients usually have no neurologic disturbances and are almost women, the possibility of a relationship between tuberous sclerosis and lymphangiomyomatosis must be considered. One feature of note in pulmonary lesions of tuberous sclerosis is the presence of adenomatoid proliferations of epithelium. Such changes were also observed in 2 patients of the present series, and it is remarkable that both of these women had "retarded"children. At present the question of whether by lymphangiomyomatosis is a forme fruste of tuberous sclerosis must be considered as unresolved. It may yield to further investigation, possibility including chromosomal studies.

Adolescent↗
PubMedDate not supplied

[Superficial siderosis of the central nervous system: an uncommon cause of spastic paraparesia].

INTRODUCTION: Superficial siderosis of the central nervous system (CNS) is an uncommon neurological condition, characterized clinically by cerebellar ataxia, neurosensorial deafness, anosmia, myelopathy and cognitive deterioration. It is associated with the deposition of haemosiderin in the subpial layers of the brain, cerebellum and spinal cord, following chronic bleeding (often clinically silent) in the subarachnoid space. Histopathologically there is gliosis, neurone loss and demyelination of the CNS. CLINICAL CASE: We present the case of a 60 year old woman with a history of progressive worsening of a disorder of myelopathic type with signs of pyramidal liberation and sphincter incontinence associated with cerebellar ataxia, anosmia and bilateral hypoacusia. Initially she had unsteadiness, frequent falls and weakness of the legs. Her deafness was more obvious during the previous year. On lumbar puncture there was hemorrhagic CSF with increased red blood cells, iron, ferritin and protein. High field encephalic MR showed a hypointense image in T2 which surrounded the fissure of Sylvius, the brain stem, medulla oblongata, cerebellar hemispheres and sulci of the cerebellar vermis, suggestive of hemosiderosis, atrophy of the cerebellar vermis and slight cortical atrophy. Angioresonance of the intracranial vessels showed that there were no signs of aneurysms or vascular malformations. Transcranial Doppler studies were normal. CONCLUSIONS: Superficial siderosis of the CNS should be included in the differential diagnosis of the parethospastic and ataxic syndromes. The extensive study done ruled out any secondary cause such as chronic bleeding secondary to an arteriovenous malformation or bleeding aneurysm.

Ataxia↗
PubMed2001

[Indices of chronic intravascular hemolysis in patients with different prosthetic heart valves in long-term follow-up].

An examination of 153 patients with valve replacement was performed at late stages of (6.8 +/- 0.2) years, in 84 patients the mitral valve replacement (MVR) was fulfilled, in 64--the aortic replacement (AVR) and in 5--two valve replacement were performed. Higher indices of hemolysis were noted in patients with AVR, in cases of two valve replacement and in patients with the paravascular fistula. The lowest indices of hemolysis take place in patients with bileaflet prostheses (MEDING/CarboMedics, St. Jude Medical), especially in patients with AVR. The complications of intravascular hemolysis are: cholelithiasis, anemia, hemosiderosis of the kidneys, sideropenia. The timely diagnosis, treatment and prophylactics are necessary to prevent these complications.

Anemia↗
PubMed1975

Influence of iron and ascorbic acid on tryptophan metabolism in man.

South African Bantu patients with a scurvy-type skin, which developed after a prolonged, iron-induced hemosiderosis, were studied with ascorbic acid-1-C14 and the 2 gm tryptophan load test. The metabolism of the two compounds was found to be abnormal in these patients. The data suggested that ascorbic acid was rapidly (iron accelerated) metabolized to monodehydroascorbate, a compound that rapidly reacts with tissue DPNH to form DPN. This mechanism could reduce tissue levels of DPNH such that the feed-back control of tryptophan pyrolase enzyme was depressed. The change in control level of the pyrrolase enzyme permitted large quantities of tryptophan to be converted into the kynurenine pathway products, and a smaller quantity for the serotonin pathway. This mechanism could contribute to the abnormal tryptophan metabolism found in chronic pellagrins with dementia.

Ascorbic Acid↗
PubMed2003

[Churg-Strauss syndrome with alveolar hemorrhage].

A 35 year-old man was admitted to our hospital because of cough, wheezing, and paresthesia of the right upper extremity. He demonstrated marked eosinophilia, bronchial asthma, and mononeuritis multiplex. We diagnosed Churg-Strauss syndrome. Serum MPO-ANCA was elevated to 189 U/ml. Chest high-resolution computed tomography showed panlobular ground-glass attenuation in both lungs. BALF showed bloody fluid, and TBLB revealed findings consistent with eosinophilic pneumonia. Lung biopsy by VATS revealed eosinophilic pneumonia, pulmonary vasculitis, capillaritis, and hemosiderosis. The patient recovered after treatment with prednisolone and cyclophosphamide. We concluded that alveolar hemorrhage due to pulmonary capillaritis could be a complication in cases of Churg-Strauss syndrome.

Adult↗
PubMed1976

[Hemosiderin pigmentation in Sezáry's syndrome].

A 75 year old woman suffering from Sezary syndrome with edemas and excessive pigmentations is presented. In previous observations, pigmentation was caused by melanosis. In our patient it is caused by hemosiderosis. The origin of the pigmentation by a purpura-pigmentosa-progressiva-like mechanism or by the tumor disease itself is discussed.

Aged↗
PubMed2005

[Modified hemispherectomy for intractable epilepsy in patients with infantile hemiplegia].

OBJECTIVE: To explore the effectiveness of modified hemispherectomy for intractable epilepsy in patients with infantile hemiplegia. METHODS: Eighteen cases of patients were treated with modified hemispherectomy and the effectiveness was studied and followed up. RESULTS: The seizures in all 18 cases of patients were controlled effectively and stopped completely in 16 cases of them, without nervous disfunction worsened. The patients' cerebral peduncles on healthy side were much thicker than those on sick side (t = 58.32, P < 0.001) and healthy peoples' (t = 14.63, P < 0.001) and the patients' cerebral peduncles on sick side were much thinner than those of healthy peoples' (t = 51.27, P < 0.001). CONCLUSION: The modified hemispherectomy can effectively control the seizures of patients with infantile hemiplegia without superficial cerebral hemosiderosis happened.

Adolescent↗
PubMedDate not supplied

Subtotal splenectomy preserving the lower pole in rats: technical, morphological and functional aspects.

PURPOSE: To assess the possibility of preserving the lower pole of the spleen, supplied by the inferior lobar vessels and segmental vessels, or by vessels of the gastrosplenic ligament, in subtotal splenectomy; to study the viability and function of the lower pole of the spleen. METHODS: Thirty-six male Wistar rats were used in this study. Said animals weighed 273-390 g ( 355.2 +/- 30.5 g ), and were randomly distributed into three groups. Group 1 comprised ten animals which were submitted to exploratory laparotomy with spleen manipulation (sham operation). Group 2 comprised 16 animals which were submitted to total splenectomy. Group 3 comprised ten animals which were submitted to subtotal splenectomy, preserving the lower pole of the spleen. Blood was collected from all animals before and 90 days after surgery to measure the levels of cholesterol and triglycerides. The animals were sacrificed 90 days after surgery. Spleens and remaining spleens were removed for macroscopic and microscopic examination. RESULTS: Surgery was performed with no complications in all groups. Six animals died in group 2. Spleens of groups 1 and 2, and lower poles of group 3 were macroscopically viable. Apparent white pulp hyperplasia was observed in group 1. In group 3, slight inflammation and capsular fibrosis were observed at the incision site, as well as diffuse hemosiderosis in the red pulp. Average mass of remaining spleen was 35.84% +/- 4.31%. No significant difference was observed between preoperative and late postoperative lipid levels in groups 1 and 3 (p > 0.05). Late postoperative lipid levels significantly increased in group 2. CONCLUSIONS: Preservation of the lower pole of the spleen (supplied by gastrosplenic vessels or inferior lobar vessels and segmental vessels) was possible with subtotal splenectomy. The lower pole was macroscopically and microscopically viable in all cases. Subtotal splenectomy preserving the lower pole prevented changes in lipid levels, which were observed in rats submitted to total splenectomy. Plasma lipid levels in rats submitted to subtotal splenectomy were similar to those observed in sham operated rats.

Animals↗
PubMed1991

Transfusion-related chronic liver disease in sickle cell anemia.

The medical records and liver biopsies of nine sickle cell patients with chronically elevated liver function tests were retrospectively reviewed to determine the etiology of chronic liver disease. There were eight women and one man with a mean age of 30 yr. All patients had hemoglobin SS. Eight patients were referred for elevated aminotransferases and one for an elevated alkaline phosphatase. Hemosiderosis was present in all of the biopsies. Two patients had cirrhosis. Chronic hepatitis was noted in two patients, and five patients had cholestasis. Two patients had serologic markers demonstrating HBV exposure but no patients were HBsAg positive. Erythrophagocytosis, sinusoidal dilatation, and Kupffer cell hyperplasia were present in all of the liver biopsies. Transfusion-related causes were the most common significant pathologic findings in our patients, and appeared to be the etiologies of chronic liver disease in sickle cell patients.

Adult↗
PubMed1991

[The value of nuclear magnetic resonance in the study of iron overload in thalassemia patients].

In patients with thalassemia treated by long-term chronic blood transfusions who survive beyond the first ten or twenty years of life but received no or inadequate chelating therapy during the first years, evaluation of iron overload and its consequences on tissues may prove an arduous task. MRI is a non-invasive means of measuring the amount of iron in the liver and the consequences of the iron overload on the heart and other tissues. For this purpose, MRI is more satisfactory than CT scan studies. In this investigation, 20 patients with thalassemia major underwent MRI. Multiple spin echos were used to allow determination of the transversal relaxation constant T2. This constant, expressed in ms, is related to the concentration of iron in the liver as in the following expression: (C) = 5 410/T2-110. MRI studies disclosed a discrepancy between the severity of hepatic hemosiderosis and development of decompensated iron overload cardiomyopathy. In a unique case, in which a heart transplant and two MRI studies were performed, the severe iron overload that failed to respond to several years of subcutaneous chelating therapy was more than halved by intensive intravenous chelation through a central catheter. MRI studies of the heart provide valuable morphologic and functional data. Although the amount of iron in the myocardium cannot as yet be quantified, modifications of the transversal relaxation time provide information on the severity of the overload and the presence of other myocardial alterations.

Adolescent↗
PubMed1990

[Possibilities of instrumental biopsy methods in the diagnosis of rarely occurring disseminated processes of the lungs].

Fifty eight patients with disseminated lung lesions were investigated, including 19 with histiocytosis X, 5 with alveolar microlithiasis, 8 with pulmonary phospholipoproteinosis, 16 with idiopathic hemosiderosis, 1 with primary pulmonary amyloidosis and 9 with primary diffusive tumors of the lungs. In all the above cases, a complex bronchological examination was undertaken with the use of different biopsy transbronchial procedures as well as an open biopsy of a lung. Diagnostic value and efficiency of bronchobiopsies were considered. Recommendations concerning the use of different instrumental techniques were formulated.

Adolescent↗
PubMed1987

[Pathologico-morphologic picture of subchronic trimorfamide poisoning in dogs].

Animals in groups of six dogs and six bitches were given daily in their food, individually, for a period of six months, the fungicide trimorphamide, at dosages of 0, 300 and 500 mg.kg-1 of their weight. After they had been killed, a significant reduction in their weight was observed along with hemosiderosis of the liver, kidneys and spleen of all the experimental animals. No other important structural changes were found by dissection and pathologico-histological examinations of almost all organs and tissues.

Animals↗
PubMed1986

Paediatric liver disorders in Singapore.

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.

Adolescent↗
PubMed1986

Allergic diseases in the lungs.

Four basic immune reactions in the lung are fundamental to our understanding of allergic lung diseases. The first, immediate hypersensitivity, principally involves airway obstruction because of hypersecretion and bronchospasm. Resulting roentgenographic changes are those of large and small airway disease. The second reaction, cytotoxic antibody-mediated disease, produces air space filling by hemorrhage. The third form, immune complex-mediated hypersensitivity, causes mixed patchy consolidation and interstitial roentgenographic patterns caused by inflammation stimulated by antigen-antibody complexes. Fourth, cell-mediated immunity usually produces a granulomatous reaction, roentgenographically manifest by nodules, masses, and enlarged lymph nodes. Applying our understanding of these four basic reactions permits understanding the common allergic disease of the lungs: hypersensitivity pneumonitis, asthma, allergic bronchopulmonary fungal disease, chronic eosinophilic pneumonia, hypereosinophilic syndrome, Goodpasture's syndrome and idiopathic pulmonary hemosiderosis (IPH), Wegener's granulomatosis, and allergic granulomatosis (Churg-Strauss disease). The clinical, pathologic, and roentgenographic manifestations of these diseases are explained and related to the basic immune mechanisms.

Alveolitis, Extrinsic Allergic↗
PubMed1987

[Iron reserves in patients with chronic renal insufficiency].

Hemorrhage values and the amount of iron entering the body with drugs and blood transfusions were determined in 107 patients with the terminal CRF stage. Of them 59 received regular hemodialyses. The level of serum iron and ferritin as well as iron reserves in the body were investigated at the start and end of the study. In the end a histochemical study of the content of hemosiderin in the bone marrow, liver and spleen was performed. A close interrelationship of iron reserves determined with a modified desferal test and the level of serum ferritin (r = 0.94) was established. The highest iron reserves were revealed in the patients receiving blood transfusions and parenteral iron drugs. Criteria for the assessment of iron reserves in patients with renal failure were determined by means of the modified desferal test and investigation of serum ferritin. Normal ferritin reserves in such patients corresponded to serum ferritin values within the range of 50-400 micrograms/l and indices of the modified desferal test ranging from 0.4 to 2.0/0.5 g of desferal. Of a degree of hemosiderosis one could judge on the basis of a histochemical investigation of tissue hemosiderin only. Iron drugs per os were proposed for the prevention of disorders of iron balance in patients with renal failure.

Adolescent↗