The adult respiratory distress syndrome bronchogenic pulmonary tuberculosis

In three cases of pulmonary tuberculosis associated with the adult respiratory distress syndrome the clinical features, which were similar to those of patients with miliary tuberculosis and adult respiratory distress syndrome, included a history of cough, fever, and dyspnoea on effort, and the physical signs of fever, tachypnoea, pulmonary adventitious sounds, tachycardia, and hepatomegaly. In these cases the radiological features, though suggestive of diffuse pulmonary oedema, were more prominent on the side in which the cavitatory lesion appeared. The diagnosis of tuberculosis was made easily from direct examination of sputum. Despite early ventilatory support and antituberculous therapy, two of the three patients died. Postmortem examination of the lungs in these cases showed evidence of acute alveolar damage (loss of type 1 pneumocytes and the presence of hyaline membranes within alveolar ducts) and of chronic alveolar damage (interstitial and alveolar fibrosis).     

Lymphoid hyperplasia and eosinophilic pneumonia as histologic manifestations of amiodarone-induced lung toxicity

Amiodarone use is often limited by pulmonary toxicity. Amiodarone lung disease (ALD) classically manifests as organizing pneumonia with intra-alveolar foamy macrophages, but other patterns may also occur. Here we report 2 previously unreported patterns of ALD: lymphoid hyperplasia (LH) and eosinophilic pneumonia (EP). We identified patients with LH or EP as a prominent feature among 75 cases of probable ALD from the authors' teaching files collected from 1997 to 2010. Clinical history and lung wedge biopsies were reviewed. Twelve patients (7 men) met inclusion criteria (median age, 71 y). The exact amiodarone dose was known in all cases (median, 200 mg/d). Treatment duration was known in 10 cases and ranged from 1 to 12 years. Thoracic imaging showed diffuse infiltrates causing concern for a diagnosis of ALD. Histologic review revealed intra-alveolar foamy macrophages in all cases. Eight cases prominently displayed patterns of LH, including diffuse LH (7), follicular bronchiolitis (5), lymphoid interstitial pneumonia (2), and lymphocytic perivascular cuffing (2). Two showed features of acute EP, including diffuse alveolar damage with abundant eosinophils. Two showed features of chronic EP, including interstitial pneumonia with abundant eosinophils, patchy organization, and fibrinous exudates with macrophages and eosinophils. One chronic EP case also showed focal LH. Additional features included intra-alveolar giant cells (6), pleuritis (3), small poorly formed granulomas (3), and thrombi (2). LH and EP are previously unrecognized histopathologic manifestations of ALD, and amiodarone exposure should be included in their differential diagnosis.     

Cerebral alveolar echinococcosis. A case report with MRI and review of the literature

Alveolar echinococcosis is an important zoonotic infection caused by the larval stage of the Echinococcus multilocularis. It is endemic to North America, Central Europa, Russia, China and Turkey. The liver and the lung are the organs most commonly involved. Cerebral alveolar echinococcosis is rare accounting for only 1% of cases. We present a 55-year-old patient with a right frontal mass. T2-weighted MRI series revealed a grape like multilobular, heterogeneous mass with low density. A diagnosis of glial tumor was made. The mass was totally removed. The histopathological examination showed a diffuse growth composed of compartments that are filled with a gelatinous matrix and many brood capsules and protoscolices filled with necrotic tissue. Histopathological findings were consistent with the diagnosis of alveolar echinococcosis. No postoperative complications were observed. There were no lesions in the liver and lungs. The patient was started on albendazole (ABZ) at a daily dosage of 800 mg for 3 months. The patient has remained free of any mass lesion for 5 years. Hypointense grape-like mass with calcification and surrounding white matter edema in T2-weighted MRI should suggest cerebral alveolar echinococcosis. Radical surgery and an adjuvant therapy with ABZ provides useful prolongation of life.     

Multiple nodular pulmonary amyloidosis. A case report and comparison with diffuse alveolar-septal pulmonary amyloidosis.

A case of multiple nodular pulmonary amyloidosis in a 54-year-old Caucasian man is presented. Discrete symptomless radiodensities had developed in this patient's lungs within a period of three years, leading to a suspicion of a neoplastic process. The amyloid nature of these nodules was demonstrated by biopsy. In this case, as in others previously reported, there was no evidence of systemic disease, and immunoglobulins were normal. Local factors probably play an important part in the pathogenesis of this disease. This entity is to be distinguished from the diffuse type of pulmonary amyloidosis, which has a far graver prognosis. Diffuse alveolar septal amyloidosis is usually associated with primary systemic amyloidosis or multiple myeloma and leads rapidly to respiratory distress.     

Pulmonary alveolar microlithiasis with involvement of the sympathetic nervous system and gonads

Pulmonary alveolar microlithiasis is a relatively rare disease, of obscure aetiology, manifested by the presence of intra-alveolar laminated concretions distributed diffusely throughout the lungs. In all the recorded cases disease has been limited to the lungs and similar lesions have not been observed in other organs or tissues.     

Crescentic glomerulonephritis in children: a review of 43 cases.

Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 +/- 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in greater than 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocellular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis.     

Liver Transplantation in Management of Alveolar Echinococcosis: Two Case Reports

Hepatic alveolar echinococcosis is an infectious disease caused by the larval stage of Echinococcus multilocularis, which grows primarily in the liver of an infected person and develops as a tumorlike lesion. In advanced cases, the organisms infiltrate every organ neighboring the liver and spread hematogenously to distant organs such as lungs and brain. Surgical resection and liver transplantation are accepted treatment options for early and advanced disease, respectively. Herein, we present case reports of 2 patients with advanced alveolar echinococcal disease that invaded both lobes of the liver and neighboring vital structures including the inferior vena cava. Despite the technical difficulty of the surgery, both patients were successfully treated with living donor liver transplantation. Liver transplantation should be accepted as a life-saving treatment of choice in patients with alveolar echinococcosis for whom there is no other medical or surgical treatment options.     

Dialysis Dementia - The Chicago Experience

In the four years between 1972 and 1976 twenty out of 160 maintenance dialysis patients developed dialysis dementia. Their illness was characterized by an insidious onset of mental deterioration, speech disturbance, apraxia, and myoclonus. The disease progressed inexorably to a fatal outcome, the onset of seizures being an ominous sign, and the average duration of the illness being seven months. Routine biochemical studies were unremarkable, and osteodystrophy was not a prominent feature. Serial electroencephalograms (EEC) showed progressive slowing of the rhythm, usually antedating the neurologic symptoms. Brain scan and flow studies were normal. Radio-iodinated serum albumin (RISA) scans in seven patients showed changes suggesting altered cerebrospinal fluid (CSF) dynamics. Treatment was generally ineffective, but ventriculo-peritoneal shunting produced transient neurologic improvement in one patient. Epidemiologic investigations showed high aluminum levels in city water during the period of the outbreak.     

Pulmonary changes and cor pulmonale in mucoviscidosis

The lungs of eight cases of cystic fibrosis in patients ranging from age 19 days to 23 years were examined by the large-section technique of Gough and Wentworth (Gough, 1960). All cases showed in life a susceptibility to staphylococcal and pseudomonal bronchopneumonia. In all cases the bronchial tree was plugged with mucus. The lungs from the older patients showed diffuse and pronounced bronchiectasis with luxuriant peribronchial granulation tissue and dilatation of the pulmonary arterial system. The distal air spaces in all cases showed only minimal distension and only in the eldest was there any evidence of destructive emphysema. Three cases developed cor pulmonale which we thought was the result of hypoxaemia due to a ventilation-perfusion imbalance.     

Alveolar integrity and ultrastructure in pigs remain undamaged after exposure to sevoflurane

Previous studies have shown that both halothane and isoflurane have adverse but reversible effects on alveolar physiology. The present study was designed to test the hypothesis that also sevoflurane may affect alveolar integrity.
Fifteen pigs were randomly selected to receive either thiopentone infusion (control group, n=8) or sevoflurane (n=7) at 4.0% inspiratory concentration (1.5 MAC) in air for 6 h. Tissue samples from the lungs were obtained at the end of the experiment. Both histopathological light microscopy and electron microscopy were used to assess the structural integrity of the alveoli.
Pulmonary hemodynamics were comparable in both groups. Light microscopy showed no difference between the groups in the amount of alveolar macrophages, red blood cells or edema. Electron microscopy showed minor changes such as moderate local swelling of alveolar epithelium in both study groups. Alveolar type II cells were ultrastructurally unaltered in both study groups.
We conclude that long-term, high concentration exposure to sevoflurane has no detrimental effect on the alveolar integrity in pigs.     

Morphological changes in the lungs and characteristics of thromboemboli in patients with thromboembolism of the pulmonary artery

Basing on results of pathologoanatomical investigations, performed in 26 cases, the lungs changes in pulmonary thromboembolism were analyzed. It was shown, that an acute thromboembolism of a. pulmonalis main branches did not cause an acute necrosis of pulmonary alveolar tissue, but induces progressive diffuse changes of their microcircular bed, concomitant with pulmonary hypertension. In cases of chronical pulmonary thromboembolism the diffuse vascular sclerosis of the nonaffected lung and the alveolar tissue emphysema are formed, and in such cases the signs of pulmonary hypertension showing hypertrophy of the muscular vessels middle layer as well as their intima hyperplasia are revealed. This determines the expediency of earlier start of thrombolytic therapy conduction as well as surgical intervention in an acute pulmonary thromboembolism.     

Crescentic Glomerulonephritis Associated with Non-Tuberculous Mycobacteria Infection

A 72-year-old woman with a previous diagnosis of non-tuberculous mycobacteria (NTM) pulmonary disease was admitted because of hemoptysis and acute renal failure. A chest x-ray showed interstitial infiltration over bilateral lung fields. A kidney biopsy showed immune complex-mediated crescentic glomerulonephritis and diffuse endocapillary hypercellularity with exudative neutrophils. Reactive NTM infection of the lungs was suspected when mycobacterial cultures of the sputum repeatedly yielded Mycobacterium avium. A lung biopsy revealed chronic inflammation without evidence of alveolar capillaritis. NTM pulmonary disease was further confirmed by tissue culture of the lung biopsy specimens. Anti-tuberculous drugs in combination with clarithromycin were given for the treatment of NTM infection. Because of the risk of aggravating underlying infectious disease, immunosuppressive therapy for crescentic glomerulonephritis was not carried out. Pulmonary symptoms promptly responded to treatment. Furthermore, renal function steadily improved after the initiation of anti-NTM therapy. To our knowledge, this is the first report of crescentic glomerulonephritis associated with NTM infection.     

Sirolimus-Induced Pneumonitis: Three Cases and a Review of the Literature

Interstitial pneumonitis is a rare disease that is seen in the context of some infections (e.g. PCP and CMV pneumonia), as side-effects of drugs (e.g. beta-blockers, amiodarone) and rarely in the context of renal transplantation. It manifests itself usually as a pneumonic illness; with symptoms of dyspnea, cough, fatigue and sometimes fever. Characteristic radiological changes are bilateral lower zone haziness. Interstitial pneumonitis is now emerging in solid organ transplant patients secondary to sirolimus). We describe three cases of sirolimus-induced pneumonitis in two patients who started sirolimus to permit cyclosporin withdrawal and in one patient initially started on sirolimus. The presentations in these cases ranged from insidious to fulminant; there was a rapid response to sirolimus withdrawal. This is an important syndrome, with an unknown frequency.     

Crescentic glomerulonephritis associated with non-tuberculous mycobacteria infection

A 72-year-old woman with a previous diagnosis of non-tuberculous mycobacteria (NTM) pulmonary disease was admitted because of hemoptysis and acute renal failure. A chest x-ray showed interstitial infiltration over bilateral lung fields. A kidney biopsy showed immune complex-mediated crescentic glomerulonephritis and diffuse endocapillary hypercellularity with exudative neutrophils. Reactive NTM infection of the lungs was suspected when mycobacterial cultures of the sputum repeatedly yielded Mycobacterium avium. A lung biopsy revealed chronic inflammation without evidence of alveolar capillaritis. NTM pulmonary disease was further confirmed by tissue culture of the lung biopsy specimens. Anti-tuberculous drugs in combination with clarithromycin were given for the treatment of NTM infection. Because of the risk of aggravating underlying infectious disease, immunosuppressive therapy for crescentic glomerulonephritis was not carried out. Pulmonary symptoms promptly responded to treatment. Furthermore, renal function steadily improved after the initiation of anti-NTM therapy. To our knowledge, this is the first report of crescentic glomerulonephritis associated with NTM infection.     

宏基因二代测序协助诊断毛霉菌病一例并文献复习

目的:探讨宏基因二代测序(mNGS)协助诊断侵袭性真菌病的临床应用价值,为临床诊断和治疗毛霉菌病提供病原学依据。方法:通过mNGS技术检测1例患有不明原因肺部感染者的肺泡灌洗液以确定病原体,并根据mNGS检测报告调整其治疗方案。结果:患者间断胸痛、咳嗽5月余,胸部CT示双肺呈多发性阴影伴空洞形成,肺组织活检提示凝固性坏死,常规实验室及病原学检查均未见异常,行多种抗感染治疗方案均未见明显好转,后取肺泡灌洗液送检mNGS提示病原体为总状横梗霉菌,改用两性霉素B行抗感染治疗后,患者感染病灶缩小,症状明显缓解。结论:mNGS是鉴定罕见病原体的重要辅助诊断方法,对协助诊断肺部侵袭性真菌病具有重要意义。     

Influence of intravenous injection of sclerosing agents on the respiratory function

Severe lung injury (ARDS) has occasionally been observed after sclerotherapy for bleeding oesophageal varices. In order to study the effects of sclerosing agents, which may escape into the systemic circulation during treatment, seven sheep were given either ethanolamine oleate (n = 4) or sodium tetradecyl sulfate (n = 3) intravenously. A control group (n = 3) was treated identically with the others except it did not receive any sclerosing agent. The study showed that both sclerosing agents caused an immediate and severe fall in total respiratory compliance and arterial oxygen tension. There was a marked trapping of platelets in the lungs, which was also reflected by a drop in platelet count in peripheral blood. The lungs from the animals receiving sclerosing agents appeared moderately to severely congested and the wet/dry weight ratio of the lungs was significantly increased compared with untreated normal lungs (p less than 0.01). Histopathological examination revealed severe damage to the alveolar membranes, intraalveolar fibrino-haemorrhagic exudate, collapse of alveolar spaces and numerous eosinophilic leukocytes in the broadened, oedematous alveolar walls. It was concluded that the sclerosing agents used in this study, ethanolamine oleate and sodium tetradecyl sulphate, cause severe lung injury if given intravenously in sheep in doses corresponding to 25-50% of what is normally used during sclerotherapy in patients. The mechanism of this action may be that of an increased microvascular permeability causing marked alveolar damage and destruction of the blood gas barrier of the lungs.     

Busulphan lung

A 61-year-old man with chronic myeloid leukaemia was treated with busulphan (Myleran). After receiving 1 g. of this drug over a period of 20 months he became dyspnoeic and developed crepitations in the lungs. Two months later radiographs of the chest revealed peri-hilar infiltrates and subsequently diffuse mottling throughout both lungs. Lung function tests showed a gross impairment of the transfer factor to a quarter of the predicted normal. At necropsy the lungs showed a striking proliferation of granular pneumocytes, many of which had disintegrated to produce intra-alveolar debris, some of which showed organization by fibrous tissue. There was associated interstitial pulmonary fibrosis. Electron microscopy confirmed the desquamated alveolar cells to be type II (granular) pneumocytes containing characteristic lamellar bodies. Many of these osmiophilic bodies, believed to be the source of pulmonary surfactant, had been liberated into the alveolar spaces, with the formation of phospholipid myelin figures and lattices. We think that the basic pathology of busulphan lung is a chemically induced alveolitis with proliferation of granular pneumocytes followed by fibrosis of alveolar walls and intra-alveolar contents.     

Multifocal subacute pyogenic osteomyelitis in a child. A case report.

The clinical features of multifocal subacute pyogenic osteomyelitis are: insidious onset; the absence of toxic reaction; radiographic appearance of a bone neoplasm. In a 14-year-old boy, immobilization and appropriate antibiotics produced symptomatic improvement and radiographic evidence of healing reactive bone above both bone lesions.     

Nodular amiodarone lung disease

The antiarrhythmic drug amiodarone accumulates in many organs of the body. Amiodarone lung disease (ALD) most commonly manifests clinically as an interstitial pneumonitis. The few reports of nodular ALD generally have been in the clinical and radiographic literature. No detailed histopathologic analysis of nodular ALD is available. We report 4 patients with nodular ALD, all of whom had excision of a nodule and none had a preoperative diagnosis of ALD. The radiographic suspicion before excisional biopsy in all 4 cases was malignancy. The initial pathologic suspicion in all 4 cases was either an abscess or vasculitis. In 3 of the 4 cases, where the dosages were known, each patient received 800 mg/d for 7 or more months. All cases have strikingly similar histopathology, with vacuolated histiocytes massed within alveoli to form macroscopic nodules with tissue breakdown. Ultrastructural examination of lung and peripheral nerve in 1 case showed the characteristic inclusions of amiodarone in the cytoplasm of swollen histiocytes. Vacuolated histiocytes filled with these inclusions indicate the effect of the drug, but in these 4 cases there was tissue destruction, which indicated disease. The necrotizing nature of the massed histiocytes in the absence of infection or obstruction suggests the correct etiologic diagnosis even when the history of administration of the drug is not available at initial review.     

Pulmonary bacterial defense: Effect of the burn wound on transfer of alveolar macrophage activation in rats by parabiosis

The alveolar macrophage was studied in parabiotic rats using an inbred strain. Parabiotic pairs were sutured together at five weeks of age. Rats were subjected to a full thickness cutaneous burn of 20 per cent of the body surface area at seven weeks of age, and alveolar macrophages were washed from the lungs at six days post burn. The number of alveolar macrophages, their per cent of activation, and their ability to phagocytize and kill P aeruginosa in vitro were significantly increased at six days post burn in the burned controls and in both the burned and unburned members of the parabiotic pairs. No change in the alveolar macrophages was found in either unburned parabiotic pairs or in those which were sham-burned. These results indicate that a humoral or cellular agent produced either within the cutaneous burn wound or elsewhere as a response to the injury, traverses the parabiotic cross circulation to stimulate the alveolar macrophages.