Fulminant hepatic failure related to immunosuppressive therapy in an asymptomatic HBsAg carrier with idiopathic interstitial pneumonia

A 72-year-old woman who was an asymptomatic HBsAg carrier was admitted because of productive cough and dyspnea on exertion. After close examination, she was given a diagnosis of idiopathic interstitial pneumonia (IIP), and treated with glucocorticoids and cyclophosphamide. However, fulminant hepatitis type B developed and the patient died of respiratory complications stemming from the acute exacerbation of IIP. To avoid such outcomes, extra caution should be taken when planning immunosuppressive therapy for HBsAg carriers.     

High levels of elastolytic activity in bronchoalveolar lavage fluid from a patient with idiopathic interstitial pneumonia]

A 63-year-old female with acure exacerbution of idiopathic interstitial pneumonia (IIP) showed high levels of elastolytic activity in bronchoalveolar lavage fluid (BALF). She was admitted to our hospital with progressive dyspnea. Arterial blood gas analysis and pulmonary function tests were abnormal. Examination of the bronchoalveolar lavage fluid revealed an increased numbers of neutrophils. Despite the administration of corticosteroids, the patient died of respiratory failure. A high level of elastolytic activity was present in the BALF. Western immunoblot analysis, using an anti-alpha 1-protease inhibitor (PI) antibody, revealed a truncated alpha 1-PI in the BALF. These findings suggest that an imbalance between protease and PI in the lower respiratory tract contribute to lung tissue damage in patients with IIP.     

Dermatological indicators of coronary risk: a case-control study

Varicella pneumonia is the most common complication of adult varicella. Symptoms may be severe and the mortality rate high in patients who are immunodeficient or pregnant. Symptoms may be mild and progression more favorable in adults previously in good health. We report two cases of varicella infection complicated by severe pulmonary involvement in adult patients who were previously healthy. Case 1 was a 36-year-old male who 6 days after developing varicella was clinically observed to have dyspnea and hemopytsis. He died of acute respiratory failure on the following day. Case 2 was a 28-year-old male whose respiratory symptoms started the third day after developing varicella. These symptoms were relieved by treatment with acyclovir and gammaglobulin. Careful observation is and an early treatment of varicella should be undertaken not only for patients with suppressed cellular immunity, but also for healthy adults, to prevent severe complications.     

Reduction of islet amylin expression and basal secretion by adenovirus-mediated delivery of amylin antisense cDNA

A 55-year-old woman was admitted to our hospital with progressive dyspnea that had begun one month before. Chest rentogenogram revealed groundglass appearance and reticular shadows bilaterally. Pulmonary function tests showed both decreased vital capacity and diffusing capacity. Bronchoalveolar lavage fluid had a high lymphocyte fraction with a low CD4+/CD8+ ratio. Thoracoscopic lung biopsy revealed thick, fibro-edematous interstitium and diffuse infiltration of lymphocytes. We also observed an intra-alveolar exudate with infiltration of histiocytes and lymphocytes. The clinical features and pathological findings were consistent with subacute interstitial pneumonia, which was the entity proposed by Kawabata and colleagues. The patient developed acute respiratory failure four days after lung biopsy and died despite steroid pulse therapy. Although subacute interstitial pneumonia has been reported to respond to steroid therapy, and to have a good prognosis, we believe that subacute interstitial pneumonia could fatally worsen when associated with lung biopsy, infection, or some other stimulus.     

A case of sarcoidosis associated with chronic eosinophilic pneumonia

A 38-year-old man was hospitalized in our university hospital because of pulmonary opacities with bilateral hilar and mediastinal lymphadenopathy seen on chest radiograph. Eosinophilia was observed in the circulation and bronchoalveolar lavage (BAL) fluid. Histological examination revealed noncaseating epithelioid granulomas and eosinophilic infiltration in the lung. Based on these findings, a diagnosis of sarcoidosis combined with chronic eosinophilic pneumonia was made. The infiltrates on chest radiograph and BAL eosinophilia were promptly reduced with corticosteroid therapy, but only mild reduction was observed in diffuse nodular shadows and hilar and mediastinal lymphadenopathy, and high amounts of lymphocytes in BAL fluid remained. Increased IFN-gamma, IL-4 and IL-5 were detected in the BAL fluid, and corticosteroid therapy reduced IL-4 and IL-5 (Th-2 cytokines) but not IFN-gamma (Th-1 cytokine). These cytokine levels in BAL fluid were intimately correlated with the clinical course of sarcoidosis and chronic eosinophilic pneumonia.     

Myasthenia gravis presenting as isolated respiratory failure

We present 2 cases of idiopathic CD4+ T-lymphocytopenia (ICL) in elderly patients. Case 1, a 73-year-old man, with pneumonia had received several antibiotics with unsuccessful results at another hospital. On admission, his CD4+ T-lymphocyte count was 109/microl and Pneumocystis carinii was detected by bronchoalveolar lavage fluid staining. No evidence of human immunodeficiency virus (HIV) infection was found. Despite therapy, the patient died of respiratory failure. Case 2, a 72-year-old man, contracted severe pneumonia, and Hemophillus influenzae was believed to be the pathogen. On admission, his CD4+ T-lymphocyte count was 238/microl. No evidence of HIV infection was found. He received antibiotics and improved successfully. We suggest that ICL may currently be incubating in a number of elderly pneumonia patients.     

Acute interstitial pneumonia (Hamman Rich syndrome)

We report the case of a 65-year-old woman with no history of respiratory disease who suffered onset of dyspnea after an episode of pseudoinfluenza. Dyspnea progressed such that within 15 days it was triggered by minimal effort. The patient died 15 hours after admission to our hospital, with a clinical picture of adult respiratory distress. Autopsy allowed us to rule out several diseases and arrive at a diagnosis of acute interstitial pneumonia, consistent with clinical course, anatomical and pathological findings as described in the literature.     

High levels of interleukin-8 in the blood and alveolar spaces of patients with pneumonia and adult respiratory distress syndrome

There is ample experimental evidence that polymorphonuclear neutrophils (PMN) play a critical role in the pathogenesis of the adult respiratory distress syndrome (ARDS). Since interleukin-8 (IL-8) is a strong chemotactic factor for PMN, we measured IL-8 levels in plasma and bronchoalveolar lavage (BAL) fluid of 18 patients, 12 with ARDS and 6 with severe pneumonia uncomplicated by ARDS, all of whom had an increased number of PMN in BAL fluid. Seven healthy subjects served as controls. We found elevated levels of IL-8 in the alveolar spaces of all patients tested. Elevated BAL IL-8 levels were related to a fatal outcome and the presence of shock and correlated with a general clinical severity index (simplified acute physiological score). BAL fluid levels of IL-8 were significantly higher in patients with ARDS than in patients with pneumonia. In plasma, IL-8 levels were increased similarly in all patients and did not correlate with survival or the presence of shock. The BAL fluid-to-plasma ratio of IL-8 was significantly greater than that of tumor necrosis factor alpha, indicating higher local production of IL-8. Moreover, the presence of a primed subpopulation of blood PMN with respect to H2O2 production indicates that IL-8 may contribute to the neutrophil-mediated process in the pathogenesis of ARDS and pneumonia.     

Leukotriene B4 and interleukin-8 in human immunodeficiency virus-related pulmonary disease

STUDY OBJECTIVE: To investigate the pathogenesis of lung injury in Pneumocystic carinii pneumonia and nonspecific interstitial pneumonitis (NIP), common pulmonary complications of human immunodeficiency virus (HIV) infection. The efficacy of corticosteroid therapy in P carinii pneumonia and the observation that bronchoalveolar lavage (BAL) neutrophilia predicts a poor prognosis support the premise that the lung injury of P carinii pneumonia is due to the host's inflammatory response to the infection. DESIGN: In vitro measurements on previously collected BAL fluid samples. SETTING: The Clinical Center of the National Institutes of Health, a research hospital and tertiary care referral center. PATIENTS: Five normal volunteers, 5 asymptomatic HIV-positive patients, 10 HIV-positive patients with NIP (5 asymptomatic and 5 with respiratory symptoms), and 19 HIV-positive patients with P carinii pneumonia. MEASUREMENTS AND RESULTS: BAL leukotriene B4 (LTB4), interleukin 8 (IL-8), and phospholipase A2 (PLA2) were measured. IL-8 and PLA2 were elevated in patients with P carinii pneumonia, and IL-8 correlated with BAL fluid absolute neutrophil count. LTB4, IL-8, and PLA2 levels were elevated in patients with NIP; LTB4 and PLA2 levels correlated with absolute neutrophil count, and IL-8 correlated with alveolar-arterial oxygen pressure difference. IL-8 was elevated in the asymptomatic HIV-positive patients, and there was a trend toward elevation of PLA2 in this group. CONCLUSION: IL-8 appears to play a role in the pathogenesis of lung injury in P carinii pneumonia and may be the principal neutrophil chemotaxin in this disease; PLA2 may also be involved in the pathogenesis of P carinii pneumonia. Both LTB4 and IL-8 may be involved in the recruitment of neutrophils and subsequent lung injury of NIP. These data suggest that there are varying mechanisms by which inflammatory cells are recruited to the lung in different HIV-related lung diseases.     

Radiological evidence of involvement of dust exposure in pathogenesis of pulmonary alveolar proteinosis

A 52-year-old man was admitted because of increasing dyspnea on exersion and presence of pulmonary infiltrates. The patient had pulmonary tuberculosis at the age of 31, which resulted in volume loss and calcified foci in the upper lobe of his left lung. As a construction worker for more than 20 years, he had been exposed to inorganic dusts. Chest radiographs showed a symmetrical consolidation of infiltrates in both lungs with the exception of the left upper lobe, where no apparent infiltrates were shown. A computed tomographic scan of the chest revealed widely panlobular consolidation with the exception of the left upper lobe. A diagnosis of pulmonary alveolar proteinosis (PAP) was established by analysis of bronchoalveolar lavage fluid. Although the patient underwent segmental bronchoalveolar lavage four times under general anesthesia, he suffered frequent pulmonary infection and died two years after the onset his symptoms. Interestingly, the patient had a markedly narrowed orifice in the left upper lobe, as demonstrated by fiberoptic bronchoscopy. Chest radiographs of this lung field revealed no infiltrative shadows. These results suggest that some inhalative agent was involved in the pathogenesis of PAP in this case. In addition, significantly increased levels of KL-6 detected in both serum and bronchoalveolar lavage fluid were attributable to overproduction of KL-6 by Type II pneumocytes that had been stimulated or damaged by PAP.     

A clinical study of chronic lower respiratory infection with Haemophilus influenza by transtracheal aspiration

We have performed a clinical study on chronic lower respiratory tract infection (CLRTI) with Haemophilus influenzae (H. influenzae) by transtracheal aspiration (TTA) and analyzed clinical factors of the acute exacerbation. In 40 episodes (38 cases) of H. influenzae isolated from CLRTI, monobacterial infection with H. influenzae were 21 episodes and polymicrobial infection were 19 episodes. We classified the disease into acute exacerbated (27 episodes) and stable (13 episodes) phase and the former episodes were divided into bronchitis type (7 episodes) and pneumonia type (20 episodes). Polymicrobial infections were seen more in the pneumonia type (13 episodes) than in the bronchitis type (2 episodes). The principal organism detected with H. influenzae were alpha-Streptococcus and Neisseria sp. in the bronchitis type and S. pneumoniae in the pneumonia type. The acute exacerbated cases were divided into the following 4 patterns; 1. polymicrobial infection with continuous infection of P. aeruginosa, 2. monomicrobial infection after acute upper respiratory tract infection, 3. polymicrobial infection with S. pneumoniae after continuous infection of H. influenzae, 4. bacterial replacement by P. aeruginosa after acute exacerbation. The results of the study suggests that polymicrobial infection is an important chronic lower respiratory tract infection when caused H. influenzae.     

Colchicine induced rhabdomyolysis

A 59-year-old man with chronic obstructive pulmonary disease (COPD), atrial fibrillation, and gout developed acute dyspnea, cough, and diffuse muscle aches and pains. He had commenced colchicine (0.6 mg b.i.d. p.o.), for the first time, one month earlier for recurrent gout attacks. Clinical examination revealed atrial fibrillation, an exacerbation of his pulmonary disease, tender muscles, especially calves, and diffuse muscle weakness. Laboratory results included creatinine phosphokinase 6961 U/l (1% MB), microscopic hematuria, myoglobinuria, elevated creatinine 1.6 mg/dl, and blood urea nitrogen 17 mg/dl. COPD and atrial fibrillation were treated and colchicine was discontinued. The patient made a full recovery. This 2nd reported case of colchicine induced rhabdomyolysis is the first reported in the treatment of gout.     

Respiratory manifestations of carbamazepine. Apropos of a case

A 14-year-old girl developed skin rash, fever and dyspnea. The chest roentgenogram showed diffuse reticulonodular infiltration. Pulmonary function tests revealed mild restrictive defect and blood oxygen pressure at 71 mm Hg. BAL showed increased cell counts with lymphocytosis at 15% and neutrophilia at 3%. Outcome was good after carbamazepine withdrawal and without corticosteroid therapy. Relapse was observed after patient-induced rechallenge.     

Edward Tyson''s case of an American with gastric perforation

Pulmonary alvelolar proteinosis (PAP) is a rare cause of chronic respiratory failure due to progressive alveolar accumulation of a periodic acid-schiff (PAS) positive proteinaceous material. In some cases, the rapid accumulation of intra-alveolar material leads to acute respiratory failure (ARF). We report the causative role of secondary PAP in the case of a 26-year-old man with acute myeloid leukemia who developed fever, increased serum lactate dehydrogenase level and ARF, and required mechanical ventilation. The diagnosis of PAP was established by the examination of material obtained by bronchoalveolar lavage (BAL). Respiratory improvement occurred several days after the patient had recovered from neutropenia. This report underlines the importance of the early diagnosis of PAP as a potential cause of ARF in leukemic patients. Adequate stain on BAL fluid provides the diagnosis and avoids repeated invasive procedures and inappropriate treatments.     

Endoscopic injection of botulinum toxin for biliary sphincter of Oddi dysfunction

A 66-year old man was admitted to our hospital because of vomiting, diarrhea and progressive dyspnea. Acute respiratory distress syndrome (ARDS) due to bilateral pneumonia was diagnosed and he was also in septic shock. The patient had a history of partial hepatectomy for hepatoma, and suffered from liver cirrhosis. Emergency bronchoalveolar lavage (BAL) revealed abundant gram-positive cocci and polymorphonuclear leukocytes in the collected sample. Blood culture revealed corynebacterium. Treatment consisted of mechanical ventilation and administration of fluids, effective antibiotics, and high-dose methylprednisolone (MPS). MPS was administered from the onset of ARDS with a starting dose of 1,000 mg, which was gradually reduced to 60 mg over 8 days. Pulmonary infiltrates shown on the chest X-ray film were alleviated, and arterial blood gas data rapidly improved. The patient was successfully extubated on the 10th hospital day, and discharged on the 30th hospital day. Serial BAL and plasma levels of inflammatory cytokines decreased rapidly in parallel with the improvement of the patient's clinical condition. This is a case report of severe bacterial pneumonia that was successfully treated with effective antibiotics and high-dose MPS for several days from the onset of ARDS.     

Two cases of the congenital posterolateral diaphragmatic hernia were reported

Case 1, a 9-year-old woman, was admitted to our hospital because of nausea, vomiting, and epigastralgia. Diagnosis of Bochdalek hernia was made by the unusual course of naso-gastric tube. At surgery through the left posterorateral thoracotomy, the herniation of the stomach, small intestine, and colon to the thoracic cavity through the dorsolateral defect of the diaphragm were revealed. Case 2, a 35-year-old man, was admitted to our hospital because of dyspnea. Similar diagnosis was made by the examination of upper G1 series and barium enema, which demonstrated the presence of multiple loops of the small intestine and colon in the left thoracic cavity. Their postoperative courses were uneventful. Most of Bochdalek hernia is observed in infancy, and adolescent or adult case is is rarely reported (approximately 10% of all cases). Since this often misdiagnosed as pleuritis or pulmonary tuberculosis, a cautious examination is necessary for the establishment of the correct diagnosis.     

Two cases of Swyer-James syndrome

Case 1: 55-year-old male was admitted to our hospital for further examination of increased transparency of X-ray in the left lower lung. He had history of pneumonia in his childhood. Left bronchography revealed mild cylindrical bronchi-ectasia in the proximal bronchi but poor filling by contrast in the peripheral bronchi. Case 2: 61-year-old male was referred to our hospital with palpitation and dyspnea. Chest X-ray film revealed hyperlucency of the left lower lung. The ventilation scan showed a marked decrease in ventilation to the left lung and air trapping was present in the left lung. The perfusion scan showed a matched decrease in blood supply to the left lung. In both cases, pulmonary arteriogram showed narrowed and withered-tree-like left pulmonary arteries. From these clinical findings, we diagnosed these two cases as Swyer-James syndrome. We are reporting two cases of Swyer-James syndrome and describing the clinical feature, differential diagnosis and etiology of the syndrome.     

Genetic changes in colorectal adenoma and cancer in relation to various morphologic aspects

Diffuse hypoxic pneumonia was found to be caused by angiotensin converting enzyme (ACE) inhibitors in two patients given enalapril and fosinopril for hypertension. Both patients developed sub-acute respiratory failure and lost weight. Imaging explorations showed multiple areas of alveolar consolidation, moderate pleural effusion and in one case linear opacities. In both cases, peripheral eosinophila was found and the bronchoalveolar lavage fluid contained lymphocytes. Progressive improvement was achieved after withdrawal of the ACE and corticosteroid therapy for three months. Subsequent x-rays and respiratory function tests returned to normal apart from persistently low CO diffusion in one patient. In view of other cases reported in the literature, ACE inhibitors should probably be included in the list of drugs capable of inducing pneumonia, notably eosinophilic pneumonia.     

Effective treatment of thymic carcinoma with operation and combination chemotherapy against acute monocyte leukemia: case report and review of the literature

Thymic carcinoma associated with acute monocyte leukemia (AMoL) and a history of choriocarcinoma was diagnosed in a 58-year-old female. We found no other such case in a literature search. She was first treated with DCMP therapy: daunorubicin, cytosine arabinoside, 6MP-riboside, and prednisolone against AMoL. After induction chemotherapy, complete AMoL remission was attained. Chest CT scan after chemotherapy revealed regression of the mediastinal tumor. Resection of the tumor included the left upper lobe of the lung, phrenic nerve and pericardium. Pathological diagnosis showed poorly or moderately differentiated squamous cell carcinoma. Although the patient died of pneumonia during chemotherapy for relapsed AMoL, chest X-ray and CT revealed no recurrence of the mediastinal tumor after the original operation. Judging from this case and other successful cases of chemotherapy, we feel that intensive chemotherapy may be a beneficial strategy against thymic carcinoma.     

Bronchiolitis obliterans organizing pneumonia in a patient with AIDS

A 35-year-old homosexual man who had a remote history of cocaine abuse presented to the hospital with fever, chills, drenching night sweats, and progressive dyspnea of 3 months' duration. His condition had been diagnosed as AIDS 1 1/2 years before presentation. Multiple blood cultures and serological tests failed to yield an infective etiology. Bronchoscopy with transbronchial biopsy, both performed twice, also failed to reveal an etiology. Empirical treatment for infection with the Mycobacterium avium complex yielded no response; empirical treatment, based on abnormalities revealed by gallium scanning, for Pneumocystis carinii pneumonia led to some clinical improvement. Because of rapid respiratory deterioration at the end of this treatment course, a thoracoscopic lung biopsy was performed; this procedure demonstrated classic bronchiolitis obliterans organizing pneumonia. Corticosteroid therapy resulted in a rapid salutary response. It is important to aggressively pursue a definitive diagnosis for selected patients with a nonidentifiable infectious cause so that patients receive the correct treatment.