21/874. Pleural fibrosis associated with dihydroergocryptine treatment. OBJECTIVES: This is the first report of a histologically confirmed pleuropulmonary fibrosis (PPF) associated with the dopamine agonist dihydroergocryptine. CASE REPORT: A 67-year-old male patient with Parkinson's disease developed a severe restrictive pulmonary disorder with dyspnea and nonproductive cough after a daily intake of 45 mg dihydroergocryptine for 2 years. After changing the dopamine agonist to the non-ergoline substance pramipexole, marked improvement of the clinical symptoms of PPF occurred, while radiological signs showed only a moderate decrease. CONCLUSION: PPF is a possibly fatal complication. Chest x-rays and specific pneumological diagnostics should be done if typical symptoms or nonspecific signs of PPF occur while a patient is on treatment with an ergoline dopamine agonist. ( info) |
22/874. Thoracic epidural anesthesia for modified radical mastectomy in a patient with cryptogenic fibrosing alveolitis: a case report. A case of advanced cryptogenic fibrosing alveolitis (CFA) with multiple bullae and extensive pulmonary fibrosis, scheduled for modified radical mastectomy for carcinoma of breast, is presented. This patient had ischemic heart disease, corticosteroid-induced hypertension, diabetes mellitus, and a difficult airway. Thoracic epidural segmental anesthesia was successfully given to this patient. Preoperative problems, perioperative management, and alternative anesthetic techniques are discussed. ( info) |
| The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome. ( info) |
24/874. Pulmonary fibrosis in a steel mill worker. We report a case of pulmonary fibrosis in a 32-year-old man, who had worked at a steel mill and who died of respiratory failure due to interstitial fibrosis despite vigorous treatment. He showed SLE-associated symptoms, such as pleural effusion, malar rashes, discoid rashes, arthritis, leukopenia, and positive antinuclear antibody and anti-histone antibody. However, he did not present anti-dna antibody. A thoracoscopic lung biopsy showed interstitial fibrosis, chronic inflammation and a small non-caseating granuloma in lung tissues, which could be induced by external agents such as metals. The manganese concentration in the lung tissue was 4.64 microg/g compared to 0.42-0.7 microg/g in the controls. The levels of other metals, such as iron, nickel, cobalt and zinc in patient's lung tissue were higher than those in the controls. The patient was probably exposed to Si and various metal dusts, and the lung fibrosis was related to these exposures. Exposure to Si and metal dusts should be sought in the history of any patient with SLE, especially in a male with pulmonary signs, and if present, exposure should be stopped. In the meantime, steps should be taken to ensure that workers exposure to Si and metal dusts in all environments have adequate protection. ( info) |
| A 78-year-old farmer presented with symptomless skin lesions for evaluation. Two years prior, he had developed idiopathic pulmonary fibrosis (IPF) and had been treated thereafter with oral prednisolone 20 mg/day and occasionally with colchicine 1 mg/day. On examination, erythematoviolaceous, slightly infiltrated plaques, measuring approximately 5 x 9 cm, rubbery in consistency, intermingled with pustules, sometimes eroded, with distinctive borders, were noted on the dorsum of both hands and on the extensor surface of both forearms. The lesions had developed over a 20-day period. The skin of these areas was atrophic or eroded with multiple ecchymoses (Fig. 1). The abnormal laboratory findings included an elevated white blood cell count of 17,100/mm3, with 79% neutrophils, 16% lymphocytes, and 5% monocytes, c-reactive protein of 33.15 mg/dL (normal, <0.8 mg/dL), and immunoglobulin g of 598 mg/dL (normal, 701-1545 mg/dL). Other blood and urine tests performed were within normal limits. The diagnosis of IPF was reconfirmed through radiology, high-resolution computed tomography, and spirometry, as well as bronchoscopy and bronchoalveolar lavage fluid analysis. Coexistence of presumptive pulmonary alternariosis was excluded. hematoxylin and eosin stained sections of the excised cutaneous specimen showed focal ulceration of the epidermis adjacent to a mainly intradermal abscess cavity. Within the latter, remnants of a partly destroyed hair follicle were seen amongst degenerating polymorphonuclear leukocytes, as well as many histiocytes and a few Langhans-type multinucleated giant cells. Minute collections of polymorphonuclear leukocytes were seen in the adjacent epidermis. periodic acid-Schiff (PAS) and Gomori's silver methenamine stains showed a multitude of broad branching fungal hyphae and large spores within the aforementioned cavity, both free and within the cytoplasm of giant cells (Fig. 2). immunohistochemistry was performed by means of the alkaline phosphatase anti-alkaline phosphatase (APAAP) method. Sections showed that the infiltrate consisted of an almost equal number of B and T lymphocytes, whereas histiocytes and the few giant cells were labeled with anti-CD68 antibodies. skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid structures. biopsy material was plated on Sabourand's dextrose agar with cloramphenicol (0.05 mg/mL). After 7 days at 27 degrees C, dark, gray-white colonies with a dark brown underside appeared. Microscopic examination of the colonies revealed hyphae with typical conidia having transverse and longitudinal septa. Based on macroscopic and microscopic examination, the isolates were identified as alternaria alternata (Fig. 3). Treatment with prednisolone was reduced to 10 mg/day and the patient received oral itraconazole (200 mg/day). This resulted in progressive improvement of alternariosis, and the lesions healed completely within 3 months, when treatment was interrupted. Two years later, there is no evidence of recurrence. ( info) |
| immunologic factors have been incriminated in the pathogenesis of lymphoid interstitial pneumonia. The discovery of a patient with coexistent lymphoid interestitial pneumonia, pernicious anemia, and common variable hypogammaglobulinemia focused attention on the possible autoimmune nature of this pulmonary disease. Extensive immunologic studies demonstrated a noticeably impaired bonemarrow-dependent (B cell) system and intact thymus-dependent (T cell) system. No evidence of humoral or cellular hypersensitivity to homologous lung determinants was found. ( info) |
27/874. Pulmonary involvement by histiocytosis X in the pediatric age group. The lungs may be involved by histiocytosis X either as part of a generalized disease or as a separate entity, primary pulmonary histiocytosis X. The former is much more frequent in the pediatric age group, and although it occurs primarily in those who are quite sick with widely disseminated disease, the lung involvement rarely caused symptoms and almost invariably clears. By contrast, primary pulmonary histiocytosis X is mainly seen in young adult males and frequently leads to severe disability. Twelve patients less than 20 years old with lung involvement by histiocytosis X are presented. ( info) |
28/874. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. We present a case of persistent spontaneous pneumomediastinum precipitated by an upper respiratory infection in a patient with interstitial fibrosis associated with rheumatoid arthritis who was receiving chronic corticosteroid treatment. The persistent nature of the mediastinal emphysema over 2 months eventually required treatment with high concentrations of inhaled oxygen that resulted in rapid resolution of the pneumomediastinum without recurrence over 6 months of follow-up. This case, along with others in the medical literature, emphasizes the need for early use of high-concentration inhaled oxygen in the treatment of pneumomediastinum in high-risk patients, such as those with connective tissue disorders. ( info) |
| A middle-aged man developed multiple subcutaneous rheumatoid granulomata, high titer of rheumatoid factor, diffuse interstitial pulmonary fibrosis and digital clubbing in the absence of clinicoradiological evidence of synovial disease. This patient supports the concept of rheumatoid disease without arthritis. ( info) |
30/874. Haemophilia B in a female caused by skewed inactivation of the normal X-chromosome. hemophilia b (factor ix deficiency) is an X-linked recessive disorder with a prevalence of 1:30,000 male births, which rarely affects females. A missense mutation T38R (6488C>G) of the factor ix (FIX) gene was characterized in a young female with moderate-to-severe hemophilia b. She is heterozygous for this mutation, which she inherited from her carrier mother. Analysis of the methyl-sensitive HpaII sites in the first exon of the human androgen-receptor locus indicated a de novo skewed X-chromosomal inactivation. This indicates that the paternal X-chromosome carrying her normal FIX gene is the inactive one, which has led to the phenotypic expression of hemophilia b in this patient. ( info) |