Glomerular disease and lung transplantation.

Three patients with lung or heart/lung transplants developed nephrotic-range proteinuria 2 to 5 years posttransplantation. Kidney biopsy showed focal segmental glomerulosclerosis in two patients and probable focal sclerosis in the third. A retrospective review of postmortem kidney specimens from 18 lung transplant recipients who died did not indicate additional cases of glomerular disease. The three patients with glomerular disease after lung transplantation had very few clinical similarities other than nephrotic-range proteinuria and lung transplantation. Their underlying lung diseases were different, and their posttransplantation courses were very different in terms of pulmonary function, cyclosporine nephrotoxicity, and other complications. We did not find in the literature previous reports of de novo focal segmental glomerulosclerosis or other glomerular lesions after lung transplantation. We suspect that additional cases will be identified in the future.

Unusual forms of pulmonary embolism.

The first word of the title, unusual, captures the theme of this article. The entities discussed here: fat embolism, tumor embolism, venous air embolism, and amniotic fluid embolism, as well as other unusual embolic events are uncommon disorders that occur in specific circumscribed clinical settings. We have attempted to provide a cognitive jolt to remind the reader to consider these unusual events in appropriate differential diagnoses. No laboratory test, physical finding, or patient complaint will yield a timely diagnosis. Yet, these entities can be acutely life threatening; swift recognition is imperative. Timely and effective therapy rests on the clinical certainty that a test is not likely to establish. It is, perhaps, the greatest demand placed upon the agile clinical mind--to think of it!

Tracheobronchial obstruction due to silicosis.

Broncholithiasis can result in airway obstruction through the erosion of calcified lymph nodes into the bronchial lumen or by extrinsic compression of the tracheobronchial tree. We report an unusual case of broncholithiasis in a patient with silicosis who developed airway obstruction from endobronchial polypoid masses of granulation tissue adjacent to calcified mediastinal lymph nodes. The production of granulation tissue may have been the result of broncholiths in the early stages of erosion into the tracheobronchial tree. Efforts to ablate the endobronchial polyps using YAG laser phototherapy were only temporarily successful and surgical removal of the calcified mediastinal lymph nodes was required to halt further polyp growth. Surgical specimens grew Mycobacterium avium-intracellulare (MAI), a common pathogen in patients with silicosis. MAI may have contributed to the local inflammatory milieu provoking the exuberant tissue response.

Combined laser phototherapy and growth factor treatment of bronchial obstruction after lung transplantation.

Lung transplantation has resulted in dramatic functional improvement in patients with end-stage pulmonary diseases. Among the complications of lung transplantation are dehiscence and stenosis at the site of the bronchial or tracheal anastomosis. In this case report, we describe a single lung transplant recipient in whom partial bronchial dehiscence, followed by exuberant growth of granulation tissue, resulted in obstruction of the bronchial lumen. After mechanical dilation failed to produce lasting relief of bronchial obstruction, a novel approach to this problem was successfully employed: YAG laser phototherapy was used to remove obstructing granulation tissue, followed by application of a preparation derived from autologous blood platelets to promote epithelialization of the bronchial anastomosis. The bronchus remains patent and fully epithelialized six months after therapy.

Albinism and Hermansky-Pudlak syndrome in Puerto Rico.

Five types of oculocutaneous albinism and two types of ocular albinism were found among 349 Puerto Rican albinos. The most prevalent type of albinism was the Hermansky-Pudlak syndrome (HPS). HPS was observed in five of every six albinos in Puerto Rico. The prevalence of HPS was highest in the northwestern quarter of the island, affecting approximately one in 1,800 persons, and approximately one in 22 are carriers of the gene. HPS is an autosomal recessively inherited triad of a tyrosinase-positive type of albinism, a hemorrhagic diathesis due to storage pool deficient platelets and accumulation of ceroid in tissues. The pigmentary phenotype of HPS albinos resembled that of any other type of oculocutaneous or ocular albinism. The most reliable method of diagnosing HPS is by a deficiency of platelet dense bodies observed by electron microscopy. The accumulation of ceroid in the tissues is associated with fibrotic restrictive lung disease and granulomatous enteropathic disease. The enteropathic disorder resembles Crohn's disease and with few exceptions, had its onset after 13 years of age. The major causes of death were fibrotic restrictive pulmonary disease, hemorrhagic episodes and sequelae of granulomatous enteropathic disease. Menometrorrhagia was common in women with HPS. No immune deficiency was found in HPS patients. The majority of patients with HPS had visual acuities of 20/200 or worse and consequently were legally blind. Albinos of all types, including HPS, lacked binocular vision due to nearly complete crossing of the optic tracts.

Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension.

Intravascular lymphomatosis is a rare lymphoma characterized by proliferation of malignant cells within the lumen of small blood vessels. We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea. This lymphoma occasionally responds to combination chemotherapy, suggesting that pulmonary hypertension secondary to intravascular lymphomatosis may be reversible. Intravascular lymphomatosis should be considered in the differential diagnosis of pulmonary hypertension.

Respiratory manifestations of connective tissue disease.

Pleuropulmonary disease is a common feature of the following connective tissue diseases: systemic lupus erythematosis (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), mixed connective tissue disease (MCTD), polymyositis/dermatomyositis (PM/DM), and Sjogren's syndrome (SS). Features common to most of these disorders include pleurisy with effusion and interstitial lung disease. Pleural effusions caused by SLE and RA have certain characteristics on pleural fluid analysis that aid in diagnosis, but infection and other causes of effusion must be excluded. Interstitial lung involvement is usually indolent in onset, but a more rapidly progressive course over weeks to a few months may mimic infection. Several drugs used to treat connective tissue diseases may cause interstitial disease, increase susceptibility to infection, or both. This complicates differential diagnosis. Acute lupus pneumonitis and SLE-related alveolar hemorrhage are usually fulminant processes, often associated with fever. Diagnosis of these conditions always requires exclusion of infection. Rheumatoid nodules may mimic infectious and neoplastic lung diseases. Needle biopsy helps reduce the likelihood of infection or malignancy, but open lung biopsy is needed if a firm diagnosis of rheumatoid nodules is required.