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Post-mortem examinations were conducted in 28 patients with the acquired immune deficiency syndrome (AIDS) and biopsy-proven Pneumocystis carinii pneumonia (PCP) who had been treated with trimethoprim-sulfamethoxazole (Bactrim, intravenous infusion [Roche]) and/or pentamidine isethionate. According to the evolution of the pulmonary process, the cases were classified into three groups. Group I ("fulminant" PCP) was composed of eight patients who died during the first week of the disease. Although treatment had eradicated most of the organisms, one third of the alveolar space volume, on the average, was filled by foamy exudates characteristic of PCP. This accounted for the respiratory insufficiency and death of these patients. Group II ("nonresolving" PCP) was comprised of nine patients who died within eight days and 2 months of diagnosis. PCP was less severe than in group I, but fatal respiratory insufficiency was the result of fibroblastic organization of the intraalveolar exudates (fibrosing alveolitis). In seven of the nine patients (78%), the latter resulted from oxygen toxicity; in the remaining two patients (22%) PCP, per se, was the original stimulus for the fibrosis. Patients in group II also had a high incidence of thromboembolic pulmonary lesions. Group III ("cured" PCP) was composed of 11 patients who responded dramatically well to therapy but died months or years later of other manifestations of AIDS. In group III patients, the roentgenographic picture at diagnosis was consistently less severe than in groups I and II.

In the 1980s, under the impact of the acquired immune deficiency syndrome, Pneumocystis carinii Pneumonia (PCP) has become the prime infectious manifestation of this condition. In addition to the well-recognized "classic" features of this disease, several unusual manifestations are being recognized with increasing frequency. We presently review and illustrate the following "atypical" manifestations of PCP: (1) interstitial lung responses that include diffuse alveolar damage, bronchiolitis obliterans, interstitial fibrosis, and lymphoplasmacytic infiltrates; (2) striking localized processes frequently exhibiting granulomatous features; (3) extensive necrosis and cavitation; and (4) extrapulmonary dissemination of the disease. Close clinico-pathologic correlation and attention to roentgenographic detail are invaluable aids in arriving at the correct diagnosis.

Two homosexual men with disseminated Kaposi's sarcoma presented with paroxysms of a nonproductive cough probably related to the presence of tumor in the airways. At bronchoscopy, multiple, flat, brightly red to violaceous lesions were seen throughout the tracheobronchial tree. In both patients the bronchial biopsies were nondiagnostic, and one patient had excessive bleeding after the procedure. Autopsies confirmed the presence of Kaposi's sarcoma in the trachea and bronchi. The bronchoscopic appearance of Kaposi's sarcoma of the tracheobronchial tree is characteristic, and bronchial biopsy is probably unnecessary when the diagnosis of Kaposi's sarcoma has already been made by biopsy of more accessible lesions. Furthermore, bronchial biopsy may be hazardous because of excessive bleeding.

We report the case of a patient with tricuspid and pulmonic valve atresia, with survival to the age of 21 years. Postmortem examination revealed an anomalous vessel connecting the ascending aorta with the main pulmonary artery. This fistula measured 20 mm by 4 mm, arose independently of the coronary arteries and had the histologic features of an elastic artery. The remarkable longevity was ascribed in part to the maintenance of pulmonary arterial flow by the left-to-right shunt through the anomalous arterial connection.

An unusual wound manifestation associated with toxic shock syndrome (TSS) is reported. The patient fulfilled all four major criteria for TSS and demonstrated involvement of the gastrointestinal, muscular, and renal systems. Rapid resolution of the symptoms was associated with wound drainage, fluid therapy, steroids, and broad spectrum antibiotic coverage.

Bronchocentric granulomatosis is a chronic pulmonary disease treated with short-term therapy with corticosteroids, and the disease has an excellent prognosis. We describe a patient, and review an additional case from the literature, in whom bronchocentric granulomatosis was accompanied by glomerulonephritis. A misdiagnosis of Wegener's granulomatosis was made, and therapy with cyclophosphamide was either considered or given in each of these cases. We emphasize the need for careful histopathologic evaluation of open lung biopsies in patients suspected of having Wegener's granulomatosis in order to rule out the possibility of bronchocentric granulomatosis with concurrent renal disease, and thus avoid the unnecessary use of cytotoxic agents.

Chronic subluxation of the extensor tendons of the metacarpal phalangeal joint has been documented in six patients on active duty in the United States Navy. These patients had painful full flexion and gripping in the knuckle, especially when they were performing their jobs. No extension lag was noted. Three patients had a severed junctura tendinum between the long and index fingers, which was believed to be a contributing factor to extensor tendon subluxation. Local anesthesia was administered to these patients, and the lesions were surgically corrected by reefing of the extensor hood and the sagittal band and repair of the junctura tendinum.

Primary choriocarcinomas of the lung are extremely rare. Like choriocarcinomas elsewhere, they possess rapid growth ability and a high propensity to metastasize. There is minimal information available on the treatment of lung choriocarcinoma. In the case reported herein, neoadjuvant chemotherapy with 5-fluorouracil (5-FU) infusion, etoposide, and cisplatin induced a partial response permitting complete excision of a massive tumor of the right upper lobe involving the chest wall and superior vena cava. The patient relapsed with a metastasis to the brain that was surgically excised. Contralateral lung metastases were soon noted and responded well to systemic chemotherapy; yet the patient died of a new brain metastasis. To our knowledge, this is the first example of a primary choriocarcinoma of the lung treated with intensive multimodality therapy. The latter seems to offer a potential benefit if certain guidelines are followed.

A 14-year-old nonasthmatic boy who presented with multiple skin lesions and a mediastinal tumor was found to have allergic angiitis and granulomatosis (AAG) (Churg-Strauss syndrome), with prominent involvement of the thymus. The occurrence of AAG in the thymus merits recognition because of possible confusion with a variety of other lesions characterized by proliferation of histiocytes and tissue eosinophilia that affect this organ. In addition, this case illustrates that the absence of asthma should not be used as a definitive criterion for exclusion of the diagnosis of AAG.

Reported herein is the case of a 30-year-old white woman who had a congenitally double uterus with two cervixes (uterus didelphys). Each cervix was diffusely involved by squamous cell carcinoma in situ that had progressed to a microinvasive stage of the same depth (0.8 mm) on each side. This coincidence was the more remarkable because it had taken place synchronously. A transmissible coital factor of a viral nature, ie, human papillomavirus infection, was suggested by the presence of koilocytotic atypia in both uterine cervixes.

Periarticular calcifications are the hallmark of a rare entity: tumoral calcinosis. We have followed for 90 months a nine-year-old black girl with involvement of both shoulders. Seven initial local excisions of the mass on the right shoulder were attempted without complete removal and prompt recurrence after each attempt. The entire lesion on the right side, including a cutaneous ulceration, was managed by en masse surgical excision. Preoperative inpatient medical management in the form of low calcium and low phosphorus diet was unsuccessful. Postoperatively, she has remained free of ulceration; however, after two and a half years, the right mass has again increased in size with compression of the brachial plexus. This recurrence occurred despite strict dietary control starting immediately postoperatively. Although there are many advocates of surgical excision of these lesions and, more recently, several cases reported of successful medical management, we find that often a combination approach is necessary to effectively treat tumoral calcinosis and reduce the rate of recurrence.

Mixed connective tissue disease is a syndrome having clinical features of scleroderma, systemic lupus erythematosus, and polymyositis with a unique combination of clinical and laboratory findings. Not all patients with clinical features of more than one connective tissue disease fit the mixed connective disorders category; therefore, the term "overlap syndrome" is applied to patients with features of two or more connective tissue diseases. Gastrointestinal involvement in progressive systemic sclerosis is common, with the esophagus being the usual site, followed by the small bowel, colon, and stomach. Colonic involvement is reported to occur in 10 to 50 percent of cases and is typically manifested radiographically as wide-mouthed diverticular saculations. Symptoms secondary to colonic involvement are rare but occasionally serious, such as impaction of barium or feces. A case is described in which systemic lupus erythematosus coexisted with previously undiagnosed scleroderma involving predominantly the gastrointestinal tract and resulting in megacolon.

In children, primary tumors of the lung constitute a unique subset of quasineoplastic and unequivocally neoplastic lesions whose benign or malignant potential is not always predictable on the basis of morphologic findings. One such neoplasm in the latter category is the primary bronchopulmonary fibrosarcoma (PBPF). This clinicopathologic, ultrastructural, and immunohistochemical study documents our experience with five PBPFs in newborns and children up to 11 years of age at diagnosis. The tumors were either endobronchial or intraparenchymal in location. A uniform population of interlacing bundles and sheets of densely arranged spindle cells with variable mitotic activity was observed microscopically in each case. Ultrastructurally, the cells had the features of fibroblasts and vimentin was the only immunohistochemical marker identified. Despite the disturbing pathologic findings, the four children with more than 1 year of follow-up have survived well 4 to 9 years after surgical resection. Our results are compared with the 21 cases of PBPF reported in the literature and the differential diagnosis is discussed.

Observations on 35 patients with pulmonary angiitis and granulomatosis show the value of separating pulmonary lesions of this type into three categories. Fifteen patients had Wegener's granulomatosis characterized by necrotizing granulomatous inflammation of lung, frequent upper airway and renal involvement, and responsiveness to cyclophosphamide. Nine had lymphomatoid granulomatosis characterized by necrotic atypical lymphoreticular infiltrates and frequent cutaneous and neurologic involvement, usually fatal despite intensive cytotoxic drug therapy. Eleven had benign lymphocytic angiitis and granulomatosis, in the past included in the above categories and characterized by nodular collections of mature lymphocytes and plasma cells, with predominantly pulmonary involvement and consistent responsiveness to chlorambucil. Wegener's granulomatosis and benign lymphocytic angiitis and granulomatosis were frequently associated with serum immunoglobulin elevations, with intact cell-mediated responses. Clinical and immunologic assessment was useful indistinguishing benign lymphocytic angiitis and granulomatosis from lymphomatoid granulomatosis.

The histologic findings, natural history, and response to treatment were investigated in 62 patients with pulmonary angiitis andgranulomatosis seen over a 23 year period. Three histologic types were recognized: lymphocyte depleted angiitis and granulomatosis, 24 cases (39 per cent); benign lymphocytic angiitis and granulomatosis, 14 cases (22 per cent); and malignant lymphoproiferative angiitis and granulomatosis, 24 cases (39 per cent). Lymphocyte depleted angiitis and granulomatosis corresponds to Wegener's granulomatosis of either the "limited" or "classic" variant. The lesions frequently show extensive liquefactive necrosis. Eosinophils may be abundant, but lymphocytes and plasma cells are relatively scarce. Histiocytic proliferation with the occasional presence of sarcoid-like granulomas was observed only in cases with no evidence of glomerular involvement. Cyclophosphamide is highly effective regardless of whether extrapulmonary manifestations are present. Benign lymphocytic angiitis and granulomatosis is characterized by dense benign infiltrates of lymphocytes, plasma cells, and histiocytes. Necrosis may be absent or minimal; the angiitis is usually mild to moderate. Only one of 14 patients (7 per cent) had extrapulmonary involvement (skin). Chlorambucil is highly effective, sometimes resulting in dramatic cures. Malignant lymphoproliferative angiitis and granulomatosis corresponds to lymphomatoid granulomatosis. Its lesions show extensive coagulative necrosis, a highly atypical lymphoreticular infiltrate with frequent mitoses, and angioinvasion. Extrapulmonary involvement is frequent (83 per cent), particularly in the skin (46 per cent) and central nervous system (33 per cent). The mortality is high: 65 per cent of the patients were dead within the first year of disease. Although cytotoxic drugs were of no value, corticosteroids were associated with prolonged remissions in three young patients.

A comparative prospective study of the surgical management of the tendon sheath after repair of flexor tendons in zone II is reported. The study included only patients with lacerations of both flexor tendons and no other associated injuries. A modified Kessler suture was used to repair the profundus tendon and the superficialis tendon was repaired with a horizontal mattress suture. In 48 fingers the flexor tendon sheath was left open and it was closed in the second group of 42 fingers. When it was impossible to close the tendon sheath, a vein patch was taken from the dorsal veins of the hand. Both groups of patients were treated with the same regimen of controlled motion rehabilitation and supervised by the same hand therapist. Results were evaluated by the Strickland formula for total active motion of the proximal and distal interphalangeal joints. There was no statistical difference between the results of open sheath versus closed sheath in these two groups of patients treated postoperatively with the same controlled motion rehabilitation program.

Arthrodesis of the metacarpophalangeal joint of the thumb is a well-accepted procedure. Recommendations for position of fusion have varied from 0 to 30 degrees of flexion. Two hundred of these joints in normal subjects were measured in thirteen positions of function for different tasks. The mean of all thirteen measured positions was considered to be the "optimal functional position". Based on this study, we recommend the metacarpophalangeal joint of males be fused in approximately 25 degrees of flexion and females in 20 degrees of flexion.

Approximately 5 percent of patients with systemic lupus erythematosus by clinical and pathologic criteria have no demonstrable antinuclear antibodies. This figure is likely to be an underestimate, as it does not include the antinuclear antibody-negative patients with limited manifestations in whom the diagnosis of systemic lupus erythematosus is missed. A 23-year-old woman is described who had a history of perplexing bilateral pleural effusions with development of peritoneal effusion after 18 months and positive antinuclear antibody results after 22 months. Tissue pathologic features, initially interpreted as nonspecific, on review revealed striking lymphocytic periarteritis with endothelial swelling and leukocytoclastic vasculitis often seen in systemic lupus. A selective defect in the suppression of T cell effector function, such as direct cell-mediated cytotoxicity, with intact suppressor systems for B cell effector function, such as antibody production, can be postulated in this patient. This would explain the active cellular tissue pathology with the lack of prominent antinuclear antibody production.

Nodular regenerative hyperplasia of the liver is a rare condition that primarily affects adult patients. We report a case of this disease in a 4-month-old infant with coexisting globoid cell leukodystrophy (Krabbe's disease). Based on an analysis of this case and a review of the literature, we concluded that the two disorders were probably unrelated.