Glomerulonephritis.
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Biomedical subjects
Publications and source records attributed to M Chung-Park.
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We reviewed clinical data, autopsy reports, and microscopic slides on 10 patients with sleep apnea/obesity hypoventilation syndrome (SA/OHS) to define the cardiopulmonary pathological features and establish clinicopathologic correlations. Ten obese (>136 kg) patients without SA/OHS were studied as controls. Patients with SA/OHS exhibited biventricular cardiac failure and pulmonary hypertension with a higher prevalence of moderate/severe pulmonary hemosiderosis (8 v 0 patients), alveolar hemorrhage (7 v 4 patients), capillary proliferation (4 v 0 patients), iron encrustation of elastica (1 v 0 patients) and medial hypertrophy of muscular pulmonary arteries (11.9 +/- 2.4 v 9.7 +/- 1.6%) (P < .05). In two patients capillary proliferation resembled capillary hemangiomatosis. Mean right ventricular thickness was higher in the SA/OHS group (0.71 +/- 0.17 v 0.42 +/- 0.1 cm) (P < .01). Four patients with SA/OHS and three controls had moderate/severe myocardial fibrosis. Biventricular cardiac failure caused death in seven patients with SA/OHS. Hypoxia is probably the most important cause of pulmonary hypertension, arterial muscularization, and right ventricular hypertrophy in SA/ OHS. Left ventricular failure in some SA/OHS patients may be the result of hypertensive cardiac disease. In others, the etiology of left ventricular failure was not determined morphologically, suggesting functional abnormalities related to obesity and/or apneic episodes.
Acute renal crisis as an early manifestation of scleroderma is underemphasized, and its recurrence after initial successful therapy is rare. We describe a 32-year-old woman who presented with scleroderma renal crisis. A second episode of apparent renal crisis, however, was complicated by thrombotic thrombocytopenic purpura, which led to pancreatitis, a large cerebral infarction, and fatal outcome despite intensive therapy. This case illustrates the complexity and severity of diffuse systemic sclerosis presenting with multiple, major organ complications.
We report an HLA-B27 positive patient with severe psoriatic spondyloarthropathy who developed secondary reactive amyloidosis. The clinical course was characterized by progressive renal failure with nephrotic syndrome and cardiac dysfunction. He died suddenly and autopsy revealed diffuse amyloid deposits in the heart, kidneys, and many other organs. Amyloid deposits were present in the cardiac conduction system including the sinoatrial and atrioventricular nodes, myocardial interstitium, and intramyocardial small blood vessels. The severe AA amyloid deposit in the heart with involvement of conduction system is unusual, and has not been described in patients with psoriasis or psoriatic arthritis.
A case of benign inflammatory pseudotumor of the spleen is described in a 71-year-old man who presented with left upper quadrant pain and anemia. Definitive diagnosis was made by a combination of microscopic examination with immunohistochemical stains and flow cytometry studies. To our knowledge this is only the 24th reported case of inflammatory pseudotumor involving the spleen. Recognition of this rare entity is important because the clinical manifestations and radiographic features may be indistinguishable from a malignant lymphoproliferative disorder. The clinical presentation, radiographic findings, gross and microscopic features, results of surgery, and long-term follow up are reviewed for our case and the previously reported cases in the literature.
This study examines the indications for performing the Kleihauer-Betke (KB) test and makes recommendations for its use. Results of 523 KB tests performed during 1993 at our hospital (Cleveland, Ohio) are reviewed in conjunction with surgical pathology reports of placental findings, obstetric records, and toxicology results. We conclude that the KB test should be performed following a positive screening test on all Rh negative mothers of Rh positive infants. Additional indications include cases of maternal trauma, unexplained increased maternal alpha-fetoprotein levels, fetal distress with abnormal heart tracings, intrauterine fetal death, and cases of unexplained neonatal anemia. We note that the KB test should not be performed to detect suspected placental abruption.
Although rounded atelectasis was recognized in 1928, its relationship to fibrotic pleural disease almost has gone unnoticed. We present four cases of lobar rounded atelectasis that help to clarify this relationship. Rounded atelectasis is part of the spectrum of fibrous pleural disease. Any portion of lung may become entrapped by the pleural process. When focal areas are involved rounded atelectasis results. When a larger portion of the thorax is involved with this process, lobar rounded atelectasis may occur. Very extensive disease is recognized as fibrothorax.
The superior vena cava syndrome associated with an intraluminal vascular proliferative lesion is extremely rare. A case of intravascular papillary endothelial hyperplasia of the superior vena cava causing obstructive symptoms is reported that was successfully managed by surgical excision.
We describe a patient with hypocomplementemic urticarial vasculitis syndrome complicated by recurrent pericarditis and cardiac tamponade. The episodes of pericarditis were associated with urticarial vasculitis, hypocomplementemia, and circulating IgG antibodies to the collagen-like region of Clq. Histopathologic examination of the skin and pericardium demonstrated vasculitis associated with immunoglobulin and complement deposition suggesting an immune complex mediated etiology. Recurrent pericarditis should be included in the clinical spectrum of hypocomplementemic urticarial vasculitis syndrome.
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A 67-yr-old man, known to have chronic obstructive lung disease, developed bilateral localized pulmonary densities on chest radiographs after cardiopulmonary resuscitation. An autopsy disclosed bilateral intrapulmonary hematomas without communication with bronchi, pulmonary arteries, or pleural cavities. We suggest blunt pulmonary injury is the most probable cause of the hematomas and discuss its pathogenic mechanism. Intrapulmonary hematomas should be considered in the differential diagnosis of pulmonary densities developing after a vigorous resuscitation.
A case of unusual cholecystitis that developed on completion of interleukin-2 and lymphokine-activated killer cell therapy is described. A 62-year-old man was treated with interleukin-2 and lymphokine-activated killer cells for disseminated renal cell carcinoma. During the course of the immunotherapy, his serum alkaline phosphatase level increased, as did the peripheral eosinophil count (0.31). Subsequently, clinical and radiologic evidence of acute cholecystitis was noted. The removed gallbladder showed acalculus cholecystitis with extensive diffuse infiltrates of numerous eosinophils and T lymphocytes, but sparse polymorphonuclear leukocytes. The authors name this unusual cholecystitis acalculus lymphoeosinophilic cholecystitis and believe it to be associated with interleukin-2 and lymphokine-activated killer cell therapy. The pathogenic relationship is discussed.
Round atelectasis (shrinking pleuritis) is typically a localized process characterized by focal pleural scarring and subjacent peripheral atelectasis. We report three patients, studied at autopsy, with an unusual variant of round atelectasis, termed shrinking pleuritis with lobar atelectasis, which is characterized by lobar atelectasis, visceral pleural fibrosis involving multiple lobes, interlobar fibrous cords, pleural effusion, and nonspecific, persistent infiltrates on chest radiogram. The possible causes of shrinking pleuritis with lobar atelectasis in our patients were multiple and included environmental dust exposure, infection, uremia, and recurrent pleural effusions. Our findings support both the folding (pleural effusion) and fibrosing (pleural injury) theories of pathogenesis of round atelectasis and emphasize the spectrum of morphologic variability in this condition.
We report 3 cases of acquired cystic disease of the kidneys with associated renal carcinoma in 2 of the cases. In all 3 cases, the patients had chronic renal insufficiency due to hypertension but had never required dialysis. Review of 176 reported cases of acquired cystic disease of the kidneys and renal tumors disclosed that 18 patients (including 1 previously reported by us) had never received dialysis treatment. These cases support the hypothesis that acquired cystic disease of the kidney is not restricted to patients treated with maintenance dialysis. Among the 18 patients, hypertension was the most common underlying cause of renal failure. Patients with chronic renal failure due to or associated with severe hypertension should be monitored carefully for the development of both renal cysts and tumors even though they have not started on chronic dialysis.
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This paper reports two cases of acquired renal cysts and multiple renal cell tumors in end-stage kidneys with a discussion of the pathogenesis of these lesions. The first patient had been on maintenance dialysis for approximately three years when he was found to have multiple renal cysts and renal cell tumors. He subsequently developed multifocal urothelial carcinomas of the lower urinary tract. The second patient had progressive renal failure due to hypertensive vascular disease when he underwent left nephrectomy because of multiple renal cysts and renal cell carcinoma. He later developed terminal renal failure and was maintained on chronic dialysis. He expired three years later. At autopsy, the right kidney also demonstrated multiple cysts and renal cell tumors.
The renal extraction of beta 2-microglobulin (beta 2M) was investigated under steady-state conditions achieved by constant infusion of human beta 2M. Fifteen animal experiments were conducted. Beta 2 microglobulin was infused at rates ranging from 51 o 269 micrograms/min. The renal arterial and venous blood levels remained constant throughout the study period. The data showed that renal extraction of beta 2M exceeded the rate of filtration at all levels of beta 2M delivered to the kidney. The tubular uptake of filtered beta 2M increased linearly as did the extraglomerular extraction throughout the range investigated. There was no evidence of beta 2M (FE beta 2M) increased linearly with the fractional excretion of filtered water (FE H2O). The results are interpreted to indicate that beta 2M is extracted from renal blood by glomerular filtration and, in addition, by a mechanism independent of glomerular filtration rate (GFR). Under the conditions existing in these experimental animals, the linear relationship between FE beta 2M and FE H2O is evidence to suggest that factors affecting proximal tubular water reabsorption also affect beta 2M reabsorption.