Moonja Chung-Park

Cardiopulmonary pathology in patients with sleep apnea/obesity hypoventilation syndrome

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.

Acquired Cystic Disease of the Kidneys and Renal Cell Carcinoma in Chronic Renal Insufficiency without Dialysis Treatment

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.

Adenomatoid tumor of the adrenal gland with micronodular adrenal cortical hyperplasia.

We report a case of an adenomatoid tumor (AT) of an adrenal gland with micronodular adrenal cortical hyperplasia (ACH). A 51-year-old man was found to have newly developed hypertension with clinical evidence of primary aldosteronism. A computerized tomogram of the abdomen revealed a solitary mass in the right adrenal gland. He underwent a right adrenalectomy for a presumptive clinical diagnosis of a solitary aldosterone-producing adrenal cortical adenoma. On histopathologic examination, the adrenal gland demonstrated an AT, diagnosed by the characteristic histological features, immunohistochemical stain results, and electron microscopic findings. The surrounding adrenal cortex showed multiple small hyperplastic cortical nodules. After the adrenalectomy, the patients blood pressure normalized. Primary AT of the adrenal gland coexisting with micronodular ACH associated with hypertension has not been previously reported.

Efficacy of early immunosuppressive therapy in a child with carbamazepine-associated vanishing bile duct and Stevens-Johnson syndromes.

Acute drug-related vanishing bile duct syndrome (VBDS) is a rare, rapidly progressive destruction of the intrahepatic bile ducts with unknown pathogenesis. Immune-mediated mechanisms have been proposed. Prognosis is variable, and treatment unclear, as biliary cirrhosis has resulted despite aggressive immunosuppression (1, 2). Stevens-Johnson syndrome (SJS) is an acute mucocutaneous disorder secondary to drugor infection-induced immune complex deposition, treated supportively and with steroids (3). We report on a 4-year-old boy who developed carbamazepine-induced SJS concurrent with pancreatitis, hepatitis, and cholestasis. Liver biopsy on day 4 of illness demonstrated early vanishing bile duct syndrome. With aggressive immunosuppression using corticosteroids followed by tacrolimus, the patient’s liver insult did not progress to biliary cirrhosis.

Rounded atelectasis and fibrotic pleural disease: the pathologic continuum

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.

IgA Nephropathy Associated With Sarcoidosis

PATIENTS with sarcoidosis may develop renal insufficiency due to hypercalcemia, granulomatous or nongranulomatous interstitial disease or, rarely, necrotizing arteritis. I An increasing number of glomerular diseases, most frequently membranous glomerulopathy, have been also recognized in patients with sarcoidosis. 2 Taylor et aP described a case of IgA nephropathy followed by the development of sarcoidosis 2 years later, and Murray et al4 reported a case of simultaneously diagnosed sarcoidosis and IgA nephropathy. This report describes a case of IgA nephropathy that developed after a long history of systemic sarcoidosis, and discusses the possible pathogenic relationship between sarcoidosis and IgA nephropathy.

Shrinking pleuritis with lobar atelectasis, a morphologic variant of "round atelectasis"

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.

Renal Metabolism of β2-Microglobulin

Abstract. β2‐microglobulin (β2M) is a protein of 11,800 daltons which occurs in the plasma of normal individuals at concentrations of approximately 2 μg/ml. It is presumed to be relatively freely filterable. More than 99% of the filtered β2M is taken up by an active reabsorptive mechanism and catabolized by the renal tubule. The data presented here demonstrate that renal extraction is only slightly diminished by complete ureteral obstruction. The renal extraction of β2M is greater than can be accounted for by filtration alone. These data indicate that some uptake of β2M occurs from the peritubular capillary circulation. The loading of animals with β2M is associated with a marked tubular proteinuria suggesting that this protein may play a part in inducing tubular injury.

Immunofluorescence Studies of Lung Tissue in Cystic Fibrosis

Previous studies have suggested that immune mechanisms contribute to lung injury in cystic fibrosis (CF); however, there have been no comprehensive studies of immunofluorescent staining patterns in CF lung tissue. We performed immunofluorescence (IF) studies for immunoglobulins, C3, and fibrinogen on autopsy frozen lung tissue from 21 CF patients. Results were compared with lung findings in patients without CF. In CF-derived lung tissue fibrinogen was ubiquitous along the alveolar wall, alveolar space, and interstitium. Free immunoglobulin G (IgG) and IgA coated the alveolar surface segmentally in 14 and 6 cases, respectively. Unequivocal interstitial deposits were infrequent and IgM was present in blood vessels in one patient only. Intra-alveolar and interstitial inflammatory cells demonstrated cytoplasmic IgG, IgA, and IgM, respectively, in 18, 14, and 6 patients. C3 was seen only segmentally along the alveolar wall in two patients and in blood vessels in one. Antinuclear antibody (ANA) staining of interstitial cells for C3 and immunoglobulins was seen in five patients, four of whom had interstitial pneumonitis. Insignificant amounts of alveolar or interstitial fibrinogen and immunoglobulins in inflammatory cells were seen in controls in the absence of lung inflammation. The IF patterns were similar in the inflammatory lesions of CF and control specimens. The IF patterns observed in CF lung tissue are consistent with nonspecific vascular leakage and chronic inflammation with little evidence of immune complex deposition in the interstitium or blood vessels. This study confirms previous reports of ANA activity in CF patients, although the significance of this finding is unknown.

Immune and Inflammatory Glomerular Diseases

This chapter is mainly devoted to immune-mediated kidney disorders, and is primarily focused on glomerular diseases. Lupus nephritis and ANCA-associated vasculitis are addressed in separate chapters. The reader is reminded that the renal involvement may be the initial manifestation of systemic disease, and the results of renal pathology may provide the first clues to diagnosis. In this chapter, glomerular diseases have been divided into diseases that present as either acute glomerulonephritis or the nephrotic syndrome, although there is overlap in these clinical presentations. For example, some patients present with features of both, while diseases that most often present as nephrotic syndrome, sometimes present as acute glomerulonephritis, and vice versa. Nevertheless, we find this distinction to be useful as starting point in the differential diagnosis, although as indicated below, the clinical presentation is dependent on the primary pathophysiologic events that predominate in individual patients.