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Arteriohepatic dysplasia (syndromatic ductular hypoplasia, Alagille syndrome) is a condition of chronic cholestasis dating from infancy accompanied by characteristic facies, pulmonic stenosis, and other somatic abnormalities. The pathologic hallmark of arteriohepatic dysplasia is a paucity or absence of intrahepatic bile ducts, wildely regarded as a congenital deficiency. We present a longitudinal study of six infants and children with arteriohepatic dysplasia, stressing evolution of the characteristic pathology on liver biopsy. All patients were biopsied twice. All five liver biopsies performed during infancy (

Background. Smooth muscle tumors of the vulva are rare and sometimes create diagnostic difficulty because of unusual morphological features. Case. A 29-year-old African–American female presented with a vulval mass during her 5th week of pregnancy. The lesion was surgically removed under the clinical impression of a Bartholin cyst. Histopathological findings of the lesion and immunohistochemical stain results are characteristic of myxoid epithelioid leiomyoma. Conclusion. Up to now, there are only a few cases of myxoid epithelioid leiomyoma of the vulva reported in English literature. We review previously reported cases and discuss the morphologic differential diagnosis. The mass presented in this report was the largest benign epithelioid leiomyoma in the literature.

We report a case of gallbladder paraganglioma that was discovered during nonrelated surgery. Retrospective study disclosed a family history of pheochromocytoma. The occurrence of gallbladder paraganglioma in the presence of family history of endocrine neoplasia supports that gallbladder paraganglioma may indeed occur as a part of the multiple endocrine neoplasm syndrome. Gallbladder paraganglioma is a rare tumor, and so far to our knowledge only 6 cases have been reported in the literature. Three cases were discovered incidentally during cholecystectomy for cholelithiasis, 2 presented with right upper quadrant pain, and 1 manifested with gastrointestinal bleeding. We herein review all reported cases of paraganglioma of gallbladder and biliary system.

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 patient's blood pressure normalized. Primary AT of the adrenal gland coexisting with micronodular ACH associated with hypertension has not been previously reported.

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.

Attachment of bacillus Calmette-Guerin (BCG) organisms to the bladder during intravesical therapy is thought to be mediated exclusively by the glycoprotein fibronectin, which is expressed variably on epithelial surfaces and on basement membranes. We examined the relationship between the degree of fibronectin expressed on surgical specimens obtained from 50 candidates for BCG therapy and the subsequent clinical response. Immunoperoxidase staining for fibronectin was performed on tumor, nonadjacent normal mucosa and basement membrane tissues, and the intensity of the staining was scored on a scale of 0 to 3+ (control 2+). In the absence of recurrence at quarterly surveillance cystoscopy, a course of Tice BCG therapy consisted of 6 weekly and 12 monthly instillations. Recurrence of noninvasive tumor prompted a second BCG course. Followup ranged from 24 to 66 months (median 40 months). Of the 50 patients (11 with carcinoma in situ) disease progression occurred in 9 (none with carcinoma in situ). Compared to the results for tumors or for basement membranes, the degree of fibronectin expression on normal mucosa was well correlated with the clinical response (r = 0.59, p0.001 by Kendall Tau B). Routine assessment of fibronectin expression on the normal mucosa associated with superficial bladder cancer may be useful for predicting the clinical response to BCG therapy.

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.

Objectives This investigation attempted to determine whether the degree of fibronectin expression in the bladders of patients with invasive transitional cell carcinoma correlated with their clinical response to intravesical bacille Calmette-Guerin (BCG) therapy. Methods Following transurethral resection of all invasive disease, 13 patients with Stages T2–T4 bladder cancer were administered intravesical BCG (for 6 weeks followed by monthly instillations). Fibronectin expression in the patients' resected tumors and normal mucosa was determined by immunohistochemical staining techniques. Minimum disease-free follow-up was 60 months. Results Only 1 of 13 patients had neither local nor systemic disease recurrence; 10 of 13 patients developed systemic disease and 7 patients died from metastases. Fibronectin expression was not correlated with the clinical response to BCG. Conclusions Intravesical BCG therapy for the treatment of muscle invasive transitional cell carcinoma of the bladder is ineffective. Fibronectin expression in the bladder of patients with invasive disease is variable and does not correlate significantly with the clinical response to BCG therapy.

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.

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.

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.

beta 2-microglobulin (beta 2M) is a protein of 11,800 daltons which occurs in the plasma of normal individuals at concentrations of approximately 2 microgram/ml. It is presumed to be relatively freely filterable. More than 99% of the filtered beta 2M 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 beta 2M is greater than can be accounted for by filtration alone. These data indicate that some uptake of beta 2M occurs from the peritubular capillary circulation. The loading of animals with beta 2M is associated with a marked tubular proteinuria suggesting that this protein may play a part in inducing tubular injury.

Carotid artery rupture is a rare complication of irradiation therapy of the neck and it may present with mild continuous bleeding. A prompt surgical intervention should be considered to prevent sudden fatal outcome when the patient develops oropharyngeal bleeding after neck irradiation without previous history of trauma or other medical reasons, such as hypertension or hematologic disorders. A case of carotid artery rupture after the usual 6000 rads of Cobalt 60 therapy for squamous cell carcinoma of the vocal cord is. herein reported.

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.

The effect of cholestyramine and bile acid feeding upon fecal, red blood cell, and plasma protoporphyrin levels was evaluated in a patient with erythropoietic protoporphyria and evidence of hepatic damage. After a basal period on a controlled diet, bile acids alone were administered (300-900 mg) daily for 9 days, followed by cholestyramine (12 g) plus bile acids (900 mg) daily for 7 days, and cholestyramine alone (12 g) daily for 6 days. There was no substantial change in either the red blood cell or plasma protoporphyrin concentrations during any treatment period. However, cholestyramine and cholestyramine combined with bile acids caused a threefold increase in fecal protoporphyrin excretion, whereas bile acids alone had no effect in this regard. After 1 yr of cholestyramine therapy, fecal excretion of protoporphyrin remained elevated with concurrent improvement in liver function tests and photosensitivity. This study indicates that cholestyramine but not bile acids can substantially increase protoporphyrin excretion and therefore may be capable of ameliorating the hepatotoxicity that may occur in selected patients with erythropoietic protoporphyria.

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.

Direct immunofluorescent staining with fluorescein isothiocyanate conjugated goat anti-human beta 2-microglobulin (beta 2M) was used to determine the localization of beta 2M in kidney tissue, obtained by biopsies or nephrectomies for various renal diseases. beta 2M was found in the renal tubular epithelial cell cytoplasm and occasionally in the tubular basement membranes in 48 out of 79 pathologic specimens tested, but not in 3 normals. beta 2M was seen in the tubular cells in all cases of primary tubulointerstitial disease and in all cases with secondary tubular damage associated with glomerular disease or systemic disease. This supports the concept that beta 2M is metabolized in proximal tubular epithelial cells, and its deposition indicates impairment of tubular handling.

UNTIL the mid-1930s, tuberculosis had traditionally been considered to be the most frequent cause of primary adrenal insufficiency. With a progressive decrease in the prevalence of this granulomatous process, however, autoimmune disease has superseded tuberculosis as the most frequent cause of primary adrenal insufficiency.1It is amply documented, nevertheless, that certain fungal diseases such as histoplasmosis as well as neoplasms may destroy sufficient adrenal tissue to produce the signs and symptoms of Addison's disease.2-4It is our purpose herein to point out the less well-known fact that other fungal diseases, by means of massive destruction of the adrenal glands, may likewise produce adrenal insufficiency. Specifically, we detail what to the best of our knowledge is the second reported, antemortem biochemically and histopathologically confirmed documentation of cryptococcal-induced adrenal cortical insufficiency. We likewise point out the value of computed tomographic (CT) scanning of the adrenal glands to assist in the

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.