Vijay V. Joshi

ARTERIOPATHY IN CHILDREN WITH ACQUIRED IMMUNE DEFICIENCY SYNDROME

Pathologic features of the arteries of different organs (heart, lungs, kidneys, spleen, intestine, brain) seen at autopsy in 6 children with acquired immune deficiency syndrome (AIDS) are described. Small and medium-sized arteries, which were the most commonly involved, showed intimal fibrosis with fragmentation of elastic tissue, fibrosis and calcification of media with variable luminal narrowing, and a vasculitis or perivasculitis that was seen only in the brain in association with AIDS encephalopathy. In 1 case aneurysms of the right coronary artery with thrombosis and myocardial infarction were seen. Vascular inflammation, seen only in the brain, may be related to the agent associated with AIDS encephalopathy. The fibrocalcific arterial lesions most closely resemble idiopathic arterial calcification of infancy, but because of differences in age incidence, clinicopathologic and immunologic features, and the size and distribution of the involved arteries, the arterial lesions of pediatric AIDS appear to constitute a distinctive arteriopathy. Infection, secondary to immunodeficiency and resulting in increased exposure to endogenous and exogenous elastases, may be the pathogenesis. Luminal narrowing caused by arterial lesions may play a contributory role in the pathogenesis of the atrophy, cell depletion, scarring, and necrosis or infarction found in organs of children with AIDS. Pediatricians should be alerted to the possibility of arterial involvement in pediatrics AIDS.

Disseminated leiomyosarcoma in a child with acquired immune deficiency syndrome

A child with disseminated leiomyosarcoma and acquired immune deficiency syndrome (AIDS) is reported. She was originally believed to have peptic ulcer disease by radiographic and endoscopic evaluation but was found subsequently to have hypergastrinemia, hypochlorhydria, and a smooth muscle tumor. Leiomyosarcoma in children and its evolution in AIDS are discussed.

Handbook of placental pathology

Introduction Development of the Placenta Structure of the Placenta Examination of the Placenta in the Clinical Setting Clinical Data to be Sent to the Pathologist Indications for Pathological Examination Pathologic Examination of the Placenta Gross Abnormalities of the Placenta Histologic Lesions of the Placenta Lesions of the Placenta as a Whole of of the Placental Disk Lesions of the Umbilical Cord Lesions of the Membranes Abnormalities of the Decidua Lesions of the Placenta in Maternal Disorders Lesions of the Placenta in Fetal Disorders Iatrogenic Lesions of the Placenta Traumatic Lesions of the Placenta Placenta in Pregnancies After In Vitro Fertilization (IVF) and Embryo Transfer (ET) Specimens Related to Placental Laser Surgery Comment Index

Pathology of suspected acquired immune deficiency syndrome in children: a study of eight cases.

Biopsy and/or autopsy material from lymphoreticular and other organs was studied in 8 children with suspected acquired immune deficiency syndrome (AIDS). One or both parents of each of these children had one or more of the recognized risk factors for AIDS, such as intravenous drug abuse, prostitution, Haitian origin. The following histologic patterns were noted in the lymph nodes: (1) follicular hyperplasia with normocellular paracortex, (2) follicular hyperplasia with depletion of paracortex, and (3) atrophy of follicles with depletion of paracortex. Lymphoid interstitial pneumonitis (LIP), a previously unreported lesion in AIDS, was present in 4 cases. It is suggested that the pulmonary lymphoid lesion may be part of a more generalized lymphoid hyperplasia involving B cells. The gross and microscopic features of the thymus, available in 2 of the 8 cases, indicated that the immunologic defect in these children was not of congenital type. Pathologic findings can be helpful in the diagnosis of the syndrome when correlated with clinical and immunologic features of suspected cases and of the pulmonary lesion. The latter is of importance in deciding the type of therapy to be given for the pulmonary disease process.

Polyclonal Polymorphic B-Cell Lymphoproliferative Disorder With Prominent Pulmonary Involvement in Children With Acquired Immune Deficiency Syndrome

Four cases of pediatric Acquired Immune Deficiency Syndrome (AIDS) with lymphoproliferative disorder are described and other lymphoid lesions in previously reported cases of pediatric AIDS are reviewed. The lymphoproliferative disorder was characterized by polyclonal, polymorphic B‐cell content without evidence of cellular atypia, necrosis or prominent mitotic activity but with predominantly extranodal systemic and prominent pulmonary involvement. Since the lesion has overlapping features it is considered to be intermediate between benign and malignant lymphoproliferations and designated as polyclonal, polymorphic B‐cell lymphoproliferative disorder (PBLD) of pediatric AIDS. The PBLD is part of a spectrum of lymphoid lesions in pediatric AIDS consisting of follicular lymphoid hyperplasia of nodal and extranodal sites, pulmonary lymphoid hyperplasia/lymphoid interstitial pneumonitis complex (PLH/LIP complex) in cases previously reported by the authors, and also malignant lymphoma reported by others. It is possible that Epstein‐Barr virus (EBV) by itself or in synergism with human T‐lymphotropic virus‐type III (HTLV‐III) is related to pathogenesis of PBLD in children with AIDS.

Acquired immunodeficiency syndrome-associated renal disease in children

Five children with acquired immunodeficiency syndrome (AIDS) and clinically significant renal disease had detailed pathologic examination of renal tissue (biopsy specimens, autopsy specimens, or both). All patients had proteinuria, hypoalbuminemia, and edema; one patient had persistent azotemia. In two cases, renal disease was the first manifestation of human immunodeficiency virus (HIV) infection. All patients had progressive renal disease, and four of the five died. Pathologic studies revealed focal glomerulosclerosis and mesangial proliferative glomerulonephritis with deposits of immunoglobulins and complement demonstrated by immunofluorescence and electron microscopy. Characteristic tubuloreticular structures were also demonstrated in the glomerular endothelial or epithelial cells in two cases. Renal disease is part of the multisystem involvement in children with AIDS. The pathogenesis of renal disease is not known, but circulating immune complexes are known to occur in children with HIV infection and may be involved.

Cystic partially differentiated nephroblastoma. A clinicopathologic entity in the spectrum of infantile renal neoplasia

Three cases of cystic partially differentiated nephroblastoma (CPDN) are presented and ten cases from literature are reviewed. CPDN has been designated by various terms; it is a cystic encapsulated tumor occurring before 2 years of age. Cysts are lined by epithelium; septa of the cysts show a mixture of partially differentiated and undifferentiated metanephrogenic blastema. This histologic feature distinguishes CPDN from multilocular cyst of kidney. In seven cases simple nephrectomy, and in remaining cases nephrectomy with radiation and/or chemotherapy, had been the treatment. The disease‐free interval ranged from 5 to 72 months, without reports of recurrence or metastasis. CPDN appears to take a benign course and simple nephrectomy seems to be the treatment of choice. However, in view of the possibility of recurrence as shown in rare instances by congenital mesoblastic nephroma, another less aggressive lesion in the spectrum of infantile renal neoplasia, regular follow up is recommended. Cancer 40:789–795, 1977.

Dilated cardiomyopathy in children with acquired immunodeficiency syndrome:: A pathologic study of five cases

Clinicopathologic features with special reference to the heart are presented in five fatal cases of acquired immunodeficiency syndrome (AIDS) in children. Three children showed clinical evidence of cardiovascular compromise or congestive heart failure. Autopsy was performed in all cases. The enlarged heart showed biventricular dilatation with grossly unremarkable valves and coronary arteries and absence of mural thrombi. Microscopic examination of the heart revealed primarily myopathic abnormalities with hypertrophy of the myocardium and only rare foci of sparse inflammatory infiltrate. The pathogenesis of dilated cardiomyopathy in these children with AIDS is not known. Infection, immunologic factors, anemia, deficiency of nutritional factor(s), and longer survival may be related to the pathogenesis. Pediatricians should be alert to the possibility of cardiac involvement in pediatric AIDS.

Severe gastrointestinal involvement in children with the acquired immunodeficiency syndrome.

Five children with the acquired immunodeficiency syndrome (AIDS) and unusual gastrointestinal disease are described. Two children presented with malnutrition, abdominal distention, and diarrhea. One was found to have moderately severe villus atrophy on jejunal biopsy and was initially thought to have celiac disease. Jejunal biopsy from the second child revealed infiltration of the mucosa with acid-fast bacilli-laden macrophages. A third child suffered recurrent abdominal pain, progressive weight loss, diarrhea, and severe gastrointestinal hemorrhage secondary to infection with cytomegalovirus. Pseudomembranous necrotizing jejunitis associated with overgrowth of Klebsiella pneumoniae in the duodenal fluid occurred in one patient. The fifth child presented in the newborn period with Serratia marcescens cholecystitis. Gastrointestinal disease in children with AIDS may be due to idiopathic villus atrophy and bacterial or opportunistic infection.

Zollinger-Ellison syndrome associated with a renal gastrinoma in a child

Renal gastrinoma has not been previously reported. A 12-year-old boy with Zollinger-Ellison syndrome was found to have a renal tumor. No other tumor was detectable by imaging techniques, and selective venous sampling for gastrin showed a significant renal vein to vena cava gradient. Nephrectomy was performed, and examination of the tumor showed typical histologic features of an endocrine tumor. G cells were apparent by electron microscopy, and immunoperoxidase staining for gastrin, neuron-specific enolase, and chromogranin were positive. The gastrin content was unusually low for gastrinomas: 128 pg/g. Following nephrectomy, fasting gastrin and secretin stimulation testing were normal. Basal acidity was reduced by 60% but remained elevated at 39 mmol H +/h (hydrogen ion per hour). We speculate that renal gastrinoma may be characterized by uniquely poor gastrin storage and that curative resection of all gastrinoma tissue may not necessarily be associated with immediate complete suppression of hyperacidity.