Sudesh Kapur

Atypical hemolytic uremic syndrome in human immunodeficiency virus-1-infected children

Abstract. We describe the clinical and pathological findings of the hemolytic uremic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolytic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both patients. None of these children presented with bloody diarrhea, evidence of circulating antibody response to Escherichia coli O157 lipopolysaccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma infusion and renal replacement therapy. Their clinical course was characterized by non-oliguria and lack of significant hypertension throughout the acute phase of the disease. Despite these favorable clinical parameters, both patients developed end-stage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unknown and the role of HIV infection in its pathogenesis, although possible, is unclear.

Malignant schwannoma with melanocytic and neuroepithelial differentiation in an infant with congenital giant melanocytic nevus: A complex neurocristopathy

We describe an infant girl, born with a pigmented giant nevus, who developed a malignant schwannoma in the retroperitoneum at 16 months of age. At birth the nevus covered over 50% of her body and histologically was a compound nevus with extension into the deep dermis surrounding dermal appendages. The malignant schwannoma was biphasic with areas composed of spindle and round cells. Ultrastructurally, the majority of the tumor cells exhibited a Schwann cell phenotype, but neuroepithelial and melanocytic cells were identified as well. We believe that this constellation of findings represents a form of neurocristopathy. Neurocristopathy, as defined by Bolande (Hum Pathol 5:409-429, 1974), is a disease that results from aberrations in the migration, growth, or cytodifferentiation of neural crest tissues. These diseases may be simple (a singular pathologic process, usually localized) or complex (multiple neuroectodermal lesions). We report this case because the occurrence of retroperitoneal malignant schwannoma arising in a 16-month-old infant born with a pigmented giant nevus is unique, and may represent a previously undescribed form of a complex neurocristopathy.

Reconstruction of the renal artery after unsuccessful percutaneous transluminal angioplasty in children.

The use of percutaneous transluminal angioplasty as the primary treatment of renovascular stenosis in adults has recently been described. Previously, only three children have been reported to have undergone transluminal angioplasty for stenosis of the renal artery and hypertension. At our hospital, transluminal angioplasty was attempted in four children with renal artery stenosis; one attempt was successful and three were unsuccessful. The three patients who required surgical repair of the renal artery after unsuccessful transluminal angioplasty have been described in detail. The histopathology of the stenotic vessels is also discussed. Based on the analysis of the three children, certain criteria have been derived to select pediatric patients with renovascular hypertension either for attempted transluminal angioplasty or for primary surgical revascularization.

Diffuse pulmonary arteriovenous malformation in a child with polysplenia syndrome.

Pulmonary arteriovenous malformation (PAVM) is usually seen as a well-circumscribed cystic mass. In this communication we describe a diffuse arteriovenous malformation in a 5-month-old infant. The lesion was seen only at the microscopic level and was associated with abdominal heterotaxy, atrial situs solitus, polysplenia, interrupted inferior vena cava, atrial septal defect, and atrioventricular canal. This uncommon association may be within the spectrum of polysplenia syndrome.

Hepatocellular Adenoma in a Young Woman with β-Thalassemia and Secondary Iron Overload

The occurrence of a hepatocellular adenoma is described in a young woman with beta-thalassemia and secondary iron overload. This patient had no history of oral contraceptive use, and in fact was hypogonadal as a result of iron deposition in pituitary and gonadal tissue. Although hepatocellular carcinoma frequently occurs in diseases associated with iron overload, this is the first report of a benign liver tumor associated with secondary iron overload.

Premature Ovarian Failure in a 15-Year-Old

6 months,’ can be due to a number of different nonovarian or ovarian causes. Among the ovarian causes are failure secondary to gonadal dysgenesis; premature ovarian failure (POF) or premature menopause; ovarian tumors that secrete large amounts of estrogens or androgens; ovarian resistance to gonadotropins; and surgical removal of the ovaries or damage by irradiation or chemotherapi. POF in adolescent girls has been rarely reported. We wish to report a 15-year-old female with POF.

Rapid Tissue Diagnosis of Herpes Simplex Infections in the Newborn

The presence of intranuclear inclusions in tissues is considered to be consistent with viral infections. Further identification is done by viral cultures. In this study, the direct immunofluorescence technique was utilized to identify herpes simplex virus in the tissues. Positive fluorescence was seen in 8 of 12 specimens. In all these specimens, diagnosis was further confirmed by electron microscopy, viral cultures, or both. Thus, the technique is a specific, sensitive method of diagnosis for herpes simplex infections. Rapidity and simplicity of the technique warrant regular use.