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

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. Received March 5, 1996; received in revised form and accepted October 15, 1996     

Spontaneous regression of extramedullary plasmacytoma--a case report

A case of extramedullary plasmacytoma in the soft tissues of the posterior chest wall of an 80-year old man is reported herein. Immunofluorescence study showed that the tumor cells produced IgG lambda. An M-component was also detected in the patient's serum by paper electrophoresis. Two months following the open biopsy done to establish diagnosis, the tumor underwent spontaneous regression and the M-component in the serum also disappeared. This is the first case report of spontaneous regression of an extramedullary plasmacytoma and the probable reasons for this spontaneous regression are discussed herein.     

Role of the dopaminergic system in depression.

A hypothesis implicating dopamine in depression was proposed over 15 years ago (Randrup et al 1975). The identification of multiple new subtypes of dopamine receptors and evolving views regarding the function of the dopamine systems in the brain require a reexamination of this hypothesis. Results from studies in depression, Parkinson's disease, and animal models of depression suggest a deficiency of dopamine in depression. Dopamine precursors, dopamine agonists, and dopamine reuptake inhibitors show therapeutic efficacy in depression. Electroconvulsive therapy (ECT) and standard pharmacological antidepressants enhance dopamine function. Studies using receptor-specific drugs in clinical trials and neuroimaging studies are needed to further clarify the role of dopamine in depression.     

Adult Wilms' tumor: report of a case and review of the literature

A 48 year old male presented with a one and a half year history of a progressively increasing, asymptomatic lump in the left hypochondrium and no history of hematuria. His general physical examination was unremarkable, and an abdominal examination revealed a large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. Chest X-ray was normal. An intravenous urogram revealed a normally functioning right kidney with non-visualization of the left kidney. CT-scan of the abdomen revealed a large, mixed attenuating mass replacing the left kidney. At laparotomy, a large, fleshy, well-encapsulated tumor was found in the left kidney with no surrounding infiltration and a left radical nephrectomy was performed. Microscopic examination revealed a poorly differentiated tumor comprised of small round cells with focal areas of abortive embryonal tubular and glomerular differentiation suggestive of Wilms' tumor. The patient was advised chemotherapy and radiotherapy but he absconded and was lost to follow-up.     

Regional cerebral activity associated with the incidental processing of pseudo-words

In order to study brain activity associated with “incidental” cognitive processing, regional cerebral blood flow (rCBF) was measured in six volunteers while they monitored a sequence of pseudo-words (e.g., FLOPE) for the rare occasions when the letters were displayed in blue rather than white. In the control condition, the same pseudo-word was presented repeatedly. In one experimental condition all 60 pseudo-words were different, while in the other there were 18 repetitions. Although it was not necessary to “read” the pseudo-words to perform the monitoring task, subsequent forced choice recognition memory for these stimuli was significantly greater than chance. Furthermore, there were significant differences in blood flow between the three conditions. When different pseudo-words were presented there was significantly greater activity in brain areas concerned with shape and object identity (extrastriate cortex bilaterally), with visual word form (left inferior temporal gyrus), and with articulatory word form (Broca's area) even though none of this information about the pseudo-words was needed for performance of the monitoring task. In the condition in which some of the words were repeated, there was significantly reduced activity in the right lingual gyrus. This area may therefore be a possible anatomical locus for repetition priming with verbal stimuli. These results indicate the importance of taking into account incidental processing when designing tasks for functional imaging experiments. © 1995 Wiley-Liss, Inc.     

Primary breast lymphoma.

Primary breast lymphoma (PBL) is a rare tumor of the breast. Three cases of PBL are being described. All three cases achieved remission following biopsy, chemotherapy, and/or radiotherapy. The literature is extensively reviewed with emphasis on true incidence of PBL and survival rates.     

A strategy for resection of Wilms' tumor with vena cava or atrial extension.

Resection of a Wilms' tumor that extends into the vena cava or right atrium results in excellent survival when combined with adjuvant therapy. Preoperative identification of the presence of intravascular tumor thrombus and the level of vascular involvement is essential. It facilitates safe surgical resection, with cardiopulmonary bypass immediately available for retrohepatic and atrial tumors. Six patients with intracaval or intracardiac tumor thrombus were treated over a 5-year period with no perioperative deaths. Preoperative chemotherapy was useful in two patients with extensive tumors and pulmonary metastases. Our results using an integrated management plan suggest that an aggressive surgical approach is justified for this extensive variant of Wilms' tumor.     

A 38-kilobase pathogenicity island specific for Mycobacterium avium subsp. paratuberculosis encodes cell surface proteins expressed in the host

We have used representational difference analysis to identify a novel Mycobacterium avium subsp. paratuberculosis-specific ABC transporter operon (mpt), which comprises six open reading frames designated mptA to -F and is immediately preceded by two putative Fur boxes. Functional genomics revealed that the mpt operon is flanked on one end by a fep cluster encoding proteins involved in the uptake of Fe(3+) and on the other end by a sid cluster encoding non-ribosome-dependent heterocyclic siderophore synthases. Together these genes form a 38-kb M. avium subsp. paratuberculosis-specific locus flanked by an insertion sequence similar to IS1110. Expression studies using Western blot analyses showed that MptC is present in the envelope fraction of M. avium subsp. paratuberculosis. The MptD protein was shown to be surface exposed, using a specific phage (fMptD) isolated from a phage-peptide library, by differential screening of Mycobacterium smegmatis transformants. The phage fMptD-derived peptide could be used in a peptide-mediated capture PCR with milk from infected dairy herds, thereby showing surface-exposed expression of the MptD protein in the host. Together, these data suggest that the 38-kb locus constitutes an M. avium subsp. paratuberculosis pathogenicity island.