Cognitive deterioration in adults with Down syndrome: effects on the individual, caregivers, and service use

Individuals with Down syndrome (N = 49) who had participated in serial neuropsychological assessments were assigned to one of three groups comparable in level of premorbid mental retardation: those showing cognitive deterioration, those comparable in age but not showing cognitive deterioration, and those not showing cognitive deterioration but younger. Those experiencing cognitive deterioration were less likely to receive day services, had more impoverished life experiences, and required more support compared to groups without cognitive deterioration. When age was controlled for, cognitive deterioration was significantly positively associated with caregiver difficulties and service use and negatively associated with life experiences for the individual. Results suggest a potential role for caregiver difficulties in influencing life experiences of adults with Down syndrome showing cognitive decline.     

Intravascular lymphomatosis manifesting in the lung

A case of intravascular lymphomatosis with predominant symptoms in the lung (cough and interstitial pulmonary infiltrates) is presented. Fever and confusion were lateness symptoms. Examination postmortem established the diagnosis. In the lung alveolar walls, small arterioles and the capillaries were occupied by atypical cells positives for CD45 and CD20, leukocyte and pan-B markers, and negatives for CD45-Ro and CD34, pan-T and endothelial markers. The main characteristics and the lung participation of this entity were reviewed.     

Dynamic intrahepatic flow and cellular alterations during radiofrequency ablation of liver tissue in mice

PURPOSE: The purpose of this study was to identify microvascular and other associated changes that occur in the liver during focal heating with monopolar radiofrequency (RF). MATERIALS AND METHODS: Intravital video microscopy was performed on exteriorized transilluminated livers of 15 live mice during RF-induced heating of liver parenchyma. Microvascular flow parameters, flow reversibility, microbubble formation, phagocytic activity, and endothelial permeability were recorded throughout a range of tip temperatures (40 degrees C-95 degrees C). RESULTS: During RF application, five discrete zones extended outward from the electrode surface: (i) tissue coagulation, (ii) cellular edema/necrosis, (iii) sinusoidal stasis, (iv) parenchymal shunting, and (v) normal liver tissue. Reversal of stasis in sinusoids and small (<25 microm) vessels occurred at tip temperatures below 50 degrees C. This zone of stasis corresponded to the hyperemic zone on histologic analysis. Although alterations in permeability and phagocytic activity were first identified at 43 degrees C, tip temperatures higher than 55 degrees C always produced local endothelial leakiness to carbon microparticles at the periphery and always inhibited phagocytic activity. At tip temperatures higher than 95 degrees C, microbubble formation occurred with bubbles ultimately tracking through necrotic tissue into patent sinusoids. Larger peripheral vessels (>30 microm) limited extension of coagulation. CONCLUSION: Although coagulation occurs at tip temperatures higher than 50 degrees C, RF heating induced reversible microvascular stasis at temperatures lower than 50 degrees C. Increased sinusoidal endothelial permeability occurs at near-coagulative temperatures. Therefore, targeted endovascular microparticle delivery through this leaky endothelium may provide an additional and complimentary adjunct for RF ablation therapy.     

Heme oxygenase-1 induction in islet cells results in protection from apoptosis and improved in vivo function after transplantation

Transplantation of islets of Langerhans represents a viable therapeutic approach for the treatment of type 1 diabetes. Unfortunately, transplanted islets are susceptible to allogeneic recognition and rejection, recurrence of autoimmunity, and destruction by local inflammation at the site of implantation. The last of these phenomena might not only result in functional impairment and death of islet cells but could also contribute to amplifying the subsequent specific immune response. Induction of islet cell protection against inflammation could therefore be postulated to be a powerful means to improve overall graft fate. Heme oxygenase-1 (HO-1) has been described as an inducible protein capable of cytoprotection via radical scavenging and apoptosis prevention. The purpose of the present study was to analyze whether HO-1 upregulation in a beta-cell line and in freshly isolated murine islets could result in protection from apoptosis and improve in vivo functional performance. HO-1 upregulation was induced reproducibly with protoporphyrins and was correlated with protection from apoptosis induced in vitro with proinflammatory cytokines or Fas engagement. Furthermore, in vivo HO-1 upregulation resulted in improved islet function in a model of marginal mass islet transplantation in rodents. Strategies aimed at inducing HO-1 upregulation might result in improved success in islet transplantation.     

Preliminary experience with the angiotensin II receptor antagonist irbesartan in chronic kidney disease

Blocking the formation of angiotensin II with converting enzyme inhibitors is an established intervention for kidney disease. The advent of antagonists of the angiotensin II receptor has increased the options for inhibiting the renin-angiotensin-aldosterone system. In adults, angiotensin II antagonists have antihypertensive and antiproteinuric effects similar to those of converting enzyme inhibitors and an adverse effect profile similar to that of placebo. In children, no information is available on angiotensin II antagonists. A total of 20 children (aged 4 to 17 years) with chronic kidney disease received the angiotensin II antagonist irbesartan given once daily. They had hypertension (n=11), overt proteinuria (n=3), or both (n=6). At last follow-up, 2 to 17 months after starting irbesartan (median dosage: 3.3 mg/kg body weight daily), arterial pressure was significantly reduced: the systolic value by 16 [6–22] and the diastolic value by 11 [4–22] mmHg (median and interquartile range). In nine patients with proteinuria, the urinary albumin/creatinine ratio significantly decreased by 145 [105–209] mg/mmol. The frequency of reported adverse events was similar before and with irbesartan. Conclusion In children with chronic kidney disease the effects of the angiotensin II antagonist irbesartan on arterial pressure and proteinuria mimic those observed with the converting enzyme inhibitors. The effectiveness of a single daily dose and the paucity of side-effects suggest that angiotensin II antagonists expand therapeutic options for inhibiting the renin-angiotensin-aldosterone system in children. Received: 7 January 2000 and in revised form: 24 February and 29 February 2000 Accepted: 29 February 2000     

Elevated aminotransferase as a presenting finding in a patient with occult muscle disease

We report a patient with occult muscle disease who presented with raised serum aminotransaminases. This case report emphasises the importance of considering muscle disease in the differential diagnosis of raised serum aminotransaminases, as it may negate the need for invasive investigations such as liver biopsy.     

Functional outcomes of posterior intravaginal slingplasty: report on its impact on urinary,bowel and psychosexual function

The functional impact of any clinical intervention should be the ultimate arbiter of the success of such interventions. This study evaluated the outcomes of urinary, bowel and psychosexual functions in 31 patients undergoing posterior vaginal slingplasty for pelvic organ prolapse. The results show significant improvement in all prolapse symptoms. Urinary symptoms of overactive bladder and stress incontinence improved significantly, as well as the bowel symptoms of obstructed defecation and urgency. Sexual function and psychological state also improved significantly with the procedure. Significantly, to date, this is the first study assessing the psychological impact of pelvic organ prolapse and the remission of these symptoms with the slingplasty. The posterior vaginal slingplasty is an effective procedure in prolapse correction, along with alleviation of the majority of bothersome symptoms caused by the prolapse.     

Compensatory weight gain due to dopaminergic hypofunction: new evidence and own incidental observations

There is increasing evidence for a role of dopamine in the development of obesity. More specifically, dopaminergic hypofunction might lead to (over)compensatory food intake. Overeating and resulting weight gain may be induced by genetic predisposition for lower dopaminergic activity, but might also be a behavioral mechanism of compensating for decreased dopamine signaling after dopaminergic overstimulation, for example after smoking cessation or overconsumption of high palatable food. This hypothesis is in line with our incidental finding of increased weight gain after discontinuation of pharmaceutical dopaminergic overstimulation in rats. These findings support the crucial role of dopaminergic signaling for eating behaviors and offer an explanation for weight-gain after cessation of activities associated with high dopaminergic signaling. They further support the possibility that dopaminergic medication could be used to moderate food intake.