Acquired C1 esterase inhibitor deficiency

Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. In this paper, we summarize experience with 22 cases of acquired C1 esterase inhibitor deficiency in the context of a review of the published literature on diagnosis and treatment of this condition.     

The health of immigrant women: Queensland women from the former Yugoslavia

A study of the social and health status of women from the former Yugoslavia was conducted in Queensland, Australia. Study participants were predominantly refugee women who had migrated to Australia between 1991 and 1996. A significant number of the women rated their health status as poor or fair. Most women did not perceive any change in health following migration, but more felt that their health had deteriorated than improved. Applying a social model of health, we explored the social contexts of countries of origin and destination that impact on women's health. We analyze how preimmigration trauma, settlement problems, health risk behaviors, and participation in screening programs affect women's health status and health needs. Data analysis indicated that government and nongovernment services can reduce the impact of preimmigration experience on health risk behaviors and poor health outcomes only to a limited degree. Since the low socioeconomic status of immigrants following immigration was identified by women as a main contributing factor to their poor health status, government support in tackling structural barriers in accessing the Australian labor market is essential to achieve positive health outcomes.     

Serial position learning effects in patients with aneurysms of the anterior communicating artery

Ruptured and repaired Anterior Communicating Artery (ACoA) aneurysm can result in devastating impairments involving memory, executive function, confabulation, and personality changes. This study tested serial position learning effects (SPEs) in patients following repaired and ruptured ACoA aneurysm, using results on the Rey Auditory Verbal Learning Test (RAVLT). Thirty patients with ruptured aneurysms of the ACoA and 31 matched controls were included in the study. The primacy-recency effects were maintained during five learning trials in ACoA group, albeit at an overall lower level than in the controls. There was no difference in primacy-recency relation across five learning trials in ACoA group. On the delayed recall trial the patient group demonstrated neither a primacy, nor a recency phenomenon, reflecting a lack of recall of any parts of the word list. This kind of primacy-recency profile across learning trials in ACoA group has no similarity with SPE results in frontal lesion groups, or with SPE distributions in other amnesic disorders, despite the fact that memory and executive deficits were evident in our ACoA group.     

Conversion of a fused hip to total hip arthroplasty

BACKGROUND: Arthrodesis of the hip remains a viable treatment for severe unilateral arthritis after traumatic injury or infection in a young but otherwise healthy individual. The goal of the present study was to review the long-term clinical and radiographic results after conversion of a fused hip to a total hip arthroplasty and to identify the risk factors that would lead to a higher rate of failure. METHODS: We performed a retrospective review of the charts and radiographs of 187 patients (208 hips) who had conversion of a fused hip to a total hip arthroplasty. The mean duration of follow-up after the conversion to total hip arthroplasty was 9.2 years (range, two to twenty-six years). RESULTS: The mean age at time of the arthroplasty was fifty-one years. The mean time-interval between the arthrodesis and the conversion to a total hip arthroplasty was twenty-seven years. According to the information in the charts, at a mean duration of follow-up of 9.2 years after the total hip arthroplasty, 79% of hips were either pain-free or had minimal pain, 83% had good-to-excellent function, and 79% had good-to-excellent range of motion. Complications, which included fifteen nerve palsies, occurred in twenty-four hips. Twenty-eight hips had heterotopic ossification, but it was not associated with a recurrence of ankylosis or a marked reduction of motion. Revision arthroplasty was performed in twelve hips. The probability of survival of the implant was 96.1% (95% confidence interval, 91.5% to 98.2%) at ten years, 89.9% (95% confidence interval, 85.3% to 96.1%) at fifteen years, and 72.8% (95% confidence interval, 36% to 90.6%) at twenty-six years. CONCLUSIONS: Conversion of a fused hip to a total hip arthroplasty has a favorable outcome. However, the technically demanding nature of the procedure should not be underestimated. Patients should be cautioned with regard to the possibility of a higher rate of complications than that seen with primary total hip arthroplasty.     

Phase II trial of KW2189 in patients with advanced malignant melanoma

KW-2189, a semisynthetic duocarmycine antibiotic has been shown to exert antiproliferative effects against human tumor cell lines in vitro and animal tumor models in vivo. Phase I studies identified myelosuppression as the most noteworthy adverse effect. Presented are two concurrent phase II studies assessing the antitumor and toxicity profile of KW-2189 in metastatic melanoma patients. One of the studies accrued patients with a history of prior melanoma therapy and the other accrued patients without a history of prior melanoma therapy. KW-2189 was administered at 0.4 mg/m2 to previously treated patients and 0.5 mg/m2 to the previously untreated. Treatment was administered intravenously on day 1 of a 6-week cycle. Thirty previously untreated and 15 previously treated patients were accrued. The toxicity profiles of the both groups of patients were similar. Of the 15 previously treated patients, 8 completed once cycle of treatment, 2 completed 2 cycles, and 5 completed 3 cycles. Dose modification for neutropenia/ thrombocytopenia was necessary in six patients. Among the previously untreated cohort (30 patients), 16 completed 1 cycle, 5 completed 2 cycles, 4 completed 3 cycles, 3 completed 4 cycles, and 2 completed 6 cycles. Doses were modified (neutropenia or thrombocytopenia) in 11 patients. None of the 15 previously treated patients responded to therapy. Four patients remained stable during two cycles. Five of the previously untreated patients achieved a partial remission/regression. Response duration ranged from 2.8 to 16.6 months. Overall objective response rate was 17%. Regarding survival, one previously treated patient is still alive 2.9 years after study entry, and three previously untreated patients are still alive 1.6, 2.3, and 2.9 years after study entry. The 1-year survival rate for previously treated patients is 27% and for the untreated patients is 23%. In summary, the lack of significant antitumor activity of KW-2189 and its associated toxicity suggest that further testing of this regimen in patients with stage IV melanoma is not warranted.     

The influence of mecamylamine on trigeminal and olfactory chemoreception of nicotine.

Nicotine presented to the nasal cavity at low concentrations evokes 'odorous' sensations, and at higher concentrations 'burning' and 'stinging' sensations. A study in smokers and nonsmokers provided evidence of a relationship between the experience with the pharmacological action of S-(-)-nicotine and the perceived pleasantness/unpleasantness following nasal stimulation with S-(-)-nicotine. Mecamylamine, a nicotinic acetylcholine-receptor-(nAch-R) antagonist, was able to block painful responses following chemical stimulation of the human tongue and to block responses from the rat's ethmoidal nerve. The aim of our study in humans was to investigate the effects of mecamylamine on the olfactory and the trigeminal chemoreception of nicotine enantiomers. In order to achieve this aim, we determined-before and after mecamylamine-(1) detection thresholds, trigeminal thresholds, and intensity estimates (stimulus intensity) and (2) recorded the negative mucosal potential (NMP) following nasal stimulation with nicotine in a placebo-controlled double blind study (n = 15). CO(2) was used as a trigeminal and H(2)S as an olfactory control stimulus. Mecamylamine significantly increased trigeminal thresholds of S-(-)-nicotine and reduced intensity estimates and NMPs following stimulation with nicotine enantiomers, whereas mecamylamine did not influence NMPs and trigeminal intensity estimates following stimulation with CO(2). In contrast, mecamylamine did neither influence detection thresholds nor olfactory intensity estimates following stimulation with olfactory nicotine concentrations. These results demonstrate that the trigeminal nasal chemoreception of nicotine enantiomers, in contrast to CO(2), is mediated by nAch-Receptors and give evidence that the olfactory chemoreception of nicotine is independent from peripheral nAch-Receptors.     

The effect of absolute lymphocyte count recovery kinetics on survival after autologous stem cell transplantation for non-Hodgkin's lymphoma

Absolute lymphocyte count (ALC) >or=500 cells/microl at day 15 after autologous stem cell transplantation (ASCT) is a powerful independent, prognostic indicator for survival in multiple hematological malignancies. A limitation in these studies was the selection of a single time point (day 15 post-ASCT) as the only discriminator of clinical outcome in relation to ALC recovery. We hypothesized there is a continuous and not discrete relationship between ALC recovery and clinical outcome post-ASCT in NHL. Therefore, we analyzed 274 consecutive patients who underwent ASCT for NHL between 1987 and 2001. The primary end point was to assess the impact of the kinetics of post-ASCT lymphocyte recovery>or=500 cells/microl (K-ALC) on overall survival (OS) and progression-free survival (PFS). K-ALC was a predictor of OS and PFS when the Cox proportional hazards model was used with K-ALC entered as a continuous variable (p<0.0001). Multivariate analysis demonstrated K-ALC recovery post-ASCT to be an independent prognostic indicator for OS and PFS. These data support our hypothesis that the K-ALC post-ASCT is associated with clinical outcome in NHL.