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1.
Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients presenting with these symptoms. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis. Overlooking the diagnosis of light chain amyloidosis leading to therapy delay is common, and it represents an error of diagnostic consideration. Herein we provide a review of established and investigational treatments for patients with AL amyloidosis and provide algorithms for workup and management of these patients.Subject terms: Myeloma, Chemotherapy 相似文献
2.
An American kindred with systemic amyloidosis presenting with carpal tunnel syndrome, peripheral neuropathy, and cardiomyopathy is reported. The transthyretin gene of a patient was analysed by direct DNA sequencing and both cytosine and thymine were present at the first base of codon 24. This new point mutation in exon 2 results in the amino acid substitution of serine for proline in the A-B loop of the transthyretin molecule. DNA testing for this mutant allele by restriction fragment length polymorphism analysis based on the polymerase chain reaction is described. 相似文献
3.
HJ Aubin S Tilikete C Laureaux HT Nguyen Hac MC Roullet-Volmi S Troupel D Barrucand 《European psychiatry》1995,10(8)
The aim of this study was to assess alcoholic inpatients' smoking and coffee intake variation following withdrawal. Only moderate smokers (less than 30 cigarettes/day) showed a significant increase of cigarette consumption after alcohol withdrawal. However, their urinary cotinine level did not vary, suggesting a behavioral, and not biological, compensation through smoking following alcohol withdrawal. Heavy smokers (30 cigarettes/day or more) showed no significant clinical or biological variation of smoking behavior. Coffee consumption increased after alcohol withdrawal in all patients, irrespective of smoking habits. 相似文献
4.
BACKGROUND: A simple, rapid, inexpensive method for measuring the flow in a
patient's vascular access would permit routine monitoring during
haemodialysis, and hence provide information of access graft deterioration
sufficiently early to increase the success of minimally invasive remedial
procedures. This paper reports the validation of such a method in animals.
METHODS: A PTFE graft was implanted in sheep between the carotid artery and
the jugular vein. While the sheep was under general anaesthesia and on an
haemodialysis circuit, ultrasound velocity in its blood was perturbed by
the injection of a 5-10 ml bolus of isotonic NaCl. The pump tubing flow was
measured by a transit-time blood flow meter. This flow was combined with
the areas of perturbation generated by the injection before and after
mixing in the access flow to estimate graft flow. The calculated graft flow
was compared to flow measured directly by a transit-time probe on the same
carotid artery. RESULTS: Over a 10-fold range, 120-1260 ml/min, graft flow
measured by ultrasound velocity dilution agreed well with graft flow
measured directly with a scatter of 76 ml/min about the regression line.
CONCLUSION: Ultrasound velocity dilution provides a method for measuring
flow in the graft accurate enough for clinical evaluation of patients on
dialysis.
相似文献
5.
M Kaplan HJ Vreman C Hammerman C Leiter B Rudensky MG MacDonald DK Stevenson 《Acta paediatrica (Oslo, Norway : 1992)》1998,87(4):455-457
The incidence (%) of hyperbilirubinemia (serum bilirubin ≥257 μmol/l) was similar in neonates with a combination of ABO incompatibility and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency (45%), with ABO incompatibility (54%) or G-6-PD deficiency (37%), alone (ns). Carboxyhemoglobin values, corrected for inspired CO, were similarly elevated in all three groups (0.87 ± 0.32%, 0.82 ± 0.29%, 0.76 ± 0.18%, respectively, ns), but correlated with bilirubin only in those with ABO incompatibility alone. ABO-incompatible/G-6-PD-deficient neonates, compared with those with either condition alone, are not at increased risk for hemolysis or hyperbilirubinemia. 相似文献
6.
Yuning Xiong Kathleen A Donovan Michael P Kline Michael K Gornet Laurie L Moon-Tasson Martha Q Lacy Angela Dispenzieri Morie A Gertz Philip R Greipp John A Lust 《Journal of interferon & cytokine research》2006,26(2):83-95
Interleukin-1beta (IL-1beta) is abnormally expressed by the plasma cells obtained from myeloma patients, and it is a potent inducer of the important myeloma growth factor, IL-6. We investigated whether levels of IL-1beta biologic activity might distinguish different groups of patients with smoldering multiple myeloma (SMM). We measured the ability of IL-6 production by bone marrow stromal cells to serve as a surrogate marker for IL-1beta biologic activity. Using this IL-1beta bioassay, we found that it is sensitive at < 1 pg/ml of recombinant IL-1beta and that IL-1beta biologic activity is detectable with either mature or pro-IL-1beta-transduced myeloma cell lines. Patients with active myeloma induced quantitatively higher levels of stromal cell IL-6 production when compared with those with monoclonal gammopathy of undetermined significance (MGUS). The bioassay distinguished two groups of SMM patients, those who were high producers, similar to patients with active MM, and those who were low producers, comparable to MGUS patients. IL-1 antagonists inhibited the paracrine IL-6 production by > or = 90% in the majority of patients with an elevated IL-6 level. Based on such studies, it may be possible to predict patients that will progress to active MM and to delay or prevent this progression with IL-1 antagonists. 相似文献
7.
The effects of co-culture with human fibroblasts on human embryo development in vitro and implantation 总被引:5,自引:0,他引:5
Wetzels AM; Bastiaans BA; Hendriks JC; Goverde HJ; Punt-van der Zalm AP; Verbeet JG; Braat DD 《Human reproduction (Oxford, England)》1998,13(5):1325-1330
In a human in-vitro fertilization (IVF) programme, the effect of co-
culture of embryos with human fibroblasts was evaluated with respect to
pregnancy rate and embryo development. Patients were included in the study
after giving informed written consent. The IVF treatments were randomly
assigned by stratification of both age (<36 versus > or =36 years)
and previous IVF attempts (yes versus no). After fertilization was
established, the zygotes were transferred to a 4-well dish with or without
fibroblasts and cultured for 2 days. On the third day after ovum pick-up
(OPU), cell number and quality [5 (good) to 1 (poor)] of the embryos were
scored and a maximum of three embryos was transferred. Supernumerary
embryos of good quality were cryopreserved. The design of this study was a
group sequential trial with the objective of detecting differences between
pregnancy rates following IVF with conventional incubation or incubation in
co-culture with fibroblasts. This design included one evaluation at
half-way data collection. In the study, 148 patients had an OPU, of whom 77
were allocated to the co-culture group. There was no statistically
significant difference in pregnancy rate, cell number and embryo quality
between the two groups. The ongoing pregnancy rate per embryo transfer was
27% in co-culture and 30% in the conventional culture group. The
implantation rates per transferred embryo were 17 and 18% respectively.
Using a multivariate logistic regression model for the probability of
ongoing pregnancies, the odds ratio of co-culture, adjusted for age and
previous IVF attempts, was not statistically significant. In conclusion,
co-culture with human fibroblasts does not contribute to an improvement of
embryo quality nor to a higher pregnancy rate after IVF in an unselected
group of patients.
相似文献
8.
Pulmonary marginal zone lymphoma of MALT type as a cause of localised pulmonary amyloidosis
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AIM: To describe six patients with pulmonary marginal zone lymphoma in whom amyloid deposition was identified. Marginal zone lymphoma is a recently recognised type of low grade non-Hodgkin's lymphoma. METHODS: A computerised search was performed of all patients seen at the Mayo Clinic with a diagnosis of pulmonary amyloidosis. Six patients with pulmonary amyloidosis who had biopsy confirmed extranodal marginal zone lymphoma of mucosa associated lymphoid tissue type were identified. All were women, ranging in age from 45 to 85 years. RESULTS: Five patients had amyloid deposition in conjunction with pulmonary marginal zone lymphoma at the time of the original diagnosis. One patient was referred for evaluation of localised pulmonary amyloidosis and was found to have coexisting pulmonary marginal zone lymphoma. Clinical presentation was limited to pulmonary symptoms (two of the six) or constitutional symptoms (two), or was asymptomatic (two). In all six cases, initial findings of nodular densities on screening chest roentgenograms led to further evaluation and eventual lobectomy; these findings included multiple pulmonary nodules (four), single nodule (one), and single nodule with diffuse bilateral interstitial infiltrates (one). Bone marrow was examined in five patients and was normal in all. Protein studies performed in four patients revealed no monoclonal protein. No patients had manifestations of systemic amyloidosis, such as renal, neurological, or cardiac involvement, at a median follow up of 50 months. Four of the six patients remain alive at a median of five years. CONCLUSIONS: Pulmonary marginal zone lymphoma may be found in association with localised amyloid deposition and should be considered in the differential diagnosis of localised pulmonary amyloidosis. 相似文献
9.
Endothelin receptor expression in human decidua 总被引:3,自引:1,他引:3
Kohnen G; Campbell S; Irvine GA; Church HJ; MacLachlan F; Titterington M; Cameron IT 《Molecular human reproduction》1998,4(2):185-193
The endothelins are signalling peptides that act via two receptors, ET(A)
and ET(B). In the human endometrium, endothelin receptors have been
demonstrated in glands and stroma and have been shown to vary during the
course of the menstrual cycle. The present study was undertaken to
determine whether or not expression of endothelin receptors changes during
pregnancy or after administration of exogenous progestagens. The expression
of the receptors was correlated with the appearance of basement membrane
components during decidualization of the endometrial stroma. Decidual
specimens (n = 15) were obtained during the first trimester of pregnancy
and 10 at term. Sixteen pairs of endometrial biopsies were obtained from
women with menorrhagia before and after exposure to exogenous progestagens.
A total of 15 hysterectomy specimens were used as controls for the
expression of stromal basement membrane proteins in the absence of
decidualization. Autoradiography was carried out with selective ligands for
ET(A) ([125I]-PD 151242) and ET(B) ([125I]-BQ3020). The distribution of
ligand binding was then compared with the distribution of laminin alpha2
light chain and collagen IV. ET(A), ET(B), laminin alpha2 light chain, and
collagen IV were expressed in stromal decidual cells in the first trimester
of pregnancy. ET(B) was also found on endometrial glandular epithelium.
Quantitative macro-autoradiography and multiple regression analysis
demonstrated a highly significant positive correlation (P < 0.001)
between expression of ET(B) and laminin alpha2 light chain. In the third
trimester qualitative examination suggested a reduction of ET(A) in the
stroma. Progestagen-induced decidua exhibited a similar pattern to that
found in first trimester decidua. This study has demonstrated up-regulation
of ET(B) during the progesterone- dependent process of decidualization and
suggests a paracrine or autocrine role for endothelins in the decidua.
相似文献
10.
Arterial spasm was induced by application of calcium chloride to the adventitial surface of the rabbit common carotid artery in vivo. Sodium chloride (NaCl) was applied to the contralateral vessel as control. Vessels were fixed in situ by intravascular perfusion after 15 min, 1 hr, or 24 hr and prepared for light and scanning electron microscopy (SEM). With SEM, the luminal surface at the site of calcium application showed severe longitudinal folding accompanied by endothelial desquamation with extensive platelet deposition on exposed subendothelium. The luminal cross-sectional area was reduced by 53 +/- 19.5% after 15 min and by 44 +/- 12% after 1 hr as compared with the contralateral control. Furthermore, the luminal area at the site of calcium application was found to be reduced by 42 +/- 8% after 1 hr when compared with segments of the same vessel distal to the site of calcium application. Blood flow rate, as measured by electromagnetic flow probe, was not reduced. Vessels examined after 24 hr showed a significant increase in luminal cross-sectional area as compared with contralateral control vessels (136 +/- 70%). Control vessels (NaCl) showed no significant change in luminal cross-sectional area and no endothelial desquamation or platelet deposition after 15 min, 1 hr, or 24 hr. Examination of histologic sections showed calcium precipitation within the attached thrombus after 15 min with calcium deposits also adherent to the adjacent luminal aspect of the internal elastic lamina (IEL). By 24 hr, this precipitation extended throughout the media. Marked deposition of leukocytes was seen after 24 hr which showed a preferential attachment for areas of endothelial damage and discontinuity of IEL. 相似文献