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31.
OBJECTIVE: To determine the mortality of a population of patients diagnosed with Charcot neuropathic osteoarthropathy managed by a single specialist unit and to compare the results with a control population. METHODS: We have undertaken a retrospective analysis of all cases of Charcot foot on the comprehensive database which has been maintained at the specialist diabetic foot clinic at the City Hospital, Nottingham since 1982. Survival and the incidence of amputation (major and minor) was compared with a control population referred with uncomplicated neuropathic ulceration. Controls were individually matched for gender, age (+/-2 years), disease type, disease duration (+/-2 years) and year of referral (+/-3 years). RESULTS: Forty-seven cases (21 female, 26 male) of Charcot foot were identified, of whom 18 (38.3%) had Type 1 diabetes. Mean age and disease duration at presentation were 59.2 +/- 13.4 (sd) and 16.2 +/- 11.2 years, compared with 59.7 +/- 12.6 and 16.3 +/- 11.2 years, respectively, in the controls. Twenty-one (44.7%) of those with Charcot had died, after a mean interval of 3.7 +/- 2.8 years. This compared with 16 (34.0%) after a mean 3.1 +/- 2.7 years in the control group. Mean duration of follow-up in the survivors was 4.7 +/- 4.9 years (Charcot) and 5.3 +/- 3.9 years (controls). A total of 11 (23.4%) Charcot patients had had a major amputation on the side of the index lesion, compared with five (10.6%) controls. There was no difference between the two groups (P > 0.05, Chi-square). CONCLUSIONS: The mortality in this group of patients with Charcot foot was higher than expected. Nevertheless, there was no difference between those with Charcot and those with uncomplicated neuropathic ulceration. It is possible that it is neuropathy, rather than Charcot osteoarthropathy, which is independently associated with increased mortality in diabetes. The mechanism underlying any such association is not known. There is a need for a formal, prospective, multicentre study to investigate the life expectancy and cardiovascular risk of those with Charcot osteoarthropathy. 相似文献
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33.
Nobuyuki Oka Teruaki Kawasaki Kotaro Mizutani Hiroshi Sugiyama Ichiro Akiguchi 《Neuropathology》2007,27(6):509-515
Neuromuscular biopsy is still an essential method for diagnosing vasculitic neuropathy, although its diagnostic sensitivity is at most 60%. Our objective was to examine the expression of hypoxia‐inducible factor 1α (HIF‐1α) in peripheral nerves and to evaluate its usefulness in diagnosing vasculitic neuropathy, especially for discrimination from other axonal neuropathies. Forty‐one patients with vasculitic neuropathy consisting of 20 definite, 14 probable and seven possible diagnoses, 15 patients with metabolic neuropathy, five with motor neuron disease and six with chronic inflammatory demyelinating polyneuropathy were included. Nerve biopsy specimens were immunohistochemically examined for HIF‐1α and various cell markers. Distinct immunoreactivity (IR) was observed in nuclei of endoneurial cells in 54% (22/41) of vasculitic patients, while specimens from metabolic neuropathies showed less nuclear IR and the difference of mean density of HIF‐1α‐positive nuclei was significant. Two patients with possible vasculitis who showed HIF‐1α‐positive nuclei in endoneurium, were later confirmed to have vasculitis by skin biopsies. Most of the cells expressing HIF were demonstrated to be Schwann cells. There was a trend in the vasculitic patients with early phase nerve damage to display higher endoneurial HIF‐1α‐IR. HIF‐1α may be an immunohistochemical marker for vasculitic neuropathy, especially when the observed section contains no vasculitic lesions. 相似文献
34.
J. Finsterer G. Miltenberger H. Rauschka A. Janecke 《European journal of neurology》2006,13(10):1149-1152
The objective of this study was to report a novel exon-1 mutation in the myelin protein zero (MPZ) gene, resulting in axonal Charcot–Marie–Tooth neuropathy with recurrent hyper-CK-emia. In a 64-year-old woman slowly progressive distal lower limb weakness, muscle cramps in the lower limb muscles, and stocking-type numbness had developed from the age of 61. Neurologic examination revealed discrete hip flexor weakness, weakness for foot extension, diffuse wasting of the distal lower limb muscles, reduced patella tendon reflexes, and absent Achilles tendon reflexes. There was recurrently elevated creatine kinase with a maximum of 607 U/l ( n , <145 U/l). Stimulation of the peroneal and tibial nerves did not evoke a muscular response. Electromyography was neurogenic. Biopsy of the right sural nerve showed diffuse axonal degeneration and loss of axons of all diameters. Muscle biopsy showed increased fiber-size variability, angulated fibers, internalized nuclei, accumulations of nuclei, grouped atrophic muscle fibers, and fiber splitting. Molecular genetic analysis by PCR and direct nucleotide sequencing revealed the heterozygous C59T exon-1 MPZ gene mutation, resulting in the amino acid exchange S20F of the MPZ signal protein domain (leader peptide). The novel C59T mutation in the leader peptide of the MPZ gene is pathogenic and manifests as severe, late-onset, axonal, symmetric sensorimotor polyneuropathy (CMT2) and hyper-CK-emia. 相似文献
35.
36.
赵桂芬 《山东医学高等专科学校学报》1992,14(2):135-137
30例儿童糖尿病患者年龄2~14岁,83.3%病前有感染史,起病多较急,年龄越小“三多一少”症状越不典型,多以酮症酸中毒昏迷就诊,其中20%被误诊。建议采用小剂量胰岛素静脉滴注治疗。 相似文献
37.
Emmanuel Ellie Anne Vital Andreas Steck Jean-Michel Boiron Claude Vital Jean Julien 《Journal of neurology》1996,243(1):34-43
We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG. 相似文献
38.
39.
P. Lombrail T. Lang P. Degoulet F. Aimee C. Devries C. Fouriaud M. C. Jacquinet-Salord 《European journal of epidemiology》1988,4(3):371-376
Alcohol consumption and glycosuria were found to be associated (p < 0.001) in a population of 6571 salaried employees who underwent a systematic examination. The prevalence of glycosuria was found to range from 1.3% among 2609 non-drinkers to 5% among 816 heavy drinkers (six glasses or more of alcoholic beverage daily). This association was still significant after adjustement for age, sex and body mass index. Similarly, a positive association was observed between fasting glycemia and alcoholic intake in a subgroup of 998 subjects when such a result was available (p < 0.05). 相似文献
40.
Francine Ratner Kaufman Mary Halvorson Neal D. Kaufman 《Diabetes research and clinical practice》1995,30(3):205-209
Objective: To determine if uncooked cornstarch, as part of the evening snack, can avert nocturnal hypoglycemia in type 1 diabetes. Research Design and Methods: Fifty-one campers and counselors at the American Diabetes Association Camp in San Bernardino, CA were randomly assigned to receive 5 g of uncooked cornstarch as part of the 21:00 evening snack vs. a standard snack of equivalent carbohydrate content. Each snack was given for five nights and the participants and medical personnel were blinded as to assignment. Midnight and 07:00 finger stick blood glucose levels were compared with values <60 mg/dl defined as hypoglycemia and values >250 mg/dl defined as hyperglycemia. Results: There were 218 midnight and 222 07:00 values for comparison. There were six episodes of hypoglycemia at midnight and nine episodes of hypoglycemia at 07:00 for the cornstarch snack nights vs. 30 hypoglycemia episodes at midnight and 21 at 07:00 for the standard snack nights (P < 0.001 and < 0.05, respectively). There was no difference in the number of hyperglycemic events at midnight or 07:00 for the cornstarch vs. standard snack nights. At midnight, 12% of campers had hypoglycemia after the cornstarch snack vs. 46% after the standard snack (P < 0.001), and at 07:00, 16% had hypoglycemia after cornstarch vs. 26% after the standard snack (P = 0.327). Conclusions: These data suggest that uncooked cornstarch, as part of the evening snack, can diminish the nighttime and morning hypoglycemia associated with type 1 diabetes, without causing hyperglycemia. 相似文献