Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031117 (peripheral neuropathy)
 10,577 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The median survival in primary systemic (AL) amyloidosis is less than 18 months. No published series of patients with AL amyloidosis have reported survival of more than 10 years. The records of all Mayo Clinic patients with a diagnosis of AL amyloidosis between January 1, 1966 and March 1, 1987 were reviewed. Patients with secondary amyloidosis, familial amyloidosis, senile systemic amyloidosis, and localized amyloidosis were excluded. During the 21 years of the study, 841 patients with AL amyloidosis were seen. Of these, 29 were excluded because the diagnosis was made at autopsy, and 2 others were excluded because no follow-up data were available. Actuarial survival for the 810 patients was 51% at 1 year, 16% at 5 years, and 4.7% at 10 years. Thirty patients survived for 10 years or more after the histologic diagnosis of AL amyloidosis; all received alkylating-agent therapy. In 14 patients, the monoclonal protein disappeared from the serum or urine. Of 10 patients with nephrotic syndrome, 4 had an objective response. Congestive heart failure, older age, creatinine value of 2 mg/dL or more, bone marrow plasma cell value of 20% or more, platelet count of 500 x 10(9)/L or less, and the presence of peripheral neuropathy were underrepresented in the 10-year survivors and are unfavorable prognostic features. Five percent of patients with AL amyloidosis survived for 10 years or more.
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PMID:Long-term survival (10 years or more) in 30 patients with primary amyloidosis. 992 Aug 56

Multiple myeloma is characterized by the presence of bone pain, weakness, and fatigue. Ninety-eight percent of patients have an M-protein in the serum or urine at the time of diagnosis. Skeletal roentgenograms are abnormal in nearly 80%. Renal insufficiency (creatinine > or = 2 mg/dL) is present in one-fourth. The major causes of renal insufficiency are "myeloma kidney" and hypercalcemia. The diagnosis of multiple myeloma depends upon the presence of more than 10% plasma cells or a plasmacytoma plus an M-protein in the serum or urine or lytic bone lesions. Multiple myeloma must be differentiated from monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. The plasma cell labeling index and the presence of circulating plasma cells in the peripheral blood are helpful in the differential diagnosis. Plasma cell leukemia, osteosclerotic myeloma (POEMS syndrome), and plasmacytomas are discussed. The heavy-chain diseases consist of alpha, gamma, and mu heavy-chain disease. The fibrils of primary amyloidosis consist of kappa or lambda monoclonal light chains. Weakness, fatigue, and weight loss are the most frequent symptoms. Macroglossia occurs in 10%. An M-protein is found in the serum or urine in 90%. The presence of nephrotic syndrome, renal insufficiency, congestive heart failure, orthostatic hypotension, or sensorimotor peripheral neuropathy, and an M-protein in the serum or urine suggest the possibility of primary amyloidosis. The diagnosis depends upon the demonstration of amyloid in tissues. The subcutaneous fat aspirate is positive in 80% while the bone marrow is positive in 55%. If these tissues are negative, one should obtain tissue from an involved organ.
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PMID:Clinical aspects of multiple myeloma and related disorders including amyloidosis. 1019 81

Insulin-dependent diabetes mellitus (IDDM) is rare in Chinese children. There have been no reports on the prevalence of peripheral neuropathy in Chinese children with IDDM. This study aimed to determine prevalence of subclinical peripheral neuropathy in Chinese children with IDDM. Motor and sensory nerve conduction studies of both median, ulnar, peroneal, and tibial (motor nerves) and median, ulnar, and sural (sensory nerves) were performed in 38 children with IDDM (18 males, 20 females). The age was 4-21 years (mean = 12.7 years; median = 12 years, 6 months). The duration of diabetes was less than 5 years in 15, 5-10 years in 14, and more than 10 years in nine. Neurophysiologic evidence of subclinical peripheral neuropathy was present in 26 patients (68.4%) of which motor, sensory, or motor and sensory involvement was 26 (68.4%), eight (21.1%), and 26 (68.4%), respectively. Twelve (31.6%) and 14 (36.8%) children had mild and moderate degrees of peripheral neuropathy, respectively. Among the 26 children with abnormal nerve-conduction studies, two (7.7%) had symptoms of numbness and pain in the lower limbs. Thus, two children had symptomatic neuropathy and most (n = 24) had asymptomatic peripheral neuropathy. Two children had systemic hypertension, and one (3.8%) had laboratory evidence of early renal complications. Analysis of demographic and laboratory risk factors for the development of subclinical peripheral neuropathy revealed that the age of onset, duration of diabetes, level of hemoglobin A1c, triglyceride, cholesterol, serum creatinine, and urea, microalbumin/creatinine ratio, and urinary microalbumin excretion rate were significantly related to the development of subclinical peripheral neuropathy in specific nerves.
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PMID:Prevalence of peripheral neuropathy with insulin-dependent diabetes mellitus. 1020 29

POEMS syndrome is a multisystem disorder with signs such as peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein, skin lesions, papilledema, and increased cerebrospinal fluid proteins, which can also evolve with renal and cardiac affection. It is considered a result of a plasma cell cyscrasia with the production of a monoclonal protein. A 46-year old man was seen as an outpatient referring progressive weakness of legs and arms, fever, impotence, inguinal and cervical lymphadenopathies, peripheral edema, hepatomegaly and skin hyperpigmentation. In laboratory test, platelet count was between 528 x 10(9)/L and 599 x 10(9)/L, creatinine clearance 27.2 ml/min, proteinuria 0.8 g/dl, IgA 455 mg/dl, T3 30 ng/100 ml, T4 2.6 vg/dl, T4F 0.5 ng/dl, TSH 12.4 vU/ml; testosterone 1.56 ng/ml. The electromyography showed a mixed sensitive-motor pattern. On the pelvis radiography, an osteosclerotic lesion on the left sacroiliac joint was identified. Bone biopsy of the site of the sclerotic lesion revealed plasma cell dyscrasia. The patient was treated with diuretics, digitalis and prednisone. Diagnosis of this disorder is difficult because of the multipathology it is necessary to establish differential diagnosis.
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PMID:[POEMS syndrome. Report of a case]. 1042 26

Neuropeptide Y (NPY) is a potent vasoconstrictor peptide that is abundant in the brain and the peripheral sympathetic nervous system. In the present study we investigated possible changes in plasma immunoreactive (IR)-NPY concentrations and urinary IR-NPY excretion in patients with non-insulin dependent diabetes mellitus (NIDDM) and the relationship to diabetic complications, such as nephropathy and neuropathy. IR-NPY in plasma and urine was measured by radioimmunoassay in 69 patients with NIDDM. Plasma IR-NPY concentrations in patients with advanced nephropathy (creatinine clearance <30 ml/min) (100.5 +/- 10.3 pmol/l, n=9, mean +/- SEM) were higher than in the control subjects (55.0 +/- 6.8 pmol/l, n=15) (P<0.02). Urinary excretion of IR-NPY and fractional excretion of NPY were also increased in the patients with advanced nephropathy. Sephadex G-50 column chromatography of the urine extracts obtained from healthy subjects, diabetic patients with renal failure and non-diabetic patients with renal failure showed an immunoreactive peak eluting in the NPY position. On the other hand, neither plasma nor urinary IR-NPY was high in patients with retinopathy, or in patients with peripheral neuropathy. The present study has, for the first time, shown high plasma IR-NPY concentrations and urinary IR-NPY excretion in NIDDM patients with advanced nephropathy.
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PMID:Elevated plasma immunoreactive neuropeptide Y concentrations and its increased urinary excretion in patients with advanced diabetic nephropathy. 1042 78

Thirty patients with primary hepatocellular carcinoma or liver metastases were entered into a program of chemoembolization with cisplatin, lipiodol, and escalating doses of thiotepa. Doses of cisplatin were 100/m2, and thiotepa doses ranged from 9 mg/m2 to 24 mg/m2. Two of three patients with ocular melanoma had partial responses in the liver metastases for 3+ and 16 months. In patients with either hepatocellular carcinoma (15 patients) or primary cholangiocarcinoma of the liver (three patients), there were two partial responses, for 22 and 33 months. Five patients had minor responses: four with a 40% reduction in tumor and one with a mixed response. There were four early deaths, which involved sepsis in two patients, respiratory failure in one, and acute myocardial infarction in one. Otherwise, toxicity was tolerable and reversible and included abdominal pain and transient elevation of serum creatinine, bilirubin, and transaminases. Less common toxicities included ototoxicity and peripheral neuropathy. Chemoembolization of the liver with cisplatin, thiotepa, and lipiodol can produce responses, but toxicity can be significant. The recommended starting phase II dose for future studies is thiotepa 24 mg/m2 and cisplatin 100 mg/m2.
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PMID:A phase I study of chemoembolization with cisplatin, thiotepa, and lipiodol for primary and metastatic liver cancer. 1044 Jan 93

The primary aim of this phase II study was to determine the response rate of a combination of paclitaxel and carboplatin in advanced non-small cell lung cancer (NSCLC) in a multicentre setting. The secondary aim was to determine time to progression (TTP), 1-year survival rate and toxicity. 65 patients were treated and all of them were included in the follow up for survival and toxicity. 60 patients were followed for response rate and time to progression. 55% were stage IV patients and 45% stage IIIB patients. The treatment consisted of paclitaxel 200 mg/m2 given as a 1-h i.v. infusion and carboplatin given as a 30 min i.v. infusion and the latter was dosed by using the Calvert formula at an area under the concentration time curve (AUC) of 5. The glomerular filtration rate (GFR) was determined by iohexol clearance and was not calculated from the serum creatinine level. The chemotherapy courses were given every third week with a maximum number of six or eight courses for patients who responded late. As premedication we used 8 mg betamethasone 40 min prior to infusion and then 10 min later clemastin and cimetidine. One complete response and 18 partial responses were seen giving a response rate of 29%. 40% of the patients progressed during the treatment and 28% had stable disease. The median TTP was 22 weeks. At a minimum follow up of 1 year, the 1-year survival rate was 38% and the median survival rate was 41 weeks. Haematological toxicity was mild with no grade 4 leucopenia and only seven patients (11%) had grade 3 leucopenia. There was no grade 4 toxicity. Grade 3 toxicity was seen as myalgia 5%, allergic reaction 3% and peripheral neuropathy 6%. 15% of the patients had a dose reduction due to neurotoxicity. The haematological toxicity was much milder than we expected, probably because of more exact determination of the GFR. This trial confirms the results of earlier reported trials of the efficacy and the ease of the regimen as an out-patient treatment option in advanced NSCLC. The main problem with this treatment is peripheral neuropathy.
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PMID:Treatment with paclitaxel 1-h infusion and carboplatin of patients with advanced non-small-cell lung cancer: a phase II multicentre trial. Joint Lung Cancer Study Group. 1044 61

India is amidst a demographic transition showing an ageing trend. This will increase non-communicable diseases including diabetes which is already showing an increasing trend. With scanty literature existing on elderly diabetics (> 60 years of age), it was decided to study the clinico-laboratory and complication profile of this group of patients. Fifty consecutive elderly diabetics were studied and evaluated for ECG, chest x-ray, blood sugar, urea, creatinine, lipid profile, proteinuria, motor nerve conduction velocity and autonomic neuropathy. Duration of diabetes varied from one month to 28 years. Fifty-six per cent of the patients presented with classical symptoms of polyuria, polyphagia and polydipsia. Hypertension was present in 40% and cataract in 54% of the patients. Eighteen per cent were obese, 52% had evidence of peripheral neuropathy while 56% had autonomic neuropathy. Background diabetic retinopathy was present in 56%, pre-proliferative retinopathy and maculopathy in 4% each; hypertensive retinopathy in 10% of patients; 44% had microproteinuria and 8% had chronic renal failure. Hypercholesterolaemia was present in 64% and hypertriglyceridaemia in 42% of the patients with 26% having coronary artery disease. Sixty per cent were harbouring infections--20% had foot infections, 14% had tuberculosis and 10% had urinary tract infections. Ninety-two per cent of the patients were aware of their disease but 62% were not aware of the complications and of the need for strict dietary and drug compliance. There was a high prevalence of associated diseases viz, osteoarthritis, cataract, hypertension, hepatitis and parkinsonism. Therefore, this study brings out the need to have a holistic and multidisciplinary approach for management of elderly diabetics who constitute a heterogeneous group with distinct health care problems.
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PMID:Clinical and laboratory profile of diabetes in elderly. 1065 95

Tumor necrosis factor alpha (TNF alpha) is usually excreted by the kidney. In dialysis patients, it accumulates. TNF alpha has been implicated in the pathogenesis of malnutrition, diabetic neuropathy, and erythropoietin resistance. We studied TNF alpha plasma levels in 49 stable peritoneal dialysis (PD) patients, with the aim of correlating those levels with the presence and severity of peripheral neuropathy, hypertrophic cardiomyopathy, and anemia. Kt/Vurea' residual renal creatinine clearance (CrC), nutritional markers, and general biochemistry were also determined. The average plasma level of TNF alpha was 67 +/- 32 pg/mL (range: 18.1-156.3 pg/mL; normal value 3-20 pg/mL). No correlation was observed between TNF alpha and KT/Vurea' but a negative correlation with CrC was seen (r: -0.37, p < 0.05). TNF alpha levels were higher in patients with neuropathy as compared to patients with normal results (72.5 +/- 32 pg/mL vs 44 +/- 22 pg/mL, p < 0.05). Patients with neuropathy also showed a lower CrC value (1.5 +/- 1.7 mL/min vs 3.9 +/- 2.6 mL/min, p < 0.01). TNF alpha levels were higher in patients with left ventricular hypertrophy (LVH) with respect to normal individuals (70.4 +/- 32 pg/mL vs 38.5 +/- 20.8 pg/mL, p < 0.05). Patients with severe LVH showed the lowest CrC value. A direct, significant relationship was found between TNF alpha levels and weekly erythropoietin dose (r: 0.41, p < 0.05). Patients with hypertriglyceridemia or taking lipid-lowering agents showed a positive linear correlation between TNF alpha and triglycerides (r = 0.7, n = 14, p < 0.05). These data suggest that accumulation of TNF alpha may contribute to the development or maintenance of some neurologic, hematologic, and cardiac complications of uremic syndrome. Loss of residual renal function conditions an increment in TNF alpha levels. These data continue to add support to the idea that TNF alpha may be considered a uremic toxin.
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PMID:Tumor necrosis factor alpha as a uremic toxin: correlation with neuropathy, left ventricular hypertrophy, anemia, and hypertriglyceridemia in peritoneal dialysis patients. 1068 77

One hundred and twenty-nine (87%) of a total county population of 150 eligible diabetic children together with 144 age- and sex-matched control children participated in a longitudinal, epidemiological study of the evolution of diabetic microvascular disease. At enrollment the median (range) age of the diabetic children was 12.5 (3.7-16.8) years with a median diabetes duration of 2.9 (0.1-13.4) years and a median HbAl of 11.1 (6.8-17.9)%. Two sets of measurements were made over a period of 18 months for all indices of microvascular disease, while autonomic function was studied on one occasion. Urinary albumin excretion in diabetic children was assessed from all voidings during two timed 48-h urine collections and was expressed as urinary albumin/creatinine ratios (ACR). Blood pressure (BP) was measured using a random zero sphygmomanometer. Autonomic function was assessed by pupillary adaptation in darkness, using a portable Polaroid pupillometer, and by heart rate (HR) variation recorded by dedicated computer. Vibration sensation thresholds (VST) (as indices of peripheral neuropathy) were recorded using a Biothesiometer. Limited joint mobility (LJM) was assessed by the "prayer sign". Five (3.9%) diabetic children presented raised mean ACR in more than two of four 24-h urine collections. Fourteen (10.8%) diabetic children were identified as having persistently raised BP during both study periods. Impaired HR response in one HR test was observed in 20 (15.5%) diabetic children, while ten (7.7%) diabetic children demonstrated abnormalities in two or more HR tests. Reduced pupillary adaptation in darkness was found in eight (7.9%) diabetic children. Persistent vibration sensation impairment (VST) in lower limbs was detected in eight (6.2%) diabetic children, while LJM was present in 12 (9.3%) diabetic children. Eight of the 129 diabetic children (6.2%) were found to have abnormality in two and one in three indices of microvascular and autonomic function. Six of nine children had coexistence of impaired autonomic neuropathy and nephropathy. These nine children were diagnosed at a younger age than the rest of the diabetic population (5.1 vs 8.0 yr, p=0.002). Four of nine were aged >11 yr and five of nine had had diabetes for >5 yr. Thus, a constellation of microvascular and neurological abnormalities were demonstrable in a small proportion of diabetic children, who were younger than the rest of the population at the time of onset of their disease. Longitudinal study of this population will demonstrate the clinical significance of these findings.
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PMID:Prevalence of microvascular and neurologic abnormalities in a population of diabetic children. 1082 Dec 21


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