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T P Links

Publications and source records attributed to T P Links.

At least 19 recordsLinked to original sources

[Prophylactic thyroidectomy in children who are carriers of a multiple endocrine neoplasia type 2 mutation: description of 20 cases and recommendations based on the literature].

OBJECTIVE: Evaluation of treatment of children who are proven carriers of a multiple endocrine neoplasia type 2 (MEN 2)-associated rearranged during transfection (RET) gene mutation. DESIGN: Retrospective case study and review of the literature. METHOD: Between 1976 and 2005, 6 boys and 14 girls with a proven RET mutation or biochemical indication of MEN 2 had thyroid surgery at the University Medical Center, Groningen, The Netherlands. The median age was 10 years (range: 0-08). Preoperative assessment, surgical procedure, pathological findings, postoperative complications and treatment results were studied and compared with data from the literature. RESULTS: All 20 children underwent total thyroidectomy. In 17 children with preoperatively abnormal basal or stimulated calcitonin levels, total thyroidectomy was combined with tracheo-oesophageal exploration (n = 6) or central compartment dissection (n = 11). C-cell hyperplasia was found in 19 cases (95%) and medullary thyroid carcinoma in 14 (70%; aged 3-18 years). Lymph-node metastases were found in 3 children (15%), all over the age of 10. They underwent additional selective lateral neck dissection, unilateral in 2 cases and bilateral in 1. Two children developed hypoparathyroidism postoperatively, no recurrent laryngeal-nerve palsy was observed. All patients are clinically free of disease after a median follow-up of 9 years (range: 0.6-27). The patients with node metastases still have biochemical evidence of disease. The literature indicates that the progression of the malignant transformation to medullary thyroid carcinoma is connected to the type of RET-mutation. The treatment plan depends on the type of mutation. CONCLUSION: Medullary thyroid cancer occurs at a very young age in carriers ofgermline RET mutations. In patients with high-risk mutations prophylactic thyroidectomy is likely to be recommended before the child reaches the age of 2. Elective central lymph-node dissection can be omitted in this instance. After this age, however, the risk of lymph-node metastases increases and, for cases with increased basal or stimulated calcitonin levels, total thyroidectomy with central compartment dissection is indicated.

Adolescent↗

Improving self-management in insulin-treated adults participating in diabetes education. The role of overprotection by the partner.

AIMS: To examine the role of overprotection by the partner--i.e. excessive protection, unnecessary help, excessive praise for accomplishments, or attempts to restrict activities as a consequence of underestimating the patient's capabilities--in changes in patient self-management in the context of diabetes education. METHODS: Sixty-seven insulin-treated patients with a partner completed questionnaires on admission to a Multidisciplinary Intensive Education Programme (MIEP) and 3 months after completing the core module of MIEP. Factors assessed were overprotection by their partner and three aspects of diabetes self-management, namely internal locus of control, diabetes-related distress and HbA1c. Regression analyses were used to test the independent associations of patient sex, baseline overprotection and the interaction between sex and overprotection with diabetes self-management at the follow-up stage, controlling for the baseline value of the dependent variable. RESULTS: The increase in internal locus of control and decrease in HbA1c were both significantly less for female patients who perceived their partner to be rather overprotective than for female patients who did not perceive their partner to be overprotective. The more patients, both male and female, perceived their partner to be overprotective, the less their diabetes-related distress decreased. CONCLUSIONS: Overprotection by the partner showed a negative association with improvement in diabetes self-management, especially for female patients. Thus, an intervention programme with the aim of reducing overprotection by the partner, or the perception of this, may enhance self-management in patients participating in diabetes education.

Adolescent↗

Increased accumulation of skin advanced glycation end-products precedes and correlates with clinical manifestation of diabetic neuropathy.

AIMS/HYPOTHESIS: The accumulation of AGE is related to the progression of the renal, retinal and vascular complications of diabetes. However, the relationship with diabetic neuropathy remains unclear. We recently showed that skin autofluorescence, measured non-invasively with an AutoFluorescence Reader (AFR), could be used to assess skin AGE accumulation. We evaluated the relationship between skin autofluorescence and the severity of diabetic neuropathy. MATERIALS AND METHODS: Skin autofluorescence in arbitrary units (AU) was assessed in 24 diabetic patients with a history of neuropathic foot ulceration (NP(+)), 23 diabetic patients without clinical neuropathy (NP(-)) and 21 control subjects, using the AFR. Arterial occlusive disease was excluded in all. The severity of foot ulceration was assessed by the Wagner score. Peripheral nerve function was assessed by neurography, measuring motor and sensory nerve conduction velocity and amplitude of the median, peroneal and sural nerves. Heart rate variability (HRV) and baroreflex sensitivity (BRS) were measured by Finapres to assess autonomic nervous function. RESULTS: Autofluorescence was increased in NP(-) compared with control subjects. In NP(+) patients, autofluorescence was further increased and correlated with the Wagner score. Autofluorescence correlated negatively with nerve conduction velocity and amplitude, HRV and BRS in both NP(+) and NP(-) groups. Autofluorescence correlated with age, diabetes duration, mean HbA(1)c of the previous year, serum creatinine level, presence of microalbuminuria and severity of diabetic retinopathy. CONCLUSIONS/INTERPRETATION: Skin autofluorescence correlates with the severity of peripheral and autonomic nerve abnormalities in diabetes, even before being clinically manifest. The AFR may be a convenient and rapid clinical tool for assessing risk of progression of long-term diabetic complications.

Aged↗

Cost and benefits of a multidisciplinary intensive diabetes education programme.

OBJECTIVES: To determine the cost and benefits of an intensive diabetes education programme for patients with prolonged self-management problems and to determine the inclusion criteria for optimal outcomes. METHODS: Sixty-one participants of a multidisciplinary intensive diabetes education programme (MIDEP) were measured before they started the intervention (T0), and at 1-year follow-up (T1). Data on glycaemic control (HbA1c), diabetes-related distress (PAID) and costs were obtained. Changes over time were analysed and means at T0 and T1 were compared to a reference group of 230 non-referred consecutive outpatients. The number needed to treat (NNT), that is, the number of patients to be treated to achieve one successful case, was calculated for different baseline values of HbA1c and PAID to determine optimal inclusion criteria. RESULTS: Diabetes-related costs decreased significantly and participants improved significantly in HbA1c and diabetes-related distress following MIDEP. HbA1c and distress reached the levels of the reference group. The T1 costs remained higher than in the reference group, but the reduction in costs outweighed the intervention costs. Including patients with baseline HbA1c>or=8.0% and/or PAID scores>or=40 would improve the NNT to achieve clinically relevant outcomes, while 76% of the patients matched these inclusion criteria. CONCLUSIONS: MIDEP is effective in improving glycaemic control and diabetes-related distress for patients with prolonged self-management difficulties. Besides the immediate reduction in costs found in the present study, improved glycaemic control may reduce future costs of diabetic complications. Stricter inclusion criteria with respect to HbA1c and PAID scores may further improve the programme's efficiency.

Adult↗

Life expectancy in differentiated thyroid cancer: a novel approach to survival analysis.

In differentiated thyroid carcinoma 10-year survival rates amount to 80-95%. Because age at diagnosis varies widely, these survival rates strongly depend on age at presentation. The aim of the present study was to analyse the attributable risk factors, including therapy per se, on survival in thyroid cancer after proper adjustment for the baseline mortality rate in the general population and to elucidate the adverse treatment effects on survival. Initial treatment in 504 patients consisted of thyroidectomy and 131I ablation. High-dose 131I was administered for residual disease. Patients in complete remission underwent an annual physical examination and thyroglobulin measurements during TSH suppression. Survival time was studied after transformation to standardised survival time to adjust for the baseline mortality rate in the general population. Median follow-up since diagnosis was 9 years. The 10-year overall survival was 83% and disease-specific survival 91%. After initial treatment, persistent disease occurred in 75 patients (15%). In univariate analysis, T4, N1, M1 status and Hürthle cell type were prognostic for persistent and recurrent disease. Age was not prognostic for recurrent disease in multivariate analysis. The standardised survival time was not altered in disease-free patients. However, patients with persistent disease had a median standardised survival time of only 0.60 (95% confidence interval 0.47;0.72), ranging from 0 to above 1, independent of initial tumour status or age. The cumulative proportion of persistent disease was at least 20% of the whole group. Disease-free patients after thyroid carcinoma have a normal residual life span. In contrast, in cases of persistent disease the life expectancy ranges widely with its median being reduced to 60%. Overall, treatment including radioiodine is safe but unsuccessful in 20% of the patients. Age is not a disease-specific risk factor and should not be used as an independent factor in treatment algorithms.

Adult↗

Simple non-invasive assessment of advanced glycation endproduct accumulation.

AIMS/HYPOTHESIS: The accumulation of AGE is thought to play a role in the pathogenesis of chronic complications of diabetes mellitus and renal failure. All current measurements of AGE accumulation require invasive sampling. We exploited the fact that several AGE exhibit autofluorescence to develop a non-invasive tool for measuring skin AGE accumulation, the Autofluorescence Reader (AFR). We validated its use by comparing the values obtained using the AFR with the AGE content measured in extracts from skin biopsies of diabetic and control subjects. METHODS: Using the AFR with an excitation light source of 300-420 nm, fluorescence of the skin was measured at the arm and lower leg in 46 patients with diabetes (Type 1 and 2) and in 46 age- and sex-matched control subjects, the majority of whom were Caucasian. Autofluorescence was defined as the average fluorescence per nm over the entire emission spectrum (420-600 nm) as ratio of the average fluorescence per nm over the 300-420-nm range. Skin biopsies were obtained from the same site of the arm, and analysed for collagen-linked fluorescence (CLF) and specific AGE: pentosidine, N(epsilon)-(carboxymethyl)lysine (CML) and N(epsilon)-(carboxyethyl)lysine (CEL). RESULTS: Autofluorescence correlated with CLF, pentosidine, CML, and CEL ( r=0.47-0.62, p</=0.002). In 32 of 46 diabetic patients (70%), autofluorescence values were above the 95% CI of the mean value in control subjects, and correlated with age, diabetes duration, mean HbA(1)c of the previous year and creatinine levels. CONCLUSIONS/INTERPRETATION: The AFR offers a simple alternative to invasive measurement of AGE accumulation and, to date, has been validated in non-pigmented skin. The AFR may prove to be a useful clinical tool for rapid risk assessment of AGE-related long-term complications in diabetes mellitus and in other conditions associated with AGE accumulation.

Adult↗

[From gene to diseases; hypokalemic periodic paralysis].

Hypokalaemic periodic paralysis is an autosomal, dominantly inherited disorder, characterised by attacks of partial or total muscle weakness and serum hypokalaemia. the mutations responsible are located in the CACNA1S gene (type 1) and in the SCN4A gene (type 2), and are all missense mutations where arginine is mostly replaced by histidine or sometimes glycine. The mutation has been localised in the voltage sensor of the transmembrane segment of calcium channel (type 1) and sodium channel (type 2) respectively. How muscle weakness develops is not known. Oral or intravenous administration of potassium is helpful in treating partial or complete attacks. Acetazolamide can reduce the frequency of attacks in type-1 hypokalaemic periodic paralysis.

Acetazolamide↗

Do diabetologists recognise self-management problems in their patients?

OBJECTIVE: The aim of this study was to determine whether diabetologists recognise patients' needs for additional intensive multidisciplinary care due to glycaemic and diabetes-related psychosocial difficulties. RESEARCH DESIGN AND METHODS: We compared 114 participants in a diabetes intervention programme with 201 as yet non-referred outpatients, of whom 54 outpatients were considered eligible for the intervention by their diabetologists; thus, 147 outpatients were considered non-eligible. RESULTS: Analysis revealed that the eligible patients had poorer glycaemic control but all other parameters were similar to non-eligible patients. Significantly, 22 (15%) of the 147 non-eligible patients clearly had diabetes distress and could potentially benefit from the intervention. CONCLUSION: The results suggest that in regular care, patients' needs with respect to glycaemic control are recognised by their diabetologists, but patients with high psychosocial diabetes-related distress are often overlooked, though they also may be in need of additional care. Integrated monitoring of diabetes-related distress in outpatients could improve this area of diabetes care.

Adult↗

Outcome in patients with differentiated thyroid cancer with negative diagnostic whole-body scanning and detectable stimulated thyroglobulin.

BACKGROUND: Management of patients with differentiated thyroid carcinoma with negative diagnostic radioiodide scanning and increased serum thyroglobulin (Tg) concentrations is a widely debated problem. High-dose iodine-131 treatment of patients who have a negative (131)I diagnostic whole-body scan (WBS) is advocated. However, the therapeutic benefit of this "blind" treatment is not clear. OBJECTIVE: To investigate the course of serum Tg during thyroid hormone suppression therapy (Tg-on) and clinical outcome in patients with negative diagnostic (131)I scanning and increased serum Tg concentrations during thyroid hormone withdrawal (Tg-off), after treatment with high-dose (131)I. DESIGN: Retrospective single-center study. METHODS: Fifty-six patients were treated with a blind therapeutic dose of 150 mCi (131)I. Median follow-up from this treatment until the end of observation was 4.2 Years (range 0.5-13.5 Years). RESULTS: The post-treatment WBS revealed (131)I uptake in 28 patients, but none in the remaining 28 patients. In this study the Tg-on values did not change after treatment in either the positive or the negative post-treatment WBS group. During follow-up, 18 of the 28 patients with a positive post-treatment WBS achieved complete remission, compared with 10 of the 28 patients with a negative post-treatment WBS. Nine patients in the negative group died, but no patients died in the positive post-treatment group (P=0.001). CONCLUSIONS: High-dose iodine treatment in diagnostically negative patients who have a negative post-treatment scan seems to confer no additional value for tumor reduction and survival. In patients with a positive post-treatment scan, high-dose iodine treatment can be used as a diagnostic tool to identify tumor location, and a therapeutic effect may be present in individual cases.

Adenoma, Oxyphilic↗

Symptom scoring systems to diagnose distal polyneuropathy in diabetes: the Diabetic Neuropathy Symptom score.

AIMS: To provide one of the diagnostic categories for distal diabetic polyneuropathy,several symptom scoring systems are available, which are often extensive and lack in validation. We validated a new four-item Diabetic Neuropathy Symptom (DNS) score for diagnosing distal diabetic polyneuropathy. METHODS: We compared score characteristics of the generally accepted Neuropathy Symptom Score (NSS) with the DNS score, and tested construct validity,predictive value and reproducibility with the Diabetic Neuropathy Examination score, Semmes-Weinstein monofilaments and Vibration Perception Threshold(clinical standards) in 73 patients with diabetes (24 Type 1, 49 Type 2;43 male/30 female; mean age 57 years (19-90);mean diabetes duration 15 years (1-43)). RESULTS: Correlation between NSS and DNS score was high (Spearman r= 0.88). Patient scores were more differentiated on the DNS score. The relation of the NSS and DNS scores, respectively, with clinical standards was good (Spearman r= 0.21-0.60). Reproducibility of the DNS score was high (Cohen weighted kappa 0.78-0.95). The DNS score was easier to perform in clinical practice. CONCLUSIONS: The DNS is validated, fast and easy to perform, with a high predictive value when screening for diabetic polyneuropathy.

Adult↗

Quality of life in patients with diabetic foot ulcers.

PURPOSE: To compare Quality of Life (QoL) between diabetic patients with (former or present) and without foot ulcers. METHODS: Two patient groups of comparable age, sex distribution, type distribution and duration of diabetes were studied. Fourteen patients with former or present, but clinically stable diabetic foot ulcers (DFUs) were studied. The control group was 24 unknown patients with DFUs. None of the participants had other diabetic complications or conditions that would potentially affect QoL. A diabetic foot risk score and QoL were assessed. QoL was scored with the RAND-36, the Barthel Score (ADL) and the Walking and Walking Stairs Questionnaire (WSQ). RESULTS: Marked and significant differences were found in physical functioning (p < 0.001), social functioning (p < 0.05), physical role (p < 0.001) and health experience (p < 0.05) between the two groups with the RAND-36 and the four subscales of the WSQ (all p < 0.001). On all these scales, QoL was significantly poorer in the study group. A correlation was found between the risk scores and QoL (physical functioning and physical role Spearman's r: -0.66, -0.56 and WSQ -0.63, -0.64, -0.67 and 0.71, respectively). CONCLUSION: Presence or history of DFUs has a large impact on physical role, physical functioning and mobility. Physical impairments especially influenced QoL. Probably, QoL can be increased by providing attention that will enhance mobility and by giving advice about adaptations and special equipment.

Aged↗

[Muscle weakness or rigidity due to hereditary ion channel diseases].

Three men, aged 36, 16 and 66 years, had suffered for several years from muscular weakness; after a low serum potassium level had been established, supplementary examination revealed hypokalaemic periodic paralysis. A woman aged 25 had suffered since youth from muscular stiffness on sudden movements; she suffered from hereditary myotonia of the recessive type (Becker's disease). Both rare skeletal muscle ion channel diseases are characterized by the fact that the variable clinical expression complicates making the diagnosis. Since the causal mutations are known, genetic analysis is an essential step in confirming the diagnosis. Additional EMG procedures may be of diagnostic value, even in cases that cannot be clarified genetically.

Adolescent↗