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Introduction: The 2015 American Thyroid Association guidelines recommend to de-escalate treatment such as Thyroid lobectomy instead of total thyroidectomy for 1–4 cm papillary thyroid cancer (PTC). Dutch guidelines endorse restricted work-up for thyroid incidentalomas recommending only fine needle aspiration in case of a 'palpable thyroid nodule'. This diagnostic work-up algorithm may result in the identification of less indolent PTCs and may lead to a patient population with relatively more aggressive PTCs. This study aims to retrospectively analyze recurrence rates of low-risk 1–4 cm PTC in the Netherlands. Methods: From the national cancer registry, patients with low-risk 1–4 cm PTC between 2005 and 2015 were included for analysis. Disease free survival (DFS) and overall survival were compared between patients who underwent TT ± RAI and TL without RAI. Post-hoc propensity score analysis was performed correcting for age, sex, T-stage, and N-stage. Results: In total 901 patients were included, of which 711 (78.9%) were females, with a median follow-up of 7.7 years. TT was performed in 893 (94.8%) patients. Recurrence occurred in 23 (2.6%) patients. Multivariable analysis showed no significant correlation between extent of surgery and DFS (p = 0.978), or overall survival (p = 0.590). After propensity score matching, multivariable analysis showed no significant difference on extent of surgery and recurrence. Conclusion: Low-risk PTC patients with 1–4 cm tumor who underwent TL showed similar recurrence rates as those who underwent TT ± adjuvant RAI, which suggests that TL can be sufficient in treating low-risk 1–4 cm PTC, possibly reducing morbidity of these patients in the Netherlands. [ABSTRACT FROM AUTHOR]

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors that are hereditary in up to 50% of patients. The gene encoding transmembrane‐protein‐127 (TMEM127) is one of the PCC/PGL‐susceptibility genes with an autosomal dominant inheritance pattern. Here, we report 2 patients with bilateral PCC who both harbored a homozygous TMEM127‐mutation. In a 31‐year‐old mentally retarded patient, the homozygous c.410‐2A > G mutation was discovered during an update of DNA analysis. A 26‐year‐old mentally retarded patient was found to have a homozygous c.3G > A mutation. The parents of both patients were consanguineous. We reviewed previously reported clinical features of TMEM127 mutation carriers and compared our findings with case descriptions of homozygous mutations in other PGL/PCC‐susceptibility genes. Homozygosity for an autosomal dominant inherited disorder is an extremely rare phenomenon and has, to our knowledge, not been reported before for the gene encoding TMEM127. In the present cases, the clinical picture does not seem to be very different from heterozygous TMEM127 mutation carriers, except for a relatively large tumor size and more pronounced plasma metanephrine concentration. It is unclear whether the mental retardation is causally related to homozygosity of the TMEM127 mutations. Updating genetic screening in patients in whom PCC/PGL has been diagnosed in the past should be considered as it might provide clinically relevant information. [ABSTRACT FROM AUTHOR]

Aims To investigate: (1) the willingness of patients with diabetes to participate in a screening programme; (2) the extent to which patients with diabetes who screen positive endorse need for psychosocial care; (3) the rate of referral to psychosocial care during screening vs. usual care. Methods Four hundred and ninety-nine patients with diabetes were invited to complete the Center for Epidemiologic Studies Depression and the Problem Areas in Diabetes questionnaires. Patients screening positive on either instrument were invited for an interview. One year after screening was withdrawn, rates of referral to psychosocial care were assessed from physician reports of patient referrals. Results In total, 349/499 (70%) patients with diabetes completed the questionnaire. Patients who did not take up the screening were younger, smoked more often and had higher HbA1c values. 'No-shows' for clinical appointments accounted for 74% of non-participation. Of the 104 (30% of 349) patients screening positive, 45 accepted an invitation for an interview. Finally, 36/104 (35%) would like a referral for psychological care. Seven per cent of patients were referred to psychological care during screening compared with 1% when screening was withdrawn. Conclusions Results raise questions as to whether screening is the most efficient way to identify patients with psychological problems. Many patients did not take up the screening, especially those with low adherence to diabetes care in general. Furthermore, few patients screening positive wanted to be referred. Screening should be evaluated in the context of consideration of alternative ways to identify at-risk patients, including providing resources to deal with patients with already known adjustment and adherence problems. [ABSTRACT FROM AUTHOR]

To assess the prognostic value of detectable thyroglobulin (Tg) after initial surgery and radioactive iodine (I) therapy by comparing patients with a negative post-therapeutic whole body scan (WBS) with either detectable or undetectable Tg. Differentiated thyroid cancer has a good prognosis. However, recurrences can occur up to 30 years after initial treatment. Because life-long follow-up is necessary, it is important to explore possible risk factors associated with recurrence and mortality. We studied 539 patients who were treated between 1980 and 2007. After the last therapeutic dosage of 5550 MBq I, 72 patients had negative post-therapeutic WBS and positive Tg levels (Tg+ group) and 399 patients had negative post-therapeutic WBS and negative Tg (Tg- group). The 68 remaining patients had proven residual macroscopic disease. We investigated recurrences and overall mortality in the Tg+ and Tg- group compared with the Dutch population. In the Tg+ group, detectable recurrences occurred significantly earlier and more frequently than in the Tg- group (19% vs 13%, P = 0·024). Survival between these groups was comparable, but shorter than the general Dutch population [Standardised Mortality Rate (SMR) 1·38 (95% CI 1·12;1·63) ( P = 0·003)]. Disease-free survival in the Tg groups was comparable and not significantly different from the Dutch population [SMR = 1·09 (95% CI 0·81;1·34) ( P = 0·569)]. Patients with detectable Tg during the last I treatment and a negative post-therapeutic WBS have significant earlier and more recurrences than patients without detectable Tg. Survival in both groups is comparable. After initial therapy, the combination of a negative high dose post-therapeutic WBS with detectable Tg is a valuable predictor for earlier and more recurrences, but is not associated with survival. [ABSTRACT FROM AUTHOR]

Background Somatostatin analogues are administered to control hormone hypersecretion in acromegaly and carcinoid patients. Somatostatin analogues can increase fat in the stools, which can lead to loss of fat-soluble vitamins. The effect of long-term somatostatin analogue use on vitamin levels remains unknown. Aim To investigate the prevalence of fat-soluble vitamin deficiencies in longterm somatostatin analogue users. Methods All acromegaly and carcinoid patients using somatostatin analogues for ≥ 18 months visiting the University Medical Center Groningen between December 2008 and April 2009 were eligible. Vitamin levels of fat-soluble vitamins in blood, clinical and vitamin-dependent laboratory parameters were collected. Results In all, 19 acromegaly and 35 carcinoid patients were included. Twelve patients experienced steatorrhoea; two carcinoid patients experienced night blindness. Forty-two (78%) were deficient for one or more vitamins, and 32% (n = 17) had multiple deficiencies. Deficiencies for vitamin A, D, E, K1 and E in erythrocytes occurred in 6%, 28%, 15%, 63% and 58% of the patients. Prevalence of vitamin D, E and K1 deficiencies was similar in both patient groups. Treatment duration did not influence vitamin levels. The length of intestinal resection and age correlated negatively with vitamin A levels. Conclusions Fat-soluble vitamin deficiencies are frequent during long-term somatostatin analogue treatment. Therefore, fat-soluble vitamins should be monitored in these patients. [ABSTRACT FROM AUTHOR]

The clinical management of patients with persistent or recurrent medullary thyroid carcinoma (MTC) is still under debate, because these patients either have a long-term survival, due to an indolent course of the disease, or develop rapidly progressing disease leading to death from distant metastases. At this moment, it cannot be predicted what will happen within most individual cases. Biomarkers, indicators which can be measured objectively, can be helpful in MTC diagnosis, molecular imaging and treatment, and/or identification of MTC progression. Several MTC biomarkers are already implemented in the daily management of MTC patients. More research is being aimed at the improvement of molecular imaging techniques and the development of molecular systemic therapies. Recent discoveries, like the prognostic value of plasma calcitonin and carcino-embryonic antigen doubling-time and the presence of somatic RET mutations in MTC tissue, may be useful tools in clinical decision making in the future. In this review, we provide an overview of different MTC biomarkers and their applications in the clinical management of MTC patients. [ABSTRACT FROM AUTHOR]

The current clinical diagnosis of Von Hippel-Lindau (VHL) disease demands at least one specific a sporadic VHL manifestation in a patient with familial VHL disease, or, in asporadic patient, at least two or more hemangioblastomas or a single hemangioblastoma in combination with a typical visceral lesion. To evaluate this definition, we studied the frequency of germline VHL mutation in three patients groups: (i) multi-organ involvement (classic VHL), (ii) limited VHL manifestations meeting criteria (non-classic VHL) and (iii) patients with VHL-associated tumors not meeting current diagnostic VHL criteria. In addition, we validated multiplex ligation-dependent probe amplification (MLPA) as a rapid and reliable quantitative method for the identification of germline VHL deletions. The frequency of germline VHL mutations was very high in classic VHL cases with multi-organ involvement (95%), lower in non-classic cases that meet current diagnostic criteria but have limited VHL manifestations or single-organ involvement (24%) and low (3.3%), but tangible in cases not meeting current diagnostic VHL criteria. The detection of germline VHL mutations in patients or families with limited VHL manifestations, or single-organ involvement is relevant for follow-up of probands and early identification of at-risk relatives. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.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.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≥ 8.0% and/or PAID scores≥ 40 would improve the NNT to achieve clinically relevant outcomes, while 76% of the patients matched these inclusion criteria.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. [ABSTRACT FROM AUTHOR]

Presents letters to the editor about endocrinology. Contribution of a kozak snp in the CD40 gene to graves' disease; Impact of DHEA levels in treated Cushing's disease on the quality of life; Significance of pre-operative medical therapy with rosiglitazone.

Abstract Aims To provide one of the diagnostic categories for distal diabetic polyneuro-pathy,several symptom scoring systems are available, which are often extensive andlack in validation. We validated a new four-item Diabetic Neuropathy Symptom (DNS) scorefor diagnosing distal diabetic polyneuropathy. Methods We compared score characteristics of the generally accepted NeuropathySymptom Score (NSS) with the DNS score, and tested construct validity,predictive value and reproducibility with the Diabetic Neuropathy Examinationscore, 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 scoreswere more differentiated on the DNS score. The relation of the NSS andDNS scores, respectively, with clinical standards was good (Spearman r = 0.21–0.60). Reproducibility ofthe DNS score was high (Cohen weighted κ 0.78–0.95). The DNSscore was easier to perform in clinical practice. Conclusions The DNS is validated, fast and easy to perform, with a high predictive valuewhen screening for diabetic polyneuropathy. Diabet. Med. 19, 962–965 (2002). [ABSTRACT FROM AUTHOR]

BACKGROUND The outcome for patients with bone metastases from differentiated thyroid carcinoma is worse compared to the overall prognosis of patients with differentiated thyroid carcinoma. The aim of this study is to evaluate the effect of embolization with concomitant radioiodine treatment on the serum thyroglobulin (Tg) level, pain and neurological symptoms in patients with large bone metastases from differentiated thyroid carcinoma. PATIENTS AND METHODS Five symptomatic patients, who presented with a large unresectable bone metastasis of differentiated thyroid carcinoma were treated with radioiodine and embolization. The effect of this combined treatment was compared to the effect of radioiodine without embolization in a previously treated control group of 6 patients. Serum Tg levels, pain and neurological symptoms were scored. Both groups were treated similarly with total thyroidectomy followed by ablation with 5.55 GBq 131I and a second dose of 5.55 GBq 131I three months later, except for embolization in the embolization group, which took place between the 2 radioiodine treatments. RESULTS In the embolization group, serum Tg at the second 131I therapy had decreased by 88.7% (median, range: 77.1–99.3%), which was significantly more compared to the decrease of serum Tg in the control group (18.6%, range: -4.7–95%, P < 0.05). CT-scanning showed a median volume reduction of the metastasis after radioiodine treatment combined with embolization of 52.5% (range: 39–80%). Both strategies resulted in a rapid relief of pain and neurological symptoms. Embolization was not accompanied with severe complications. CONCLUSIONS This preliminary study suggests that embolization of bone metastases of differentiated thyroid carcinoma in combination with radioiodine treatment results in a significant initial reduction of serum Tg level compared to radioiodine treatment alone. This suggests a beneficial reduction in tumour... [ABSTRACT FROM AUTHOR]

An abstract of the article "Skin Autofluorescence, a Noninvasive Measure of Advanced Glycation End Product Accumulation, Is a Predictor of Mortality in Hemodialysis Patients," by R. Meerwaldt, R. Graff, P. Links, J.W. Baynes, G.J. Navis, R.M. Huisman, R.O.B. Gans and A.J. SMIT is presented.

This study was conducted to analyse the effect of childhood-onset diabetes mellitus on adult height. The height at time of diagnosis of 35 children with insulin-dependent diabetes mellitus (IDDM) was compared with growth reference data. Predictions of the adult height were made at the time of diagnosis using the target height and the Tanner-Whitehouse II method. The adult height was compared with both the predicted values and the height of healthy adults. The height at time of diagnosis of the prepubertal children was increased compared with growth reference data, in contrast to pubertal children who had normal heights. Only the prepubertal boys were taller at time of diagnosis. The adult height of the prepubertal patients was taller than growth reference data. The mean adult height in all patients did not differ significantly from the predicted heights. In conclusion, the increased height at the start of IDDM in prepubertal children persists until adulthood.