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Adult hyperinsulinemic hypoglycemia not caused by an insulinoma: a report of two cases 总被引:1,自引:0,他引:1
van der Wal BC de Krijger RR de Herder WW Kwekkeboom DJ van der Ham F Bonjer HJ van Eijck CH 《Virchows Archiv : an international journal of pathology》2000,436(5):481-486
Nesidioblastosis is rare in adults and accounts for 0.5–5% of cases of organic hyperinsulinemia. The diagnosis of nesidioblastosis
should be considered when peroperative imaging modalities fail to localize a lesion in patients with hyperinsulinism. Two
female patients, aged 55 and 16 years, with hyperinsulinemic hypoglycemia are reported. Somatostatin receptor scintigraphy
showed slight focal activity in both patients. The first patient underwent a Whipple procedure and became diabetic. The second
patient underwent a distal hemi-pancreatectomy and suffered from recurrent hypoglycemic episodes 3 months after surgery, for
which she is presently being treated with octreotide. Histological examination of the resected pancreata revealed focally
increased islet tissue and a number of slightly hypertrophic beta cells. Such histological abnormalities have been related
to functional changes of β-cells. In infantile nesidioblastosis, a proportion of cases has been associated with mutations
in one of several genes. Whether such mutations, leading to hyperinsulinism, also play a role in adult nesidioblastosis is
presently unknown.
Received: 5 July 1999 / Accepted: 19 January 2000 相似文献
3.
Prednisolone does not affect direct‐acting antivirals against hepatitis C,but inhibits interferon‐alpha production by plasmacytoid dendritic cells
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A. S. W. Tjon T. Tha‐In H. J. Metselaar R. van Gent L. J. W. van der Laan Z. M. A. Groothuismink P. A. W. te Boekhorst P. M. van Hagen J. Kwekkeboom 《Clinical and experimental immunology》2013,173(2):259-267
Intravenous immunoglobulin (IVIg) is used to treat autoimmune and systemic inflammatory diseases caused by derailment of humoral and cellular immunity. In this study we investigated whether IVIg treatment can modulate regulatory T cells (Tregs) in humans in vivo. Blood was collected from IVIg-treated patients with immunodeficiency or autoimmune disease who were treated with low-dose (n = 12) or high-dose (n = 15) IVIg before, immediately after and at 7 days after treatment. Percentages and activation status of circulating CD4+CD25+forkhead box protein 3 (FoxP3+) Tregs and of conventional CD4+FoxP3− T-helper cells (Tconv) were measured. The suppressive capacity of Tregs purified from blood collected at the time-points indicated was determined in an ex-vivo assay. High-dose, but not low-dose, IVIg treatment enhanced the activation status of circulating Tregs, as shown by increased FoxP3 and human leucocyte antigen D-related (HLA-DR) expression, while numbers of circulating Tregs remained unchanged. The enhanced activation was sustained for at least 7 days after infusion, and the suppressive capacity of purified Tregs was increased from 41 to 70% at day 7 after IVIg treatment. The activation status of Tconv was not affected by IVIg. We conclude that high-dose IVIg treatment activates Tregs selectively and enhances their suppressive function in humans in vivo. This effect may be one of the mechanisms by which IVIg restores imbalanced immune homeostasis in patients with autoimmune and systemic inflammatory disorders. 相似文献
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Hendrik Bergsma Mark W. Konijnenberg Boen L. R. Kam Jaap J. M. Teunissen Peter P. Kooij Wouter W. de Herder Gaston J. H. Franssen Casper H. J. van Eijck Eric P. Krenning Dik J. Kwekkeboom 《European journal of nuclear medicine and molecular imaging》2016,43(3):453-463
Purpose
In peptide receptor radionuclide therapy (PRRT), the bone marrow (BM) is one of the dose-limiting organs. The accepted dose limit for BM is 2 Gy, adopted from 131I treatment. We investigated the incidence and duration of haematological toxicity and its risk factors in patients treated with PRRT with 177Lu-DOTA0-Tyr3-octreotate (177Lu-DOTATATE). Also, absorbed BM dose estimates were evaluated and compared with the accepted 2 Gy dose limit.Methods
The incidence and duration of grade 3 or 4 haematological toxicity (according to CTCAE v3.0) and risk factors were analysed. Mean BM dose per unit (gigabecquerels) of administered radioactivity was calculated and the correlations between doses to the BM and haematological risk factors were determined.Results
Haematological toxicity (grade 3/4) occurred in 34 (11 %) of 320 patients. In 15 of the 34 patients, this lasted more than 6 months or blood transfusions were required. Risk factors significantly associated with haematological toxicity were: poor renal function, white blood cell (WBC) count <4.0?×?109/l, age over 70 years, extensive tumour mass and high tumour uptake on the OctreoScan. Previous chemotherapy was not associated. The mean BM dose per administered activity in 23 evaluable patients was 67?±?7 mGy/GBq, resulting in a mean BM dose of 2 Gy in patients who received four cycles of 7.4 GBq 177Lu-DOTATATE. Significant correlations between (cumulative) BM dose and platelet and WBC counts were found in a selected group of patients.Conclusion
The incidence of subacute haematological toxicity after PRRT with 177Lu-DOTATATE is acceptable (11 %). Patients with impaired renal function, low WBC count, extensive tumour mass, high tumour uptake on the OctreoScan and/or advanced age are more likely to develop grade 3/4 haematological toxicity. The BM dose limit of 2 Gy, adopted from 131I, seems not to be valid for PRRT with 177Lu-DOTATATE.9.
Informal caregivers’ views on the division of responsibilities between themselves and professionals: A scoping review
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Yvette Wittenberg MSc Rick Kwekkeboom PhD Janneke Staaks MSc Arnoud Verhoeff PhD Alice de Boer PhD 《Health & social care in the community》2018,26(4):e460-e473
This scoping review focuses on the views of informal caregivers regarding the division of care responsibilities between citizens, governments and professionals and the question of to what extent professionals take these views into account during collaboration with them. In Europe, the normative discourse on informal care has changed. Retreating governments and decreasing residential care increase the need to enhance the collaboration between informal caregivers and professionals. Professionals are assumed to adequately address the needs and wishes of informal caregivers, but little is known about informal caregivers’ views on the division of care responsibilities. We performed a scoping review and searched for relevant studies published between 2000 and September 1, 2016 in seven databases. Thirteen papers were included, all published in Western countries. Most included papers described research with a qualitative research design. Based on the opinion of informal caregivers, we conclude that professionals do not seem to explicitly take into account the views of informal caregivers about the division of responsibilities during their collaboration with them. Roles of the informal caregivers and professionals are not always discussed and the division of responsibilities sometimes seems unclear. Acknowledging the role and expertise of informal caregivers seems to facilitate good collaboration, as well as attitudes such as professionals being open and honest, proactive and compassionate. Inflexible structures and services hinder good collaboration. Asking informal caregivers what their opinion is about the division of responsibilities could improve clarity about the care that is given by both informal caregivers and professionals and could improve their collaboration. Educational programs in social work, health and allied health professions should put more emphasis on this specific characteristic of collaboration. 相似文献
10.
Nasir A Gardner NM Strosberg J Ahmad N Choi J Malafa MP Coppola D Kwekkeboom DJ Teunissen JJ Kvols LK 《Pancreas》2008,36(3):309-313
We present an unusual case of a 52-year-old woman with severe, uncontrollable, refractory diarrhea attributable to pancreatic endocrine carcinoma (ECA) with markedly elevated serum vasoactive intestinal polypeptide (VIP) and calcitonin levels. After initial correction of fluid and electrolyte abnormalities, the patient was treated with high-dose octreotide. Shortly thereafter, due to the intractable nature of her diarrhea, she underwent cytoreductive hepatic surgery. The pancreatosplenectomy specimen showed a poorly differentiated ECA of the distal pancreas, immunoreactive for synaptophysin, CD56, and S100 protein, with morphologically similar hepatic and lymph node metastases. Postoperatively, her diarrhea improved, along with decline in serum VIP and calcitonin levels. Systemic chemotherapy with etoposide and cisplatin did not result in any radiographic and biochemical improvement. Having radiologically stable disease with depot-octreotide and short-acting octreotide (Sandostatin), she was subjected to peptide receptor radiotherapy with [177Lu-DOTA0,Tyr]octreotate (LuTate) that resulted in marked clinical and biochemical improvement, along with dramatic reduction in the number and size of hepatic metastases. In summary, this is a unique case of metastatic VIP- and calcitonin-secreting pancreatic ECA with dramatic sustained clinical, biochemical, and objective tumor response to peptide receptor radionuclide therapy. 相似文献