During a period of 9 years, 71,500 patients underwent thyroid investigations at our department. Sixteen patients with thyroid hemiagenesis, 13 women and 3 men, were seen during this period. Fifteen had no left lobe and only 1 had no right lobe, the isthmus was present in 5 patients. Associated thyroid diseases of the lobe that was present could be observed in 11 patients (9 diffuse or nodular goiters, 2 thyroid autoimmune diseases). One patient was hyperthyroid and 7 were hypothyroid. Hypothyroidism associated with hemiagenesis has rarely been reported in the literature. In our survey, the high percentage of hypothyroidism may be explained by coexisting iodine deficiency, which could be verified in 4 hypothyroid patients. Ultrasonography is the key investigation to diagnose thyroid hemiagenesis. Fine-needle aspiration biopsies, laboratory tests, and scintigraphies are useful to diagnose other diseases within the remaining lobe or to visualize ectopic thyroid tissue. Review of the literature, including our cases, presented a total of 256 patients with thyroid hemiagenesis. Its prevalence can be estimated between 1:1900 and 1:2675. Left to right ratio of thyroid hemiagenesis is 3.6:1 with an isthmus present in 44%. The female-to-male ratio is 3:1; however, the larger number of females is probably based on a bias due to a female predominance of the populations investigated. On the basis of an equal distribution of both sexes, the female-to-male ratio of thyroid hemiagenesis would be only 1.3:1 in our survey.
A variety of indications for radiosynoviorthesis have been reported in literature, but the clinical outcome differs and depends on the primary disease and the pre-existing degenerative changes. This metaanalysis was carried out to establish groups for radiosynoviorthesis based on clinical outcome with respect to primary disease, clinical stage and the pre-existing degenerative changes. The literature search was carried out using the MEDLINE search term 'radionuclide synovectomy'. Based on reports in the literature we determined groups for radiosynoviorthesis for clinical use. Our literature list comprised 2190 joints that were treated with radiosynoviorthesis. The overall response rate for all treated joints was 72.5+/-17%. The mean improvement rate for the treated joints in rheumatoid arthritis was 66.7+/-15.4%. For osteoarthritis the success rate was 56+/-11%, with better results in case of minimal radiological changes. Radiosynoviorthesis in patients with changes according to Steinbrocker I and II was successful in 72.8+/-12.3%, and in 64+/-17.3%, respectively. Steinbrocker III and IV had a mean success rate of 52.4+/-23.6%. In the case of haemophilia and Willebrand's disease a reduction of joint bleedings and factor usages after radiosynoviorthesis was evident in 91+/-4.3%. In patients with pigmented villonodular synovitis radiosynoviorthesis was successful in 77.3+/-25.3%. It is concluded that radiosynoviorthesis provides better results in rheumatoid arthritis than in osteoarthritis. Minimal or moderate changes according to Steinbrocker stages I and II respond better to radionuclide therapy than do stages III and IV. Deformed or unstable joints might fail treatment and therefore surgical interventions should be considered. Close cooperation with orthopaedists and rheumatologists is necessary to consider radiosynoviorthesis in each patient to ensure optimal medical care.
Gaucher disease (GD) is a rare, genetic lysosomal disorder leading to lipid accumulation and dysfunction in multiple organs. Involvement of the skeleton is one of the most prevalent aspects of GD and a major cause of pain, disability, and reduced quality of life. Uniform recommendations for contemporary evaluation and management are needed. To develop practical clinical recommendations, an international group of experienced physicians conducted a comprehensive review of 20 years’ of the literature, defining terms according to pathophysiological understanding and pointing out best practice and unmet needs related to the skeletal features of this disorder. Abnormalities of bone modeling, reduced bone density, bone infarction, and plasma cell dyscrasias accompany the displacement of healthy adipocytes in adult marrow. Exposure to excess bioactive glycosphingolipids appears to affect hematopoiesis and the balance of osteoblast and osteoclast numbers and activity. Imbalance between bone formation and breakdown induces disordered trabecular and cortical bone modeling, cortical bone thinning, fragility fractures, and osteolytic lesions. Regular assessment of bone mineral density, marrow infiltration, the axial skeleton and searching for potential malignancy are recommended. MRI is valuable for monitoring skeletal involvement: It provides semiquantitative assessment of marrow infiltration and the degree of bone infarction. When MRI is not available, monitoring of painful acute bone crises and osteonecrosis by plain X‐ray has limited value. In adult patients, we recommend DXA of the lumbar spine and left and right hips, with careful protocols designed to exclude focal disease; serial follow‐up should be done using the same standardized instrument. Skeletal health may be improved by common measures, including adequate calcium and vitamin D and management of pain and orthopedic complications. Prompt initiation of specific therapy for GD is crucial to optimizing outcomes and preventing irreversible skeletal complications. Investing in safe, clinically useful, and better predictive methods for determining bone integrity and fracture risk remains a need. © 2019 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.
The aim of this study was to evaluate the usefulness of 18F-FDG PET in the diagnosis and staging of primary and recurrent malignant head and neck tumours in comparison with conventional imaging methods [including ultrasonography, radiography, computed tomography (CT) and magnetic resonance imaging (MRI)], physical examination, panendoscopy and biopsies in clinical routine. A total of 54 patients (13 female, 41 male, age 61.3+/-12 years) were investigated retrospectively. Three groups were formed. In group I, 18F-FDG PET was performed in 15 patients to detect unknown primary cancers. In group II, 24 studies were obtained for preoperative staging of proven head and neck cancer. In group III, 18F-FDG PET was used in 15 patients to monitor tumour recurrence after radiotherapy and/or chemotherapy. In all patients, imaging was obtained at 70 min after the intravenous administration of 180 MBq 18F-FDG. In 11 of the 15 patients in group I, the primary cancer could be found with 18F-FDG, yielding a detection rate of 73.3%. In 4 of the 15 patients, CT findings were also suggestive of the primary cancer but were nonetheless equivocal. In these patients, 18F-FDG showed increased 18F-FDG uptake by the primary tumour, which was confirmed by histology. One patient had recurrence of breast carcinoma that could not be detected with 18F-FDG PET, but was detected by CT. In three cases, the primary cancer could not be found with any imaging method. Among the 24 patients in group II investigated for staging purposes, 18F-FDG PET detected a total of 13 local and three distant lymph node metastases, whereas the conventional imaging methods detected only nine local and one distant lymph node metastases. The results of 18F-FDG PET led to an upstaging in 5/24 (20.8%) patients. The conventional imaging methods were false positive in 5/24 (20.8%). There was one false positive result using 18F-FDG PET. Among the 15 patients of group III with suspected recurrence after radiotherapy and/or chemotherapy, 18F-FDG was true positive in 7/15 (46.6%) and true negative in 4/15 (26.6%). The conventional imaging methods were true positive in 5/15 (33.3%) and true negative in 4/15 (26.6%). One false negative (6.6%) and three false positive findings (20%) on 18F-FDG PET were due to inflamed tissue. The conventional imaging methods were false positive in three (20%) and false negative in three cases (20%). It is concluded that in comparison to conventional diagnostic methods, 18F-FDG PET provides additional and clinically relevant information in the detection of primary and metastatic carcinomas as well as in the early detection of recurrent or persistent head and neck cancer after radiotherapy and/or chemotherapy. 18F-FDG PET should therefore be performed early in clinical routine, usually before CT or MRI.
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