Surgical Treatment for Atlanto-Occipital Subluxation due to Destructive Spondyloarthropathy in a Patient Undergoing Long-Term Hemodialysis

Destructive spondyloarthropathy (DSA) has been reported in patients undergoing long-term hemodialysis. Cervical spinal lesions, including those of the upper cervical spine, are reported to be some of the most common. To our knowledge, we report for the first time, a case of atlanto-occipital subluxation requiring surgical treatment due to severe myelopathy and nuchal pain in a patient undergoing long-term hemodialysis. The patient was a 66-year-old woman who had undergone hemodialysis for 40 years. She visited our hospital due to an acute progression of gait disturbance and severe nuchal pain. Computed tomography showed posterior subluxation of the atlanto-occipital joints. DSA was also observed in the lower cervical spine. Magnetic resonance imaging showed spinal canal stenosis at both the upper and lower cervical levels. We performed Oc-C7 fixation, C1 laminectomy, and C3-C7 laminoplasty. We first recognized that the atlanto-occipital subluxation was caused by the extremely long-term, in this case, 40 years, hemodialysis

Differential Diagnosis of Tumoral Lesions in the Spinal Canal in Patients Undergoing Hemodialysis

Study DesignA retrospective study.PurposeTo clarify the features useful for the differential diagnosis of spinal canal tumoral lesions in patients undergoing hemodialysis.Overview of LiteratureTumoral lesions in the spinal canal are rarely found in hemodialysis patients. Therefore, the differential diagnosis of tumoral lesions in the spinal canal in hemodialysis patients has been very difficult.MethodsSpinal canal tumors in 17 patients undergoing hemodialysis or continuous ambulatory peritoneal dialysis were investigated. Histopathological analysis was conducted for all specimens obtained during surgery. The tumoral lesions were categorized into 3 groups on the basis of histopathology: spinal cord tumor, amyloidoma, and other. Patient medical history and diagnostic images of each group were reviewed.ResultsEight of 17 cases were histopathologically diagnosed as spinal cord tumors and were neurinomas, 6 were amyloidomas, and 3 were classified as other. The rate of spinal cord tumors was 47.1% (8 of 17 cases), which revealed the most frequent lesion type. The rate of amyloidomas and other types was 35.3% (6 of 17 cases) and 17.6% (3 of 17cases), respectively. In the amyloidoma group, the mean duration of hemodialysis (24.3 years) was longer than that of spinal cord tumors and other types (9.2 years and 8.6 years, respectively). All spinal cord tumors were intradural extramedullary, whereas all amyloidomas and other types were extradural.ConclusionsThe rate of each tumoral lesion, the duration of hemodialysis, and the tumoral localization are important features for the differential diagnosis of tumoral lesions in the spinal canal in hemodialysis patients

Surgical Outcome for Hemodialysis-Related Upper Cervical Lesions

Study DesignA retrospective study.PurposeTo investigate the surgical outcome for hemodialysis-related upper cervical lesions.Overview of LiteratureSurgical outcome of lower cervical lesions in patients undergoing hemodialysis has been reported. However, surgical outcome for upper cervical lesions in hemodialysis patients is unclear.MethodsUpper cervical lesions in nine patients undergoing hemodialysis were surgically treated. Mean age at surgery was 61.6 years (range, 52-68 years), and the mean follow-up period was 45.4 months (range, 2-98 months). Patients had undergone hemodialysis for an average of 25.3 years (range, 16-40 years) at surgery. Seven patients with destructive spondyloarthropathy (DSA) of the upper cervical spine were treated with atlantoaxial or occipitocervical fixation. Two patients with retro-odontoid pseudotumors were treated with C1 posterior arch resection alone. Japanese Orthopedic Association (JOA) scores for cervical myelopathy, postoperative complications, postoperative radiography, and preoperative and postoperative occipital pain were evaluated.ResultsMean preoperative and postoperative JOA score was 3.7 and 8.1, respectively. The seven patients with DSA had severe preoperative occipital pain that disappeared postoperatively. Postoperative radiography showed solid bone union in DSA cases and no instability in pseudotumor cases.ConclusionsSatisfactory surgical outcome was observed for hemodialysis-related upper cervical lesions

Effect of thyroid statuses on sodium/iodide symporter (NIS) gene expression in the extrathyroidal tissues in mice

<p>Abstract</p> <p>Background</p> <p>Iodide that is essential for thyroid hormone synthesis is actively transported into the thyroid follicular cells via sodium/iodide symporter (NIS) protein in vertebrates. It is well known that NIS expression in thyroid is regulated by the thyroid statuses mainly through thyroid stimulating hormone (TSH). Although <it>NIS </it>mRNA expressions in extrathyroidal tissues have been qualitatively reported, their regulation by thyroid statuses has not been well clarified.</p> <p>Methods</p> <p>Male ICR mice aged four weeks were assigned into three groups (control, hypothyroid, and hyperthyroid). Hypothyroid group of mice were treated with 0.02% methimazole in drinking water and hyperthyroid group of mice received intraperitoneal injection (4 μg _L-T₄twice a week) for four weeks. <it>NIS </it>mRNA expression levels in the tissues were evaluated using Northern blot hybridization and quantitative real-time RTPCR (qPCR). Additionally, end-point RTPCR for the thyroid follicular cell-characteristic genes (TSH receptor, <it>TSHR</it>; thyroid transcription factor-1, <it>TTF1</it>; and paired box gene 8, <it>Pax8</it>) was carried out.</p> <p>Results</p> <p>By Northern blot analysis, <it>NIS </it>mRNA was detected in thyroid and stomach. In addition to these organs, qPCR revealed the expression also in the submandibular gland, colon, testis, and lung. Expression of <it>NIS </it>mRNA in thyroid was significantly increased in hypothyroid and decreased in hyperthyroid group. Trends of <it>NIS </it>mRNA expression in extrathyroidal tissues were not in line with that in the thyroid gland in different thyroid statuses. Only in lung, <it>NIS </it>mRNA was regulated by thyroid statuses but in opposite way compared to the manner in the thyroid gland. There were no extrathyroidal tissues that expressed all three characteristic genes of thyroid follicular cells.</p> <p>Conclusions</p> <p><it>NIS </it>mRNA expression in the thyroid gland was up-regulated in hypothyroid mice and was down-regulated in hyperthyroid mice, suggesting that <it>NIS </it>mRNA in the thyroid gland is regulated by thyroid statuses. In contrast, <it>NIS </it>mRNA expression in extrathyroidal tissues was not altered by thyroid statuses although it was widely expressed. Lack of responsiveness of <it>NIS </it>mRNA expressions in extrathyroidal tissues reemphasizes additional functions of NIS protein in extrathyroidal tissues other than iodide trapping.</p

Pycnodysostosis with Multi-Segmental Spinal Canal Stenosis due to Ossification of the Yellow Ligament

Pycnodysostosis is an autosomal recessive disorder characterized by osteosclerosis, small stature, acro-osteolysis of the distal phalanges, loss of the mandibular angle, separated cranial sutures with open fontanels, and frequent fractures. One identified cause of the disease is reduced activity of the cysteine protease cathepsin K. A 48-year-old woman with a history of frequent fractures presented with a severe gait disturbance. Radiography, computed tomography, magnetic resonance imaging, and gene analysis were performed. Physical examination revealed open fontanels, and radiographs showed increased bone density. DNA sequence analysis revealed a deletion mutation of the cathepsin K gene. We diagnosed pycnodysostosis based on these findings. The magnetic resonance and computed tomography images demonstrated multilevel spinal canal stenosis due to ossification of the yellow ligament. We performed a laminectomy, and the patient's neurological signs and symptoms improved. To our knowledge, this is the first case of pycnodysostosis with ossification of the yellow ligament

Inactivation of the Rcan2 Gene in Mice Ameliorates the Age- and Diet-Induced Obesity by Causing a Reduction in Food Intake

Obesity is a serious international health problem that increases the risk of several diet-related chronic diseases. The genetic factors predisposing to obesity are little understood. Rcan2 was originally identified as a thyroid hormone-responsive gene. In the mouse, two splicing variants that harbor distinct tissue-specific expression patterns have been identified: Rcan2-3 is expressed predominately in the brain, whereas Rcan2-1 is expressed in the brain and other tissues such as the heart and skeletal muscle. Here, we show that Rcan2 plays an important role in the development of age- and diet-induced obesity. We found that although the loss of Rcan2 function in mice slowed growth in the first few weeks after birth, it also significantly ameliorated age- and diet-induced obesity in the mice by causing a reduction in food intake rather than increased energy expenditure. Rcan2 expression was most prominent in the ventromedial, dorsomedial and paraventricular hypothalamic nuclei governing energy balance. Fasting and refeeding experiment showed that only Rcan2-3 mRNA expression is up-regulated in the hypothalamus by fasting, and loss of Rcan2 significantly attenuates the hyperphagic response to starvation. Using double-mutant (Lepob/ob Rcan2−/−) mice, we were also able to demonstrate that Rcan2 and leptin regulate body weight through different pathways. Our findings indicate that there may be an Rcan2-dependent mechanism which regulates food intake and promotes weight gain through a leptin-independent pathway. This study provides novel information on the control of body weight in mice and should improve our understanding of the mechanisms of obesity in humans