- 著者
- Mika Kawagoe Kohei Odajima Shinichiro Asakawa Michito Nagura Shigeyuki Arai Osamu Yamazaki Yoshifuru Tamura Ryuji Ohashi Takamasa Iwakura Hideo Yasuda Shigeru Shibata Yoshihide Fujigaki
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- pp.1251-22, (Released:2023-03-22)
- 参考文献数
- 27
Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren's syndrome. The patient exhibited various other immune abnormalities. Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of SLICC 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.
- 著者
- Yukitoshi Sakao Takeshi Sugiura Takayuki Tsuji Naro Ohashi Hideo Yasuda Yoshihide Fujigaki Akihiko Kato
- 出版者
- 一般社団法人 日本内科学会
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.53, no.14, pp.1485-1490, 2014 (Released:2014-07-15)
- 参考文献数
- 34
- 被引用文献数
- 1 13
Objective The goal of this study was to clarify the clinical manifestation of hypercalcemia due to hypoadrenalism in hemodialysis (HD) patients. Methods We retrospectively analyzed the clinical characteristics of five HD patients who had presented with hypercalcemia due to adrenal insufficiency (age: 69±7 [58-75] years old, time on HD: 13±11 [2-32] years). We conducted corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) stimulation tests. We also examined serum bone turnover markers before and after glucocorticoid replacement. Results All patients had critical illnesses at the onset of hypercalcemia. They had at least one symptom, such as eosinophilia, hypoglycemia, or fever. The prevalence of hypercalcemia due to adrenal insufficiency was 1.3% in maintenance HD patients on admission. The causes of adrenal insufficiency were isolated ACTH deficiency, pituitary apoplexy, pituitary atrophy, glucocorticoid withdrawal syndrome, and unilateral adrenalectomy. Serum calcium (Ca) levels corrected by serum albumin were maximally increased to 12.9 to 14.3 mg/dL in four anuric HD patients and mildly elevated to 10.4 mg/dL in a patient with residual diuresis. Their basal serum cortisol levels ranged from <1.0 to 15.4 μg/dL. Single CRH injections failed to increase serum cortisol in any of the patients. Glucocorticoid replacement acutely normalized serum Ca and decreased levels of carboxy-terminal telopeptide of type I collagen, a marker of bone resorption. Conclusion Adrenal insufficiency could therefore be an occult cause of hypercalcemia in anuric HD patients who are critically ill.