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指定難病 — No.72

下垂体性ADH分泌異常症

検索語 Pituitary ADH Secretion Disorder ・ 最終更新 2026-09-17 13:34 ・ 最新に更新

Data Sheet
指定 No.72
Src PubMed · CT.gov · jRCT

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( 01 )EVIDENCE / PUBMED · 4件

世界の論文

直近の研究を、やさしい日本語で

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不明
MK-01 · PMID 42704014

[From syndrome of inappropriate antidiuretic hormone secretion deficiency to excess - Challenges and solutions in a unique case]

Abstract / 原文

INTRODUCTION: the sequential transition from central diabetes insipidus (CDI) to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) is an exceptionally rare condition and represents a major diagnostic and therapeutic challenge. CASE REPORT: we report the case of a 47-year-old man with Burkitt lymphoma involving the hypothalamic-pituitary region who initially presented with polyuria, polydipsia, and hypernatremia. CDI was confirmed by biochemical findings and a positive response to desmopressin. Following desmopressin treatment and intensive chemotherapy, rapid tumor regression and partial recovery of neurohypophyseal function were observed. During follow-up, the patient developed persistent euvolemic hyponatremia with low plasma osmolality, inappropriately concentrated urine, and elevated urinary sodium. After excluding adrenal insufficiency, hypothyroidism, desmopressin overtreatment, and other secondary causes, SIADH was diagnose. DISCUSSION: this case highlights the rare sequential transition from ADH deficiency to ADH excess caused by the same hypothalamic-pituitary disease and supports oral urea as an effective, safe, and cost-effective therapeutic option for chronic SIADH.

Journal
Nutricion hospitalaria(2026 Sep)
Authors
4名
Type
English Abstract, Journal Article
PubMedで原文を見る
観察研究
MK-02 · PMID 42274282

Neuroendocrine regulation of female fertility: the role of CNS-derived hormones

Abstract / 原文

Infertility is an increasing concern for many women and can affect both physical and emotional well-being. The central nervous system (CNS) - particularly the hypothalamus, pituitary gland, and pineal gland - plays a crucial role in female reproductive health. We conducted a narrative review of relevant studies published between 2015 and 2025, sourcing data from PubMed and Scopus. Our goal was to investigate how dysregulation of hormones from the hypothalamus, pituitary gland, and pineal gland contributes to fertility-related disorders, such as impairments in ovulation, oocyte quality, and embryo development. Both human and significant animal studies were considered to better understand how CNS hormones affect fertility. The findings emphasize the roles of key hormones, including gonadotropin-releasing hormone (GnRH), luteinizing hormone (LH), follicle-stimulating hormone (FSH), melatonin, adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH), and vasopressin (ADH). The proper timing and balance of these hormones are vital. For instance, GnRH pulses regulate the release of LH and FSH, which are essential for ovulation and follicle development. Melatonin supports oocyte health and helps maintain the menstrual cycle, while ACTH and TSH are also involved in reproductive function. Vasopressin affects uterine activity and hormone production. Understanding these hormonal interactions may lead to better diagnostic tools and more effective treatment strategies for conditions such as polycystic ovary syndrome (PCOS) and other fertility-related disorders. Unlike previous studies, this research provides a comprehensive investigation of how CNS-mediated hormonal regulation influences female reproductive outcomes, examining the roles of all involved hormones.

Journal
Journal of molecular endocrinology(2026 Jul)
Authors
5名
Type
Journal Article, Review
PubMedで原文を見る
観察研究
MK-03 · PMID 42088331

Consensus Guidelines on Inferior Petrosal Sinus Sampling: A Guideline From the Society of Vascular and Interventional Neurology Guidelines and Practice Standards Committee

Abstract / 原文

BACKGROUND: Bilateral inferior petrosal sinus sampling (BIPSS) is the gold-standard test to distinguish Cushing disease (CD) from ectopic adrenocorticotropic hormone (ACTH) secretion when biochemistry confirms ACTH-dependent Cushing syndrome and pituitary magnetic resonance imaging is equivocal. However, practice varies widely between centers, leading to false negatives, misinterpretation, sampling errors, and avoidable risk. Neurointerventionalists performing BIPSS have lacked dedicated, procedure-focused guidance. METHODS: Using the Society of Vascular and Interventional Neurology Guidelines and Practice Standards framework, a multidisciplinary panel (endocrinology, interventional neuroradiology, and endovascular neurosurgery) performed a systematic review through August 2025 and developed recommendations graded by class of recommendation and level of evidence through a modified Delphi process. RESULTS: The guideline standardizes (1) indications for BIPSS in biochemically confirmed ACTH-dependent Cushing with normal, equivocal, or <6 mm pituitary lesions; (2) technical approach, including bilateral IPS catheterization, heparinization, and stimulation with corticotropin-releasing hormone or desmopressin; (3) sampling protocol with 2 prestimulation draws and timed collections at 3, 5, 10, and 15 minutes; (4) lab handling, emphasizing prechilled EDTA tubes, rapid ACTH processing, and prolactin measurement as an internal control of venous effluent; and (5) interpretation, using ACTH inferior petrosal sinus to peripheral ratio cutoffs (≥2 prestimulation, ≥3 poststimulation) and techniques to identify potential false negatives. The document also defines operator/institutional competency benchmarks and quality-assurance metrics. CONCLUSIONS: When executed with standardized technique and interpretation, BIPSS offers very high sensitivity and specificity for localizing ACTH pathology. These consensus guidelines provide a practical playbook for neurointerventionalists to perform BIPSS safely, reproducibly, and with maximal diagnostic yield.

Journal
Stroke (Hoboken, N.J.)(2026 May)
Authors
14名
Type
Journal Article, Review
PubMedで原文を見る
症例報告
MK-04 · PMID 41994839

Early-Onset Hyponatremia Presenting as Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) Post-transsphenoidal Pituitary Resection

Abstract / 原文

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a disorder in which an excessive amount of antidiuretic hormone (ADH) is released, leading to water retention and subsequent hyponatremia. Delayed hyponatremia, typically beginning on postoperative day (POD) 4, is a well-recognized complication following transsphenoidal resection of pituitary tumors and is thought to result from excess release of stored ADH due to surgical trauma. In this rare case of early symptomatic hyponatremia secondary to SIADH, a 61-year-old postmenopausal woman with no significant past medical history developed symptomatic hyponatremia on POD 1 after elective transsphenoidal resection of a nonfunctioning pituitary neuroendocrine tumor. Her serum sodium reached a nadir of 126 mmol/L on POD 2. Urine osmolality was 961 mOsm/kg on POD 1, consistent with SIADH. She was treated with fluid restriction, intravenous 3% hypertonic saline, and salt tablets, with improvement in serum sodium prior to discharge on POD 4 on fluid restriction and salt tablets. This case highlights the importance of early clinical vigilance for hyponatremia following pituitary resection, as symptomatic hyponatremia can rarely occur in the early postoperative period.

Journal
Cureus(2026 Mar)
Authors
5名
Type
Case Reports, Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

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jRCT で検索日本の臨床研究実施計画 公開システム「対象疾患名」に 下垂体性ADH分泌異常症 を入力し、「募集状況」で 募集中 にチェックして検索します。ClinicalTrials.gov で全件を見る世界最大の治験データベース(英語)「下垂体性ADH分泌異常症・日本・募集中」の条件で一覧が開きます。

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