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BMJ Case Reports Jan 2021A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with...
A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with non-specific complaints of profound fatigue and 40-pound weight loss. He was seronegative for antinuclear antibodies and cytoplasmic antineutrophilic antibodies, but erythrocyte sedimentation rate and C reactive protein levels were elevated. Endocrinological testing revealed adrenal insufficiency, hypogonadism, hypothyroidism and diabetes insipidus. An MRI of the head revealed extensive sinonasal inflammation eroding through the floor of the sella turcica and into the pituitary gland and stalk. Biopsy of the sinonasal tissues was inconclusive. On review of his case, a multidisciplinary team diagnosed him with panhypopituitarism secondary to a recurrence of GPA. He responded well to glucocorticoids and methotrexate with marked reduction of pituitary enhancement on imaging and resolution of diabetes insipidus. He will require lifelong testosterone, levothyroxine and glucocorticoids for hormone replacement therapy.
Topics: Adrenal Insufficiency; Androgens; Diabetes Insipidus; Fatigue; Glucocorticoids; Granulomatosis with Polyangiitis; Hormone Replacement Therapy; Humans; Hypogonadism; Hypopituitarism; Hypothyroidism; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Methotrexate; Middle Aged; Pituitary Diseases; Pituitary Gland; Recurrence; Rhinitis; Sella Turcica; Sinusitis; Testosterone; Thyroxine; Weight Loss
PubMed: 33509867
DOI: 10.1136/bcr-2020-237774 -
Archives of Endocrinology and Metabolism Nov 2022" (Juliet, from by William Shakespeare). Shakespeare's implication is that a name is nothing but a word and it therefore represents a convention with no intrinsic...
" (Juliet, from by William Shakespeare). Shakespeare's implication is that a name is nothing but a word and it therefore represents a convention with no intrinsic meaning. Whilst this may be relevant to romantic literature, disease names do have real meanings, and consequences, in medicine. Hence, there must be a very good rational for changing the name of a disease that has a centuries-old historical context. A working group of representatives from national and international endocrinology and endocrine pediatric societies now proposes changing the name of "diabetes insipidus" to "Arginine Vasopressin Deficiency (AVP-D)" for central etiologies, and "Arginine Vasopressin Resistance (AVP-R)" for nephrogenic etiologies This editorial provides both the historical context and the rational for this proposed name change.
Topics: Humans; Child; Diabetes Insipidus; Arginine Vasopressin; Diabetes Mellitus
PubMed: 36219203
DOI: 10.20945/2359-3997000000528 -
Annals of the Academy of Medicine,... Feb 2021
Topics: Diabetes Insipidus; Diabetes Mellitus; Humans; Sodium-Glucose Transporter 2 Inhibitors
PubMed: 33733264
DOI: 10.47102/annals-acadmedsg.2020418 -
Neurology India 2020Patients with pituitary masses who undergo transsphenoidal resection are at risk for a number of medical complications postoperatively. Among these are disturbances in... (Review)
Review
Patients with pituitary masses who undergo transsphenoidal resection are at risk for a number of medical complications postoperatively. Among these are disturbances in fluid and sodium homeostasis, including diabetes insipidus (DI) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH). It is believed that these pathologic states are a result of damage to the hypothalamic-pituitary axis from surgery, as are the downstream consequences, such as the triple phase response. The triple-phase response describes the pattern of initial acute DI, subsequent rebound SIADH, and eventual chronic DI, the pathophysiology of which is described. Given the medical complexity of managing postoperative pituitary patients, it is essential to develop dedicated postoperative management protocols. Here, we describe the University of Utah's postoperative pituitary management protocol that includes immediate postoperative monitoring, treatment of DI, surveillance for the triple-phase response after discharge with outpatient serum sodium checks, and involvement of the endocrinology service for assistance with management of hypopituitarism. A complete understanding of the relevant anatomy, physiology, and development of standardized protocols for postoperative management can aid with minimizing medical complications after pituitary surgery.
Topics: Diabetes Insipidus; Disease Management; Humans; Inappropriate ADH Syndrome; Neurosurgical Procedures; Pituitary Gland; Pituitary Neoplasms; Postoperative Complications; Water-Electrolyte Balance
PubMed: 32611899
DOI: 10.4103/0028-3886.287679 -
Journal of the American Society of... Jul 2016To reduce lithium-induced nephrogenic diabetes insipidus (lithium-NDI), patients with bipolar disorder are treated with thiazide and amiloride, which are thought to... (Comparative Study)
Comparative Study
To reduce lithium-induced nephrogenic diabetes insipidus (lithium-NDI), patients with bipolar disorder are treated with thiazide and amiloride, which are thought to induce antidiuresis by a compensatory increase in prourine uptake in proximal tubules. However, thiazides induced antidiuresis and alkalinized the urine in lithium-NDI mice lacking the sodium-chloride cotransporter, suggesting that inhibition of carbonic anhydrases (CAs) confers the beneficial thiazide effect. Therefore, we tested the effect of the CA-specific blocker acetazolamide in lithium-NDI. In collecting duct (mpkCCD) cells, acetazolamide reduced the cellular lithium content and attenuated lithium-induced downregulation of aquaporin-2 through a mechanism different from that of amiloride. Treatment of lithium-NDI mice with acetazolamide or thiazide/amiloride induced similar antidiuresis and increased urine osmolality and aquaporin-2 abundance. Thiazide/amiloride-treated mice showed hyponatremia, hyperkalemia, hypercalcemia, metabolic acidosis, and increased serum lithium concentrations, adverse effects previously observed in patients but not in acetazolamide-treated mice in this study. Furthermore, acetazolamide treatment reduced inulin clearance and cortical expression of sodium/hydrogen exchanger 3 and attenuated the increased expression of urinary PGE2 observed in lithium-NDI mice. These results show that the antidiuresis with acetazolamide was partially caused by a tubular-glomerular feedback response and reduced GFR. The tubular-glomerular feedback response and/or direct effect on collecting duct principal or intercalated cells may underlie the reduced urinary PGE2 levels with acetazolamide, thereby contributing to the attenuation of lithium-NDI. In conclusion, CA activity contributes to lithium-NDI development, and acetazolamide attenuates lithium-NDI development in mice similar to thiazide/amiloride but with fewer adverse effects.
Topics: Acetazolamide; Amiloride; Animals; Aquaporin 2; Diabetes Insipidus, Nephrogenic; Diuretics; Female; Lithium Compounds; Mice; Mice, Inbred C57BL; Sodium Chloride Symporter Inhibitors
PubMed: 26574046
DOI: 10.1681/ASN.2015070796 -
Endocrine Journal Apr 2021This Review Article overviews the literature on diabetes insipidus (DI) associated with pregnancy and labor in Japan published from 1982 to 2019. The total number of... (Review)
Review
This Review Article overviews the literature on diabetes insipidus (DI) associated with pregnancy and labor in Japan published from 1982 to 2019. The total number of patients collected was 361, however, only one-third of these cases had detailed pathophysiologic information enabling us to identify the respective etiology and subtype. Pregnancy-associated DI can be divided into 3 etiologies, central (neurogenic) DI, nephrogenic DI, and excess vasopressinase-associated DI. Neurogenic DI has various causes: for example, DI associated with tumoral lesions in the pituitary and neighboring area, DI associated with Sheehan's syndrome and/or pituitary apoplexy, and DI associated with lymphocytic infundibuloneurohypophysitis (LINH, stalkitis). Nephrogenic DI results from defective response of the kidney to normal levels of vasopressin. However, the most interesting causal factor of pregnancy-associated DI is excess vasopressinase, caused either by excess production of vasopressinase by the placenta or defective clearance of vasopressinase by the liver. Hepatic complications resulting in pregnancy-associated DI include acute fatty liver of pregnancy (AFLP) and HELLP syndrome (syndrome of hemolysis, elevated liver enzymes, low platelets), as well as pre-existing or co-incidental hepatic diseases. A possible role of glucose uptake in putative stress-induced DI and the importance of correct diagnosis and treatment of pregnancy-associated DI, including use of 1-deamino 8-D arginine vasopressin, are also discussed.
Topics: Adult; Cystinyl Aminopeptidase; Diabetes Insipidus; Female; Humans; Japan; Pregnancy
PubMed: 33775975
DOI: 10.1507/endocrj.EJ20-0745 -
International Journal of Environmental... Jan 2022Wolfram syndrome 1 is a rare, autosomal recessive, neurodegenerative, progressive disorder. Insulin-dependent, non-autoimmune diabetes mellitus and bilateral progressive...
Wolfram syndrome 1 is a rare, autosomal recessive, neurodegenerative, progressive disorder. Insulin-dependent, non-autoimmune diabetes mellitus and bilateral progressive optic atrophy are both sensitive and specific criteria for clinical diagnosis. The leading cause of death is central respiratory failure resulting from brainstem atrophy. We describe the clinical features of fourteen patients from seven different families followed in our Diabetes Center. The mean age at Wolfram syndrome 1 diagnosis was 12.4 years. Diabetes mellitus was the first clinical manifestation, in all patients. Sensorineural hearing impairment and central diabetes insipidus were present in 85.7% of patients. Other endocrine findings included hypogonadotropic hypogonadism (7.1%), hypergonadotropic hypogonadism (7.1%), and Hashimoto's thyroiditis (21.4%). Neuropsychiatric disorders were detected in 35.7% of patients, and urogenital tract abnormalities were present in 21.4%. Finally, heart diseases were found in 14.2% of patients. Eight patients (57.1%) died at the mean age of 27.3 years. The most common cause of death was respiratory failure which occurred in six patients. The remaining two died due to end-stage renal failure and myocardial infarction. Our data are superimposable with those reported in the literature in terms of mean age of onset, the clinical course of the disease, and causes of death. The frequency of deafness and diabetes insipidus was higher in our patients. The incidence of urogenital diseases was lower although it led to the death of one patient. Long-term follow-up studies including large patient cohorts are necessary to establish potential genotype-phenotype correlation in order to personalize the most suitable clinical approach for each patient.
Topics: Adult; Diabetes Mellitus, Type 1; Hearing Loss, Sensorineural; Humans; Male; Urogenital Diseases; Wolfram Syndrome
PubMed: 35010780
DOI: 10.3390/ijerph19010520 -
Journal of Investigative Medicine High... 2023Either optic neuritis (neuropathy) or hypopituitarism has been known to occur separately after COVID-19 vaccination. In this report, we describe the rare combination of... (Review)
Review
Either optic neuritis (neuropathy) or hypopituitarism has been known to occur separately after COVID-19 vaccination. In this report, we describe the rare combination of hypophysitis and optic neuritis which occurred after COVID-19 vaccination. A 74-year-old woman began to have thirst, polydipsia, and polyuria, and was diagnosed as central diabetes insipidus 1 month after the fourth COVID-19 mRNA vaccine. Head magnetic resonance imaging (MRI) disclosed the thickened pituitary stalk and enlarged pituitary gland with high contrast enhancement as well as the absence of high-intensity signals in the posterior pituitary lobe on the T1-weighted image, leading to the diagnosis of lymphocytic hypophysitis. She was well with desmopressin nasal spray until further 2 months later, when she developed bilateral optic neuritis, together with gait disturbance, intention tremor of the upper extremities, urinary retention, constipation, abnormal sensation in the distal part of the lower extremities, and moderate hemiplegia on the left side. Autoantibodies, including anti-aquaporin 4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG), were all negative. She showed multifocal spinal cord lesions on MRI and oligoclonal bands in the cerebrospinal fluid obtained by spinal tap, and so underwent steroid pulse therapy with methylprednisolone in the tentative diagnosis of multiple sclerosis, resulting in visual acuity recovery and alleviation of neurological symptoms. In the literature review, the combination of optic neuritis and hypophysitis, mostly with diabetes insipidus, was reported in 15 patients as case reports before the years of COVID-19 pandemic. The COVID-19 vaccination would trigger the onset of hypophysitis and optic neuritis in this patient.
Topics: Female; Humans; COVID-19 Vaccines; Pandemics; COVID-19; Diabetes Insipidus; Hypophysitis; Optic Neuritis; Diabetes Mellitus; mRNA Vaccines
PubMed: 37431875
DOI: 10.1177/23247096231186046 -
International Journal of Environmental... Apr 2021Wolfram syndrome is a rare neurodegenerative disorder that is typically characterized by diabetes mellitus and optic atrophy. Other common features are diabetes... (Review)
Review
Wolfram syndrome is a rare neurodegenerative disorder that is typically characterized by diabetes mellitus and optic atrophy. Other common features are diabetes insipidus and hearing loss, but additional less-frequent findings may also be present. The phenotype spectrum is quite wide, and penetrance may be incomplete. The syndrome is progressive, and thus, the clinical picture may change during follow-up. Currently, two different subtypes of this syndrome have been described, and they are associated with two different disease-genes, () and . These genes encode a transmembrane protein and an endoplasmic reticulum intermembrane protein, respectively. These genes are detected in different organs and account for the pleiotropic features of this syndrome. In this review, we describe the phenotypes of both syndromes and discuss the most pertinent literature about the genotype-phenotype correlation. The clinical presentation of Wolfram syndrome type 1 suggests that the pathogenic variant does not predict the phenotype. There are few papers on Wolfram syndrome type 2 and, thus, predicting the phenotype on the basis of genotype is not yet supported. We also discuss the most pertinent approach to gene analysis.
Topics: Genetic Association Studies; Genotype; Humans; Mutation; Optic Atrophy; Phenotype; Wolfram Syndrome
PubMed: 33946243
DOI: 10.3390/ijerph18094796 -
AACE Clinical Case Reports 2022The common causes of central diabetes insipidus (CDI) include trauma to the pituitary, hypoperfusion, and malignancy. However, CDI can also be transient. An emerging...
OBJECTIVE
The common causes of central diabetes insipidus (CDI) include trauma to the pituitary, hypoperfusion, and malignancy. However, CDI can also be transient. An emerging cause of transient diabetes insipidus is through the use and withdrawal of vasopressin. Here, we present a case of transient CDI that developed during an intensive care unit admission.
CASE REPORT
A Caucasian woman presented to the emergency room after a fall. On presentation, the patient was found to be in shock and was admitted to the surgical intensive care unit. Treatment with norepinephrine, vasopressin, and intravenous antibiotics was started. On day 5 of hospitalization, the patient's blood pressure improved, and treatment with vasopressin was discontinued. On day 6 of hospitalization, the patient's urine output increased and serum sodium level was elevated. Despite increasing free water, serum sodium level continued to rise. Endocrinology division was consulted, and urine osmolality was consistent with diabetes insipidus (DI). Urine osmolality at 30 and 60 minutes after desmopressin (1-desamino-8-d-arginine vasopressin [DDAVP]) was consistent with CDI. Magnetic resonance imaging scan of the brain showed no intracranial pathology. Over the next day with scheduled DDAVP, serum sodium level decreased below the goal level. Thus, DDAVP was held. Prior to discharge, the patient did not require additional DDAVP. She was discharged without DDAVP.
DISCUSSION
Our patient's workup was initially consistent with CDI. However, the DI resolved spontaneously, supporting transient CDI secondary to vasopressin infusion. Different theories have emerged about why this occurs with vasopressin. However, further investigation is needed.
CONCLUSION
Although rare, it is important to monitor for DI after vasopressin infusion and have a suspicion that DI may be transient in the absence of a clear cause.
PubMed: 35097193
DOI: 10.1016/j.aace.2021.06.004