Idiopathic Central Diabetes Insipidus Research Articles

A Case Report on Unusual Idiopathic Central Diabetes Insipidus During the Polyuric Phase of AKI

A Case Report on Unusual Idiopathic Central Diabetes Insipidus During the Polyuric Phase of AKI

Idiopathic Central Diabetes Insipidus as an Etiology of Left Ventricular Suction and Ventricular Arrhythmias

<h3>Introduction</h3> Left ventricular assist device (LVAD) implantation has increased in patients with end-stage heart failure. Left ventricular suction is a known complication of LVAD therapy, with etiologies including hypovolemia, sepsis and ventricular arrhythmias. Prompt recognition of the underlying cause is crucial in the management and prevention of suction events. <h3>Case Report</h3> A 59-year-old male with non-ischemic cardiomyopathy status post LVAD and ventricular tachycardia (VT) presented with shocks from his implantable defibrillator. He noted 2-3 weeks of low flow alarms and daily suction events with associated lightheadedness and dizziness. On admission, device interrogation revealed two episodes of successfully terminated VT. His antiarrhythmic therapy and device settings were adjusted by the electrophysiology team. On day two of hospitalization, he had ventricular fibrillation associated with a suction event. He was noted to have increased urine output despite holding diuresis. LVAD speed was decreased and he was given intravenous fluids, however right heart catheterization revealed very low filling pressures despite these interventions. He continued to have polyuria despite liberalization of fluid intake and suction alarms with associated ventricular ectopy. Urine studies revealed low urine osmolality of 266 mOsm/Kg and urine sodium of 41 mmol/L. Hypertonic saline challenge resulted in hypernatremia and improved but suboptimal urine concentration. Workup for causes of nephrogenic diabetes insipidus (DI) was unrevealing. Computed tomography of the sella did not reveal any pituitary abnormalities. He was started on desmopressin and urine output appropriately decreased. He had no further ventricular arrhythmias, though he continued to have intermittent suction alarms associated with positional changes. <h3>Summary</h3> Treatment of left ventricular suction events includes correction of underlying etiology, maintaining preload, reducing LVAD speed and management of complications including ventricular arrhythmias. Our patient's suction events and ventricular arrhythmias were presumed secondary to hypovolemia in the setting of central DI and polyuria, which improved with treatment with desmopressin. In review of literature, this is the first documented case of diabetes insipidus resulting in suction events and ventricular arrhythmias.

Central Diabetes Insipidus in Children and Adolescents: Twenty-Six Year Experience from a Single Centre.

Introduction Paediatric cohorts of central diabetes insipidus (CDI) have shown varying prevalence for different causes of CDI. The objective of this study was to determine the causes of CDI and long-term outcome in children and adolescents from a Tertiary Paediatric Endocrinology unit. Methods The clinic database was searched to identify patients with CDI managed between 1993 and 2019. Relevant clinical information was collected from patient records. Results A total of 138 CDI patients, median age 6 years (range <1–18) at presentation, were identified. Principal CDI aetiologies were craniopharyngioma (n = 44), acute central nervous system (CNS) insult (n = 33), germinoma (n = 15), postneurosurgery (indication other than craniopharyngioma and germinoma, n = 20), midline CNS malformation (n = 14), Langerhans cell histiocytosis (n = 5), and familial (n = 2). Idiopathic CDI in this cohort was infrequent (n = 5). Patients with CNS malformations/infections presented with CDI at a younger age compared to patients with CNS tumours (p < 0.0001). Five patients, initially presenting as idiopathic CDI, were subsequently diagnosed with germinoma after a median interval of 3.3 years. All patients with CDI related to craniopharyngioma and nearly all (87%) patients with CDI related to germinoma had concomitant GH, ACTH, and TSH deficiency. The majority of patients who manifested CDI due to acute CNS insult either deceased (30%) or had transient CDI (33.3%). Conclusion Surgery for craniopharyngioma was the most common underlying aetiology of CDI with ubiquitous occurrence of panhypopituitarism in these patients. Manifestation of CDI in patients with acute CNS insult carries poor prognosis. We affirm that neuroimaging assessment in idiopathic CDI should be continued beyond 3 years from diagnosis as a significant number of patients exhibited progression of infundibular thickening 3 years post-CDI diagnosis.

Approach to the Pediatric Patient: Central Diabetes Insipidus.

Central diabetes insipidus (CDI) is a complex disorder in which large volumes of dilute urine are excreted due to arginine-vasopressin deficiency, and it is caused by a variety of disorders affecting the hypothalamic-posterior pituitary network. The differential diagnosis is challenging and requires a detailed medical history, physical examination, biochemical approach, imaging studies, and, in some cases, histological confirmation. Magnetic resonance imaging is the gold standard method for evaluating congenital or acquired cerebral and pituitary stalk lesions. Pituitary stalk size at presentation could be normal, but it may change over time, depending on the underlying condition, while other brain areas or organs may become involved during follow-up. Early diagnosis and treatment are crucial to avoid central nervous system damage and germ cell tumor dissemination and to minimize complications of multiple pituitary hormone defects. We provide a practical update on the diagnosis and management of patients with CDI and highlight several pitfalls that may complicate the differential diagnosis of conditions presenting with polyuria and polydipsia. The need for a careful and close follow-up of patients with apparently idiopathic CDI is particularly emphasized because the underlying condition may be recognized over time. The clinical scenario that we outline at the beginning of this article represents the basis for the discussion about how the etiological diagnosis of CDI can be overlooked and demonstrates how a water intake and urine output improvement can be a sign of progressive damage of both hypothalamus and anterior pituitary gland with associated pituitary hormonal deficiencies.

A good night’s sleep after two decades: a case report of idiopathic central diabetes insipidus

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Etiological Spectrum with Diagnosis and Prognosis of Central Diabetes Insipidus needs Long Term Followup: A Single Centre Experience

Introduction: Central Diabetes insipidus (CDI) is a rare disorder caused vasopressin deficiency characterized by the excretion of copious volumes of unconcentrated urine. Objective: To assess the etiological,clinical, biochemical and radiological spectrum of Central DI in our institute and long term follow up of these cases. Material and Methods:32 patients with Central DI admitted in Department of Endocrinology,Guwahati Medical College, Assam in the last 2.5 years were included.Detailed clinical assessment, biochemical evaluation and MRI (Magnetic Resonance imaging) brain were done in all the patients. Central DI without any identifiable cause was considered Idiopathic and those with structural lesion in hypothalamic pituitary region were considered organic. Result: Idiopathic CDI was present in 12(37.5%) patients and 20(62.5%) patients had organic CDI with acute onset of presentation.12(60%) patients with organic CDI present with neurological symptoms but 8(40%) patients had no neurological symptoms even with organic cause. Pituitary dysfunction was common in organic CDI as compared to idiopathic CDI. Paediatric patients commonly present with organic cause for CDI with low cortisol most common hormonal deficit. One patient of idiopathic CDI with normal stalk thickness at baseline presented with clinical and radiological features of (Langerhans cell histiocytosis) on follow up. Conclusion: Organic CDI more likely to have acute onset of presentation than idiopathic CDI and even in absence of neurological features. Paediatric patients commonly have organic cause for CDI. We propose the paramount importance of long-term clinical follow-up and reassessment of endocrine function in patients with CDI for definitive diagnosis of autoimmune and inflammatory causes of idiopathic CDI and timely treatment of pituitary ypofunction.

Chapter 16 - Neuroimaging of central diabetes insipidus

Central diabetes insipidus (CDI) occurs secondary to deficient synthesis or secretion of arginine vasopressin peptide from the hypothalamo-neurohypophyseal system (HNS). It is characterized by polydipsia and polyuria (urine output >30mL/kg/day in adults and >2l/m2/24h in children) of dilute urine (<250mOsm/L). It can result from any pathology affecting one or more components of the HNS including the hypothalamic osmoreceptors, supraoptic or paraventricular nuclei, and median eminence of the hypothalamus, infundibulum, stalk or the posterior pituitary gland. MRI is the imaging modality of choice for evaluation of the hypothalamic-pituitary axis (HPA), and a dedicated pituitary or sella protocol is essential. CT can provide complimentary diagnostic information and is also of value when MRI is contraindicated. The most common causes are benign or malignant neoplasia of the HPA (25%), surgery (20%), and head trauma (16%). No cause is identified in up to 30% of cases, classified as idiopathic CDI. Knowledge of the anatomy and physiology of the HNS is crucial when evaluating a patient with CDI. Establishing the etiology of CDI with MRI in combination with clinical and biochemical assessment facilitates appropriate targeted treatment. This chapter illustrates the wide variety of causes and imaging correlates of CDI on neuroimaging, discusses the optimal imaging protocols, and revises the detailed neuroanatomy required to interpret these studies.

Chapter 15 - Autoimmune diabetes insipidus

Once central diabetes insipidus (CDI) has been diagnosed, every effort should be made to reveal its underlying cause. Autoimmune CDI should be considered in the differential diagnosis of idiopathic CDI and also of mass lesions of the sella region. An autoimmune etiology of CDI was first suggested in 1983 by the detection of autoantibodies to hypothalamic vasopressin-producing cells (AVPcAb) in adults and also in children with the disease, using the indirect immunofluorescence test. The major autoantigen for autoimmune CDI has now been recognized as rabphilin-3A, a protein of secretory vesicles of the neurohypophyseal system. The detection of autoantibodies to rabphilin-3A by Western blotting or of AVPcAb provides strong evidence for the diagnosis of autoimmune CDI. Autoimmune CDI is recognized mostly in patients who had also been diagnosed with endocrine autoimmune disorders. The radiological and morphological correlate with autoimmune DI is lymphocytic infundibuloneurohypophysitis (LINH) as detected by magnetic resonance imaging and biopsies that show massive infiltration of the posterior pituitary and the infundibulum with lymphocytes and some plasma cells, and fibrosis in the later stages of the disease. LINH may be associated with lymphocytic anterior hypophysitis. Both may either appear spontaneously or on treatment with immune checkpoint inhibitors.

MYELODYSPLASTIC SYNDROME PRESENTING AS CENTRAL DIABETES INSIPIDUS

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Central diabetes insipidus (CDI) is characterized by a decreased release of antidiuretic hormone (ADH) and often caused by disorders that act at sites involved in either ADH secretion or delivery. Common causes of CDI include primary/secondary brain tumors, infiltrative diseases, trauma, and idiopathic CDI. Non-traumatic causes of CDI are less studied. Myelodysplastic syndrome (MDS), which describes a group of hematopoietic stem cell disorders that is characterized by dysplasia and cytopenia, is a rare but reported non-traumatic cause of CDI. We present a patient with a history of MDS that was found to have CDI during his hospital course. CASE PRESENTATION: A man in his 60s, with history of relapsed acute myeloid leukemia with induction chemotherapy one month prior to admission (PTA), presented with one week history of fevers and melena. Stated he had been urinating several times a day, with at least two episodes of nocturia, and severe thirst. Patient denied headache, nausea, vomiting, or trauma. On presentation, he was mentating well with dry mucous membranes. His initial sodium (Na) levels of 141 mEq/L increased to 160 mEq/L after two liters (L) of normal saline (NS) resuscitation, peaking at 164 mEq/L. Urinalysis revealed specific gravity of 1.006 when Na was 148 mEq/L. His urine osmolality became more concentrated with desmopressin (DDAVP) administration, increasing from 192 mOsm/kg to 381 mOsm/kg, suggestive of central diabetes insipidus (DI) over nephrogenic. Daily urine output progressed from 5.7L to 2.6L to 0.65L. MRI brain was negative for sella or pituitary findings. His Na levels improved to normal limits with DDAVP and 1/2 NS. Patient received a bone marrow cancer karyotype one year PTA, which revealed a t(6;16), (p21;q24) chromosome abnormality. DISCUSSION: There are three leading mechanisms for CDI as a complication of MDS: DNA methylation, chromosomal abnormality, and micro-infiltration of cancer cells. In a study of 184 patients with MDS and AML, DNA methylation microarray was used to assess the relative contributions of aberrant DNA methylation during disease progression and was notably seen in every case. With alterations of the mRNA-expression and promoter-related DNA methylation of vasopressin, particularly ANP precursor genes, as a pathophysiologic mechanism for CDI, DNA methylation is noted to be involved in both processes. Regarding chromosomal anomalies, the most common associated with MDS and CDI are 2,3,7,12, and 20. Our patient had a balanced translocation between the short arm of chromosome 6 and the long arm of chromosome 16. To our knowledge, this is a novel finding that warrants further research. Lastly, reported micro-infiltration of cancer cells to the hypothalamus leading a presentation of CDI have been documented. CONCLUSIONS: CDI should be considered in all AML/MDS patients presenting with polyuria and polydipsia with an unremarkable MRI of the brain. Reference #1: Jiang, Y., Dunbar, A., Gondek, L. P., Mohan, S., Rataul, M., Okeefe, C., … Maciejewski, J. P. (2009). Aberrant DNA methylation is a dominant mechanism in MDS progression to AML. Blood, 113(6), 1315–1325. doi: 10.1182/blood-2008-06-163246 Reference #2: Sun, R., Wang, C., Zhong, X., & Wu, Y. (2016). Diabetes Insipidus as an Initial Presentation of Myelodysplastic Syndrome: Diagnosis with Single-Nucleotide Polymorphism Array-Based Karyotyping. The Tohoku Journal of Experimental Medicine, 238(4), 305–310. doi: 10.1620/tjem.238.305 DISCLOSURES: No relevant relationships by Narjes Akhlaghi, source=Web Response No relevant relationships by Rohit Aloor, source=Web Response No relevant relationships by sai phani sree cherukuri, source=Web Response

Central Diabetes Insipidus in a Patient with Stiff Person Syndrome

Background: Central diabetes insipidus (CDI) is a rare hypothalamic-pituitary disease due to the deficiency of arginine vasopressin (AVP) synthesis of which more than one third of etiologies are unidentified. Autoimmunity is associated with one third of patients with apparently idiopathic CDI. The most common antibody detected in auto immune CDI is autoantibodies to AVP-secreting cells (AVPcAb). Stiff-person syndrome (SPS) is a rare neurological disorder with features of an autoimmune disease. It is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli. It is well known that SPS is associated with multiple auto immune endocrinopathy including auto immune diabetes and auto immune thyroid disease. The association between SPS and CDI is not well documented in the literature. Case information: We report a case of 51 year old female who developed CDI while being treated for SPS unmasked by high dose steroids. Result: Anti amphiphysin is the only antibody detected in our patient. Animal studies showed a high expression of amphiphysin in anterior and posterior pituitary gland, data in human are still vague .One theory is that anti-amphiphysin antibodies attack the amphiphysin molecules in the AVP secreting cells and inhibit release of AVP. Conclusion: This antibody could hint towards the autoimmune cause of CDI.

Endocrine Outcomes In Central Diabetes Insipidus: the Predictive Value of Neuroimaging "Mismatch Pattern".

The etiology of central diabetes insipidus (CDI) in children is often unknown. Clinical and radiological features at disease onset do not allow discrimination between idiopathic forms and other conditions or to predict anterior pituitary dysfunction. To evaluate the evolution of pituitary stalk (PS) thickening and the pattern of contrast-enhancement in relation with etiological diagnosis and pituitary function. We enrolled 39 children with CDI, 29 idiopathic and 10 with Langerhans cell histiocytosis (LCH). Brain magnetic resonance images taken at admission and during follow-up (332 studies) were examined, focusing on PS thickness, contrast-enhancement pattern, and pituitary gland size; T2-DRIVE and postcontrast T1-weighted images were analyzed. Seventeen of 29 patients (58.6%) with idiopathic CDI displayed "mismatch pattern," consisting in a discrepancy between PS thickness in T2-DRIVE and postcontrast T1-weighted images; neuroimaging findings became stable after its appearance, while "mismatch" appeared in LCH patients after chemotherapy. Patients with larger PS displayed mismatch more frequently (P = 0.003); in these patients, reduction of proximal and middle PS size was documented over time (P = 0.045 and P = 0.006). The pituitary gland was smaller in patients with mismatch (P < 0.0001). Patients with mismatch presented more frequently with at least one pituitary hormone defect, more often growth hormone deficiency (P = 0.033). The PS mismatch pattern characterizes patients with CDI, reduced pituitary gland size, and anterior pituitary dysfunction. The association of mismatch pattern with specific underlying conditions needs further investigation. As patients with mismatch show stabilization of PS size, we assume a prognostic role of this peculiar pattern, which could be used to lead follow-up.

MON-078 WFS1 Related Disorder in A 4-Month Old Girl

Background: Idiopathic early-onset central diabetes insipidus (CDI) may be due to mutations of arginine vasopressin-neurophysin II (AVP-NPII (AVP)) or wolframin (WFS1/2) genes (1). Clinical Case: A 4-month old girl presented to our pediatric endocrinology clinic due to severe polydipsia-polyphagia and polyuria. Plasma and urine glucose and HbA1c were normal and 24h monitoring of urinary output was elevated (4.2ml/kg/h). We proceeded to a modified 5-h water deprivation test followed by 0.1 mcg DDAVP IM. Results were compatible with partial central DI: at 5-hrs weight loss 5%, urine osmolality 155 from 111 at 0΄and 355 mOsm/kg 2hrs after DDAVP administration.Pituitary MRI was normal with presence of posterior pituitary bright spot and normal pituitary stalk.We initiated therapy with oral desmopressin acetate titrated at the dose of 70mcg x 3/day under close clinical and biochemical surveillance. Hyponatremia, with high natriuresis >100 mmol/L and elevated BNP occurred the 3rd day. Fludrocortisone 100 mcg x 2/day controlled natriuresis and supplemental oral NaCl 15% 15ml/day was needed to restore normal electrolytes (2).Marked catch-up growth was observed already at 1 month for height, weight and head circumference. Methods: Whole exome sequencing was performed targeted to a gene panel related to DI, containing AVP gene, WFS1/2 genes and AVPR2/AQP2 genes. Result: Two heterozygous variants were revealed: WFS1:NM_001145853:exon8:c. G997A:p.V333I,WFS1:NM_006005:exon8:c.G997A:p.V333I and WFS1:NM_00114585 3:exon8:c.G1832A:p.R611H,WFS1:NM_006005:exon8:c.G1832A:p.R611H reported as possibly damaging in 1/6 and 4/6 prediction programs respectively. These variants will be checked in both parents to confirm the presumed compound heterozygosity pattern in the child. Conclusion: We present a 4-month old girl with two heterozygous variants of WFS1 gene which may cause early-onset central diabetes insipidus and possibly a WFS1 related disorder (1).Reference:(1) Perrotta S et al. Early-onset central diabetes insipidus is associated with de novo arginine vasopressin-neurophysin II or Wolfram syndrome 1 gene mutations. Eur J Endocrinol. 2015 Apr;172(4):461-72(2) Papadimitriou DT, Spiteri A, Attilakos A, Papadimitriou A. Cerebral Salt Wasting Complicated by Central Diabetes Insipidus and Growth Hormone Deficiency. Indian J Pediatr. 2018 Jul;85(7):580-581

Thickening of the pituitary stalk in children and adolescents with central diabetes insipidus: Causes and consequences

Central diabetes insipidus (CDI) is a rare disorder in children. The aetiology of CDI in childhood is heterogeneous. The aim of this study is to illustrate the importance of a careful clinical and neuro-radiological follow-up of the pituitary and hypothalamus region in order to identify the aetiology and the development of associated hormonal deficiencies. Clinical and auxological variables of 15 children diagnosed with CDI were retrospectively analysed in a paediatric hospital. Evaluations of adenohypophyseal function and cranial MRI were performed periodically. The mean age at diagnosis of CDI was 9.6 years (range: 1.32-15.9). The aetiological diagnosis could be established initially in 9 of the 15 patients, as 7 with a germinoma and 2 with a histiocytosis. After a mean follow-up of 5.5 years (range: 1.6-11.8), the number of idiopathic cases was reduced by half. At the end of the follow-up, the aetiological diagnoses were: 9 germinoma (60%), 3 histiocytosis (20%), and 3 idiopathic CDI (20%). There is a statistically significant association between stalk thickening and tumour aetiology. At least one adenohypophyseal hormonal deficiency was found in 67% of cases, with the majority developing in the first two years of follow-up. Growth hormone deficiency (60%) was the most prevalent. The follow-up of CDI should include hormone evaluation with special attention, due to its frequency, to GH deficiency. In addition, a biannual MRI in an idiopathic CDI should be performed, at least during the first 2-3 years after diagnosis, as 50% of them were diagnosed with a germinoma or histiocytosis during this period.

Central Diabetes Insipidus in a Patient with Stiff Person Syndrome

Background: Central diabetes insipidus (CDI) is a rare hypothalamic-pituitary disease due to the deficiency of arginine vasopressin (AVP) synthesis of which more than one third of etiologies are unidentified. Autoimmunity is associated with one third of patients with apparently idiopathic CDI. The most common antibody detected in auto immune CDI is autoantibodies to AVP-secreting cells (AVPcAb). Stiff-person syndrome (SPS) is a rare neurological disorder with features of an autoimmune disease. It is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli. It is well known that SPS is associated with multiple auto immune endocrinopathy including auto immune diabetes and auto immune thyroid disease. The association between SPS and CDI is not well documented in the literature. Case information: We report a case of 51 year old female who developed CDI while being treated for SPS unmasked by high dose steroids. Result: Anti amphiphysin is the only antibody detected in our patient. Animal studies showed a high expression of amphiphysin in anterior and posterior pituitary gland, data in human are still vague .One theory is that anti-amphiphysin antibodies attack the amphiphysin molecules in the AVP secreting cells and inhibit release of AVP. Conclusion: This antibody could hint towards the autoimmune cause of CDI.

Intracranial pathologies associated with central diabetes insipidus in infants.

Background Idiopathic central diabetes insipidus (CDI) has been associated with intracranial pathologies that do not involve the structural pituitary gland or hypothalamus. The objective was to study the association between non-structural hypothalamic/pituitary intracranial pathologies (NSHPIP) with CDI and to review etiologies that may be contributory to the development of CDI. Methods A retrospective query of our intra-institutional database from 2006 to 2015. Children admitted diagnosed with diabetes insipidus (DI) (ICD-9 253.5) between the ages of 0-1 year were included. Patient charts were reviewed to include those who have a documented diagnosis of CDI, hypernatremia (>145 mmol/L), high serum osmolality (>300 mOsm/kg), low urine osmolality (<300 mOsm/kg), and brain imaging reports. Diagnoses of nephrogenic DI were excluded. Results Twenty-three infant patients were diagnosed with CDI. Eleven subjects (48%) had NSHPIP. Of those, 18% had cerebral infarction, 27% had intracranial injury and hemorrhage due to traumatic brain injury, 18% had isolated intraventricular hemorrhage, and 27% had meningitis. Hospital prevalence for NSHPIP, age 0-1 year, ranged from 0.05% to 0.3%. Conclusions Rates of NSHPIP in those with CDI are higher than expected hospital rates (p<0.001), suggesting a possible association between CDI and NSHPIP.

Clinical characteristics of central diabetes insipidus: a retrospective analysis of 230 cases

Objective: To evaluate the clinical characteristics and etiologies of central diabetes insipidus (CDI). Methods: The clinical data of 230 patients with CDI in the Department of Endocrinology of Chinese PLA General Hospital from 2008 June to 2014 December were collected and analyzed retrospectively. Results: The three most common causes of CDI were idiopathic CDI, lymphocytic hypophysitis and intracranial germ cell tumors. Among all the CDI, the idiopathic CDI accounted for 37.48%. There were significant differences in age onset and gender distribution among the different causes of CDI. The patients with intracranial germ cell tumors [age of onset(19.2±10.2) years] were younger than the other types of CDI. Germ cell tumors patients were more common in male, and lymphocytic hypophysitis patients were more common in female. The most frequent abnormality of anterior pituitary in patients with CDI was growth hormone deficiency, followed by hypogonadism, adrenal insufficiency and hypothyroidism. The dysfunction of thyroid axis and adrenal axis in patients with germ cell tumor was more common than those in patients with idiopathic and lymphocytic hypophysitis. Conclusions: The most common causes of central diabetes insipidus were idiopathic CDI, lymphocytic hypophysitis and intracranial germ cell tumors. There were differences in age of onset, gender distribution and abnormal production of anterior pituitary hormones among all causes of CDI patients.

Central Diabetes Insipidus: Clinical Characteristics and Long-Term Course in a Large Cohort of Adults

Central diabetes insipidus (CDI) is a rare heterogeneous condition with various underlying causes. This study sought to increase the still-limited data on the clinical characteristics and long-term course in adults diagnosed with CDI. Data on demographics, presentation, imaging findings, affected pituitary axes, treatment, and complications were collected retrospectively from the files of 70 adult patients with CDI followed at a referral endocrine clinic. Forty women and 30 men were included. Mean age was 46.8 ± 15 years at the time of this study and 29.3 ± 20 years at CDI diagnosis. Twenty-eight patients were diagnosed in childhood. Forty patients (57%) acquired CDI following surgery. Main sellar pathologies were: craniopharyngioma, 17 patients (11 diagnosed in childhood); Langerhans histiocytosis, 10 patients (5 diagnosed in childhood); 7 patients (all diagnosed as adults) had a growth hormone-secreting adenoma; 12 patients (17%; 6 diagnosed in childhood) had idiopathic CDI. At least one anterior pituitary axis was affected in 73% of the cohort: 59% had growth hormone deficiency, 56% hypogonadism, 55% central hypothyroidism, 44% adrenocorticotropic hormone-cortisol deficiency. Patients with postoperative/trauma CDI (n = 44) tended to have multiple anterior pituitary axes deficits compared to the nonsurgical group of patients. All patients were treated with vasopressin preparations, mostly nasal spray. Hyponatremia developed in 32 patients, more in women, and was severe (<125 mEq/L) in 10 patients. Hypernatremia (>150 mEq/L) was noticed in 5 patients. Overall, the calculated complication rate was 22 in 1,250 treatment-years. Most adult patients with CDI have anterior pituitary dysfunction. Stability is usually achieved with long-term treatment. Women were more susceptible to desmopressin complications, albeit with an overall relatively low complication rate. ACTH = adrenocorticotropic hormone CDI = central diabetes insipidus GH = growth hormone MRI = magnetic resonance imaging.

Transient idiopathic central diabetes insipidus: is severe sepsis a possible cause?

Idiopathic central diabetes insipidus (CDI) is a disorder characterized by hypotonic polyuria and polydipsia, without any identified etiology. Here we report a case of a 57-year-old woman, with idiopathic CDI, admitted to our department with severe sepsis and acute kidney failure. After clinical and radiological investigations, she was diagnosed with idiopathic CDI. In this case report the findings suggest that severe sepsis could be the trigger for this disease. In addition, we hypothesise that apelin, a diuretic neuropeptide, plays a role in such a process. Apelin levels are known to increase during severe sepsis, which in turn counteracts vasopressin actions through inhibition of vasopressin neuron activity and vasopressin release.

Pituitary Morphology and Function in 43 Children with Central Diabetes Insipidus.

Objective. In pediatric central diabetes insipidus (CDI), etiology diagnosis and pituitary function monitoring are usually delayed. This study aimed to illustrate the importance of regular follow-up and pituitary function monitoring in pediatric CDI. Methods. The clinical, hormonal, and neuroradiological characteristics of children with CDI at diagnosis and during 1.5–2-year follow-up were collected and analyzed. Results. The study included 43 CDI patients. The mean interval between initial manifestation and diagnosis was 22.29 ± 3.67 months (range: 2–108 months). The most common complaint was polyuria/polydipsia. Causes included Langerhans cell histiocytosis, germinoma, and craniopharyngioma in 2, 5, and 4 patients; the remaining were idiopathic. No significant changes were found during the 1.5–2 years after CDI diagnosis. Twenty-three of the 43 cases (53.5%) had ≥1 anterior pituitary hormone deficiency. Isolated growth hormone deficiency was the most frequent abnormality (37.5%) and was not associated with pituitary stalk diameter. Multiple pituitary hormone deficiencies were found in 8 cases with pituitary stalk diameter > 4.5 mm. Conclusion. Diagnosis of CDI is usually delayed. CDI with a pituitary stalk diameter > 4.5 mm carries a higher risk of multiple pituitary hormone deficiencies. Long-term MRI and pituitary function follow-ups are necessary for children with idiopathic CDI.

Central diabetes insipidus: clinical profile that suggests organicity in Peruvian children: Lima - Peru 2001-2013.

Central diabetes insipidus (CDI) is a heterogeneous disease caused by arginine vasopressin deficiency; its management implies a profound understanding of the pathophysiology and the clinical spectrum. The aim of the study was to describe the clinical characteristics that indicate organicity in children and adolescents with central diabetes insipidus treated at the Department of Endocrinology from The Child Health's Institute during 2001 to 2013. Cross-sectional, retrospective study. 79 cases of patients diagnosed with CDI (51 males and 28 females) from 1 month to 16 years of age were reviewed. For the descriptive analysis, measures of central tendency and dispersion were used; groups of organic and idiopathic CDI were compared using χ2-test and t-test. A p-value<0.05 was considered significant. The average age of patients was 8.1±4.2 years. Organic causes were intracranial tumors, 44 (55.7%), Langerhans cell histiocytosis (LCH), 11 (13.9%) and cerebral malformations in 7 (8.9%) patients, while the idiopathic group was 14 (17.7%) patients. Regarding clinical characteristics suggestive of organicity, headache (p=0.02) and visual disturbances (p=0.01) were found statistically significant. The anterior pituitary hormonal abnormalities were documented in 34 (52.3%) organic CDI patients. Furthermore, we did not find a significant difference in the average daily dose of desmopressin between patients with permanent vs. transitory CDI (0.81±0.65 vs. 0.59±0.62; p=0.363). The main clinical features suggestive of organicity in pediatric patients with central diabetes insipidus were headache and visual disturbances; furthermore, anterior pituitary hormonal abnormalities suggest an underlying organic etiology.