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

2021 ◽  
Vol 2 (1) ◽  
pp. 15
Author(s):  
Dipti Sarma ◽  
Manoj Gedam
Keyword(s):  
Diabetes Insipidus ◽  
Acute Onset ◽  
Neurological Symptoms ◽  
Central Diabetes Insipidus ◽  
Endocrine Function ◽  
Pituitary Dysfunction ◽  
Paediatric Patients ◽  
Low Cortisol

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.

scholarly journals Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis

2000 ◽  
Vol 142 (4) ◽  
pp. 365-372 ◽  
Author(s):  
C Bullmann ◽  
M Faust ◽  
A Hoffmann ◽  
C Heppner ◽  
F Jockenhovel ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Case Reports ◽  
Lymphocytic Hypophysitis ◽  
Central Diabetes Insipidus ◽  
Presenting Symptoms ◽  
Pituitary Dysfunction ◽  
Mri Scans ◽  
Primary Evaluation ◽  
Therapy Result

OBJECTIVES: We retrospectively reviewed 5 patients with neurosarcoidosis, who all presented with central diabetes insipidus and hypogonadism. DESIGN: This was a single-centre, retrospective analysis of 5 cases with a minimum follow-up of 2 years. METHODS: Case analysis included clinical, biochemical, and endocrinological evaluation and frequent CT/MRI scans of involved organs as primary evaluation and in response to immunosuppressive therapy. RESULT: Neurosarcoidosis was diagnosed in all patients. Two patients had no proven extracerebral manifestation and had a stable disease over 3 and 5 years. One patient showed deterioration with corticosteroids alone but partial remission after additional cyclophosphamide. Pituitary dysfunction remained unchanged in all patients, despite total clinical and radiological remission in two patients. However, one of these patients died of acute granulomatous meningoencephalitis after two years of follow-up. CONCLUSION: Although the presenting symptoms of neurosarcoidosis may vary, the occurrence of central diabetes insipidus associated with typical radiological features is suggestive of neurosarcoidosis. However, there is an increasing number of case reports on lymphocytic hypophysitis. Without the bioptic diagnosis, the differentiation between potentially lethal isolated neurosarcoidosis and lymphocytic hypophysitis is difficult. These cases demonstrate the difficulties in diagnosing neurosarcoidosis and reflect experiences with follow-up parameters.

Endocrine Outcomes In Central Diabetes Insipidus: the Predictive Value of Neuroimaging “Mismatch Pattern”

2020 ◽  
Vol 105 (11) ◽  
Author(s):  
Deborah Bianco ◽  
Flavia Napoli ◽  
Giovanni Morana ◽  
Angela Pistorio ◽  
Anna Elsa Maria Allegri ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Diabetes Insipidus ◽  
Contrast Enhancement ◽  
Anterior Pituitary ◽  
Magnetic Resonance Images ◽  
Central Diabetes Insipidus ◽  
Pituitary Hormone ◽  
Gland Size ◽  
Pituitary Dysfunction

Abstract Context 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. Objective To evaluate the evolution of pituitary stalk (PS) thickening and the pattern of contrast-enhancement in relation with etiological diagnosis and pituitary function. Methods 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. Results 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). Conclusions 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.

scholarly journals Clinical features and molecular analysis of arginine-vasopressin neurophysin II gene in long-term follow-up patients with idiopathic central diabetes insipidus

2010 ◽  
Vol 54 (3) ◽  
pp. 269-273 ◽  
Author(s):  
Sergio L. Batista ◽  
Ayrton C. Moreira ◽  
Jose Antunes-Rodrigues ◽  
Margaret de Castro ◽  
Lucila L. K. Elias ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Clinical Features ◽  
Molecular Analysis ◽  
Arginine Vasopressin ◽  
Central Diabetes Insipidus ◽  
Sequencing Analysis ◽  
Long Term Follow Up ◽  
Intron 2

INTRODUCTION: Central diabetes insipidus (DI) characterized by polyuria, polydipsia and inability to concentrate urine, has different etiologies including genetic, autoimmune, post-traumatic, among other causes. Autosomal dominant central DI presents the clinical feature of a progressive decline of arginine-vasopressin (AVP) secretion. OBJECTIVE: In this study, we characterized the clinical features and sequenced the AVP-NPII gene of seven long-term follow-up patients with idiopathic central DI in an attempt to determine whether a genetic cause would be involved. METHODS: The diagnosis of central DI was established by fluid deprivation test and hyper-tonic saline infusion. For molecular analysis, genomic DNA was extracted and the AVP-NPII gene was amplified by polymerase chain reaction and sequenced. RESULTS: Sequencing analysis revealed a homozygous guanine insertion in the intron 2 (IVS2 +28 InsG) of the AVP-NPII gene in four patients, which represents an alternative gene assembly. No mutation in the code region of the AVP-NPII gene was found. CONCLUSIONS: The homozygous guanine insertion in intron 2 (IVS2 +28 InsG) is unlikely to contribute to the AVP-NPII gene modulation in DI. In addition, the etiology of idiopathic central DI in children may not be apparent even after long-term follow-up, and requires continuous etiological surveillance.

Long-term tumor control after endoscopic endonasal resection of craniopharyngiomas: comparison of gross-total resection versus subtotal resection with radiation therapy

2021 ◽  
pp. 1-9
Author(s):  
Saniya S. Godil ◽  
Umberto Tosi ◽  
Mina Gerges ◽  
Andrew L. A. Garton ◽  
Georgiana A. Dobri ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Diabetes Insipidus ◽  
Endocrine Function ◽  
Gross Total Resection ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Total Resection ◽  
Endoscopic Endonasal

OBJECTIVE Surgical management of craniopharyngiomas (CPAs) is challenging. Controversy exists regarding the optimal goals of surgery. The purpose of this study was to compare the long-term outcomes of patients who underwent gross-total resection with the outcomes of those who underwent subtotal resection of their CPA via an endoscopic endonasal approach. METHODS From a prospectively maintained database of all endoscopic endonasal approaches performed at Weill Cornell Medicine, only patients with CPAs with > 3 years of follow-up after surgery were included. The primary endpoint was radiographic progression. Data were collected on baseline demographics, imaging, endocrine function, visual function, and extent of resection. RESULTS A total of 44 patients with a mean follow-up of 5.7 ± 2.6 years were included. Of these patients, 14 (31.8%) had prior surgery. GTR was achieved in 77.3% (34/44) of all patients and 89.5% (34/38) of patients in whom it was the goal of surgery. Preoperative tumor volume < 10 cm3 was highly predictive of GTR (p < 0.001). Radiation therapy was administered within the first 3 months after surgery in 1 (2.9%) of 34 patients with GTR and 7 (70%) of 10 patients with STR (p < 0.001). The 5-year recurrence-free/progression-free survival rate was 75.0% after GTR and 25.0% after STR (45% in subgroup with STR plus radiotherapy; p < 0.001). The time to recurrence after GTR was 30.2 months versus 13 months after STR (5.8 months in subgroup with STR plus radiotherapy; p < 0.001). Patients with GTR had a lower rate of visual deterioration and higher rate of return to work or school compared with those with STR (p = 0.02). Patients with GTR compared to STR had a lower rate of CSF leakage (0.0% vs 30%, p = 0.001) but a higher rate of diabetes insipidus (85.3% vs 50%, p = 0.02). CONCLUSIONS GTR, which is possible to achieve in smaller tumors, resulted in improved tumor control, better visual outcome, and better functional recovery but a higher rate of diabetes insipidus compared with STR, even when the latter was supplemented with postoperative radiation therapy. GTR should be the goal of craniopharyngioma surgery, when achievable with minimal morbidity.

scholarly journals FRI0172 Long-term follow-up of paediatric patients with positive anti-phospholipid antibodies

2001 ◽  
Author(s):  
A Ince ◽  
A Roumany ◽  
U Daud ◽  
PH Pepmueller ◽  
TL Moore
Keyword(s):  
Paediatric Patients ◽  
Long Term Follow Up

Approach To The Pediatric Patient: Central Diabetes Insipidus

2022 ◽  
Author(s):  
Giuseppa Patti ◽  
Flavia Napoli ◽  
Daniela Fava ◽  
Emilio Casalini ◽  
Natascia Di Iorgi ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Diabetes Insipidus ◽  
Central Diabetes Insipidus ◽  
Pituitary Stalk ◽  
Central Nervous System Damage ◽  
Gold Standard Method ◽  
Underlying Condition ◽  
Detailed Medical History ◽  
Over Time

Abstract 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 in order to avoid central nervous system damage, 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.

scholarly journals Pituitary Morphology and Function in 43 Children with Central Diabetes Insipidus

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Wendong Liu ◽  
Limin Wang ◽  
Minghua Liu ◽  
Guimei Li
Keyword(s):  
Diabetes Insipidus ◽  
Central Diabetes Insipidus ◽  
Pituitary Function ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Initial Manifestation ◽  
Stalk Diameter ◽  
And Function

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.

scholarly journals Long term outcomes of acute and transient psychotic disorders: The missed opportunity of preventive interventions

2018 ◽  
Vol 52 ◽  
pp. 126-133 ◽  
Author(s):  
Grazia Rutigliano ◽  
Sergio Merlino ◽  
Amedeo Minichino ◽  
Rashmi Patel ◽  
Cathy Davies ◽  
...  
Keyword(s):  
High Risk ◽  
Psychotic Disorders ◽  
Cumulative Risk ◽  
Acute Onset ◽  
Kaplan Meier ◽  
Schizophrenia Spectrum ◽  
National Health Service Trust ◽  
Icd 10

AbstractBackground:Acute and transient psychotic disorders (ATPD) are characterized by an acute onset and a remitting course, and overlap with subgroups of the clinical high-risk state for psychosis. The long-term course and outcomes of ATPD are not completely clear.Methods:Electronic health record-based retrospective cohort study, including all patients who received a first index diagnosis of ATPD (F23, ICD-10) within the South London and Maudsley (SLaM) National Health Service Trust, between 1 st April 2006 and 15th June 2017. The primary outcome was risk of developing persistent psychotic disorders, defined as the development of any ICD-10 diagnoses of non-organic psychotic disorders. Cumulative risk of psychosis onset was estimated through Kaplan-Meier failure functions (non-competing risks) and Greenwood confidence intervals.Results:A total of 3074 patients receiving a first index diagnosis of ATPD (F23, ICD-10) within SLaM were included. The mean follow-up was 1495 days. After 8-year, 1883 cases (61.26%) retained the index diagnosis of ATPD; the remaining developed psychosis. The cumulative incidence (Kaplan-Meier failure function) of risk of developing any ICD-10 non-organic psychotic disorder was 16.10% at 1-year (95%CI 14.83–17.47%), 28.41% at 2-year (95%CI 26.80–30.09%), 33.96% at 3-year (95% CI 32.25–35.75%), 36.85% at 4-year (95%CI 35.07–38.69%), 40.99% at 5-year (95% CI 39.12–42.92%), 42.58% at 6-year (95%CI 40.67–44.55%), 44.65% at 7-year (95% CI 42.66–46.69%), and 46.25% at 8-year (95% CI 44.17–48.37%). The cumulative risk of schizophrenia-spectrum disorder at 8-year was 36.14% (95% CI 34.09–38.27%).Conclusions:Individuals with ATPD have a very high risk of developing persistent psychotic disorders and may benefit from early detection and preventive treatments to improve their outcomes.

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