Central diabetes insipidus is a disease of the pituitary gland or hypothalamus and is caused by a deficiency of antidiuretic hormone (ADH). The symptoms are polyuria and polydipsia, while dehydration can develop if fluid intake is inadequate. The condition is treated by hormone replacement – desmopressin, a synthetic analog of ADH.
Central diabetes insipidus is due to a deficiency of antidiuretic hormone (ADH) and therefore its defining characteristic is polyuria. Nephrogenic diabetes insipidus is caused by a failure of the kidney to recognize or respond ADH and therefore also leads to polyuria. Both conditions stem from an inability of the kidneys to reabsorb water. The consequences of polyuria are polydipsia and nocturia-other important characteristics of central diabetes insipidus. A serious complication is a dehydration if the patient is unable to drink enough water to replace the fluids lost.
The origins of central diabetes insipidus (central DI) are varied. Only about ten percent of central DI cases have a genetic background . The pattern of inheritance may be autosomal dominant , which is due to the accumulation of hormone precursors toxic to neurons. Autosomal recessive forms are also observed, owing to genetic changes in the arginine vasopressin-neurophysin II gene.
Most of the central DI cases are due to non-genetic effects, such as brain injury, brains tumors, or brain surgery. Thus, a history of such events will suggest a neurogenic, or central origin of the condition as opposed to nephrogenic disease. Idiopathic cases are also fairly common. The disease may be caused by autoimmune and inflammatory conditions. Among tumors associated with central DI is craniopharyngioma, the most common pediatric intracranial tumor. Postoperative central DI may occur after various surgeries; almost 20% of patients undergoing transsphenoidal pituitary adenoma surgery developed central DI, which, in most cases, resolved spontaneously in a little over a week . Of patients with serious head injuries, about 15% developed central DI. The most important risk factors were severe head trauma and penetrating trauma .
Central DI may be transient, permanent, or triphasic. The triphasic condition starts with a few days of polyuria, followed by amelioration of the symptoms for another few days (owing to release of stored antidiuretic hormone), and ends with permanent central DI.
The symptoms in infants are less specific than in adults and include irritability, crying, weight loss, and growth delays. Apart from nephrogenic DI, central DI needs to be differentiated from psychogenic polydipsia.
Entire Body System
Here, we report on a female infant who presented with fever, convulsions, and polyuria. Examination revealed weight and length below the 3rd centile along with signs of severe dehydration. Fundal examination showed bilateral chorioretinitis. [ncbi.nlm.nih.gov]
[…] liquid per day In severe cases, or if a person can't get enough liquid to drink, central diabetes insipidus can cause: Dehydration Confusion Loss of consciousness Symptoms of central DI in children may be: Low energy Irritability Slow growth Weight loss Fever [webmd.com]
In infants, additional symptoms may occur including irritability, lethargy, vomiting, constipation and fever. [rarediseases.org]
A middle-aged woman who was receiving treatment for Graves' disease suddenly complained of polydipsia, polyuria and general fatigue. Laboratory tests showed hyperthyroidism, hypernatremia, hypoosmolar urine and a decreased plasma vasopressin level. [ncbi.nlm.nih.gov]
Signs of dehydration include: thirst, dry skin, sluggishness, fatigue, dizziness, confusion and nausea. If you are severely dehydrated, you can even experience seizures, permanent brain damage and death. [draxe.com]
Dehydration can cause: Dry mouth Changes in skin elasticity Thirst Fatigue Electrolyte imbalance Diabetes insipidus can cause an imbalance in electrolytes — minerals in your blood, such as sodium and potassium, that maintain the fluid balance in your [mayoclinic.org]
- Developmental Delay
Many of these patients have developmental delays and may be diapered later into childhood, which could make polyuria more difficult to appreciate. [academic.oup.com]
If left untreated, repeated episodes of dehydration can potentially result in seizures, brain damage, developmental delays, and physical and mental retardation. [rarediseases.org]
However, persistent polyuria can cause not only patient discomfort, including polyuria and polydipsia, but also volume depletion that can produce allograft dysfunction. [ncbi.nlm.nih.gov]
Two months after the transplantation, she was admitted for persistent polyuria, polydipsia, and nocturia with urine output of more than 4 L/d. [ncbi.nlm.nih.gov]
Case report 1: Forty-five year old female, previously healthy, was observed in April 2011 complaining of polydipsia, polyuria, nocturia and weight loss since January. [endocrine-abstracts.org]
Laboratory studies should include determination of serum electrolyte and glucose, urinary osmolality with simultaneous plasma osmolality values, and the specific gravity of the urine. A large volume of dilute urine, the osmolality (<300 mOsm/kg) of which is lower than that of plasma, is a strong indication for diabetes insipidus (DI) . A reliable test for the detection of DI is the water deprivation test which needs to be performed under supervision to avoid dangerous dehydration. During the test, healthy people will pass urine with an osmolality significantly higher than the plasma osmolality; urine osmolality does not change much after antidiuretic hormone administration. In patients with DI, the urinary osmolality remains low after water deprivation. It will be raised by antidiuretic hormone administration in central DI, but not in nephrogenic DI . Measurement of antidiuretic hormone levels during the water deprivation test increases the accuracy of diagnosis .
Determination of levels of other pituitary hormones is recommended in cases of traumatic head injury . Magnetic resonance imaging (MRI) may aid in the diagnosis of central DI because the hyperintense signal seen in T1-weighted images of a healthy posterior pituitary gland is lost in patients suffering from central DI.
After eliminating two questions about DDAVP treatment, the sum of QOL scores was 15.3 ± 6.5 in untreated CDI patients, 24.4 ± 5.2 in those with nasal treatment, 28.9 ± 4.9 in those with oral DDAVP, and 29.5 ± 3.6 in healthy controls. [ncbi.nlm.nih.gov]
The objective of this study was to investigate the prognosis of adipsic CDI patients. [ncbi.nlm.nih.gov]
[…] idiopathic peripheral DI : increased end-organ resistance to vasopressin congenital renal insensitivity to vasopressin (rare) long-term lithium use (15% of patients) metabolic, e.g. hypokalemia, hypercalcemia chronic kidney disease pregnancy Treatment and prognosis [radiopaedia.org]
Even with intensive treatment, prognosis remains dismal with an average overall survival of 18–26 months ( 12 ). CNS lymphomas in immunocompetent patients have no known risk factors. [edmcasereports.com]
Outlook (Prognosis) Outcome depends on the cause. If treated, central diabetes insipidus usually does not cause severe problems or result in early death. [mountsinai.org]
Their medical records were thoroughly reviewed and their clinical symptoms and signs, laboratory data, and etiologies were analyzed. [ncbi.nlm.nih.gov]
Etiology of Diabetes Insipidus Etiology is a reference to the cause or a set of causes to a specific condition or disorder. It can also refer to the investigation, attri... [diabetesinsipidus.org]
CONTEXT: Epidemiological data for central diabetes insipidus (CDI) are sparse. OBJECTIVE: The purpose of this study was to provide accurate epidemiological data on CDI on a national level. [ncbi.nlm.nih.gov]
A post-infectious disease process and histiocytosis X cause CDI in 2% and 16% of children, respectively. 54 No data on ethnic/racial differences in the epidemiology of CDI are available. 220.127.116.11 Life Expectancy and Mortality In a prospective study of [endocrinefacts.org]
Following the case presentation, we describe the etiology, pathophysiology, diagnosis, and treatment of CDI. [ncbi.nlm.nih.gov]
OVERVIEW Diabetes insipidus (DI) is a condition caused by loss of the effect of antidiuretic hormone on the collecting ducts of the kidneys, resulting in loss of free water. diabetes insipidus can be central or nephrogenic This article will focus on CDI PATHOPHYSIOLOGY [lifeinthefastlane.com]
English, hypothalamic%20diabetes%20insipidus Record 1, Textual support, English Record number: 1, Textual support number: 1 CONT Polyuria is the excretion of dilute urine in excess of 3 liters per 24 hours, and results from one of three fundamental pathophysiologic [btb.termiumplus.gc.ca]
Prevention Many of the cases may not be preventable. Prompt treatment of infections, tumors, and injuries may reduce risk. References Brimioulle S. Diabetes insipidus. In: Vincent J-L, Abraham E, Moore FA, Kochanek PM, Fink MP, eds. [mountsinai.org]
For new-onset CDI with LCH, chemotherapy with cytarabine might be useful for preventing APHD and ND-CNS. [ncbi.nlm.nih.gov]
This controls urine output and fluid balance and prevents dehydration. In mild cases, drinking more water may be all that is needed. [medlineplus.gov]
- Fujiwara TM, Bichet DG. Molecular biology of hereditary diabetes insipidus. J Am Soc Nephrol. 2005;16(10):2836-2346.
- Hedrich CM1, Zachurzok-Buczynska A, Gawlik A, et al. Autosomal dominant neurohypophyseal diabetes insipidus in two families. Molecular analysis of the vasopressin-neurophysin II gene and functional studies of three missense mutations. Horm Res. 2009;71(2):111-119.
- Kristof RA, Rother M, Neuloh G, Klingmüller D. Incidence, clinical manifestations, and course of water and electrolyte metabolism disturbances following transsphenoidal pituitary adenoma surgery: a prospective observational study. J Neurosurg. 2009;111(3):555-562.
- Hadjizacharia P, Beale EO, Inaba K, Chan LS, Demetriades D. Acute diabetes insipidus in severe head injury: a prospective study. J Am Coll Surg. 2008;207(4):477-484.
- Seckl J, Dunger D. Postoperative diabetes insipidus. BMJ. 1989;298(6665):2-3.
- Di Iorgi N, Napoli F, Allegri AE, et al. Diabetes insipidus--diagnosis and management. Horm Res Paediatr. 2012;77(2):69-84.
- Zerbe RL, Robertson GL. A comparison of plasma vasopressin measurements with a standard indirect test in the differential diagnosis of polyuria. N Engl J Med. 1981;24;305(26):1539-1546.
- Krahulik D, Zapletalova J, Frysak Z, Vaverka M. Dysfunction of hypothalamic-hypophysial axis after traumatic brain injury in adults. J Neurosurg. 2010;113(3):581-584.