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 [1]. The pattern of inheritance may be autosomal dominant [2], 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 [3]. Of patients with serious head injuries, about 15% developed central DI. The most important risk factors were severe head trauma and penetrating trauma [4].

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.

• Fever

  A 58-year-old woman was admitted due to visual field abnormalities, thirst, polydipsia, polyuria and fever. [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]

  Symptoms may include: Excessive thirst Excessive urine production Dehydration Infants with diabetes insipidus may also exhibit the following symptoms: Irritability Poor feeding Failure to grow High fevers The symptoms of diabetes insipidus may resemble [stanfordchildrens.org]

• Fatigue

  We here report a 77-year-old Japanese male who suffered general fatigue with progressive thirst and polyuria. Central diabetes insipidus was diagnosed by depletion of vasopressin secretion in response to increases in serum osmolality. [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]

• Enuresis

  Abstract Nocturnal enuresis is a common symptom in children. It is usually attributed to benign causes and diagnostic evaluation is not carried out. [ncbi.nlm.nih.gov]

  New onset nocturia and enuresis in young children is also frequently seen in DI. Age of onset is important to consider when evaluating a patient with suspected DI. [pedclerk.bsd.uchicago.edu]

  Affected children may develop bedwetting (enuresis), fatigue, weight loss, and growth retardation. [rarediseases.org]

  A careful history should focus on establishing daily fluid intake and urinary output, including an assessment for nocturia and enuresis. [clinicaladvisor.com]

• Asymptomatic

  AVP gene testing also has the potential to identify which asymptomatic children will later develop FNDI. [ncbi.nlm.nih.gov]

  Agents such as chlorpropamide or thiazide diuretics can be useful when only a modest decrease in urine volume will make the patients asymptomatic. [endocrinologyadvisor.com]

• 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]

• Rhinitis

  Absorption from the nasal mucosa may be erratic, especially when an upper respiratory infection or allergic rhinitis occurs. [msdmanuals.com]

• Polydipsia

  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]

  Apart from nephrogenic DI, central DI needs to be differentiated from psychogenic polydipsia. [symptoma.com]

• Loss of Appetite

  Symptoms of an electrolyte imbalance may include: Weakness Nausea Vomiting Loss of appetite Muscle cramps Confusion Feb. 16, 2019 [mayoclinic.org]

  Signs of low levels of sodium in the blood include: loss of appetite, severe nausea, vomiting, severe headache, mental and mood chances, muscle weakness, cramps and spasms, shallow breathing and loss of consciousness. [draxe.com]

• Abdominal Pain

  Abdominal pain. Nausea. A blocked or runny nose. Nosebleeds. If you take too much desmopressin or drink too much fluid while taking it, it can cause your body to retain too much water, which can cause the following symptoms: Headaches Dizziness. [my.clevelandclinic.org]

• Hypotension

  Affected adults may develop orthostatic hypotension, a condition in which there is a dramatic decrease in blood pressure upon standing or sitting. Orthostatic hypotension can result in dizziness or momentary loss of consciousness (syncope). [rarediseases.org]

  Note that despite absent or markedly blunted AVP responses to hyperosmolality, patients with osmoreceptor dysfunction respond normally to baroreceptor stimulation induced by hypotension. [slideshare.net]

  Dehydration is continued until orthostatic hypotension and postural tachycardia appear, ≥ 5% of the initial body weight has been lost, or the urinary concentration does not increase > 0.001 specific gravity or > 30 mOsm/L in sequentially voided specimens [msdmanuals.com]

  This patient’s baroreflex-mediated vasopressin release was not formally tested, but he was not able to concentrate his urine and developed hypernatremia despite relative hypotension and volume depletion at the time of admission. [link.springer.com]

• Tachycardia

  Dehydration is continued until orthostatic hypotension and postural tachycardia appear, ≥ 5% of the initial body weight has been lost, or the urinary concentration does not increase > 0.001 specific gravity or > 30 mOsm/L in sequentially voided specimens [msdmanuals.com]

  Our patient’s tachycardia and relatively low blood pressure suggest volume depletion that is further corroborated by the elevated serum creatinine and contraction alkalosis. Notably, he had hyperglycemia but no ketoacidosis. [link.springer.com]

  Her electrocardiogram (ECG) was consistent with sinus tachycardia and initial computed tomogram (CT) scan of the brain and chest X-ray were unremarkable. [cureus.com]

  It has off-label uses and is used in the treatment of gastrointestinal bleeding, ventricular tachycardia and ventricular defibrillation. Vasopressin is used to treat diabetes insipidus related to low levels of antidiuretic hormone. [en.wikipedia.org]

• Orthostatic Hypotension

  Affected adults may develop orthostatic hypotension, a condition in which there is a dramatic decrease in blood pressure upon standing or sitting. Orthostatic hypotension can result in dizziness or momentary loss of consciousness (syncope). [rarediseases.org]

  Dehydration is continued until orthostatic hypotension and postural tachycardia appear, ≥ 5% of the initial body weight has been lost, or the urinary concentration does not increase > 0.001 specific gravity or > 30 mOsm/L in sequentially voided specimens [msdmanuals.com]

• Vascular Disease

  breast, lymphoma, leukaemia) Hypoxic brain injury Brain stem death Profound hyponatraemia -> cerebral oedema Radiotherapy Inflammatory conditions – sickle cell, sarcoid, Wegener’s, histiocytosis X Infections – Tb, abscess, encephalitis, meningitis Vascular [lifeinthefastlane.com]

  The etiology of central diabetes insipidus in children and young adults is highly varied, ranging from autoimmune, vascular diseases, infections, surgical or other trauma [ 12 ], and genetic defects [ 13 ] to infiltrative diseases such as Langerhans’ [link.springer.com]

• Fracture

  Causes of central diabetes insipidus include: Accidental damage to your pituitary gland during surgery A brain injury, especially a fracture to the base of the skull A brain tumor Blocked blood flow to your brain Encephalitis (swelling of your brain caused [msdmanuals.com]

  The deficiency may be Inherited Caused by another disorder Of unknown cause Other disorders that can cause central diabetes insipidus include accidental damage done during surgery on the hypothalamus or pituitary gland; a brain injury, particularly a fracture [merckmanuals.com]

• Seizure

  Abstract A previously healthy 30-day-old girl presented with seizures, irritability and inability to sleep for three days. Vitamin K was not given just after birth. She was lethargic. A multifocal clonic seizure was evident during examination. [ncbi.nlm.nih.gov]

  Seizure 0001250 Percent of people who have these symptoms is not available through HPO Abnormality of metabolism/homeostasis Laboratory abnormality Metabolism abnormality [ more ] 0001939 Autosomal dominant inheritance 0000006 Gliosis 0002171 Hypertelorism [rarediseases.info.nih.gov]

  This can cause an abnormal dilution of the urine, leading to hyponatremia, which can cause drowsiness, seizures and even death. [pituitary.ucla.edu]

  Hypernatraemia Mind map (PDF) CCC – Hyponatraemia CCC – Hyponatraemia Mind map (PDF) CCC – Hyponatraemia Interpretation (PNG) CCC – SIADH – SIADH DDx CCC – Diabetes Insipidus Central – Diabetes Insipidus DDx Case – Exercise-associated Hyponatremia Case – Seizures [lifeinthefastlane.com]

• Irritability

  Abstract A previously healthy 30-day-old girl presented with seizures, irritability and inability to sleep for three days. Vitamin K was not given just after birth. She was lethargic. A multifocal clonic seizure was evident during examination. [ncbi.nlm.nih.gov]

  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. [symptoma.com]

  […] often drinking more than 1 gallon of 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 [webmd.com]

  Symptoms may include: Excessive thirst Excessive urine production Dehydration Infants with diabetes insipidus may also exhibit the following symptoms: Irritability Poor feeding Failure to grow High fevers The symptoms of diabetes insipidus may resemble [stanfordchildrens.org]

• Lethargy

  She had a 3-month history of lethargy, reduced appetite, weight loss, cough and intermittent fevers. [ncbi.nlm.nih.gov]

  If you lose too much water, you could have: Unexplained weakness Lethargy Muscle pains Irritability How Is It Diagnosed? If you have this condition, you’ll probably wind up at the doctor for help with your thirst and constant need for a bathroom. [webmd.com]

  At the time of presentation, he had exhibited progressive lethargy over 2 weeks. While he was in school, his teachers noted that he was more awake and interactive if urged to drink cold water. [link.springer.com]

  Nocturia 0000017 Polydipsia 0001959 Weight loss 0001824 30%-79% of people have these symptoms Anxiety Excessive, persistent worry and fear 0000739 Depressivity Depression 0000716 Excessive daytime somnolence 0001262 Fever 0001945 Headache Headaches 0002315 Lethargy [rarediseases.info.nih.gov]

• Convulsions

  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]

• Polyuria

  We report, to our knowledge, the first case of new-onset symptomatic CDI causing nocturnal polyuria in an ADPKD patient with ESRD, before now on hemodialysis. [ncbi.nlm.nih.gov]

• Nocturia

  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]

  The authors present the case of a 37-year-old male, diagnosed of CVID, who had thirst, polyuria and nocturia for several years. [ncbi.nlm.nih.gov]

  Symptoms • Nocturia is often the first sign of CDI. • This is because urine is usually most concentrated in the morning due to lack of fluid ingestion overnight. • As a result, nocturia is usually the first manifestation of a loss of concentrating ability [slideshare.net]

• Nocturnal Enuresis

  Abstract Nocturnal enuresis is a common symptom in children. It is usually attributed to benign causes and diagnostic evaluation is not carried out. [ncbi.nlm.nih.gov]

• Urinary Incontinence

  Previous case reports often refer the occurrence of polyuria or ‘urinary incontinence’ later in the disease course, being frequently preceded by confusion, epileptic seizures and/or focal neurological signs ( 4 ). [edmcasereports.com]

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) [5]. 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 [6]. Measurement of antidiuretic hormone levels during the water deprivation test increases the accuracy of diagnosis [7].

Determination of levels of other pituitary hormones is recommended in cases of traumatic head injury [8]. 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]

[…] isotonic saline if hypernatraemia is > 155 -> then also use hypertonic saline to reduce the rate of reduction ADH supplementation if patient is euvolaemic -> DDAVP (V2 receptor agonist) 1-4mcg/day IV/IM/SC/IN can infuse and titrate against urine output PROGNOSIS [lifeinthefastlane.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]

CONCLUSIONS: Etiological diagnosis is possible in a significant proportion (70.6%) of children with central DI. [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. 1.2.1.3 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]

Timely diagnosis and treatment with lyophilized desmopressin may prevent severe morbidity and mortality. [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]

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