- Hypokalemia
- Hypothyroidism
- Myasthenia Gravis
- Systemic Lupus Erythematosus
CODES
ICD9
- 710.3 Dermatomyositis
- 710.4 Polymyositis
ICD10
- M33.20 Polymyositis, organ involvement unspecified
- M33.90 Dermatopolymyositis, unspecified, organ involvement unspecified
- M33.92 Dermatopolymyositis, unspecified with myopathy
DIABETES INSIPIDUS
Melissa H. White
•
Carolyn Maher Overman
BASICS
DESCRIPTION
- Disorder in which large volumes of dilute urine are excreted (polyuria) as an inappropriate response to arginine vasopressin (AVP)
- Polyuria defined as >3 L in 24 hr
- Often characterized by excessive fluid intake (polydipsia)
- 2 types:
- Central diabetes insipidus (DI, CDI; failure or deficiency of AVP release):
- 4 types:
- No AVP to release (loss or malfunction of posterior pituitary neurons)
- Defective osmoreceptors—release AVP only in response to severe dehydration
- Elevated threshold for AVP release
- Subnormal amount of AVP released
- Familial cases have been reported (autosomal dominant).
- Nephrogenic DI (lack of renal response to AVP):
- Differentiate from primary polydipsia.
- Some cases are X-linked recessive in males.
ETIOLOGY
- Central DI:
- Any condition that disrupts the osmoreceptor-hypothalamus-hypophyseal axis:
- Highest incident in ages 10–20 yr
- Trauma (skull fractures, hemorrhage)
- Pituitary or hypothalamic surgery
- CNS neoplasm: DI can be considered a tumor marker:
- Pituitary adenomas
- Craniopharyngiomas
- Germinomas
- Pinealomas
- Meningiomas
- Metastatic tumors:
- Granulomatous:
- Congenital CNS defects
- CNS infections (e.g., meningitis, encephalitis)
- Pregnancy (Sheehan syndrome)
- Idiopathic (autoantibodies, occult tumor)
- Wolfram syndrome (DI, DM, optic atrophy, deafness)
- Ethanol
- Nephrogenic DI:
- Any condition that disrupts the kidney:
- Congenital renal disorders
- Obstructive uropathy
- Renal dysplasia
- Polycystic kidney disease
- Systemic disease with renal involvement
- Sickle cell disease
- Sarcoidosis
- Amyloidosis
- Drugs:
- Amphotericin
- Phenytoin
- Lithium (most common and persists past discontinuation of drug)
- Aminoglycosides
- Methoxyflurance
- Demeclocycline
- Electrolyte disorders:
Pregnancy Considerations
- Transient in the 2nd trimester:
- Unclear etiology, but there is an increase of circulating vasopressinase.
- Leads to a decrease in AVP and transient DI
- Watch patient closely during anesthesia and periods of water restriction.
- Typically clears after 2–6 wk after delivery
- Desmopressin (DDAVP) resists this vasopressinase.
- Sheehan syndrome may cause DI.
DIAGNOSIS
SIGNS AND SYMPTOMS
History
- Polyuria (up to 16–24 L/d of urine):
- Note the voiding frequency.
- Polydipsia (often craves cold fluids):
- Note the amount of PO fluid intake per day.
- Drug ingestion
- Signs and symptoms of hypothalamic tumors:
- Headache
- Visual disturbances
- Growth disturbances
- Obesity
- Hyperpyrexia
- Sleep disturbances
- Sexual precocity
- Emotional disturbances
Physical-Exam
- Dehydration
- Cachexia
- Head trauma
- Visual field defects
- Seizures
Pediatric Considerations
- Polyuria and polydipsia may not be recognized by caregivers until symptoms of dehydration develop.
- In neonates:
- Often present at birth
- If unrecognized, dehydration and hypernatremia may cause permanent CNS damage.
- In infants:
- Irritability
- Poor feeding/weight loss
- Constipation
- Growth failure
- Intermittent high fever
- Abnormal behavior (hyperactivity, restlessness, excessive crying)
- In children:
- Enuresis
- Difficulty with toilet training
ESSENTIAL WORKUP
- Clinical diagnosis in the ED:
- Elevated serum sodium concentration
- Copious amounts of dilute urine
- History:
- Usually an increased amount of PO fluid intake per day
- Voiding frequency
- Medication use history
- Physical exam
- Labs below
DIAGNOSIS TESTS & NTERPRETATION
Lab
- Urinalysis:
- Specific gravity will be low.
- Serum and urine osmolality:
- High serum osmolality
- Low urine osmolality
- Electrolytes, BUN, creatinine, and glucose:
- Hypernatremia
- Hypercalcemia
- Hypokalemia
- CBC:
- Anemia may be a sign of a neoplasm.
- Serum and urine AVP tests are expensive and unnecessary in the ED.
Imaging
- As needed to evaluate for trauma or search for neoplasm
- CXR
- CT of brain
- MRI of pituitary axis is usually outpatient.
Diagnostic Procedures/Surgery
Water deprivation test (dehydration test):
- Unnecessary in the emergency setting
- Can be dangerous in cases of hypotension or small children
- Performed as a confirmatory test for those receiving treatment
- Measures urine and plasma osmolality after fluid restriction
- Urine osmo <300 is significant for DI
- Desmopressin is administered
- Central DI—urine osmo increased by >50%
- Nephrogenic DI—urine osmo increased by <50%
- Further testing is needed if urine osmo 300–800
- Primary polydipsia if urine osmo >800
DIFFERENTIAL DIAGNOSIS
- Primary water deficit:
- Inadequate access to free water
- Increased insensible water loss (e.g., premature infants)
- Inadequate breast-feeding
- Primary sodium excess:
- Excessive sodium bicarbonate during resuscitation
- Hypernatremic enemas
- Ingestion of seawater
- Hypertonic saline administration
- Accidental substitution of salt (sodium chloride) for glucose in infant formulas
- Intentional salt poisoning
- High breast milk sodium
- Primary polydipsia (psychogenic polydipsia):
- Solute-induced polyuria
- Diuretic use
- Resolving acute renal failure
- Osmotic diuresis
- Uncontrolled
DM
TREATMENT
PRE HOSPITAL
- ABCs
- Immobilize if trauma is suspected.
- Serum blood glucose
- IV access and fluids if signs of dehydration exist
- Control seizures according to medical direction guidelines.
INITIAL STABILIZATION/THERAPY
- Manage ABCs.
- Manage traumatic injuries accordingly.
- High index of suspicion for head trauma
ED TREATMENT/PROCEDURES
- Correction of hypotension:
- Use of 0.9% NaCl is indicated for shock.
- Intravascular losses represent only about 1/12 of total water losses.
- Central DI (vasopressin deficient):
- AVP (aqueous vasopressin):
- Half-life is too short.
- May induce coronary vasospasm
- Used only for dehydration test
- Lysine vasopressin (lypressin):
- Can be given intranasally
- Frequent instillation needed
- Desmopressin:
- Drug of choice to control symptoms
- Administer intranasally, SC, IV, or PO in 2 divided doses as necessary to control polyuria or polydipsia.
- Caution in postoperative patients as cerebral edema may develop
- Chlorpropamide (Diabinese):
- Enhances effect of vasopressin at renal tubule
- May stimulate AVP release
- Useful only in partial CDI
- Clofibrate stimulates the release of endogenous vasopressin.
- Nephrogenic DI:
- Diuretics:
- Induce natriuresis
- Thiazides 1st line
- Amiloride often used in combination with thiazides
- Dietary sodium restriction
- Restrict solutes and avoid excessive drinking to prevent water intoxication.
- Avoid alcohol (especially beer) intake.
- Check daily weights.
- NSAIDs (indomethacin)
- Parenteral correction of initial water deficit in cases where PO is not an option:
- Usually only in symptomatic hypernatremic cases
- For fluid replacement, refer to “Hypernatremia.”