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Central Diabetes InsipidusAka: Central DI

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  1. Definition
    1. Antidiuretic Hormone (ADH) deficiency
  2. Pathophysiology
    1. Polyuria
      1. Decreased ADH release
    2. Permanent Polyuria
      1. Central lesion above median eminence
    3. Transient Polyuria
      1. Central lesion below median eminence
      2. ADH passes via hypothalamus to portal capillaries
      3. Results in ADH release below the median eminence
  3. Etiology
    1. Idiopathic (30%)
      1. Autoimmune disease (common)
        1. Lymphocyte inflammation
          1. Pituitary stalk (thickened stalk on MRI)
          2. Posterior pituitary
        2. Anterior Pituitary deficiency
          1. Growth Hormone
          2. ACTH deficient
      2. Familial Diabetes Insipidus (very rare)
        1. Point mutation in ADH precursor gene
          1. Precursor accumulates
          2. Toxicity to ADH synthesizing cells
        2. Enhancement within hypothalamus on MRI
    2. Neurosurgery (Transsphenoidal)
      1. Results from Hypothalamus or Pituitary trauma
      2. Most common cause of polyuria post neurosurgery
      3. Differential Diagnosis
        1. Excess fluids
        2. Mannitol
        3. Corticosteroids
      4. Approach
        1. Check Urine Osmolality
        2. Observe response to water restriction
    3. Malignancy
      1. Examples: Lung Cancer, Leukemia, Lymphoma
      2. Polyuria may be presenting symptom
    4. Langerhans Histiocytosis (Histiocytosis X)
      1. Infiltrative disease
      2. Sarcoidosis causes similar infiltration
    5. Post SVT resolution
    6. Anorexia Nervosa
    7. Pregnancy exacerbates any of above forms
  4. Diagnosis
    1. Fluid Deprivation Test
      1. No response to water deprivation
      2. Response to exogenous ADH administration
    2. Hare-Hickey Test
      1. Decreased ADH to Serum Osmolality ratio
  5. Radiology: MRI Head
    1. Central DI: Diminished signal at posterior pituitary
  6. Management
    1. General Measures that potentiate ADH
      1. Low sodium diet
      2. NSAIDs
    2. dDAVP (Desmopressin)
      1. Synthetic ADH replacement hormone
      2. dDAVP 10 to 20 ug bid intranasally
    3. Chlorpropamide 125-250 mg PO qd-bid
      1. Antidiuretic effect - may lower Urine output by 50%
      2. Risk of Hypoglycemia at higher doses
    4. Carbamazepine 100-300 mg bid
      1. Enhances ADH response
      2. May lower urine output by 50%
    5. Hydrochlorothiazide with low salt intake
      1. Decreases polyuria
      2. Dose: 25 mg qd to bid

Central Diabetes Insipidus (C0687720)

Definition (MSH)A genetic or acquired polyuric disorder caused by a deficiency of VASOPRESSINS secreted by the NEUROHYPOPHYSIS. Clinical signs include the excretion of large volumes of dilute URINE; HYPERNATREMIA; THIRST; and polydipsia. Etiologies include HEAD TRAUMA; surgeries and diseases involving the HYPOTHALAMUS and the PITUITARY GLAND. This disorder may also be caused by mutations of genes such as ARVP encoding vasopressin and its corresponding neurophysin (NEUROPHYSINS).
ConceptsDisease or Syndrome (T047)
ICD9253.5
MSHD020790
Englishantidiuretic hormone defective syndrome, Central Diabetes Insipidus, Cranial diabetes insipidus, Diabetes insipidus - pituitary, Diabetes insipidus secondary to vasopressin deficiency, Neurogenic diabetes insipidus, Neurohypophyseal diabetes insipidus, Pituitary diabetes insipidus, Primary central diabetes insipidus, vasopressin defective diabetes insipidus, Vasopressin deficiency, Vasopressin deficiency syndrome
Spanishdiabetes insipida craneal, diabetes insipida neurogenica, diabetes insipida neurohipofisaria, diabetes insipida pituitaria, diabetes insipida primaria central, diabetes insipidus secundaria a deficiencia de vasopresina, sindrome de deficiencia de vasopresina
Parent ConceptsDiabetes Insipidus (C0011848), Duplicate concept (C1274013), Ambiguous concept (C1274012)
SourcesCSP, DXP, MSH, MTH, MTHICD9, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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