Diabetes Insipidus
1. ÀÓ»óƯ¡
AVPºÐºñ ȤÀº ÀÛ¿ëÀÌ °¨¼ÒÇÏ¿© ¹ß»ýÇÏ¸ç ºñÁ¤»óÀûÀ¸·Î ¸¹Àº ¾çÀÇ Èñ¼®µÈ ¼Òº¯ÀÌ Æ¯Â¡ÀÌ´Ù.
24hr urine volume > 50 mL/kg, U-osmolality < 300 mmol/kg
polyuria, urinary frequency, enuresis and/or nocturia, ÀÌ·Î ÀÎÇÑ sleep disturbance, mild
daytime fatigue or somnolence°¡ »ý±ä´Ù. polydipsia¸¦ ÅëÇØ º¸»óµÇ¹Ç·Î dehydration sign
Àº ¾ø´Ù.
2. ¿øÀÎ
1) deficient secretion : primary or secondary
(1) primary deficiency: agenesis or irreversible destruction of the neurohypophysis
= neurohypophyseal DI, neurogenic DI, pituitary DI, cranial DI, or central DI
congenital, acquired, or genetic disorder¿¡ ¹ß»ýÇÏÁö¸¸ ¹ÝÁ¤µµ´Â idiopathic.
¨ç genetic form : ÈçÈ÷ autosomal dominant mode & diverse mutation
(AVP-neurophysin II gene)
¨è gestational DI : ÀÓ½ÅÁß¿¡ ¹ß»ýÇϹǷΠgestational DI¶ó ÇÏ¸ç ºÐ¸¸ÈÄ ¼öÁÖ³»¿¡ ÈçÈ÷
ȸº¹µÈ´Ù. ±×·¯³ª subclinical AVP deficiency°¡ ÈçÈ÷ °üÂûµÈ´Ù.
(2) secondary deficiency: excessive fluid intake¿¡ ÀÇÇØ 2Â÷ÀûÀ¸·Î AVPºÐºñ°¡ ¾ïÁ¦µÇ¾î
¹ß»ý
¨ç primary polydipsia
i) dipsogenic DI: osmoregulatory mechanismÀÇ "set" reductionÀ¸·Î ÀÎÇÏ¿©
ºñÁ¤»óÀûÀÏ Á¤µµ·Î thirstÁõ°¡
ii) psychogenic polydipsia: psychosis·Î ÀÎÇÑ polydipsia
iii) iatrogenic polydipsia: ÀÇ·áÀο¡°Ô Ä¡·á ¹× ¿¹¹æÀû ¸ñÀûÀ¸·Î ¹°À» ¸¹ÀÌ ¸¶½Ç °ÍÀ»
À̾߱⸦ µéÀºÈÄ ¸¹ÀÌ ¸¶½É.
¨è drug: lithium, carbamazepine
2) nephrogenic DI : genetic, acquired, or drug
¨ç genetic form : ÈçÈ÷ X-linked mode, V2 receptor gene mutation
¨è drug : lithium, demeclocycline, methoxyflurane, amphotericin B, aminoglycoside,
cisplatin, rifampin, foscarnet
(¾Ï±â: ¸¶Ãë[flurane]ÇÒ ¶§ ABC-R lithiumÀ» ¾²¸é DI°¡ »ý±ä´Ù)
¨é metabolic: hypercalcemia, hypercalciuria, hypokalemia
3. º´Å»ý¸®
AVPÀÇ ºÐºñ ȤÀº ÀÛ¿ëÀÌ 80-85%ÀÌ»ó °¨¼ÒÇÏ¸é ¼Òº¯À» ³óÃà½ÃŰ±â¿¡ ºÒÃæºÐÇÏ°Ô µÈ´Ù.
AVP defect°¡ ÀÏÂ÷ÀûÀ̶ó¸é(pituitary, gestational, or nephrogenic DI) polyuria¿¡ µÚÀ̾î
body water°¡ ¾à°£ °¨¼ÒÇϰí(1-2%), Áõ°¡µÈ plasma osmolality & sodium concentration
À¸·Î ÀÎÇÏ¿© °¥ÁõÀÌ ÀÚ±ØµÇ¾î º¸»óÀûÀ¸·Î water intake°¡ Áõ°¡ÇÏ°Ô µÈ´Ù. ±× °á°ú thirst
defect°¡ ¾ø´ÂÇÑ dehydration signÀº ¾ø´Ù.
»ç¶÷¸¶´Ù ¿øÀο¡ µû¶ó ºÐºñ ¹× ÀÛ¿ëÀÇ ¼Õ»óÁ¤µµ°¡ ´Ù¸£Áö¸¸ ½ÉÇÑ °æ¿ì basal urine outputÀÌ
ÃÖ´ë 10-15 mL/min°¡ µÇ±âµµ ÇÑ´Ù.
* Fig 329-3 water deprevation test
i) primary polydipsia : water depreviationÀ¸·Î ¼Òº¯ÀÌ ÃÖ´ë·Î ³óÃàµÇ¾ú´Ù°¡ AVPÅõ¿©ÈÄ
¿¡´Â º¯È°¡ ¾ø´Ù.
ii) pituitary DI : water deprivationÀ¸·Î ¼Òº¯ÀÌ ¾à°£ ³óÃàµÇ¾ú´Ù°¡ AVPÅõ¿©ÈÄ¿¡ Å©°Ô
³óÃàµÈ´Ù.
iii) nephrogenic DI: water deprivationÀ¸·Î ¼Òº¯ÀÌ ¾à°£ ³óÃàµÇ¾ú´Ù°¡ AVPÅõ¿©ÈÄ¿¡ º°
¹ÝÀÀ¾ø´Ù.
4. °¨º°Áø´Ü
24hr urine output >50 mL/kg/d(>3500 mL in a 70 kg man)ÀÏ ¶§ DI¸¦ ÀǽÉÇÑ´Ù.
24hr urine osmolality >300 mosmol/kg¶ó¸é solute diuresis¸¦ ÀǹÌÇϸç uncontrolled DM or
other less common causes of excessive solute excretion¿¡ ´ëÇØ Á¶»çÇØ¾ß ÇÑ´Ù.
300 mosmol/kg¹Ì¸¸À̶ó¸é water diuresis¸¦ ÀǹÌÇϸç DI type¿¡ ´ëÇØ Á¶»çÇÑ´Ù.
fluid deprevationÈÄ urine concentration(urine osmol >300 mosmol/kg, SG>1.010)µÇÁö
¾ÊÀ¸¸é primary polydipsia & partial AVP secretion or action defect´Â ¹èÁ¦ÇÒ¼ö ÀÖ´Ù.
ÀÌ¿Í °°ÀÌ ¼Òº¯ÀÌ ³óÃàµÇÁö ¾Ê´Â °æ¿ì¿£ severe pituitary or nephrogenic DI¸¦ »ý°¢Çϴµ¥
ÀÌÀÇ °¨º°À» À§Çؼ desmopressin(DDAVP, 0.03 ug/kg SC or IV)dmf Åõ¿©ÇÑÈÄ 1-2½Ã°£ÈÄ
urine osmolality¸¦ ÃøÁ¤ÇÑ´Ù.
50%ÀÌ»ó Áõ°¡Çϸé severe pituitary DI¸¦ ÀǹÌÇÏ¸ç ¹ÝÀÀÀÌ À۰ųª ¾øÀ¸¸é nephrogenic DI¸¦
ÀǹÌÇÑ´Ù. ±×·¯³ª ÀÌ·¯ÇÑ indirect criteria´Â ¼Òº¯ÀÌ ³óÃàµÉ °æ¿ì¿¡ À־ Áø´Ü¿¡ µµ¿òÀÌ
µÇÁö ¸øÇÑ´Ù. ¿Ö³ÄÇϸé primary polydipsia, partial pituitary and partial nephrogenic DI¿¡¼
urine osmolality°¡ À¯»çÇϱ⠶§¹®ÀÌ´Ù. ÀÌ·± °æ¿ì °¨º°À» À§Çؼ fluid deprivationÀüÈÄ·Î
plasma or urine AVP¸¦ ÃøÁ¤Çϸé partial nephrogenic DI¿Í partial pituitary DI & primary
polydipsia¸¦ °¨º°ÇÒ¼ö ÀÖ´Ù.
¶ÇÇÑ plasma osmolality or sodiumÀÌ normal rangeÀÌ»óÀÏ ¶§ pituitary DI¿Í primary
polydipsia¸¦ °¨º°ÇÒ¼ö ÀÖ´Ù. ±×·¯³ª ¼Òº¯ÀÌ ³óÃàµÇ¾úÀ» ¶§ fluid deprivation´Üµ¶À¸·Î´Â ÃæºÐ
ÇÑ hypertonic dehydrationÀ» ¾ò±â ¾î·Æ´Ù. ±×·¯¹Ç·Î ÀϹÝÀûÀ¸·Î plasma osmolality°¡ 300
mmol/kg(Na>145mmol/L)ÀÌ»ó µÇ¾úÀ»¶§ hypertonic(3%) saline infusionÇÏ¿© ¹Ýº¹Çؼ AVP
¸¦ ÃøÁ¤ÇÒ Çʿ䰡 ÀÖ´Ù.
DI °¨º°Áø´Ü¿¡ ÀÖ¾î pituitary and hypothalamus MRI°¡ µÇ¿òÀÌ µÇ±âµµ ÇÑ´Ù. °Ç°Àο¡¼
posterior pituitary´Â T1 WI¿¡¼ hyperintense signalÀ» º¸À̴µ¥ ÀÌ·¯ÇÑ "bright spot"ÀÌ
pituitary DI¿¡¼´Â ¼Ò½ÇµÇ°Å³ª ºñÁ¤»óÀûÀ¸·Î ÀÛÁö¸¸ primary polydipsiaȯÀÚ¿¡¼´Â 80-90%
¿¡¼ °üÂûµÈ´Ù. ±×·¯¹Ç·Î ÀÌ·¯ÇÑ bright spotÀÇ Á¸Àç´Â pituitary DIÀÇ Á¸À縦 ¹èÁ¦ÇÒ ¼ö
ÀÖÀ¸³ª ¹Ý¸é À̰ÍÀÌ ¾ø´Ù°í ÇØ¼ pituitary DI¶ó°í ÇÒ ¼ö´Â ¾ø´Ù. ±×·¯¹Ç·Î MRI¸¦ ÇØ¼®Çϴµ¥
ÁÖÀǸ¦ ¿äÇÑ´Ù.
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* DI Áø´Ü ¹× °¨º°Áø´Ü¿¡ »ç¿ëÇÏ´Â °Ë»çµé
i) water deprivation test(=dehydration test)
ii) hypertonic saline infusion test
iii) plasma or urine AVP
iii) pituitary & hypothalamus MRI
5. Ä¡·á
¨ç DDAVP : synthetic analogue of AVP
uncomplicated pituitary DIÀÇ Áõ»óÀ» ¿ÏÀüÈ÷ ¾ø¾Ù¼ö ÀÖ´Ù.
V2 receptor¿¡ ¼±ÅÃÀûÀ¸·Î ÀÛ¿ëÇÏ¿© ¿ë·®¿¡ ºñ·ÊÇÏ¿© ¼Òº¯À» ³óÃà½Ã۰í urine flow¸¦
°¨¼Ò½ÃŲ´Ù.
IV or SC injection, nasal inhalation, oral tabletÀ¸·Î »ç¿ëÇÒ¼ö ÀÖ´Ù.
¨è chlorpropamide(Diabinese)
pituitary DI´Â chlorpropamide·Î Ä¡·áÇÒ¼öµµ Àִµ¥ ÀÌ ¾àÀÇ Ç×ÀÌ´¢ÀÛ¿ë ±âÀüÀº ºÐ¸íÄ¡´Â
¾ÊÀ¸³ª ¼Ò·®ÀÇ AVPÀÛ¿ëÀ» °È½ÃŰ°Å³ª V2 receptor¸¦ direct activation½ÃŰ´Â °ÍÀ¸·Î
»ý°¢µÈ´Ù. gestational DI Ä¡·á¿¡ À־ ±Ý±âÀÌ´Ù
14ÆÇ¿¡¼´Â clofibrate, carbamazepineÀÌ Ä¡·á¿¡ ÀÖ¾úÀ¸³ª 15ÆÇ¿¡¼´Â ºüÁü