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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־´Â 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.

<Á¤¸®>

* 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־´Â ±Ý±âÀÌ´Ù

14ÆÇ¿¡¼­´Â clofibrate, carbamazepineÀÌ Ä¡·á¿¡ ÀÖ¾úÀ¸³ª 15ÆÇ¿¡¼­´Â ºüÁü