Sheehan's syndrome medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Sheehan's syndrome}} | {{Sheehan's syndrome}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{IQ}}. | ||
==Overview== | ==Overview== | ||
Treatment involves appropriate [[hormone replacement therapy]] that results in complete recovery and reversal of symptoms. | |||
==Medical Therapy== | ==Medical Therapy== | ||
*Treatment involves appropriate hormone replacement therapy, which must be taken for the rest of your life that results in significant improvement of not only the physical symptoms but also the psychological symptoms.<ref name="pmid27034575">{{cite journal |vauthors=Parikh R, Buch V, Makwana M, Buch HN |title=The price of a 15-year delay in diagnosis of Sheehan's syndrome |journal=Proc (Bayl Univ Med Cent) |volume=29 |issue=2 |pages=212–3 |year=2016 |pmid=27034575 |pmc=4790577 |doi= |url=}}</ref> | *Treatment involves appropriate [[hormone replacement therapy]], which must be taken for the rest of your life that results in significant improvement and reversal of not only the physical symptoms, but also the [[psychological]] symptoms.<ref name="pmid27034575">{{cite journal |vauthors=Parikh R, Buch V, Makwana M, Buch HN |title=The price of a 15-year delay in diagnosis of Sheehan's syndrome |journal=Proc (Bayl Univ Med Cent) |volume=29 |issue=2 |pages=212–3 |year=2016 |pmid=27034575 |pmc=4790577 |doi= |url=}}</ref><ref name="pmid19697029">{{cite journal |vauthors=Laway BA, Bhat JR, Mir SA, Khan RS, Lone MI, Zargar AH |title=Sheehan's syndrome with pancytopenia--complete recovery after hormone replacement (case series with review) |journal=Ann. Hematol. |volume=89 |issue=3 |pages=305–8 |year=2010 |pmid=19697029 |doi=10.1007/s00277-009-0804-9 |url=}}</ref><ref name="pmid15921942">{{cite journal |vauthors=Tanriverdi F, Unluhizarci K, Kula M, Guven M, Bayram F, Kelestimur F |title=Effects of 18-month of growth hormone (GH) replacement therapy in patients with Sheehan's syndrome |journal=Growth Horm. IGF Res. |volume=15 |issue=3 |pages=231–7 |year=2005 |pmid=15921942 |doi=10.1016/j.ghir.2005.03.005 |url=}}</ref><ref name="pmid9059561">{{cite journal |vauthors=Bülow B, Hagmar L, Mikoczy Z, Nordström CH, Erfurth EM |title=Increased cerebrovascular mortality in patients with hypopituitarism |journal=Clin. Endocrinol. (Oxf) |volume=46 |issue=1 |pages=75–81 |year=1997 |pmid=9059561 |doi= |url=}}</ref><ref name="pmid12675508">{{cite journal |vauthors=Arafah BM |title=Medical management of hypopituitarism in patients with pituitary adenomas |journal=Pituitary |volume=5 |issue=2 |pages=109–17 |year=2002 |pmid=12675508 |doi= |url=}}</ref><ref name="pmid20719838">{{cite journal |vauthors=Grossman AB |title=Clinical Review#: The diagnosis and management of central hypoadrenalism |journal=J. Clin. Endocrinol. Metab. |volume=95 |issue=11 |pages=4855–63 |year=2010 |pmid=20719838 |doi=10.1210/jc.2010-0982 |url=}}</ref><ref name="pmid1986026">{{cite journal |vauthors=Esteban NV, Loughlin T, Yergey AL, Zawadzki JK, Booth JD, Winterer JC, Loriaux DL |title=Daily cortisol production rate in man determined by stable isotope dilution/mass spectrometry |journal=J. Clin. Endocrinol. Metab. |volume=72 |issue=1 |pages=39–45 |year=1991 |pmid=1986026 |doi=10.1210/jcem-72-1-39 |url=}}</ref><ref name="pmid16584509">{{cite journal |vauthors=Arlt W, Rosenthal C, Hahner S, Allolio B |title=Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements |journal=Clin. Endocrinol. (Oxf) |volume=64 |issue=4 |pages=384–9 |year=2006 |pmid=16584509 |doi=10.1111/j.1365-2265.2006.02473.x |url=}}</ref><ref name="pmid17437510">{{cite journal |vauthors=Thomson AH, Devers MC, Wallace AM, Grant D, Campbell K, Freel M, Connell JM |title=Variability in hydrocortisone plasma and saliva pharmacokinetics following intravenous and oral administration to patients with adrenal insufficiency |journal=Clin. Endocrinol. (Oxf) |volume=66 |issue=6 |pages=789–96 |year=2007 |pmid=17437510 |doi=10.1111/j.1365-2265.2007.02812.x |url=}}</ref><ref name="pmid4182323">{{cite journal |vauthors=Martin MM |title=Coexisting anterior pituitary and neurohypophyseal insufficiency. A syndrome with diagnostic implication |journal=Arch. Intern. Med. |volume=123 |issue=4 |pages=409–16 |year=1969 |pmid=4182323 |doi= |url=}}</ref><ref name="pmid2019265">{{cite journal |vauthors=Shibata H, Ogishima T, Mitani F, Suzuki H, Murakami M, Saruta T, Ishimura Y |title=Regulation of aldosterone synthase cytochrome P-450 in rat adrenals by angiotensin II and potassium |journal=Endocrinology |volume=128 |issue=5 |pages=2534–9 |year=1991 |pmid=2019265 |doi=10.1210/endo-128-5-2534 |url=}}</ref><ref name="pmid8015573">{{cite journal |vauthors=White PC |title=Disorders of aldosterone biosynthesis and action |journal=N. Engl. J. Med. |volume=331 |issue=4 |pages=250–8 |year=1994 |pmid=8015573 |doi=10.1056/NEJM199407283310408 |url=}}</ref><ref name="pmid11158009">{{cite journal |vauthors=Miller KK, Sesmilo G, Schiller A, Schoenfeld D, Burton S, Klibanski A |title=Androgen deficiency in women with hypopituitarism |journal=J. Clin. Endocrinol. Metab. |volume=86 |issue=2 |pages=561–7 |year=2001 |pmid=11158009 |doi=10.1210/jcem.86.2.7246 |url=}}</ref><ref name="pmid16478814">{{cite journal |vauthors=Miller KK, Biller BM, Beauregard C, Lipman JG, Jones J, Schoenfeld D, Sherman JC, Swearingen B, Loeffler J, Klibanski A |title=Effects of testosterone replacement in androgen-deficient women with hypopituitarism: a randomized, double-blind, placebo-controlled study |journal=J. Clin. Endocrinol. Metab. |volume=91 |issue=5 |pages=1683–90 |year=2006 |pmid=16478814 |doi=10.1210/jc.2005-2596 |url=}}</ref> | ||
== | === '''ACTH deficiency''' === | ||
==== Acute setting ==== | |||
* Preferred regimen: [[Hydrocortisone]] 100 mg IV bolus, then 300 mg/day IV divided q8hr '''or''' continuous infusion for 48 hours | |||
** Once patient is stable: 50 mg PO q8hr for 6 doses, later on tapered to 30-50 mg/day PO in divided doses | |||
'''Chronic setting''' | |||
* Preferred regimen: [[Hydrocortisone]] 15-25 mg/day PO divided q8-12hr | |||
'''NOTE''' | |||
*Dosage is increased in patients with severe deficiency, increased body weight and in times of [[surgery]], [[illness]], [[Procedure|procedures]], and other [[Stress|stresses]]. | |||
*Unfortunately, there is no established test to assess adequate [[Hormone replacement therapy|hormonal replacement]]. Plasma [[Adrenocorticotropic hormone|ACTH]] measurement and [[serum]]/[[Saliva|salivary]]/[[urinary]] [[Cortisol|cortiso]]<nowiki/>l values are all unreliable. So assessment of adequate hormonal replacement is based on [[clinical]] basis with [[Cushingoid appearance|Cushingoid features]] showing excessive replacement while [[symptoms]] of [[adrenal insufficiency]] suggesting insufficient hormonal replacement. | |||
*[[Glucocorticoid]] replacement can cause [[polyuria]] due to unmasking of underlying [[central diabetes insipidus]]. [[DDAVP]] is the treatment of choice for patients with [[Diabetes insipidus|DI]].<ref name="pmid18797595">{{cite journal |vauthors=Soares DV, Conceição FL, Vaisman M |title=[Clinical, laboratory and therapeutics aspects of Sheehan's syndrome] |language=Portuguese |journal=Arq Bras Endocrinol Metabol |volume=52 |issue=5 |pages=872–8 |year=2008 |pmid=18797595 |doi= |url=}}</ref> | |||
*[[Mineralocorticoids]] are rarely required as [[aldosterone]] secretion is primarily regulated by [[angiotensin II]] and [[potassium]] not by [[Adrenocorticotropic hormone|ACTH]]. | |||
=== '''TSH deficiency''' === | |||
==== Mild hypothyroidism ==== | |||
* [[Levothyroxine]] 1.7 mcg/kg qDay '''or''' 100-125 mcg PO qDay | |||
==== Severe hypothyroidism ==== | |||
* [[Levothyroxine]] 12.5-25 mcg PO qDay and later on dose can be adjusted by 25 mcg/day q2-4 Week PRN | |||
==== NOTE ==== | |||
*In patients with combined [[hypothyroidism]] and [[hypocortisolism]], [[glucocorticoids]] ([[physiologic]] [[doses]] and increased doses in [[stress]]) are replaced before [[thyroid hormone]] replacement, because treating the [[hypothyroidism]] alone by [[levothyroxine]] can worsen the severity of [[Cortisol|cortiso]]<nowiki/>l deficiency by increasing the [[Clearance (medicine)|clearance]] of [[cortisol]]. So, it is important to assess [[adrenal]] function, including [[corticotropin]] ([[Adrenocorticotropic hormone|ACTH]]) reserve, before administering [[T4]] ([[levothyroxine]]). | |||
*American Thyroid Association (ATA) recommends dose adjustment to keep serum [[free T4]] [[concentration]] in upper half of [[reference range]]. | |||
=== '''LH and FSH deficiency''' === | |||
* '''If fertility required:''' | |||
** Such women are offered [[ovulation]] induction. [[Pregnancy]] can be made possible by giving [[exogenous]] [[gonadotropins]] or [[Gonadotropin-releasing hormone|pulsatile GnRH]]. | |||
** Women with [[GnRH|GnRH deficiency]] can be offered either [[Gonadotropin-releasing hormone|pulsatile GnRH]] or [[gonadotropin]] [[therapy]]. | |||
** Women with [[gonadotropin]] deficiency are given [[gonadotropins]] only. | |||
* '''If fertility not required:''' | |||
** Such women are treated with [[Estrogen and Progestin (Hormone Replacement Therapy) (patient information)|estrogen-progestin replacement therapy]] by using the traditional regimen of [[estradiol]] on days 1 through 25 of each month and [[progesterone]] on days 16 through 25 of each month. | |||
** Another regimen includes continuous [[transdermal]] [[estradiol]] throughout the month, with [[progestin]] added days 1 to 10 of the calendar month. | |||
=== '''Androgen replacement''' === | |||
*[[Androgens]] can be given to females having low [[libido]].<ref name="pmid28615049">{{cite journal |vauthors=Matsuzaki S, Endo M, Ueda Y, Mimura K, Kakigano A, Egawa-Takata T, Kumasawa K, Yoshino K, Kimura T |title=A case of acute Sheehan's syndrome and literature review: a rare but life-threatening complication of postpartum hemorrhage |journal=BMC Pregnancy Childbirth |volume=17 |issue=1 |pages=188 |year=2017 |pmid=28615049 |pmc=5471854 |doi=10.1186/s12884-017-1380-y |url=}}</ref> | |||
=== '''Growth hormone replacement''' === | |||
*[[Growth hormone|GH]] is replaced on case to case basis starting with a low dose (0.1-0.3 mg/day) and [[Titrate|titrated]] upwards by 0.1 mg/d/month with repeated measurement of [[hormone]] levels every month, initially for the first 6 months followed by yearly measurements; replaced once all other [[hormones]] have been replaced.<ref name="pmid20944496">{{cite journal |vauthors=Tessnow AH, Wilson JD |title=The changing face of Sheehan's syndrome |journal=Am. J. Med. Sci. |volume=340 |issue=5 |pages=402–6 |year=2010 |pmid=20944496 |doi=10.1097/MAJ.0b013e3181f8c6df |url=}}</ref> | |||
=== '''Prolactin deficiency''' === | |||
* A study was done on 5 women with [[prolactin]] deficiency caused by Sheehan's syndrome or other causes that showed increased milk production upon [[subcutaneous]] administration of r-hPRL ([[recombinant]] human [[prolactin]]) every 12 hours for 28 days.<ref name="pmid20718766">{{cite journal |vauthors=Powe CE, Allen M, Puopolo KM, Merewood A, Worden S, Johnson LC, Fleischman A, Welt CK |title=Recombinant human prolactin for the treatment of lactation insufficiency |journal=Clin. Endocrinol. (Oxf) |volume=73 |issue=5 |pages=645–53 |year=2010 |pmid=20718766 |doi=10.1111/j.1365-2265.2010.03850.x |url=}}</ref> | |||
==References== | |||
<nowiki/>{{Reflist|2}} | <nowiki/>{{Reflist|2}} | ||
| |||
{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
[[Category:Medicine]] | |||
[[Category:Endocrinology]] | |||
[[Category:Up-To-Date]] | |||
[[Category:Emergency medicine]] | |||
[[Category:Obstetrics]] | |||
Latest revision as of 00:09, 30 July 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2].
Overview
Treatment involves appropriate hormone replacement therapy that results in complete recovery and reversal of symptoms.
Medical Therapy
- Treatment involves appropriate hormone replacement therapy, which must be taken for the rest of your life that results in significant improvement and reversal of not only the physical symptoms, but also the psychological symptoms.[1][2][3][4][5][6][7][8][9][10][11][12][13][14]
ACTH deficiency
Acute setting
- Preferred regimen: Hydrocortisone 100 mg IV bolus, then 300 mg/day IV divided q8hr or continuous infusion for 48 hours
- Once patient is stable: 50 mg PO q8hr for 6 doses, later on tapered to 30-50 mg/day PO in divided doses
Chronic setting
- Preferred regimen: Hydrocortisone 15-25 mg/day PO divided q8-12hr
NOTE
- Dosage is increased in patients with severe deficiency, increased body weight and in times of surgery, illness, procedures, and other stresses.
- Unfortunately, there is no established test to assess adequate hormonal replacement. Plasma ACTH measurement and serum/salivary/urinary cortisol values are all unreliable. So assessment of adequate hormonal replacement is based on clinical basis with Cushingoid features showing excessive replacement while symptoms of adrenal insufficiency suggesting insufficient hormonal replacement.
- Glucocorticoid replacement can cause polyuria due to unmasking of underlying central diabetes insipidus. DDAVP is the treatment of choice for patients with DI.[15]
- Mineralocorticoids are rarely required as aldosterone secretion is primarily regulated by angiotensin II and potassium not by ACTH.
TSH deficiency
Mild hypothyroidism
- Levothyroxine 1.7 mcg/kg qDay or 100-125 mcg PO qDay
Severe hypothyroidism
- Levothyroxine 12.5-25 mcg PO qDay and later on dose can be adjusted by 25 mcg/day q2-4 Week PRN
NOTE
- In patients with combined hypothyroidism and hypocortisolism, glucocorticoids (physiologic doses and increased doses in stress) are replaced before thyroid hormone replacement, because treating the hypothyroidism alone by levothyroxine can worsen the severity of cortisol deficiency by increasing the clearance of cortisol. So, it is important to assess adrenal function, including corticotropin (ACTH) reserve, before administering T4 (levothyroxine).
- American Thyroid Association (ATA) recommends dose adjustment to keep serum free T4 concentration in upper half of reference range.
LH and FSH deficiency
- If fertility required:
- Such women are offered ovulation induction. Pregnancy can be made possible by giving exogenous gonadotropins or pulsatile GnRH.
- Women with GnRH deficiency can be offered either pulsatile GnRH or gonadotropin therapy.
- Women with gonadotropin deficiency are given gonadotropins only.
- If fertility not required:
- Such women are treated with estrogen-progestin replacement therapy by using the traditional regimen of estradiol on days 1 through 25 of each month and progesterone on days 16 through 25 of each month.
- Another regimen includes continuous transdermal estradiol throughout the month, with progestin added days 1 to 10 of the calendar month.
Androgen replacement
Growth hormone replacement
- GH is replaced on case to case basis starting with a low dose (0.1-0.3 mg/day) and titrated upwards by 0.1 mg/d/month with repeated measurement of hormone levels every month, initially for the first 6 months followed by yearly measurements; replaced once all other hormones have been replaced.[17]
Prolactin deficiency
- A study was done on 5 women with prolactin deficiency caused by Sheehan's syndrome or other causes that showed increased milk production upon subcutaneous administration of r-hPRL (recombinant human prolactin) every 12 hours for 28 days.[18]
References
- ↑ Parikh R, Buch V, Makwana M, Buch HN (2016). "The price of a 15-year delay in diagnosis of Sheehan's syndrome". Proc (Bayl Univ Med Cent). 29 (2): 212–3. PMC 4790577. PMID 27034575.
- ↑ Laway BA, Bhat JR, Mir SA, Khan RS, Lone MI, Zargar AH (2010). "Sheehan's syndrome with pancytopenia--complete recovery after hormone replacement (case series with review)". Ann. Hematol. 89 (3): 305–8. doi:10.1007/s00277-009-0804-9. PMID 19697029.
- ↑ Tanriverdi F, Unluhizarci K, Kula M, Guven M, Bayram F, Kelestimur F (2005). "Effects of 18-month of growth hormone (GH) replacement therapy in patients with Sheehan's syndrome". Growth Horm. IGF Res. 15 (3): 231–7. doi:10.1016/j.ghir.2005.03.005. PMID 15921942.
- ↑ Bülow B, Hagmar L, Mikoczy Z, Nordström CH, Erfurth EM (1997). "Increased cerebrovascular mortality in patients with hypopituitarism". Clin. Endocrinol. (Oxf). 46 (1): 75–81. PMID 9059561.
- ↑ Arafah BM (2002). "Medical management of hypopituitarism in patients with pituitary adenomas". Pituitary. 5 (2): 109–17. PMID 12675508.
- ↑ Grossman AB (2010). "Clinical Review#: The diagnosis and management of central hypoadrenalism". J. Clin. Endocrinol. Metab. 95 (11): 4855–63. doi:10.1210/jc.2010-0982. PMID 20719838.
- ↑ Esteban NV, Loughlin T, Yergey AL, Zawadzki JK, Booth JD, Winterer JC, Loriaux DL (1991). "Daily cortisol production rate in man determined by stable isotope dilution/mass spectrometry". J. Clin. Endocrinol. Metab. 72 (1): 39–45. doi:10.1210/jcem-72-1-39. PMID 1986026.
- ↑ Arlt W, Rosenthal C, Hahner S, Allolio B (2006). "Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements". Clin. Endocrinol. (Oxf). 64 (4): 384–9. doi:10.1111/j.1365-2265.2006.02473.x. PMID 16584509.
- ↑ Thomson AH, Devers MC, Wallace AM, Grant D, Campbell K, Freel M, Connell JM (2007). "Variability in hydrocortisone plasma and saliva pharmacokinetics following intravenous and oral administration to patients with adrenal insufficiency". Clin. Endocrinol. (Oxf). 66 (6): 789–96. doi:10.1111/j.1365-2265.2007.02812.x. PMID 17437510.
- ↑ Martin MM (1969). "Coexisting anterior pituitary and neurohypophyseal insufficiency. A syndrome with diagnostic implication". Arch. Intern. Med. 123 (4): 409–16. PMID 4182323.
- ↑ Shibata H, Ogishima T, Mitani F, Suzuki H, Murakami M, Saruta T, Ishimura Y (1991). "Regulation of aldosterone synthase cytochrome P-450 in rat adrenals by angiotensin II and potassium". Endocrinology. 128 (5): 2534–9. doi:10.1210/endo-128-5-2534. PMID 2019265.
- ↑ White PC (1994). "Disorders of aldosterone biosynthesis and action". N. Engl. J. Med. 331 (4): 250–8. doi:10.1056/NEJM199407283310408. PMID 8015573.
- ↑ Miller KK, Sesmilo G, Schiller A, Schoenfeld D, Burton S, Klibanski A (2001). "Androgen deficiency in women with hypopituitarism". J. Clin. Endocrinol. Metab. 86 (2): 561–7. doi:10.1210/jcem.86.2.7246. PMID 11158009.
- ↑ Miller KK, Biller BM, Beauregard C, Lipman JG, Jones J, Schoenfeld D, Sherman JC, Swearingen B, Loeffler J, Klibanski A (2006). "Effects of testosterone replacement in androgen-deficient women with hypopituitarism: a randomized, double-blind, placebo-controlled study". J. Clin. Endocrinol. Metab. 91 (5): 1683–90. doi:10.1210/jc.2005-2596. PMID 16478814.
- ↑ Soares DV, Conceição FL, Vaisman M (2008). "[Clinical, laboratory and therapeutics aspects of Sheehan's syndrome]". Arq Bras Endocrinol Metabol (in Portuguese). 52 (5): 872–8. PMID 18797595.
- ↑ Matsuzaki S, Endo M, Ueda Y, Mimura K, Kakigano A, Egawa-Takata T, Kumasawa K, Yoshino K, Kimura T (2017). "A case of acute Sheehan's syndrome and literature review: a rare but life-threatening complication of postpartum hemorrhage". BMC Pregnancy Childbirth. 17 (1): 188. doi:10.1186/s12884-017-1380-y. PMC 5471854. PMID 28615049.
- ↑ Tessnow AH, Wilson JD (2010). "The changing face of Sheehan's syndrome". Am. J. Med. Sci. 340 (5): 402–6. doi:10.1097/MAJ.0b013e3181f8c6df. PMID 20944496.
- ↑ Powe CE, Allen M, Puopolo KM, Merewood A, Worden S, Johnson LC, Fleischman A, Welt CK (2010). "Recombinant human prolactin for the treatment of lactation insufficiency". Clin. Endocrinol. (Oxf). 73 (5): 645–53. doi:10.1111/j.1365-2265.2010.03850.x. PMID 20718766.