Síndrome deSheehan;diabetesinsípidocentral;testeprivaçãoágua Descritores Arq Bras Metab. Endocrinol 2011;55/2 S desmopressina paracontrolarapoliúria. resposta àvasopressinaindicaramdiabetesinsípidocentral. A pacientefaziausocontínuode deáguaeexame A jovem tambémapresentoupoliúria.Osresultadosdotestedeprivação que foiconfirmadoporpesquisahormonaleexamesdeimagemevidenciaramselavazia. parto, apresentouhemorragia pós-parto grave,seguidapelaevolução depan-hipopituitarismo síndrome deSheehan.Nesteartigo, descrevemosocasodeumajovem que,apósosexto de das funçõesdaglândulahipofisáriaposterior. Adisfunçãodahipófiseposterior, sobaforma se caracterizapelainsuficiênciadaglândulahipofisáriaanterior, porémcomaconservação do hipopituitarismoobservadoempaísesdesenvolvimento comoaÍndia.Essasíndrome mente precedidoporhemorragia pós-parto. Essacondiçãoclínicaaindaconstitui causacomum A síndromedeSheehanestárelacionadaàocorrência dehipopituitarismopós-parto, geral- SUMÁRIO .Sheneededoraldesmopressinonacontinuousbasistocontrolpolyuria. Polyuria. The watertestandresponsetovasopressin testresultsindicatedcentral deprivation nal investigation anddemonstrationofemptysellaonimaging.Inaddition,shedeveloped hemorrhage followedbydevelopmentofpanhypopituitarism which was confirmedbyhormo- ofayoungladywhoafterthe clinicalcourse hersixthchildbirth developedseverepostpartum form ofcentraldiabetesinsipidusisrareinpatientswithSheehan’s syndrome. We describe failure withpreservationofposteriorpituitaryfunctions.Posterior pituitarydysfunctioninthe India. tuitarism indevelopingcountrieslike The disorderusuallypresentswithanteriorpituitary ded by postpartum hemorrhage. The condition still continuesto be a common cause ofhypopi - Sheehan’s totheoccurrence syndromerefers ofhypopituitarism after delivery, usuallyprece- SUMMARY Laway Bashir Ahmad Síndrome deSheehanediabetes insípidocentral central diabetes insipidus Sheehan’s syndrome with Sheehan’s syndrome;centraldiabetesinsipidus;waterdeprivationtest Keywords Bras EndocrinolMetab. 2011;55(2):171-4 Mohd IqbalDar itary necrosis (1).The syndrome manifests withlactation (1).Thesyndrome necrosis itary spasm insmallervessels,apoplexy, andsubsequent pitu- causesarterial artery. Anyhypotensionduringdelivery ofthesuperiorhypophyseal incompression nancy results - enlargement during preg Pituitary tum hemorrhage. bypostpar whichisusuallypreceded during pregnancy gland ofthephysiologicallyenlarged pituitary necrosis and asaconsequenceofinfarction pituitarism results diabetes insipiduscentral,éalgoraramenteobservadoempacientesqueapresentama tuitary hormone deficiency after child birth. Hypo- deficiencyafterchildbirth. hormone tuitary withanteriorpi- (SS)presents heehan’s syndrome 1 , Abdul HamidZargar 1 , Shahnaz Ahmad Mir Arq BrasEndocrinolMetab. 2011;55(2):171-4 1 1 ,

- itary (4-6). Although subtle posterior pituitary distur (4-6).Althoughsubtle posteriorpituitary itary hypofunction of the anterior pitu- complete or partial witheither (3).Most patients present autoimmunity anddelayed antigens, triggeringpituitary sequestered mayrelease is believedthattissuenecrosis development ofhypopituitarismhasbeensuggested.It esis ofSSisnotclear. ofautoimmunity inthe Arole deficiencies(2).Pathogen- hormone typicalofother andpubichairoffeatures of axillary andloss involutionofthebreasts, amenorrhea, failure, Arq Arq

clinical casereport Jammu andKashmirIndia SKIMS, Soura,Srinagar, 1 Accepted onFev/2/2011 Received onDec/30/2010 [email protected] Correspondence to: Department ofEndocrinology, 171 -

Copyright© ABE&M todos os direitos reservados. Copyright© ABE&M todos os direitos reservados. procedure waterdeprivation test(10). procedure both ofthem.ThepatientwassubjectedtotheMiller consentwasobtainedfrom her spouseandinformed water deprivationtestwasexplainedtothepatientand ofthe ter deprivationtestwasplanned.Theprocedure ted aftertwomonthsforevaluationofpolyuriaandwa- ters/day.- Thepatientwasdischarged andwasreadmit 4.5to6li- from volumeincreased worsened, urinary overthefollowingtwoweeksbutpolyuria improving 5 mg/day.prednisolone Someof her symptoms started 75µg/dayand thyroxine receiving put onreplacement 1).The patientwas of completelyemptysella(Figure evidence revealed nance imaging(MRI)ofthepituitary tive ofpanhypopituitarism(Table- 1).Magneticreso sugges- features (PRL),and gonadotrophins; (GH), hormone growth (TSH) withlowbasalcortisol, stimulatinghormone thyroid normal inappropriately (T4), totalthyroxine undetectableserum tions revealed estima- limits.Basalhormone withinnormal gram were - and calciumlevels.HerchestX-rayelectrocardio creatinine, glucose,sodium,potassium,urea, normal leukocyte count.Biochemicalinvestigationrevealed re, urine volume, and urine osmolality were measured measured urinevolume,and osmolalitywere re, - herweight,bloodpressu at 7o’clockinthemorning; osmolality, measured plasmasodium,andglucosewere urinevolume,urinary test. Herweight,bloodpressure, the 9p.m.intheeveningbefore of foodandwaterfrom 172 bocytopenia (plateletcountof66´10 - anemia(Hbof9.3 g/dL,throm vestigation revealed In- ofhypothyroidism. andpubichairwithfeatures ry atrophy,facial hypopigmentation,breast absentaxilla- of95/60mmHg, bloodpressure mination revealed noticingexcessive thirst and polyuria. Exa- also started andwasfatigable.Ayear earlier,menstruate shehad shefailedtolactate,didnot seeking us.Afterdelivery yearsbefore units ofbloodafterhospitalizationthree three bleeding,andreceived loped massivepostpartum A 35-year-old hersixthchild,deve- womandelivered C mopressin. tooraldes- was foundtohaveCDIwhichresponded polyuriaduringfollow-upandoninvestigation severe with whoalsopresented a caseofSheehan’ssyndrome nance imaging(7-9).We theclinicalcourseof report - theavailabilityofmagnetic reso before reported are central complete bances inthesepatientshavebeenreported, Sheehan’s syndrome withcentral diabetes insipidus ase repor (CDI) is rare andmostcases diabetes insipidus(CDI)israre t

She wasdeprived 3 )

with normal with normal tions todate. inpolyuriaandiscontinuing medica- a markedrelief 0.1mgatbedtime,following whichshehad mopressin method. The patient was put on oraldes- depression point withanosmometerusingthefreezing performed 2).Allosmolalitystudieswere 302 mOsm/kg(Figure osmolalitydespiteplasmaof ze hisurinary didnotstabili- suggestive ofcentralDI.Thecontrol osmolality of AVP inurinary wasa74%increase there osmolality was300mOsm/kg.Afteradministration hours ofwaterdeprivationwhensimultaneousplasma 16 osmolalitystabilizedataround The patient’surinary the testsinsamemannerandactedasacontrol. minutes. Thepatient’shusbandwasalsosubmittedto minutesfornext90 thirty every measured lality were subcutaneously. Subsequently, urineandplasmaosmo- (AVP)units ofarginine vasopressin administered were and 5 measured plasma sodiumandosmolality were osmolalitystabilized.Atthistime, hourly untilurinary radioimmunoassay. * Peak values after insulin tolerance test. Hormone assays performed with specific hormone; LH:luteinizingGH:growthhormone. (arrow) suggestiveofcomplete emptysella. atthebase cerebrospinal fluidandthestalktouching theatrophicpituitary fossa filledwith coronalviewshowingpituitary Figure 1.MRIpituitary T T 3 Basal thyroid and pituitary hormonalparameters able 1.Basalthyroidandpituitary T T (Plasma) Hormone Prolactin* FSH LH TSH GH* Cortisol* : tri-iodothyronine; T 4 3 4 : thyroxine; TSH: thyroid stimulating hormone; FSH: follicle stimulating μIU/mL μg/mL μg/dL μg/dL Units μg/L μg/L IU/L IU/L Arq Bras Metab. Endocrinol 2011;55/2 Values 10.47 < 0.3 6.53 1.18 3.31 0.25 8.35 < 1 Normal values 5.5-13.5 0.7-2.5 0.5-6.5 2-6.6 3-12 > 20 > 2 > 3 Arq Bras Metab. Endocrinol 2011;55/2 join withthosefrom Thesearteries and lateralarteries. divideintomedial artery carotid oftheinternal portion the cavernous arisingfrom inferior hypophysialarteries The invascularsupplythe two regions. the difference explainedby ispartly than thatoftheanteriorpituitary islesscommon involvement oftheposteriorpituitary only.SS anddataislimitedtocasereports Thefactthat inpatientswith basisisrare onapermanent treatment all ofthem(12).Polyuriathemagnitudeneeding in forthirstwasincreased insipidus andthethreshold central diabetes of thepatientshadpartial 29 percent publishedseriesaround (7-10). Inoneoftherecently and subtledefectsinAVP havebeendetected secretion tions havebeentestedinmanyseriesofpatientswithSS func- (11).Posteriorpituitary in theposteriorpituitary changes of thedisease,whenSheehannoticedatrophic sincetheoriginal description function hasbeenthere deficiency,hormone intheposteriorpituitary interest ofanterior pituitary to medical attention with features central ted tisolemic states. The water deprivation test documen- andeucor an exacerbationafterachievingeuthyroid was admission andthere polyuria foroneyearbefore of ofemptysella.Shehadahistory features revealed evidence of panhypopituitarism and MRI sis revealed analy- Hormonal ofhypothyroidism. rhea, andfeatures amenor followed by lactation failure, tum hemorrhage ofpostpar evidencedbyhistory Sheehan’s syndrome of patienthadclassicalpresentation The present Discussion mOsm/kg, urineosmolalitycontinuedtobeofapproximately800mOsm/kg. 600 mOsm/kginnexttwohours.Control:spiteofserumosmolality300 osmolality was 300 mOsm/kg. After giving AVP urine osmolality increased to a.m., urineosmolalitystabilizesat1p.m.whensimultaneousplasma and controlduringdehydrationtest.Case: Figure 2.UrineandplasmaosmolalityresponsetoAVP inthepatient

and response toAVPdiabetes insipidusandresponse confirmed Urine Osmolality mOsm/kg UrOS Case 1,000 100 200 300 400 500 600 700 800 900 diabetes insipidus. AlthoughSSclinicallycomes 0 7 8 UrOS Control 9 10 T 11 ime hours 12 After starting dehydration test at 7 Afterstartingdehydrationtestat7 PDS case 13 given A VP 14.5 15 PDS control 15.5 - - - rone; anddiabetesinsipidushadresolved. rone; - andestrogen/progeste corticosteroids, on thyroxine, she wasdocumentedtohavepanhypopituitarism; forafewdaysonly.with desmopressin Onfollow-up pituitary. normal treatment phy revealed Sherequired computedtomogra- centralDIandcerebral confirmed fourunitsofblood. Investigations she wasadministered 9.8g/Lto5.7within24hours,and from dropped an estimatedbloodlossof500mL,herhemoglobin section. Shehad polyuria afterlowersegmentCesarean agrandmultiparaladywhodevelopedsevere ported - gland.Kanandcols.(9)re ofthepituitary infarction privation test.Imagingshowedevidenceofischemic andaftera10-hourwaterde- testingbefore electrolyte with Diabetesinsipiduswasconfirmed failure. adrenal andsecondary hypoprolactinemia, tral , cen - function tests revealed vision. Pituitary and blurry headaches, developed excessivethirst,polyuria,severe subtotalhysterectomy.requiring Postoperatively, she andhypotension hemorrhage sented withpostpartum - gestational diabetes who pre sia and insulin requiring a35-year-old- womanwithmildpreeclamp reported emptysella.Westonthe brainrevealed andcols.(15) was diagnosed tohavediabetesinsipidus, imaging of section. She developed polyuria seven months later and bleeding and hypotension after caesarean loped severe bed thecaseofa31-year-old womanwhohaddeve- died aftersixweeks,(5).Tulandi andcols.(14)descri- ofpolyuriaand forcontrol needed nasaldesmopressin outcomehadCentralDI.Both adverse postdelivery shed; twooutoftheeightysixpatientsSSwhohad excessivedamage(13). itfrom protect and oftheneurohypophysis the infundibularprocess ananastomotic ringaround the oppositesideforming after institution of steroid treatment whenthesubtle treatment after institutionofsteroid ADH deficiencyusuallycomestoattention If present, state. mainly contributedby a hypocortisol treatment, before sensitivitytothehormone levels andincreased supplementation. Patientswith SShaveelevatedAVP significantly withintranasal Desmopressin tus improved evidenceofdiabetesinsipidusandherclinicalsta- tory postoperatively,drome, shedevelopedpolyuria,labora- intravascular coagulation followedby Sheehan’s syn- disseminated hemorrhage, postpartum veloped severe a multigravida woman, who de- and cols. (16) reported sectionorelseDIwastransient.RecentlyKumar rean duringthecesa- tomatic onlyafterwaterwasrestricted water and became symp- compensating by taking more andwas delivery that thesaidpatienthadDIbefore In one of our large clinical studies previously publi- In oneofourlarge clinicalstudiespreviously Sheehan’s syndrome withcentral diabetes insipidus

It ispossible 173

Copyright© ABE&M todos os direitos reservados. Copyright© ABE&M todos os direitos reservados. 174 1. Referncs was reported. relevant tothisarticle nopotentialconflictofinterest Disclosure: of her6 neededbloodtransfusionafterdelivery hemorrhage, after documentationofcentraldiabetesinsipidus. treatment permanent andrequired lone andthyroxine - ofpredniso symptomaticafterreplacement came more ent patient had Polyuria for one year,the pres she be - deficiency becomessymptomatic(17,18).Although Sheehan’s syndrome withcentral diabetes insipidus 5. 4. 3. 2. in patients with Sheehan’s syndrome israre. dus inpatientswithSheehan’ssyndrome centraldiabetesinsipi- ofpolyuria.Permanent to befree permanently vation test.Sheneededoraldesmopressin med tohavecentraldiabetesinsipidusbyawaterdepri- imaging. Shealsodevelopedpolyuriaandwasconfir investigationand byhormonal confirmed syndrome

In summary a young woman developed postpartum ayoungwomandevelopedpostpartum In summary Saudi Med.1996;16(3):338-41. Siddiqi M,etal.ClinicalspectrumofSheehan’s syndrome. Ann Zargar AH, MasoodiSR,Laway BA,ShahNA,Salahuddin M, Kelestimur F. Sheehan’s syndrome.Pituitary. 2003;6(4):181-8. Obstet Gynecol.1954;68(1):202-23. Sheehan HL. The incidenceofpostpartum hypopituitarism. Am J crinol Metab.2002;87(9):4137-41. autoimmunity inpatientswithSheehan’s syndrome.JClinEndo- Goswami R, Kochupillai N, Crock PA, Jaleel A, Gupta N. Pituitary fferential diagnosisinacutephase.JInternMed.1988;244(3):261-6. Dejager S,GerberFoubert L, Turpin G.Sheehan’s syndrome:di- th child,andsubsequentlydevelopedSheehan’s - 18. 7. 1 16. 15. 14. 13. 12. 11. 10. 9. 8. 7. 6.

cases. EurJEndocrinol.2003;148(6):609-17. due tohypopituitarism with adrenalinsufficiency:report on28 Diederich S,Franzen NF, Bahr V, Oelkers W. Severe hyponatremia 2002;16(5):419-23. poglycemia inacuteSheehan’s syndrome.GynecolEndocrinol. Bunch TJ, Dunn WF, Basu A, GosmanRI.Hyponatremiaandhy uncommon disorder. EndocrinePractice. 2010;1:1-23. presenting ascentraldiabetesinsipidus:ararepresentationofan Kumar S,Burrows D,Dang S, SimmonsD.Sheehan’s syndrome col. 2005;45(3):249-50. ting post-partum withdiabetesinsipidus. Aust NZObstetGyne- Weston G,ChavesN,Bowditch J. Sheehan’s syndromepresen- complication. ObstetGynecol.1987;70(3):492-5. Tulandi T, Yusuf N,Posner BI.Diabetesinsipidus:apostpartum adenomatous hyperplasia. Br JNeurosurg.1995;9(3):331-46. the adenohyophysis andalteredpatterns inoestrogen-induced Jakubowski J. Bloodsupply, bloodflowandautoregulationin 2007;156(5):563-7. terior pituitaryfunctioninSheehan’s syndrome.EurJEndocrinol. Atmaca H, Tanriverdi F, GokceC,UnluhizarciK,Kelestimur F. Pos- hypopituitarism. JPathol Bacteriol.1963;85:145-69. Sheehan HL, Whitehead R. The neurohypophysis inpostpartum Intern Med.1970;73(5):721-9. nition ofpartial defectsinantidiuretichormonesecretion. Ann Miller M,Dalakos T, Moses AM, Fellerman H,StreetenDH.Recog - 1998;38(2):224-6. senting withdiabetesinsipidus. Aust NZJObstetGynaecol. Kan AK, Calligerous D. A case report ofSheehansyndrome pre- (Oxf). 1987;27(1):91-5. function inpatientswithSheehan’s syndrome.ClinEndocrinol Jialal I, Desai RK,Rajput MC. An assessmentof posterior pituitary me. BrMedJ. 1984;289(6445):579-80. Bakiri F, BenmiloudM. Antidiuretic functioninSheehan’s syndro- ph function.Endocrinologist.2009;19(6):253-4. pregnancies inSheehan’s syndromewithpreservedgonadotro- Laway BA, Ganie MA, Wani IR, Butt TP. Multiple spontaneous Arq Bras Metab. Endocrinol 2011;55/2 -