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Insuficienca antehipofizare
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EtiologjiaEtiologjia
Funksioni i reduktuar i hipofizes mund të rezultojë nga çrregullime te trasheguara
por më shpesh,ajo është e fituar dhe reflekton efektet mase të tumoreve apo pasoja te inflamacionit ose dëmtim të enëve të gjakut.
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Shkaqet gjenetikeShkaqet gjenetike te te zhvillimit zhvillimit të të HypopituitarismHypopituitarism
Displasia Pituitare Disfunksioni hipotalamik i rritjes:
Kallmann Syndrome (Hypogonadotropic hypogonadism)
Laurence-Moon-Bardet-Biedl Syndrome (involves many body systems)
Fröhlich Syndrome (childhood metabolicdisorder) Prader-Willi Syndrome.
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Shkaqet e hypopituitarismitShkaqet e hypopituitarismitTumore (tu’) Pituitary tumor Adenoma,craniopharyngiom
a Cerebral tumor
Crregullime Hypothalamike
Tumore Crregullime Funksionale - Eg -Anorexia nervosa
Semundje infiltrative Sarcoidosis Histocytosis X Haemochromatosis Te tjera Empty Sella syndrome Lymphocytic hypophysitis
Vasculare Necrosis (Sheehan’s synd) Infarkti I hipofizes Hypotension I thelle Kranial arteritisTraumaInfection Meningitis esp TB, syphilisIatrogenic Kirurgji Rrezatime Medical Therapy
– DA drugs– Pegvisomant– Anti-adrenal drugs
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Hypopituitarism Mortaliteti 1.75-2.2 here me I madh se ne popullaten e
pergjithshme Mund te jete total (panhipopituitarizem) ose
parcial Deficiti I isoluar I hormoneve adenohipofizare
mund te vij dhe nga patologji te tjera vec anomalive gjenetike
Isolated gonadotrophin deficiency-Haemochromatosis
Isolated ACTH deficiency -Lymphocytic hypophysitis
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Karakteristika te pergjithshmeKarakteristika te pergjithshme
Hipopituitarismi parcial eshte me I shpeshte se Hipopituitarismi parcial eshte me I shpeshte se hipopituitarismi I plotehipopituitarismi I plote
Simptomat nuk manifestohen deri ne Simptomat nuk manifestohen deri ne shkaterrimin e me shume se 75% te qelizave te shkaterrimin e me shume se 75% te qelizave te hipofizes anteriore hipofizes anteriore
Sekretimi I GH secretion eshte shfaqja me e Sekretimi I GH secretion eshte shfaqja me e heret e hipopituitarismit me efekte te heret e hipopituitarismit me efekte te shprehura tek femijet por me pak sinjifikante shprehura tek femijet por me pak sinjifikante tek te rriturittek te rriturit
LH/ FSH preken perpara ACTHLH/ FSH preken perpara ACTH
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ANTERIOR PITUITARY HYPOFUNCTION ANTERIOR PITUITARY HYPOFUNCTION (hypopituitarism(hypopituitarism))
3 shkaqet me te shpeshta:3 shkaqet me te shpeshta:
Adenomat jo sekretore te hipofizes anterioreAdenomat jo sekretore te hipofizes anteriore
Sheehan’s syndrome Sheehan’s syndrome ((postpartum pituitary necrosis)
Empty sella syndrome Empty sella syndrome
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Sindroma Sheehan
Sheehan (1937): nje sindrom qe karakterizohet me nekroze akute te hipofizes anteriore si pasoje e hemorragjise postpartum .
Sheehan's syndrome: destruksion total I hipofizes anteriore, klinikisht panhypopituitarism
Incidence: 1/10,000 lindje. Klinikisht, 70-75% e indit hipofizar humbet perpara se te
shfaqen shenjat klinike. Humbja sequenciale e sekretimit hormonal
GH --> FSH & LH --> TSH --> ACTH --> PRL.
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Hypofisiti limfocitar
Hipofisiti limfocitar eshte nje lesion I rralle inflamator I hipofizes qe zakonisht prek grate e reja gjate fazes se fundit te gravidances ose ne periudhen postpartum. Ky crregullim mendohet te kete nje baze patogjenetike autoimune dhe mund te shoqerohet me semundje te tjera autoimune si tiroiditi Hashimoto apo morbus Addison .Manifestimet klinike mund te jene ne varesi te efektit mase ose te procesit inflamator si psh dhembje koke ,prekje te fushes vizive dhe parcial apo total hipopituitarizem.Axi me I prekur eshte ai I ACTH.Kirurgjia trans sfenoidale eshte terapia e zgjedhur per ato raste me shprehje te efektit mase,kurse terapia zevendesuese hormonale ne varesi te deficiteve hormonale.
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Empty sella(Sella bosh)
Empty sella konsiderohet si nje selle e cila pavaresisht nga permasat e saj eshte plotesisht ose pjeserisht e mbushur me leng cerebrospinal.
Nje Empty sella(Sella bosh) me permase normale eshte nje gjetje kryesisht aksidentale gjate autopsive.
Kjo mund te quhet sekondare kur gjendet pas interventeve kirurgjikale radioterapive,apo trajtimeve per patologji hipofizare.Gjetja e saj duhet te shoqerohet me nje vleresim hormonal per te percaktuar deficitet e mundshme hormonale dhe ne ato raste behet zevendesimi hormonal perkates
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Empty sella(Sella bosh)
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Apoplexia hipofizare
Apoplexia hipofizare eshte nje emergjence e ralle endokrine qe rezulton si pasoje e nje infarkti hemorragjik I nje tumori hipofizar pre egzistues.manifestimet klinike te kesaj sindrome jane te lidhura me hemorragjine dhe komresionin e gjendres hipofizare qe con ne dhimbje koke ,prekje te fushes vizive dhe hipopituitarizem.Dhimbja e kokes eshte me evidente tek keta persona dhe sapo te dyshohet nje diagnoze e tille duhet te fillojme dexamethasone 2mg cdo 6 ore iv dhe te bejme nje RMN te hipofizes
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Shenjat dhe simptomat e Apoplexys Pituitare
Signs and Symptoms Prevalence (%)
Dhimbje koke 80
probleme te shikimit 62
renie te palpebres 40
Nausea, te vjella 21
Alterim I statusit mental 20
Meningismus 16.5
temperature 2.5
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Pituitary apoplexy
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Clinical featuresClinical features
Hormone Shfaqjet e deficitit
GH femije: vonese ne rritjeAdults: ↓mases musculore Tendence per hypoglicemia.
Prolactin Mungese lactacioni
Gonadotrophins Femije:pubertet I vonuarFemra: oligomenorrhoea, infertilitet,atrophy e gjoksit & gjenitaleMeshkuj:Impotence,azoospermia,atrofi testiculareTe dy sexet: humbje te libidos,dhe te qimeve
ACTH Humbje peshe, hypotension, hypoglycaemia, mungese pigmentimi te lekures
TSH Shtim ne peshe, intolerance ndaj te ftohtit,lodhje
Vasopressin etje, poliuria
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Insuficienca e Hormonit te rritjes
Insuficienca somatotrope eshte deficiti hipofizar me i shpeshteKlinikisht manifestohet tek femijet nga nje vonese ne rritje .kjo vonese eshte me e dukshme pas viteve te para te jetes dhe shoqerohet me nje ngadalesim te shpejtesise te rritjes . Tek te rriturit mungesa e hormonit te rritjes shoqerohet me :•Asteni fizike dhe psiqike •Tendence depresive•Alterim te troficitetit kutan •Nje modifikim i shperndarjes se mases dhjamore qe rritet ne raport me masen muskulare qe zvogelohet•Zvogelim i densiteti kockorBiologjikisht vihet re Nje tendence per anemi dhe nje tendence per hipoglicemi sidomos ne efort dhe esell (sidomos kur existon dhe nje insuficience kortikotrope shoqeruese)Konfirmimi i deficitit somatotrop behet nepermjet matjes te nivelit plazmatik te GH qe nuk rritet nga provat e stimulimit dhe nga niveli plazmatik i IGF1 vlerat e te cilit gjenden ne nivelet e ulta te normes per moshen
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Insuficienca e GH Dhjamosje e shtuar, raportit bark/vithe BMD 1-2SD poshte mesatares per moshen, frakture x2 total cholesterol and LDL/HDL insulin, insulin resistance Lodhje dhe dobesi muskulare
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Insuficienca e Gonadotropinave Burrat
volumit testicular Humbje te qimeve te trupit dhe fytyres te fuqise sexuale dhe te libidos
Meshkuj Peripubertal : mungese ne rritjen e volumit testikular dhe
nivelit te testosteronit Grate
Amenorrea Tharje te vagines
Grate ne Postmenopause: LH, FSH I ulet
Te dy sexet Lekure te holle BMD (densiteti kockor)
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Insuficienca e ACTH
Dobesi trupore,lodhje Hypotension Te vjella Hypoglycaemia Hyponatraemia Dhimbje muskulare Mungese pigmentimi
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Insuficienca e TSH
FT4 I ulur TSH mund te jete normal Hypothyroidism me I lehte se ai primar
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Egzaminimet e hypopituitarismit
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Nivelet baze te hormoneve
Prolactin FT4 (TSH) Cortisol (8-9am) Testosterone/Estradiol LH, FSH IGF-1, GH
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Growth hormone deficiency Assume deficient if TSH, ACTH, gonadotrophin
deficiency Isolated GH deficiency predominant form in children Pulsatile secretion – majority of adult levels low Single level of limited use Insulin tolerance test = “gold standard” Other tests:
Arginine Glucagon Clonidine (unsuitable in adults) IGF-1:
Stable throughout day Integrated GH level Dependent on age, nutrition, hepatic, thyroid function
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Sekretimi I GH
06:00 06:0024:0018:0012:00
Normal
Acromegaly
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Insuficienca e ACTH Niveli I kortizolit
Piku I kortisolemise ne 6-8 te mengjesit Kortisoli ngrihet nga stresi fizik dhe emocional
Insulin tolerance test (Hipoglicemia e provokuar) “Gold standard” Glicemia duhet te arrije < 40 (2.2mmol/l) Normal: cortisol > 550nmol/l, increment > 170nmol/l Kundraindikacione: mosha te vjetra, Insuficienca kardiake,
konvulsione Synacthen test Injektim im ose iv I synacthen Matet kortizolemia bazale dhe pastaj 30 min dhe 60 min pas injeksionit.
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Insuficienca e TSH
FT4 I ulur , TSH I ulur TRH test – jo shume I vlefshem behet rralle
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Trajtimi i hypopituitarismit
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Growth hormone
Injeksione te GH ne rruge S-C Permiresime ne formimin trupor ne
performancen fizike,ne densitetin kockor dhe ne permiresimin e cilesise se jetes
Perfitime ne gjatesi ne femijet qe su jane mbyllur epifizat
Vetem deficitet severe te GH pergjigjen mire ndaj zevendesimit te te rriturit
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Gonadotrophin Deficiency
Femrat Estrogen + cyclical/continuous progesterone Fertiliteti: Pulsatile GnRH apo gonadotropina Shtatzania arrihet ~85%
Meshkujt Testsosterone: injeksion, implant, patch, gel, oral ( absorbim jo I mire) Fertiliteti: hCG injections ± FSH
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Insuficienca e ACTH
Hydrocortisone/Cortisone acetate Monitorimi I dozes :
symptomatik,TA,elektrolitet dhe dozimi periodik I kortizolemive
Rritja e dozes gjate Stressit (fizik, emocional, shendetesor) dyfishim I dozes ose marrja parenterale sidomos ne rast te vjellash
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Insuficienca e TSH
Nuk mund te perdoret per monitorim TSH, perdoret FT4
Duhet pritur deri ne korrektimin e deficitit te kortikotropit pasi mund te keqesoje deficitin e kortizolemise (Krize adissoniene)
Duhet shmangur mbidozimi (osteoporoze, probleme kardiake)
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Insuficienca e hipofizes Insuficienca e hipofizes posterioreposterioreShkaqetShkaqet
prodhimit te ADH prodhimit te ADH diabet insipid qendror diabet insipid qendror (DI)(DI) Tumore-craniopharyngioma, tumore Tumore-craniopharyngioma, tumore
sekondare(metastatic CA) tumore hipofizare me rritje sekondare(metastatic CA) tumore hipofizare me rritje suprasellar .suprasellar .
Semundje granulomatoze.Semundje granulomatoze. Meningitis, absces dhe encephalite.Meningitis, absces dhe encephalite. Vaskulare.Vaskulare. Trauma.Trauma. Kirurgji.Kirurgji. Idiopathik.Idiopathik.
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Shkaqet e Central Diabetes Insipidus
Shkaqe kongjenitaleShkaqe kongjenitale
Trauma, postkirurgjiTrauma, postkirurgji
Neoplasms—craniopharyngioma, germinoma, meningioma, leukemia, Neoplasms—craniopharyngioma, germinoma, meningioma, leukemia, lymphomalymphoma
Pituitary macroadenoma me rritje suprasellarePituitary macroadenoma me rritje suprasellare
MetastasisMetastasis
Semundje Granulomatose —histiocytosis, sarcoidosis, tuberculosisSemundje Granulomatose —histiocytosis, sarcoidosis, tuberculosis
Infeksione—meningitis, encephalitisInfeksione—meningitis, encephalitis
Shkaqe vaskulare—aneurysm, Sheehan's syndromeShkaqe vaskulare—aneurysm, Sheehan's syndrome
Shkaqe Autoimmune Shkaqe Autoimmune
Shkaqe FamiljareShkaqe Familjare
Shkaqe IdiopatikeShkaqe Idiopatike
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Insuficienca hipofizes Insuficienca hipofizes Posteriore Posteriore EfektetEfektet
Polyuria-shtim I diurezes tendence per Polyuria-shtim I diurezes tendence per dehidrimdehidrim
Polydipsia-shtim I etjes qe con ne Polydipsia-shtim I etjes qe con ne stimulimin e qendres se etjes dhe ne stimulimin e qendres se etjes dhe ne rritjen e marrjes se ujit rritjen e marrjes se ujit
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Investigation, treatment Water deprivation test
Normal: urine osmolality rises DI: Urine fails to concentrate (urine : plasma <
2x) Plasma osmolality rises ADH deficiency: urine osmolality with desmopressin Nephrogenic DI: no response to desmopressin
Treatment Desmopressin
More potent, long acting than ADH Nasal fluid/aerosol, tablet
Large individual variation in dose