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Hypopituitarism & Hyperpituitarism

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Hypopituitarism & Hyperpituitarism

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Hypopituitarism & Hyperpituitarism

  1. 1. Hypothalamic - pituitaryHypothalamic - pituitary disorders.disorders. Hypopituitarism andHypopituitarism and HyperpituitarismHyperpituitarism
  2. 2. SponsoredSponsored Medical Lecture Notes –Medical Lecture Notes – All SubjectsAll Subjects USMLE Exam (America) –USMLE Exam (America) – PracticePractice
  3. 3. The hypothalamic-pituitary axesThe hypothalamic-pituitary axes
  4. 4. HYPOTHALAMUS ANDHYPOTHALAMUS AND PITUITARY GLANDPITUITARY GLAND HYPOPHYSISHYPOPHYSIS ANTERIORANTERIOR LOBELOBE INTERMEDIATEINTERMEDIATE LOBELOBE POSTERIORPOSTERIOR LOBELOBE MelanotropinMelanotropin ADHADH (vasopresin)(vasopresin) OxytocinOxytocin Basophile cellsBasophile cells EosinophilicEosinophilic cellscells ChromophobesChromophobes cellscells ACTHACTH TSHTSH FSHFSH ICSHICSH TTHTTH STGSTG ProlactinProlactin
  5. 5. Hypothalamic releasing hormonesHypothalamic releasing hormones Hypothalamic releasing hormone Effect on pituitaryEffect on pituitary Corticotropin releasingCorticotropin releasing hormone (CRH)hormone (CRH) Stimulates ACTH secretionStimulates ACTH secretion Thyrotropin releasingThyrotropin releasing hormone (TRH)hormone (TRH) Stimulates TSH andStimulates TSH and Prolactin secretionProlactin secretion Growth hormone releasingGrowth hormone releasing hormone (GHRH)hormone (GHRH) Stimulates GH secretionStimulates GH secretion SomatostatinSomatostatin Inhibits GH (and otherInhibits GH (and other hormone) secretionhormone) secretion Gonadotropin releasingGonadotropin releasing hormone (GnRH)hormone (GnRH) Stimulates LH and FSHStimulates LH and FSH secretionsecretion Prolactin releasingProlactin releasing hormone (PRH)hormone (PRH) Stimulates PRL secretionStimulates PRL secretion Prolactin inhibitingProlactin inhibiting hormone (dopamine)hormone (dopamine) Inhibits PRL secretionInhibits PRL secretion
  6. 6. The hormones are secreted by the anterior pituitary:The hormones are secreted by the anterior pituitary: HORMONEHORMONE FUNCTIONFUNCTION Thyroid StimulatingThyroid Stimulating HormoneHormone (TSH)(TSH) causes the thyroid gland tocauses the thyroid gland to produce and release thyroidproduce and release thyroid hormoneshormones Growth HormoneGrowth Hormone (GH)(GH) regulates growth and metabolismregulates growth and metabolism AdrenocorticotropicAdrenocorticotropic Hormone (ACTH)Hormone (ACTH) triggers the adrenals to releasetriggers the adrenals to release the hormone cortisolthe hormone cortisol Luteinizing Hormone (LH)Luteinizing Hormone (LH) menstrul cycle and reproductionmenstrul cycle and reproduction Follicle StimulatingFollicle Stimulating Hormone (FSH)Hormone (FSH) in the ovary stimulates the growthin the ovary stimulates the growth of immatureof immature Graatian folliclesGraatian follicles to maturation.to maturation. In men enhances the productionIn men enhances the production of androgen-bilding protein by theof androgen-bilding protein by the Sertoli cellsSertoli cells of the testes and isof the testes and is critical for spermatogenesiscritical for spermatogenesis stimulates production of breaststimulates production of breast milk and is necessary for normalmilk and is necessary for normal milk production duringmilk production during breast feedingbreast feedingProlactinProlactin (PRL)(PRL)
  7. 7. The hormones are secreted by theThe hormones are secreted by the posterior pituitary:posterior pituitary: HORMONEHORMONE FUNCTIONFUNCTION stimulates contractions of the uterusstimulates contractions of the uterus during labor and the ejection of milkduring labor and the ejection of milk during breast-feedingduring breast-feeding increases reabsorption of water intoincreases reabsorption of water into the blood by the kidneys andthe blood by the kidneys and therefore decreases urine productiontherefore decreases urine production Intermediate lobeIntermediate lobe producesproduces melanocyte – stimulating hormonemelanocyte – stimulating hormone (MSH)(MSH) regulates the production of melanin, a dark pigment, byregulates the production of melanin, a dark pigment, by melanocytes in the skinmelanocytes in the skin OxytocinOxytocin oror VasopressinVasopressin AntidiureticAntidiuretic HormoneHormone (ADH)(ADH)
  8. 8. DISORDERS OF PITUITARY GLANDDISORDERS OF PITUITARY GLAND with the decreased function HYPOPITUITARISMHYPOPITUITARISM with the increased function HYPERPITUITARISMHYPERPITUITARISM Pituitary dwarfismPituitary dwarfism (nanism)(nanism)  Sheehan’sSheehan’s syndromesyndrome (Simmond’s desease)(Simmond’s desease)  Diabetes insipidusDiabetes insipidus AcromegalyAcromegaly GigantismGigantism Icsenko-CushingIcsenko-Cushing diseasedisease HyperprolactinaemiaHyperprolactinaemia
  9. 9. HypopituitarismHypopituitarism is loss of function in an endocrine gland due to failure ofis loss of function in an endocrine gland due to failure of the pituitary gland to secrete hormones whichthe pituitary gland to secrete hormones which stimulate that gland's function.stimulate that gland's function. PanhypopituitarismPanhypopituitarism This condition represents the loss ofThis condition represents the loss of all hormonesall hormones released by the anterior pituitary gland.released by the anterior pituitary gland. PanhypopituitarismPanhypopituitarism is also known as complete pituitaryis also known as complete pituitary failure.failure. The terms Reye syndrome, Sheehan’s andThe terms Reye syndrome, Sheehan’s and Simmond’sSimmond’s syndromesyndrome refers to necrosis of therefers to necrosis of the pituitary during the postpartum period.pituitary during the postpartum period. Only the difference inOnly the difference in Simmond’s syndromeSimmond’s syndrome is thatis that although it is very similar medical condition can takealthough it is very similar medical condition can take place in both males and females and it is independentplace in both males and females and it is independent from the postpartum complications.from the postpartum complications.
  10. 10. Causes of hypopituitarismCauses of hypopituitarism Ischemia and infarctionIschemia and infarction – Sheehan’s syndrome,– Sheehan’s syndrome, apoplexyapoplexy  IatrogenicIatrogenic - Radiation, surgery, withholding previous- Radiation, surgery, withholding previous chronic glucocorticoid replacementchronic glucocorticoid replacement  Trauma to the hypophysisTrauma to the hypophysis TumorsTumors - Craniopharyngiomas, pituitary adenomas- Craniopharyngiomas, pituitary adenomas Infiltrative processesInfiltrative processes - Sarcoidosis, histiocytosis X,- Sarcoidosis, histiocytosis X, hemochromatosishemochromatosis InfectionsInfections -- Tuberculosis, syphilis, meningitisTuberculosis, syphilis, meningitis Empty sella syndromeEmpty sella syndrome CongenitalCongenital – Kallmann’s syndrome– Kallmann’s syndrome AutoimmuneAutoimmune - Lymphocytic hypophysitis- Lymphocytic hypophysitis
  11. 11. Sheehan’s syndromeSheehan’s syndrome Synonyms:Synonyms: Simmond’s disease, PostpartumSimmond’s disease, Postpartum pituitary necrosis, Postpartum ishemic necrosis ofpituitary necrosis, Postpartum ishemic necrosis of the anterior pituitary, Postpartumthe anterior pituitary, Postpartum panhypopituitarismpanhypopituitarism Sheehan’s syndromeSheehan’s syndrome, or, or necrosis of thenecrosis of the pituitary glandpituitary gland, is a rare complication of, is a rare complication of postpartum hemorrhage initiallypostpartum hemorrhage initially described in 1937described in 1937.. Sheehan’s syndrome was named when theSheehan’s syndrome was named when the English pathologistEnglish pathologist Harold LeemingHarold Leeming Sheehan (1900-1988)Sheehan (1900-1988) reviewed andreviewed and described the syndrome.described the syndrome. Polish physicianPolish physician Leon Konrad Glinski (1870-Leon Konrad Glinski (1870- 1918)1918) counts for the other name,counts for the other name, GlinskiGlinski Simmon’s syndrome.Simmon’s syndrome.
  12. 12. Laboratory diagnostic ofLaboratory diagnostic of Sheehan’s syndromeSheehan’s syndrome • Blood tests:Blood tests:  Serum thyroid stimulating hormoneSerum thyroid stimulating hormone (TSH):(TSH): decreased (decreased (↓↓) or normal (N)) or normal (N)  TT44 (thyroid hormone):(thyroid hormone): ↓↓  Serum luteinizing hormoneSerum luteinizing hormone (LH):(LH): ↓ or N↓ or N  Serum follicle stimulating hormoneSerum follicle stimulating hormone (FSH):(FSH): ↓ or N↓ or N  Serum testosteroneSerum testosterone:: ↓↓  Serum estradiolSerum estradiol (estrogen):(estrogen): ↓↓  Serum cortisolSerum cortisol:: ↓↓  Serum ACTHSerum ACTH:: ↓↓  Serum growth hormoneSerum growth hormone (GH):(GH): ↓↓ • Bone x-rays of the hand:Bone x-rays of the hand: • to determine bone ageto determine bone age
  13. 13. Standard therapy ofStandard therapy of Sheehan’s syndromeSheehan’s syndrome Hormone replacement medications mayHormone replacement medications may include:include: CorticosteroidsCorticosteroids are required if the ACTH-are required if the ACTH- adrenal axis is impairedadrenal axis is impaired Treat secondary hypothyroidismTreat secondary hypothyroidism -- LevothyroxineLevothyroxine Sex hormones:Sex hormones: testosterone in men andtestosterone in men and estrogen or a combination of estrogen andestrogen or a combination of estrogen and progesterone in womenprogesterone in women
  14. 14. GlucocorticoidsGlucocorticoids used in adrenal insufficiency:used in adrenal insufficiency: HydrocortisoneHydrocortisone 20-30 mg/d PO divided bid20-30 mg/d PO divided bid (often 15 mg in the morning and 10 mg in the afternoon)(often 15 mg in the morning and 10 mg in the afternoon) Thyroid hormonesThyroid hormones used in hypothyroidism:used in hypothyroidism: LevothyroxineLevothyroxine 100-200 mcg/d p/o100-200 mcg/d p/o Growth hormonesGrowth hormones used in the treatment of children:used in the treatment of children: Somatropin (Humatrope, Genotropin)Somatropin (Humatrope, Genotropin) 6-125 mcg/kg/d s/c6-125 mcg/kg/d s/c Pediatric dose:Pediatric dose: GenotropinGenotropin - 160-240 mcg/kg SC q week divided- 160-240 mcg/kg SC q week divided in 6-7 dosesin 6-7 doses Humatrope -Humatrope -180 mcg/kg IM/SC q week divided in 3-7 doses180 mcg/kg IM/SC q week divided in 3-7 doses Sex hormonesSex hormones used in hypogonadism:used in hypogonadism: Recombinant human GHRecombinant human GH (rhGH)(rhGH) by SC injection daily:by SC injection daily: 0.3 mg/kg/week0.3 mg/kg/week TestosteroneTestosterone -- 50-400 mg i/m q 2-4 week50-400 mg i/m q 2-4 week EstrogensEstrogens -- 0.3-0.625 mg/d p/o for 3 week; off 1 week,0.3-0.625 mg/d p/o for 3 week; off 1 week, repeat cyclerepeat cycle Treatment of Sheehan’s syndromeTreatment of Sheehan’s syndrome
  15. 15. PITUITARY NANISMPITUITARY NANISM HYPOPHYSIAL MICROSOMIA, HYPOPHYSIALHYPOPHYSIAL MICROSOMIA, HYPOPHYSIAL NANOCORMIANANOCORMIA it is a genetic disease caused byit is a genetic disease caused by absolute or relative deficiency of STHabsolute or relative deficiency of STH in the organism.in the organism. The sudden growth inhibition is markedThe sudden growth inhibition is marked the age of 2-3 years in genetic nanism.the age of 2-3 years in genetic nanism. First the disease was described byFirst the disease was described by A. Paltuff in 1891.A. Paltuff in 1891.
  16. 16. Pituitary dwarfismPituitary dwarfism is a condition in which the growth of theis a condition in which the growth of the individual is very slow or delayed,individual is very slow or delayed, resulting in less than normal adultresulting in less than normal adult stature.stature. Abnormally short stature.Abnormally short stature. The average adult height ofThe average adult height of malemale andand femalefemale dwarfismdwarfism sufferers aresufferers are 130130 cmcm andand 120 cm120 cm respectively.respectively. Also known asAlso known as nanismnanism..
  17. 17. Etiology and pathogenesisEtiology and pathogenesis pituitary nanismpituitary nanism CongenitalCongenital insufficiencyinsufficiency autosomal – recessiveautosomal – recessive inheritanceinheritance (idiopathic forms)(idiopathic forms) Acquired insufficiencyAcquired insufficiency pituitary tumorpituitary tumor craniopharyngiomacraniopharyngioma injury of the pituitaryinjury of the pituitary sarcoidosissarcoidosis toxoplasmosistoxoplasmosis infectioninfection vascular pathologyvascular pathology PeripheralPeripheral resistanceresistance of GHof GH Growth deceleration and differentiation of skeletonGrowth deceleration and differentiation of skeleton AbsoluteAbsolute deficiency ofdeficiency of GHGH RelativeRelative deficiency ofdeficiency of GHGH
  18. 18. Classification of pituitary dwarfismClassification of pituitary dwarfism Organic:Organic: traumatrauma neoplasmsneoplasms infectioninfection Idiopatic:Idiopatic: primaryprimary secondarysecondary due to hypothalamicdue to hypothalamic deficiencydeficiency PanhypopituitarismPanhypopituitarism Isolated GH deficiencyIsolated GH deficiency (may be hereditary and transmitted(may be hereditary and transmitted as an autosomal recessive trait,as an autosomal recessive trait, in other instancesin other instances a hereditary basis cannot be established)a hereditary basis cannot be established)
  19. 19. PhysicalPhysical ChildrenChildren The standing heightThe standing height standard deviation scorestandard deviation score is usually below -2is usually below -2 Growth velocity is below theGrowth velocity is below the 10-25 th percentile,10-25 th percentile, which reflects growthwhich reflects growth decelerationdeceleration Increased subcutaneous fatIncreased subcutaneous fat is present, especiallyis present, especially around the trunkaround the trunk The face is immature, with aThe face is immature, with a prominent forehead andprominent forehead and depressed midfacialdepressed midfacial developmentdevelopment Dentition is delayedDentition is delayed The average age of pubertalThe average age of pubertal onset is delayed in bothonset is delayed in both boys and girlsboys and girls Adults Reduced lean body massReduced lean body mass and increased weight, withand increased weight, with body fat massbody fat mass predominantly in thepredominantly in the abdominal regionabdominal region Thin and dry skinThin and dry skin Cool peripheriesCool peripheries Poor venous accessPoor venous access Reduced muscle mass andReduced muscle mass and strength and reducedstrength and reduced exercise performanceexercise performance Depressed affectDepressed affect Labile emotionsLabile emotions
  20. 20. S., 13 years old. Height – 85 cm, weight – 12 kg G., 3 years old. Height – 68 cm, weight – 7 kg.
  21. 21. Girl , 4 years old, her height -120 cmGirl , 4 years old, her height -120 cm
  22. 22. MEDICATIONMEDICATION GenotropinGenotropin NutropinNutropin 0.15 - 0.3 mg/kg/week S/C initially divide into equal doses to be given daily or 6 times/week as subcutaneous injections
  23. 23. Diabetes insipidus (DI)Diabetes insipidus (DI) is a condition that results from insufficient production of theis a condition that results from insufficient production of the antidiuretic hormone (ADH).antidiuretic hormone (ADH). First, it was described by Thomas Willis in 1674. The family formFirst, it was described by Thomas Willis in 1674. The family form of hypothalamic DI was described by Lacomb in 1841.of hypothalamic DI was described by Lacomb in 1841. Hypothalamic Diabetes Insipidus (HDI)Hypothalamic Diabetes Insipidus (HDI) also known asalso known as neurogenic, central, or cranial DI is the result of partial orneurogenic, central, or cranial DI is the result of partial or complete lack of osmoregulated ADH secretion.complete lack of osmoregulated ADH secretion. Nephrogenic Diabetes Insipidus (NDI)Nephrogenic Diabetes Insipidus (NDI) is due to renalis due to renal resistance to the antidiuretic effects of ADH.resistance to the antidiuretic effects of ADH. Dipsogenic Diabetes Insipidus (DDI)Dipsogenic Diabetes Insipidus (DDI) is a polyuricis a polyuric syndrome secondary to excess fluid intake. Though structuralsyndrome secondary to excess fluid intake. Though structural abnormalities may be the cause, it is generally a manifestationabnormalities may be the cause, it is generally a manifestation of primary polydipsia, psychiatric disease, or secondaryof primary polydipsia, psychiatric disease, or secondary to drug effects.to drug effects.
  24. 24. Classification of Diabetes Insipidus (DI)Classification of Diabetes Insipidus (DI) Hypothalamic DIHypothalamic DI PrimaryPrimary Genetic:Genetic: DIDMOAD (Wolfram)DIDMOAD (Wolfram) syndromesyndrome Autosomal dominantAutosomal dominant Autosomal recessiveAutosomal recessive DevelopmentalDevelopmental syndromes:syndromes: Septo-optic dysplasiaSepto-optic dysplasia IdiopathicIdiopathic Secondary/Secondary/ acquiredacquired Trauma:Trauma: Head injuryHead injury Post surgeryPost surgery (transcranial,(transcranial, transphenoidal)transphenoidal) Tumour:Tumour: CraniopharyngiomCraniopharyngiom Germ cell tumoursGerm cell tumours MetastasesMetastases PituitaryPituitary macroadenomamacroadenoma Inflammatory:Inflammatory: GranulonulomasGranulonulomas SarcoidosisSarcoidosis HistiocytosisHistiocytosis InfectionInfection Infundibulo-Infundibulo- neurohypophysitisneurohypophysitis Guillaine-BarreGuillaine-Barre SyndromeSyndrome AutoimmuneAutoimmune Vascular:Vascular: AneurysmAneurysm InfarctionInfarction Sheehan'sSheehan's syndromesyndrome Sickle cell diseaseSickle cell disease PregnancyPregnancy (associated with vasopressinase)(associated with vasopressinase) Nephrogenic DINephrogenic DI Dipsogenic DIDipsogenic DI CompulsiveCompulsive water drinkingwater drinking AssociatedAssociated with affectivewith affective disordersdisorders Structural/Structural/ organicorganic hypothalamichypothalamic diseasedisease:: Sarcoid Tumours involving hypothalamus Head injury Tuberculous meningitis PrimaryPrimary SecondarySecondary Genetic:Genetic: X-linked recessiveX-linked recessive Autosomal recessiveAutosomal recessive Autosomal dominantAutosomal dominant Idiopathic:Idiopathic: Chronic renal diseaseChronic renal disease Metabolic diseaseMetabolic disease Drug inducedDrug induced Osmotic diureticsOsmotic diuretics Systemic disordersSystemic disorders PregnancyPregnancy
  25. 25. Diabetes insipidus (DI)Diabetes insipidus (DI) HYPOTHALAMIC DIHYPOTHALAMIC DI NEPHROGENIC (RENAL)NEPHROGENIC (RENAL) DIDI absolute deficiency ofabsolute deficiency of antidiuretic hormoneantidiuretic hormone genetic pathology ofgenetic pathology of ADH receptors,ADH receptors, it inheritsit inherits as recessive signas recessive sign which linkedwhich linked with sex (in male)with sex (in male)
  26. 26. Causes of DICauses of DI  malfunctioning hypothalamusmalfunctioning hypothalamus  malfunctioning pituitary glandmalfunctioning pituitary gland  damage to hypothalamus or pituitary gland duringdamage to hypothalamus or pituitary gland during surgerysurgery  brain injurybrain injury  tumortumor  tuberculosistuberculosis  blockage in the arteries leading to the brainblockage in the arteries leading to the brain  encephalitisencephalitis  meningitismeningitis  sarcoidosis (a rare inflammation of the lymph nodessarcoidosis (a rare inflammation of the lymph nodes and other tissuesand other tissues throughout the body)throughout the body)
  27. 27. SYMPTOMS OF DIABETES INSIPIDUSSYMPTOMS OF DIABETES INSIPIDUS  excessive thirstexcessive thirst  excessive urine production (up to a dozen or moreexcessive urine production (up to a dozen or more quarts a day) of diluted, colorless urinequarts a day) of diluted, colorless urine  dehydrationdehydration  dry handsdry hands  constipation (due to "dry" bowels)constipation (due to "dry" bowels) LABORATORY TESTS OF DI:LABORATORY TESTS OF DI:  low ADH levelslow ADH levels  electrolyte imbalanceelectrolyte imbalance  polyuriapolyuria (> 3 litre)(> 3 litre)  urinalysis shows a low specific gravityurinalysis shows a low specific gravity (< 1008)(< 1008)
  28. 28. NameName Central Diabetes InsipidusCentral Diabetes Insipidus Neprhogenic Diabetes InsipidusNeprhogenic Diabetes Insipidus SituationSituation Lack of or insufficient ADHLack of or insufficient ADH Structural or functional defectsStructural or functional defects in ADH receptors or aquaporinsin ADH receptors or aquaporins OnsetOnset •congenital defect ofcongenital defect of hypothalamus or pituitaryhypothalamus or pituitary •acquiredacquired •congenital defect of receptorscongenital defect of receptors or aquaporinsor aquaporins •acquiredacquired CausesCauses trauma or disease of pituitary ortrauma or disease of pituitary or hypothalamushypothalamus trauma or disease of the kidneytrauma or disease of the kidney Signs &Signs & TestsTests polyuriapolyuria •polydispsiapolydispsia •electrolyte imbalanceelectrolyte imbalance •possible dehydrationpossible dehydration •low ADH levelslow ADH levels •urinalysis low specific gravityurinalysis low specific gravity polyuriapolyuria •polydispsiapolydispsia •electrolyte imbalanceelectrolyte imbalance •possible dehydrationpossible dehydration •low ADH levelslow ADH levels •urinalysis low specific gravityurinalysis low specific gravity DiagnosisDiagnosis •rule out other causesrule out other causes •imagery of pituitary andimagery of pituitary and hypothalamushypothalamus •water deprivation testwater deprivation test •ADH trialADH trial •rule out other causesrule out other causes •rule out CDIrule out CDI TreatmentTreatment desmopressindesmopressin oral chlorothiazideoral chlorothiazide •chloropropamidechloropropamide •NSAIDsNSAIDs •restrict saltrestrict salt PrognosisPrognosis variablevariable •not life-threatening if treatednot life-threatening if treated and fluid intake maintainedand fluid intake maintained congenital NDI--chroniccongenital NDI--chronic •acquired NDI--variableacquired NDI--variable
  29. 29. Treatment of Diabetes InsipidusTreatment of Diabetes Insipidus Central DI:Central DI: long-acting VPlong-acting VP analogue DDAVP:analogue DDAVP: intranasal sprayintranasal spray H-DesmopressinH-Desmopressin single dosesingle dose consists ofconsists of 10 mcg10 mcg of Desmopressin acetateof Desmopressin acetate (5-100 mcg daily),(5-100 mcg daily), AdiupressinAdiupressin («Ameda Pharma», India) is used(«Ameda Pharma», India) is used intranasalintranasal 2–8 gutters2–8 gutters (10-40 mcg)(10-40 mcg) a day.a day. Antidiuretic effect showsAntidiuretic effect shows in an hourin an hour,, maximal actionmaximal action 1–5 hours1–5 hours,, effect longevity is 8–20 hourseffect longevity is 8–20 hours.. Parenteral injection (0.1-2.0 mcg daily).Parenteral injection (0.1-2.0 mcg daily). OralOral MinirinMinirin is used byis used by 100–200 mcg a100–200 mcg a dayday (1–3 tablets), in divided doses.(1–3 tablets), in divided doses.
  30. 30. Nephrogenic DI:Nephrogenic DI: is usually treated withis usually treated with thiazide diureticsthiazide diuretics hydrochlorothiazidehydrochlorothiazide 25 mg/day25 mg/day,, which are among the class of "water pills“.which are among the class of "water pills“. Non-steroidal anti-inflammatory drugs:Non-steroidal anti-inflammatory drugs: Ibuprofen 200 mg/dayIbuprofen 200 mg/day. Low salt dietLow salt diet .. Dipsogenic DI:Dipsogenic DI: Clozapine 100 mgClozapine 100 mg may reduce polydipsia in those patients with refractory schizophrenia on other dopamine antagonistsdopamine antagonists. Reduced fluid intakefluid intake is the only rational treatment. Treatment of Diabetes InsipidusTreatment of Diabetes Insipidus
  31. 31. HyperpituitarismHyperpituitarism AcromegalyAcromegaly GigantismGigantism Itsenko-Cushing’sItsenko-Cushing’s syndromesyndrome  HyperprolactinaemiaHyperprolactinaemia
  32. 32. PITUITARY ADENOMAPITUITARY ADENOMA MICROADENOMAMICROADENOMA MACROADENOMAMACROADENOMA CATEGORIESCATEGORIES Diameter = / < 10 mmDiameter = / < 10 mm IntrasellarIntrasellar Presents usually withPresents usually with hormonal hypersecrationhormonal hypersecration syndromesyndrome Diameter > 10 mmDiameter > 10 mm Extends outsideExtends outside the sellathe sella Presents often withPresents often with chiasmal compressionchiasmal compression syndromesyndrome
  33. 33. Causes of acromegalyCauses of acromegaly SOMATOTROPH ADENOMASSOMATOTROPH ADENOMAS  Eosinophilic pituitary adenoma  Pituitary tumors: microadenomas (pituitarymicroadenomas (pituitary tumors less than 1 cm in size);tumors less than 1 cm in size); macroadenomas (pituitary tumors greatermacroadenomas (pituitary tumors greater than 1cm)than 1cm)  Nonpituitary tumors:Nonpituitary tumors: by tumors of theby tumors of the pancreas, lungs, and other parts of the brainpancreas, lungs, and other parts of the brain
  34. 34. Symptoms of acromegalySymptoms of acromegaly Facial change, acral enlargement, and soft-Facial change, acral enlargement, and soft- tissue swellingtissue swelling Excessive sweatingExcessive sweating Acroparesthesiae/ carpal tunnel syndromeAcroparesthesiae/ carpal tunnel syndrome Tiredness and lethargyTiredness and lethargy HeadachesHeadaches Oligo- or amenorrhea, infertilityOligo- or amenorrhea, infertility Erectile dysfunction and/or decreasedErectile dysfunction and/or decreased libidolibido ArthropathyArthropathy Impaired glucose tolerance/ diabetesImpaired glucose tolerance/ diabetes GoiterGoiter Ear, nose throat and dental problemsEar, nose throat and dental problems Congestive cardiac failure/ arrythmiaCongestive cardiac failure/ arrythmia HypertensionHypertension Visual field defectsVisual field defects AA – Arthralgias/– Arthralgias/ ArthritisArthritis BB – BP raised– BP raised CC – Carpal– Carpal TunnelTunnel DD – Diabetes– Diabetes EE – Enlarged– Enlarged OrgansOrgans FF – Field defect– Field defect
  35. 35. It be showed largenessIt be showed largeness in the size of nose, ears , lipsin the size of nose, ears , lips It be showed largenessIt be showed largeness in the size of tonguein the size of tongue It be showedIt be showed growth in handsgrowth in hands Typical facies of acromegalyTypical facies of acromegaly
  36. 36. Typical facies of acromegalyTypical facies of acromegaly Frontal bossingFrontal bossing Thickening of the noseThickening of the nose MacroglossiaMacroglossia PrognathismPrognathism
  37. 37. Separation of the teethSeparation of the teeth on the lower jawon the lower jaw
  38. 38. Image of a radiotherapy machine.Image of a radiotherapy machine. The patient lies within a fixed mask that targets the radiation preciselyThe patient lies within a fixed mask that targets the radiation precisely A magnetic resonance imaging (MRI) machine.A magnetic resonance imaging (MRI) machine. The patient slides into the machine andspinning magnets areThe patient slides into the machine andspinning magnets are used to create an image of the pituitary gland and the surrounding tissueused to create an image of the pituitary gland and the surrounding tissue
  39. 39. Typical Skull X-RayTypical Skull X-Ray (Thickening of the Calvarium)(Thickening of the Calvarium) of an Acromegalic patientof an Acromegalic patient Lateral skull X-rayLateral skull X-ray The bones of the skull are normal.The bones of the skull are normal. Regular sella (arrow)Regular sella (arrow)
  40. 40. In the cefalometric radiograms, an enlargementIn the cefalometric radiograms, an enlargement in the sella tursica and prognathismin the sella tursica and prognathism and obliquity in angulus mandibula were observedand obliquity in angulus mandibula were observed
  41. 41. Complications of acromegalyComplications of acromegaly Cardiovascular:Cardiovascular:  Ischemic heart diseaseIschemic heart disease  CardiomyopathyCardiomyopathy  Congestive heart failureCongestive heart failure  ArrhythmiasArrhythmias  HypertensionHypertension Respiratory:Respiratory:  KyphosisKyphosis  Obstructive sleep apneaObstructive sleep apnea Metabolic:Metabolic:  Diabetes mellitus/IGTDiabetes mellitus/IGT  HyperlipidemiaHyperlipidemia Neurologic:Neurologic: Carpal Tunnel syndromeCarpal Tunnel syndrome StrokeStroke Neoplastic:Neoplastic: CoorectalCoorectal Breast and prostate -Breast and prostate - uncertainuncertain Musculoskeletal:Musculoskeletal: DegenerativeDegenerative arthropathyarthropathy Calcific discopathy,Calcific discopathy, pyrophosphatepyrophosphate arthropathyarthropathy
  42. 42. Treatment of acromegalyTreatment of acromegaly Somatostatin analogues (SSAs):Somatostatin analogues (SSAs): OctreotideOctreotide ((SandostatinSandostatin)) and lanreotideand lanreotide ((SomatulineSomatuline DepotDepot)) 50 mcg s/c tid; can increase to 500 mcg tid; doses50 mcg s/c tid; can increase to 500 mcg tid; doses of 300-600 mcg/day or higher seldom result in additionalof 300-600 mcg/day or higher seldom result in additional benefit.benefit. LanreotideLanreotide is given as a long-actingis given as a long-acting subcutaneous injection once a month.subcutaneous injection once a month. Dopamine agonists:Dopamine agonists: BromocriptineBromocriptine (Parlodel)(Parlodel) 20-30 mg PO qd (10-6020-30 mg PO qd (10-60 mg/day)mg/day) in divided doses. Safety not demonstrated atin divided doses. Safety not demonstrated at >100 mg/d.>100 mg/d. CabergolineCabergoline (Dostinex)(Dostinex) Growth hormone antagonistsGrowth hormone antagonists:: blocks the effect of growth hormone on body tissues.blocks the effect of growth hormone on body tissues. PegvisomantPegvisomant (Somavert)(Somavert) 40 mg s/c40 mg s/c 10 mg s/c qd initially; may increase or decrease q 4 – 610 mg s/c qd initially; may increase or decrease q 4 – 6 week by 5-mg increments as determined by IGF-I levels;week by 5-mg increments as determined by IGF-I levels; not to exceed 30 mg/d.not to exceed 30 mg/d.
  43. 43. SurgerySurgery  Acromegaly is traditionally treated withAcromegaly is traditionally treated with transsphenoidaltranssphenoidal pituitary surgery and adenoma removalpituitary surgery and adenoma removal  Endonasal Transphenoidal surgeryEndonasal Transphenoidal surgery  Septal Pushover/Direct SphenoidotomySeptal Pushover/Direct Sphenoidotomy  Endoscopic approachEndoscopic approach Radio-therapyRadio-therapy Conventional radiation therapyConventional radiation therapy this type of radiation isthis type of radiation is usually given every weekday over four to six weeks. It may takeusually given every weekday over four to six weeks. It may take five to 10 years or more for your growth hormone levels tofive to 10 years or more for your growth hormone levels to return to normalreturn to normal Stereotactic radiosurgeryStereotactic radiosurgery Radiation can also be givenRadiation can also be given stereotactically, with precisely focused, intense beams aimed atstereotactically, with precisely focused, intense beams aimed at a tumor from multiple directions. This strategy can deliver aa tumor from multiple directions. This strategy can deliver a high dose of radiation to tumor cells while limiting the amount ofhigh dose of radiation to tumor cells while limiting the amount of radiation to nearby normal tissuesradiation to nearby normal tissues Current stereotactic technologies deliver radiation with aCurrent stereotactic technologies deliver radiation with a gamma knifegamma knife, a linear accelerator or a proton beam, a linear accelerator or a proton beam
  44. 44. Icsenko-Cushing’s diseaseIcsenko-Cushing’s disease CORTICOTROPH ADENOMASCORTICOTROPH ADENOMAS (small basophilic microadenomas that(small basophilic microadenomas that secret ACTH)secret ACTH) is a disease, which is manifested by theis a disease, which is manifested by the bilateral hyperplasia of adrenal glands,bilateral hyperplasia of adrenal glands, increased secretion of ACTH and hormonesincreased secretion of ACTH and hormones of adrenal cortex.of adrenal cortex. First, the disease was described by theFirst, the disease was described by the RussianRussian neuropatolologist N.M. Icsenkoneuropatolologist N.M. Icsenko in 1924in 1924.. In 1932In 1932 the same symptom wasthe same symptom was described by the Americandescribed by the American neurosurgeonneurosurgeon Harvey Cushing.Harvey Cushing.
  45. 45. Icsenko-Cushing’s disease andIcsenko-Cushing’s disease and Icsenko-Cushing’s syndromeIcsenko-Cushing’s syndrome Icsenko-Cushing’s syndromeIcsenko-Cushing’s syndrome is a syndrome due to excess cortisolis a syndrome due to excess cortisol from pituitary, adrenal or other sourcesfrom pituitary, adrenal or other sources (exogenous glucocorticoids, ectopic(exogenous glucocorticoids, ectopic ACTH, etc.)ACTH, etc.) Icsenko-Cushing’s diseaseIcsenko-Cushing’s disease is hypercortisolism due to excess pituitaryis hypercortisolism due to excess pituitary secretion of ACTH (about 70% of cases ofsecretion of ACTH (about 70% of cases of endogenous Icsenko-Cushing’sendogenous Icsenko-Cushing’s syndrome)syndrome)
  46. 46. Icsenko-Cushing’s diseaseIcsenko-Cushing’s disease Centripetal obesityCentripetal obesity Moon faceMoon face Buffalo humpBuffalo hump Skin atrophySkin atrophy Easily bruisedEasily bruised StriaeStriae Cutaneous fungalCutaneous fungal infectionsinfections HyperpigmentationHyperpigmentation Oligo- or amenorrheaOligo- or amenorrhea Hirsutism and VirilizationHirsutism and Virilization with adrenal tumorswith adrenal tumors
  47. 47. Icsenko-Cushing’s diseaseIcsenko-Cushing’s disease Proximal muscle wasting &Proximal muscle wasting & weaknessweakness OsteoporosisOsteoporosis Glucose intolerance orGlucose intolerance or steroid diabetessteroid diabetes HypokalemiaHypokalemia ThromboembolismThromboembolism DepressionDepression InfectionInfection GlaucomaGlaucoma
  48. 48. Progressive Obesity ofProgressive Obesity of Icsenko-Cushing’s DiseaseIcsenko-Cushing’s Disease Age 6Age 6 Age 7Age 7 Age 8Age 8 Age 9Age 9 Age 11Age 11
  49. 49. TREATMENT OF ICSENKO-CUSHING DISEASETREATMENT OF ICSENKO-CUSHING DISEASE ACTH-DependentACTH-Dependent HypercortisolismHypercortisolism Pituitary MRI Petrosal sinusPituitary MRI Petrosal sinus ACTH samplingACTH sampling ACTH-secreting pituitary adenomaACTH-secreting pituitary adenoma Consider chest/Abd imagingConsider chest/Abd imaging Ectopic ACTH excludedEctopic ACTH excluded Transsphenoidal surgical resectionTranssphenoidal surgical resection Biochemical cureBiochemical cure Persistent hypercortisolismPersistent hypercortisolism PituitaryPituitary irradiationirradiation andand//oror SteroidogenicSteroidogenic inhibitorsinhibitors ADRENALECTOMYADRENALECTOMY Serial biochemical and MRISerial biochemical and MRI evaluationevaluation GlucocorticoidGlucocorticoid replacement,replacement, if neededif needed
  50. 50. GONADOTROPH ADENOMASGONADOTROPH ADENOMAS  Majority produce FSH, some FSH and LH, rarely only LHMajority produce FSH, some FSH and LH, rarely only LH  Occur in middle-aged men and women usually areOccur in middle-aged men and women usually are macroadenomasmacroadenomas  May cause amenorrhea or galactorrhea,May cause amenorrhea or galactorrhea, ↓ libido in men↓ libido in men THYROTHROPH ADENOMASTHYROTHROPH ADENOMAS  produce TSH►hyperthyroidismproduce TSH►hyperthyroidism NON-SECRETORY ADENOMASNON-SECRETORY ADENOMAS  in 4th decade of lifein 4th decade of life  may grow to large size- macroadenomas 1 cmmay grow to large size- macroadenomas 1 cm  local mass effect: headache, visual disturbances andlocal mass effect: headache, visual disturbances and panhypopituitarism:panhypopituitarism: hypogonadism, hypothyroidism,hypogonadism, hypothyroidism, hypoadrenalismhypoadrenalism  most consist of chromophobic cells or intenselymost consist of chromophobic cells or intensely eosinophilic cellseosinophilic cells
  51. 51. GigantismGigantism oror giantismgiantism, (from, (from GreekGreek gigasgigas,, gigantasgigantas "" giantgiant") is a condition characterized by") is a condition characterized by excessive height growth and bignessexcessive height growth and bigness significantly abovesignificantly above average heightaverage height.. Height isHeight is 2.25 - 2.402.25 - 2.40 metres.metres.
  52. 52. The world's tallest peopleThe world's tallest people Leonid StadnikLeonid Stadnik, Ukraine, 258 cm, Ukraine, 258 cm Alexander SizonenkoAlexander Sizonenko, Russia, 250 cm, Russia, 250 cm Yunsay ChangYunsay Chang, China, 242 cm, China, 242 cm Radhuan CharbybaRadhuan Charbyba, Tunis, 237 cm, Tunis, 237 cm Bao Si ShunBao Si Shun, China, 236 cm, China, 236 cm Nasir SoomroNasir Soomro, Pakistan 236 cm, Pakistan 236 cm Besad HusseinBesad Hussein, Britain, 236 cm, Britain, 236 cm Yao DefenYao Defen,, JapanJapan, 236 cm, 236 cm Leonid StadnikLeonid Stadnik Alexander SizonenkoAlexander Sizonenko Bao Si ShunBao Si Shun Yao DefenYao Defen
  53. 53. Features of acromegaly/gigantism.Features of acromegaly/gigantism. A 22-year-old man with gigantism due to excess growth hormone isA 22-year-old man with gigantism due to excess growth hormone is shown to the left of his identical twin.shown to the left of his identical twin. The increased height and prognathism.The increased height and prognathism. Enlarged hand and foot of the affected twin are apparent. Their clinical features began to diverge at the age of approximately 13 years.
  54. 54. HYPOTHALAMIC SYNDROMEHYPOTHALAMIC SYNDROME  Obesity is not cushingoid (not central)Obesity is not cushingoid (not central)  Striae (pink and not very large)Striae (pink and not very large)  Hypertension (constant or permanent)Hypertension (constant or permanent)  Glucose intoleranceGlucose intolerance
  55. 55. Increased activateIncreased activate of leptin receptorsof leptin receptors in hypothalamusin hypothalamus HyperleptinemiaHyperleptinemia in plasmain plasma Adipose depotAdipose depot IncreasedIncreased fat accumulationfat accumulation High level ofHigh level of leptin synthesisleptin synthesis Increased food intakeIncreased food intake reduced energy consumptionreduced energy consumption
  56. 56. HYPOTHALAMIC SYNDROMEHYPOTHALAMIC SYNDROME Autonomic-vascular formAutonomic-vascular form Sympatho-adrenaline crisisSympatho-adrenaline crisis: Increasing pressureIncreasing pressure TachycardiaTachycardia Cardiac respirationCardiac respiration PallorPallor FearFear TremblingTrembling AgitationAgitation Vago-insular crisis:Vago-insular crisis: HypotentionHypotention BradycardiaBradycardia SweatingSweating Heat sensationHeat sensation Redness of the faceRedness of the face Neuroendocrine formNeuroendocrine form Violations of water-salt metabolismViolations of water-salt metabolism Disturbance of thermoregulationDisturbance of thermoregulation Oligo- or amenorrheaOligo- or amenorrhea ObesityObesity HypertensionHypertension Neurotrophic formNeurotrophic form Change the color of the skinChange the color of the skin NarrowNarrow Bright deviceBright device Dryness and rashDryness and rash on the skinon the skin Early graying and hair lossEarly graying and hair loss Sleep Disorders and VitalitySleep Disorders and Vitality The attack sleepiness in other moment Cataplexy Acoustic and color nightmarish dreams
  57. 57. Treatment of hypothalamic syndromeTreatment of hypothalamic syndrome Sympatho-adrenaline crisis:Sympatho-adrenaline crisis: PiroksanPiroksan 1% - 1,0 g i/m1% - 1,0 g i/m PiroksanPiroksan 0.015 1 tablet 3 times/day 3 weeks0.015 1 tablet 3 times/day 3 weeks DopehitDopehit 0.25 1 tablet 3 times/day 3 weeks0.25 1 tablet 3 times/day 3 weeks Vago-insular crisis:Vago-insular crisis: Atropine sulfateAtropine sulfate 1% - 1,0 s/c1% - 1,0 s/c Extract of belladonnaExtract of belladonna 0.015 1 tablet 3 times/day 3 weeks0.015 1 tablet 3 times/day 3 weeks Nootropics:Nootropics: LutsetamLutsetam 800 mg 2 times/day 3 weeks800 mg 2 times/day 3 weeks Symptomatic therapy:Symptomatic therapy: ATPATP 1.0 g i/m (10 days)1.0 g i/m (10 days) Glutamic acidGlutamic acid 0.25 3 times/ day 3 weeks0.25 3 times/ day 3 weeks Dehydration therapy:Dehydration therapy: HypothiasidHypothiasid 50 – 100 mg/day50 – 100 mg/day MgSO4MgSO4 25 % solution i/m 10 – 15 times25 % solution i/m 10 – 15 times Physiotherapy:Physiotherapy: Galvanizing hypothalamic nuclesGalvanizing hypothalamic nucles 10 days to 15 minutes10 days to 15 minutes Hypocaloric diet:Hypocaloric diet: 1000-1500 kcal/day1000-1500 kcal/day

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