Endokrin DR Jimmy-May2010

ENDOKRIN
dr. Jimmy H.W. , Sp.PA
ENDOKRIN
Kelenjar mengeluarkan hormon
Figure 24-1 Hormones released by the anterior pituitary. The adenohypophysis (anterior pituitary) releases five hormones that are in turn under the control of various stimulatory and inhibitory hypothalamic releasing factors. TSH, thyroid-stimulating hormone (thyrotropin); PRL, prolactin; ACTH, adrenocorticotrophic hormone (corticotropin); GH, growth hormone (somatotropin); FSH, follicle-stimulating hormone; LH, luteinizing hormone. The stimulatory releasing factors are TRH (thyrotropin-releasing factor), CRH (corticotropin-releasing factor), GHRH (growth hormone-releasing factor), GnRH (gonadotropin-releasing factor). The inhibitory hypothalamic influences are comprised of PIF (prolactin inhibitory factor or dopamine) and growth hormone inhibitory factor (GIH or somatostatin).
Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 6 April 2005 03:46 AM) 2005 Elsevier
Hormon :
1. Molekul pemberi sinyal permukaan sel
GH, Insulin Epinefrin, Histamin
interaksi
2. Steroid

masuk sel membran
Estrogen, Progesteron Glukokortikoid Tiroksin Asam retinoat
Figure 24-2 A, Photomicrograph of normal pituitary. The gland is populated by several distinct cell populations containing a variety of stimulating (trophic) hormones. B, Each of the hormones has different staining characteristics, resulting in a mixture of cell types in routine histologic preparations. Immunostain for human growth hormone.
Kelenjar Hipofise ( Pituitary )

1 cm, 0,5 gr, pada sella tursica Jenis hormon :
1. Adenohipofise - GH - PRL 2. Neurohipofise - ACTH - TSH - FSH - LH
- Oksitosin - Vasopresin - ADH
Figure 24-7 Homeostasis in the hypothalamus-pituitary-thyroid axis and mechanism of action of thyroid hormones. Secretion of thyroid hormones (T3 and T4) is controlled by trophic factors secreted by both the hypothalamus and the anterior pituitary. Decreased levels of T3 and T4 stimulate the release of thyrotropin-releasing hormone (TRH) from the hypothalamus and thyroid-stimulating hormone (TSH) from the anterior pituitary, causing T3 and T4 levels to rise. Elevated T3 and T4 levels, in turn, suppress the secretion of both TRH and TSH. This relationship is termed a negative-feedback loop. TSH binds to the TSH receptor on the thyroid follicular epithelium, which causes activation of G proteins, and cyclic AMP (cAMP)-mediated synthesis and release of thyroid hormones (T3 and T4). In the periphery, T3 and T4 interact with the thyroid hormone receptor (TR) to form a hormone-receptor complex that translocates to the nucleus and binds to so-called thyroid response elements (TREs) on target genes initiating transcription.
Hiperpituitarism
Oleh karena : Adenoma Hiperplasia Carcinoma Kelainan Hipotalamus
ADENOMA HIPOFISE :
10 % tumor otak Usia 30 50 tahun Satu jenis tumor 1 jenis hormon Makroskopis : Batas jelas, lunak Kecil (mm) besar (cm) Lesi besar invasive adenoma Perdarahan apoplexi
Mikroskopis :
Sel uniform, poligonal, jalur-
jalur/lembaran Jaringan ikat penyangga Inti uniform pleomorfik
Klinik :
bayangan pada sella tursica ekspansi sellar erosi tulang kerusakan diafragma Gangguan produksi hipopituitarisme Penekanan tumor gangguan chiasmo opticum (bitemporal hemianopsi) Tekanan intracranial naik
R
Pusing Mual/muntah
Figure 24-4 Pituitary adenoma. This massive, nonfunctional adenoma has grown far beyond the confines of the sella turcica and has distorted the overlying brain. Nonfunctional adenomas tend to be larger at the time of diagnosis than those that secrete a hormone.
Figure 24-4 Pituitary adenoma. This massive, nonfunctional adenoma has grown far beyond the confines of the sella turcica and has distorted the overlying brain. Nonfunctional adenomas tend to be larger at the time of diagnosis than those that secrete a hormone.
PROLAKTINOMA
Tumor hipofise terbanyak ( 30 % ) Usia 20 40 th, pria > wanita Mikro atau makro Efek dari tumor PRL naik - amenorrhea - galactorrhea - libido kurang - infertil
PROLAKTINOMA
Prolaktin tinggi juga karena : Hamil Stress Hiperplasi sel laktotrof
PROLAKTINOMA
Hiperplasi sel laktotrof karena : Hipotalamus rusak neuron dopaminergik rusak Obat yang menekan reseptor dopamin pada hipofise - phenotiazin - reserpin - haloperidol
PROLAKTINOMA
Terapi : Bromocriptin dopamin
sebagai antagonist receptor
Somatotrof Adenoma
Mutasi somatik monoclonal Efek tumor G H meningkat Anak gigantisme Dewasa acromegali G H tinggi juga dapat karena : - Disfungsi gonad - Arthritis - DM - Congestive jantung - Hipertensi - Ca Gastro intestinal
KORTIKOTROF ADENOMA
Sering sebagai mikroadenoma Efek ACTH naik hiperkortikoisme (penyakit Cushing) Nelson sindrom : - Kortikotrof adenoma lebih besar (setelah adrenal dioperasi) - Hiperpigmentasi (karena MSH tinggi)
Hipopituitarisme, karena :
Fakta Hipofise : Tumor non fungsionil / kista Operasi / radiasi Apoplexi Ischemic necrosis/post partum necrosis (Sheehan syndrome) Empty sella syndrome Genetik
Fakta Hipotalamus : Tumor primer / sekunder Infeksi / degenerasi Klinik hipopituitarisme : 1. Fungsi kelenjar perifer turun
Adrenal Thyroid Gonad
2. Wajah pucat MSH rendah 3. Atrofi genitalia
Hipofise posterior
Hormon produksi
ADH Oksitosin
Diabetes insipidus
ADH rendah Etiologi : trauma, infeksi, tumor Klinik : - haus - urine banyak - Na serum tinggi, osmositas
Syndrome of Inappropriate ADH secretion :

ADH tinggi Etiologi : Ca small cell paru-paru Klinik : - urine sedikit - Na serum rendah
Tumor Hipotalamus
Glioma Craniopharyngioma
Craniopharyngioma
Dari : Vestigical Remnants Rathke Pouch Usia : anak dewasa muda Morfologi : Umumnya jinak Soliter, kistik, multiloculated Mirip adamantinoma
Thyroid
Asal : evaginasi epitel pharyngeal Normal : 15-20 gr Hormon aktif : - T3,T4 bebas - ikatan dengan TBG
Thyroid
fungsi :
Katabolisme : - karbohidrat - lemak Sintesa : - protein
Thyroid
Goitrogen (bahan penghambat sintesa hormon)
Propil tiourasil oksidasi Jodium Jodium pelepasan T3,T4 Jodium dosis tinggi proteolisis tiroglobulin
Thyroid
C cell produksi Calcitonin kerja untuk - absorbsi calcium - kerja osteoclast
Hipertiroidisme
lab : T3,T4 tinggi Gejala : - nervous - lemah otot - palpitasi - kurus - tremor - diare - kulit panas - tiroid besar
keringatan emosionil capek gangguan siklus M
Hipertiroidisme
Tirotoxicosis dapat karena :

Diffuse hiperplasi (85% Graves) hipertiroidisme Tx hormon tiroid berlebihan Multinodular goiter Neoplasma tiroid Tiroiditis
Hipertiroidisme
Terjadi :
1. Hipermetabolik 2. Overaktif simpatetik
Hipertiroidisme
Gejala Hipertiroid : 1. Cardiac : aritmi/palpitasi/cardiomegali 2. Otot : atrofi / fatty changes 3. Tulang : osteoporose, fraktur 4. Limfoid : hiperplasi
Hipertiroidisme
5. Ocular : Staring gaze, lid lag 6. Neuromuscular : tremor, cemas, insomnia, emosional 7. Kulit : berkeringat, rasa panas, kemerahan 8. GI : rasa haus, lapar
Hipertiroidisme
Dx :
Tanda klinik o Lab : - T4 bebas >> - T S H <<
o
Tx :
F blocker fungsi adrenergic propil tiouracil sintesa T3T4 jodium pelepasan T3T4 radioactive jodium
Hipotiroidi
Sebab : 1. Primer gangguan tiroid 2. Sekunder
Hipotiroidi
Hipotiroidi karena parenchim tiroid Embrional Radiasi Operatif Hashimoto :
Hipotiroidi
Hipotiroidi karena sintesa : Idiopatik Cacat sintesa turunan Jodium intake kurang Bahan-bahan goitrogen
Hipotiroidi
Hipotiroidi karena supratiroidal : Lesi hipofise Lesi hipotalamus
Cretinisme
Bayi, anak-anak Daerah rendah jodium, pegunungan Gangguan metabolisme
Cretinisme
Ciri klinik : Pertumbuhan skeletal dan syaraf Antara lain : Retardasi mental Tubuh pendek, kecil Wajah kasar Lidah menjulur Hernia umbilicus
Mixedema
Pada anak besar dewasa Aktifitas fisik dan mental Tanda-tanda klinik : - fatique - apatis - bicara & intelektual - tidak tahan dingin
Mixedema
Tanda-tanda klinik : - gemuk - simpatetik : konstipasi, kulit kering/dingin/pucat - cardiac output : napas pendek - tidak tahan dingin
Mixedema
PA : - matrix glycosaminoglycan - matrix hyaluronicacid Lab : - T4 , T3 - TSH
seluruh jaringan
Hashimoto Thyroiditis
Penyakit autoimmune hipotiroidi Umur 45-65, : = 10-20 : 1 Ada unsur familiar twin monozigote = 30-60% Sering disertai Rh, arthritis, SLE
Patogenesis
Terdapat HLA DR3, HLA DR 5 Defect T cell mengenai MHC Ag T cell suppressor T cell helper CD8+ cytotoxic Ab B cell epitel rusak
Figure 24-9 Pathogenesis of Hashimoto thyroiditis. Three proposed models for mechanism of thyrocyte destruction in Hashimoto disease. Sensitization of autoreactive CD4+ T cells to thyroid antigens appears to be the initiating event for all three mechanisms of thyroid cell death. See the text for details.
Ab yang timbul
Ab Tiroglobulin Ab Tiroperoxidase Ab TSH rec menghambat T3 T4 Ab Jodium transporter
Morfologi
Diffuse, berbatas jelas Pucat, abu-abu, kenyal, noduler Kapsul intak
Klinik
Struma tidak nyeri, simetrik diffuse Kadang-kadang noduler Hipotiroidisme, kadang-kadang hipertiroidisme transien Risk factor timbul limfoma
Figure 24-9 Pathogenesis of Hashimoto thyroiditis. Three proposed models for mechanism of thyrocyte destruction in Hashimoto disease. Sensitization of autoreactive CD4+ T cells to thyroid antigens appears to be the initiating event for all three mechanisms of thyroid cell death. See the text for details.
De Quervain Tiroiditis :
Jarang terjadi Usia 30 50 tahun Wanita : Pria = 3 5 : 1
Patogenesis :
Ada faktor infeksi virus, seperti : - coxsachie virus - measles - mumps - adenovirus Ada HLA B 35 virus Ag makrofag (HLA B35) jaringan rusak
Sel rusak
cytotoxic
T cell
Morfologi :
Unilateral / bilateral Kenyal, kapsul intak Kadang-kadang perlekatan jaringan sekitar Warna kuning pucat, kecoklatan
Klinik :
Terjadi mendadak / bertahap Nyeri leher, panas, capek, malas, anorexi, myalgin Terdapat struma Dapat sembuh spontan T3 T4 , TSH
Figure 24-11 Subacute thyroiditis. The thyroid parenchyma contains a chronic inflammatory infiltrate with a multinucleate giant cell (above left) and a colloid follicle (bottom right).
Graves Disease
Triad yang harus ada :

1. Hipertiroidi, dengan goiter aktif 2. Ophthalmopathy exophthalmos 3. Dermatopathy pretibial myxedema
Klinik : o Usia 20-40 tahun, wanita : pria = 7 : 1 o Ada faktor genetik o Terdapat MHC - haplotype HLA DR3 HLA DR8
Figure 24-8 A patient with hyperthyroidism. A wide-eyed, staring gaze, caused by overactivity of the sympathetic nervous system, is one of the features of this disorder. In Graves disease, one of the most important causes of hyperthyroidism, accumulation of loose connective tissue behind the eyeballs also adds to the protuberant appearance of the eyes.
Patogenesis
Terdapat Ab :
1. TSI = Thyroid stimulating immnuoglobulin 2. TGI = Thyroid growth stimulating immnuoglobulin 3. TSH binding inhibitor immunoglobulin ( antibody anti TSH receptor )
Figure 24-12 Diffusely hyperplastic thyroid in a case of Graves disease. The follicles are lined by tall, columnar epithelium. The crowded, enlarged epithelial cells project into the lumens of the follicles. These cells actively resorb the colloid in the centers of the follicles, resulting in the scalloped appearance of the edges of the colloid.
Teori terjadinya Graves :

1. Molecular mimicry :
Proses infeksi Bahan2 exogen
proses imunitas
Ab
2. Primary T cell autoantibody : MHC Ag HLA DR gene protein Ag Ab B cell T cell active
Ophthalmopathy graves
Jaringan ikat orbita Otot extra ocular Ab B cell
Mirip TSH receptor
T cell, CD4+ / CD8+
Morfologi : Struma, Sp 80 gr, simetri berkapsul Konsistensi lunak, halus, merah seperti daging Sel-sel silindris, papil-papil kecil Colloid sedikit, dengan scalloped margin Infiltrasi limfosit ( B cell )
Terapi : Jodium
involusi epitel sekresi tiroglobulin turun Propilthiouracil sintesa kurang Radioaktif jodium pembedahan
Diffuse Non Toxic ( simple ) Goiter

a. Endemic Pada semua usia terutama muda Pada daerah rendah jodium pegunungan 10 % populasi (+) Ada faktor goitrogen : - calcium - lobak - kubis - singkong - bloomkol
b. Sporadik Wanita > pria Usia pubertas dewasa muda Terdapat perubahan kelainan pada : transport jodium - dehalogenase - organification - iodotyrosin coupling
Morfologi : 2 stadium 1. Hiperplasi membesar simetrik diffuse 100 150 gr 2. Involusi colloid lebih besar, colloid lebih banyak colloid goiter Klinik : pada anak2 hipotiroidi cretinisme Pada dewasa keluhan sedikit
Multinodular goiter
Dapat sporadik atau endemik Usia lebih tua Menyerupai neoplasm
Patogenesis
TSH
Tiroid
Hiperplasi Involusi colloid fibrosis kalsifikasi
Folikel besar pecah
Nodul2 bergabung
Perdarahan
Multinodular goiter
Morfologi :
Multilobulated sp 2.000 gr Asimetrik, masuk ke substernal goiter plunging
Bila 1 nodul dominan solitary nodule (adenomatous goiter) Irisan warna coklat, gelatinous, fibrosis, perdarahan, kalsifikasi, kistik Mikroskopis : folikel banyak colloid, epitel pipih, atrofik/hiperplasi
Klinik :
Bila besar gangguan kosmetik tekanan pada trachea, oesophagus, vena2
Bila salah satu nodulnya hiperfungsi Plummer Syndrome
Figure 24-13 Nodular goiter. The gland is coarsely nodular and contains areas of fibrosis and cystic change.
Neoplasma Tiroid
Bentukan soliter, palpable Wanita : pria = 4 : 1 Sebagian besar nodul soliter jinak Nodul neoplastik 90% adenoma
Beberapa kriteria penyokong Dx

Nodul soliter neoplasma Usia muda neoplasma Jenis kelamin laki-laki neoplasma Pernah diterapi R Ca Hot nodule jinak
Adenoma tiroid
Soliter Folikel follicular adenoma Beberapa jenis, tersering : simple colloid adenoma Adenoma sangat jarang menjadi Carcinoma
Figure 24-14 Follicular adenoma of the thyroid. A solitary, well-circumscribed nodule is seen.
Patogenesis
TSH receptor merupakan keluarga 7 anggota transmembrane kelompok protein G receptor TSH rec subunit stimulating guanine nucleotide binding protein Gs adenyl cyclose C AMP gene aktif hormon , proliferasi
Patogenesis
Mutasi somatik stimulasi C AMP kronik sel tumbuh lebih cepat autonomously functioning monoclonal adenoma
Morfologi adenoma
Tumor soliter Bentuk speris Berkapsul, tekanan jaringan sekitar Ukuran sekitar 3 cm Warna abu-abu putih, merah kecoklatan Kadang2 perdarahan, fibrosis, kalsifikasi, kistik
mikroskopis
Folikel ukuran sama, isi folikel Jenis : - Simple colloid (macrofolicular) - Fetal (microfolicular) - Embryonal (trabecular) - Hurthle cell (oxiphyl, oncocyte) - Atypical - Adenoma with papillae (=Papillae adenoma) (=Encapsuled Papillary Ca)
Tumor Tiroid Jinak yang lain

Kista tiroid : - deri adenoma folicular - dari multinodular goiter Kista dermoid Lipoma Hemangioma Terratoma
Carcinoma Thyroid
Umumnya usia dewasa Wanita > pria, khususnya usia muda Terdapat reseptor estrogen pada sel-sel tumor
Jenis Carcinoma
Papillary Ca Follicular Ca Medullary Ca Anaplastic Ca 75-85 10-30 5 5 % % % %
Patogenesis
Faktor radiasi terutama usia dibawah 20 th Adanya colloid goiter adan Thyroiditis Terjadinya aktivasi/mutasi RET oncogen terutama papillary & medullary Carcinoma
Patogenesis
Mutasi RAS gene terjadi pada adenoma dan carcinoma Mutasi inaktivasi pada gene tumor suppressor P53 khususnya pada anaplastic carcinoma
Papillary Carcinoma
Semua usia, terutama 20-40 tahun Erat hubungannya dengan fakta radiasi
Figure 24-17 Papillary carcinoma of the thyroid. A, The macroscopic appearance of a papillary carcinoma with grossly discernible papillary structures. This particular example contains well-formed papillae (B), lined by cells with characteristic empty-appearing nuclei, sometimes termed "Orphan Annie eye" nuclei (C). D, Cells obtained by fine-needle aspiration of a papillary carcinoma. Characteristic intranuclear inclusions are visible in some of the aspirated cells.
Figure 24-17 Papillary carcinoma of the thyroid. A, The macroscopic appearance of a papillary carcinoma with grossly discernible papillary structures. This particular example contains well-formed papillae (B), lined by cells with characteristic empty-appearing nuclei, sometimes termed "Orphan Annie eye" nuclei (C). D, Cells obtained by fine-needle aspiration of a papillary carcinoma. Characteristic intranuclear inclusions are visible in some of the aspirated cells.
Morfologi
Soliter atau multipel Berbatas jelas / berkapsul / menyebar diluarnya Kadang2 fibrosis, kalsifikasi, kistik Pada irisan granula / papil-papil kecil
Mikroskopis :
Papil dengan fibrovasculer, dilapisi epitel

Inti dengan ground glass / orphan annie
Intra nuclear inclusion / groves

Psammoma bodies
Klinik
Sering a symptomatic Sering dengan metastasis kelenjar leher Radioactive jodium cold nodule FNA, cara Diagnosa yang tepat
Follicular Carcinoma
Wanita > Pria Usia 40 50 tahun Sering sudah didapatkan colloid goiter
Morfologi :
Single nodule Batas jelas / infiltratif Tumor besar infiltrasi ke jaringan sekitar Warna abu-abu coklat merah muda Kadang2 fibrosis, kalsifikasi
Figure 24-18 Follicular carcinoma. Cut surface of a follicular carcinoma with substantial replacement of the lobe of the thyroid. The tumor has a light-tan appearance and contains small foci of hemorrhage.
Figure 24-19 Follicular carcinoma of the thyroid. A few of the glandular lumens contain recognizable colloid.
Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 6 April 2005 02:32 PM) 2005 Elsevier
mikroskopis :
Folikel2 seperti normal, atau dengan diferensiasi yang rendah Kadang2 dengan sel Hurthle Invasi sel pada kapsul atau vascular
Klinik :
Nodul kecil, lambat laun membesar R cold nodule Metastasis hematogen ke organ-organ jauh
Medullary Carcinoma
Dari para follicular cell Hormon yang dikeluarkan -Calcitonin - Serotonin -CEA - Somatostatin -VIP (Vasoactive Intestinal Peptide)
Medullary Carcinoma
80% type sporadic 20% a. termasuk MEN syndrome b. familial Sporadic & familial dewasa 40-50 tahun MEN syndrome anak-anak
Figure 24-21 Medullary carcinoma of thyroid. These tumors typically show a solid pattern of growth and do not have connective tissue capsules. (Courtesy of Dr. Joseph Corson, Brigham and Women's Hospital, Boston, MA.)
Figure 24-21 Medullary carcinoma of thyroid. These tumors typically show a solid pattern of growth and do not have connective tissue capsules. (Courtesy of Dr. Joseph Corson, Brigham and Women's Hospital, Boston, MA.)
Morfologi
Soliter type sporadic Multipel type familial Jaringan tumor halus, warna abu2coklat Kadang2 nekrosis, perdarahan
Klinis
Nodul di thyroid Kadang2 disertai diare karena VIP Type sporadic / MEN tumbuh agresif Type familial low grade
Mikroskopis
Sel poligonal, spindle, dalam sarang/trabekula/folikel Deposit amiloid ( dari molekul calcitonin)
Anaplastic Carcinoma :
Sangat agresif Usia tua, 65 tahun Sering didahului multinodular goiter
Morfologi : Large, pleomorfik giant cell Spindle cell Small anaplastic cell
Congenital anomali Tiroid

Ductus/cyst thyroglossus Sisa2 vestigial remnant Lesi kecil 2-3 cm Letak antara Glossus - Thyroid
Parathyroid
Dari kantung pharyngeal, ada 4 kelenjar Berat 35-40 mg Terdiri dari - chief cell germal parathormon - oxyphil cell Kerja parathyroid dikendalikan oleh Ca ion darah
Figure 24-24 Parathyroid adenomas are almost always solitary lesions. Technetium-99m-sestamibi radionuclide scan demonstrates an area of increased uptake corresponding to the left inferior parathyroid gland (arrow). This patient had a parathyroid adenoma. Preoperative scintigraphy is useful in localizing and distinguishing adenomas from parathyroid hyperplasia, where more than one gland would demonstrate increased uptake.
Figure 24-25 Parathyroid adenoma. A, Solitary chief cell parathyroid adenoma (low-power photomicrograph) revealing clear delineation from the residual gland below. B, High-power detail of a chief cell parathyroid adenoma. There is some slight variation in nuclear size but no anaplasia and some slight tendency to follicular formation.
Hormon PTH bekerja :

a. Pada tulang menambah aktivitas osteoclast b. Pada ginjal meningkatkan - resorbsi calcium - konversi vit D aktif - ekskresi phosphat c. Pada usus menambah absorbsi kalsium
Tumor2 ganas yang lain darah tinggi
calcium
1. Metastasis tulang osteolisis 2. PTH related protein ( PTH rP )
Hiperparatiroidisme primer
Sebabnya :
Adenoma 75-80 Hiperplasia 10-15 Carcinoma 5 % % %
Usia tersering pada dewasa 50 th lebih Wanita lebih sering dp laki-laki Ada faktor radiasi sebelumnya
Figure 24-26 Cardinal features of hyperparathyroidism. With routine evaluation of calcium levels in most patients, primary hyperparathyroidism is often detected at a clinically silent stage. Hypercalcemia from any other cause can also give rise to the same symptoms.
Morfologi
Tumor soliter, kecil 0,5 5 gr Lunak, batas jelas, kecoklatan Mikroskopis :
Sel2 poligonal, uniform Inti kecil, central
Klinik, dapat berupa :

o A symptomatic hyperparathyroidism o Symptomatic hyperparathyroidism
Pada symptomatic timbul : Tulang osteoporosis Ginjal nephrolithiasis Gastrointestinal constipasi, ulcus dll CNS depresi Neuromuscular lemah Cardiac kalsifikasi katup
Adrenal Cortex :
Adrenal gland Adrenal cortex :
cortex medulla
1. Glucocorticoid (cortisol) 2. Mineralocorticoid (aldosteron) 3. Sex steroid (estrogen/androgen)
Adrenal medulla (epinephrin)
catecholamine
Figure 24-47 Nodular hyperplasia of the adrenal contrasted with normal adrenal gland. In cross-section, the adrenal cortex is yellow, thickened, and multinodular, owing to hypertrophy and hyperplasia of the lipid-rich zonae fasciculata and reticularis.
Figure 24-48 Consequences of C-21 hydroxylase deficiency. 21-Hydroxylase deficiency impairs the synthesis of both cortisol and aldosterone. The resultant decrease in feedback inhibition (dashed line) causes increased secretion of adrenocorticotropic hormone, resulting ultimately in adrenal hyperplasia and increased synthesis of testosterone. The sites of action of 11-, 17-, and 21-hydroxylase are shown by the numbers in circles.
Adrenocorticol hyperfunction (hyperadrenalism) :
1. Cushing syndrome 2. Hyperaldosteronism 3. Adrenogenital syndrome
Cushing syndrome
Produk glucocortikoid Sebab2 glucocorticoid tinggi :
1. Pemberian obat2 glucocorticoid luar (iatrogenic) 2. Primary hypothalamic pituitary disease 3. Hypersecretion of cortisol in adrenal 4. Secretion of ectopic ACTH in non endocrine neoplasm
Figure 24-43 A schematic representation of the various forms of Cushing syndrome, illustrating the three endogenous forms as well as the more common exogenous (iatrogenic) form. ACTH, adrenocorticotropic hormone.
Figure 24-44 The major causes of primary hyperaldosteronism and its principal effects on the kidney.
Pituitary Cushing syndrome (50%)

Adenoma hipofise ACTH Hyperplasia hipofise karena trofik hormon hipofise ciri : - cortisol - ACTH
Adrenal Cushing syndrome (15-30%)

Adenoma / carcinoma adrenal Hyperplasia adrenal ciri : - cortisol - ACTH
Paraneoplastic Cushing syndrome

Small cel Ca paru Carcinoid tumor Medullary Ca thyroid Islet cell Ca pancreas ciri : - cortisol - ACTH (karena corticotropin releasing factor dari tumor) Morfologi Jaringan hipofise & adrenal kondisinya tergantung jenis kelainannya
Klinik hypercorticolisme
Hipertensi Kegemukan Lemah otot atrofi Hyperglicemia Glicosuria Polidipsi Kulit tipis, fragil, striae Truncal obesity Moon faces Buffalo hump Osteoporosis Mental disturbances Hersuitisme
Figure 24-45 Adrenal cortical adenoma. The adenoma is distinguished from nodular hyperplasia by its solitary, circumscribed nature. The functional status of an adrenal cortical adenoma cannot be predicted from its gross or microscopic appearance.
Figure 24-46 Histologic features of an adrenal cortical adenoma. The neoplastic cells are vacuolated because of the presence of intracytoplasmic lipid. There is mild nuclear pleomorphism. Mitotic activity and necrosis are not seen.
Insufisiensi Adrenocortical
1. Primary acute adrenocortical insufficiency (adrenal crysis) 2. Primary chronic adrenocortical insufficiency (Addison Disease) 3. Secondary adrenocortical insufficiency
Adrenal crysis
Chronic adrenocortical insufficiency U pasien tambah parah Tx corticosteroid withdrawl Adrenal hemorrhage :
Bayi lahir dg masalah Tx anticoagulant DIC Bacteremia
Waterhouse Friderichsen Syndrome anak2
Bacterial infection :
Neisseria meningitis Pseudomonas Haemofilus influinza
Hypotensi DIC
shock
Figure 24-49 Waterhouse-Friderichsen syndrome in a child. The dark, hemorrhagic adrenal glands are distended with blood.
Figure 24-50 Waterhouse-Friderichsen syndrome. At autopsy, the adrenals were grossly hemorrhagic and shrunken; microscopically, little residual cortical architecture is discernible.
Friderichsen Syndrome
Adrenocortical insuff karena Adrenal haemorrhage dan bacterial infection Ciri-ciri : Infeksi karena - neisseria meningitis - pseudomonas - Pneumococci - haemophilus influenza Hipotensi progresif shock
Friderichsen Syndrome
Ciri-ciri :
Coagulasi menyebar purpura (pada kulit) Adrenocorticol insufficiency karena perdarahan
Klinik :
Biasanya pada anak-anak
Addison disease
= Primary chronic adrenocortical insufficiency Gejala klinik timbul bila lebih 90 % adrenal rusak Patogenesis 90% karena autoimune adrenalitis, TBC, metastasis Ca
1. Autoimmune adrenalitis (60-70%)

Selain adrenal organ lain sering ikut terjadi proses autoimmune tsb, yaitu pada
Hashimoto Pernicious anemia DM type II Idiopathic hypoparathyroidism
Didapatkan antiadrenal antibodies Didapatkan HLA B8 DR 3
2. infection
Sering karena TBC 90% Addison (dulu)
3. Metastatic Carcinoma
Ca dari paru-paru, mamma dll
Figure 24-52 Adrenal carcinoma. The hemorrhagic and necrotic tumor dwarfs the kidney and compresses the upper pole.
Figure 24-52 Adrenal carcinoma. The hemorrhagic and necrotic tumor dwarfs the kidney and compresses the upper pole.
Medulla Adrenalis
Sel chromaffin catecholamines (epinephrin, norepinephrin) Sel ini diinduksi oleh serat syaraf preganglion sistem sympathetic Norepinephrin : = adrenalin, pada sirkulasi aliran darah E/F adrenergic receptor sel > aktif (mis: myocard , vasoconstricsi )
Sel chromaffin adalah termasuk sel neuroendocrin (epinephrin, norepinephrin) Sel neuroendocrin (diluar Medulla adrenalis) membentuk kelompok2 /nodul paraganglion sistem ini termasuk sistem syaraf otonomic ada 3 : - bronchiomeric - intravasal - aortico sympathetic
Pheochromocytoma
Dari sel chromaffin catecholamin 85% timbul pada medulla, yang lain dari paraganglion extra adrenal 90% sporadic 10% autosomal dominant, familial syndrome
Pheochromocytoma
Type sporadic usia 40-60 th Wanita sedikit lebih banyak Type familial anak-anak, laki-laki > Dx ganas hanya ditentukan metastasis +/-
Klinik : Tanda utama hipertensi Nyeri dada, nusea, vomiting Pada jantung catecholamin cardiomyopathy
Figure 24-55 Pheochromocytoma. The tumor is enclosed within an attenuated cortex and demonstrates areas of hemorrhage. The comma-shaped residual adrenal is seen below.
Figure 24-55 Pheochromocytoma. The tumor is enclosed within an attenuated cortex and demonstrates areas of hemorrhage. The comma-shaped residual adrenal is seen below.
Neuroblastoma
Lokasi : o Adrenal medulla o Tempat lain : sistem syaraf sympathetic
Pineal
Pine cone shaped 100 180 gr Tumor pinealoma
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Endokrin DR Jimmy-May2010

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ENDOKRIN

dr. Jimmy H.W. , Sp.PA

masuk sel membran

Estrogen, Progesteron Glukokortikoid Tiroksin Asam retinoat

Kelenjar Hipofise ( Pituitary )

- Oksitosin - Vasopresin - ADH

jalur/lembaran Jaringan ikat penyangga Inti uniform pleomorfik

sebagai antagonist receptor

2. Wajah pucat MSH rendah 3. Atrofi genitalia

Syndrome of Inappropriate ADH secretion :

keringatan emosionil capek gangguan siklus M

Sebab : 1. Primer gangguan tiroid 2. Sekunder

Bayi, anak-anak Daerah rendah jodium, pegunungan Gangguan metabolisme

Triad yang harus ada :

Teori terjadinya Graves :

Jaringan ikat orbita Otot extra ocular Ab B cell

Mirip TSH receptor

T cell, CD4+ / CD8+

Diffuse Non Toxic ( simple ) Goiter

Hiperplasi Involusi colloid fibrosis kalsifikasi

Folikel besar pecah

Bila salah satu nodulnya hiperfungsi Plummer Syndrome

Beberapa kriteria penyokong Dx

Tumor Tiroid Jinak yang lain

Papil dengan fibrovasculer, dilapisi epitel

Intra nuclear inclusion / groves

Congenital anomali Tiroid

Hormon PTH bekerja :

Tumor2 ganas yang lain darah tinggi

1. Metastasis tulang osteolisis 2. PTH related protein ( PTH rP )

Klinik, dapat berupa :

Adrenal gland Adrenal cortex :

1. Glucocorticoid (cortisol) 2. Mineralocorticoid (aldosteron) 3. Sex steroid (estrogen/androgen)

Adrenal medulla (epinephrin)

Adrenocorticol hyperfunction (hyperadrenalism) :

1. Cushing syndrome 2. Hyperaldosteronism 3. Adrenogenital syndrome

Pituitary Cushing syndrome (50%)

Adrenal Cushing syndrome (15-30%)

Paraneoplastic Cushing syndrome

Waterhouse Friderichsen Syndrome anak2

1. Autoimmune adrenalitis (60-70%)

Didapatkan antiadrenal antibodies Didapatkan HLA B8 DR 3

Terima kasih Selamat belajar dr. Jimmy H.W., Sp.PA

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