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HYPOPITUITARISM and GH DEFICIENCY

HORMONE LOSSwith pituitary lesions the hormones are lost in the following order:

GH > FSH/ LH > TSH > ACTH(an !e thought of as whih are most ruial to sur"i"al # an sur"i"e w/o GH !ut not w/o ACTH$

AETIOLOGY

Cause Isolated GH deficiency Multile ituita!y "o!#one deficiency

Congenital(geneti$

GH%H reeptorGH& A% (type &$

A' (type $)*lin+ed (type ,$

GH insensiti"ityGH reeptor (Laron$-GF*& geneGH a!normal

.%.& (prophet of .it &$ 0 GH1 TSH1 .%L1 FSH1 LH.it& (a+a .2&F&$ 0 GH1 TSH1 .%L onlyLH),H3S)& (septo*opti dysplasia$.T)*%-3G& (%ieger syndrome$

Congenital

(un+nown$

anenephaly

holoprosenephalypituitary aplasiasingle entral inisorempty sella syndromeHall*.allister syndrome

A4uired post radiationidiopathi

tumours (raniopharyngioma$traumaautoimmune/ infiltrati"e/ infeti"e

Isolated GH deficiencyIdioat"ic(567 of all auses$Post$!adiation (GH most susepti!le # an !e only hormone affeted$Genetic GH& A% (type &$ # omplete a!sene GH (no response to stimulation$

se"ere post*natal growth failureA' (type $)L (type ,$ 8/* hypogammaglo!ulinaemia

GH%H reeptorGH insensiti"ity Laron9s syndrome (num!er of mutations$

GH reeptor !inding: GH (!ut ation$

response to stimulation/ GH%H

-GF*& (no response to eogenous GH$

GH reeptor may !e / normal/

-GF*& gene defets a!sent -GF*& GH/ growth

Multiple pituitary hormone deficiency

Con%enitale!elopmental defectsanenephalyholoprosenephaly (ylopia1 e!oephaly1 or!ital hypotelorism$pituitary aplasia # pro!a!le A%1 rare

present with hypoglyaemia 8 miropenis 8 a!sene orpus allosum ( -;$

midfaial anomalies (hyperterlorism1 left lip/ palate1 solitary maillary entral inisor$empty sella syndrome # herniation of suprasellar su!arahnoid spae into sella turia

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flattening of pituitary 8/* hypopituitarism

Hall*.allister syndrome* a!sent pituitary gland 8 hypothalami hamarto!lastoma8 ardiorespiratory anomalies8 !ifid oesophagus 8 imperforate anus 8 renal anomalies8 polydatyly and nail dysplasia

Genetic defects

Tye Essence Ho!#onedeficient

Ho!#one!ese!&ed

.%.&(A% # type &$

una!le to !ind or ati"ate transriptionfators

regressed pu!erty

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Infecti!e!asal meningitis (espeially TD$1 tooplasmosis

"LINI"AL #EAT$RESCon%enital Hyoituita!is#

a!normal appearane due to GH

round head with prominent foreheadheru! fae (immature round and !road$ with !ulging eyes

midline hypoplasia # small Eaw and hindelayed dental eruption * fre4uently rowdedshort ne+hi pithed "oie (e"en after pu!erty$ # to small larynsmall hands and feetunderde"eloped genitalia miropenis 82'T (FSH/LH in utero$pale and hairless s+in (ala!laster s+in$

-; usually normal

tend to present with neonatal emergeniesapnoea and yanosis

se"ere DSL (should stimulate GH/ ACTH if normal or thin+ hypopituitarism$

slow feeding/ prolonged Eaundie/ hypothermia/ persistent post fontanelle (TSH$

growth failure: normal si@e and weight at !irth (may !e &S' !elow mean for length$gradual growth failure if se"ere>S' !elow !y &yr

without treatment adult height *& S' !elow mean

Pituita!y (esionompressi"e effets: -C.

"isual field defet (!itemporal hemianopia raniopharyngioma$

hydroephalus (,rd"entrile ompression$sei@ures

with destrution other pituitary hormones (only &67 at presentation$

FSH/ LH delayed pu!erty or regression (pu!i hair1 amenorrhea1 gonads$TSH hypothyroidism

ACTH DSL (symptomati DSL in &6*&?7$

'- may !e present early tends to resol"e as ant pituitary progressi"ely destroyed

IN%ESTIGATIONS)loodsGH le!el

sin%le le&elpro!a!ly not useful to ma+e diagnosis !ut does elude GH def if high

sti#ulation testsa!sent/GH in response to stimulus (67 normal ha"e no response$

l*dopa1 insulin1 arginine1 eerise1 lonidine1 gluagonafter stimulation &6 0 definitely a!normal (>6 0 normal$

*+$+, "! sa#lin% GH(6 minute inter"als$

some short hildren with normal GH ha"e little spontaneous GH seretion

!ut wide range of spontaneous GH seretion in normal hildren

Other pituitary hormonesTSH/T

ACTH/ ortisol (at Iam$'H3AS1 testosterone1 oestradiol1 LH1 FSHwater depri"ation testing (for A'H$

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I#a%in%s+ull )*ray

CT or K/&

contra)indications: malignany1 -'' & (girls$ and > &K (!oys$

ris+s: leu+emia pseudo*tumour ere!rislipped femoral epiphysis worsening soliosisgynaeomastia re"ersi!le hypothyroidism

in total !ody water in &st&* wee+s of treatment

CM' (pre"ious preparations$ reom!inant GH eliminated this ris+

outcome( maimal response in &

st

yearwith eah suessi"e year response

Ot"e! t"e!aies aa!t f!o# GH

-GF*& may !e used in: Laron9s sydrome (a!normal GH N$

GH& gene deletion 8 high titres GH anti!odies

GH%H in those with hypothalami auses of hypopituitarism (also need daily S/C inEetion$