Transcript In children

Behavioural changes in
endocrine diseases
DIABETES INSIPIDUS
DIABETES INSIPIDUS
Diabetes insipidus is a disorder of a large volume of urine
(diabetes) that is hypotonic, dilute, and tasteless (insipid).
This is opposed to the hypertonic and sweet urine of
diabetes mellitus (honey).
Definition:
polyuria-thirst- polydipsia (>3l/24h)
associated with urine hypoosmolality (<300mOsm/l)
Clinical characteristics
• sudden onset of symptoms
• persistent thirst throughout the day and night
• associated with a desire for cold liquids
Classification of diabetes insipidus
• Vasopressin (AVP) and
oxytocin are synthesized
in the magnocellular
neurons of the
supraoptic and
paraventricular nuclei.
• They migrate along the
axons of the
magnocellular neurons,
to be stored and
released at the ends of
the neuronal tracts.
• Renal action
The physiologic regulation of vasopressin synthesis
and secretion involves two systems:
Osmotic and Pressure/volume
• Pressure/volume
• High pressure arterial baroreceptors are located
in the carotid sinus and aortic arch and
• Low pressure volume receptors are located in the
atria and pulmonary venous system.
• The afferent signals are carried through cranial
nerves IX and X.
• When the hypovolemia is sufficient to cause a
decrease in blood pressure (5-10%), there is a
sudden and exponential increase in the level of
vasopressin in plasma
Osmotic system
• The primary receptors for sensing changes in
osmolality are located in the brain.
• !Osmoreceptors must be outside the bloodbrain barrier.
• Primary osmoreceptors: organum vasculosum
of the lamina terminalis (OVLT) and areas of
the anterior hypothalamus
• Extracellular fluid osmolality (predominantly
determined by sodium concentration) varies from
280 to 295 mOsm/kg H2O in normal subjects but in
any individual is maintained within a narrow range.
• As little as a 1% increase or decrease in plasma
osmolality will cause a rapid increase/decrease in
plasma vasopressin by release of vasopressin from
the store of hormone in the posterior pituitary.
• When vasopressin is absent, 18 to 20 L/day are
excreted, but with an increase to as little as 0.5 to 1
pg/mL, urine volume is reduced to less than 4 L/day.
Thirst
• Represents the body's defense mechanism and water
consumption is increased in response to perceived deficits of
body fluids.
• As with to vasopressin, increases in osmolality of the
extracellular fluid (ECF) or decreases in intravascular volume
can stimulate thirst.
• ! Most humans consume the bulk of their ingested water as a
result of the relatively unregulated components of fluid
intake, such as
- the consumption of beverages in association with food intake,
- for reasons of palatability or
- for desired secondary effects (e.g., caffeine), or
- for social or habitual reasons (e.g., sodas or alcoholic
beverages);
Sexual hormones
Hypogonadism=sexual hormones deficiency
• In children : delayed puberty
• In women: chronic anovulation, amenorhoeae,
infertility, reduced libido, genitourinary atrophy,
osteoporosis
• In men: reduced libido, impotence, reduced
muscle mass, increased fat, osteoporosis, lethargy,
reduced testicular volume, hypospermia, hair loss,
fine wrinkling
Androgen excess
• Such cases may present
in early childhood with
signs of precocious
pseudopuberty such as
sexual precocity, pubic
hair development,
and/or growth
acceleration due to
premature androgen
excess.
GLUCOCORTICOIDS
• Clinical observations on patients with glucocorticoid
excess and deficiency reveal that the brain is an
important target tissue for glucocorticoids, with
depression, euphoria, psychosis, apathy, and lethargy
being important manifestations.
• Both glucocorticoid and mineralocorticoid receptors are
expressed in discrete regions of the rodent brain,
including hippocampus, hypothalamus, cerebellum, and
cortex.
• Glucocorticoids cause neuronal death, notably in the
hippocampus, which may underlie the recent interest in
glucocorticoids and cognitive function, memory, and
neurodegenerative diseases such as Alzheimer's.
GLUCOCORTICOIDS
• Central action on circuits involved in energy
homeostasis
• GC simulate hunger and weight gain
• Hypercortisolism => central fat distribution
Features of chronic adrenal insufficiency
• Weakness, tiredness, weight loss, nausea,
intermittent vomiting, abdominal pain, general
malaise, muscle cramps, and symptoms
suggestive of postural hypotension
• Psychiatric symptoms may occur in longstanding
cases and include memory impairment,
depression, and psychosis.
• Patients may be inappropriately diagnosed as
suffering from chronic fatigue syndrome or
anorexia nervosa.
Hypercortisolism
Hypercortisolism
1.01.2006
5.09.2007
21.11.2007
Deficiency of thyroid hormone in adult life
• Causes less severe manifestations that usually respond to
treatment with the hormone.
• All intellectual functions, including speech, are slowed in
thyroid hormone deficiency.
• Loss of initiative is present, memory defects are common,
lethargy and somnolence are prominent, and dementia in
elderly patients may be mistaken for senile dementia.
• Psychiatric disorders are common and are usually of the
paranoid or depressive type and may induce agitation
(myxedema madness).
• Cerebral hypoxia due to circulatory alterations may
predispose to confusional attacks and syncope, which may
be prolonged and lead to stupor or coma.
Hormonii necesită concentraţii optime pentru
determinismul comportamental
Modificările SN şi
comportamentale:
-letargie, astenie fizică,
scăderea atenţiei şi gândirii,
tulburări de memorie, încetinire
mentală, bradilalie şi ezitări în
vorbire, depresie, diminuarea
auzului, somnolenţă
- rar: sindroame neurologice,
reversibile la tratamentul de
substituţie tiroidiană, disfuncţie
cerebelară cu ataxie, tremor de
intenţie şi nistagmus
♀, 65 ani, mixedem
La vârstnici: alterarea funcţiei cognitive şi simptome depresive
Congenital myxedema
• Thyroid hormone is essential for the
development of the central nervous system.
Deficiency in fetal life or at birth impairs
neurologic development, including hypoplasia
of cortical neurons with poor development of
cellular processes, retarded myelination, and
reduced vascularity.
• ! If the deficiency is not corrected in early
postnatal life, the damage is irreversible.
Alterations in the function of the nervous
system in thyrotoxicosis
• Are manifested by nervousness, emotional lability, and
hyperkinesia.
• Fatigue may be due to both the muscle weakness and the
insomnia that are commonly present.
• Emotional lability is common and in rare cases mental
disturbance may be severe; manic depressive, schizoid, or
paranoid reactions may emerge.
• The hyperkinesia During the interview, the patient shifts
positions frequently, and movements are quick, jerky,
exaggerated, and often purposeless.
Hormonii necesită concentraţii optime
pentru determinismul comportamental
Manifestări SN şi comportamentale:
- nervozitate, iritabilitate, labilitate
emoţională, anxietate persistentă,
nelinişte, senzaţie de tensiune
interioară, instabilitate în modul de a
fi, somn neodihnitor, insomnii,
scăderea puterii de concentrare,
tulburări de memorie, fugă de idei,
delir
♀, 22 ani, boală Graves Basedow
- reacţie exacerbată în situaţii
stresante
Thyrotoxicosis in children
• In children, in whom such manifestations tend
to be more severe, inability to focus may lead
to deterioration of school performance,
suggesting attention deficit hyperactivity
disorder.
SIGNS AND SYMPTOMS ASSOCIATED WITH
CATECHOLAMINE-SECRETING TUMORS
• SPELL RELATED
Anxiety and fear of impending death
Diaphoresis
Dyspnea
Epigastric and chest pain
Headache
Hypertension
Nausea and vomiting
Pallor
Palpitation (forceful heartbeat)
Tremor