Adrenal disorder.

The Growth disorder and a

sexual development at children
and teenagers.
 Надпочечники состоят из :

Коркового слоя
Мозгового слоя
 Корковый слой состоит из:

Клубочковой зоны
Пучковой злны
Сетчатой зоны
 Клубочковая зона
Клубочковая зона –
минералокортикоиды (альдостерон)
Пучковая зона –глюкокортикоиды
(кортизол)
Сетчатая зона – андрогены
 Диагностика
Определение электролитов (натрия,
калия, хлора), глюкоза
крови,мочи,ЭКГ,рентгенограмма
поясничного отдела позвоночника.
Костный возраст
Проведение функциональных тестов
 Гипофункция коры
надпочечников
Гиперпигментация
Слабость , утомляемостью.
Артериальная гипотония
Задержка роста
Снижение массы тела
Диарея
Задержка полового развития.
 Лечение
Гидрокортизон 50 – 75 мг / м2 / в сутки или
1-2 мг/кг внутривенно или внутримышечно
Поддерживающая терапия: гидрокортизон
7 – 20 мг / м2 / в сутки за 2 – 3 приёма.
Если нет эффекта- минералокортикоиды:
флудрокортизон – 0,05- 0,2 мг/ сутки
доза подбирается от 1/8 таблетки.
 Гиперфункция коры
надпочечников
 Диспластической ожирение
 Атрофические изменения кожи
 Гиперпигментация
 Артериальная гипертензия
 Слабость
 Иммунодефицит
 Стероидный диабет
 Нарушение толерантности к углеводам
 Задержка роста
 Гипогонадизм
 Лечение
Оперативное
Радиационное облучение
Ципрогептадин и бромгептин.
 Body height of the child – genetical
programmed process of augmentation of the
linear and volumetric dimensions of an
organism at optimum entering
инградиентов deliveries and the favorable
environmental conditions, compounds one of
the basic performances of nursery age.
 The Biosynthesis and secretion GH
descends in somatotropes – the numerous
cells amounting about 50 % of mass of an
adenohypophysis.
GH represents одноцепочный a peptide
containing 191 amino acid.
 Synthesis and secretion GH is
checked by two subthalamic
neuropeptids – Somatotropinum –
рилизинг – a hormone – GHRH
and somatostatin SRIH
.
GHRH it is synthesized preferentially
in neurones аркуатного kernels, and
axons come to an end in the field of a
median prominence of a hypothalamus.
GH it is synthesized in neurones
перивентрикулярного plexuses,
паравентрикулярного,
супраоптического, аркуатного and
вентромедиального kerns of a
hypothalamus.
 FACTORS INFLUENCING
SECRETION of the GROWTH
HORMONE.
Inhibiting Boosting
 Somatoliberin
 Somatostatin  Androgens
 Reception of peep  Starvation
 Physical exercises
 Obesity  Hypoglycemia
 Antagonists of opiates  Agonists a beta – receptors
 L-допа
 (Naloxonum)  Arginine
 Glucocorticoids  Acetylcholinum
 Thyroid hormones
(At constantly raised level)  АКТГ
 Vasopressin
Hormones influencing body
height:

- the Somatostatin
-insulin
-TSH
-ACTH
-LH, FSH,GnR
 DIAGNOSTICS of GROWTH DISORDER
The Procedure of survey of patients with a growth disorder
includes:
 1. An estimate of body height and growth rate, proportions of a
body.
 2. Definition of biological (osteal) age
 3. The detailed anamnesis, including family, with analysis of
growth rate since a birth till the moment of the treatment.
 4. Standard survey for revealing the most spread somatopathies.
 5. Calculation of medial expected final body height.
 6. Examination of level GH.
 7. Carrying out of physiological functional assays.
 8. Carrying out of medicamental standard tests
 9. КТ or МРТ.
Pharmacological assays:
- with an insulin
- L – DOPA
- СТГ-РГ
- with a glucose
 Physiological (not
pharmacological)
Assays.
- examination спонтнанной
diurnal secretion.
- recording of peak GH during a
sleep.
A-exercise tolerance test.
CLASSIFICATION of the GROWTH
INHIBITION

1. Endocrino – dependent variants.

1.1. Insufficiency of GH

1.1.1. A panhypopituitarism

1.1.1.1. An idiopathic variant

1.1.1.2. An organic variant

1.1.2. The isolated deficit of a growth hormone

1.1.2.1. The expressed deficit

1.1.2.2. A particulate deficit
1.1.2.3. The selective deficit
1.1.2.4. A psychosocial nanism
1.1.3. Syndrome Laronae
1.2 Deficit of thyroid hormones
1.3. Disturbance of a level of
sexual hormones
1.4. Excess of glucocorticoids.
2. Endocrino – independent
variants.
2.1.Соматогеннообусловленные
2.2.Pathologic osteal system
2.3. Genetical and chromosomal
diseases
3. Features of physical
development
3.1. A syndrome serotinal
puberty

3.2. Family stunting

 The Pituitary nanism – educes
as a result of primary failure ГР
and is characterized by a
proportional growth inhibition
and developments of the child.
 Clinic
Backlog in body height is displayed up to 5 –
years age.
Excess of mass of a body
Спланхомикрия
Bones of a facial skull are abortive, the bridge
sinks down (the pupal face)
Skin dry with a yellow tone
Hair thin
Voice high
The sexual development is sharply impeded
The osteal age compounds l passport
 Subthalamic (the cerebral
nanism) – is characterized by
the expressed growth inhibition
вследствии depressions or lack
of secretion of a somatoliberin a
hypothalamus.
 Clinic
 Congenital small-for-date fetus
 Микро – or a macrocephalia
 Acromicria (decrease of the dimensions of a nose, a
chin, stacks, brushes - птицеголовые nanuses
 Backlog in body height appreciablly on the first to
year of life
 Disproportionality of a constitution of a body
 The wrong body height of dens
 Trophic changes of a skin and its appendages
 Disturbance водно – salt metabolism
 The sexual development comes self-maintainedly
 The osteal age lags behind from passport for 2 - 5
years.
 Treatment

-replaceable therapy ГР:

Сайзен, Генотропин,
Нордитропин.
In a dose 0,1 ЕД on kg in days
-high-grade delivery.
-vitamin therapy.
 Shereshevskogo-Turner's Syndrome –
the shape of a dysgenesis of the gonads,
bound to lack in a chromosomal
complement of one X – хромососмы (a
karyotype 45, X).
Views of mosaicism:
45, Х/46ХХ; 45, Х/46ХУ;
45, Х/46ХХ/47,
 Clinic:
 During newborn: a lymphatic edema of stacks, brushes, a upper
of a trunk and a neck
 With the years children the characteristic outward – they of low
body height. Final body height 142,0 ± 5,6 see
 Wide barrel thorax
 Neck коротнкая with the characteristic "pterygoid" cords
 Auricles are distort ed low had.
 Anomalies urinations systems
 The mass of a body is normally raised
 The osteal age lags behind from passport.
 Sexual infantilism.
 The Gigantism-proportional body
height of bones of an atomy and
augmentation of the dimensions of an
internals in comparison with medial age
norms вследствии supersecretion GH
at faces with not terminated
physiological body height.
 The parents of disease:
Eosinophilic adenoma of a forward
share of a pituitary body
Tumour of a hypothalamus
Cranial – a cerebral trauma
Contagious or inflammatory processes
Hypersensibility of epiphyseal cartilages
to a growth hormone.
 Clinic
Черезмерный proportional body height with a
little bit greater length of an extremity.
Muscular delicacy
Undue fatiguability
The back полусогнута, educes a kyphosis or a
kyphoscoliosis.
At early offensive disease the long bones
promptly grow
Спланхомегалия
The sexual development at early stages is
enhanced, but then, in process of an attrition
of pituitary functions peters out.
 Treatment
Surgical or by means of radial therapy.
If the tumour is not taped, the certain
effect is reached by application of sexual
hormones.
 The Acromegalia –
disproportional body height of
an atomy, the soft tissues and an
internals вследствии a
supersecretion of a growth
hormone at the ended
physiological body height.
 Clinic
Sleepiness, nervousness, disturbance of
appetite, parasthesia, giddiness.
Disproportionate body build.
High body height, large skull, the large
features which are given away forward
надбровные of an arc.
The occlusion, tongue
увеличиваетсянарушается an articulation
is broken
Brushes and stops, утолщаются calcaneuses
are considerably enlarged.
During puberty retardation and a
underdevelopment of secondary sexual
attributes is scored.
 Treatment
Surgical and radial therapy.
Medicamental treatment: Sexual hormones:
estrogens, Progesteronum,
медроксипрогестерон – an acetate,
Parlodelum and other agonists of Dofaminum.
Last years there is begun application of
analogs of a somatostatin (сандостатин,
октреоид) – selectively inhibiting release GH
for a long time (till 8 o'clock)
 The Premature sexual
development – occurrence of
secondary sexual attributes in
girls before 7 years, at boys –
before 10 years.
 Classification
1. The true premature sexual development
Constitutional or идиопатитческая the shape;
Premature sexual development of a cerebral
genesis
2. A false premature sexual development
PSD an adrenal genesis
PSD gonadal a genesis
PSD it is endogenous – medicamental character
3. The incomplete shape: телархе and
адренархе.
 Treatment
Bottom консервативнлй therapies –
drugs with antigonadotrophic activity
(ципротеронацетат, декапептил,
pregnenoldione, андрокур, диферелин)
In case of revealing a tumour of ovaries
or adrenal glands surgical treatment is
shown.