mudr Congential Heart Disease

Document Sample
mudr Congential Heart Disease Powered By Docstoc
					    Pathology of growth and development in the postnatal
                              Programme schedule
                         Modul C, course 17, 4th year

Total number of teaching hours: 60 ( 2 weeks, 6 hours/day)

Taught by departments:

     Dept. of Paediatrics and Adolescent Medicine

Course co-ordinator:

     as. MUDr. Daniela Palyzová, CSc.


   The programme is liased to the 5 day course Introduction to Paediatrics in the 3rd year
     which was focused on the basic means of clinical examination of children.

   Teaching of Clinical Paediatrics in 5th and 6th year is based on the programme.

   The teaching covers the preclinical problems of paediatrics.

Recommended reading:

The list of recommended reading will be presented actually during the tutorials.

                                   Detailed programme
Day 1 : Physiology of growth and development in childhood

   Introduction (1 hr, As. Palyzová, As. Schneidrová)
   Development of human individual from birth to adulthood (2 hrs, Dr. Vosáhlo)
   Monitoring of growth and development (nationwide surveys, growth standards) (2 hrs, As.
   Practical use of growth charts (1 hr)

Day 2 : Congenital and developmental abnormalities

   The cardiovascular system (2 hrs, Doc. Votava)
   The digestive system (2 hr, As. Marx, Dr. Volf)
   Bedside teaching (2 hr)

Day 3 : Congenital and developmental abnormalities

   The urogenital system (2 hr, As. Palyzová)
   The respiratory system (2 hr, Doc. Votava)
   Bedside teaching (2 hr)

Day 4 : Nutrition

   The principles of nutrition, clinical aspects of disturbances of nutrition (3 hrs, As.
   Marx, Dr. Volf)
   Nutrition in adolescence, feeding disorders in adolescence (2hr, Dr. Volf)
   Bedside teaching (1 hr)

Day 5 : Psychomotoric development of child

   Pathology of neuropsychical development (2 hrs, Dr. Jahnová)
   Practical tutorial: developmental assessment (2 hrs, Dr. Jahnová)
   Handicapped child – organisation of care (1hrs, As. Palyzová)
   Study time (1 hr)

Day 6 : Bedside teaching - Practical tutorial (6hrs)

Day 7 : Adolescence
 Clinical approach to problems of pubertal development in boys and girls (3 hrs, Dr.
 Bedside teaching (3 hrs)

Day 8 : Child Abuse and Neglect
   Drug abuse in children and adolescents (2 hrs, As. Marx)
   Child abuse and neglect-panel discussion (2 hrs, As. Marx)

   Study time (2 hrs)

Day 9 : Handicapped child

   Excursion to the pediatric specialised care institution (4 hrs, Dr. Drahota)
   Study time (2 hrs)

Day 10 :

   Active and passive immunisation principles (2 hrs, As. Dáňová)
   Bedside teaching (2 hrs)
   Examination, crediting (1 hr, As. Palyzová, As. Marx, Doc. Votava, Dr, Volf)

Evaluation and disorders of psychomotoric development
1)  Introduction
    a. characteristics of psychomotoric development
    b. approaches to examination (observation, anamnestic data, informal testing, standard
       examination, combination of the all approaches mentioned above)
    c. great research workers in the field of developmental kinesiology
    d. conditions of physiological psychomotoric development
    e. main components of psychomotoric development (motor skills, sensoric functions,
       speech, social behaviour, sleep, others)
2) Development of motor skills
    a. necessity of standard conditions during examination (appropriate time and milieu,
       standard sequence of the both, testing positions and testing methods)
    b. important periods of motoric development (newborn age, premature newborn, second
       part of the Ist trimenone, the IInd trimenone, the IIIrd trimenone, the IVth trimenone, the
       Vth trimenone, toddler age, preschool age, school age and adolescence)
    c. description of the typical motor skills in particular periods of psychomotoric
       development (depending on lecture)
3) Development of speech
4) Development of social behaviour and fine motor skills
5) Development of sleep (amount and organization)
6) Development of electroencephalogram (EEG)
7) Disorders of psychomotoric development
    a. dividing (global or with predominance of the particular component, harmonic or
    b. causes of psychomotoric retardation (genetic, prenatal nociceptive agents, damage of
       brain during perinatal period, damage of brain in consequence of different adverse
       effects in the postnatal life, secondary retardation in consequence of failure of other
       systems and organs, negative influence of the social environment)
8) Genetic basis of psychomotoric retardation (examples)
    a. aneuploidias (Down syndrome)
    b. microdeletion syndromes
    c. syndrome of the fragile X-chromosome
    d. inherited metabolic disorders (examples depending on lecture – phenylketonuria,
       peroxisomal biogenesis disorders, syndrome Smith-Lemli-Opitz)
9) Embryopathy, fetopathy
10) Syndrome of cerebral palsy
    a. characteristics
    b. main causes (hypoxia – asphyxia, perinatal injury, sepsis, metabolic dysbalance,
       frequently in combination)
    c. main types (according motoric pathology)
    d. complications, associated disorders (mental retardation, epileptic seizures, secondary
       orthopedic changes)
    e. complex care, physiotherapy (common methods – according Vojta, Bobaths´, etc.)
11) Psychomotoric retardation as result of postnatal damage of brain
    a. injuries of brain
    b. serious neuroinfections
    c. neoplastic lesions of brain
    d. metabolic breakdown, hypoxia, vascular diseases
    e. secondarily in sensory deficiencies (hypacusis, amaurosis) or some malformation
    f. chronic social deprivation, child abuse

12) Developmental regression in monogenic inherited diseases
    a. characteristics
    b. examples depending on lecture (some inherited metabolic disorders, spinal
       amyotrophy type I, II, tuberose sclerosis)
13) Syndrome of mental retardation
    a. characteristics
    b. dividing on the basis of mental status level (inteligence quocient)and possible causes
14) Disorders of autistic spectrum (autism)
15) Disorders of speech development
16) Attention and hyperactivity disorders
17) Learning disabilities

Assessment of growth and development in childfren and
adolescents (population standpoints)
Monitoring of growth and development is an important preventive measure for:
- assessment of nutritional and health status;
- early detection of growth and development disorders due to malnutrition, illness or
psychosocial problems;
- follow-up efficiency of the treatment.

Biological and social determinants of growth and development
- genetic factors;
- environmental factors:
    * social environment (differences in ethnic groups, socioeconomic groups);
   * nutrition (obesity – main nutrition related problem in western countries);
- health status (health care, immunization).

WHO growth reference data for infants and young children
In 1977, the WHO recommended referential growth charts based on longitudinal studies of
the American population. These data were collected during a time when breastfeeding was in
decline and complementary foods were introduced at a very early age. A number of studies
revealed significant differences in growth between breastfed and formula fed infants.
Breastfed infants grow and gain weight faster in the first 2 months and then their growth and
weight gain slow down. Formula fed infants grow faster in the second half of infancy
probably due to an increased intake of proteins which stimulates the growth due to the
influence of some aminoacids on the secretion of insulin or a direct influence on the
secretion of IGF-1. Formula fed infants also gain higher weights and are at a greater risk for
In 1997-2003, the WHO carried out a Multicentre Growth Reference Study (MGRS) in
order to develop international growth standards based on a collection of data on growth
and development in 8500 breastfed infants from different ethnic and cultural backgrounds of
good socioeconomic conditions (Brazil, Ghana, India, Norway, Oman, USA) which replaced
the referential data (height, weight, weight/height ratio) based on the American population.
The new referential data on a triceps and subscapular skinfold, head and arm
circumference, body mass index (BMI) and growth velocity of followed up parameters were
published in 2006. These data are important for monitoring the childhood obesity and
malnutrition. The referential data of a motor development in children up to 5 years will be
also published. The revision of growth monitoring by health professionals will be undertaken

Monitoring of growth in CR (National Institute of Public Health)

    -   A long tradition of anthropological research of children and adolescents, first
        research carried out in 1895 by prof. Matiegka
    -   6 nationwide anthropological surveys every ten years from 1951 to 2001
    -   Basic bodily characteristics (weight, length, height, circumference of head, chest,
        waist, hip, arm) obtained in the representative sample – 3-5% of the population of
        the given age from 0 to 18 years (80-100.000) together with data on SES, dietary
        habits, sports activity, health status, etc.
    -   percentile graphs - standard instruments constructed on the basis of the growth
        referential data related to age and gender
    -   indexes calculated – BMI = weight(kg)/(height (m))2
                              - weight/height ratio – for younger children
    -   BMI = one of the most often used indices for the evaluation of actual body mass
        regarding the height. The percentile chart enables to assess the corpulence of an
        individual with regard to a reference population.

1995-96 – research on head dimensions, secular trend of height has stopped in girls and
boys in postpubertal age.

1997-99 – a semilongitudinal survey of growth in schoolchildren
2.000 schoolchildren (6 to 15 years old) from different regions (measured 6 times in 6
months intervals)
    - growth rate of basic parametres (1cm lower in girls than boys)
    - growth spurt in girls 4 months earlier than in 1975 (11 years – 7 cm/year)
    - growth spurt in boys 3 months earlier than in 1975 (13 year – 8 cm/year)
    - education of parents – correlated with the height positively and with the weight
    - children with severe illness were smaller (17% children).

1999-2000 – prevalence of obesity (European Child Obesity Group)
   - schoolchildren – 7-11 year (1529 boys, 1493 girls)
   - in comparison to 1991 – no increase in average BMI
   - increased prevalence of extremely obese children (5 % over 97th percentile) – found
       more frequently in the families with one child, in obese children inappropriate eating
       habits (frequency) found
   - distribution related to residence – lower prevalence in Prague in comparison to
       smaller towns.

2001 – 6th nationwide anthropological research
- anthropometric data (height, body weight and head, arm, waist and hip circumferences) on
18,584 children under 6 years of age and 40,525 school children and adolescents were
collected with the participation of pediatricians, teachers (kindergartens, schools), public
health staff and parents:
- the period of the fastest growth (maximum growth velocity) shifted gradually to younger age
categories (13 years for boys, 11 years for girls)
- the mean age of menarché in girls was 13 years, the mean age of voice breaking in boys
was 13.8 years
- these observations correspond to a more prominent slow-down of the secular trend in
height in girls and a less prominent height increment in boys
 - the proportion of 7 to 11 years old Czech children with overweight and obesity remains, in
comparison with other European countries is lower, however, the increasing trend towards
higher values is evident, the rates of obese children (>97th centile) were 2.4 % for boys and

1.6 % for girls in 2001, the rates of overweight (90th – 97th centile) were 12.1 % for boys and
9.8 % for girls, the overwight an obesity rates in children are declining with age.

Growth charts
In centile charts, represented lines correspond to the 3rd, 10th, 25th, 50th, 75th, 90th and 97th
centiles for reference data at a given age. The 50th centile represents the most frequent value
for the body parameter found in the reference population. Values above the middle line are
higher than the average of the population of a given age while those below the middle line
are lower than the average.
Under favourable conditions leading to full development of the genetic potential, i.e. when
adequate health care, nutrition and socio-economic conditions are available, the growth
curve of the followed child is parallel to centile curves in the range of the 25th to 75th centiles.

Growth charts based on the nationwide research are available for:

a) an individual assessment
- regular child growth monitoring is a part of preventive pediatric examinations (Health and
Immunization Record);
- endocrinology- used for the dg. of growth disorders;
- plastic surgery, etc.

b) population groups assessment
- environmental studies;
- nutritional assessment (weight/height ratio, BMI, skinfolds percentile graphs).

Bláha, P., Vignerová, J.: Investigation of the growth of Czech children and adolescents.
    National Institute of Public Health, Prague, 2002.
Garza, C., de Onis, M.: Introduction. Symposium: A New 21st-Century International Growth
    Standard for Infants and Young Children. Journal of Nutrition, 2007, 137:142-143.
Lener, J. et. al.: Medical Hygiene. Karolinum, 1997, s. 5-25.
Vignerová, J., Riedlová, J. et al.: 6th Nation-wide Anthropological Survey of Children and
     Adolescents 2001 Czech Republic. Summary results. Faculty of Natural Sciences and
     National Institute of Public Health, Prague 2006, 238 pp.
WHO Working Goup on the Infant Growth. An evaluation of infant growth. Geneva: World
     Health Organization, 1994.

Development of human individual : From birth to the onset of
Periodization of paediatric age
Newbort – main tasks, physical characteristics, abilities, psychosocial development
Infant – main tasks, physical characteristics, abilities, psychosocial development
Toddler – main tasks, physical characteristics, abilities, psychosocial development
Pre-school child – main tasks, physical characteristics, abilities, psychosocial development
School child – main tasks, physical characteristics, abilities, psychosocial development
Growth curve
Sandwich model of growth – infantile, childhood and pubertal growth period
Infantile growth period – main characteristics, regulation
IGF-I – function, regulation
Pathophysiological mechanisms of growth disorders in infantile growth period

Childhood growth period – main characteristics, regulation
Pathophysiological mechanisms of growth disorders in childhood growth period

Morphological basis of puberty (hypothalamo-pituitary-gonadal axis)
Minipuberty of newborn age
Transition of a child into an adult – patterns, participating hormonal systems
Pubertal development in boys
Pubertal development in girls
Growth curve – pubertal growth period, accumulation of peak bone and muscle mass
Cognitive and social development during puberty
Pubertal growth – main characteristics, regulation
Pathophysiological mechanisms in pubertal growth period
Puberty stage evaluation in boys and girls
Puberty disorders – precocious puberty
Puberty disorders – delayed puberty

Congenital developmental defects of respiratory tract
1. Development and functions of lung
Development of lung – stadias:
a) prenatal - morphogenesis
b) adaptation to atmospheric breathing, birth
c) proporcional growth 25 mil. alveoli (newborn.. 300 mil alveoli (adults)
Function of lung:
1) Prenatal: - production of amniotic fluid
2) Postnatal:
   - gas exchange
   - mechanical defense (mucus, ciliar movement)
   - immunological defence (fagocytosis, APC, secretory IgA
   - metabolic functions: .. conversion of angiotensin I .. II
   - communication by voice

2.Adaptation to atmospheric breathing, surphactant
Hydrophobic complex mixture of phspholipids and proteins producted by pneumocytes, type
II, „lining complex“ since gestation age 24 weeks, glukocorticoids enhaces its production,
lecithine-sphingomyeline ratio. First breaths are a complex event with aeration of lung with
creation of FRC, amniotic fluid drainage and change of circulation.

3. Congenital developmental defects
Choanal atresia
Uni or bilateral, dyspnea, danger of aspiration during feeding – it is a life threatening
condition, could be a part of a syndrome – f.e. CHARGE.

Stridor laryngis congenitus
this relatively common condition involves the collapse of the arytenoid cartilages during
inspiration which produce an inspiratory stridor. Usually is benign and self-limiting.
Diagnosis: laryngoscopy to rule out other laryngeal stenosis such a hemangioma.

The tracheal cartilage rings normally extend through an arc of approximately 300 degrees,
thus maintaining rigidity of the trachea during changes in intrathoracic pressure during

breathing. In tracheomalacia, the cartilage rings do not extend nearly so far around the
circumference, and thus a larger portion of tracheal wall is membranous. Therefore, the
lumen of the intrathoracic portion of trachea tends to collapse during exspiration and produce
exspiratory stridor. Diagnosis: bronchoscopy to different from extrinsic compressing lesion -
f.e. vascular ring.

Tracheo-esophageal fistulas
relatively common 1:4000 live birth. Recurrent aspiration, dyspnea during feeding.
Diagnosis: bronchoscopy, esophagoscopy, bronchography, esophageal manometry
Treatment: surgery.

Bronchial anomalies
Stenosis, mucosal web, anomaly of branching, bronchiectasia

Lung sequestration
A significant part of lung is sequesterd fromperfusion of a.pulmonalis. Bronchiectasias,
repeated pneumonias. Perfusion scintigraphy.

Congenital lobar emphysema
A significant part of lung (lobe-whole lung) has higher compliance – volume expansion,
compression of healthy part of lung, respiratory insufficiency of different degree.

Lung hypoplasia
Different degree from insignificant up to lethal. Could be a part of a syndrome – f.e. Potter

Diaphragmatic hernias
Dyspnoe and empty abdomen in a newborn, live threatening condition – intubation and
surgery. Not use a face mask for vantilation. F.e. Bochdalek type.

Congenital heart defects
1.Fetal circulation
Placenta serves as the exchange organ for respiration and metabolismus. Oxygenated blood
(80%) passes from the placenta through the umbilical vein to the heart. As the flows toward
the heart, it mixes with blood from the inferior vena cava and from hepatic veins, so that
blood entering the right atrium is approximately 65% saturated. A considerable amount of
this blood is shunted across the foramen ovale into the left atrium. The venous blood from
upper part is much less saturated - 30% and most of it enters the right ventricule through
tricuspid valve. Thus, the blood in right ventricle is a mixture of both relatively highly
saturated blood from the umbilical vein and desaturated blood from venae cavae. This
mixture result in saturation 50% in the right ventricle.
   The blood in the left atrium is derived from the blood shunting across the foramen ovale
and the blood returning from pulmonary veins. Saturation is 60%. A great deal of the left
ventricular output goes to the head, the lower portion of the body is supplied by blood both
from right ventricle, through the patent ductus arterious, and from the left ventricle.

2.Adaptation of circulation to extrauterine enviroment
Transient neonatal circulation – closing of ductus arteriosus and forman ovale.

 3. Basic Principles
Cardiovascular diseases are a significant cause of death and chronic illness in pediatric
population. Frequency, incidency: about 1% of newborns have congenital heart disease.
Note ! The most important clues to the presence of heart disease requiring prompt attention,
diagnosis and managment is congestive heart failure and/or cyanosis.
(Cyanosis = more than 5g of reduced Hb/l)
The cardiologist and neotologist use for this situation a therm: „critical heart disease“.
The presence of a heart murmur is not a significant sign of heart disease. The murmur may
suggest the possibility of heart disease in an infant, or the murmur can be a functional or
innocent murmur. On ohter hand not all serious heart diseases are accompanied by a

4. Etiology of Congenital Heart Defects
With incidence about 1% the CHD are the most common structural malformation.
- cca 8% of all CHD are know to be associated with gene or chromosome abnormalities - M.Down,
Turner syndrome, DiGeorge syndrome.
- multiple enviromental factors: diabetes mellitus, alcohol consumption, progesteron use, and
other teratogenes.
- congenital - embryo and fetus infections, certain viruses. Typicall example is rubella syndrom
(Gregg syndrom - CHD, pulmonary stenosis + cataracta, microphtalmia + deafness, hearing loss)
In dealing with families of children with CHD the physician must often answer the question of
risk for feature pregnancies. For siblings is cca 2-5%. More higher is the risk for child of
affected mother - 10-15%.

(A) Critical heart defects
Immediately afte birth life threatening congenital heart defects – hypoxia, cyanosis, heart
failure. Basic diagnostic approach.

(B) Defects with left-right shunt
- ventricular septal defect 20%
- patent ductus arteriosus 12%
- atrial septal defect 10%
- atrioventricular septal defect - AV canal 4%

(C)Defects with right-left shunt
- tetralogy of Fallot 10-15%
- complete transposition of the great arteries 10-12%
- pulmonary atresia 3 %
- tricuspid atresia 1%
- hypoplastic left heart 1%
- persistent truncus arteriosus 1%
- origin of both great vessels from the right ventricule
- total anomalous pulmonary venous return rare

(D)Defects without shunt
1) Malformations associated with obstruction to blood flow on the right side of the heart:
- valvular pulmonary stenosis with intact vent. septum 10%
- infundibular pulmonary stenosis with intact v.s.
- distal pulmonary stenosis
2) Malformations associated with obstruction to blood flow on the left side of the heart:
- coarctation of the aorta 6%
- aortic stenosis 0.2%
- mitral valve prolaps rare
- congenital mitral stenosis rare

- cor triatriatum extrem rare
- Ebstein´s malformation of the tricuspid valve rare
- congenital mitral regurgitation rare
- congenital aortic regurgitation - bicuspidal aortic valve
3) Congenital myocardial diseases: all are very rare
- glycogen storage disease - glykogenosis type II. Pompe
- anomalous origin of the left coronary artery
- endocardial fibroelastosis

Congenital defects of kidney and urogenital tract
High frequency of congenital anomalies of the urogenital tract in childhood
Antenatal diagnosis using ultrasound investigation
Intrauterine intervention

Incidence of urinary tract anomalies; pathophysiology of development of the kidney and the
excretory system … diferentiation from the ureteral bud and metanephritic blastema (fetal
period of development)
Nephrogenesis is finished in the 36.week

Dysgenesis of the kidney
Unilateral or bilateral developmental defects, morphology-based definition,
Functional consequences, clinical symptoms, diagnostic and therapeutic options
    Aplasia, dysplasia, hypoplasia, cystic disease

Antenatal diagnosis – ultrasound investigation
The aim: prevention of renal and pulmonary dysplasia, preservation of the renal function
Methods:fetal intervention – decompression, permanent shunt …)
         risks: bleeding, infection, perforation of the gut, sepsis, abortion

(A) Non-obstructive anomalies
Agenesis of kidney (unilateral, bilateral – sy Potteri, oligohydramnion…)
Dysplasia of kidney (focal, segmental, diffuse, altered structural organization and abnormal
  nephronic and ductal differentiation (conglomerates of disorganized tubules, glomeruli,
  and ducts that have a primitive or fetal appereance)
     Patogenesis: (1) the theory of pathological development of the ureteral pad
                        (2) the consequnec of early obstructive uropathy
Multicystic dysplasia focal, difuse or segmental abnormal structures- mainly primitive ducts
  or non-renal structures
     Pathogenesis: multifactorial
Hypoplasia (hypofunctional kidney, low number of the nephrons, risks of early development
  of renal insufficience and hypertension

Anomalies of shape, number and localization: the result of disturbance of renal
   embryogenesis anatomy, pathogenesis, diagnostics, clinical symptoms
Horseshoe kidney, sigmoid kidney, polar fusion, crossed ectopia, ren migrans, ren
duplex, ureter fissus, ureter duplex (Results from disturbances in renal embryogenesis)
Bladder diverticulum: herniation of a pocket of mucosa via a defective muscular layer of
   the bladder wall (acquired -secondary to infravesical or bladder neck obstruction..urethral

   valves…., congenital -a solitary lesion)
Bladder extrophy:-results from a failure of abdominal wall closure with eversion and
   protuberance of the posterior bladder wall and wide separation of the symphysis pubis
Hypospadia: dislocation of the external urethral meatus (is located proximaly to its normal
   position (ventral position at any point along the course of the anterior urethra from the
   perineum to the tip of the glans).
   Multifactorial, polygenic mode of inheritance
Undescended testes: disturbance of intrauterine descent of testes into the scrotum.
    between 7th and 9th month of intrauterine life..the testis passes from the intra-abdominal
    position to the scrotum. Further testicular descent during the 1st year of life
Polycystic kidney disease (infantile-autosomal recesive, adult type – autosomal dominant

(B) Obstructive uropathies: basic types, description, pathogenesis, consequence and
   symptomatic treatment options
      congenital versus acquired
      consequences of the obstruction: impaired kidney growth and function
Posterior urethral valve in boys: serious obstructive uropathy - 30% CHRF, kidney
   dysplasia, hydronephrosis, VUR, incontinence

Obstruction of pelvi- or vesico-ureteral junction
Hydronephrosis: causes, pathophysiology, principal clinical symptoms, diagnostics and
  treatment - ureteropelvic junction stenosis or ureterovesical junction stenosis
Vesicoureteral reflux (VUR): retrograde urine flow from the urinary bladder to the ureter
  and/or to the renal pelvis. VUR is free flow of infected urine into the upper tract is a
  potential risk of renal parenchymal scarring and atrophy.
  Scarring (the most common in the polar region sof the kidney) is related to intrarenal
  reflux (the extension of reflux into the collecting tubules and nephrons).
Phimosis: is the inability to retract the prepuce and expose the glans penis, risk- obstruction
  and VUR, hydronephrosis, UTI

Developmental problems of GIT I
This seminars provides to the students information about general patophysiology of the
development of the gut and presents the most frequent clinical conditions including stenosis
and atresia of the gut.

Development of the gut, risk points for developmental anomalies
Stenosis and atresia of the gut

1) Oesophageal atresia
       classification, epidemiology
       pathophysiology of complications
       general approach to treatment
2) Pyloric stenosis
       etiology, epidemiology
       pathophysiology of clinical symptoms
       general approach to treatment

3) Meckel´s diverticulum
       pathophysiology of clinical symptoms
       general approach to treatment
4) Hirschprung disease
       etiology, epidemiology
       pathophysiology of clinical symptoms
       general approach to treatment

Feeding in childhood
Seminar presents principles of breast and formula feeding in childhood, specifies
macronutrients and micronutrients and describes the most frequent complications.

Parenteral nutrition in children
Seminar presents basic principles of total parenteral nutrition (TPN) in children, specifies the
requirementsof macronutrients and micronutrioents a describes the most frequent

Indications of TPN in children
Basic requirements of macronutrients and micronutrients

Principles of active and passive immunisation
- Explain term „specific immunity“

 passive immunity attained naturally
 passive immunity attained artificially
 active immunity attained naturally
 active immunity attained artificially

Importance of active immunisation of children as a most effective procedure in
prevention of infectious diseases
 most important types of vaccines (live attenuated, killed, toxoids, polysaccharide, split,
 types of vaccinations (routine, special, emergency, in injuries, before travelling, according
 adverse reaction and contraindication
 vaccination schedule -intervals between several types of vaccination

- Prophylactic and therapeutic effect of passive immunisation
 Heterologous immunoglobulins
 Homologous immunoglobulins

containes antigen of one or more microorganisms and after aplication to human or animal
body causes antibody response – active immunization

Types of vaccines
according method of preparation
   live attenuated
   inactivated (killed)
   anatoxin (toxoid)

Causes of low immunity response
immunodeficiency inborn or acquired
insufficiency of intervals between vaccination
bad nutritional status

Organization of vaccination in Czech republic
vaccination is important among children, they are in childhood mostly exposed to infectious
strategy of vaccination is regulated by statement of Ministry of Health
vaccination in CR against infectious diseases is divided to several groups

Types of vaccination in Czech republic
routine vaccination
special vaccination
emeregency vaccination
vaccination in injueries
vaccination of people going abroad
vaccination on request

Routine vaccination
diphtheria, tetanus, pertussis and diseases caused by Hemophilus influenzae b
measles, mumps, rubella
viral hepatitis B

Handicapped child
General definition of a handicapped child

Classification of handicapped children according to the type of affliction:

1. Somatic and locomotor defects (congenital anomalies of organs or systems; acquired
   defects; abnormalities of somatic growth and of onset of puberty
2. Sensory defects – hearing, vision, speech (congenital; acquired)

3. Mental defects as a consequence of congenital anomaly (chromosome aberrations,
   congenital hypothyreosis, phenylketonuria, defects of CNS development)
   pathological course of pregnancy or delivery (fetal infections, CNS hypoxemia,
     hyperbilirubinemia, hypoglycemia, prematurity, teratogens, toxins, intrauterine
     malnutrition, intracranial haemorrhage, etc.)
   postnatal disease (hyperpyrexia, epilepsy, asphyxia, meningitis, degenerative CNS
     disease, CNS trauma, metabolic disorders, poisoning
   psychogenic deprivation
   chronic disease (renal insufficiency, cystic fibrosis, diabetes mellitus, epilepsy, etc.)
   social problems (family rejects the affected child, family unable to care for the child)

Cathegory of handicap: gr. I.-V.

Handicap from the point of the child
       limitation of sensory perception, movement, diet
       repeated hospitalisations, painful diagnostic and therapeutic procedures, side effects
        of medications
       limited access to education
       subjective complaints

Handicap from the point of the family
       alteration of family dynamics
       economic problems
       social isolation of the child and family

Team care for the handicapped child
       family
       pediatrician
       medical specialist
       physiotherapist
       teacher
       psychologist
       social worker

Additional care options for the handicapped child and the family
Specialized institutions, professional centres, boarding schools (integrated versus special
education). Civil associations, centres for dissemination of information and sharing
experience, self-help, development of psychological support among families with a specific
type of a child’s handicap

    individual approach to education depending on the type and severity of the handicap
    educable child: in special schools or integrated in regular schools

Prevention of handicap
   primary
   secondary
   tertiary
   preconception (aimed at the mother and her health)
   during gestation (genetic counseling, aminocentesis, chorion villi biopsy, optimal
    pregnancy conditions, careful guidance of the delivery process)

   postnatal (monitoring, medical examinations and treatment, breast-feeding suppoort,
    systematic conventional screening methods)
   regular follow-ups of each individual from birth to 18 years of age through systematic
    medical examinations

Child Abuse and Neglect
This seminar provides to the students basic information on the the issues concerning Child
Abuse and Neglect – epidemiology, classification, diagnostic tools and overview of therapy.
Short case studies are included in the seminar.

Plan of the seminar:
Child Abuse and Neglect (CAN) – definition
        present situation in the Czech Republic and worldwide
        physical abuse
        emotional abuse
        sexual abuse (contact vs non contact)
        Munchausen syndrome by proxy
CAN – diagnosis
CAN – treatment
CAN – prevention
Legas aspects – reporting, contacts with Police and social services


Shared By: