Term
| when would you give IV glucose to a new born? |
|
Definition
if an asymptomatic infant has 2 low glucose values despite being fed (<2.6mmol/L)
OR 1 very low value <1.6
OR becomes symptomatic; jittery irritability, apnoea
the aim is to keep glucose above 2.6mmol/L |
|
|
Term
if baby fails to respond adequately to glucose IV what else could be given?
how should you give glucose i.e though central or peripheral line? explain why |
|
Definition
you can give glucagon or hydrocortisone instead.
give glucose via central line as extravasation can occur causing tissue necrosis. |
|
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Term
| how is a cleft lips formed? |
|
Definition
| failure of fusion of the frontonasal and maxillary processes. |
|
|
Term
| what is the pierre Robin sequence? |
|
Definition
combination of
micrognathia,
posterior displacement of tongue and
midline cleft of soft palate
problems: difficuty feeding, airway obstruction, FTT in 1st few months
mx: if airways obstruction then lie infant prone as this allows tongue and jaw to fall forward.
eventually mandible grows and problems resolve. then the cleft palate can be repaired. |
|
|
Term
| what condition during pregnancy is oesophageal atresia associated with? |
|
Definition
polyhydramnios.
usually there is a tracheo-oesophageal fistula.
almost half of babies will have other malformations as this is associated with VACTERL anomalies.
vertebral
anorectal
cardiac anomalie
trecheo esophageal
renal
radial Limb |
|
|
Term
| characteristic signs of duodenal atresia on xr? |
|
Definition
double bubble sign
absence of air distally |
|
|
Term
| how many a meconium illeus be dislodged? |
|
Definition
using a gastrograffin contrast medium.
meconium ileus is due to the putty like consistency of the meconium. |
|
|
Term
| define exomphalos/omphalocele |
|
Definition
abdominal contents protrude through umbilical ring they are covered with a transparent sac formed by amniotic membrane and the peritoneum. often associated with other congenital abnormalities.
this is different to gastroschisis where
bowel protrude through defect in anterior abdominal wall, adjacent to umbilicus . there is no covering sac and it is not associated with other congenital abnormalities. |
|
|
Term
out of exomphalos and gastrochisis which one is associated with other congenital abnormalities?
which one is covered with a peritoneal sac and amniotic membrane?
which carries a much greater risk of dehydration and protein loss? |
|
Definition
exomphalos/omphalocele associated with other congenital abnormalities. it is also covered with a layer of amniotic membrane and peritoneum.
gastroschisis carries higher risk of dehydration and protein loss. |
|
|
Term
|
Definition
wrap abdomen of infant in several layers of cling film to minimise fluid and heat loss
nG tube used to aspirate frequently
IV dextrose infusion
colloid support for protein loss
many lesions repaired by primary closure of abdomen
in large lesions, intestine enclosed in a silastic sac sutured to the edges of the abdominal wall and contents gradually returned into the peritoneal cavity. |
|
|
Term
| what are the 4 phases of human growth? |
|
Definition
fetal- fastest period of growth
infantile
child hood
pubertal |
|
|
Term
| feral growth accounts for 30% of eventual height. list factors that affect this |
|
Definition
| size of mother and placental nutrient supply. |
|
|
Term
infantile phase accounts for 15% of eventual height. name important factors here
period spans from infancy to 18months. |
|
Definition
adequate nutrition
good thyroid function
FTT is when there is inadequate weight gain during this period. |
|
|
Term
childhood phase of growth contributes to 40% of eventual height.
what factors are important here? |
|
Definition
GH is important
adequate nutrition
good health
profound unhappiness can decrease GH and cause psychological short stature |
|
|
Term
| pubertal growth adds 15% to final height. what factors are important here? |
|
Definition
testpsterone and estradiol
these cause the back to lengthen and boost GH secretion.
same sex steroids cause fusion of the epiphyseal plates. |
|
|
Term
| what are the features of puberty in females? |
|
Definition
breast development is the 1st sign
pubic hair growth and rapid height spurt follow almost immediately
periods start on average 2.5 years after the start of puberty.
start of periods signal that growth is coming to an end with only ~5cm of growth left. |
|
|
Term
| what are the 1st signs of puberty in males? |
|
Definition
testicular enlargement then
pubic hair growth
then ~18m later a height spurt. in contrast in females, growth spurt occurs shortly after breast development. |
|
|
Term
|
Definition
height below 2nd centime or
0.4th centile |
|
|
Term
what s height velocity?
how can it be calculated? |
|
Definition
height velocity is a sensitive indicator of growth failure
you need 2 accurate measurements at least 6months (preferably 1 year) apart. this is plotted on a chart and gives a height velocity in cm/year.
height velocities persistently below 25th centime is abnormal and that child will eventually become short. |
|
|
Term
| how to measure potential genetic height from parents? |
|
Definition
mean height of mum and dad THEN
add 7cm if its a boy
OR
subtract 7cm if its a girl
to get 9th-91st centimes it is +/-10cm for boy
+/- 8.5cm for a girl |
|
|
Term
|
Definition
defective GH receptiors.
there are high levels of GH but low levels of the further active upstream product which is IGF 1
does not respond to GH rx but does to IGF 1 therapy |
|
|
Term
| in GH deficiency what would the bone age show? |
|
Definition
it would be markedly delayed.
also delayed in hypothyroidism |
|
|
Term
| in disproportionate heights there are 2 classes of disorders to consider. these are skeletal dysplasias and storage disorders. how can they be differentiated using measurements? |
|
Definition
skeletal dysplasias: Leg> back
storage disorders: back> legs |
|
|
Term
| when does the posterior and anterior fontanelles close? |
|
Definition
posterior by 8 weeks
anterior 12-18months |
|
|
Term
| a baby is found to have markedly raised plasma levels of 17-alpha-hydroxyprogesterone in the blood. which condition are you thinking baby has? |
|
Definition
congenital adrenal hyperplasia.
there is a deficiency in 21-hydroxylase. |
|
|
Term
| features of congenital adrenal hyperplasia |
|
Definition
AR disorder of adrenal steroid biosynthesis
females present with virilisation of external genitalia
males present with salt loss or tall stature and precocious puberty
long term mx: lifelong glucocorticoids, mineralocorticoids/sodium chloride if salt loss
addiitonal steroids to cover illness
salt losing crisis needs urgent rx with hydrocortisone, saline and IV glucose
monitor growth, skeletal maturity, plasma androgens and 17-alpha-hydroxyprogesterone levels
surgery for females |
|
|
Term
cows milk contains long chain triglycerides where as specialised formula feed contains a mix of medium and long chain triglycerides.
what is so special about medium chain triglycerides? |
|
Definition
| medium chain triglycerides are directly absorbed into small intestine and need neither pancreatic enzymes nor bile salts for the process. |
|
|
Term
| why should a soya formula not be used below 6 months of age? |
|
Definition
| it contains high aluminium content and also phytoestrogens that mimic endogenous effects of oestrogen's. |
|
|
Term
| define mild and severe FTT |
|
Definition
mild : dropped across 2 centile lines
severe: drop across 3 |
|
|
Term
| define broad causes of FTT? |
|
Definition
non organic and organic
inadequate intake
inadequate retention
malabsorption
failure to utilise nutrient
increased requirements |
|
|
Term
| name some non organic causes of FTT |
|
Definition
inadequate availability of food; due to money, malnutrition of mother etc
psychosocial deprivation: poor attachment, maternal depression, low education in mother
Neglect or child abuse |
|
|
Term
| name some conditions where the child may have increased requirements? |
|
Definition
thyrotoxicosis
CF
malignancy
chronic infections (HIV)
chronic heart or renal failure. |
|
|
Term
features of Marasmus
definition: weight for height more than 3 SD below the median. |
|
Definition
protein energy malnutrition
wasted wizened appearance
oedema is NOT present |
|
|
Term
|
Definition
generalised oedema
severe wasting
flaky paint skin rash and hyperkeratosis
angular stomatitis
sparse and depigmented hair
enlarged liver and diarrhoea |
|
|
Term
| pathophysiology of vitamin D deficiency |
|
Definition
vit D deficiency causes low serum calcium
this triggers secretion of PTH which normalises the serum calcium but demineralises the bone.
PTH causes renal losses of phosphate and consequently low serum phosphate levels, further reducing the potential for bone calcification. |
|
|
Term
|
Definition
malabsorbtion
inadequate intake
poor sunlight
anticonvulsants e.g. phenobarbital and phenytoin
hepatic and renal disease can interfere with the conversion or activation of vitamin D |
|
|
Term
| clinical manifestation of rickets |
|
Definition
ping pong ball head (craniotabes)
palpable costochondral junctions (rachitic rosary)
wrists and ankles may be widened
harrison sulcus due to attachment of softened ribs to diaphragm.
bow legs |
|
|
Term
| being deficient in which vitamin increases you susceptibility to measles as well as other infections? |
|
Definition
|
|
Term
|
Definition
weight in kg divided
height in metres squared |
|
|
Term
BMI >91st centile is?
BMI> 98th centile is? |
|
Definition
>91 st is overweight
>98th is obese |
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