Paeds 3 Flashcards
How is nocturnal enuresis defined?
involuntary wetting during sleep, without any inherent suggestion of frequency or pathophysiology.
generally considered to be normal in children younger than 5 years of age
Describe the different types of enuresis
primary without daytime symptoms - the child has never achieved sustained continence at night and does not have daytime symptoms.
primary with daytime symptoms - the child has never achieved sustained continence at night and has daytime symptoms such as urgency, frequency, daytime wetting, abdominal straining, or poor urinary stream, or pain passing urine.
secondary - bedwetting occurs after the child has been dry at night for more than 6 months
What are the causes of primary enuresis without daytime symptoms
Sleep arousal difficulties — inability to wake to noise, the sensation of a full bladder, or bladder contractions.
Polyuria — a larger than normal production of urine at night which is greater than bladder capacity.
Bladder dysfunction — a small bladder capacity or overactive bladder.
What are the causes of primary enuresis with daytime symptoms
An overactive bladder.
Structural abnormalities (for example ectopic ureter).
Neurological disorders (for example neurogenic bladder secondary to spinal dysraphism).
Chronic constipation.
Urinary tract infection.
What are the causes of secondary enuresis
underlying cause:
diabetes,
urinary tract infection,
constipation,
psychological problems (for example behavioural or emotional problems),
family problems (vulnerable child or family)
What are the risk factors for enuresis
FH male delay in development constipation psych or behavioral problems - ADHD, ASD, anxiety, depression, conduct disorder sleep apnoea
What questions are important to ask in a history of enuresis
primary or secondary any day time symptoms medical or physical tigger social, emotional or enviromental problems ?child maltreatment
What would be red flags for child maltreatment in an enuresis history
parents blaming the child - think the child is wetting onpurpose
punishments for bedwetting
bedwetting persists after adequate managment
What investigaitons could be done in enuresis
urinalysis if started recently, daytime symptoms, child seems unwell or history suggesting UTI/diabetes
ask parents to keep diary with fluid intake, toilet use and wetting recorded
What is the management for enuresis
<7y - usually resolves over time. ensure fluid intake not excessive, encourage to empty bladder before bed
> 7y - first line is enuresis alarm
next desmopressin
if daytime symptoms, need referral
if secondary, exclude cause before referral
Describe the enuresis alarm and its usefulness
most effective long term treatment
useful in those >7y
An enuresis alarm has a sensor pad which senses wetness. The sensor is linked to an alarm which wakes the child if it becomes wet. There are two main types of enuresis alarms:
Beside alarms — where a noise box is placed next the child’s bed and a sensor pad is positioned under a draw sheet beneath the child in the bed.
Body-worn alarms — where a tiny sensor is attached to the child’s pants for example between two pairs of tightly fitting underpants and the alarm is worn on the pyjama top.
contunue to use until 14 dry nights in a row
Who might an enuresis alarm not be suitable for?
The child or parents and carers do not want to use one.
The child wets the bed (infrequently) less than once or twice a week.
Parents or carers have emotional difficulty coping with the burden of bedwetting.
Parents or carers express anger, negativity, or blame towards the child.
The child is younger than 7 years of age and is not able to use an alarm.
What are the signs of response to enuresis alarm treatment
Smaller wet patches.
Waking to the alarm.
The alarm going off later and fewer times per night.
Fewer wet nights.
Describe the mechanism of action of desmopressin and its usefulness in managing enuresis
ADH analogue so reduces quantity of urine produced
used in short term management if:
A rapid onset in improvement or a short-term improvement is required (for example for sleepovers or school trips).
The child or parents or carers are unable to use an alarm or do not want to use a alarm as first-line treatment.
The child or parents and carers are currently using an alarm and want to stop.
How should desmopressin be taken?
take at bedtime
sips only from one hour before taking until 8 hours after taking - reduces risk of hyponatraemia and fluid overload
When can desmopressin not be prescribed
heart failure
HTN
taking diuretics
psychogenic polydipsia
What is necrotising enterocolitis
vascular, mucosal, toxic and other insults to the immature gut leads to serious intestinal injury
damage to the mucosa leads to the spread of commensal organisms
What are the features of necrotising enterocolitis
occurs in preterm or very low birth weight neonates in first two weeks of life bilious vomiting bloody mucoid stool feeding difficulties abdominal distension and erythema intestinal loops visible abdominal mass present/ascites reduced bowel sounds bradycardia, lethargy, shock, apnoea, resp distress
What investigations should be done in suspected necrotising enterocolitis
FBC CRP VBG U+E
AXR
What can be seen on AXR in necrotising enterocolitis
wall thickening
gas filled loops of bowel
pneumatosis intestinalis = intramural gas
portal venous gas
What is the management of necrotising enterocolitis
NBM - bowel rest NG tube to decompress bowel with intermittent suction IV fluids IV abx - cefotaxime + metronidazole TPN intubation or ventilation if apnoea surgery if perforation/necrotic bowel
What are the potential complications of necrotising enterocolitis
Perforation. Acquired short bowel syndrome (following surgery). DIC. Sepsis and shock. Intestinal strictures (~30%). Enterocolic fistulae. Abscess formation. Iatrogenic complications - eg, central venous catheter-related thrombotic events and nosocomial infections, metabolic complications secondary to prolonged hyperalimentation (a nutrient mixture given to premature babies before giving milk).
How can necrotising enterocolitis be prevented?
breast milk
small feeds - increase volume slowly
probiotics
What is coeliac disease?
autoimmune condition in which glutens activate an abnormal mucosal response leading to chronic inflammation and damage (mucosal atrophy) to the lining of the small intestine
What are the risk factors for coeliac disease
FH - first degree relatives
genetic - HLA DQ2 and HLA DQ8
other automimmune - T1DM, automimmune thyroid
down’s, turner’s william’s syndromes
What are the symptoms of coeliac disease
failure to thrive prolonged and unexplained diarrhoea, bloating, constipation, indigestion fatigue weight loss mouth ulcers - severe or persistent
What skin condition is associated with coeliac disease
dermatitis herpetiformis
Describe dermatitis herpetiformis
symmetrical
on extensor surfaces, scalp and natal cleft
vesciles or papules on erythematous patch of skin
intensely itchy
How is dermatitis herpetiformis treated
Dapsone PO
How is coeliac disease investigated
serology testing - IgA and tTGA
FBC, B12, ferritin, LFTs, calcium
jejunal biopsy
What is important to ask patients before serology testing for coeliac disease
have they been eating gluten??iac d
need to have been eating gluten at least twice a day for past 6 weeks in order for test to give reliable answer
What does tTGA stand for?
tissue transglutaminase
What is seen on jejunal biopsy in coeliac disease?
subtotal villous atrophy
crypt hyperplasia
increase in intraepithelial lymphocytes
lamina propria infiltration with lymphocytes
What is the management of coeliac disease?
gluten free diet
supplements if needed eg. calcium or vit D
Give some differentials for coeliac disease?
Crohn's disease Ulcerative colitis. Malignancy Infections such as HIV and tuberculosis infection — presenting with possibly mouth ulcers (in HIV infection and AIDS), prolonged fatigue, and unexpected weight loss. Irritable bowel syndrome Cow's milk protein allergy Aphthous ulcer
What are the potential complications of coeliac disease
Anaemia, due to deficiency of iron, folate, or vitamin B12 due to malabsorption
Osteoporosis, due to malabsorption of calcium and/or vitamin D.
Increased risk of fragility fractures.
Chronic pancreatitis with pancreatic insufficiency.
Hepatobiliary abnormalities such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis.
Splenic dysfunction - hyposplenism
Subfertility.
Lactose intolerance.
Faltering growth.
Delayed puberty.
enteropathy-associated T-cell lymphoma of small intestine
What is VUR
vesicoureteric reflux
abnormal backflow of urine from bladder to ureter and kidneyu
primary is due to ureters being displaced, entering bladder at a perpendicular angle and having a shorter intramural course.
VUJ does not close as bladder distends
so urine flows up the ureters
What is the function of the ureteric opening being 2cm in to the bladder
so that as the bladder distends with urine, the entrance to the ureter closes
acts a valve and prevents backflow of urine into the ureters
Describe the stages of VUR
I - Reflux into the ureter only, no dilatation
II - reflux into ureters and renal pelvis on micturition, no dilation
III - mild/moderate dilation of ureter, renal pelvis and calyces
IV - dilation of ureter, renal pelvis adn calyces with moderate ureteral tortuosity
V - gross dilation of ureter, renal pelvis and calyces with ureteral tortuosity
What complications can VUR lead to
recurrent UTI
pyelonephritis
renal scarring
HTN due to renal scarring
What investigations need to be done for VUR
abdo USS
micturating cystourethrogram
DMSA scanning - looks for renal scarring
How can VUR be managed
reassurance - in most cases resolves as the ureter and bladder grow over time and valve function improves
can have surgery to repair VUJ valve
What is Turner syndrome
45X
How common is Turner syndrome
1 in 2000 live births
What are the features of Turner syndrome in a neonate
SGA
lymphoedema in hands and feet
excessive skin at nape of neck
cardiac abnormalities
What cardiac abnormalities are most common in Turner syndrome
bicuspid aortic valve
coarctation of aorta
also
pulmonary stenosis
aortic stenosis
What are the features of Turner syndrome in an infant
feeding difficultues
poor weight gain
length close to and parallel to the 3rd centile
poor sleep
What are the features of Turner syndrome in a preschool age child
short statre high activity behavioural problems recurrent middle ear infections sensorineural hearing loss
What are the features of Turner syndrome in a school age child
gonadal dysgenesis obesity learnign difficulties social vulnerability renal anomolies foot problems (eg, toenail involution, cellulitis),
What renal anomolies are common in Turner syndrome
double collecting system absent kidney malrotation of kidney horseshoe kidney hydronephrosis
What are the features of Turner syndrome in an adolescent
impaired prepubertal growth spurt obesity ovarian failure HTN autoimmune disease - autoimmune thyroiditis, coeliac disease, inflammatory bowel disease specific learning difficulties
What are the features of Turner syndrome in an adult
infertility obesity HTN aortic dilatation or dissection autoimmune thyroid disease osteoporosis
What are the dysmorphic features in Turner syndrome.
Eyesm ears, mouth, neck, chest, joints, hands, skin
Eyes - epicanthic folds, oblique palpebral fissures, ptosis, squint, nystagmus, cataracts, amblyopia, and hypermetropia.
Ears - low-set, posteriorly rotated ears, prominent upturned lobules.
Mouth - micrognathia, high palate and mandible abnormalities can result in crowding of teeth and malocclusion.
Neck - short, webbed; low hairline.
Chest - broad chest, pectus excavatum, inverted, hypoplastic, widely-spaced nipples.
Joints - cubitus valgus, congenital hip dislocation.
Hands - short 4th/5th metacarpals, short fingers, forearm and carpal developmental abnormalities; nail hypoplasia, hyperconvex nails, nail-fold oedema.
Skin lymphoedema - hands, feet, neck (pterygium coli), pigmented naevi, telangiectasias, vitiligo, keloid, seborrhoeic dermatitis; increased body hair with alopecia. There is a risk of keloid formation with surgery.
What investigations can be done in Turner’s
ECG, hearing tests
U+E, TFT, HbA1c, FSH, LH, bone profile
chromosome analysis
renal USS, heart echo
How can the growth failure in Turner’s be managed
GH and oestrogen
How is maintenance fluids for children calculated?
100ml for 10kg
50ml for next 10kg
20ml for any more kg
given over 24 hours
How are fludi replacement amount calculated in children?
according to % fluid deficit
weight (kg) x % replacement x 10 given over 48 hours
Who is screened for coeliac disease
T1DM,
autoimmune thyroid disease,
Trisomy 21,
1st degree relatives with coeliac
What is the difference between a simple and complex febrile convulsion
Simple: lasts <15mins, not recurring in same illness,
generalised
Complex: >15mins, recur in same illness, focal
