Paeds New from PassMed Flashcards
what is the triad in shaking baby syndrome
Retinal haemorrhages, subdural haematoma and encephalopathy
what is another word for exomphalos
omphalocele
what occurs in omphalocele
Abdo contents protrude THROUGH the umbilical ring
they are covered by sac (amniotic membrane + peritoneum)
what are RF for omphalocele
other syndromes (Down’s, Beckwith Wiederman)
low socioecon status
young maternal age
tobacco, alcohol
what occurs in gastroschisis
bowel protrudes through defect in anterior abdo wall ADJACENT to umbilicus
no covering
how do omphalocele and gastroschisis differ in terms of location
omphalocele is THROUGH umbilical ring
gastroschisis is ADJACENT to umbilicus
what metabolic abnormality is pyloric stenosis associated with and why=
HYPOCHLORAEMIC HYPOKALAEMIC ACIDOSIS
HYPOCHLORAEMIC: vomiting up stomach contents which is acidic (hydrogen chloride - HCl),
HYPOKALAEMIC: Potassium is also lost in the vomitus.
ACIDOSIS: loss of hydrogen ions due to the vomiting up of stomach acid
what is the appearance of the abdomen of a child with a diaphragmatic hernia
CONCAVE chest
as all the contents are displaced superiorly
what kind of delivery do you do for omphalocele
ECS to prevent sac rupture
What kind of delivery do you do for gastroschisis
Attempt vaginal
Which one needs an urgent repair, omphalocele or gastroschisis
Gastroschisis
describe the classical presentation of meckel’s diverticulum
PAINLESS rectal bleeeding (1-2yo)
LOTS of dark red blood
what followup scan can you do if suspecting perthes and hip X ray is negative
MRI
what kind of worms commonly cause anal itching in children
threadworms
How do you manage faecal impaction in first line and second line
1 polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) using an escalating dose regimen as the first-line treatment
2. add a stimulant laxative e.g. SENNA if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks
What are fts of cows milk protein allergy
regurgitation and vomiting diarrhoea urticaria, atopic eczema 'colic' symptoms: irritability, crying wheeze, chronic cough rarely angioedema and anaphylaxis may occur
what are investigations you can get for cows milk prot allergy
Dx is clinical (e.g. improvement with cow’s milk protein elimination). Investigations include:
skin prick/patch testing
total IgE and specific IgE (RAST) for cow’s milk protein
Describve roseola infantum esp spread of rash
high fever > maculopapular rash
- rash starts on chest
- spreads to limbs
Nagayama spots: papular spots on the uvula and soft palate
febrile convulsions
diarrhoea and cough
What virus causes roseola infantum
HSV6
What is a rare complication of roseola infantum
encephalitis and febrile fits (after cessation of the fever)
what are the criteria for admission with bronchioloitis
- Apnoea
- Oxygen sat <90 in room air
- Insuff fluid intake <50% normal
- severe resp distress (grunting, chest recession, resp rate >70)
What age is a child with bronchiolitis
<1
what is the condition called where bronchiolitis causes permanent airway damage
and what virus causes it
bronchiolitis obliterans
adenovirus
What gender is Hirscprungs more common in and by how much
MALES 80%
females 20%
What percetage of Hirschprung is assoc with Downs
2%
Explain sx of measles
describe spread of rash
Prodrome: irritable, conjunctivitis, fever
Koplic spots (white spots on buccal mucosa)
Rash: starts behind ears, then to whole body. Maculopapular
Explain sx of mumps
fever, malaise, muscular pain
Parotitis (earache, pain on eating) - unilateral then bilateral
Explain sx of rubella, especially spread of rash\
Pink maculopapular rash - initially on face then to whole body
fades in 3-5days
Lymphadenopathy
Explain rash in Erythema infectiosum
Red rash SLAPPED CHEEK (red on both cheeks, white pallor in mouth), spreads to arms and extensor surface
Explain rash and other symptoms in scarlet fever
Fever, malaise, tonsillitis
Strawberry tongue
Rash - fine punctuate erythema around the mouth
Hand, mouth and foot disease sx
mild systemic upset
sore throat
fever
vescicles in mouth and soles of feet
Explain features of Fragile X
FMR1 gene Repeat CGG Autism Giant brain, testes (macrocephaly, macroorchidism) Intellectual disability Long face, large jaw Eveerted yes X linked
Explaain features of Noonan syndrome
PEWS
Pulmonary stenosis
Pectus excavatum
Webbed neck
Short stature
Pierre robin sequence
Microagnathia
Glossoptisis
Cleft palate
Prader WIlli
Hypotonia
Hyopogonadism
Obesity
What are features of WIlliams syndrome
Weight low at birth, slow to gain Iris (stellate) Large philtrum Large mouth Increased calcium Aortic stenosis SUPRAVALVULAR Mental retardation (learning difficulties, friendly, extrovert) Swelling around eyes
Cri du chat syndrome
characteristic cry (due to larynx and neuro problems) feeding difficulties, poor weight gain learning difficulties microcephaly, microagnathism hypertelorism (far set eyes)
what is cri du chat due to
5p deletion
what are features that occur with de George
CATCH 22
Cardiac anomalies (interrupted aortic arch, TOF) Abnormal facies Thymic hypoplasia Cleft palate Hypocalcaemia 22q11
what are abnormal facies with diGeorge
smooth philtrum
small chin
Explain Turners syndrome (genotype and phenotype)
45X
Phenotypically female, with CLOWNS
Cardiac abnormalities Low set ears Ovaries underdeveloped (streak gonads) Webbed neck Nipples far apart Short stature
How does Downs syndrome occur
Trisomy 21, due to:
- non-dysjunction
- translocation
- mosaic
What is the biggest RF for Downs
high maternal age
What are the most important complications assoc with downs
AAAA
duodenal Atrasia, Hirschoprung
Atrioventricular septal defect
AML/ALL
Alzheimers early onset
What are the facial features of Downs
Upward slanting palpebral fissures, epicantheal folds
Eyes: strabismus, cataracts, hypertelorism, brushfiends spots
Broad, flat nasal bridghe
high arched palate
What are other physical features of Downs
Transverse palmar creasee
Sandal gap
Short stature
What are other non-physical fts of downs
Motor and intellectual disability
Milestones reached at double the age of healthy children
Intellectual - also have autism, ADHD, conduct disorder
What investigations do you do for Downs
screening: combined test / quadruple test
(low hCG, low PAPP-A, high inhibin A. Low oestriol, low AFP)
Confirm with amniocentesis, CVS, umbilical cord sampling > karyotyping
what is a reflex anoxic seizure
fit resulting from a brief stoppage of the heart through excessive activity of the vagus nerve
syncopal episode (or presyncope) that occurs in response to pain or emotional stimuli.
caused by neurally-mediated transient asystole in children with very sensitive vagal cardiac reflexes.
Who do reflex anoxic seizures occur in
young children - 6 months to 3 years
what are typical features of reflex anoxic seizures
very pale
falls to floor
secondary anoxic seizures are common
rapid recovery
What is the typical preesentation of pityriasis versicolor
lots of light brown macules and confluent patches on back and chest.
more noticeable after spending time in the sun -as healthy skin becomes darker, the white/light brown patches become more prominent.
How do you manage pityriasis versicolor
ketoconazole shampoo / topical antifungal
what are features of a simple febrile seizure
< 15 minutes
generalised
no recurrence within 24 hours
complete recovery within an hour
what are RF for neonatal hypo
preterm birth (< 37 weeks) maternal diabetes mellitus IUGR hypothermia neonatal sepsis inborn errors of metabolism nesidioblastosis Beckwith-Wiedemann syndrome
what heart complication is fragile X associated with
mitral valve prolapse
what other condition has similar presentation to appendicitis but is not malignant
Mesenteric adenitis - inflamed mesenteric lymph nodes
commonly post-viral
needs no mx
When do you give VZIG vs acyclovir for chickenpox
give VZIG if patient has had exposure but no symptoms yet!! e.g. pregnant/immunosuppr/neonate
give acyclovir if symptomatic
what is the method of inheritance of prader willi? explain
IMPRINTING
deletion of gene from paternal chromosome
what would occur similar to prader willi if the imprinting i.e. gene removal occurred from the mother
angleman syndrome
what must you do if neonate has glucose <1
IMMEDIATE transfer to ICU and 10% dextrose IV
How do you manage threadworms
oral mebendazole
what is the first sign of puberty in boys
testicular growth
at what age does puberty in boys usually start
12
what testicular volume indicates onset of pubery
> 4ml
what are findings for DDH on inspection + gait assessment
Inspection: asymmetry of skinfolds, shortening of one leg
Gait: positive trendelenberg (sways towards healthy side), waddle
when should you treat DDH
ALWAYS - aim to treat EARLY to maximise prognosis
the earlier the child is treated, the better the prognosis
what is delayed puberty in boys
no testicular growth by age 14
when is delayed puberty in girls
no breast development age 13
no menarche age 15
what are RF for DDH that require USS at 6 weeks
Breech >36 weeks regardless of presentation at delivery
Breech delivery <36 weeks
first degree relative with DDH/hip problem
what do you need to make a diagnosis of CF
ALL THREE
- Typical pulmonary and/or gastrointestinal tract manifestations
- A family history
- A positive ‘sweat-test’
OUTLINE the CONSEQUENCES of CF - based on which ORGAN
Intestine:
- meconium ileus (vomiting, abdo distension, failure to pass meconium)
- rectal prolapse
Pancreas: pancreatic insufficiency (ducts bocked by thick secretions)
- DM
- recurrent pancreatitis
- pancreatic enzyme deficiency, malabsorption
LIVER:
- chronic hepatitis
- cirrhosis
NUTRITIONAL:
- failure to thrive
MALE UROGENITAL:
- obstructive azoospermia
what is the average life prognosis for CF
50-55
what is the percentage of caucasians who are CF carriers
1:100
Summarise Tay Sachs disease
Developmenntal regression in late infancy exaggerateed startle responnse to noise visual inattention sociaal unresponsiveness severe hypotonia, enlarged hear CHERRY RED SPOT in macula
what is Tay Sachs MoI and WHAT POPULATION is it more common in
AUTOSOMAL RECESSIVE
ashkenazi jews
summarise gaucher disease presentation
splenomegaly, hepatomegaly, bone marrow suppression, bone involvement
NORMAL IQ
Who is gaucher disease more common in
Ashkenazi jews
what two diseasees have cherry red spot in macula
Tay Sachs
Niemann-Pick
what are the 5 types of Autism Spectrum Disorder
Autism Asperger's Rett Child Disintegrative DIsorder Pervasive Developmental Disorder not Otherwise Specified
Which type of ASD is most common
Pervasive Developmental Disorder not Otherwise Specified
What GI condition has a DOUBLE BUBBLE APPEARANCE
duodenal atresia
What investigations must you get for Hirschprung’s
Full thickness rectal biopsy
Anorectak mamometry / contrast barium study
why is meningococcal septicaemia a contraindication for LP
apnoea risk
deranged clotting
expain most common features of Wilms tumour
abdominal mass (most common presenting feature)
painless haematuria
flank pain
what are medical conditions ASSOCIATED WITH (not caused by) cystic fibrosis
short stature, delayed puberty diabetes mellitus rectal prolapse (due to bulky stools) nasal polyps male infertility, female subfertility
when in meningitis must you never give corticosteroids?
if UNDER 3 MONTHS
what is persistent pulmonary HTN of newborn
high pulmomary vascular resistance
leads to right to left shunting of blood > cyanosis
What are causes of persistent pulmonary HTN of newborn
birth asphyxia
meconium aspiration
septicaemia
RDS
What investigations for persistent pulmonary HTN of newborn
CXR: normal sized heart
Echo: exclude congenital heart defect
How do you manage persistent pulmonary HTN of newborn
- inhaled nitric oxide (vasodilator)
- sildenafil (viagra, also a vasodiltor)
- high freq oscillatory ventilation
- extracorporeal membrane oxygenation (ECMO)
what is an umbilical granuloma
overgrowth of tisue occurring during healing process on belly button
it prevents involutiuon of umbilicus
how do you manage umbilical granuloma
silver nitrate cream
ligature to stump
what sound is indicative of an inhaled foreign body
MONOPHASIC WHEEZE
what is the centor score used for
used to assess likelyhood of bacterial cause of tonsillitis
What are criteria of CENTOR score, and what do you do with the results?
Each point scores 1:
- Tonsillar exhudate
- Tender anterior cervical lymphadenopathy
- Fever >38
- no cough
if score 3/4, there is up to 50% chance that it is due to bacteria > prescribe antibiotics
what are complications of measles
Neuro:
- febrile convulsions
- encephalitis
- subacute sclerosing panencephalitis
Resp:
- pneumonia
- otitis media
Cardiac:
- myocarditis
what is - subacute sclerosing panencephalitis
RARE BUT DEVASTATING
7 years after measles infection
loss of neurological function, dementia, death
what med can you use for measles in immunocompromised pt
ribavarin
What are indications for antibiotics in OM?
1 perforated eardrum
2 years old or less, with OM on both sides
3 months old or less
4 days duration or longer
how do you confirm constitutional growth delay?
hand/wrist X ray to assess bone age
What are indications for commencing intensified phototherapy
- serum bilirubin rising >8.5 micromoles/ L/ hour
- serum bilirubin within 50 micromoles/L below exchange transfusion threshold
- serum bilirubin levels static or rising after 6 hours of commencing phototherapy.
list infective causes of bloody diarrhoea in children
CHESS-Y: Champylobacter, histolytica entamoeba, e.coli, shigella, salmonella, yersinia
