Day 7 Flashcards
Chickenpox features (4)
spread via the respiratory route
can be caught from someone with shingles
infectivity = 4 days before rash, until 5 days after the rash first appeared*
incubation period = 10-21 days
A 3-month-old boy is brought to surgery as his mother is concerned he is ‘floppy’.
Examination confirms a greater degree of hypotonia than would be expected at this age.
What could the possible diagnosis be?
Central causes
- Down’s syndrome
- Prader-Willi syndrome
- hypothyroidism
- cerebral palsy (hypotonia may precede the development of spasticity)
Neurological and muscular problems
- spinal muscular atrophy
- spina bifida
- Guillain-Barre syndrome
- myasthenia gravis
- muscular dystrophy
- myotonic dystrophy
A 9-month-old baby is brought to the emergency department by his father.
He has had a barking cough for the past 3 days and his father says he has been feeding poorly for the past 4 days.
On examination the child does not appear agitated and is easily distracted by his toys.
The barking cough is audible at rest and there is mild sternal retraction. Observations are otherwise stable.
What is the diagnosis?
What is the treatment?
Croup
A single dose of oral dexamethasone (0.15 mg/kg) is to be taken immediately regardless of severity
A mother brings her 14-month-old son into surgery.
Since yesterday he seems to be straining whilst passing stools.
She describes him screaming, appearing to be in pain and pulling his knees up towards his chest.
These episodes are now occurring every 15-20 minutes.
This morning she noted a small amount of blood in his nappy. He is taking around 50% of his normal feeds and vomiting ‘green fluid’ every hour.
On examination, he appears irritable and lethargic but is well hydrated and apyrexial.
On examination, his abdomen seems distended but no discrete mass is found.
What is the most likely diagnosis?
(3)
Intususseption
Whilst constipation is common, it would not cause the bilious vomiting as described.
An abdominal ‘sausage-shaped’ mass, whilst classical, is only found in around 25% of cases.
Management of intususseption
(2)
the majority of children can be treated with reduction by air insufflation under radiological control, which is now widely used first-line compared to the traditional barium enema
if this fails, or the child has signs of peritonitis, surgery is performed
Features of intususseption
(5)
paroxysmal abdominal colic pain
during paroxysm the infant will characteristically draw their knees up and turn pale
bilious vomiting
bloodstained stool - ‘red-currant jelly’ - is a late sign
sausage-shaped mass in the right upper quadrant
A 5-year-old boy presents to the emergency department with severe pain in their right hip and a sudden inability to weight-bear. His parents are very concerned about the possibility of an infection. He had a viral upper respiratory tract infection 1 week ago, from which he has recovered. He appears well. He has had no trauma to the hip.
Blood tests revealed normal WCC and ESR. An ultrasound scan of the hip shows a mild effusion in the joint capsule.
What is the most appropriate next step?
What is the most likely diagnosis?
Transient synovitis is self-limiting, requiring only rest and analgesia
An 15-day old baby presents to the emergency department with his mother. His mother states he has not been feeding or drinking well for the last two days. She believes he is not gaining much weight and his stools have been more pale than usual. On examination, you note the baby is visibly jaundiced and has hepatomegaly. Your team conducts a newborn jaundice screen, with one of the differentials being biliary atresia.
What finding would support this diagnosis most?
Conjugated bilirubin is elevated in biliary atresia
Biliary atresia can present with prolonged jaundice
(present > 14 days of age), hepatomegaly, splenomegaly, abnormal growth, cardiac murmurs if associated cardiac abnormalities are present.
Findings of billiary attresia
(4)
Conjugated bilirubin is elevated in biliary atresia and therefore this is the correct answer.
Biliary atresia can present with prolonged jaundice (present > 14 days of age),
hepatomegaly,
splenomegaly,
abnormal growth,
cardiac murmurs if associated cardiac abnormalities are present.
Complications of biliary atresia
(3)
unsuccessful anastomosis formation
progressive liver disease
cirrhosis with eventual hepatocellular carcinoma
Investigations for biliary atresia
(6)
- Serum bilirubin including differentiation into conjugated and total bilirubin: Total bilirubin may be normal, whereas conjugated bilirubin is abnormally high
- Liver function tests (LFTs) including serum bile acids and aminotransferases are usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis
- Serum alpha 1-antitrypsin: Deficiency may be a cause of neonatal cholestasis
- Sweat chloride test: Cystic fibrosis often involves the biliary tract
- Ultrasound of the biliary tree and liver: May show distension and tract abnormalities
- Percutaneous liver biopsy with intraoperative cholangioscopy
A 3-year-old boy presents with 6 days of fever, increasing irritability and a rash. His mother is worried because she has been giving him Calpol and ibuprofen for the last few days but has seen no improvement. On examination his temperature is 38.9°C, respiratory rate is 30 breaths/min, and heart rate is 136 beats/min. On further inspection, the child is noted to have bilateral conjunctivitis with no exudate, cervical lymphadenopathy, erythema of the oral mucosa, and a non-vesicular rash that is spreading from his hands and feet. What immediate treatment should be given?
(3)
High dose aspirin and a single dose of intravenous immunoglobulin
The child presents with at least five of the six diagnostic criteria for Kawasaki disease. Therefore the correct answer is high dose aspirin and a single dose of intravenous immunoglobulin.
Initial aspirin dose of 7.5-12.5 mg/kg 4 times a day for 2 weeks or until afebrile, then 2-5 mg/kg once daily for 6-8 weeks (source: BNF for Children)
Intravenous immunoglobulin 2 g/kg daily for 1 dose, treatment should be given within 10 days of onset of symptoms (source: BNF for Children)
A 5-month-old girl presents to the emergency department with a 24-hour history of cough and wheeze, on a background history of one week of mild fever and coryzal symptoms. She is otherwise well and has no past medical history of note. Respiratory examination identifies generalised wheeze. Observations show:
- Respiratory rate 50/min
- Blood pressure 90/50mmHg
- Temperature 38.1ºC
- Heart rate 122 bpm
- Oxygen saturation 97% on room air
What is the most appropriate management for this infant?
Bronchiolitis does not require antibiotics, children require supportive management only
The correct answer is supportive management only. This child is presenting with cough and wheeze on a background history suggestive of a viral illness; this should raise suspicion of bronchiolitis. Bronchiolitis is a condition characterised by bronchiole inflammation in response to a recent viral illness, most commonly respiratory syncytial virus (RSV). As this patient’s observations show only a mild fever, the most appropriate management is supportive. Alternatively, if her oxygen saturation was persistently below 92% or her feeding was affected, admission would be considered.
A 4-year-old girl is brought to the GP surgery by her father. She has a one-year history of asthma which has previously been controlled with a salbutamol inhaler twice daily and beclometasone 50 micrograms bd.
She has an audible wheeze that has been gradually worsening over the last few weeks and has not responded to additional doses of salbutamol. Her father also reports that she has a night-time cough for the past 4 weeks.
What is the most appropriate next step in management?
Add a trial of a leukotriene receptor antagonist e.g. montelukast
A 25-days-old newborn presents to the emergency department with his parents. He has a distended abdomen and looks lethargic. The parents report that he has been feeding less during the last three days and vomited repeatedly. They describe the vomit as bilious. This morning he produced stools with blood in them but he has no fever. He was born at 35 weeks following premature rupture of membranes. Now he is otherwise healthy.
What is the most likely diagnosis?
Which investigations should be performed?
an x-ray should be performed
the most likely diagnosis is necrotising enterocolitis
What would an x-ray of a child with necrotising enterocolitis show?
(7)
x-rays with necrotising enterocolitis;
- dilated bowel loops (often asymmetrical in distribution)
- bowel wall oedema
- pneumatosis intestinalis (intramural gas)
- portal venous gas
- pneumoperitoneum resulting from perforation
- air both inside and outside of the bowel wall (Rigler sign)
- air outlining the falciform ligament (football sign)
Which main feature is pyloric stenosis characterised by?
What is the test for confirmation of pyloric stenosis?
Projectile vomiting
confirmed with feeding test
Laparoscopy is the investigation of choice for which GI paeds condition
PERFORATION
What is the diagnostic method of choice for the diagnosis of GI perforation?
laparoscopy
What is the diagnostic method of choice for intussusception?
ultrasound
What is the classical presentation of intussusception?
(3)
red-current jelly stool
paroxsysmal abdominal pain
What are the hallmark symptoms of necrotising enterocolitis?
(5)
bloody stools
abdominal distention
abdominal discolouration
perforation
peritonitis
What is the diagnostic method of choice for necrotising enterocolitis?
What might the investigation show?
(7)
dilated bowel loops
bowel wall oedema
portal venous gas
peneumo-peritooneum
inter-mural gas (pneumatosis intestenalis)
Rigler’s sign (air inside + outside the bowl wall)
football sign (air outlining the falciform ligament
Triad of cardiac malformations associated with Turner’s
(3)
coarctation of aorta
aortic root dilation
bicuspid aortic valve
A baby boy born 6 hours ago has an APGAR score of 10. He is not cyanosed, has a pulse of 140, cries on stimulation, his arms and legs resist extension and he has a good cry,
He appears jaundiced. What is the most appropriate action?
Measure and record the serum bilirubin level urgently (within 2 hours) in all babies with suspected or obvious jaundice in the first 24 hours of life since this is likely to be pathological rather than physiological jaundice. NICE CG98
Causes of jaundice in the first 24 hrs
- rhesus haemolytic disease
- ABO haemolytic disease
- hereditary spherocytosis
- glucose-6-phosphodehydrogenase
Physiological causes of jaundice in the first 2-14 days
(3)
more RBCs
more fragile RBCs
less developed liver function
causes of jaundice within the first 24 hours
rheasus haemolytic disease
ABO hemolytic disease
hereditary spherocytosis
glucose-6-phosphodehydrogenase deficiency
decribe the pathophysilogy of Glucose-6-phosphate dehydrogenase deficiency
a defect in an enzyme called glucose-6-phosphate dehydrogenase causes red blood cells to break down prematurely
Describe the pathophysiology of ABO haemolytic disease
(2)
maternal IgG antibodies with specificity for the ABO blood group system pass through the placenta to the fetal circulation
here they can cause hemolysis of fetal red blood cells which can lead to fetal anaemia and HDN.
Hereditary spherocytosis pathophysiology
(4)
most common hereditary haemolytic anaemia in people of northern European descent
autosomal dominant defect of red blood cell cytoskeleton
the normal biconcave disc shape is replaced by a sphere-shaped red blood cell
red blood cell survival reduced as destroyed by the spleen
A 2-day-old neonate born at term presents with tachypnoea, grunting and cyanosis. He is unable to feed. Oxygen saturation is at 70% on room air. On auscultation there is a loud S2 and systolic murmur that is loudest at the left sternal border. A diagnosis of transposition of the great arteries is suspected. He is started on intravenous fluids, antibiotics, and scheduled for surgery.
What is the diagnosis?
Which medications should be given?
should be given prostaglandin E1
In ductal dependent cyanotic heart diseases, prostaglandin E1 (alprostadil) is infused to prevent closure of the patent ductus arteriosus until a surgical correction can be carried out.
This will allow mixing of deoxygenated and oxygenated blood so as to provide adequate systemic circulation. Antibiotics should also be given as prophylaxis for bacterial endocarditis.
Anatomical changes seen in transposition of the great arteries
(2)
the right ventricle outputs to the aorta
the left ventricle outputs to the pulmonary trunk
What is the embryonic cause of TGA?
What increases the risk?
failure of the aorticopulmonary septum to spiral during septation
children of diabetic mothers are at increased risk
Which murmur is heard in patients with TGA?
Where is the murmur heard?
loud S2 and systolic murmur
at left sternal border
Which drug is used to keep at PDA open?
Which drug is used to close a PDA?
Prostaglandin E1
Indomethacin/ibuprofen
Causes of cyanotic congenital heart disease
(3)
tetralogy of Fallot (TOF)
transposition of the great arteries (TGA)
tricuspid atresia
What is a “nitrogen washout test”?
(3)
An infant is given 100 percent oxygen for 10 minutes
an ABG is then taken
if the oxygen is less than 15kPa, a congenital cyanotic heart disease is indicated
5 T’s for cyanotic congenital heart diseases (CHD):
It is useful to remember the Tetralogy of fallot
Transposition of great vessels (TGA)
Tricuspid atresia
Total anomalous pulmonary venous return
Truncus arteriosus
What is measured for an AGPAR score?
What is the highest possible AGPAR score?
- Pulse
- Respiratory effort
- Colour
- Muscle tone
- Reflex irritability
Highest possible score of 10/10
You are working on the neonatal unit and examine a new-born baby. On examination you note a small left sided haematoma over the parietal bone.
It does not extend beyond the margins of the parietal bone and is soft to touch. The baby is otherwise well.
The baby was born by spontaneous vaginal delivery at term. There were no problems prenatally or during labour.
What is the most likely diagnosis?
A cephalhaematoma can often be confused by medical students with a caput succedaneum.
Distinguishing features of a cephalhaematoma are that they usually develop after birth and do not cross the suture lines of the skull as the blood is confined between the skull and periosteum.
How can cephalohematoma be differentiated from caput secundum?
(2)
In cephalohematoma, the swelling develops after birth
it also doesn’t cross the suture lines of the skull as blood is arrested between the skull and periosteum
You are called to theatres for the forceps delivery of a baby of 38 week gestation who developed fetal distress during labour.
The obstetrician hands you the baby for resuscitation. What is the first step?
Dry the baby
A 4-year-old boy is brought to the clinic.
He gives a history of difficult, painful defecation with bright red rectal bleeding.
What is the most likely diagnosis?
Anal fissure
Painful rectal bleeding in this age group is typically due to a fissure. Treatment should include stool softeners and lifestyle advice.
A 2-year-old has a history of rectal bleeding.
The parents notice that post defecation, a cherry red lesion is present at the anal verge.
What is the most likely diagnosis?
Juvenile polyps
These lesions are usually hamartomas and this accounts for the colour of the lesions.
Although the lesions are not themselves malignant they serve as a marker of an underlying polyposis disorder.
What is the sniffing position?
