450 SBA's Flashcards
What is the most common congenital heart defect? A. Coarctation of the aorta B. Ventricular septal defect (VSD) C. Atrial septal defect D. Patent ductus arteriosus E. Transposition of the great arteries
VSD
You are doing a baby check on the post-natal ward on a baby who is 23 hours old.
His mother tells you that he is not feeding well. On examination he is unsettled
with a respiratory rate of 76 and a heart rate of 182. You think his hands and feet
look blue and there is a soft systolic murmur heard at the left upper sternal border.
You ask the midwives to check his saturations which are 85 per cent in air and
start some oxygen. You explain to the mother that he needs to be managed on the
neonatal unit. What is the next step in your management?
A. Stop the oxygen as this may drive the closure of the ductus arterioles
B. Give prostaglandin intravenously to open the duct while organizing an
echocardiogram
C. Give antibiotics and prostaglandin intravenously while organizing an
echocardiogram
D. Give indomethacin intravenously to open the duct while organizing an
echocardiogram
E. Give indomethacin
C - The baby could have C
Teenager presents with new murmur, cyanosis, crepitations, and tachypnoea
Eisenmengers
Signs of cardiovascular insufficiency/heart failure
Which of the following is not a feature of cardiac insufficiency?
A. Scattered wheeze on auscultation of the chest
B. Central cyanosis
C. Sacral oedema
D. Tachypnoea with the apex beat palpable in the 7th intercostal space just
lateral to the mid-clavicular line
E. Hepatomegaly
B) Cyanosis could be a sign of resp failure
Scattered wheeze or bibasal creps are a sign of heartfailure
A 5-year-old girl was brought to hospital at midnight by her mother with 5 per
cent partial thickness burns to her chest and abdomen. Her mother states that she
pulled on the kettle at 2 pm and the boiling water scalded her. On examination she
is tachycardic, and drowsy with cool peripheries. Her initial blood tests: sodium
150 mmol/L, potassium 7.8 mmol/L, urea 10.2 mmol/L, creatinine 104 μmol/L,
haemoglobin 14 g/dL. What is the most likely aetiological factor to account for
these results?
A. Post-renal cause of acute kidney injury
B. Poisoning
C. Renal cause of acute kidney injury
D. Dehydration
E. Pre-renal cause of acute kidney injury
C - The electrolytes are so high to point to AKI over simply dehydration
An 11-year-old girl presents to the out of hours GP while on holiday in England
with abdominal pain. She tells you she has polycystic kidney disease which was
diagnosed early in life. She has bilateral palpable kidneys and hepatosplenomegaly,
with visible distended veins on the abdomen and ascites. Abdominal ultrasound
shows liver fibrosis. What is the inheritance of this condition?
A. Autosomal dominant
B. X-linked
C. Sporadic mutation
D. Autosomal recessive
E. Microdeletion
D - Autosomal recessive version presents as young child. AD version presents in older kids and adults
A 4-year-old boy is brought to accident and emergency with a limp for 1 day. He
was unhappy to weight bear on his right leg. He had been with his grandparents
all day and his mother brought him to hospital when she returned from work that
evening. He was afebrile with a heart rate of 110 bpm but had had a cold last week.
Mum reports no history of trauma. What is the most important diagnosis to exclude?
A. Behavioural
B. Acute leukaemia
C. Reactive arthritis
D. Soft tissue injury
E. Septic arthritis
Acute leukaemia (B) can present with a limp due to bone pain secondary
to dissemination of the disease. It is important to do a full blood count
(FBC) and blood film on these patients to look for pancytopenia and the
presence of blast cells. The other diagnoses are also of importance to
investigate for, but may be more or less likely depending on the history.
A 14-year-old girl was diagnosed with idiopathic thrombocytopenic purpura (ITP)
last week after she attended the children’s assessment unit with recurrent epistaxis.
She had a platelet count of 16 × 109/L last week and now re-presents to accident and
emergency with further episodes of epistaxis, haematemesis and petechiae. She had a
heart rate of 110 bpm and her blood pressure is 100/70 mmHg. What is the next best
management step?
A. Give a platelet transfusion and red cell transfusion
B. Arrange an urgent upper gastrointestinal endoscopy
C. Give IV immunoglobulin and steriods
D. Admit and monitor the haemodynamic status and administer a fluid bolus
E. Discharge home with advice to return if the symptoms continue for
more than 24 hours
D - She’s bleeding and haem unstable
Platelets will most likely be destroyed so only used in life threatening bleeding
Bleeding can be explained by ITP so OGD unnecessary and may just cause more harm
A 14-year-old girl went to her GP with a sore throat and cervical lymphadenopathy.
She had a blood test done and you are called later that day with results. Haemoglobin
6.0 g/dL, WCC 230 × 109/L, neutrophils 0.9 × 109/L, platelets 77 × 109/L; blood film
showed blasts and Auer rods. What is the most important management priority for
this child in the first 24 hours from diagnosis?
A. Overwhelming sepsis
B. Febrile neutropenia
C. Heart failure
D. Uncontrollable bleeding
E. Tumour lysis syndrome
E - All the others are possible, but initial treatment could cause tumour lysis which will kill
A 12-year-old girl has been seeing her GP for the last year with heavy periods and had
suffered with bleeding gums when she was younger. She is otherwise well and lives
with her adoptive parents who now have parental responsibility. Her coagulation tests
reveal normal prothrombin time (PT) and activated partial thromboplastin time (APTT),
low factor VIII, low von Willibrand factor (vWF), abnormal platelet aggregation and
increased bleeding time. What is the likely inheritance of her condition?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked
D. Robertsonian translocation
E. Sporadic mutation
A There are three types as follows: • Type 1 – partial deficiency of vWF • Type 2 – defective vWF • Type 3 – complete deficiency of vWF vWD type 1 and 2 are autosomal dominant (A) (type 3 is autosomal recessive) and a family history may be useful in the history.
A 9-month-old boy presented to his GP with lethargy and a prominent forehead. He is pale
on examination and has yellow sclerae. He is the first child of his non-consanguineous
parents. His haemoglobin is 6.5 g/dL, WCC 5.0 × 109/L, platelets 300 × 109/L. His blood film
shows evidence of haemolysis, no spherocytes, no sickle cells and a good reticulocyte
count. Direct antiglobulin test (DAT) is negative. What is the most likely diagnosis?
A. Beta thalassaemia
B. Sickle cell disease
C. ABO incompatibility
D. Hereditary spherocytosis
E. G6PD deficiency
Beta thalassaemia (A) is a haemolytic anaemia of autosomal recessive
inheritance. Thalassaemia presents with anaemia after 6 months of
age, when HbA is starting to be produced. Thalassaemia is present in
Mediterranean and Asian populations and is due to a defect in the
production of haemoglobin beta chains or alpha chains (beta and alpha
thalassaemia, respectively). Patients can be homozygous (disease) or
heterozygous (carriers). They present with anaemia and jaundice and
may show evidence of extramedullary haemopoeisis (frontal bossing and
maxillary overgrowth).
On the day 1 baby check a mother is very concerned about a rash on her baby’s face.
Over the right eye, forehead and temple there is a pink-red, flat area of erythema. He
is opening the eye, and his eye movements seem intact. The child’s observations and
rest of the examination are normal. What should you tell the mother?
A. This is a strawberry naevus and it may get bigger before it goes away
by about 5 years of age
B. This is a port wine stain and the baby needs an MRI scan to check for
intracranial involvement
C. This is a capillary haemangioma (stork mark) and is normal; it will fade
over the first year or so of life
D. This is orbital cellulitis and he needs intravenous antibiotics
E. This is erythema toxicum which is a normal baby rash and will go away
within the first few weeks
This is likely to be a port wine stain or naevus flammeus (B) which
is a capillary vascular malformation in the dermis, present from birth
and will persist for life. When present in the trigeminal distribution, a
small proportion of children will have underlying brain involvement
(Sturge–Weber syndrome) and should have an MRI brain to look for this
as the child will be at risk of seizures and developmental problems. A
strawberry naevus (A) or cavernous haemangioma is not usually present
at birth and typically appears in the first month. Strawberry naevi will
grow larger before shrinking and disappearing, typically before the age
of 5 years. They may compress neighbouring structures as they grow
and sometimes ulcerate with troublesome bleeding. They are not flat. A
capillary haemangioma or ‘stork bite’ (C) is a pink macule found on the
eyelids, central forehead or nape of the neck which is due to distension
of the dermal capillaries; they mostly fade over the first year. Those on
the neck may persist but will be covered by hair. They are not found on
the lateral face; therefore this is not the right answer. The child is well,
with normal observations, making cellulitis (D) very unlikely. In addition
you have been given no information on whether the lesion feels warm, which you would expect in infection. Erythema toxicum (E) is a common
innocent rash of the newborn which has an erythematous base with small
pustules. It comes and goes all over the body for the first few weeks of
life.
A 4-year-old is brought into accident and emergency by very anxious parents.
She has had a bad cough which makes her vomit and a fever for 2 days. She has
now developed a rash on her face which does not pass the ‘glass test’, in that the
spots are still visible when a glass is pressed against the skin. On examination she
is alert and comfortable at rest, with fine petechiae on her cheeks and neck which
are non-blanching. She has red, enlarged tonsils without pus and the chest is clear.
What is the most likely cause of her rash?
A. Meningococcal sepsis
B. Idiopathic thrombocytopenia
C. Henoch–Schönlein purpura
D. Non-accidental injury
E. Capillary rupture secondary to raised pressure in the superior vena cava
distribution
E - Pattern of petichiae follow SVC pattern. Coughing and vomiting can cause ruptures
ITP unlikely WHILE they’re still ill
You are asked to examine a tall 15-year-old boy. His height is above the 98th
centile for his age and he has other concerns about the development of breast
tissue. He was told this was normal as he develops through puberty but his father
states he has no facial or underarm hair. Jake allows a brief examination of his
genitalia and you note he has a small penis and testicular volume. He has no
arachnodactyly or visual problems. What is the most likely diagnosis?
A. Delayed onset of puberty
B. Klinefelter’s syndrome
C. Precocious puberty
D. Marfan’s syndrome
E. Normal variation
Both Klinefelter’s and Marfan’s syndromes (D) can result in tall stature
(though associated features with Marfan’s syndrome include high arched
palate, myopia, lens dislocation, arachnodactly, arm span > height,
hypermobility, aortic arch abnormalities, mitral valve prolapse, chest
wall deformity). Precocious puberty (C) leads to tall stature initially but
ultimate height is often shorter than peers as the growth spurt occurs
earlier. He also has signs of delayed puberty with small testicular volume
(<4 mL) and no adrenarche. Delayed puberty (A) (in boys no pubertal
development by the age of 15 years) alone would usually be associated
with short rather than tall stature. The tall stature, hypogonadism
and delayed puberty with gynaecomastia is suggestive of Klinefelter’s
syndrome (B) and a karyotype should be done, showing 47XXY. This is
not a normal variation (E) as failure to develop signs of puberty by the
age of 15 years in boys requires investigation.
A 9-year-old girl presents to accident and emergency with fever, vomiting and
dysuria. She is wearing a steroid bracelet and has a steroid card stating she is
on daily prednisolone for severe asthma and eczema and is therefore at risk of
adrenal suppression. She is tachycardic at 140 bpm and you are concerned that
her blood pressure is low. Her capillary glucose is 3.0 mmol/L. What is the single
most important investigation?
A. Cortisol
B. Full blood count
C. Renal function tests
D. Urine culture
E. Blood culture
This girl is having an Addisonian crisis. She has been on long-term
corticosteroids and is therefore at high risk of having developed adrenal
suppression. She has subsequently developed a urinary tract infection
and the physiological response would be to increase endogenous steroid
production. However, with her hypothalamic-pituitary-adrenal axis
down-regulated due to her exogenous steroid use, this is not possible.
She therefore is steroid depleted. She has hypotension, hypoglycaemia
and is likely to develop hyperkalaemia and hyponatraemia. Cortisol (A)
is likely to be low, but this will not change your management of her
condition. full blood count (B), urine culture (D) and blood culture (E)
may help to elucidate the cause of the deterioration and treat it, but will
not provide information about the Addisonian crisis. Renal function (C)
is important to identify hyponatraemia and hyperkalaemia which may
require treatment. Hydrocortisone and IV dextrose would be the initial
treatment of choice, with antibiotics.
