Paeds Flashcards
What happens if VSD uncorrected?
Eisenmenger’s syndrome - Left to right shunt leads to compensatory pulmonary vascular hypertrophy. This in turn causes pulmonary hypertension and shunt reversal (right to left). Pulmonary hypertension leads to CHF.
Are newborns with VSD cyanotic?
No - often become cyanotic at later age when oxygen demand increases (can be during exercise)
Presumed diagnosis of unwell child with unhealed burn
Toxic shock syndrome
Idiopathic pulmonary haemosiderosis features
First decade of life (mean 4.5) and more females. Alveolar capillary bleeding causes haemosiderin accumulation causing anaemia. May have haemoptisis. Immunosuppressants/steroids may work. May be linked to gluten sensitivity. May resolve sponateously
Conditions to exclude prior to Idiopathic Pulmonary Haemosiderosis diagnosis
Common causes alveolar haemorhage - Goodpastures and SLE
What is Neimann-Pick’s?
Glycogen storage disorder - can have lung manifestations - foamy macrophages on biopsy. Multi-system disease
What is vanishing lung syndrome?
Found in SLE and idiopathic giant bullous emphysema - common in young male smokers. Starts in upper lobes and lungs appear to be disappearing on X-ray. Chronic and progressive.
Intussusception features, investigation and management
Classically presents with slimy or jelly-like (red currant jelly) stools and palpable mass/fullness and abdo pain. Child described as drawing their legs up. Most common in 6-18 months old. Diagnose with ultrasound. Treat with air enema usually - surgery if unsuccessful. Often associated with viral gastroenteritis. Can cause vomiting (bile stained as small bowel).
Hirschsprung’s cause
Congenital distal bowel aganglionosis
Hirschsprung’s symptoms and demographics
Bowel obstruction - vomiting and not passing stools in very young neonates
Hirschsprung’s diagnosis and treatment
Diagnosed via rectal biopsy and treated via surgical removal of the affected part of bowel.
Pyloric stenosis features
Occurs within first 4 weeks of life. Projectile vomiting immediately after feeds. Diagnosed with ultrasound and treated with pyloromyotomy
Congenital Adrenal Hyperplasia
Autosomal recessive mutation in adrenal hormone/steroid enzymes. 95% secondary to 21-hydroxylase deficiency. They can’t produce cortisol so ACTH production goes up - adrenal hyperplasia and overactivity of steroid-producing cells. Produce excess androgens/oestrogens and sometimes mineralocorticoids. Women develop pcos, men have large penis and scortal pigmentation at birth. Can present hyponatraemia and hyperkalaemia. Can cause hypoglycaemia and hypotension too as involved in adrenaline synthesis. Replace hormones.
Usual presentation age for pyloric stenosis
2-4 weeks
Pyloric stenosis features
Projectile, non-bilious vomiting, hypochloraemia, hypokalaemia and metabolic acidosis, more commonly in males.
Tracheo-oesophageal fistula presentation
Often associated with oesophageal stenosis. presents with cough/choke during feeding and lrti’s. Symptoms can be subtle and not present until later in childhood.
Biliary atresia presentation and treatment
Conjugated bilirubinaemia. Surgical treatment with Kasai portoenterostomy
What is a child protection plan?
A plan drawn up by local authority to protect children at risk of significant harm. Aims to ensure safety of child, promote childs health and development and support family in looking after welfare of child
Long term complications of ASD
Right atrial enlargement and pulmonary trunk dilation. Can present fatigue and exertional dyspnoea
Teratogenic effect of lithium during pregnancy.
Ebsteins anomaly - poor right ventricular function. Can lead to wolff-parkinson white
Age at which hand preference developed
12 months
Age at which inability to walk becomes red flag
18 months - potentially cerebral palsy, muscular dystrophy or other developmental issue
Age by which baby should be able to sit unsupported
8 months
Age at which should be able to stand on one leg
4 years
Which defects comprise tetralogy of fallot
- Right ventricular hypertrophy
- VSD
- Right sided outflow tract obstruction (pulmonary valve stenosis)
- Overriding aorta
Most common cause of congenital cyanotic heart disease
Tetralogy of fallot
If cyanosis is present at birth is tetralogy of fallot or transposition of the great vessels likely the causative defect?
Transposition of the great vessels
Clotting factor affected by haemophilia A
Factor 8
Treatment of significant bleed in haemophilia A
Recombinant factor 8
When is desmopressin used in acute bleeds in haemophilia
Mild-moderate - not severe
Clotting factor affected in haemophilia b
Factor 9
Common complications of Down’s
Congenital heart disease (AVSD), anal atresia, duodenal atresia and increased leukemia risk
What is myelomeningocele?
Complex spinal anomaly due to failure of dorsal neural fold fusion. Present with skin defects, lower limb paralysis, sensory loss and bladder and bowel dysfunction. Usually have Chiari ii malformations
Features of Edwards syndrome
Congenital heart disease, central nervous system abnormalities, IUGR, rocker bottom feet, single umbilical artery and facial abnormalities
Hyper IgE features
aka Buckley or Job syndrome
Recurrent respiratory infections, chronic eczematoid eruptions, Cold abscesses and mucocutaneous candidiasis. Strange teeth and coarse facial features
Low CD4 count associations
Advanced HIV, viral infections, pregnancy, malnutrition, TB, drug abuse and severe sepsis
Febrile convulsion red flags for urgent admission
Children <18months Diagnostic uncertainty Convulsion >15mins Focal features Convulsion recurring within same illness or 24 hours Incomplete recovery after 1 hour No focus of infection identified Exam findings suggest serious cause of fever eg pneumonia Child on/or been on antibiotics
Are patients at risk of infective endocarditis after VSD repair?
No
Up to what age is spontaneous VSD closure common?
2 years old
Symptoms that cause suspicion of appendicitis in children
Abdo pain, anorexia, vomiting and low grade fever
Initial management if appendicitis expected
Admit, Iv fluids, nil by mouth and surgical review
What are Auer rods?
Needle like bodies in the cytoplasm of myeloblast and/orproganulocytes in leukemia
What is factitious hypoglycaemia?
A deliberate attempt to induce hypoglycaemia - often via insulin or oral hypoglycaemic agents. C-peptide will be low, glucose low and insulin high
Typical age of ASD/VSD manifestation?
Childhood or adulthood - not infancy
Encephalitis symptoms
Altered behavious/encephalopathy, decreased consiousness, focal neurology/seizure on a background of viral prodrome of fever and lethargy
Main causes of viral encephalitis - children/adults and neonates
Children/adults - HSV-1
Neonates - HSV-2 from the genital tract during birth
