Paeds IV Flashcards
(31 cards)
A baby is born at full term following a vaginal delivery complicated by a shoulder dystocia. The baby is born in very poor condition and accordingly, the neonatal team resuscitate and intubate the baby. The baby is profoundly acidotic and demonstrates global hypotonia with abnormal neonatal reflexes; the neonatal team decide on the most appropriate step in management as they are concerned about hypoxic brain injury.
Which intervention is most important for this baby?
Therapeutic cooling at 33-35 degrees attempts to reduce the chances of severe brain damage in neonates with hypoxic injury
How do you dx UTIs in children? [4]
Describe the mx of UTIs in:
- infants
- children with upper UTI [2]
- children with lower UTI [2]
infants less than 3 months old
- should be referred immediately to a paediatrician
children aged more than 3 months old with an upper UTI
- should be considered for admission to hospital.
- If not admitted oral antibiotics such as cephalosporin or co-amoxiclav should be given for 7-10 days
children aged more than 3 months old with a lower UTI
- should be treated with oral antibiotics for 3 days according to local guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin.
- Parents should be asked to bring the children back if they remain unwell after 24-48 hours
How do you further investigate all children under 6 months [1] recurrent UTIs [1] or atypical UTIs [1]?
Ultrasound Scans:
All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria
Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks
Children with atypical UTIs should have an abdominal ultrasound during the illness
A child has a UTI that is found to be caused by Klebsiella.
Why is this clinically significant? [1]
Atypical causes of infection point towards a structural cause of UTIs (as opposed to typical)
Lecture:
What are the investigations for UTIs for < 6 months, 6months-3yrs and > 3 years [+]
Patients with vitamin D deficiency and rickets may not have any symptoms. Potential symptoms are [6]
Bone deformities that can occur in rickets include: [4]
Patients with vitamin D deficiency and rickets may not have any symptoms. Potential symptoms are:
* Lethargy
* Bone pain
* Swollen wrists
* Bone deformity
* Poor growth
* Dental problems
* Muscle weakness
* Pathological or abnormal fractures
Bone deformities that can occur in rickets include:
* Bowing of the legs, where the legs curve outwards
* Knock knees, where the legs curve inwards
* Rachitic rosary, where the ends of the ribs expand at the costochondral junctions, causing lumps along the chest
* Craniotabes, which is a soft skull, with delayed closure of the sutures and frontal bossing
* Delayed teeth with under-development of the enamel
Investigation, diagnosis and management will be made by specialists in paediatric endocrinology using which tests? [1]
Describe this test [1]
Growth hormone stimulation test:
- glucagon, insulin, arginine and clonidine can be used to stimulate release of growth hormone
- Growth hormone levels are monitored regularly for 2-4 hours
- In growth hormone deficiency there will be a poor response to stimulation.
Mx of Growth Hormone Deficiency? [3]
Daily subcutaneous injections of growth hormone (somatropin)
Treatment of other associated hormone deficiencies
Close monitoring of height and development
The investigation of delayed puberty involves a structured approach to identify underlying causes and guide appropriate management.
Initial investigations should focus on first-line tests, followed by more specific assessments if necessary.
What are the first line tests and what do they show? [5]
Bone Age Assessment:
- A radiograph of the left hand and wrist is typically performed to evaluate skeletal maturity.
- Bone age significantly behind chronological age may indicate constitutional delay of growth and puberty (CDGP).
Serum Gonadotropins:
- Measurement of luteinising hormone (LH) and follicle-stimulating hormone (FSH) levels can distinguish between hypogonadotropic hypogonadism (low or normal LH/FSH) and hypergonadotropic hypogonadism (elevated LH/FSH).
Sex Steroid Levels:
- Serum testosterone in males or estradiol in females helps assess gonadal function.
- Low levels are indicative of delayed puberty.
Thyroid Function Tests:
- Evaluate thyroid-stimulating hormone (TSH) and free thyroxine (T4) to rule out hypothyroidism, which can contribute to delayed puberty.
Karyotype Analysis:
- Particularly indicated in females with primary amenorrhoea, to diagnose conditions such as Turner syndrome
How do you treat delayed puberty in the cases of permanent hypogondatropic hypogonadism due to structural hypothalamic/pituitary disease or genetic defects?
How do you treat delayed puberty in the cases of primary gonadal failure (hypergonadotropic hypogonadism)? [1]
permanent hypogondatropic hypogonadism due to structural hypothalamic/pituitary disease or genetic defects
- long-term sex steroid replacement
- In males, testosterone can be used; in females, a combination of oestrogen and progesterone.
primary gonadal failure (hypergonadotropic hypogonadism)
- lifelong sex steroid replacement therapy is required for induction of puberty and maintenance of secondary sexual characteristics
[] is indicated by NICE as a treatment for Prader-Willi Syndrome, aimed at improving muscle development and body composition.
Growth hormone is indicated by NICE as a treatment for Prader-Willi Syndrome, aimed at improving muscle development and body composition.
Describe the clinical features of William syndrome [+]
Include TT [5]
- Broad forehead
- Starburst eyes (a star-like pattern on the iris)
- Flattened nasal bridge
- Long philtrum
- Wide mouth with widely spaced teeth
- Small chin
- Very sociable trusting personality
- Mild learning disability
TOM TIP:
- The distinctive features to remember with William syndrome are the very sociable personality, the starburst eyes and the wide mouth with a big smile. It is worth remembering the association with supravalvular aortic stenosis and hypercalcaemia, as these are unique features that are easy to test in exams.
What are the associated conditions of William syndrome/ [4]
Supravalvular aortic stenosis (narrowing just above the aortic valve)
Attention-deficit hyperactivity disorder
Hypertension
Hypercalcaemia
Ix for ITP? [3]
Full blood count
- Platelets: An isolated thrombocytopenia with a platelet count of < 100x109/L is usually the only blood abnormality
- White blood cells - Should be within normal limits
Haemoglobin - Usually within normal limits
Peripheral blood smear:
- Platelets will appear reduced in number
Red and white blood cells will be of normal count and morphology
Bone marrow biopsy
- BMJ recommend performing this test only if there is an atypical blood film. Should appear normal
The management of ITP is different in children compared to adults.
How is it managed? non severe [+]
Conservative management
* ITP in children will resolve spontaneously within 3 weeks in 30-70% of children, according to BMJ
Active management
- This is restricted to children with major bleeding symptoms (e.g. ongoing epistaxis, mucosal bleeding) or a severely low platelet count
- Prednisolone 1-2mg/kg/day orally, with tapering as soon as symptoms begin to clear
- IVIg and anti-D immunoglobulin - - Reserved for if corticosteroids are contraindicated or ineffective
- chronic ITP: medications such as mycophenolate, rituximab and thrombopoietin receptor agonists can be considered (with specialist input)
Tx for severe life-threatening ITP ? [+]
The first line management is with: IVIg, corticosteroids, plus platelet transfusions
- IVIg 1g/kg intravenously as a single dose
- Prednisolone 1-2mg/kg/day orally
- Alternatively, UptoDate suggest methylprednisolone 30 mg/kg per day (up to 1 g) intravenously for 3-4 days
Platelet transfusions
- UptoDate recommend platelet transfusions of a bolus dose of 10-30 mL/kg, then followed by a continuous infusion
- The role of these platelet transfusions is to ensure that the child maintains a haemostatic platelet count and to guide further treatment
There are several conditions that predispose to a higher risk of developing leukaemia.
What are they? [4]
Down’s syndrome
Kleinfelter syndrome
Noonan syndrome
Fanconi’s anaemia
Down’s syndrome patients are most likely to suffer from which type of ASD? [1]
Ostium primum
ASD:
TOMTIP: There appears to be a link between [condition] and [type of ASD].
There appears to be a link between migraine with aura and patent foramen ovale.
However, there are no recommendations in the guidelines for testing or treating patients with migraines for a PFO. It is unclear whether testing or treating improves symptoms or outcomes.
In the vast majority of cases the cause of the maldescent of tests is unknown. A proportion may be associated with other congenital defects including: [5]
- Patent processus vaginalis
- Abnormal epididymis
- Cerebral palsy
- Mental retardation
- Wilms tumour
- Abdominal wall defects (e.g. gastroschisis, prune belly syndrome)
Describe what is meant by a posterior urethral valve, what it causes a risk of [2]
A posterior urethral valve is where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output.
- It occurs in newborn boys.
- The obstruction to the outflow of urine creates a back pressure into the bladder, ureters and up to the kidneys, causing hydronephrosis.
- A restriction in the outflow of urine prevents the bladder from fully emptying, leading to a reservoir of urine that increases the risk of urinary tract infections.
A 1 year child has abdominal pain
They have this imaging.
What is the dx? [1]
What is the tx? [1]
Intestinal malrotation - whirlpool sign
- Tx with Ladd’s procedure
