Paeds VII Flashcards
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
What are intrinsic [2] and systemic [3] diseases that cause nephrotic syndrome
Most common cause: MCD
It can be secondary to intrinsic kidney disease:
* Focal segmental glomerulosclerosis
* Membranoproliferative glomerulonephritis
It can also be secondary to an underlying systemic illness:
* Henoch schonlein purpura (HSP)
* Diabetes
* Infection, such as HIV, hepatitis and malaria
Vulvovaginitis is a common presentation in young girls before puberty. It presents with: [6]
- Soreness
- Itching
- Erythema around the labia
- Vaginal discharge
- Dysuria (burning or stinging on urination)
- Constipation
- A urine dipstick may show leukocytes but no nitrites. This will often result in misdiagnosis as a urinary tract infection.
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 an atypical / recurrent UTI.
Which mode of investigation should be given after 4-6 months? [1]
DMSA (Dimercaptosuccinic Acid) Scan
* DMSA scans should be used 4 – 6 months after the illness to assess for damage from recurrent or atypical UTIs.
* This involves injecting a radioactive material (DMSA) and using a gamma camera to assess how well the material is taken up by the kidneys.
* Where there are patches of kidney that have not taken up the material, this indicates scarring that may be the result of previous infection.
What is the Vesico-Ureteric Reflux (VUR)? [1]
Why is this significant with regards to UTIs? [1]
How is it diagnosed? [1]
Vesico-ureteric reflux (VUR) is where urine has a tendency to flow from the bladder back into the ureters when the bladder contracts
- This predisposes patients to developing upper urinary tract infections and subsequent renal scarring
- Means that have urine sitting in bladder and back pressure in kidneys
- This is diagnosed using a micturating cystourethrogram (MCUG).
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 [+]
What is the simplified pathophysiology of rickets? [3]
Which populations are at higher risks of rickets? [4]
Vitamin D is a hormone (not technically a vitamin) created from cholesterol by the skin in response to UV radiation.
Inadequate vitamin D leads to a lack of calcium and phosphate in the blood
- Since calcium and phosphate are required for the construction of bone, low levels result in defective bone mineralisation
Low calcium causes a secondary hyperparathyroidism as the parathyroid gland tries to raise the calcium level by secreting parathyroid hormone. Parathyroid hormone stimulates increased reabsorption of calcium from the bones. This causes further problems with bone mineralisation.
Those at higher risk:
- Patients with darker skin require a longer period of sun exposure to generate the same quantity of vitamin D
- Patients with malabsorption disorders (such as inflammatory bowel disease) are more likely to have vitamin D deficiency.
- CKD: The kidneys are essential in metabolising vitamin D to its active form, therefore vitamin D deficiency is common in chronic kidney disease.
- Limited exposure to sunlight
- Low Ca & P diets
- Medications like glucocorticoids and ARVs
TOM TIP: Think about the risk factors for vitamin D deficiency in your exams and clinical practice. Patients with rickets are likely to have risk factors such as darker skin, low exposure to sunlight, live in colder climates and spend the majority of their time indoors.
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
Investigations for Rickets?
Serum 25-hydroxyvitamin D is the laboratory investigation for vitamin D.
- A result of less than 25 nmol/L establishes a diagnosis vitamin D deficiency, which can lead to rickets.
Xray is required to diagnose rickets.
- X-rays may also show osteopenia (more radiolucent bones).
Bone profile
* Serum calcium may be low
* Serum phosphate may be low
* Serum alkaline phosphatase may be high
* Parathyroid hormone may be high
Why are breastfed babies more at risk of rickets? [1]
How do you avoid this? [1]
Prevention is the best management for rickets. Breastfed babies are at higher risk of vitamin D deficiency compared with formula fed babies, as formula feed is fortified with vitamin D.
- Breastfeeding women and all children should take a vitamin D supplement. NICE clinical knowledge summaries recommend supplements containing 400 IU (10 micrograms) per day for children and young people.
How do you treat vitamin D deficiency in children? [1]
How do you treat rickets? [2]
Children with vitamin D deficiency can be treated with vitamin D (ergocalciferol). The doses for treatment of vitamin D deficiency depend on the age (see the BNF).
- The dose for children between 6 months and 12 years is 6,000 IU per day for 8 – 12 weeks.
Children with features of rickets should be referred to a paediatrician.
- Vitamin D and calcium supplementation is used to treat rickets.
- Surgical Intervention: In severe cases where there are bone deformities, surgical correction might be necessary. This includes procedures like osteotomies and epiphysiodesis.
The most common cause of hypothyroidism in children [1]
The most common cause of hypothyroidism in children (juvenile hypothyroidism) is autoimmune thyroiditis.
What is meant by congenital hypothyroidism? [1]
How is it detected? [1]
Congenital hypothyroidism is where the child is born with an underactive thyroid gland.
- This occurs in around 1 in 3000 newborns. It can be the result of an underdeveloped thyroid gland (dysgenesis) or a fully developed gland that does not produce enough hormone (dyshormonogenesis).
- Very rarely it can be the result of a problem with the pituitary or hypothalamus. This usually occurs without any other problems and the cause is not clear.
Congenital hypothyroidism is screened for on the newborn blood spot screening test. Where it is not picked up a birth, patients can present with:
What is the most common cause of acquired hypothyroidism in children? [1]
How would you detect? [1]
The most common cause of acquired hypothyroidism is autoimmune thyroiditis, also known as Hashimoto’s thyroiditis.
- It is associated with antithyroid peroxidase (anti-TPO) antibodies and antithyroglobulin antibodies
Describe the basic physiology of the release of GH [2]
Growth hormone is produced by the anterior pituitary gland
- It is responsible for stimulating cell reproduction and the growth of organs, muscles, bones and height.
- It stimulates the release of insulin-like growth factor 1 (IGF-1) by the liver, which is also important in promoting growth in children and adolescents.
Describe the differences in causes between congenital and acquired growth hormone deficiency [2]
Congenital growth hormone deficiency
- results from a disruption to the growth hormone axis at the hypothalamus or pituitary gland.
- It can be due to a known genetic mutation such as the GH1 (growth hormone 1) or GHRHR (growth hormone releasing hormone receptor) genes
- or due to another condition such as empty sella syndrome where the pituitary gland is under-developed or damaged.
Acquired growth hormone deficiency:
- can be secondary to infection, trauma or interventions such as surgery.
How may GHD present at birth? [3]
How many GHD present later in childhood? [4]
Growth hormone deficiency may present at birth or in neonates with:
* Micropenis (in males)
* Hypoglycaemia
* Severe jaundice
Older infants and children can present with:
* Poor growth, usually stopping or severely slowing from age 2-3
* Short stature
* Slow development of movement and strength
* Delayed puberty
* Obesity
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.
Growth hormone deficiency is related to numerous other conditions. Which tests would you conduct to assess for these? [4]
Test for other associated hormone deficiencies, for example thyroid and adrenal deficiency
MRI brain for structural pituitary or hypothalamus abnormalities
Genetic testing for associated genetic conditions such as Turner syndrome and Prader–Willi syndrome
Xray (usually of the wrist) or a DEXA scan can determine bone age and help predict final height
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
