PBL B11 W3

Question 1

What is pre-term birth?

Answer 1

Pre-term birth is live birth before 37 weeks gestation.

Question 2

What are the risk factors for pre-term birth?

Answer 2

Higher maternal age, low/high BMI, Short interval pregnancy, infection, Uterine anomalies, Cervical weakness.

Question 3

Which organs mature in the 3rd trimester?

Answer 3

Limbs
Ears
Bones
Skin
Lungs
Kidney
CNS

Question 4

When does the heart fully develop?

Answer 4

Second trimester.
-> However, the shunts of the heart close during birth due to a drop in prostaglandin and increase in oxygen. This includes the ductus arteriosus, ductus venosus and foramen ovale.

Question 5

When are the ears fully developed?

Answer 5

32-35 weeks gestation

Question 6

Which organs require full-term birth for mature development?

Answer 6

Lungs
Kidneys
CNS including brain and spinal cord

Question 7

Which organ is the last to mature/develop?

Answer 7

Lungs

Question 8

When does lung surfactant production begin?

Answer 8

Begins production at 30-32 weeks gestation and reaches sufficient levels at 35 weeks.

Question 9

What are the stages of lung development?

Answer 9

Pseudo glandular stage with formation of terminal bronchioles

Canalicular phase with formation of respiratory bronchioles

Terminal sac stage with formation of alveoli.

Question 10

What are the acute complications in premature babies?

Answer 10

Less subcutaneous fat so risk of hypothermia.
Jaundice
Failure of heart shunts to close, eg, Patent ductus arteriosus
Intraparenchymal haemorrhage
Susceptibility to infection
Anaemia
Reflux and aspiration
Apnoea and respiratory distress
Necrotising entercolitis
Retinopathy of immaturity

Question 11

GI issues in pre-term babies?

Answer 11

1)Gastro-oesophageal reflux due to weakness of the sphincter with regurgitation leading to malnutrition and risk of aspiration of the contents so a feeding tube is required.
2)Necrotising enterocolitis is inflammation of the intestines which necrose and form a perforation that allows the contents of the intestines to leak into the abdomen, leading to peritonitis and sepsis. This can occur due to immaturity of the bowels, low blood flow or increased infection risk. Reduced risk with breastmilk.

Question 12

What are the long term complications in pre-mature babies?

Answer 12

Bronchopulmonary dysplasia and asthma
Cerebral palsy, mental retardation
Hearing impairment
Behavioural and social issues
Hypertension and cardiovascular disease in adulthood.

Question 13

Acute Lung issues in pre-term babies?

Answer 13

Respiratory distress syndrome
Atelactasis
Apnoea
-> Occurs due to pulmonary hypoplasia, breathing using terminal bronchioles and primitive alveoli. Insufficient surfactant levels mean alveoli are more prone to collapse and insufficient gas exchange, leading to hypercapnia and respiratory acidosis. Artificial surfactant can be administered for treatment and corticosteroids and cortisol for maturation.

Question 14

Chronic lung issues in pre-term babies?

Answer 14

Bronchopulmonary dysplasia is a chronic breathing disorder where the bronchi are damaged, resulting in the tissue destruction of the alveoli. This occurs typically because of high pressure mechanical ventilation that causes damage and scarring of pulmonary tissue in the treatment of abnormal or immature lung development. Increases the risk of infection and lung inflammation. There is no treatment so the focus is nutrition to support repair and development.

Question 15

What is a common severe complication in premature babies due to anaemia or lung/heart conditions?

Answer 15

Hydrops fetalis- severe accumulation of fluid in 2 or more areas in the foetal body as a result of reduced blood flow, hypoxia, allowing the accumulation of interstitial fluid.

Question 16

Eye issues in pre-term babies?

Answer 16

Formation of abnormal blood vessels in the retina impairs vision in Retinopathy of Immaturity due to fluctuations in oxygenation at birth.

Question 17

Short-term CNS issues in pre-term babies

Answer 17

Issues with autoregulation of cerebral blood flow results in Intraparenchymal Haemorrhage. This is bleeding into the parenchyma of the brain tissue consisting of the neurons and glial cells

Question 18

Long-term CNS issues in pre-term babies

Answer 18

Cerebral Palsy, a disorder of movement due to inadequate blood flow. Cognitive impairment and behavioural and psychological issues.

Question 19

What is a medical error?

Answer 19

A medical error is a preventable adverse effect on patient care, whether or not it occurs regarding either treatment or diagnosis.

Question 20

What is the term used to refer to harm caused to patients by medical interventions?

Answer 20

Iatrogenesis

Question 21

What are common types of medical errors?

Answer 21

1)Prescribing error
2)Diagnosis in a timely manner
3)Acute conditions such as wound infection, pressure ulcers and nosocomial infections. The most common are E-coli causing UTIs, Pneumonia due to environment or ventilators and surgical site infections
4)Communication failure in a MDT.
5)Using outdated tests or guidelines.

Question 22

What increases risk of medical errors?

Answer 22

Schedule instability in the care providers
Staffing shortages
Inadequate training and supervision
Sleep deprivation and provider burnout.

Question 23

How should medical errors be dealt with?

Answer 23

Complete and timely explanation to patients and their families to demonstrate compassion and empathy for the impact, as well as to senior supervisiors. When addressing patients, the SPIKES protocol may be useful in order to address and provide all necessary information on those affected by the error. Supervised practise and training of the physician may be required to identify and manage the cause for concern in the error.

Question 24

How can medical errors be dealt with lawfully?

Answer 24

Compensation, accountability or retributive punishment from physician to patients or community

Question 25

What is the impact of medical errors on physicians?

Answer 25

Physicians after a medical error deal with intense feelings of guilt, shame and loss of confidence in practise, leading to significant impacts ranging from time off from profession to leaving it entirely. Counselling or discussion with a trusted colleague may be helpful because medical errors are part of practise.

Question 26

What are the types of hearing loss?

Answer 26

Conductive and sensory hearing loss.

Question 27

What is conductive hearing loss?

Answer 27

This is when sounds cannot travel through the outer and middle ear canal, treated with medication or surgery.

Question 28

What is the cause of conductive hearing loss?

Answer 28

Buildup of earwax or object in ear canal

Fluid behind ear drum

Injury to eardrum

Otitis media: infection to middle ear

Multiple infections or allergy leading to fluid build-up and scarring of eardrum

Benign tumours

Question 29

What is sensory hearing loss?

Answer 29

Inner ear damage, affecting the nerve endings to transmit sound to the brain.

Question 30

What is the cause of sensory hearing loss?

Answer 30

Genetic disorders that cause deformity in structure such as Down syndrome

Ototoxic drugs
-> Includes aminoglycoside antibiotics such as Gentamycin
-> Cisplatin, a cancer chemotherapy drug

Temporary damage includes loop diuretics, quinine to treat malaria and aspirin for pain relief and myocardial infarction.

Congenital infections such as toxoplasmosis, measles and herpes from mother in utero

Meningitis, cytomegalovirus, rubella and measles contracted after birth cause inner ear damage

Tumour in ear

Question 31

Which infections can lead to hearing damage?

Answer 31

Toxoplasmosis
Measles
Herpes
HIV
Meningitis
Rubella

Question 32

What are the hearing screening tests?

Answer 32

Hearing screen test must be offered between 0-5 weeks to identify babies with permanent hearing loss at an early stage, because many babies with hearing loss are born to families with no history. It is provided in hospital births before discharge or alternatively from 3-4 weeks post birth up to 3 months.

Question 33

What are the types of hearing tests?

Answer 33

Automated Oto-Acoustic Emission where a microphone and earphone are placed inside the ear to test for healthy cochlea, if an echo is reflected back from ear canal into microphone This is limited based on fluid in ear canal or loud noises in the room. Baby must be quiet and still.

Automated Auditory Brainstem Response: Electrodes placed on the baby’s head in order to test auditory pathway between ear canal and brain via playing sounds in the ear and detecting brainwaves. Must be done while baby is asleep to test brainstem response.

Question 34

What are the domains of child development?

Answer 34

Gross motor skills
Fine motor skills
Hearing, speech and language skills
Social, psychological and behavioural skills

Question 35

What are the milestones for gross motor skills?

Answer 35

Raises head at 6 weeks

Sitting without support at 6-8 months

Crawling at 8-9 months

Independently standing and cruising at 10 months

Walks unsteadily from 12-18 months

Runs and jumps by 2 years

Question 36

What are the milestones for fine motor skills?

Answer 36

6wks: tracking objects

6 mths: palmar grip and transferring objects between hands

9 months: inferior pincer grip and object permanence

12 mths: make a brick of 2

18ths: build 4 bricks

2 yrs: 8 bricks and draws line

3 yrs: turns book page and draws circle

Question 37

What are the milestones for speech and language skills?

Answer 37

Startle to loud noise as newborn

6 mths: babbles and understands BYE and NO

9 mths: responds to name

12mths: understands nouns

18 mths: knows 1-6 words

2 yrs: understands verbs

2.5 yrs: understands what “in, out and on” mean

3 yrs: understands negatives and adjectives

Question 38

What are the milestones for social skills?

Question 39

When does developmental surveillance occur?

Answer 39

Neonatal examination at birth

6 weeks check by GP

1 year old check by health visitor

2/2.5 yr old check by health visitor

Question 40

What are the limitations of pre-natal testing?

Answer 40

Prenatal testing can only be used to determine risk, not diagnosis of genetic conditions. There are limitations and may have limited predictive value. There is a risk of a false positive or false negative.

Question 41

Which genetic conditions are commonly screened in pre-natal testing?

Answer 41

Patau’s: extra copy of chromsome 13

Down’s: Trisomy 21

Edward’s: extra copy of chormosome 18

Question 42

What are the types of prenatal screening tests?

Answer 42

A-fetoprotein test: indicates genetic abnormalities and foetal distress such as Down’s syndrome

Amniocentesis: extraction of amniotic fluid

Percutaneous umbilical cord sampling: sample from the umbilical vein

Infection screening test for Hep B, HIV, syphyllis

Question 43

What are the child screening tests?

Answer 43

Newborn blood spot test for sickle cell, cystic fibrosis , congenital hypothyroidism or 6 metabolic disorders

Neonatal hearing screening programme

Child Health surveillance programmes to reduce childhood obesity, encourage breastfeeding and identify areas for concern in development. Occurs in Reception and Year 6.

Question 44

What is Duchenne muscular dystrophy?

Answer 44

Duchenne muscular dystrophy is a progressive muscle wasting X-linked condition caused by deficiency in dystrophin.DMD is the most inherited neuromuscular condition. It is typically diagnosed with a muscle biopsy. There is normal achievement of milestones, but it is delayed, and growth velocity is reduced resulting in a normal stature.

Question 45

What are the features of DMD?

Answer 45

Around 2-3 years of age, gross motor skills may regress and toe walking, difficulty running or climbing upstairs may present. Frequent falling increases the risk of fractures.

Trendenleburg gait is common and by 12 years old, patients are typically in wheelchairs.

Weakness of the pharyngeal muscles leads to aspiration, regurgitation of food and nasally voice are common. Posture is a common issue due to weakness of muscles and may result in scolisosis.

Due to weakness of cardiac muscles, dilated cardiomyopathy occurs where the left ventricle is weaker and less able to pump blood efficiently to the body.

Question 46

How does significant illness affect family?

Answer 46

Stress, financial pressures due to tme of work, sleep deprivation,

Question 47

What is sickle cell disease?

Answer 47

Sickle cell is a recessive disease that is autosomal caused by a point mutation on chromosome 11 in the HBB gene for glutamine -> val, resulting in HBS globin, a sickle shaped erythrocyte. Under hypoxic conditions, this results in crystallisation which can block blood vessels and result in a severe pain crisis. The malformed shape means they are cleared at a high rate by the liver faster than bilirubin is excreted, leading to jaundice.

Question 48

What is thalassemia?

Answer 48

Thalassemia is when there is underproduction of haemoglobin categorised into alpha thalassemia and beta thalassemia depending on the globin chain affected. Results in anaemia, enlarged spleen due to clearing of affected RBC. Carriers may be protected against malaria and coronary artery disease due to a lower blood haematocrit.

Question 49

What is cystic fibrosis?

Answer 49

Caused by deletion of 3 nucleotides in the 7q31.2 chromosome, resulting in the loss of phenylalanine that is important in the CFTR gene. This encodes for the CFTR pump, for Chloride extrusion, affecting Na+ and H20 absorption and to production of thicker mucus

Question 50

What is creatinine?

Answer 50

Creatinine is a breakdown product from protein or muscle metabolism of creatine and is used as an indicator of kidney function because it is majorly cleared by the kidneys into urine.

Question 51

What are the investigations for muscle disease?

Answer 51

Muscle biopsy is taken for testing. Blood test will reveal high levels of creatinine due to muscle lysis occurring in excess.
Electromyography to examine electrical activity in nerves and muscles at rest and when stimulated.
MRI and CT scans of the limbs.
Genetic testing is also performed such as PCR testing.

Question 52

What are the developmental red flags?

Answer 52

Regression, toe walking, not responding to sounds, early hand preference, not fixing and following.