2016 remembered Flashcards
Mechanism of action of phenytoin
Na channel blocker
What is best measure of lung function in kid with respiratory muscular disease? (eg
myasthenia gravis)
FVC
Best measure of lung function in kid with obstructive disease?
FEV1
Which drug to use for listeria?
Ampicillin
Child has EEG and features consistent with absence epilepsy. First line tx? Ethosuximide Carbamazepine Sodium valproate Lamotrigine Keppra
Ethosuximide
What cells does MHC II attach to? Macrophages Natural Killer cells T cells Neutrophils
T cells
What gene is responsible for APACED?
AIRE gene
What of the following is resistance to cephalosporins?
Enterococcus faecalis
?6yo Indigenous girl who has been unwell 3 times in last 6 months. Now has had 3 days of
coryzal symptoms with the X-Ray shown. On examination has reduced air entry right side.XRay (Borderline large heart with right pleural effusion)
Myocarditis
Pericarditis
Dilated cardiomyopathy
?Myocarditis
What do you give for anti freeze swallowed by a child?
Sodium bicarb to correct systemic acidosis
fomepizole to inhibit enzyme alcohol dehydrogenase
Child with sickle cell anaemia, lethargy, weakness, no fevers. Amoxicillin week before. No splenomegaly, slightly enlarged liver.
Aplastic anaemia
Parvovirus
Splenic sequestration
Hyperhaemolysis
Aplastic crisis is defined as an acute illness associated with haemoglobin below baseline for that patient associated with a substantially decreased reticulocyte count (usually <1%). Usually associated with acute infection including parvovirus.
May be associated with enlarged spleen as
Splenic sequestion is defined as a haemoglobin drop of at least 20gm/l below baseline level for that patient with an acutely enlarged spleen. Mild to moderate thrombocytopenia is often present. Reticulocyte count is equal to or greater than patient’s usual baseline. Consider co-existent aplastic anaemia if reticulocyte count is low.
Girl with Turner’s syndrome presents with bruising from early age. APTT high PT normal Fibrinogen normal Hb low Platelets normal
Haemophilia A
Von Willebrand’s Disease
Haemophilia A (deficiency of Factor 8)
Hemophilia is a disease almost exclusively of males because the defective gene is found on the X chromosome. The deficiency or absence of either of 2 clotting elements—factor VIII or factor IX—leads to the clinical condition described as hemophilia A or hemophilia B,
Females are generally asymptomatic carriers; this condition is so rare in females that another cause of unexplained or problematic bleeding should be considered before this condition. For example, a traumatic effusion or pigmented villonodular hyperplasia is more likely than hemophilia as an etiology for a bleeding disorder in a female
e.g. a girl not only inherited 1 diseased X chromosome with mild factor IX hemophilia from her father, but she also has Turner (XO) syndrome. The child’s only X chromosome had the hemophilia gene.
You suspect a child has hereditary angioedema. What is the best screening test?
C4 level
CH50 haemolytic complement test
C4 level
Child with fevers, pneumonia. Found to have low sodium 124, normal potassium, normal
Cr?
Cause
SIADH
Diabetes Insipidus
SIADH
Child with polyuria, polydipsia. Preferred water over formula milk from 12mo on.
Bloods Na
Water deprivation test:
Serum osmolality normal at 1 hour, 2 hours
Urine osmolality slightly increased at 1 and 2 hours after water deprivation then more
increase with ddAVP
How much is the increase. If >100%, then DI
Mum and dads bloods are shown. It appears mum has beta thal and dad has sickle cell. No adult Hb is shown on the cord blood. Is this;
Normal variation Beta trait Sickle cell trait beta/sickle beta thal
beta/sickle
Sickle cell beta thalassemia is inherited in an autosomal recessive pattern. Sickle cell beta thalassemia occurs when one abnormal gene for the production of hemoglobin S is inherited from one parent and one abnormal gene for the production of beta thalassemia is inherited from the other parent.
A baby testing positive for sickle cell beta thalassemia will have higher than normal fetal hemoglobin Hgb F with Hgb S and little to no presence of adult hemoglobin (Hgb A).
In a person affected with sickle cell beta thalassemia, some of the red blood cells sickle in shape, subsequently hemolyzing resulting in anemia, which is a hallmark of sickling diseases. And because of the reduced production of beta-globin chains prevents the development of normal red blood cells, the production of both quantity and quality of red blood cells is affected. As a result, the sickled cells do not live as long as normal red blood cells, tend to get stuck in blood vessels and block flow of blood to certain parts of the body. These consequences can lead to poor growth, impaired physical activity, bone deformities, fragile bones and enlargement of the liver and spleen.
Homeless 14yo girl has baby. Refuses blood test and has had no antenatal care.
What immunisation regime do you give baby?
Hep B vaccine
Hep B vaccine + Hep B immunoglobulin
All immunoglobulin
Hep B vaccine + Hep B immunoglobulin
Urine dipstick gets mistaken for blood with which agent?
Options – ascorbic acid, myoglobin, urates, rifampicin
myoglobin
7 yo girl with pubic hair and body odour. No breast development. Bone age 7years
21 OHP – 2.7 (upper normal 2.7)
Androgen – N
DHEAS – N
Cause?
Premature adrenarche
Central precocious puberty
CAH
Premature adrenarche
Question about mums of kids with brain tumours (50) and mums of kids without brain tumours (50). Total in study is 100.
Case control study of who took folate beforehand (20 of mums with kids with brain tumours) and 10 of mums in control who didn’t have brain tumour took folate
What is odds ratio?
1/5
2/5
3/7
~ 1/5
Odds Ratio = AD/BC = 200/1200
Kid with Sa02 85%RA and wheeze. Given tx for asthma but no improvement. CXR AP and
lateral – decrease volume in UL
Cause:
Upper lobe collapse
Mediastinal mass
Bronchogenic cyst
Upper lobe collapse
Child comes in with fatigue, weakness, limp and left hip pain. ?Visual problems. X Ray pelvis
and hips shows lytic lesion in left hip.
Dx? Neuroblastoma Osteosarcoma Ewings Osteochondral cyst
Ewing’s
Osteosarcoma
- an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin (and thus a sarcoma) and that exhibits osteoblastic differentiation and produces malignant osteoid.
- any patients first complain of pain that may be worse at night, may be intermittent and of varying intensity and may have been occurring for some time.
- Teenagers who are active in sports often complain of pain in the lower femur, or immediately below the knee. If the tumor is large, it can present as overt localised swelling.
- Sometimes a sudden fracture is the first symptom
- A characteristic often seen in an X-ray is Codman’s triangle, which is basically a subperiosteal lesion formed when the periosteum is raised due to the tumor. Films are suggestive, but bone biopsy is the only definitive method to determine whether a tumor is malignant or benign.
Ewings
- a type of cancer that forms in bone or soft tissue.
- Symptoms may include swelling and pain at the site of the tumor, fever, and a bone fracture.
- The most common areas where it begins are the legs, pelvis, and chest wall.
- In about 25% of cases, the cancer has already spread to other parts of the body at the time of diagnosis.[3]
- Complications may include a pleural effusion or paraplegia.
- more common in males (1.6 male:1 female) and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age. It can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones, especially around the growth plates. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus.
- Thirty percent are overtly metastatic at presentation
- most common osseous presentation is a permeative lytic lesion with periosteal reaction - “onion-skin” type periosteal reaction.
Child knocked in eye during some trauma at school. Normal neurology except left RAPD.
Damage to which structure caused this? Retinal detachment Orbital blow out fracture Hyphema Subconjunctival haemorrhage
Retinal detachment or Orbital blow out fracture
Causes:
- Optic nerve disorders: Unilateral optic neuropathies are common causes of RAPD.
- Demyelination Optic neuritis: Even very mild optic neuritis with a minimal loss of vision can lead to a very strong RAPD.
- Ischemic optic neuropathies: These include arteritic (Giant Cell Arteritis) and non-arteritic causes. Usually there will be a loss of vision or a horizontal cut in the visual field.
- Glaucoma: While glaucoma normally is a bilateral disease, if one optic nerve has particularly severe damage, an RAPD can be seen.
- Traumatic optic neuropathy: This includes direct ocular trauma, orbital trauma, and even more remote head -injuries which can damage the optic nerve as it passes through the optic canal into the cranial vault.
- Optic nerve tumor: This is a rare cause and includes primary tumors of the optic nerve (glioma, meningioma) or tumors compressing the optic nerve (sphenoid wing meningioma, pituitary lesions, etc.).
- Compressive optic neuropathy with or without orbital disease: This could include compressive damage to the optic nerve from thyroid related orbitopathy (compression from enlarged extraocular muscles in the orbit), orbital tumors, or vascular malformations.
- Radiation optic nerve damage
- Hereditary optic neuropathies, such as Leber’s optic neuropathy (usually eventually bilateral) and other inheritable optic neuropathies.
- Other optic nerve infections or inflammations: Cryptococcus can cause a severe optic nerve infection in the immunocompromised. Sarcoidosis can cause inflammation of the optic nerve. Lyme disease can affect the optic nerve.
- Optic atrophy status: post papilledema - This is usually bilateral.
- Post Surgical damage to the optic nerve: This could include damage following retrobulbar anesthesia; damage following orbital hemorrhage related to eye, orbital, sinus, or plastic surgery; damage following neurosurgical procedures such as pituitary tumor resection; and damage related to migration of an orbital plate after surgery to correct a blow-out fracture.
Retinal conditions - Usually, retinal disease has to be quite severe for an RAPD to be clinically evident.
- Ischemic retinal disease: Causes include ischemic central retinal vein occlusion, central retinal artery occlusion, severe ischemic branch retinal or arterial occlusions, severe ischemic diabetic or sickle-cell retinopathy.
- Ischemic ocular disease (Ocular ischemic syndrome): This usually arises from obstruction of the ophthalmic or carotid artery on one side.
- Retinal detachment: A RAPD can often be seen if the macula is detached, or if at least two quadrants of retina are detached.
- Severe macular degeneration: If unilateral and severe, a RAPD can be seen. Usually the visual acuity would be less than 20/400.
- Intraocular tumor: Retinal and choroidal tumors including melanoma, retinoblastoma, and metastatic lesion could lead to a RAPD if severe.
- Retinal infection: Cytomegalovirus, herpes simplex, and other causes of retinitis can lead to a RAPD if there is extensive disease.
Child in respiratory distress at birth. Massive cardiomegaly on CXR
Cause?
Ebstein’s anomaly
Baby, respiratory distress at birth. Cyanotic.
CXR – nil cardiomegaly
Cause?
TGA
TAPVR
TGA
Some spiel about how kids away from their natural homes don’t do well. What is the most common health complication?
Dental carries
Emotional and behavioural problems
Developmental problems
Emotional and behavioural problems
Difficult 5yo child causing trouble at home and school. Yelling and out of control. Best
therapy?
CBT
Multisystem therapy
Play therapy
Parental therapy
?Parental therapy
The cornerstones of treatment for ODD usually include:
- Parent training.
- Parent-child interaction therapy (PCIT).
- Individual and family therapy.
- Cognitive problem-solving training.
- Social skills training.
As part of parent training, you may learn how to manage your child’s behavior by:
Giving clear instructions and following through with appropriate consequences when needed
Recognizing and praising your child’s good behaviors and positive characteristics to promote desired behaviors
