Peadiatrics Lectures Flashcards
0
Q
When do you give prednisolone is childhood asthma?
A
The use of oral corticosteroids should be limited to children with severe wheeze who require hospital admission. The initial dose is 2mg/kg (max 60mg), followed by daily doses of 1mg/kg for 3 days. A prolonged course may be indicated in severe cases.
1
Q
What is an asthma reducing medication plan?
A
An asthma reducing medication plan is a 3-5 day plan that a child is sent home from hospital on to reduce their medications from an acute asthma attack. The child is then meant to see their GP on day three for a medications review and assessment. If a child is <20 kegs they are given 6 puffs(600mcg) every4 hours, if they are over >29kgs they are given 12 puffs(1200 mcgs) every 4 hours. Eg. <20 kg child day one:6 puffs q4h, day two: 4-6 puffs q6h, day three 2-4 puffs q8h.
>20kg child day 1: 12 puffs q4h, day 2 8-12 puffs q6h, day three 4-8 puffs q 8h. The plan also includes and preventer meds and prednisolone.
2
Q
How do you manage mild asthma?
A
Mild asthma has O2 sats>94%, child is alert, can talk in sentences, no cyanosis, no acessory muscle use. Give salbutamol stat(<20kgs 6 puffs, >20kgs 12 puffs). 3 hourly observations.
3
Q
How do you treat moderate asthma?
A
Moderate asthma pus as a child who is easily engaged, talks in phrases, has a 02 sat of 90-94%, and has no cyanosis. They should be give oxygen and should aim for it to be >94%. They should be give stat salbutamol (6or 12 puffs ) 3 times 20 minutes apart, and then repeat as required. Consider Ipratropium (atroventmuscarinic acetylcholine inhibitor) 20 minutely with salbutamol, give oral prednisolone, give continuous observations for 3 hours then admit or discharge.
4
Q
How do you treat severe asthma?
A
Severe asthma presents with altered consciousness (agitated drowsy), quiet wheeze, O2 sats <90, talks in words, weak cry, or silent, and may have cyanosis. Give oxygen (possibly high flow), give salbutamol and Ipratropium 3 times 20 minutes apart . Give hydrocortisone or methylprednisone, have continous monitoring, consider ABGs, CXR, and UEC. If poor response give IV salbutamol for 1 hour. Consider transfer to PICU.
5
Q
What is the criteria for discharge from an asthma attack?
A
Patient must be weaned off IV salbutamol and oxygen and must not be taking salbutamol every three hours. Stretch asthma medication and discharge with an asthma medication reducing plan and instructions to see GP on third day. Review asthma action plan. Give parents any discharge medications and the children’s asthma resource pack for parents and careers.
6
Q
What are the different patterns of asthma?
A
Infrequent intermittent, frequent intermittent, and persistent (mild, moderate, or severe).
7
Q
What preventer therapy would you give to children with frequent intermittent or persistent asthma?
A
Give monteleukast or a low dose inhaled cortico steroid. If needed increase dose of corticosteroid. Consider adding a long acting beta antagonist or monteleukast. If needed, increased inhaled corticosteroid dose to as high as possible.
8
Q
What is flixotide?
A
Flixotide is fluticasone. Fluticasone is an inhaled corticosteroid that is used once a day. It comes in an inhaler, nebuliser, and accuhaler.
9
Q
What is pulmicort?
A
Pulmicort is budesonide which is a corticosteroid, it is taken once a day as a preventer. It can be taken via a nebuliser or a turbuhaler.
10
Q
What is Qvar?
A
Qvar is beclomethasone is a steroid medication that should be taken once a day.it can be taken via automaker or puffer.
11
Q
What is seretide?
A
Seretide is fluticasone (a corticosteroid) and salmeterol (long acting beta agonist).
12
Q
What is symbicort?
A
Symbicort is budesonide (a corticosteroid) and eformaterol (long acting beta 2 agonist)
13
Q
What is singular?
A
Singuliar is monteleukast. Monteleukast is a leukotriene receptor antagonist.
14
Q
What is alvesco?
A
Alvesco is ciclesonide. It is a glucocorticoid that is taken twice daily and is used to treat asthma.
15
Q
What is omalizumab?
A
Omalizumab is an anti IgE antibody that binds to an area of C3 of free IgE. It attenuates the early and late phase responses to inhaled allergen challenge. It is given in fortnightly to monthly injections and people over 12 years. Has a high rate of anaphylaxis and is expensive. Used in steroid dependent asthma.
16
Q
What is the acceptability and repeatability requirement for spirometer?
A
Good start
Acceptable exhalation (>3 seconds for <10, >6 seconds for >10)
Must have three acceptable manoeuvres
Two largest FVC and FEV1 measurements within 150 mLs of each other.
17
Q
How do you calculate reversibility after a beta agonist in spirometry?
A
100x FEV1 post meds- FEV1 baseline / FEV1 baseline
18
Q
What sort of pattern in spirometry presents with decreased FEV1, FVC (may also be normal), and decreased FEV1/FVC?
A
Obstructive (asthma)
19
Q
What presents with a decreased FEV1, FVC, but a normal or increased FEV1/FVC?
A
Restrictive eg. Scoliosis.
20
Q
What is a normal sleep pattern for a 0-3 month child?
A
95% sleep during the day every day. Take half hour to an hour to settle, may wake up six times during the night, but most commonly twice.
21
Q
What is a normal sleeping pattern for a 1 year old?
A
90% have a daytime nap for 1-2 hours, most settle between 6 and 8 pm, 80% settle quickly, 10% wake more than 3 times a night.
22
Q
What is a normal sleeping pattern for a 2 year old?
A
Half still have daytime naps, they may sleep after 8pm, half still wake once during the night requiring parents attention.
23
Q
When does reflux in a child become pathological?
A
When there is poor growth
If a baby’s vomit is blood stained ( this may indicate oesophagitis)
Rarely if causes breathing difficulties, chest infections, or apnoea
Crying and irritability uncommonly caused by reflux
24
How do you treat reflux in infants?
No treatment required for most children.
Thickened feeds
Positioning (poor evidence)
Meta loperamide (not worth harm for benefit)
PPI or H2 antagonist if suspected oesophagitis
Fundoplication if severe
25
What characterises lactose intolerance?
Abdo pain, diarrhoea, nausea, flatulence, bloating
26
What is developmental lactase deficiency?
Developmental lactase deficiency occurs in babies <34 weeks old
27
What is congenital lactase deficiency?
Congenital lactase deficiency is a very rare disorder that occurs when a child would not survive without lactose free human milk substitute.
28
What is primary lactase deficiency?
Primary lactase deficiency is a relative or absolute absence of lactase that develops in childhood and is uncommon in children < 2 years of age. It is the most common cause of lactose deficiency and is more common in some ethnic groups (Asian and Hispanic)
29
What is secondary lactase deficiency?
Secondary lactase deficiency results from small bowel injury eg.gastro, bacterial, chemo
30
What test can be used to diagnose a lactose intolerance?
A hydrogen breath test can be used to diagnose subtle lactose intolerance. However, normally it is Dx by eliminating
Lactose from the diet and then returning it and watching the correlating pattern in the symptoms.
31
How do you manage frothy stools in a breast fed infant?
Frothy stools are caused by a relative lactose malabsorption. They can be due to an oversupply - advise to feed fully from one breast only for a few feeds.
32
What is the expected weight gain in an infant?
The expected weight gain is 100-200 grams per week
33
What is positional plagiocephaly?
Positional plagiocephaly is when a baby gets a flat head from lying on it too much. There is posterior skull flattening with a n ipsilateral bulge. Benign condition. However, must exclude craniosynotosis (premature fusion of one or more cranial sutures). Also check for torticollis
34
When does the foramen ovale close?
The foramen ovale closes at birth. This results in a combination of increased left atrial pressure secondary to increases in pulmonary blood flow and pulmonary venous return, and from decreases in right atrial pressure secondary to decreases in blood returning to heart from inferior vena cava after placental circulation is removed.
35
When does the ductus venosus close and what makes it close?
The ductus venosus closes shortly after birth due to the cessation of umbilical venous return. There is complete closure 3-7 days after birth.
36
What are the acquired peadiatric heart diseases?
Rheumatic fever (acute and chronic), Kawasaki disease, endocarditis, and some cardiomyopathies.
37
What is e most common type of congenital heart disease?
VSD, then ASD, then PDA
38
What are the different grades of murmurs?
```
Grade 1 : scarcely audible
Grade 2: soft
Grade 3: loud
Grade 4: loud, faint thrill
Grade 5: very loud, easily felt thrill
Grade 6: heard without a stethoscope
```
39
What are the symptoms of an innocent murmur?
Asymptomatic, grade 1-3, changes with position, systolic ejection, musical vibratory
40
What are the symptoms on a pathological murmur?
Diastolic, or loud systolic murmur that has a thrill or radiates to other parts, cyanosis, abnormally strong or weak pulses, abnormal heart sounds eg. Clicks, abnormal CXR, abnormal ECG
41
What is a stills murmur?
Stills murmur is an innocent vibratory, musical systolic ejection murmur that is heard in the tricuspid or aortic areas. It is made louder by lying in the supine position, gets worse with illness, and disappears by puberty.
42
What are the innocent heart murmurs?
Stills murmur,
Carotid bruit,
Venous hum
Pulmonary flow murmur
43
Way is a pulmonary flow murmur?
Also know as a right ventricular outflow murmur, a pulmonary flow murmur is an innocent systolic ejection murmur heard in the pulmonary area.
44
What is a normal finding on ECGs in newborns?
It is normal to have right axis deviation and right ventricular dominance in newborns.
45
What are the causes of a cyanotic heart disease?
Left to right shunt: VSD, ASD, PDA,
| Obstructive heart disease: aortic stenosis, pulmonary stenosis, aortic coarctation
46
What causes cyanotic heart disease?
Right to left shunt:
Tetralogy of fallot, Transposition of the great arteries, truncus arteriosus, tricuspid atresia, total anomalous pulmonary venous connection, Ebstein's anomaly, or a single ventricle.
47
What changes are seen on X-ray in an ASD? What murmur would you hear?
-increase in the right pulmonary vasculature
- Very enlarged right atrium
-moderately enlarged right ventricle and left ventricle.
Cardiomagaly with an advancement of the right cardiac border.
It causes a pulmonary systolic ejection murmur with fixed splitting of the second heart sounds.
48
What changes on x ray indicate a VSD? What murmur can be heard?
There is:
-increased right pulmonary artery vasculature
-increased size of the left atria and left ventricle
-movement of the cardiac border downward and to the left.
It produces a holosyatolic murmur.
49
Where does eczema tend to occur? What are its typical features?
Occurs in flexor surfaces, cradle cap, neck, hands, breasts, . Produces exforiation, redness, oedema, itching, dryness, crusting, flaking, blistering, cracking, oozing or bleeding. Worse with change in weather, sickness and immunisation, skin dryness. Avoid any irritants.
For baby's with bad eczema:
Moisturise 3 times a day
Use a strong topical corticosteroid 3 times a day
Wet dressings may also be used.
50
What is the most characteristic mark of Tinea?
An annular ring.
51
What are the identifying characteristics of a herpes skin infection?
Painful, blistering, well demarcated scalloped edge. Peri oral. If a neonate has HSV it is an emergency.
52
What is the pathogenisis of impetigo?
Impetigo is caused by staph aureus, and also rarely by group A beta heamolytic streptococci. The strains release a toxin that splits the epidermis at the level of the stratum corneum. The split then results in blister formation.
53
What causes most cases of folliculitis in children?
Staph aureus, dermatophytes (Tinea), or pseudomonas aueroginosa
54
What is a vesicle? What are the vesicular rashes?
A vesicle is fluid filled and normally less than 0.5 cm in diameter. Causes of vesicular rashes include:
- varicella zoster
- herpes simplex
- hand foot mouth disease
- molluscum contagiousum
- dermitis heroatiformis
- steven Johnsons syndrome
55
What is the incubation period of chicken pox? When does a person first become infectious? How does it present?
Chicken pox has an incubation period of 10 - 21 days. People become infectious 48 hours prior to onset of the rash until the less crust. It presents with vesicular lesions at different stages, with a predominantly truncal distribution, present in the hair line, and with posterior cervical adenoma thy, and fever at the onset of illness.
56
What is a macular papular rash? What causes maculopapular rashes?
```
A macula is a small flat impalpable lesion, while a papular is a raised circumscribed lesion. Causes of maculopapular rashes include:
Measles
Rubella
Scarlet fever
Kawasakis disease
Erythema infectiousum
Roseola infantum
Other viral infections
```
57
How des the measles present?
The measles presents with an acute catarrhal illness, fever, Koplik spots on the membrane, followed by a rash. There is a high incidence of serious complications of the respiratory and nervous systems. Measles are rare in children less than 3-4 months of age as they have maternal protection. Diagnose using PCR and detecting the presence of measles IgM that develops 3 days after onset of rash.
58
Describe the measles rash.
The measles rash is maculopapular, blotchy, red or pink in colour, raised in places, and starts behind the ears and e face spreading downwards. The child will look miserable. The lesions become confluent on the upper body, skin becomes brown and the rash fades after 2-3 days. De summation occurs, but not on the hands and feet such as in kawasakis disease.
59
How does rubella present?
Rubella generally presents without a prodrome. Instead, the first indication of illness is the rash. It is a fine, pink, maculopapular rash which commences on the face and then the trunk and then the limbs. The lesions are discrete. There is often posterior lymph node involvement, the rash develops more quickly and dissappears more quickly than in measles. Desqumation is not characteristic.
60
What causes Scarlett fever?
Scarlett fever is caused by group A strep
61
How does scarlet fever present?
Scarlet fever presents with a punctiform rash that is dark red. It is most prominent on the neck and major skin folds. A distinctive feature is the circumoral pallor as a result of the rash sparing the mouth. Des quotation occurs on the face and then the trunk and limbs. True scarlet fever is associated with inflammation of the tongue (white and red strawberry tongue), as it is caused by group A strep, there is a risk of acute rheumatic fever, or acute glomerulonephritis. Rx requires a prolonged course of antibiotics to prevent sequelae.
62
How does Kawasaki's disease present?
Kawasaki s disease present typically in children 1-8 years old. They typically have a fever for 5 days or more, as well as
-bilateral conjunctival injection
- rash
-oral changes (red mouth, pharynx, tongue and cracked lips)
- swelling of the hands and feet then desquamation of the hands and feet
-cervical lymph node increase
The rash is discrete maculopapules on the feet,marooned the knees and in the ancillary and inguinal skin creases.
Ddx: scarlet fever, staphylococcal disease. Has a risk of coronary artery aneurysms.
63
What is erythema infectiousum or fifth disease?
Erythema infectiousum or fifth disease is infection with parvovirus B19. It presents with the 'slapped cheek' rash and that then transforms into a lacy maculopapular rash 1-2 weeks later, mainly on the arms and legs brought on by hot baths. The child may also have symmetrical transient joint aches. Diagnosis is made by PCR or parvovirus IgM. Exposure presents a risk to pregnant women who may then have children affected by hydrous details.
64
What is rosella infantum?
Roseola infantum presents is caused by HHV-6. It is milked measles, but even though it commences with a high fever, the fever disappears before the rash comes out. The rash is a widespread maculopapular rash that is mainly on the trunk. Lesions tend to be discrete whereas in measles they become confluent. It is associated with febrile convulsions during the prodrome.
65
What is given to close contacts of those with meningococcal disease as prophylaxis?
Rifampicin,or ceftriaxone, or ciprofloxacin.
66
How do you manage someone presenting with signs of meningococcal infection?
Assess for signs of meningism,
Take a gram stain culture from the blood, the petechial lesions, or the CSF. Also look for meningococcal DNA in the blood and CSF. Take a blood count. May be DIC with thrombocytopenia.
Give prompt administration of IM penicillin, hospital third generation cephalosporins.
67
How do you manage fever in a newborn?
Any newborn wi a fever may also have ether guy, anorexia, or apnoea.
They should have cultures of their blood, CSF, and urine taken, and then should be started on IV antibiotics.
68
What are the side effects of congenital rubella?
Babies develop a triad of cateracts, cardiac, CSF (deaf, microcephalic) abnormalities. They also develop late onset IDDM,. 70% are asymptomatic at birth, but may have a petechial rash in the newborn period. They shed the virus for many years
69
Who is most at risk of heamatomas of the pinna? How is it managed? What complication do you need to be careful to avoid?
Ppl at risk include rugby players. If you drain it, it will re accumulate.minstead you need to drain and stitch on a dressing.mif it recurs the cartilage will die. This then leads to cauliflower ear.
70
What is a cholesteotoma?
A cholesteotoma is a destructive and expanding growth of keratinising squamous epithelium that has gotten into the middle ear. It can either be congenital or acquired.
71
What are the complications of otitis media?
Complications of otitis media including destruction if middle ear structures including VII, localised abscesses (mastoiditis), destruction of inner ear contents (superative labyrinthitis - deafness and dizziness), intracranial complications (extradural abcess, meningitis, subdural abcess, intracranial abcess).
72
If a baby presents with conjunctivitis, what are the most likely causes?
If it presents on day 1, the most likely cause is gonnococcus, day 4-6 is other bacteria, day 10 is chlamydia
73
What is your systematic approach for looking at a chest x ray?
```
Name, date,mpast xrays
Quality (overexposure/under exposure, rotation, inspiration, expiration)
Tubes
Trachea
Heart border
Hila
Lung markings/vasculature
Bones
Soft tissues
Below the diaphragm
```
74
What is an adequate inspiratory film?
Flattened, inverted diaphragms. Anterior ribs 5-6 inspiratory, 3-4 expiratory
75
What is the most common cause of cardiac arrest in a child?
Hypo ventilation is the most common cause of cardiac arrest in a child.
76
How do you manage circulation in a hemorrhagic trauma?
Give blouses of 10-20mL per kg in hypovoleamia. Blood volume = 80mL/kg.
Control haemorrhage. Give three doses of crystalloids then give blood.
Gain venous access via
Peripheral, intereosseos, cutdown, central venous.
ALWAYS remember BSL in infants.
77
What is the urinary output for an infant, a toddler, an older child and an adult?
Up to 1 year old: 2mL/kg/hour
Toddler 1.5 mL/kg/hr
Older child 1mL/kg/hour
Adult 0.5mL/kg/hr.
78
How do you treat croup?
Salbutamol, oral dexamethasone, and nebulise adrenaline
79
DDR for an upper airway breathing problem
Epiglottitis, Croup, retropharyngeal abscess, and trancheitis.
80
Lower Ddx for lower airways breathing difficulty
Bronchilitis, asthma, pneumonia.
81
What is the main difference between IgE and non IgE mediated food allergy?
IgE mediated food allergy is a short time course <2hours, Can be mild moderate severe, can be unpredictable in severity, prior exposure history not always present. Measured using rast test and skin prick testing.
Non IgE mediated food allergies are usually delayed reactions that occur 2-24 hours post digestion. There is no reliable allergy tests for most non IgE mediated reactions. The best testis usually food elimination and re challenge.
82
What is the grading system used for IgE mediated food reactions?
Mild: localised facial erythema or urticaria/angioedema
Moderate: generalised urticaria /angioedema, or vomiting, or both
Severe: generalised urticaria/angioedema and stridor or wheeze and/or collapse pale floppy
83
When do IgE food mediated allergies present?
80% of IgE mediated food allergies present in the first year of life.
84
Which foods cause 90% of the food mediated IgE reactions?
Peanuts, treenuts, fish, shellfish, eggs, milk, soy, wheat
85
Describe the skin prick allergy test.
The skin prick allergy test is a method of diagnosis of allergies that attempts to provoke a small and controlled allergic response. A microscopic amount of the allergen is introduced by pricking or scratching the patients skin. The patient must not have taken an antihistamine beforehand. There is a positive (histamine) and negative (nothing) control. Skin tests have a high sensitivity but a lower specificity (good negative predictive value but a lower positive predictive value). The size of the reaction is an indication of the likelihood of a reaction, not a prediction of its severity. Skin prick tests are good for monitoring tolerance. They are not a good tool for assessing delayed food reactions.
86
What is a RAST test?
A radio allergo sorbent test is a test that is used to determine what substances a person is allergic to. It is a blood test, which is different to a skin prick test that uses a person's skin to see which substances they will react to. The RAST is a radioimmunoassay test used to detect specific IgE antibodies to suspected or known allergens. IgE is an immune complex associated most with an allergic response. The suspected allergen is bound to an insoluble material and the persons serum is added. If the serum contains antibodies to the allergen, then they will bind to each other. Radio labelled anti human IgE is added. Unbound human anti IgE bodies are washed away. The amount of radioactivity is proportional to the serum IgE to the allergen. RAST has a good positive predictive value at high levels, but has a poor negative predictive value.
87
Which food allergies are most likely to be grown out of?
Milk, egg, soy, wheat
88
Which food allergies are unlikely to be grown out of?
Peanut, tree nut, fish, mollusc and crustaceans.
89
What is FPIES?
Food protein induced protein intolerance syndrome. It is caused by rice, soy, egg, milk, or other solids.presents ~2hours after food with vomiting, lethargy and pallor.
90
What are the cardinal symptoms of childhood cancer?
```
Persistent/recurrent fever without cause
Persistent pain, especially bone pain and headache
A mass
Purpura
Pallor
Strabismus (acute onset)
Changes in coordination and behaviour
```
91
What are the most common childhood heamatological cancers?
Leukaemia is the most common. ALL>AML>AML>CML. CLL almost never.
92
What are the symptoms of ALL?
The symptoms of acute lymphoblastic leukaemia are
aneamia, bruising, fever,
Bone pain
Lymphadenopathy, hepatomegaly and splenomegaly
Malaise, anorexia
93
What are the blood findings on ALL?
There is aneamia (hb 50-60), thrombocytopenia (platelets 10-20,000), high WCC, neutropenia, circulating blasts.
NB:whilst the blood count is usually abnormal, it can be normal.
94
What is ALL?
Acute lymphocytic leukaemia, aka acute lymphoblastic leukaemia is a form of leukaemia characterised by excess lymphoblasts. Malignant lymphoblasts continuously multiply and are overproduced in the bone marrow. ALL causes damage and death by crowding out normal cells in the bone marrow and spreading to other organs. ALL has a peak incidence in children 2-5 years and another peak incidence in old age. It can occur in as little as a few weeks. It has a good chance of cure. A translocation between chromosome 9and 22 (Philadelphia chromosome) occurs in 20% of adult cases and 5% of peads. It is a poor prognostic indicator. Hyperdiploidy is a good prognostic indicator.
95
What is a lymphoblast?
A Lymphoblast Is a cell that has the potential to become a T lymphocyte or a B lymphocyte.
96
What is a myeloblast?
A Myeloblast is a cell that has the potential to become a basophil, a eosinophil, or a neutrophil.
97
Hat is the differential diagnosis for ALL?
ALL could also be:
- ITP
- neutropenias
- aneamia
- infectious mononucleosis
- lymphadenopathy
- arthritis
98
What are the good prognostic factors for ALL?
Age 2-10 yrs, lower WCC, prednisone responses, no CNS disease, hyperploidy, b precursor, t(12:21)
99
What are the bad prognostic factors for ALL?
Infant or age > 10 years, high WCC, CNS disease, Tcell disease, Philadelphia chromosome t(9:22), t(4:11).
100
How is ALL treated?
There is a 1 month induction period with a 98% chance of remission. Then there is a further 5-6 months of heavy chemo, 1.5 years oral 'gentle' maintenance chemo. 7-5 % are 'cured'. To treat ALL invasion of the CNS, there is usually intrathecal (into the arachnoid layer) chemotherapy, however sometimes a patient will receive radiation to the brain.
101
What is acute myeloid leukaemia?
Acute myeloid leukaemia is also known as acute myeloid nous leukaemia, or acute non lymphocytic leukaemia. It is a cancer of the myeloid line of blood cells, characterised by rapid growth of white blood cells that accumulate in the bone marrow and interfere with the production of normal blood cells. AML is the most common acute cancer affecting adults, but also accounts for 20% of childhood leukaemia. It is more serious than ALL. The best results are 60% cure achieved with a more intensive therapy. It has a similar appearance as ALL but a different appearance on bone marrow aspirate. AML requires the most intensive chemo especially high dose cytosine. It has a prolonged marrow aphasia, and inpatient stay. Lymph node swelling is a lot less common in AML than in ALL.
102
Describe the more common brain tumours that occur in children.
There are more in the posterior fossa than adults. There are medullagblastomas, ependyomas, high grade gliomas, low grade gliomas, and brain stem gliomas.
103
What are the side effects of radiation to the brain?
May affect neurologive function. This is worse of they are younger, or worse if supratentorial brain is irradiated.
It may also cause hypopituitarism, affecting growth hormone, pubertal derangement, and thyroid function. Can also be carcinogenic.
104
Describe hodgekins disease.
Hodgkin's disease is a type of lymphoma (a cancer of b or t lymphocytes that typically presents as a solid tumour of lymphoid cells). Hodgekins lymphoma is characterised by the orderly spread from lymph node to another and the development of systemic symptoms with advanced disease. When hodgekins cells are examined microscopically, multinucleated reed sternberg cells are characteristic. The disease affects those in young adulthood (15-35) and those over 55. They typically present with a neck mass, Mediastinal mass, or other adenopathy. Some, but not all, patients will present with fevers, weightloss, and sweats. There is generally good outcomes with chemotherapy and radiotherapy and the great majority are cured.
105
Describe wilds tumour?
Wilms tumour is a cancer of the kidneys that typically occurs in children 1-7 years. They have a flank mass, can be huge, often painless, patient is happy. Px with heamaturia, metastasis usually to the lungs or liver, outlook generally good (surgery, chemo, RT). renal cell carcinoma is very rare in children.
106
Which conditions should you rule out if a child has a fever?
Head injury, meningitis, drugs, encephalitis, hypoglycaemia, space occupying lesion.
107
What is the emergency treatment of seizures?
Clear the airway, lie on the side, give O2, support respiration if apnoiec.
Check for hypoglycaemia. If present, give IV 10% dextrose 5mL/kg, or glucagon 0.5 mg (<5 yr) I'm/IV followed by glucagon or CHo feed.
Diazepam or midazolam
Phenytoin or phenobarbitone.
108
Describe non Hodgkin lymphoma
Non hodgekin lymphoma is a diverse group of heamatological cancers that include any type of lymphoma other than Hodgkin lymphoma. Types of NHL can vary significantly in their severity, from indolent to aggressive. Non Hodgkin's lymphoma includes T lymphoblastic lymphoma in which an older child presents with a mediastinal mass and pleural effusion.
It also includes Burrkitts (B cell) lymphoma and large cell NHL.
109
What are the markers of B cell differentiation?
CD20, CD22' CD19
110
What are the different types of congenital anomalies?
Malformation
Deformation
Disruption
111
What are the different categories of malformations?
Agenesis
Aplasia -absence of cellular proliferation
Hypoplasia: insufficient cellular proliferation
Hyperplasia
Dysplasia
112
What is ITP?
ITP is known as idiopathic autoimmune thrombocytopenia, also known as primary immune thrombocytopenic purpura is defined as an isolated low platelet count with normal bone marrow and the absence of other causes of thrombocytopenia, it causes a characteristic purpurin rash and a tendency to bleed. It can manifest as an acute condition in children that follows an infection and has spontaneous resolution within 2 months. Or it can be chronic and persist over 6 months. ITP is an autoimmune condition with antibodies detectable against several platelet surface antigens.
113
How long does a superficial burn take to heal?
A superficial burn takes approximately 5-14 days to heal
114
What are the changes that occur in a patients hormones after a burn?
There is a decrease in anabolic hormones : growth hormone, thyroxine, and anabolic steroids. There is an increase in catabolic hormones: glucagon, cortisol, catecholamines.
115
Describe fold resuscitation for burns
Hartmanns solution, 2-4 mLs/kg/%BSA burnt in the first 24 hours. Give half in the first 8 hours after burn,MADD maintenance to children <30 kg. reassess urine output 1mL/kg/hour and look for general signs of a dehydration.
Maintenance should have glucose in it.
116
What age does toilet training usually occur?
Ages 2-3
117
What is the relapse rate of nephrotic syndrome in children?
The relapse rate is quite high 70-80%. Peadiatric nephrotic syndrome mostly occurs in Charente <6yrs. Most of these children have minimal change disease.
118
What are the potential complications of nephrotic syndrome?
Infection: urinary loss of IgG, T cell dysfunction, and decreased complement factors. Particularly vulnerable to encapsulated organisms.
Thromboembolic: increased synthesis of clotting factors, loss of coagulation inhibitors.
Cardiovascular
Drug side effects (steroids, cyclophosphamide)
119
What are some of the complications that may arise from chronic renal failure?
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Fluid imbalance -increased BP
Increased urea, K, or PO4
Decreased calcium, Hb
Renal osteodystrophy
Poor weight gain and growth
Cardiovascular complications
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120
What are the causes of hypertension in Peadiatrics?
Renal: glomerularnephropathies, polycystic kidneys, scarring. Obstructive uropathy, Liddle's syndrome
Vascular: renovascular, aortic abnormalities, vadculopathies
Endocrine: adrenal, thyroid, parathyroid, pituitary
Metabolic syndrome
Central: systemic nervous system abnormality, raised ICP, vasomotor centre abnormality
Iatrogenic: drugs, non pharmacological agents, IV fluids.
121
How do you assess a child presenting with hypertension?
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Exclude metabolic syndrome
Test EUC, CMP
Urinanalysis
Doppler us
Fundoscopy
Genetics
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122
What are the live attenuated vaccines currently on the schedule?
Measles, mumps, rubella, varicella, hep B
123
What are the conjugated vaccines?
Conjugated vaccines have a polysaccharide linked to them to act as a carrier protein. this includes HiB, meningococcal, pneumococcal.
124
What is an immunisation adjuvant?
An adjuvant is a substance which enhances the immune response to an antigen. Eg aliminium.
125
Discuss developmental dysplasia of the hip.
It is when individuals are born with dislocation or displacement of the hip that can then affect the bone structure around it. Affects females more than males. Risk factors include family history, breech, first born, packing (oligohydramnios, big baby, small mother), race, being female. Signs include asymmetrical skin creases, asymmetrical leg length, clinical features of feotal packing : plagiocephaly, torticollis, hip dysplasia and postural foot deformities. Check for limited abduction in flexion. Barlow manoeuvre: try to dislocate hip. Ortalanis manoeuvre: try to reposition hip. Image via ultrasound under 6months, over 6 months X-ray. I,ageing is indicated for breech px, fhx, or abnormal signs. Treat with closed reduction (brace in flexion and abduction), open reduction and brace, acetabuloplasty. Harness called a Pavlik harness. The Denis Browne brace is used for older children.
126
Describe transient synovitis.
Transient synovitis is the most common cause of acute hip pain in choldren aged 3-10 years old. Transient synovitis of the hip is a self limiting condition in which here is inflammation of the inner lining (the synovium) of the capsule of the hip joint.it is believed to be caused by a post viral syndrome. It is a diagnosis of exclusion and clears itself in a couple of days. On examination, joint is painful, pain in passive and active movement. May have presence of an antalgic gate.
127
What is Perthe's disease?
Perthe's disease is a vascular necrosis of the femoral head due to an unknown cause resulting in compromise of the blood supply. It occurs in children aged 4-10 years old. They present with an antalgic gait, and limited range of motion, pain may be referred to the medial thigh and knee. Diagnose on X-rays (anterior posterior and frog leg lateral views). May be normal on X-ray at first but changes with time. X rays may show cessation of the epiphyteal growth, subchondral fracture, bone resorption, bone reformation. Can occur bilaterally in 10% of cases. Treat with containment (keeping the femoral head in the acetabulum). acheive containment with braces or with a pelvic osteotomy and femoral osteotomy. Trendelenberg test-> sound side sags.
128
What is a slipped capital femoral epiphytes?
A fracture through the physis (growth plate) which results in slippage of the overlying epiphysis. Most common in obese adolescent males. Occurs in males aged 12-15 years, or females aged 10-13 years. Presents with knee pain, limp, out-toeing. Treat by putting a screw in situ. Doesn't get reduced as reducing increases risk of avascular necrosis ( still at increased risk of developing it). More likely to get osteoarthritis.
129
If someone is presenting with hip pain, what is the most likely diagnosis based on age?
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0-2 developmental dysplasia of the hip
2-5 transient synovitis
5-10 Perthe's disease
10-15 slipped capital femoral epiphysis
Always exclude septic arthritis
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130
What are the clinical features of Kawasaki s disease?
High fever for more than five days, conjunctival injection, polymorphous rash, changes in mucous membranes, cervical lymphadenopathy.
131
How is fat digested?
There is hydrolysis by gastric acid. Bile salts solubilise the fat. Pancreatic enzymes such as lipase and collapse digest the fat. Intact intestinal mucosa is required for absorption. There is diffusion across enterocyte apical membrane. . Reconstitution into chylomicrons wi carrier proteins such as apolipoprotein B. transport via the lymphatic system into systemic circulation. Small chain triglycerides can bypass this system and enter via the portal venous system directly.
132
What are the causes of steatorrhoea?
Pancreatic insufficiency: CF, chronic pancreatitis, Schwachman diamond syndrome, isolated lipase deficiency
Inadequate bile salt action: biliary atresia, cholestasis, end stage liver disease, bacterial overgrowth syndromes, bile salt malabsorption (ileal resection), congenital bile salt malabsorption.
Inadequate absorptive surfaces: coeliac disease, short bowel syndrome, milk protein intolerance, immunodeficiency
Enterocyte defect: abetalipoproteinaemia
Defective lymphatic drainage: intestinal lymohangectasia. Constrictive pericarditis
133
If fat globules are found in steatorrhoea, what do you expect? What about if fat crystals are found?
Fat globules: pancreatic insufficiency, or liver disease.
| Fat crystals: mucosal disease
134
How do you assess watery stool?
You assss watery stool by assessing for reducing substances. Osmotic diarrhoea has positive reducing substances. Secretory diarrhoea has no reducing substances.
135
Sucrose breaks down sucrose into...
Glucose and fructose
136
Lactase breaks down lactose into
Glucose and galactose
137
How do you calculate the osmotic gap in stool fluids?
Serum osmolality (280) - ( 2x Na + K). Osmotic diarrhoea has an is optic gap greater than 100
138
What can cause osmotic diarrhoea?
Monosaccharides and disaccharides (eg lactose intolerance)
Laxatives such as sorbitol or MgCl
Mail digestion of malabsorption of CHO (unabsorbed CHO in the lumen of the large bowel is fermented to cause short chain fatty acids such as butyrate).
139
What's are the two tests for osmotic diarrhoea?
Osmotic gap >100. And osmotic diarrhoea will stop when feeds are stopped.
140
What are the causes of bloody diarrhoea?
Bacterial or parasitic infection
Crohn's disease
Ulcerative colitis
Milk protein intolerance
141
What are the characteristics of humoral (B cell) deficiencies? What are the different types of B cell deficiencies?
B cells deficiencies are deficiencies in IgA, IgG, IgM, and IgE. They are characterised by recurrent bacterial infections by encapsulated organisms, and ears and sinopulmonary (eg. Otitis media with perforation, pneumonia, chronic cough).
It can be caused by x linked agammaglobinaemia (XLA), IgA deficiency, specific antibody deficiency, and common variable immunodeficiency.
142
What is SCIDS?
sCIDS is severe combined immunodeficiency. It affects T cells, B cells and NK cells. SCIDS presents with lymphopenia. It may be x linked or it may be autosomal recessive. SCIDS may have absent or present B and NK cells depending on the defect, and may have an absent thymus on CXR. SCIDS presents with unusual and opportunistic infections such as PCP, fungal, and overwhelming viral EBV, CMV. Presents with FTT, diarrhoea, and rash. The treatment for SCID is bone marrow transplant.
143
Pt presents with recurrent staph, aspergillus, and gingival infections. They also have splenomegaly and lymphadenopathy. What immunodeficiency are they most likely to have?
They have a problem with their neutrophils (phagocytes) eg chronic granulomatous disease. Chronic granulomatous disease is a failure of neutrophils to undergo oxidative burst. They could also have neutropenia, or lypmhocyte adhesion disorders where neutrophils are unable to move into tissues but they still have a high neutrophil count.mthey have an inability to form pus.
