Paediatrics 2 Flashcards

Question

What is the main treatment for utricaria?

Answer 1

Fexofenadine or oral steroids

Answer 2

Anti-leukotrienes such as montelukast Omalizumab, which targets IgE Cyclosporin

Answer 3

Urticaria Itching Angio-oedema, with swelling around lips and eyes Abdominal pain Additional symptoms that indicate anaphylaxis are: Shortness of breath Wheeze Swelling of the larynx, causing stridor Tachycardia Light-headedness Collapse

Answer 4

Intramuscular adrenalin, repeated after 5 minutes if required as it has a short half-life Antihistamines, such as oral chlorphenamine or cetirizine Steroids, usually intravenous hydrocortisone

Answer 5

Measure serum mast tryptase. It stays in the blood for **6 hours**

Answer 6

Prepare the device by removing the safety cap on the non-needle end. There is a blue cap on EpiPen and a yellow cap on Jext. Grip the device in a fist with the needle end pointing downwards. The needle end is orange on EpiPen and black on Jext. Do not put your thumb over the end, because if the device is upside down you will inject your thumb with adrenalin and could risk losing it. Administer the injection by firmly jabbing the device into the outer portion of the mid thigh until the device clicks. This can be done through clothing. EpiPen advise holding it in place for 3 seconds and Jext advise 10 seconds before removing the device. Remove the device and gently massage the area for 10 seconds. Phone an emergency ambulance. A second dose may be given (with a new pen) after 5 minutes if required.

Answer 7

Adrenaline (IM*) 1:1000

Answer 8

- Children older than 12: 500 micrograms - Children aged 6-12: 300 micrograms - Children aged 6 months to 6 years: 150 micrograms

Answer 9

It is a systemic, medium-sized vessel vasculitis. It affects young children typically under 5 year. There is no clear cause or trigger It is more common among Asian children particularly Japanese and Korean and more common in boys

Answer 10

- A persistent high fever for more than **5 days**. - A widespread **erythematous maculopapular rash and desquamation** on the palms and soles of the feet

Answer 11

Strawberry tongue (red tongue with large papillae) Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis

Answer 12

- FBC can show anaemia and Leukocytosis and thrombocytosis - LFT can show hypoalbuminemia and elevated liver enzymes - Inflammatory markers (ESR) are raised - Urinalysis can show raised white blood cells without infection - Echocardiogram can demonstrate coronary artery pathology

Answer 13

- Acute phase: the rash with the fever and lymphadenopathy 1-2 weeks - Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks. - Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.

Answer 14

- High dose aspirin to reduce the risk of thrombosis - IV immunoglobulins to reduce the risk of coronary artery aneurysms

Answer 15

Because of the risk of Reye's sydrome

Answer 16

10-12 days

Answer 17

Fever, coryzal and conjunctivitis

Answer 18

Koplik spots, they are greyish white spots on the mouth. They appear 2 days after the fever if you see them you can diagnose them (pathognomonic)

Answer 19

The rash starts on the face, classically behind the ears, 3 – 5 days after the fever. It then spreads to the rest of the body. The rash is an erythematous, macular rash with flat lesions.

Answer 20

Measles is self resolving after 7 – 10 days of symptoms. Children should be isolated until 4 days after their symptoms resolve. Measles is a notifiable disease and all cases need to be reported to public health. 30% of patients with measles develop a complication.

Answer 21

- Pneumonia - Diarrhoea - Dehydration - Encephalitis - Meningitis - Hearing loss - Vision loss - Death

Answer 22

2 weeks after exposure

Answer 23

It presents with a milder erythematous macular rash compared with measles. The rash starts on the face and spreads to the rest of the body. The rash classically lasts 3 days. It can be associated with a mild fever, joint pain and a sore throat. Patients often have enlarged lymph nodes (lymphadenopathy) behind the ears and at the back of the neck.

Answer 24

Management is supportive and the condition is self limiting. Rubella is a notifiable disease and all cases need to be reported to public health. Children should stay off school for at least 5 days after the rash appears. Children should avoid pregnant women.

Answer 25

- Thrombocytopenia - Encephalitis It is also dangerous in pregnancy and can lead to congenital rubella syndrome: - Deafness - Blindness - Congenital heart disease

Answer 26

Parvovirus B19

Answer 27

Parvovirus infection starts with mild fever, coryza and non-specific viral symptoms such as muscle aches and lethargy. After 2 – 5 days the rash appears quite rapidly as a diffuse bright red rash on both cheeks, as though they have “slapped cheeks”. A few days later a reticular mildly erythematous rash affecting the trunk and limbs appears that can be raised and itchy. Reticular means net-like.

Answer 28

- Immunocompromised patients - Pregnant women - Sickle cell - Thalassaemia - Hereditary spherocytosis Patients with haematological condition will require FBC checking

Answer 29

- Aplastic anaemia - Encephalitis - Fetal death - Hepatitis - Myocarditis - Nephritis

Answer 30

Varicella zoster virus

Answer 31

Chickenpox is characterised by widespread, erythematous, raised, vesicular (fluid filled), blistering lesions. The rash usually starts on the trunk or face and spreads outwards affecting the whole body over 2 – 5 days. Eventually the lesions scab over, at which point they stop being contagious. Other symptoms: Fever is often the first symptom Itch General fatigue and malaise

Answer 32

Bacterial superinfection Dehydration Conjunctival lesions Pneumonia Encephalitis (presenting as ataxia)

Answer 33

Usually mild. Smptoms often develop gradually, beginning with a sore throat and fever. In severe cases, a grey or white patch develops in the throat, which can block the airway, and **create a barking cough similar to what is observed in** ***croup.*** May involve lymph node swelling, and can involve skin, eyes and genitals

Answer 34

A condition caused by a type of S.aureus bacteria that produces epidermolytic toxins These toxins are **protease enzymes** that break down proteins that hold skin cells together

Answer 35

Children under age of 5 as older children have developed immunity to the toxins

Answer 36

- Patches of erythema of the skin, this causes the skin to look thin and wrinkled This is followed by the formation of fluid filled blisters called bullae which burst and leave sore skin below

Answer 37

**Nikolsky sign** is where very gentle rubbing of the skin causes it to peel away.

Answer 38

Iv antibiotics

Answer 39

An upper respiratory tract infection caused by **Bordetella pertussis** (a gram neg). It is called whooping cough because the coughing fits are so severe that the child can't take in any air between coughs

Answer 40

More severe coughing fits start after a week or more. These involve sudden and recurring attacks of coughing with cough free periods in between. This is described as a paroxysmal cough. Coughing fits are severe and keep building until the patient is completely out of breath. Patient typically produces a large, loud inspiratory whoop when the coughing ends. Patients can cough so hard they faint, vomit or even develop a pneumothorax. Bear in the mind that not all patients will “whoop” and infants with pertussis may present with apnoeas rather than a cough.

Answer 41

Pertussis typically starts with mild coryzal symptoms, a low grade fever and possibly a mild dry cough

Answer 42

A nasal swab with PCR testing or bacterial culture within 2-3 weeks if symptoms Can be tested for the **anti-pertussis toxin immunoglobulin G.** This is tested for in the oral fluid of children aged 5 to 16 and in the blood of those aged over 17.

Answer 43

Macrolide antibiotics such as **azithromycin, erythromycin and clarithromycin** can be beneficial in the early stages (within the first 21 days) or vulnerable patients. Co-trimoxazole is an alternative to macrolides. Close contacts with an infected patient are given prophylactic antibiotics if they are in a vulnerable group, for example pregnant women, unvaccinated infants or healthcare workers that have contact with children or pregnant women

Answer 44

The symptoms typically resolve within 8 weeks, however they can last several months. It is also known as the “100-day cough” due to the potential long duration of the cough. A key complication of whooping cough is bronchiectasis.

Answer 45

Poliomyelitis an acute clinical disease caused by a polio virus It remains endemic in Afghanistan and Pakistan

Answer 46

Presentation: Incubation 7–10d. Flu-like prodrome in ~25%. Pre-paralytic stage: fever, increased HR, headache, vomiting, neck stiffness, tremor, limb pain. ~1 in 200 progress to paralytic stage: LMN/bulbar signs ± respiratory failure

Answer 47

Mycobacterium tuberculosis. It is an **acid-fast bacilli** and will be seen using a Zeihl-neelson stain and turn **bright red**

Answer 48

Macrophages struggle to clear TB due to its waxy mycolic acid capsule. Instead of being broken down and cleared, A **focal caseating granuloma** typically forms in the lower lobe known as a **Ghon focus.** The Ghon focus can then spread to the **Hilar Lymph nodes** in the lungs, which together form a **ghon complex** These ghon complexes can under go **fibrosis and calcification**, leading to the appearance of **ranke complexes** on xray

Answer 49

- occurs after primary infection, immune system encapsulates sites of infection and stop the progression of the disease. - Patients remain asymptomatic and the bacteria remains dormant, resulting in negative sputum cultures but a positive Mantoux test. - These patients are not infectious. - However, if patients are immunocompromised, the disease can progress or reactivate at a later stage to become active TB.

Answer 50

Immunocompromised patients may develop secondary TB when latent TB reactivates - Patients are infectious. - Reactivation typically occurs in the lung apex where pO2 is highest, as mycobacteria are aerobic. bacteria can spread locally, to form caseating granulomata, or systemically (miliary TB).

Answer 51

Miliary TB - Where immune system cannot control the infection and it becomes disseminated Extrapulmonary TB - where TB infects other areas

Answer 52

- Close contact with active TB - Immigrants from areas with high prevalence - Immunocompromised - Malnutrition, homelessness, drug users, smokers and alcoholics

Answer 53

Involves an intradermal injection of **live attenuated Mycobacterium bovis bacteria (a close relative of M. tuberculosis that does not cause disease in humans). The vaccine protects against severe and complicated TB but less against **pulmonary TB**

Answer 54

he Mantoux test and only given the vaccine if this test is negative. They are also assessed for the possibility of immunosuppression and HIV due to the risks related to a live vaccine.

Answer 55

Cough Haemoptysis (coughing up blood) Lethargy Fever or night sweats Weight loss Lymphadenopathy Erythema nodosum (tender, red nodules on the shins caused by inflammation of the subcutaneous fat) Spinal pain in spinal tuberculosis (also known as Pott’s disease of the spine)

Answer 56

- Mantoux test - Interferon-gamma release assay

Answer 57

he Mantoux test involves injecting tuberculin into the intradermal space on the forearm. Tuberculin is a collection of tuberculosis proteins isolated from the bacteria. It does not contain any live bacteria. The infection creates a bleb under the skin. After 72 hours, the test is “read”. This involves measuring the induration of the skin at the injection site. An induration of 5mm or more is considered a positive result.

Answer 58

Primary tuberculosis may show **patchy consolidation, pleural effusions and hilar lymphadenopathy** Reactivated tuberculosis may show patchy or nodular consolidation with cavitation (gas-filled spaces), typically in the upper zones. Disseminated miliary tuberculosis gives an appearance of **millet seeds** uniformly distributed across the lung fields.

Answer 59

Isoniazid and rifampicin for 3 months or Isoniazid for 6 months

Answer 60

R – Rifampicin for 6 months I – Isoniazid for 6 months P – Pyrazinamide for 2 months E – Ethambutol for 2 months

Answer 61

can cause red/orange discolouration of secretions, such as urine and tears. It is a potent inducer of the cytochrome P450 enzymes and reduces the effects of drugs metabolised by this system, such as the combined contraceptive pill.

Answer 62

can cause peripheral neuropathy. Pyridoxine (vitamin B6) is co-prescribed to reduce the risk.

Answer 63

Pyrazinamide can cause hyperuricaemia (high uric acid levels), resulting in gout and kidney stones.

Answer 64

can cause colour blindness and reduced visual acuity.

Answer 65

The virus enters and destroys the CD4 T helper cells. Uses reverse transcriptase enzyme to transcribe a piece of complimentary proviral DNA, to make a double strand with the original RNA strand. This double stranded DNA then pops itself into the DNA of the cell (via integrase enzyme.) , ready to be transcribed into another virus cell, when the old immune cell becomes activated and starts trying to transcribe proteins for the immune response. (sneaky)

Answer 66

Sexual abuse/unprotected sex - Mother to child at **any stage of pregnancy, birth or breastfeeding** (vertical transmission) Mucous membrane, blood or open wound exposure to infected blood or bodily fluids. This could be through sharing needles, needle-stick injuries or blood splashed in an eye.

Answer 67

Will be determined by the viral load of the mother Normal vaginal is recommended for women with viral load <50 copies/ml Caesarean sections are considered in patients with > 50 copies copies / ml and in all women with > 400 copies / ml IV zidovudine should be given during the caesarean if the viral load is unknown or there are > 10000 copies / ml

Answer 68

Low risk babies, where mums viral load is < 50 copies per ml, should be given zidovudine for 4 weeks High risk babies, where mums viral load is > 50 copies / ml, should be given zidovudine, lamivudine and nevirapine for 4 weeks

Answer 69

Twice: **HIV viral load test at 3 months.** If this is negative, the child has not contracted HIV during birth and will not develop HIV unless they have further exposure. **HIV antibody test at 24 months**. This is to assess whether they have contracted HIV since their 3 month viral load, for example through breast feeding. If the 3 month test is negative and they are not breastfed, this should be negative. Note that the antibody test can be **positive in infants who do not have HIV for up to 18 months of age.** This is due to maternal antibodies that have crossed the placenta during pregnancy.

Answer 70

ART to suppress the HIV ormal childhood vaccines, avoiding or delaying live vaccines if severely immunosuppressed. Prophylactic co-trimoxazole (Septrin) for children with low CD4 counts, to protect against pneumocystis jirovecii pneumonia (PCP) Treatment of opportunistic infections The aim of antiretroviral therapy (ART) is to achieve a normal CD4 count and undetectable viral load

Answer 71

Inflammation of the meninges. They make up the lining of the spinal cord and brain. The inflammation is usually due to a bacterial or viral infection

Answer 72

Neisseria meningitidis a gram-negative diplococcus bacteria and streptococcus pneumoniae

Answer 73

Group B strep which is contracted form birth

Answer 74

Fever, neck stiffness, vomiting, headache, photophobia, altered consciousness and seizures. Can also present with non-blanching rash in meningococcal septicaemia

Answer 75

. This rash indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages.

Answer 76

- Hypotonia - Poor feeding - Lethargy - Hypothermia - Bulging fontanelle

Answer 77

- Under 1 month presenting with fever - 1 to 3 months with fever and are unwell - Under 1 year with unexplained fever and other features of serious illness

Answer 78

Kernig's test Brudzinski’s test

Answer 79

Involves lying the patient on their back, flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed at 90 degrees. This creates a slight stretch in the meninges. Where there is meningitis it will produce spinal pain or resistance to movement.

Answer 80

Involves lying the patient flat on their back and gently using your hands to lift their head and neck off the bed and flex their chin to their chest. In a positive test this causes the patient to involuntarily flex their hips and knees.

Answer 81

If they have suspected meningitis and a **non blanching rash** give IM injection of benzylpenicillin.

Answer 82

Ideally a blood culture and a lumbar puncture for cerebrospinal fluid (CSF) should be performed prior to starting antibiotics, however if the patient is acutely unwell antibiotics should not be delayed. Send blood tests for meningococcal PCR if meningococcal disease is suspected. This tests directly for the meningococcal DNA. It can give a result quicker than blood culture depending on local services, and will still be positive after the bacteria has been treated with antibiotics.

Answer 83

Under 3 months: give Cefotaxime plus amoxicillin ( the amoxicillin is to cover listeria) Above 3 months: Ceftriaxone Vancomycin should be added if there is a risk of Penicillin resistant pneumococcal infection

Answer 84

Dexamethasone given 4 times daily for 4 days

Answer 85

Single dose of ciprofloxacin

Answer 86

herpes simplex virus (HSV), enterovirus and varicella zoster virus (VZV)

Answer 87

Appearance= cloudy in bacterial Protein= High in bacterial Glucose= Low in bacterial White cells= Neutrophils in bacterial White cells= Lymphocytes in viral

Answer 88

It means inflammation of the brain. It can be the result of infective or non-infective causes. Non-infective causes are autoimmune

Answer 89

HSV-1 from cold sores

Answer 90

HSV-2 contracted from genital herpes at birth

Answer 91

Varicella zoster virus (VZV) associated with chickenpox, cytomegalovirus associated with immunodeficiency, Epstein-Barr virus associated with infectious mononucleosis, enterovirus, adenovirus and influenza virus

Answer 92

- Altered consciousness - Altered cognition - Unusual behaviour - Acute onset of focal neurological symptoms - Acute onset of focal seizures - Fever

Answer 93

- Lumbar puncture sending CSF for **viral PCR testing** - CT scan if LP contraindicated - MRI after LP - Swabs of other areas can help establish the causative organism, such as throat and vesicle swabs - HIV testing is recommended in all patients with encephalitis

Answer 94

- GCS below 9 - Haemodynamically unstable - Active seizures - Post-ictal

Answer 95

- Acyclovir treats herpes simplex virus (HSV) and varicella zoster virus (VZV) - Ganciclovir treat cytomegalovirus (CMV)

Answer 96

Lasting fatigue and prolonged recovery Change in personality or mood Changes to memory and cognition Learning disability Headaches Chronic pain Movement disorders Sensory disturbance Seizures Hormonal imbalance

Answer 97

- Bullous - Non-bullous

Answer 98

occurs around the nose or mouth. The exudate from the lesions dries to form a “golden crust”. They are often unsightly but do not usually cause systemic symptom

Answer 99

- Topical fusidic acid can be used to treat localised non-bullous impetigo - antiseptic cream (hydrogen peroxide 1% cream) first line rather than antibiotics for localised non-bullous impetigo.

Answer 100

- Always caused by S.aureus - They produce epidermolysis toxins that break down proteins that hold skin cells together

Answer 101

Neonates and children under 2

Answer 102

Can cause severe infection called staphylococcus scalded skin syndrome - Treat with flucloxacillin

Answer 103

Cellulitis if the infection gets deeper in the skin Sepsis Scarring Post streptococcal glomerulonephritis Staphylococcus scalded skin syndrome Scarlet fever

Answer 104

Nappy rash is skin inflammation, mainly due to a reaction of the skin to urine and poo. Switching to highly absorbent nappies (disposable gel matrix nappies) Change the nappy and clean the skin as soon as possible after wetting or soiling Use water or gentle alcohol free products for cleaning the nappy area Use a thin layer of barrier cream Ensure the nappy area is dry before replacing the nappy Maximise time not wearing a nappy

Answer 105

Rash extending into the skin folds Larger red macules Well demarcated scaly border Circular pattern to the rash spreading outwards, similar to ringworm **Satellite lesions,** which are small similar patches of rash or pustules near the main rash Check for oral thrush with a white coating on the tongue, as this is likely to indicate a fungal infection in the nappy area.

Answer 106

- Cease the use of a barrier cream until the candida has settled Canesten® cream has been used to treat fungal nappy rash for 25 years. Active ingredient **clotrimazole** cloh trimm azz oll

Answer 107

A severe systemic reaction to staphylococcal exotoxin It is produced by S.aureus and group A streptococci

Answer 108

Leaving tampons in too long, female barrier contraceptives, any break in the skin, nasal packing for nosebleeds

Answer 109

* fever over 39° C * hypotension * diffuse erythematous, macular rash.

Answer 110

This is an emergency, ABCDE approach. Oxygen IV Broad spec Abx + IV IG IV Fluids Surgical debridement *Antibiotics often include a third-generation cephalosporin (such as ceftriaxone) together with clindamycin, which acts on the bacterial ribosome to switch off toxin production. Intravenous immunoglobulin may be given to neutralize the circulating toxin.*

Answer 111

Associated with a group A streptococcus infection usually tonsillitis

Answer 112

Caused by an exotoxin It causes a red-pink blotchy macular rash with rough **sandpaper skin** that starts on the trunk and spreads outwards

Answer 113

Fever Lethargy Flushed face Sore throat **Strawberry tongue*8 Cervical lymphadenopathy

Answer 114

- Pen V for 10 days It is a notifiable disease and should be reported to public health. Children should be kept off school for 24 hours after starting antibiotics

Answer 115

Post-streptococcal glomerulonephritis Acute rheumatic fever

Answer 116

Coxsackie A virus

Answer 117

Incubation period of 3-5 days viral upper respiratory tract symptoms such as tiredness, sore throat, dry cough and raised temperature. After 1 – 2 days small mouth ulcers appear, followed by blistering red spots across the body.

Answer 118

Dehydration Bacterial superinfection Encephalitis

Answer 119

Salmon patch - Flat red or pink patches on a baby's eyelids, neck or forehead at birth. They're the most common type of vascular birthmark and occur in around half of all babies. Infantile Haemangioma - strawberry marks, are raised marks on the skin that are usually red, occur in 5% of birth, more common in girls. Rapidly increase in size for the first six months before shrinking

Answer 120

Port wine stain - discoloration of the human skin caused by a vascular anomaly (a capillary malformation in the skin). *Mikhail Gorbachev famously had one on his forehead* Café au lait spots, = flat, hyperpigmented birthmarks. They are caused by a collection of pigment-producing melanocytes in the epidermis of the skin. **Multiple of these birth can be a sign of neurofibromatosis type 1**

Answer 121

Mongolian spots - More common in darker-skinned people and usually occur over the lower back or buttocks. ***now called congenital dermal melanocytosis*** congenital melanocytic naevi - normal moles

Answer 122

Children have almost no immunity to infections

Answer 123

Persistent severe diarrhoea Failure to thrive Opportunistic infections that are more frequent or severe than in healthy children, for example severe and later fatal chickenpox, Pneumocystis jiroveci pneumonia and cytomegalovirus Unwell after live vaccinations such as the BCG, MMR and nasal flu vaccine

Answer 124

- More than 50% of cases are caused by a mutations in the common gamma chain on the X chromosome that codes for interleukin receptors on T and B cells. This has X-linked recessive inheritance. - JAC3 gene mutations - Mutations leading to adenosine deaminase deficiency

Answer 125

Omenn syndrome is a rare cause of SCID. It is the result of a mutation in the recombination-activating gene (RAG 1 or RAG 2) that codes for important proteins in T and B cells. It has autosomal recessive inheritance. The syndrome is caused by abnormally functioning and deregulated T cells that attack the tissues in the fetus or neonate. This leads the classic features of Omenn syndrome: A red, scaly, dry rash (erythroderma) Hair loss (alopecia) Diarrhoea Failure to thrive Lymphadenopathy Hepatosplenomegaly

Answer 126

A child displaying slow development in all domains

Answer 127

- Down's syndrome - Fragile X - Fetal alcohol syndrome - Rett syndrome - Metabolic disorders

Answer 128

- Cerebral palsy - Ataxia - Myopathy - Spina bifida - Visual impairment

Answer 129

- Dyspraxia - Cerebral palsy - Muscular dystrophy - Visual impairment - Congenital ataxia

Answer 130

- Specific social circumstances, for example exposure to multiple languages or siblings that do all the talking - Hearing impairment - Learning disability - Neglect - Autism - Cerebral palsy

Answer 131

4 months: Support of their own head 6 months: They can maintain a sitting position but they don't always have balance 9 months: Sit unsupported, start crawling, they can stand on their legs and bounce when supported 12 months: They can begin **cruising** walking whilst holding on to furniture

Answer 132

15 months: Walk unaided. 18 months: Squat and pick things up from the floor. 2 years: Run. Kick a ball. 3 years: Climb stairs one foot at a time. Stand on one leg for a few seconds. Ride a tricycle. 4 years: Hop. Climb and descend stairs like an adult.

Answer 133

8 weeks: Fixes their eyes on an object 30 centimetres in front of them and makes an attempt to follow it. They show a preference for a face rather than an inanimate object. 6 months: Palmar grasp of objects (wraps thumb and fingers around the object).

Answer 134

9 months: Scissor grasp of objects (squashes it between thumb and forefinger). 12 months: Pincer grasp (with the tip of the thumb and forefinger). 14-18 months: They can clumsily use a spoon to bring food from a bowl to their mouth.

Answer 135

3 months: Cooing noises 6 months: making noises with consonants 9 months: Babies sound more like talking but not saying any words 12 months: Says single words in context, e.g. “Dad-da” or “Hi”

Answer 136

18 months: Has around 5 – 10 words 2 years: Combines 2 words. Around 50+ words total. 2.5 years: Combines 3 – 4 words 3 years: Using basic sentences 4 years: Tells stories

Answer 137

3 months: Recognises parents and familiar voices and gets comfort from these 6 months: Responds to tone of voice 9 months: Listens to speech 12 months: Follows very simple instructions

Answer 138

18 months: 1 key word, for example “show me the spoon” 2 years: 2 key words, for example “show me the spoon and the cup” 3 years: 3 key words, for example “put the spoon under the step” 4 years: 4 key words, for example “put the red spoon under the step” 18 months: Understands nouns, for example “show me the spoon” 2 years: Understands verbs, for example “show me what you eat with” 2.5 years: Understands propositions (plan of action), for example “put the spoon on / under the step” 3 years: Understands adjectives, for example “show me the red brick” and “which one of these is bigger?” 4 years: Follows complex instructions, for example “pick the spoon up, put it under the carpet and go to mummy”

Answer 139

6 weeks: Smiles 3 months: Communicates pleasure 6 months: Curious and engaged with people 9 months: They become cautious and apprehensive with strangers 12 months: Engages with others by pointing and handing objects. Waves bye bye. Claps hands.

Answer 140

18 months: Imitates activities such as using a phone 2 years: Extends interest to others beyond parents, such as waving to strangers. Plays next to but not necessarily with other children (parallel play). Usually dry by day. 3 years: They will seek out other children and plays with them. Bowel control. 4 years: Has best friend. Dry by night. Dresses self. Imaginative play.

Answer 141

- Not able to hold an object at 5 months - Not sitting unsupported at 12 months - Not standing independently at 18 months - Not walking at 2 years - Not running at 2.5 years - No words at 18 months - No interest in others at 18 months

Answer 142

A type of seizure that occurs in children with a high fever

Answer 143

6 months to 5 years

Answer 144

Simple febrile convulsions are generalised, tonic clonic seizures. They last less than 15 minutes and only occur once during a single febrile illness.

Answer 145

as complex when they consist of partial or focal seizures, last more than 15 minutes or occur multiple times during the same febrile illness.

Answer 146

Rule out differentials: - pilepsy - Meningitis, encephalitis or another neurological infection such as cerebral malaria - Intracranial space occupying lesions, for example brain tumours or intracranial haemorrhage - Syncopal episode - Electrolyte abnormalities - Trauma (always think about non accidental injury)

Answer 147

- Stay with the child - Put the child in a safe place, for example on a carpeted floor with a pillow under their head - Place them in the recovery position and away from potential sources of injury - Don’t put anything in their mouth - Call an ambulance if the seizure lasts more than 5 minutes

Answer 148

1.8% for the general population 2-7.5% after a simple febrile convulsion 10-20% after a complex febrile convulsion

Answer 149

A paroxysmal alteration of neurological function as a result of excessive hypersynchronous discharge of neurons within the brain

Answer 150

A neurological disorder characterised by an increased tendency to have recurrent seizures that are idiopathic and unprovoked. (>2 episodes more than 24hrs apart)

Answer 151

VITAMIN DE - Vascular - Infection - Trauma - Autoimmune- SLE - Metabolic - Idiopathic - Neoplasms - Dementia and drugs (cocaine) - Eclampsia

Answer 152

- Genetic - Structural - Metabolic- visible neurological abnormalities that predispose to seizures (e.g. chronic cerebrovascular disease, congenital malformation) - Immune - Infectious- a chronic one e.g HIV - Unknown

Answer 153

- Clusters of neurons in the brain become temporarily impaired and start sending put lots of excitatory signals (said to be paroxysmal which means rapid onset). Happen either due to too much excitation glutamate or nor enough inhibition GABA - It is often noticed by obvious outward signs like jerking, moving and losing consciousness but can also be subjective and only noticed by the person experiencing it like fears or strange smells

Answer 154

- **Generalised**:when both hemispheres are affected **always a loss of consciousness** - **Focal** : when the affected area is limited to one half of the brain or sometimes even smaller like a single lobe can progress to bilateral

Answer 155

- Tonic - Atonic - Clonic - Tonic-clonic - Myoclonic - Absence

Answer 156

Simple (without impaired awareness) Complex (with impaired awareness)

Answer 157

- **Prodromal phase:** - Confusion, irritability or mood disturbances - **Early-ictal phase:** - Aura: warning felt before a seizure. These can include sensory, cognitive, emotional or behaviour changes. - **Ictal phase:** - Will vary depending on seizure type - **Post-ictal phase:** - Confused, drowsy and irritable during recovery

Answer 158

- Tonic seizure: the muscles become stiff and flexed which will cause the patients to **fall backwards** - Clonic seizures: violent muscle contractions (convulsions) Tonic-clonic: there is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking episodes). Typically the tonic phase comes before the clonic phase. (tongue biting, incontinence, groaning and irregular breathing. After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or depressed.

Answer 159

First line: **sodium valproate** Second line: **Lamotrigine** or **carbamazepine**

Answer 160

Known as **drop attacks**. This is where the muscles suddenly relax and become floppy which can cause the patient to fall **usually forward**. They don't usually last longer than 3 minuets. They typically begin in childhood. They may be indicative of **Lennox-Gastaut syndrome**.

Answer 161

First line: **sodium valproate** Second line: **Lamotrigine**

Answer 162

- They present as sudden brief muscle contractions like a sudden jump. The patient usually remains awake during the episode. - They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy.

Answer 163

First line: sodium valproate Other options: lamotrigine, levetiracetam or topiramate

Answer 164

- Impaired awareness or responsiveness. Patient becomes blank and stares into space before returning to normal. Motor abnormalities are either absent or very minor e.g. **eyelid flutters or repetitive lip smacking**. - Common in children. Most patients (> 90%) stop having absence seizures as they get older.

Answer 165

First line: sodium valproate or ethosuximide

Answer 166

Known as **West syndrome**. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free.

Answer 167

Prednisolone Vigabatrin

Answer 168

- No loss of consciousness - The patient is aware and awake - Will have uncontrollable muscle jerking

Answer 169

- There is loss of consciousness - Patient is unaware

Answer 170

Temporal lobe

Answer 171

They affect hearing, speech, memory and emotions: - Hallucinations - Memory flashbacks - Déjà vu - Doing strange things on autopilot Can also include audio symptoms such as buzzing, ringing and vertigo

Answer 172

- Motor symptoms: pelvic thrusting, bicycling and tonic posturing - Bizarre behaviour - Vocalisations - Sexual automatisms

Answer 173

- Paraesthesia - Visual hallucinations - Visual illusions More subjective and difficult to diagnose than other areas

Answer 174

- Visual hallucinations - Transient blindness - Rapid and forced blinking - Movement of head or eyes to the opposite side

Answer 175

- Must have had 2 or more seizures more than 24 hours apart - MRI/CT: examine the hippocampus look for underlying cause - EEG: 3H2 wave absence - Bloods: rule out metabolic/infection

Answer 176

Opposite to generalised: First line: **Carbamazepine** Second line: **sodium valproate**

Answer 177

It works by increasing the activity of GABA which has a relaxing effect on the brain: - **Teratogenic don't give to females of child bearing age** - Liver damage - Hair loss - Tremor

Answer 178

- **Carbamazepine** - Sodium channel blocker; prevents repetitive and sustained firing of action potentials. - Agranulocytosis - Aplastic anaemia - Induces the P450 system so there are many drug interactions

Answer 179

**Status Epilepticus**

Answer 180

ABCDE Give IV **lorazepam 4mg** and repeat 10 minuets after if it doesn't work If seizure persist then give IV **phenobarbital or phenytoin**

Answer 181

Primary - also known as genetic or idiopathic epilepsy occur in an otherwise normal person and are due to a genetic predisposition to seizures. Secondary - due to an underlying abnormality of the brain structure or chemistry formerly called symptomatic epilepsy

Answer 182

hese spasms often manifest as sudden, jerking movements of the arms, legs, or trunk. - arms going out and grimacing They can occur in clusters, with each spasm lasting only a few seconds, but they can happen many times a day. Infantile spasms can be challenging to diagnose because the spasms themselves may not seem significant at first glance and can be mistaken for normal infant movements. However, they tend to occur in specific patterns, such as upon waking or when the infant is falling asleep. Additionally, they may be associated with developmental delays or regression known as/seen in West Syndrome

Answer 183

Infantile spasms: These are brief, sudden, and symmetric muscle contractions, typically involving the neck, trunk, and limbs. Hypsarrhythmia: This refers to a specific pattern seen on electroencephalogram (EEG) recordings. Hypsarrhythmia is often present in infants with West syndrome, but it's not exclusive to this condition. Developmental regression or delay Treat with: Vigabatrin and prednisolone Eyes will always be open

Answer 184

A. **Restriction of energy intake relative to requirements**, leading to a **significant low body weight** in the context of the age, sex, developmental trajectory, and physical health (less than minimally normal/expected) B. **Intense fear of gaining weight** or becoming fat or persistent behaviour that interferes with weight gain. C. **Disturbed by one’s body weight or shape,** self-worth influenced by body weight or shape, or persistent lack of recognition of seriousness of low bodyweight.

Answer 185

Social pressure Perfectionist character traits Reversing or halting effects of puberty Some genetic links Depression may be a trigger for binges. Low self-esteem Occupation and interest (e.g. ballet dancers) Anxiety disorders Past or present events: life difficulties sexual abuse physical illness upsetting events - a death or the break-up of a relationship important events - marriage or leaving home.

Answer 186

do you make yourself Sick because you're uncomfortably full? do you worry that you’ve lost Control over how much you eat? have you recently lost more than 6 kilograms (about One stone) in three months? do you believe you’re Fat when others say you’re thin? would you say that Food dominates your life?

Answer 187

Dry skin Lanugo hair - ”peach fuzz” hair on face and trunk Orange skin and palms Cold hands and feet Bradycardia Drop in BP on standing - or **increased fainting** Oedema Week proximal muscles - squat test

Answer 188

CBT Interpersonal therapy Food diary and regular eating programme – re-establish control of diet, address underlying abnormal cognitions SSRIs – best one to use is fluoxetine

Answer 189

the people have a normal body weight, that tends to flucuate Condition involves binge eating, followed by Purging - **inducing vomiting or taking laxatives to prevent the calories being absorbed.**

Answer 190

Features of bulimia nervosa: Alkalosis, due to vomiting hydrochloric acid from the stomach Hypokalaemia Erosion of teeth Swollen salivary glands Mouth ulcers Gastro-oesophageal reflux and irritation Calluses on the knuckles where they have been scraped across the teeth. This is called Russell’s sign. *Look out for the teenage girl with a normal body weight that presents with swelling to the face or under the jaw (salivary glands), calluses on the knuckles and alkalosis on a blood gas. The presenting complaint may be abdominal pain or reflux.*

Answer 191

9 months - 3 years Separation from caregivers, sudden movements, loud noised 3-6 years, animals, dark, monsters 6-12 performance anxiety 12-18 - social anxiety 18 and above - death, illness

Answer 192

Separation aneixty Symptoms must persist for more than 4 weeks Having an ill parent can make it worse

Answer 193

Act with intent to hurt self Includes cutting, burning with ice, hitting self and overdose No intention to kill self Associated with suicidal ideas therefore check May want to release tension, make self feel, others to see distress, subcultural

Answer 194

Act with intent to kill self Includes overdose, attempted hanging, Intention includes desire to be dead Importance to assess severity and whether ongoing

Answer 195

Significant weight Loss **a BMI of less than 70% of the median for your age** Resting bradycardia < 50 bpm Postural tachycardia > 35 bp Postural drop in systolic BP > 20 Hypothermia < 35.5 degrees Severe Abdominal pain **Escalating parental Concern** - generally very good guide – usually had months of struggling before presenting to hospital and they have witnessed a significant deterioration in functioning prompting presentation to medical services The Rapidity of the weight loss is as important as its degree in determining risk

Answer 196

Loss of cardiac muscle and impaired cardiac reserve Starvation causes loss of cardiac muscle as well as skeletal muscle A weakened atrophied heart also poses a risk during refeeding due to the risk of precipitation of heart failure and even death

Answer 197

**medical complications that result from fluid and electrolyte shifts as a result of aggressive nutritional rehabilitation** Metabolism in the cells and organs dramatically slows during prolonged periods of malnutrition. As the starved cells start to process glucose, protein and fats again they use up magnesium, potassium and phosphorus. This leads to: Hypomagnesaemia Hypokalaemia Hypophosphataemia These patients are also at risk of cardiac arrhythmias, heart failure and fluid overload.

Answer 198

Osteroporposis and increased risk of fractures - *as period stops so less ostrogen circualing that promotes bone health* Growth stunting and pubertal delay Neurocognitive Superior mesenteric artery syndrome

Answer 199

Cryptorchidism

Answer 200

- FH - Low birth weight - Small for gestational age - Prematurity - Maternal smoking

Answer 201

most cases the testes will descend in the first 3 – 6 months. If they have not descended by 6 months they should be seen by a paediatric urologist. Orchidopexy (surgical correction of undescended testes) should be carried out between 6 and 12 months of age.

Answer 202

- Testicular torsion - Infertility - Testicular cancer

Answer 203

It is the twisting of the spermatic cord with rotation of the tentacle (was autocorrect but thought it's funny, I'm getting really bored)

Answer 204

- Young age - Bell clapper deformity (what a great name this is for a deformity of the testicle). It's when the testicle is high riding and it's horizontal - Cryptorchidism - Trauma

Answer 205

- Abnormal lie - Prehn's negative pain is not relieved on lifting the ipsilateral testicle - Absent cremasteric reflex

Answer 206

- Awful debilitating pain imagine what it would feel like for your testicle to be twisted round and round like a big knot. - Pain can be intermittent and be brought on by exercise - Nausea and vomiting

Answer 207

- Imaging should not be considered if testicular torsion is suspected as it will delay surgery! Think of how much pain the poor man must be in don't wait give him blood back to his testicle - Surgical exploration: should be performed immediately if there is high clinical suspicion as it allows definitive diagnosis and management. Should be performed within 6 hours to prevent irreversible damage (90% salvageable at 6 hours and 10% salvageable at ≥24 hours)

Answer 208

- Bilateral orchiopexy if the testicle is viable. This involves untwisting the testicle and fixing it to scrotal sac. Contralateral one should be fixed as well - Ipsilateral orchiectomy and contralateral orchiopexy if the testicle is not viable : removal of the affected testis and fixation of the contralateral testis to the scrotal sac to prevent contralateral torsion

Answer 209

- **Infertility/ subfertility**: torsion for 10-12 hours results in ischaemia and irreversible damage. Orchiectomy results in decreased spermatogenesis - **Pubertal delay:** may occur, particularly if bilateral orchiectomy is performed; hormone replacement may be required

Answer 210

- Within **4-6 hours** of symptoms, the testis can be saved in the majority of cases - A delay of **10-12 hours** or more results in irreversible ischaemia and necrosis - The testis is salvageable **<10% of** cases at **≥24 hours**

Answer 211

The onset of secondary sexual characteristics before **8 as a girl** and **9 as a boy**

Answer 212

- Gonadotrophin dependant- **premature activation of the hypothalamic-pituitary-gonadal axis** The sequence of pubertal development would be normal LH:FSH ratio >1 Gonadotrophin independent (pseudo, ‘false’ precocious puberty) from **excess sex steroids outside the pituitary gland.** The sequence of pubertal development would be abnormal, described as ‘dissonant’. Stimulated LH:FSH ratio < 1

Answer 213

- Central malformation or damage: Hydrocephalus, neurofibromatosis - Acquired: post sepsis, surgery, radiotherapy, trauma - Brain tumour

Answer 214

- Congenital adrenal hyperplasia - Exogenous sex steroids - Gonadal tumour - Hypothyroidism - McCune Albright syndrome- polyostotic fibrous dysplasia

Answer 215

- Short stature : early onset of puberty means a child loses 2-3 years of typical growth-hormone-dependant growth Psychological disturbance: child treated as older than their age, deprived of their childhood. Early menarche: particularly a practical consideration with onset in primary school-age – children where the school isn’t set up for it. Safeguarding concerns of early development, particularly in vulnerable special educational needs children

Answer 216

GnRH super-agonists can be given to suppress secretion *Continuous exposure to an agonist such as leuprorelin for several weeks causes pituitary GnRH receptors to become desensitised and no longer responsive, as release needs to be pulsatile* Detect and treat underlying pathology eg MRI scans to find tumour Reduce rate of skeletal maturation

Answer 217

Thyroid dysgenesis is a developmental abnormality of the thyroid gland. Either it doesn't develop at all or is poorly formed Thyroid dyshormogenesis anatomically normal thyroid gland. An enzymatic defect means the thyroid is unable to produce thyroid hormone normally. This accounts for the remaining 15% of cases Don't forget iodine deficiency as well!!, this is still the most common cause of congenital hypothyroidism world wide!

Answer 218

New born blood spot= Guthrie test 1. CF 2. Congenital hypothyroidism 3. Sickle cell disease 6 metabolic diseases e.g. MCADD, phenylketonuria and maple syrup disease etc.

Answer 219

- Feeding difficulties - Lethargy and increased sleeping - Constipation - Prolonged jaundice - Hoarse cry

Answer 220

- Starts from 8-14 and takes about 4 years to finish 1. Development of breast buds 2. Pubic hair 3. Periods- occurs around 2 years after puberty begins

Answer 221

9 – 15 in boys. It takes about 4 years from start to finish. Girls have their pubertal growth spurt earlier in puberty than boys. 1. Enlargement of testicles 2. Penis enlargement 3. Darkening of scrotum 4. Pubic hair 5. Deepening of voice

Answer 222

Tanner staging

Answer 223

Refers to a lack of sex hormones e.g. oestrogen and testosterone that normally rise prior to puberty

Answer 224

Hypogonadotropic hypogonadism: a deficiency of LH and FSH Hypergonadotropic hypogonadism: a lack of response to LH and FSH by the gonads (the testes and ovaries)

Answer 225

Where there is a deficiency of LH and FSH leading to a lack of sex hormones. This means there is nothing stimulating the gonads and they do not respond by producing sex hormones

Answer 226

- Previous damage to the hypothalamus or pituitary - GH deficiency - Hypothyroidism - Hyperprolactinaemia - Serious chronic conditions - Excessive eating or dieting - Kallman syndrome

Answer 227

Hypergonadotropic hypogonadism is where the gonads fail to respond to stimulation from the gonadotrophins (LH and FSH). There is no negative feedback from the sex hormones (testosterone and oestrogen), therefore the anterior pituitary produces increasing amounts of LH and FSH to try harder to stimulate the gonads. Therefore, you get high gonadotrophins (“hypergonadotropic”) and low sex hormones (“hypogonadism”).

Answer 228

- Previous damage to the gonads - Congenital absence of the gonads - Klinefelter's syndrome (XXXY) - Turner's syndrome (XO)

Answer 229

Kallman syndrome is a genetic condition causing hypogonadotropic hypogonadism, resulting in failure to start puberty. It is associated with a reduced or absent sense of smell (anosmia).

Answer 230

X linked recessive or dominant.

Answer 231

Full blood count and ferritin for anaemia U&E for chronic kidney disease Anti-TTG or anti-EMA antibodies for coeliac disease

Answer 232

- Early morning FSH and LH - Thyroid function tests - Growth hormone testing : **Insulin like growth factor 1** is often tested - Serum prolactin

Answer 233

- X-ray of the wrists to assess bone age and inform a diagnosis of constitutional delay - Pelvic ultrasound - MRI of brain and assess the olfactory bulbs in Kallman syndrome

Answer 234

A deficiency of the 21 hydroxylase enzyme. This causes underproduction of cortisol and aldosterone and **overproduction of androgens**

Answer 235

Autosomal recessive

Answer 236

21 hydroxylase is an enzyme that is used to convert progesterone into aldosterone and cortisol. Progesterone can also be converted to testosterone but doesn't require 21 hydroxylase Therefore due to a deficiency in this enzyme there is extra progesterone that cannot be converted into aldosterone or cortisol and it gets converted into testosterone instead

Answer 237

Female patients are born with **virilised/ambiguous genitalia** and an enlarged clitoris due to **high testosterone** They will also present after birth with **hyponatraemia** **hyperkalaemia** and **hypoglycaemia** Which will give symptoms of: - Poor feeding - Vomiting - Dehydration - Arrhythmias

Answer 238

- Tall for age - Facial hair - Absent periods - Deep voice - Early puberty

Answer 239

- Tall for their age - Deep voice - Large penis - Small testicles - Early puberty

Answer 240

**Skin hyperpigmentation** Hyperpigmentation occurs because the anterior pituitary gland responds to the low levels of cortisol by producing increasing amounts of ACTH. A by product of the production of ACTH is melanocyte simulating hormone. This hormone stimulates the production of melanin (pigment) within skin cells.

Answer 241

Hydrocortisone

Answer 242

Fludrocortisone

Answer 243

Where cells are unable to respond to androgen hormones due to a lack of receptors

Answer 244

It is X-linked recessive. Therefore patients are genetically male

Answer 245

Due to receptors not responding to testosterone extra androgens are converted into **oestrogen** This results in a female phenotype. However the patients will have testes in the abdomen or inguinal canal and the absence of a uterus, upper vagina, cervix, fallopian tubes and ovaries. The female internal organs do not develop because the testes produce **anti-Mullerian hormone** Patients will be infertile and there is an increased risk of testicular cancer if they are not removed

Answer 246

Can present in infancy with inguinal hernias containing testes but can also present with primary amenorrhoea Hormone tests will show: - Raised LH - Normal FSH - Raised/normal testosterone - Raised oestrogen (for a man)

Answer 247

Bilateral orchidectomy (removal of the testes) to avoid testicular tumours Oestrogen therapy Vaginal dilators or vaginal surgery can be used to create an adequate vaginal length Generally, patients are raised as female, but this is sensitive and tailored to the individual.

Answer 248

1. Physical injury 2. Sexual abuse 3. Emotional abuse 4. Neglect

Answer 249

- Disclosure. - Injury observed e.g. at school. - Incidental findings.

Answer 250

When the cells in the body have no fuel they think they are starving, they initiate the process of ketogenesis so they have usable fuel. Over time the glucose and ketone levels get higher, initially the kidneys produce bicarbonate to buffer the ketone acids and maintain a normal PH. However overtime the ketone acids use up the bicarbonate and the blood starts to become acidic

Answer 251

Hyperglycaemia overwhelms the kidneys and glucose starts being filtered into the urine. The glucose in the urine draws water out with it in a process called osmotic diuresis. This causes the patient to urinate a lot (polyuria). This results in severe dehydration. The dehydration stimulates the thirst centre to tell the patient to drink lots of water. This excessive thirst is called polydipsia.

Answer 252

Insulin normally drive potassium into the cells. Without insulin it is not added and stored in the cells. This means serum potassium can be high in DKA but can also be normal due to being excreted by the kidneys. However overall potassium is low in the body, So when treatment starts patients can develop severe **hypokalaemia** which can lead to fatal arrhythmias

Answer 253

the priority is fluid resuscitation to correct the dehydration, electrolyte disturbance and acidosis. This is followed by an insulin infusion to allow the cells to start taking up and using glucose and stop producing ketones.

Answer 254

Correct dehydration evenly over 48 hours. This will correct the dehydration and dilute the hyperglycaemia and the ketones. Correcting it faster increases the risk of cerebral oedema. (Give a fluid bolus of 500 mL of normal saline (0.9% sodium chloride) over 10 to 15 minutes if the initial systolic blood pressure (SBP) is <90 mmHg.) **10ml per Kilo** Give a fixed rate insulin infusion. This allows cells to start using glucose again. This in turn switches off the production of ketones.

Answer 255

Cerebral oedema This is because dehydration and high blood sugar concentration cause water to move from the intracellular space into the extra This causes the brain cells to shrink and become dehydrated. Rapid correction of this dehydration and hyperglycaemia can cause a rapid shift which causes the brain to swell and become oedematous leading to brain cell destruction and death

Answer 256

Neurological observations (i.e. GCS) should be monitored very closely (e.g. hourly) to look for signs of cerebral oedema. Be concerned when patients being treated for diabetic ketoacidosis develop headaches, altered behaviour, bradycardia or changes to consciousness. Management options for cerebral oedema are slowing IV fluids, IV mannitol and IV hypertonic saline. These should be guided by an experienced paediatrician.

Answer 257

Astrocytoma- they make up 40%

Answer 258

Medulloblastoma (~20%) – arises in the midline of the posterior fossa. May seed through the CNS via the CSF and up to 20% have spinal metastases at diagnosis. * Ependymoma (~8%) – mostly in posterior fossa where it behaves like medulloblastoma. * Brainstem glioma (6%) – malignant tumours associated with a very poor prognosis. * Craniopharyngioma (4%) – a developmental tumour arising from the squamous remnant of Rathke pouch. It is not truly malignant but is locally invasive and grows slowly in the suprasellar region.

Answer 259

Primary: they are the most common form of solid tumour in children and are the leading cause of childhood cancer death

Answer 260

- Recurrent headache - Blurred or double vision - Lethargy - Deteriorating school performance - Delayed or arrested puberty

Answer 261

- Developmental delay - Progressive increased in head circumference - Bulging fontanelle - Lethargy

Answer 262

Persistent or recurrent vomiting Problems with balance, coordination or walking Behavioural change Abnormal eye movements Seizures (without fever) Abnormal head position–wry neck, head tilt or persistent stiff neck

Answer 263

Astrocytoma

Answer 264

Craniopharyngioma

Answer 265

Cerebellar: medulloblastoma, astrocytoma, ependymoma Brainstem: brainstem glioma

Answer 266

Astrocytoma and ependymoma

Answer 267

- Seizures - Hemiplegia - focal neurological signs have poor survival rates

Answer 268

- Visual field loss - Pituitary failure have good survival rates but risk of long-term visual impairment

Answer 269

- Truncal ataxia - Coordination difficulties - Abnormal eye movements survival rates are improving with 5-year survival about 50% Other posterior fossa tumours: Astrocytoma – cystic, slow growing. Good prognosis following surgery. Ependymoma – behaves like medulloblastoma

Answer 270

- Cranial nerve defects - Pyramidal tract signs - Cerebellar signs - No raised ICP Management – palliative radiotherapy Prognosis – very poor (<10% survival)

Answer 271

Wilm’s tumour. Neuroblastoma. Rhabdomyosarcoma.

Answer 272

Originates from embryonal renal tissue and is the most common renal tumour of childhood. Over 80% of patients present before 5 years of age

Answer 273

Abdominal mass Haematuria

Answer 274

Abdominal pain Anorexia Anaemia Hypertension

Answer 275

Ultrasound/MRI is used to show mass Children will receive initial chemotherapy followed by delayed nephrectomy after which the tumour ins staged histologically

Answer 276

arise from neural crest tissue in the adrenal medulla and sympathetic nervous system. It is an unusual tumour in that spontaneous regression can sometimes occur in very young infants

Answer 277

Most children have an abdominal mass but the tumour can lie anywhere along the sympathetic chain from neck to pelvis - Pallor - Weight loss - Abdominal mass - Hepatomegaly - Bone pain - Limp

Answer 278

Retinoblastoma is a malignant tumour of retinal cells Retinoblastoma susceptibility gene is on **chromosome 13**

Answer 279

Autosomal dominant but with incomplete penetrance Can also be sporadic. All bilateral tumours are hereditary, as are about 20% of unilateral cases

Answer 280

1. Loss of red reflex 2. Pain around the eye 3. Poor vision 4. Squint

Answer 281

Laser therapy

Answer 282

Osteosarcoma and usually presents in adolescents and younger adults

Answer 283

The femur. Also tibia and humorous

Answer 284

Bone pain worse at night time Bone swelling

Answer 285

Poorly defined lesion in the bone with destruction of normal bone and a **fluffy appearance**

Answer 286

Raised ALP

Answer 287

Hepatoblastoma Wilms tumour Neuroblastoma Lymphoma/leukaemia Sarcoma Constipation Enlarged kidneys – polycystic disease

Answer 288

A malignant liver cancer composed of tissue resembling Fetal liver cells, mature liver cells or bile duct cells

Answer 289

Localised masses Lymphadenopathy Organomegaly Bone marrow infiltration - recurrent illness Airway obstruction from lymphadenopathy

Answer 290

Enlarging node without clear infective cause Persistently enlarged node Unusual site e.g. supraclavicular If have associated symptoms and signs Fever, weight loss, enlarged liver/spleen If CXR abnormal

Answer 291

Acute lymphoblastic leukaemia (ALL) is the most common in children Acute myeloid leukaemia (AML) is the next most common Chronic myeloid leukaemia (CML) is rare

Answer 292

ALL 2-3 years AML under 2 years

Answer 293

Malignant changes in a lymphocyte precursor cell, causing acute proliferation of a single type of lymphocyte **usually B-lymphocytes** This leads them to replacing the other cell types and causes pancytopenia

Answer 294

- Abdominal x-ray during pregnancy - Down's syndrome - Kleinfelter syndrome - Noonan syndrome - Fanconi’s anaemia

Answer 295

- Fever - Weight loss - Night sweats - Pallor - Petechiae and abdominal bruising - Abdominal pain - Generalised lymphadenopathy - Unexplained or persistent bone or joint pain - Hepatosplenomegaly

Answer 296

Need FBC within 48 hours - Will show anaemia, leukopenia, thrombocytopenia and a high number of abnormal WBCs - Blood film can show: **Blast cells** - Bone marrow biopsy: if 20% of cells in sample are lymphoblast this is indicative of ALL - Lymph node biopsy

Answer 297

Radiotherapy Bone marrow transplant Surgery Also give **allopurinol to prevent tumour lysis syndrome

Answer 298

Tumour lysis syndrome is caused by the release of uric acid from cells that are being destroyed by chemotherapy. The uric acid can form crystals in the interstitial tissue and tubules of the kidneys and causes acute kidney injury.

Answer 299

- Stunted growth and development - Immunodeficiency and infections - Neurotoxicity - Infertility - Secondary malignancy - Cardio toxicity

Answer 300

- Triggered by strong emotion or pain - Results in reduced perfusion of the brain - EEG will be normal - Do ECG - Most will grow out of it

Paediatrics 2 Flashcards

(333 cards)