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Paeds Flashcards

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1
Q

Discuss the phases of growth in children

A

Foetal phase: 30% of eventual height
Infantile phase: up to 18mnths, requires good health and thyroid function
Childhood phase: slow and steady prolonged period of growth. Based on genes, good health and thyroid hormones
Pubertal phase: driven by sex hormones. If puberty is early (not uncommon in girls) overall growth will be lower due to earlier fusion of the epiphyseal growth plates

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2
Q

Discuss the pubertal changes seen in females and males

A

Females:

  1. Breast development (12.5years)
  2. Pubic hair growth and rapid height spurt
  3. Menarche 2.5 yrs after initial breast development

Males:

  1. Testicular enlargement
  2. Pubic hair growth
  3. Height spurt

Both sexes

  1. Development of acne
  2. Axillary hair
  3. Body odour
  4. Mood changes
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3
Q

Discuss the causes of short stature

A 
Familial: both parents are short 
Intrauterine growth restriction/prematurity
Constitutional delay of puberty 
Endocrine: Hypothyroidism, GH deficiency
Nutritional deficiency 
Psychosocial deprivation 
Chromosomal disorders
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4
Q

Discuss the investigations for short stature

A 
Plotting previous and current height and weights 
X-ray of wrist and hands 
FBC
U&E's
Karotype 
IgA antibodies 
CPR/ESR
MRI
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5
Q

Discuss the causes of tall stature

A

Familial
Obesity
Secondary : Hyperthyroidism, increase sex steroids, excessive adrenal androgens)
Syndromes: Marfans, Klinefleters, maternal DM

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6
Q

Causes of abnormal head growth

A

A. Microcephaly

  • Familial
  • Autosomnal recessive
  • Congenital infection
  • Acquired insult

B.Macrocephaly

  • > 98th centile
  • Rapid increase = ICP
  • Hydrocephalus
  • Tumour
  • Neurofibromas
  • CNS storgage disorders

C. Craniostanosis
- Premature fusions of sutures (distortion of the head shape)

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7
Q

Define premature sexual development

A

8 years = female
9 years = males

Growth spurt + pubic hair and breast development

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8
Q

Classify precocious puberty

A

True PP: premature activation of the hypothalamic-pituitary-gondal axis

Pseudo PP: excess sex steroids

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9
Q

Discuss the causes of precocious puberty

A 
Gonadotrophin-dependent 
Idiopathic/Familial
CNS lesions (postradiation, tumours)

Gonadotrophin-indepedent
McCune-Albright
Tumours of adrenals or gonads

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10
Q

Define congenital adrenal hyperplasia (CAH)

A

Caused by a defect in pathway that synthesises cortisol to cholesterol
Deficiency in 21-hydroxylase or 11-hydroxylase
Autosomal recessive
Chromosome 6

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11
Q

Clinical features of CAH

A 
Female virilization 
Salt wasting adrenal crisis (mineralcorticoid deficiency) 
Cortisol deficiency (hypoglycaemia, hypotension, shock)
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12
Q

Diagnosis and management of CAH

A

Elevated levels of 17alpha-hydroxylprogesterone
Hyponatraemia
Hypochloridaemia

Management: 
Females (corrective surgery)
Lifelong glucocorticoids 
Mineralcorticoids 
Carry a lifelong steroid replacement card
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13
Q

List the four fields of development

A

Gross motor
Vision + fine motor
Hearing and speech language
Social, emotional and behaviour

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14
Q

List the primitive reflexes

A

Moro: extension of the head, extension of the arms (drop the baby)
Grasp: flexion of fingers when object placed in hand
Rooting: head turns to stimulus near the mouth
Stepping: Feet step when touching the floor

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15
Q

Discuss the physiology of the change in foetal Hb

A

Foetus Hb contains 4 alpha chains, higher affinity for oxygen.
HbF is gradually replaced with HbA
Foetus is born with a good store of iron, folic acid and vitb12

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16
Q

Define anaemia

A

Hb level below the normal limits
neonante < 14g/dL
1-12 months < 10g/dL
1-12years < 11g/dL

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17
Q

Causes of anaemia

A

Impaired red cell production

Increased red blood cell production

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18
Q

Iron deficiency anaemia

A 
Impaired red cell production 
Fatigue
Poor feeding 
Pallor
Pice

Microcytic, hypochromic anaemia ( decreased MCV, decreased MCH)

Oral intake
Iron supplement (sodium iron date)
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19
Q

Red cell aplasia

A

Impaired red cell production

  • Congenital
  • Erythoblastema of the new born
  • Parovirus B19 infection
Low reticulocyte
Low Hb
Normal bilirubin 
Absent RBC precursors on bone marrow 
-ve antiglobin test

Oral steroids
Blood transfusions

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20
Q

Discuss the pathophysiology of increased red blood cell destruction

A

Intravascular ( circulation)
Extravascular (liver/spleen)

Red cell membrane disorders
Red cell enzyme disorders
Haemoglobinopathies

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21
Q

Glucose-6-phophate dehydrogenase deficiency

A

G6PD: rate limiting enzyme pathway (stop oxidative damage)

Neonatal jaundice
Acute haemolysis

Measure G6PD

Avoid triggers ( blood fine outside episodes)

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22
Q

Sickle cell disease

A

Haemoglobinopathies
Autosomal recessive HbsHbs
HbsC = carrier of the disease

Anaemia 
Infection
Painful crisis
Acute anaemia 
Splenomegaly

Hydroxycarbinamide
Opiates + hydration

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23
Q

B-thalasaemia

A

Haemoglobinopathies
Decrease production of beta global chain
Two types major and minor

Anaemia
Growth failure
Haemoposis

Blood transfusions
Bone marrow transplant

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24
Q

Immune thrombocytopenia

A

Most common thrombocytopenia in childhood
Destruction of circulating antibodies by anti platelet IgG autoantibodies

Petechiae
Purpura
Superficial brusing
Epitaxis

Will resolve spontaneously
Severe: oral prednisolone

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25
Causes of anaemia of the newborn
Decrease RBS production - Red cell aplasia Bleeding - Umbilical cord - Internal haemorrhage Haemolytic anaemia - Ab destroying the RBC - Immune (haemolytic disease of the newborn) - Red membrane disorders - Red enzyme disorders - Abnormal haemoglobin disorders Blood loss - Feto-maternal haemorrhage - Twin to twin transfusions - Blood loss at delivery Anaemia of prematurity - Decreased EPO production - Decrease RBC lifespan - Iron and folate acid deficiency
26
Discuss the traffic light system for identifying a sick child
1. Colour 2. Activity 3. Respiratory 4. Circulation/Hydration
27
Discuss the pathophysiology of septicaemia
Focal infection/proliferation of blood stream Release of cytokines and endothelial cells Cause = meninoccal infection ( kids) Grp B strep ( neonates)
28
Clinical features of septicaemia
``` Fever Poor feeding Miserable/ irritable Increase HR/RR Decrease Bp Shock ```
29
Management of septicaemia
Antibiotics ( infection specific) Fluids ( correct hypovolaemia ) Catheter ( regular U/O) DIC ( FFP/platelet transfusions)
30
Discuss the pathophysiology of meningitis
Inflammation of the menages covering the brain Inflammatory cells present in the CSF Bacterial/ viral
31
List the microorganisms that cause bacterial meningitis
Neonates - 3 months: Grp B strep, E.coli, Listeria 1 month- 6 years: Nessieria meningitides, strep pneumonia > 6 years: Nessieria meningitides, strep pneumonia
32
Clinical features of meningitis
Headache Photophobia Neck stiffness ``` + rash vomiting irritable seizures poor feeding shock ```
33
Compare the CSF changes in bacterial vs viral meningitis
Bacterial - increase WCC - increased protein - decreased glucose - polymorphic cells Viral - increase in WCC - normal protein - normal glucose - lymphocytes
34
Treatment of bacterial menigitis
3rd generation cephalosporin ( cefotaxime/ ceftrixone) Bactericidal Works on the beta-lactam Disrupt the synthesis of the peptidoglycan layer forming the bacterial cell wall Prevents cross linking of the peptidoglycan Rifampacin to all household contacts
35
Discuss the causes and the management of viral meningitis
``` Enterovirus EPV Adenovirus Mumps Herpes zooster virus ``` Rx: Acyclovir
36
Meningococcal infection
Septicaemia + purpuric rash Rash: non blanching, regular size, neurotic centre
37
Impetigo
Highly contagious strep or staph infection Erythematous macules = vascular pustular or bulbous - rupture of the vesicles = honey crusted lesions Rx: Mild ( topical antiB's: muciporin) Narrow ( flucloaxicllin) May require board spec ( co-amiclav)
38
Tuberculosis
Caused by mycobacterium tuberculosis A. Unsymptomatic - Local inflammation - Latent disease - Mantoux test +ve - Hilar lymphadenopathy on CXR B. Symptomatic - Spread to the lymph system - Lung lesion + infected lymph node = Ghon's complex - Fever, Anorexia, Cough, CXR changes
39
Dx of TB
Acid fast bacili and zieh neslon's stain +ve | Mantoux test +ve
40
Rx of Tb
8/52: Rifampain, Isonazid, Pyrazinamide and Ethabutamol Further 8/52: Rifampicin, Isoniazid
41
Kawasaki's disease
Systemic vasculitis 4-6 years old Asian (Japanese) Cx: Likely to be immune hyperactivity. Associated with ITPKC gene ( decrease T cells)
42
Clinical features of Kawasaki's disease
``` + 5 day fever Bilateral non purulent conjunctivitis Oral mucositis Cervical lymphadenopathy Polymorphic rash Extremity change ( erythema dactlylisis) Coronary artery aneurysms ```
43
Rx of Kawasaki's disease
IV-IgG immunogloblins High dose aspirin Must do echo and cardio F/up
44
Discuss the transmission of HIV
Mother to child ( in vitro) @ delivery (intrapartum) Breast feeding (post partum) Vertical transmission
45
Dx of HIV in kids
>18 months: antibodies | <18 months: +ve will only confirm exposure not infection due to transplacental maternal abs
46
Clinical features of HIV
``` Persistent lymphadenopathy Hepatosplenomegaly Recurrent fever Parotid swelling Thrombocytopenia ```
47
Rx of HIV
Antiviral treatment - Clinical load - HIV viral load - CD4 count DRUGS -
48
Discuss the ways to reduce vertical transmission
Reduce the viral load of the mother Avoidance of breast feeding- reduce transmission Active management of labour and delivery
49
Causes of prolonged fever
Infective - Localized infection - Bacterial infections - Deep abscess - Infective endocarditis - TB - Viral infections Non-Infective - Systemic juvenille arthritis - SLE - Vasculitis - Inflammatory bowel disease - Sacrodoisis - Malignancy ( leukamia, lymphoma) - Drug fever
50
Discuss the mechanism of action of vaccines
Induce immunity via T-cellss Induce immunological memory Herd immunity Active: Live attenuated (MMR,BCG,Rotavirus) Passive: Inactivated ( whopping cough, tetanus) Conjugate virus: Polysacchride coat of bacteria ( pneuma, Hib, MenC) Improvement of the protein carrier Generates immunological memory
51
List the types of immunological memory
1. Primary: Intrinsic defect in the immune system | 2. Secondary: Caused by a disease or a treatment
52
List the types of primary immune deficiency
T-cell defects - Failure to thrive - Oral thrush - PCP Examples: SCID HIV infection B-cell defects - Bacterial infections - Recurrent diahorrae - Failure to thrive
53
Define wheeze and list the different types
Whistling sounds, breathlessness or persistent troublesome cough severely affecting the wellbeing of the infant 1. Transient early wheeze 2. Persistent and recurrent wheeze
54
Causes of a wheeze
``` Transient early wheeze Atopic asthma (IgE mediated) Non atopic asthma Recurrent aspiration of feeds Inhaled foreign body Cystic fibrosis Recurrent anaphylaxis Congenital abnormality ```
55
Discuss the prevalence of wheeze in childhood
Transient early wheeze (0-3yrs) Viral episodic wheeze (3-5yrs) IgG associated wheeze (5+)
56
Clinical features of viral episodic wheeze
No interval symptoms No excess atopy Improvement with age No benefit from inhaled steroids
57
Clinical features of asthma
``` Wheeze (polymorphic) Symptoms worse at night or early morning Triggers Family hx to atopy +ve response to asthma therapy ```
58
Investigations for asthma
Clinical dx Record PEFR ( peak expiratory flow rate) CXR will be normal ( avoid scanning kids) Skin prick test for common allergies
59
Management of asthma
Stepwise approach 1. Inhaled SABA (Salbutamol) 2. Inhaled SABA + inhaled steroids 3. Inhaled LABA ( must be with a corticosteroid) Seretide Consider adding in leukotriene receptor agents ( <5yrs) or oral theophylline (>5yrs) 4. Increase steroids to the max dose 5. Oral steroids (prednisolone)
60
Reason for failure to respond to treatment
Adherence Bad disease pathology Choice of drugs/devices Environment (smoke/pets)
61
Long-term side effects of steroids
Adrenal suppression Growth suppression Osteoporosis
62
Clinical features of a child with chronic asthma
Growth and nutrition may be below normal Chest: Hyperinflation, harrisons sulci, wheeze Atopic Hx: Allergic rhinitis, Eczema Time off school
63
Presentation of acute asthma attack
``` Wheeze + tachypnoea Tachycardia Use of accessory muscles and recession +ve pulse paradoxus Breathlessness prevents talking ``` Life threatening - Cyanosis - Fatigue - Drowsiness - Silent chest - Decreased consciousness
64
Management of an acute asthma attack
Nebulised B2 agonist Oral or IV steroids Nebulised ipratropium Oxygen
65
List the common pathogens that cause respiratory infection
VIRUSES - RSV ( respiratory synctical virus) - Rhinovirus - Parainfluenza virus - Adenovirus BACTERIAL - Strep pneumonia - Haemophilus influenza - Moxerella catarhalis
66
Pharyngitis
Pharynx and soft palate are inflamed Local lymph nodes are enlarged and tender Grp A Beta-haemolytic streptococus ( must do throat swab in case of further renal complications)
67
Tonsillitis
Intense inflammation of the tonsils Caused by Grp A Beta-haemolytic streptococcus and EBV Bacterial tonsillitis = pencillins Avoid amoxicillin as will cause maculopapular rash
68
Acute otitis media
6mnths- 12mnths Tympanic membrane will be bright and bulging ( loos of normal light reflection Caused by RSV, Rhinovirus, H.Influenza, Moraxella Analgesics Give Abs if severe
69
Clinical features of laryngeal and tracheal infections
Stridor Hoarseness due to inflammation Barking cough Degree of dyspnoea
70
Croup
Mucosal inflammation and increased secretions affecting the airway Narrowing of the trachea
71
Clinical features of croup
Preceding factors - Fever - Coryza - Chest recession @ activity Hoarseness Barking cough
72
Treatment of croup
``` Oral dexamethasone (0.15mg) Oral prednisalone (2mg) ``` ``` Nebulised steroid (Budesomide) Reduce severity, duration and need for admission ``` Nebulised epinephrine (adrenaline) + O2 facemask (Beware of rebound affects)
73
Acute epiglottis
Life threatening emergency Caused by H.Influenza type B Intense swelling of the epiglottis and surrounding tissues
74
Clinical features of acute epiglottis
High grade fever Painful throat Soft inspiratory stridor Increasing respiratory difficulty
75
Treatment of acute epiglottis
``` Managed in a resuscitation room Examination under anaesthetic Secure the airway Blood for culture IV abs = cefuoxime ( 3rd generation cephalosporin) ```
76
Whopping cough
Highly contagious respiratory infection Caused by bordetella pertussi ( ID via PCR) An effective vaccine does exist as part of diphtheria, tetanus and pertussis Infective 7 days post exposure to 3 weeks after cough
77
Clinical features of whopping cough
Catarrhal phase: Coryzal | Paroxysmal phase: Cough ( inspiratory whoop) can last for 3-6 weeks
78
Treatment of whopping cough
Erythromycin given early disease reduces infectivity but does not shorten the course of the illness
79
Cause of bronchiolitis
RSV respiratory syncytial virus
80
Clinical features of bronchiolitis
Corysal symptoms Dry cough Increased work of breathing Feeding difficulty ( increase dyspnoea) ``` O/E Increased RR Subcostal/Intercostal recession Hyperinflation of the chest Fine-end inspo crackles Increase HR Cyanosis/Pallor ```
81
Treatment of bronchiolitis
Supportive care Humidified oxygen Fluids if needed Admit if Resp distress Chronic lung pathology Feeding difficulties
82
Causes of pneumonia
Newborn: Grp B strep Infants: RSV, strep pneumoniae, Bordetella pertussis, Chylamydia Children: Mycoplasma pneumonia, strep pneumoniae, chlymadia pneuomia
83
Clinical features of pneumonia
``` Fever Breathing difficulties Cough Lethargy Poor feeding Unwell child ``` ``` O/E Tachypnoea Nasal flaring Chest indrawing Increased respiratory rate Dullness to percuss Bronchial breathing ```
84
Management of pneumonia
``` ADMISSION Increase RR Increase WOB Grunting Decreased feeding ``` Broad spec abs ( amoxicillin)
85
Clinical features of UTI
``` Miserable Temperature Vomiting Abdo pain Fever Poor feeding ```
86
Cause of UTI
E.Coli or Kleibsiella
87
Treatment of UTI
``` Trimethoprim ( if well) IV cefuroxime ( if unwell) ``` Neonates will require an ultrasound of the kidneys to rule out malformation. Beware of ESBL: Extended spectrum beta-lactamase producers Resistant to all penicillins and cephalosporins Rx: Meropenem
88
Treatment for osteomyelitis
Commonly caused by staph aureus Rx: IV cefuroxime
89
Differential Dx of MSK pain
1. Life threatening pain 2. Pain (-ve swelling) 3. Pain (+ve swelling) LIFE THREATENING Malignancy (leukaemia, bone tumour, lymphoma) Sepsis (septics arthritis, osteomyelitis) Non-accidental injury PAIN -VE SWELLING Hypermobile joints Metabolic disease (hypothyroidism) Tumour (benign) ``` PAIN +VE SWELLING Trauma Infection JIA Arthritis SLE CF Sarcoidosis ```
90
List the types of JIA
1. Oligoarthritis: - 1-4 joints - ANA+/ANA- 2. Polyarticular RF-Ve: - Symmetrical - Low grade fever - Anterior uveitis 3. Polyarticular RF+Ve: - Symetrical large and small joints - Rheumatoid +ve - Poor prognosis 4. Psoriatic arthritis - Asymetrical arthritis - Psoriasis - Dactylitis - Nail pitting 5. Enthesistis related arthritis - Lower limb large joint arthritis - Lumbar spine involvement - Anterior uveitis - Reiter syndrome - HLA B27+ 6. Systemic JIA - Arthritis ( + daily fever for >3 days) - Evanscant rash (salmon pink) - Lymphadenopathy - Hepatomegaly +/- splenomegaly - Serositis - Link with MAS
91
What is Macrophage Activation Syndrome
``` Potentially fatal Dysregulation of T-lymphocytes, Natural killer cells activation Excessive cytokine production Abnormal proliferation of macrophages ```
92
Clinical features of MAS
``` Unrelenting fever HSM CNS dysfunction Purpuric rash Cytopenia Increase ferritin ``` Rx: Steroids, cyclosporin and supportive care
93
Treatment for JIA
NSAID's Steroids DMARD's (methotrexate) Biologics (TNF-alpha- inhibitor)
94
Define osteoporosis
A disease characterised by low bone mass and micro architectural detonation of bone tissue, leading to enhanced fragility and an increase in fracture risk.
95
Causes of osteoporosis in children
INHERITED - Osteogenesis imperfecta - Inborn errors - Idiopathic ACQUIRED - Drug induced - Endocrinopathies - Malabsorption
96
Aetiology of osteogenesis imperfecta
Autodominat condition which results in defects in the typeI collagen genes
97
Clinical features of osteogenesis imperfecta
``` Bone fragility Fractures Deformity Bone pain Impaired mobility Poor growth ```
98
Types of osteogenesis imperfecta
I: mild II: lethal III: progressively deforming IV: moderate
99
Management of osteogenesis imperfecta
Bisphosphonates Pain management IV pamidronate
100
Define rickets
Failure of mineralisation of growing bone or osteoid tissue
101
Causes of rickets
Nutritional - Dark skin - Lack of maternal Vit D - Diets low in calcium - Strict vegan diet Intestinal absorption - Small bowel enteropathy ( coeliac disease) - Pancreatic insufficiency - Cholestatic liver disease Defective production of 25(OH)D2 -Chronic liver disease Target organ resistance to 1,25 (OH)D2 - Mutations in the D receptor gene
102
Clinical features of rickets
``` Misery Failure to thrive Frontal bossing of the skull Bowing of the weight bearing bones Seizures Hypotonia ```
103
Treatment of rickets
Balanced diet Correction of the predisposing risk factors Treatment of the underlying cause
104
Define nephrotic syndrome
Proteinuria Decreased albumin Oedema
105
Types of nephrotic syndrome
Congenital Steroid sensitive Steroid resistant
106
List the investigations of nephrotic syndrome
``` Urine protein FBC CRP/ESR Complement leves Antistreptolysin O and Anti-DNASE B Throat swab Hep B and Hep C ```
107
Steroid sensitive nephrotic syndrome
MACROSCOPIC HAEMATURIA ``` Rx: Oral corticosteroids (60g/m2) 4/52 Decrease to 40g/m2 Gradually decrease steroids Dipstick urine for 10 days to check decreasing protein levels ``` 1/3 will resolve directly 1/3 frequent relapse 1/3 infrequent relapse
108
Steroid resistant nephrotic syndrome
``` Refer to nephrologist Rx: Diuretic Fluid management Salt restriction ACE inhibitors ```
109
Congenital nephrotic syndrome
Poor prognosis | High mortality
110
Causes of acute nephritis
Post infection Vasculitis IgA nephrology Goodpastures (antiglomerular basement membrane)
111
Clinical features of acute nephritis
``` Decrease urine output HTN Oedema Proteinuria Haematuria ```
112
Treatment of post strep infection nephritis
Increased ASOT Increased antiDNASE B Reduced C3 and C4 Rx HTN Dialysis Penicillin
113
Why would a UTI require further investigations
If - Male - Recurrent - Unusual organism grown A. USS: size and drainage of the bladder and kidneys B. Micturating cytourethrogram ( vesicoureteric reflux) C. DMSA scan: radionucleotide imaging renal function
114
List the red flags in paediatric GI
``` Bile stained vomiting ( intestinal obstruction) Haematemesis (ulceration) Projectile vomiting (pyloric stenosis) Abdo pain/tenderness (Surgical abdo) Abdo distension (obstruction) Blood in stool (intusception) Splenomegaly ( liver disease) ```
115
Define GORD
Involuntary passage of gastric contents into the oesophagus
116
Complications of GORD
Failure to thrive Oesophagitis Pulmonary aspiration Dystonic neck posturing
117
Management of GORD
Thickening agents (carobel) H2 receptor antagonists ( ranitidiene) PPI (omeprozole) Nissen fundiplication
118
Define pyloric stenosis
Hypertrophy of the pyloric muscle causing gastric outlet obstruction
119
Clinical features of pyloric stenosis
Projectile vomiting Hunger Dehydration Decreased weight Will present in a HYPOKALAEMIC, HYPOCHLORAEMIC, METABOLIC ACIDOSIS
120
Treatment of pyloric stenosis
Pyloromyotomy
121
Clinical features of appendicitis
``` Anorexia Vomiting Abdo pain ( localised in the RIF) Low grade fever Tenderness Guarding ```
122
Treatment of appendicitis
Appendectomy
123
Define intussuseption
Invagination of proximal bowel into the distal segment ( ileum into caecum) 3mnths to 2yrs
124
Clinical features of intussuseption
Severe colicky pain Pallor Red current jelly Abdo distension and shock
125
Dx and treatment of intussuception
Xray of distended bowel and absence of gas in the colon ( arrowhead colon) Rx: Rectal air insufflation
126
Cause of malrotation of the gut
Lack of fixation at dudonjejunal fixture or illogical region Pc: obstruction or obstruction with compromised blood supply Bilious vomiting
127
Treatment of malrotation of the gut
Voluvulusis is untwisted and duodenum mobilised
128
Causes of gastroenteritis
``` Rotavirus Norovirus Camblyobacter Shigella E.coli ```
129
Clinical features of diarrhoea
Loose watery diarrhoea | Vomiting
130
List the types of dehydration
A. Isonatrameic and hyponatraemic dehydration Swift in the water from the extra-intra cellular compartments Can cause an increase in ICP B. Hypernatraemic dehydration Extracellular fluid is hypertonic Doughy child
131
Post gastritis syndrome
Watery diarrhoea syndrome | Temporary lactose intolerance " Climtest"
132
Causes of constipation
Hirschsprung disease anorectal abnormalities Hypothyroidism Hypercalcaemia
133
Red flag symptoms of constipation
``` First few weeks of life Meconium passed > 24hrs Faltering growth Delayed walking/ Lower limb neurology Abdominal distension or vomiting ```
134
Treatment of constipation
Laxatives Regular bowel habit May require psychological support
135
Define Hirchsprung disease
Absence of ganglion cells from mesenteric and submucosal plexus of the large bowel
136
Clinical features of Hirschsprung disease
Failure to pass meconium in the first 24hrs Abdo distension Bilious vomiting
137
Dx and treatment of Hirschsprung disease
Barium enema Rx: Colostomy and anastomosing parts of the colon
138
Define toddler diarrhoea
Chronic non specific diarrhoea Persistent loose stools Maturational delay in gut motility = intermittent explosive diarrhoea
139
Define infantile colic
Recurrent inconsolable crying Drawing up the legs Lasts about 4 months
140
Causes of inconsolable crying
``` Colic GORD Cow's milk allergy Otitis media Incarcerated hernia UTI Intussusception ```
141
Clinical syndromes of protein-energy malnutrition
MARASMUS - Wasted - Wizened - Withdrawn - Apathetic KWASHIORKOR - Occurs in children weaned late from the breast - High starch diet - Wasting - Oedema - Depigmented skin - Angular stomatitis - Hepatomegaly
142
Define Meckels Diverticulum
Remnant of the foetal vital-intestinal duct | Contains ectopic gastric mucosa
143
Clinical features of Meckels Diverticulum
Painless Severe rectal bleeding due to peptic ulceration Rx: Surgical
144
Types of eczema in children
Infantile seborrhoeic eczema Atopic eczema Napkin dermatitis
145
Presentation of infantile seborrhoea eczema
First two months of life Scaly non itchy rash Cradle cap, face, flexures and napkin area Rx: Topical steroids and emollients
146
Clinical features of eczema
Dry red itchy rash Extensors (young) Spreads to the flexures (old)
147
Dx of eczema
Clinical dx IgE is raised Food and environmental allergens
148
Treatment of eczema
MILD - Emollients - Mild topical steroid MODERATE - Emollients - Moderate topical steroids - Topical calcineurin inhibitors - Wet wraps SEVERE - Emollients - Potent topical steroids - Topical calcineurin inhibitors - Wet wraps - Phototherapy - Systemic treatment
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Effects of steroids on children
``` Reduced bone density Reduced immunity Stunted growth Suppression hypothalamic adrenal axis Weight gain ```
150
Causes of napkin dermatitis
Irritant contact dermatitis Candidiasis Seborrhoeic dermatitis
151
Define Steve-Johnson Syndrome
Immune complex hypersensitivity disorder | Often caused by an adverse drug reaction (Phenytoin, Anti-psychotics, Allopurinol, sulfsazine)
152
Clinical features of Steve-Johnson Syndrome
Lower resp tract infection | Clusters of mucocutaneous lesions (palms. soles.dorsums of the hand)
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Treatment of Steve-Johnson Syndrome
Supportive care
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Define anaphylais
Severe hypersensitivity reaction that manifests with respiratory difficultly and cardiovascular distress
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Pathophysiology of anaphylaxis
Rapid degranulation of mast cells and basophils with systemic release of inflammatory mediators, capillary leak, mucosal oedema and smooth muscle contraction. - Immunologic: mediated by IgE or immune complexes/complement - Non-immnunologic: systemic mast cells/basophil degranulation
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Treatment of anaphylaxis
``` Remove allergen where possible IM adrenaline (1mg/kg) IV hydrocortisone and antihistamine can be used to dampen down the response. ```
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Treatment of acne vulgaris
Topical treatment with keratolytic agents (Benzoyl peroxide) UV light Oral antibiotics: low dose therapy with minocycline or oxytetracycline Vitamini A analogue (13-cis-retinoic acid) for severe acne
158
Define Henoch-Schönlein Purpura (HSP)
IgA mediated autoimmune hypersensitivity vasculitis of childhood Usually preceded by a respiratory infection
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Clinical features of HSP
Skin purpura Arthritis Abdominal pain GI bleeding Orchitis Low grade fever Erythematous macular rash (will become purpuric lesions) Renal disease (nephritis, can develop into end stage renal failure) Microscopic haematuria with moderate proteinuria
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Risk factors for developing HSP
Infections - Grp A streptococci - Mycoplasma - EBV ``` Vaccinations Environmental allergens (Drug and food allergies) ```
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Dx of HSP
Clinical dx Investigations will reveal Increased WCC Increased ESR Increased IgA Must to autoantibody screen
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List the most common paediatric malignancy
``` Leukaemia Brain tumours Lymphoma Neuroblastoma Nephroblastoma ```
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Clinical features of Acute lymphoblastic leukaemia
BM features - Anaemia - Thrombocytopenia (bleeding) - Leukopenia (infections) Pallor Malaise Anorexia Lympadenopathy
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Key finding on investigation of ALL
Increase lymphoblasts Decrease RBC Decrease platelet BM aspirate = > 20% blasts
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Treatment of acute lymphoblastic leukaemia
``` Induction Consolidation Interm maintenance Delayed intensification Maintenance ``` Girls maintenance = 2 yrs Bos maintenance = 3yrs
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Types of CNS tumours
Astrocytoma Medulloblastoma Glioma of the brainstem Craniopharyningoma
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Presentation of SOL in the brain
``` Headache ( worse on lying down) Vomiting ( morning time) Papilloedema Ataxia Nystagmus Back pain New onset seizures ```
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Types of lymphoma
Hodgkins (adolescences) | Non-Hodgkins ( childhood)
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Clinical features of hodgkins
Painless Asymetrical Spread continuously to adjacent lymph node B symptoms - Fever - Weight loss - Night sweats
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Investigations of Hodgkins
LN excisions biopsy = REED STENBERYG CELLS
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List the staging system of Hodgkins
Ann Arbour (1-4)
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Treatment of Hodgkins
Combination chemo ABVD regimen BMT if relapse
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Clinical features of Non-Hodgkins lymphoma
Painless Symmetrical Discontinuous spread BM Features - Pancytopenia - Hyper viscosity
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Types of non-hodgkin lymphoma
B-cell - Small cell lymphocytic - Diffuse large B cell - Low or high grade T-cell - Anaplastic - Enteropathy
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Treatment of Non-Hodgkin lymphoma
R-CHOP regimen BMT If low grade only treat when symptomatic
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Explain the pathophysiology of neuroblastoma
Arise from the neural crest cells in adrenal medulla and SNS Benign to highly malignant <5yrs
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Clinical features of neuroblastoma
``` Pallor Wt loss Abdo pain Hepatomegaly Bone pain ```
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Investigations of neuroblastoma
Increase urinary catecholamines
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Explain the pathophysiology of Wilms tumour
Originates from embryonal renal tissue
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Clinical features of Wilms tumour
``` Abdo pain Anorexia Anaemia HTN Haematuria ```
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Explain pathophysiology of retinoblastoma
``` Malignant tumour of the retinal cells Bilateral tumour Hereditary RBI gene White pupillary reflex ( loos of red reflex) Squint ```
182
Discuss the physiology of foetal circulation
Foramen ovale and ductus arteries can remain patent beyond the neonatal period Foetus:  left atria @ low pressure, little blood returning from the lung, increase pressure @ right atria BREATHES Resistance to pulmonary blood falls Increase in blood flow to the lungs Increase in left atria pressure Pressure changes = Foreman ovale to close
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Dx of congenital heart disease
``` Antenatal murmur Heart murmur Cyanosis Shock: low cardiac output Cardiac failure ```
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Types of congenital heart disease
Acyanotic (breathless) | Cyanotic (blue)
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List the features of an innocent murmur
Asymptomatic patient Soft blowing murmur Systolic murmur Left sternal age
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Classify and list the types of left to right shunts
Acyanotic condition 1. Atrial septal defect (ASD) 2. Ventricular septal defect (VSD) 3. Patent ductus arteriosus (PDA)
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Clinical features of ASD
Recurrent chest infections | Ejection systolic murmur
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Treatment of ASD
Occlusion device using cardiac catherisation
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Clinical features of VSD
Breathlessness Heart failure Recurrent chest infections Pansystolic murmur
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Treatment of VSD
Small: will close spontaneously Large: Heart failure rx with diuretics
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Physiology of patent ductus arterious
Connects the aorta to the left pulmonary artery Closes by 4th day Risk factors - Preterm infant - Down syndrome - High altitudes
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Clinical features of PDA
Asymptomatic | Bounding pulse
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Classify and list the types of right to left shunts
Cyanotic 1. Teratology of Fallot 2. Transposition of the great arteries
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Anatomical features of TOF
Large VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy
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Clinical features of TOF
Murmur Cyanosis Tet spells Harsh ejection systolic murmur
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Treatment of TOF
Management of Tet spells - Morphine - Sodium bicarbonate - Propranolol Definitive treatment is surgical
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Anatomical features of transposition of the great arteries
Aorta connected to the right ventricle Pulmonary artery connect to the left ventricle Blue blood to the body Red blood to the lungs
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Clinical features of transposition of the great arteries
Cyanosis | Loud singular second heart sound
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Management of transposition of the great arteries
Immediate - Infusion of prostaglandin E1 to reopen the ductus arteriosus - Emergency cardiac catherization and therapeutic ballon arterial septostomy
200
List the obstructive lesions in congenital heart disease
Coarctation of the aorta (COA) Aortic stenosis Pulmonary stenosis
201
Features of coarctation of the aorta
Narrowing of the aorta Clinical dx is weak or absent femoral pulses Asymptomatic child
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Features of aortic stenosis
Thrill Asymptomatic murmur Rx: Surgical or ballon valvotomy
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Pathophysiology of rheumatic fever
Sequela of group A beta-haemolytic streptococcal infection | Abnormal immune response
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Clinical features of RF
Polyarthritis Fever Malaise Pericarditis Myocarditis Endocarditis Erythema marginatum: pink macules on trunk and limb
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Dx and Rx of rheumatic fever
Clinical dx of Ducketts Jones criteria High dose aspirin Steroids Prevention is with penicillins
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Infective endocarditis
Child with fever and significant heart murmur Rx: 4-6 weeks of IV antibiotics (High dose ampicillin + amino glycoside)
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Cardiac arrhythmias in children
Supraventricular tachycardia Narrow complex tachycardia with P waves after the QRS complex Rx: Synchronised DC cardioversion
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Down syndrome (trisomy 21)
``` Trisomy 21 1 in 650 Craniofacial appearance Delayed motor milestones Learning difficulties Increase susceptibility to infection Epilepsy Alzheimer's disease ```
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Edwards syndrome
Trisomy 18 | Die at infancy or birth
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Pateaus syndrome
Trisomy 13 | Die at birth or infancy
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Turners syndrome
``` 4 (X,) Short stature Webbed neck Delayed puberty Hypothyroidism ``` Rx: GH, oestrogen
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Klinefleters syndrome
``` (47, XXY) Infertility Hypogonadism Normal pubertal development Gynaecomastia Tall stature ```
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Unstable genetic mutations
Unstable expansion of the trinucleotide repeat Eg Fragile X syndrome - Learning difficulties - Fault @ CGC trinucleotide
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Mitochondrial inheritance
own DNA not maternal DNA Clusters of overlapping phenotypes Eg: Leber hereditary optic neuropathy
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Examples of uniparent disomy
Prader-willi syndrome - hypotonia - developmental delay - hyperplasia - obesity - paternal chromosome 15 Angleman Syndrome - Cognitive impairment - facial appearance - Ataxia - Epilepsy - Maternal chromosome 15
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Principle of gene based therapies
Involves the repair, suppression or artificial introduction of genes in genetically abnormal cells
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Cystic Fibrosis
Autosomal dominant condition Chromosome 7 CF transmembrane conductance regulator gene (CFTR) ATP responsive chloride channel Mutation DF508 Sodium transport across the respiratory epithelium Composition of the cell surface glycoprotein
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Key clinic features of cystic fibrosis
``` High sodium sweat Pancreatic insufficiency Biliary disease GI disease Resp disease ```
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Muscular dystrophy
Duchenne's - X- linked recessive condition - Deletions in the dystrophin gene - Abnormal high CK level - Progressive condition - Delayed motor signs ( not walking by 18 months is huge) Becker's - Abnormal dystrophin gene - Proximal muscle wasting and weakness - X-lined recessive pattern - Variable prognosis
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Noonan syndrome
"Male Turners Syndrome" - Autosomal dominant - Mutations in the MAPK pathway - Short stature - Broad and webbed neck - Sternal deformity - Developmental delay - Increased bleeding tendency
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Williams syndrome
Infantile Hypercalcaemia - Mental disability - Unusual facies - Supravavular aortic stenosis - Connective tissue abnormalities - Intellectual disability
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Define a seizure
Sudden disturbance in neurological function caused by abnormal or excessive neuronal discharge
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Causes of seizure
Epileptic seizure - Malformation - Idiopathic - Infection - Cerebral tumour - Neurodegenerative disorder - Neurocutanous syndromes Non epileptic seizure - Febrile - Hypoglycaemic - Hypocalcaemia - Hypernatraemia - Head trauma - Meningitis - Toxins
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Define febrile convulsion
Seizure associated with fever in a child between 6months and 6 years of age Brief ( 1-2 minutes) Tonic-clonic in nature Rx: Treat the infection
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List the types of paroxysmal disorders
``` Breath holding attacks Reflex anoxic seizures Syncope Migraine BVP ```
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Breath holding attacks
Temper tantrums in toddlers Crying Turns blue Loss of consciousness Rx: Behaviour modifications
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Types of Epilepsy
``` Generalised Focal - Frontal - Temporal - Occipital - Parietal ```
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General management of epilepsy
Anti-epileptic drug therapy ( lowest dose possible to ensure seizure free) Ketogenic diet Vagal nerve stimulation Surgery
229
Define hydrocephalus
Enlargement of the cerebral ventricles due to excessive accumulation of cerebrospinal fluid Caused by either a - Intraventricular obstruction - Extraventricular obstruction Rx: Ventriculoperitoneal shunt
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Causes of hydrocephalus
Intraventricular obstruction - Congenital malformation - Intraventricular haemorrhage - Brain tumour Extraventricular obstruction - Subarachnoid haemorrhage - TB meningitis
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Define cerebral palsy
Group of conditions affecting motor function and posture due to non progressive lesion of the developing brain
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Causes of cerebral palsy
Antenatal - Congenital infections (Toxoplasmosis, CMV, Rubella) Intrapartum - Birth asphyxia Postnatal - Hypoxia-ischaemic encephalopathy - Intraventricular haemorrhage - Meningitis - Encephalitis
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Types of cerebral palsy
Spastic cerebral palsy - Hemiparesis - Diplegia - Quadriplegia Ataxic cerebral palsy - Damage to the cerebellum - Hypotonia and poor balance Dyskinetic cerebral palsy - Hypotonia - Delayed motor development
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Infantile spasms (West syndrome)
Uncommon form of epilepsy 4-6 months Violent flexor spasms of head, trunk and limbs followed by extension Poor prognosis
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Classification of neonatal jaundice
1. First 24hrs (ALWAYS PATHOLOGICAL) - Sepsis: GBS or Herpes zooster virus - Haemolysis: ABO incompatibility or rhesus incompatibility (Hb will drop very quickly) 2. 24-48hrs - Normal physiological ( Breast fed babies) Rx: phototherapy - Sepsis ( as above) 3. > 14 days - Prolonged jaundice High levels of conjugate bilirubin Screen for GBC DCT FBC SBR TFT's Metabolic causes 1. Gal-1-put 2. G6PD 3. Gilberts syndrome 4. Alpha 1 antitrypsin USS of the liver: Look for the binary tree atresia Note uncojuncated bilirubin will cross the blood brain barrier Result in kernicterus ( build up of bilirubin in the basal ganglia)
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List the core symptoms of ADHD
Inattention - easily distracted - does not listen when spoken to - dislikes task requiring mental effort - hard to follow instructions Hyperactivity - squirms and fidgets - cannot sit still - talks excessively Impulsivity - blurts out answers to questions - difficulty waiting turn - poor road safety
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Diagnosis of ADHD
Clinical interview Behavioural observation Information from 3rd parties
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Rating scale used for ADHD
Conner's Always must screen for comorbidities or organic causes
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Treatment of ADHD
Education and parenting support School support and liaison ( behavioural management) ``` Medication Not for preschool kids - Methylpendiate - Atomexitine - Lisdexameftaime ``` Psychosocial treatment - CBT - Family therapy - Art and music therapy
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Define autism spectrum disorder
Abnormal or impaired development before the age of 3 | Abnormal functioning in social interaction, communication and repetitive behaviour
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Core symptoms of autism
Persistent deficits in social communication and social interaction across multiple contexts Restricted repetitive patterns of behaviour, interests or activities. TRIAD 1. Social interaction 2. Imagination/ Rigid pattD erns of behaviour 3. Poor range of activities/ interests
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Management of ASD
Early intensive behavioural intervention +/- speech therapy Parent training Support Social skills training Drugs ( Risperidone- aggression, melatonin- sleep, SSRI's - repetitive behaviour) Benefits: Disability living allowance
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List the types of hearing loss
CONDUCTIVE - Glue ear - Ear wax build up - Middle ear infection - Perforated eardrum SENSORY - Damage to the cochlea - Permanent - Manage with hearing aids
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When are the test of auditory function performed
NEWBORN - Conduct in the prescences of high risk 7-9 months - All children 18-24 months - speech discrimination tests - in kids with suspected hearing loss School entry - Sweep test - Screen all children
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Features of periorbital cellilutisis
Eyelid oedema Erythema Chemosis Orbital signs Proptosis Gaze restriction Blurred vision
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Treatment of periorbital cellulitis
Fluoxicillin | Cefotaxime
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Define vesicoureteric reflux
Urine refluxes up to the ureter during voiding Predisposing to infection Screening siblings due to strong genetic component Dx with micturating cystourethrogram
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Haemolytic uraemic syndrome
Common cause of acute renal failure in children Associated with diarrhoea from Shigella or E.coli Features - Glomerular microangiopathic haemolytic anaemia (RBC fragmentation - Thrombocytopenia Management - Supportive - Antibiotics are contraindicated as they induce expression and release of Shigella endotoxin.
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Features of amblyopia
Defective visual acuity, persists post correction of the refractive error and removal of pathology
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Treatment of amblyopia
Refractive adaption Occlusion of good eye Atropine eye drops in the good eye Aim: equal vision in both eyes
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Causes of undescended testes
Common congenital genitourinary anomaly
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Treatment of undescended testis
Treatment is by orchidopexy Increased risk of malignancy, sub fertility and torsion
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Causes of congenital hypothyroidism
``` Developmental defects (thyroid agencies) Dyshormonogenesis (inborn error of thyroid hormone synthesis) ```
254
Clinical features of congenital hypothyroidism
Prolonged neonatal jaundice Feeding problems ``` Coarse facies Large fontanelle Large tongue Hypotonia Goitre ``` Rx: Thyroxine
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Define Kallamans syndrome
Hypogonadotropic Hypogonadism | Delayed puberty and impaired sense of smell
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Define Perthes disease
Osteonecrosis of the femoral head prior to skeletal maturity. Leads to growth disturbance with temporary ischaemia of the upper femoral epiphysis Cycle of avascular necrosis with flattening and fragmentation of femoral head. Insidious limp between 3-12 years Intermittent pain Mild disease: analgesia and activity restriction Moderate/ Severe: fixation of the hip in abduction
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Define transient synovitis
``` Children 2-12 Sudden onset hip ain Limp Refusal to weight bear on the affected side Supportive care Resolves in 2 weeks ```
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Define developmental dysplasia of the hips
Spectrum of hip instability ranging from a dislocated hip to hip with various degrees of acetabular dysplasia.
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Clinical features of developmental dysplasia of the hips
``` Delayed walking Painless limp Waddling gait More common in girls Allis sign ( shortened femur) ```
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Management of development dysplasia of the hips
<8 months - Fixing the hips in abduction - Pavlik or Von Rosen harness Older children need surgery
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Cause of osteomyelitis
Haematogenous in origin or secondary to an infected wound Starts at the metaphysics Staphaureus or Grp B strep
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Clinical features, diagnosis and treatment of osteomyelitis
Fever Refusal to move affected limb Systemically unwell Tenderness over affected bone Increase in acute phase reactants +ve blood cultures TREATMENT IV Abx Several weeks of oral abx
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Define septic arthritis
Purulent infection of the joint space | Lead to bone destruction and considerable disability
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Clinical features of septic arthritis
Fever Irritability Refusal to weight bear Pseudoparalysis
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Treatment of septic arthritis
Prolonged IV antibiotics
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Define kohler's disease
osteochondrosis of the tarsal navicular bone.
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Define Osgood-schlatters disease
caused by multiple small avulsion fractures from contractions of the quadriceps muscles at their insertion into the proximal tibial apophysis
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Clinical features of measles
Prodrome ( 4 C's) - Cough - Coryza - Conjunctivitis - Cranky Koplik spots Rash appears ( starts behind the ears)
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Treatment of measles
Isolate (Hospitalisation if immunocompromised) Adequate nutrition (Catabolism is very high) Treat any secondary bacterial infection
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Cause and clinical features of rubella
``` Rubivirus Devastating effect on the foetus in pregnant women Low grade fever Pink-red maculopapular rash Generalised lymphadenopathy ```
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Pregnancy and rubella
Rubella is a notifiable disease | All pregnant women should be screened for anti rubella IgG/ IgM
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Cause of chickenpox
``` Varicella zoster virus Transmitted by - Droplet infection - Direct contact - Contact with soiled materials ```
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Clinical features and treatment of chicken pox
Brief coryza period Itchy vesicular rash Symptomatic treatment in healthy children Immunocompromised: VZIG
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Causes and clinical features of diphtheria
Corynebacterium diphtheria Tonsillitis + polyneuritis ( starting with the cranial nerves) Bronchopneumonia Muffled voice
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Treatment of diphtheria
Diphtheria antitoxin and erythromycin
276
Cause and clinical features of polio
Poliovirus ( enterovirus) spread by faecal oral transmission Fever Headache Malaise
277
Causes of scarlett fever
Endotoxins released from Strep progenies
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Clinical features of scarlett fever
Sore throat Fever Rash on chest, axillae or behind the ears Pin prick blanching rash Facial flushing with circumpolar pallor Strawberry tongue
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Treatment of Scarlett fever
Penicillin 10 days
280
Clinical features of coxscakie virus
Aseptic meningitis Myocarditis Pericarditis
281
Clinical features and treatment of encephalitis
Flu-like prodrome Decreased consciousness Odd behaviour Fits and vomiting Causes - Parovirus - HSV - TB - Mycoplasma - TB Treatment depends on the cause
282
Define child abuse
Deliberate infliction of harm,bullying or neglect or failure to prevent harm
283
Define neglect
Persistent failure to meet a child's basic psychological needs to result thats that is likely to result in serious impairment of the child's health and development

Paediatrics (3 decks)

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