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PAEDIATRICS 3 Flashcards

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1
Q

How many newborns become visibly jaundiced?

A

Over 50%

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2
Q

Why does jaundice affect so many newborns? (3)

A

There is a release of haemoglobin from breakdown of RBCs at birth, and RBC lifespan is shorter in neonates than adults along with a less effective bilirubin metabolism

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3
Q

What is kernicterus?

A

Encephalopathy resulting from deposits of unconjugated bilirubin in the basal ganglia if at very high levels, can be fatal

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4
Q

What is the level at which clinical jaundice occurs?

A

80umol/L

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5
Q

What is the main cause of jaundice <24 hours?

A

Haemolysis i.e. rhesus haemolytic disease, ABO incompatibility, G6PD deficiency, congenital infection
Bilirubin is unconjugated and can rise rapidly

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6
Q

What is the cause of jaundice at 2 days - 2 weeks?

A

Physiological

Possibly breast milk jaundice, dehydration, infection

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7
Q

What is the cause of jaundice over 2 weeks? (5)

A

Physiological/breast milk, infection, hypothyroidism, obstruction, neonatal hepatitis

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8
Q

How is jaundice investigated? (3)

A

Blanch skin
Transcutaneous bilirubin meter/bloods
Check bilirubin levels against age and check rate of change
Assess risk factors

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9
Q

How is jaundice managed?

A

Phototherapy if moderate

If severe exchange transfusion

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10
Q

What is biliary atresia?

A

Cause of prolonged neonatal jaundice!, is a progressive disease with destruction or absence of the extrahepatic biliary tree and intrahepatic ducts

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11
Q

Symptoms of biliary atresia? (5)

A 
Failure to thrive
Jaundice
Pale stools
Dark urine
Hepatosplenomegaly
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12
Q

How is biliary atresia investigated? (4)

A

LFTs
USS
Liver biopsy
Confirmed with laparotomy

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13
Q

How is biliary atresia treated?

A

Surgical bypass of the fibrotic ducts - success worse with age
Liver transplant

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14
Q

What are red flags in vomiting? (7)

A 
Bile stained
Bloody
Projectile
Painful
Distension
Dehydration
Failure to thrive
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15
Q

Causes of vomiting in infants? (8)

A 
GORD
Infection
Feeding problems
Obstruction
Intolerances
Metabolic errors
Congenital adrenal hyperplasia
Renal failure
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16
Q

Causes of vomiting in young children? (7)

A 
Gastroenteritis or other infection
Appendicitis
Obstruction
Increased intracranial pressure
Coeliac
Metabolism errors
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17
Q

Caused of vomiting in school age children? (10)

A 
Gastroenteritis/infection
H.pylori/ulcers
Appendicitis
Migraine
DKA
Increased ICP
Coeliac
Alcohol
Pregnancy 
Anorexia
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18
Q

What is gastro-oesophageal reflux?

A

Involuntary passage of gastric contents into the oesophagus

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19
Q

Why is GORD common in infancy?

A

Inappropriate relaxation oft he lower oeseophageal sphincter due to immaturity of the muscle, usually resolves by 1 year

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20
Q

What factors worsen GORD in infants? (3)

A

Fluid diet
Horizontal position
Short oesophagus

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21
Q

3 complications of GORD?

A

Failure to thrive
Oesophagitis
Aspiration

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22
Q

How is GORD diagnosed? (4)

A

Clinically
24hr pH monitoring if atypical
Endoscopy and biopsy
Contrast studies

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23
Q

How is uncomplicated GORD treated?

A

Add thickening agents to feeds and head up position maintained

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24
Q

How is severe GORD treated? (4)

A

H2 receptor antagonist ranitidine
Proton pump inhibitor
Investigate for intolerances
Surgery - Nissen fundoplication if refractory to reinforce closing of the sphincter

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25
What does H.pylori infection cause in children? (3)
Nodular antral gastritis | Can uncommonly cause duodenal ulceration
26
Symptoms of gastritis? (3)
Abdominal pain Nausea Epigastric pain if duodenal
27
Investigation of gastritis? (3)
Carbon 13 breath test Stool sample Possible biopsy
28
How is gastritis infection treated?
Proton pump inhibitor | If H.pylori present, add a macrolide and amoxicillin
29
Management of recurrent gastritis?
Endoscopy - if normal, diagnose functional dyspepsia | Can try hypoallergenic diet
30
Why are childhood UTIs important to diagnose and investigate?
Up to 50% have a structural abnormality of the urinary tract | If pyelonephritis develops, can damage and scar kidneys
31
How do UTIs present in infants? (8)
``` Fever Vomiting Irritability Failure to thrive Jaundice Septicaemia Smelling urine Convulsions ```
32
How do UTIs present in children? (11)
``` Dysuria Frequency Loin pain Fever Anorexia Vomiting Haematuria Cloudy/smelling urine Convulsion Enuresis FTT! ```
33
How are UTIs diagnosed?
MSU for MC+S, dipstick
34
What are common organisms causing childhood UTIs? (4)
E.coli Klebsiella Proteus (esp in boys) Pseudomonas
35
Management of initial UTI? (5)
Antibiotics USS of kidneys ureters and bladder If <1 year also do micturating cystourethrogram (MSUG) and DMSA radionuclide scan! 1-3 years DMSA Over 3 years nothing else if USS normal and infection wasn't atypical/severe
36
How are UTIs treated?
<3 months IV cefotaxime >3 months pyelonephritis 7-10 days oral ciprofloxacin or co-amoxiclav >3 months cystitis 3 days oral trimethoprim or nitrofurantoin
37
Except structural, 2 other causes of UTIs in children?
Incomplete bladder emptying | Vesicoureteric reflux
38
What is nephrotic syndrome?
Heavy proteinuria, resulting in low plasma albumin and oedema and hyperlipidaemia Heavy proteinuria. Hypoalbuminaemia. Peripheral oedema.
39
What causes nephrotic syndrome?
Glomerulonephritis | May be secondary to Henoch-Schonlein purpura, SLE, infections, diabetes
40
What is glomerulonephritis?
Inflammation and damage to the glomeruli, allowing protein/blood to leak into the urine, can present with nephrotic or nephritic syndrome
41
What types of glomerulonephritis primarily case nephrotic syndrome and why?
Minimal change glomerulonephritis - abnormal podocytes (80% of nephrotic syndrome) Focal segmental glomerulonephritis - sclerosed glomeruli Membranous glomerulonephritis - thickened basement membrane
42
Signs of nephrotic syndrome?
Oedema - periorbital, scrotal/vulval, ankle/leg, ascites | Breathlessness - pleural effusions, abdominal distension
43
Investigations of nephrotic syndrome? (5)
``` Dipstick urine for MC and S FBC, ESR, U+E, autoantibodies Creatinine and albumin Throat swab Hep B and C Renal USS ```
44
What is the treatment for minimal change GN (nephrotic)? (3)
Prednisolone at 60mg/m2 for 4 weeks then 40 on alternate days for 4 weeks Reduce oedema - fluid management, salt restriction, give diuretic and ACE inhibitor Statin for hyperlipidaemia
45
What is the treatment of focal segmental GN (nephrotic)? (4)
Salt restriction and diuretics ACE inhibitor Statin for hyperlipidaemia Transplant often required - steroids DONT work
46
What is the treatment of membranous GN (nephrotic)?
Prednisolone | 1/3 have chronic GN, 1/3 remission, 1/3 renal failure
47
What are some complications of nephrotic syndrome? (4)
Hypovolaemia Thrombosis Infection Hypercholesterolaemia
48
What is nephritic syndrome?
Haematuria with RBC casts in urine, hypertension, oliguria, small proteinuria - due to glomerular inflammation Can rapidly progress to kidney failure
49
What causes nephritic syndrome?
Glomerulonephritis
50
What types of glomerulonephritis primarily cause nephritic syndrome? (4)
IgA nephropathy Post infectious (streptococcal) GN Membranoproliferative GN Rapidly progressive GN
51
What are the types of rapidly proliferative glomerulonephritis (nephritic)? (3)
Goodpasture's syndrome Microscopic polyangitis Grnaulomatosis with polyangitis
52
How is nephritic syndrome generally treated?
Monitor fluids and electrolytes Diuretics Possible steroids If rapidly progressive - biopsy, immunosuppression and steroids, plasma exchange
53
What is post streptococcal (infectious) GN (nephritic)?
Usually follows weeks after sore throat (strep pyogenes) | Returns to normal <1 month
54
What is IgA nephropathy (nephritic)?
May present with macroscopic haematuria 24-48 hours post URTI infection 20% have end stage renal failure
55
What is Goodpasture's syndrome (nephritic)?
Autoimmune ant glomerular basement membrane antibodies Also affects lungs Treat with steroids, plasmaphoresis
56
What is microscopic polyangitis (nephritic)?
Vasculitic p-ANCA+ve, affects small vessels Treat long term steroids and cytotoxics
57
What is Wegener's granulomatosis/granulomatosis with polyangitis (nephritic)?
Vasculitic c-ANCA+ve Affects lungs and kidneys Treat with steroids, plasmaphoresis, cyclophosphamide
58
What type of diabetes do children get?
Type 1 (rare type 2)
59
What causes diabetes mellitus?
Genetic disposition - family history, HLADR3/4 and other autoimmune association Environmental - enteroviral infection, diet (cow milk proteins), overnutrition
60
When does diabetes mellitus present?
2 peaks - preschool and late teens
61
How does diabetes mellitus present? (5)
``` Polyuria Polydipsia Weight loss Young children - wet the bed, candida infection DKA uncommon ```
62
How is diabetes mellitus diagnosed? (6)
``` In symptomatic children with random blood glucose >11.1mmol/L Glycosuria Ketonuria Fasting blood test >7mmol/L HbA1c >48 ```
63
How are most new cases of diabetes mellitus managed? (5)
SC insulin injections with education, BM monitoring Reduced carb diet Adjustment for illness, exercise Hypoglycaemia education
64
Types of insulin regimes? (4)
Rapid acting Short acting - before meals lasting 8 hours Intermediate lasting 12 hours Mixed
65
How can insulin be delivered?
Continuously via a pump | Injections - watch for fatty change
66
What is the most common insulin regime in children?
Pump or 3/4 short acting injections a day
67
How are hypoglycaemic attacks managed?
Glucose tablets/glucogel/glucagon injection
68
Complications of diabetes mellitus? (7)
``` Retinopathy Nephropathy Hypertension Neuropathy Coeliac/thyroid disease Delayed puberty Obesity ```
69
Signs of diabetic ketoacidosis? (7)
``` 'Pear drop' breath Vomiting Dehydration Abdominal pain Hyperventilation Shock Coma ```
70
How is DKA managed? (6)
``` Fluids slowly over 48 hours Insulin infusion titrated with BM Give potassium Re-establish oral fluids, diet, SC insulin Identify cause ```
71
What causes type 1 diabetes?
Autoimmune destruction of the insulin producing beta cells of the islets of Langerhans in the pancreas
72
What causes type 2 diabetes?
Combination of inadequate insulin secretion by beta cells and peripheral insulin resistance
73
How common is undescended testicles?
4% of males at birth, more if preterm | 1.5% by 3 months
74
What are the types of undescended testicles? (4)
Retractile - normally descend with age Palpable in groin but can't be manipulated in scrotum Impalpable and may be in inguinal canal/abdomen Absent
75
Investigations of undescended testicles?
Ultrasound Hormonal tests if impalpable/absent Laparoscopy
76
Treatment of undescended testicles?
Orchidopexy - surgery for fertility reasons and to reduce malignancy
77
When should testicles be descended in the baby?
3-6 months
78
What is torsion of the testes?
Spermatic cord rotates and becomes twisted, cutting off testicle blood supply - emergency Most common in adolescents
79
Symptoms of testicular torsion?
Groin/scrotal/abdominal pain | Swelling
80
How is testicular torsion treated?
Within 6 hours to save testicle | Surgical exploration, and if confirmed fix both testicles in place
81
What is bell clapper deformity?
Tunica vaginalis joins high on the spermatic cord, so the testes are not anchored
82
What is hydrocoele?
Fluid around the testicles, caused by patent processus vaginalis, can occur after illness
83
Symptoms of hydrocoele? (4)
Swelling Bluish discolouration Non tender Transilluminate
84
How is hydrocoele treated?
Most resolve but surgery if persistent after 2 years
85
What is varicocoele?
Varicosities of testicular veins, often around puberty, associated with decreased fertility
86
How is varicocoele treated?
Surgery if painful or impaired growth
87
What is congenital adrenal hyperplasia?
Group of inherited conditions where the adrenal gland is large, with a lack of cortisol and increase in androgens
88
What effects does CAH cause? (4)
Male characteristics to appear early in boys and inappropriately in girls Less able to cope with stress Hyponatraemia, hypoglycaemia
89
What is the mechanism behind CAH? (3)
Lack of 21-hydroxylase deficiency for cortisol synthesis Inability to produce aldosterone Cortisol deficiency stimulates pituitary to produce ACTH increasing androgens
90
In what patients is CAH more common?
Consanguineous
91
How does CAH present? (5)
Virilisation of female genitalia Large penis and pigmented scrotum in males 80% salt losing crisis Male non salt losers - tall stature Female non salt losers - muscular, BO, pubic hair, acne
92
What is a salt losing (adrenal) crisis? (6)
``` Vomiting Weight loss Shock Hyponatraemia, hyperkalaemia, hypogylcaemia Metabolic acidosis ```
93
How is CAH treated? (5)
Females - corrective surgery externally Glucocorticoids, mineralocorticoids NaCl if salt losing Additional corticosteroids if ill
94
How is a salt losing crisis treated? (3)
Hydrocortisone Saline Glucose IV
95
What is turners syndrome?
45X, female only has one normal X chromosome, normally spontaneous
96
How is turners syndrome diagnosed?
Antenatally 95% miscarry Clinically
97
How does turners syndrome present? (9)
``` Short stature Lymphoedema of extremities Neck webbing Wide carrying angle Wide spaced nipples Coarctation of aorta Delayed puberty Infertility Hypothyroidism ```
98
How is turners syndrome treated? (3)
Growth hormone Oestrogen replacement ART for fertility
99
What is Edward's syndrome?
Trisomy 18, most die in infancy
100
Symptoms of Edward's syndrome? (7)
``` Low birthweight Prominent occiput Small mouth/chin Short sternum Overlapping flexed fingers Rocker bottom feet Heart/kidney malformation ```
101
What is Patau syndrome?
Trisomy 13, most die in infancy
102
Symptoms of Patau syndrome? (6)
``` Brain maldevelopment Scalp defects Small defected eyes Cleft lip Polydactyly Heart/kidney defects ```
103
What is Klinefelter syndrome?
47, XXY affects 1-2/1000 males
104
Symptoms of Klinefelter syndrome? (5)
``` Infertility, hypogonadism Small testes Gynaecomastia Tall Psychological problems ```
105
What is William's syndrome?
Deletion of 27 genes on chromosome 7, usually sporadic
106
Symptoms of Williams syndrome? (5)
``` Short flat nasal bridge Elf like appearance Learning difficulties Heart disease Happy demeanour ```
107
What is failure to thrive?
Suboptimal weight gain in infants and toddlers
108
Causes of failure to thrive? (5)
``` Inadequate intake Inadequate retention Malabsorption Failure to utilise Increased requirement ```
109
Causes of inadequate intake?
Non organic (environmental) or organic
110
Causes of environmental inadequate intake? (5)
``` Feeding problems Inadequate milk Low socioeconomic status Maternal depression or poor education Neglect or child abuse ```
111
Causes of organic inadequate intake? (4)
Impaired suck/swallow (oromotor dysfunction) Neurological problems Cleft palate Chronic illness - cystic fibrosis, Crohn's
112
Causes of inadequate retention? (2)
GORD | Vomiting
113
Causes of malabsorption? (5)
``` Coeliac Cystic fibrosis Cow's milk protein allergy NEC Short gut ```
114
Causes of failure to utilise? (4)
Chromosomal disorders Prematurity Infection Metabolic disorders
115
Causes of increased requirement? (6)
``` Hyperthyroid Cystic fibrosis Malignany Chronic infection Severe combined immunodeficiency Heart/renal disease ```
116
Management of failure to thrive?
Detailed history and examination for underlying disease - then treat MDT care Education and monitoring if no cause
117
Management of severe failure to thrive?
If <6 months and severe, admit for refeeding
118
What is a choledochal cyst?
Cystic dilatations of the extrahepatic biliary system
119
How does a choledocal cyst present? (4)
Abdo pain Mass Jaundice Cholangitis
120
How is a choledocal cyst treated?
Surgical excision and anastomosis to biliary duct
121
What is neonatal hepatitis syndrome?
Prolonged neonatal jaundice and hepatic inflammation, may have IUGR and hepatosplenomegaly
122
Causes of neonatal hepatitis syndrome?
``` Congenital infection Metabolism errors Alpha antitrypsin deficiency Cystic fibrosis Galactosaemia ```
123
What is acute liver failure?
Development of massive hepatic necrosis with subsequent loss of liver function
124
Symptoms of acute liver failure? (7)
``` Jaundice Encephalopathy Coagulopathy Hypoglycaemia Electrolyte disturbance Haemorrhage Cerebral oedema ```
125
Management of acute liver failure? (4)
Maintain blood glucose Prevent sepsis - Abx Vitamin K to prevent haemorrhage Liver transplant
126
Causes of chronic liver disease in children? (7)
``` Hepatitis B or C or autoimmune Drugs - NSAIDs IBD Wilson disease Cystic fibrosis Alpha antitrypsin deficiency Neonatal liver disease ```
127
Symptoms of chronic liver disease?
``` Acute hepatitis Hepatosplenomegaly Cirrhosis Portal hypertension Lethargy, malnutrition ```
128
Management of liver disease?
``` Treat specific causes Adequate nutrition Fat soluble vitamins ADEK Ursodeoxycholic acid for itching Liver transplant ```
129
What is congenital nephrotic syndrome?
Inherited disorder, infants present with LBW, oedema, hypoalbuminaemia, proteinuria, often leads to kidney failure
130
What is haemolytic uraemic syndrome?
Triad of acute renal failure, microangiopathic haemolytic anaemia and thrombocytopaenia
131
Cause of haemolytic uraemic syndrome?
Typically secondary to GI infection with e.coli of shigella Bacteria cause intravascular thrombogenesis in kidneys Coagulation pathway activated, platelets consumed RBC damage through occluded vessels
132
Symptoms of haemolytic uraemic syndrome? (7)
``` Diarrhoeal prodrome (bloody) Haematuria Oliguria Hypertension Abdo pain, vomiting Oedema Possible purpura ```
133
Complications of HUS?
Acute kidney injury
134
Treatment of HUS? (2)
Early supportive therapy | Dialysis
135
What is acute kidney injury?
Acute renal failure where there is a sudden reversible reduction in renal function
136
Types of AKI?
Prerenal (commonest in paeds) Renal Postrenal
137
Prerenal AKI causes? (5)
``` Gastroenteritis Burns Sepsis Nephrotic syndrome Circulatory failure Anything that causes hypovolaemia ```
138
Renal AKI causes? (4)
Haemolytic uraemic syndrome Acute tubular necrosis Renal vein thrombosis Glomerulonephritis
139
Postrenal AKI causes?
Obstruction
140
Treatment of AKI? (3)
Prerenal - correct hypovolaemia, circulatory support Renal - restrict fluids, diuretic, high calorie diet Postrenal - nephrostomy, bladder catheterisation, surgery Dialysis
141
When is dialysis indicated? (5)
``` Failure of treatment Severe electrolyte disturbances Pulmonary oedema or hypertension Severe acidosis Multisystem failure ```
142
Causes of chronic renal failure? (4)
Structural malformations Glomerulonephritis Hereditary nephropathies Systemic diseases
143
Presentation of chronic renal failure? (6)
``` Anorexia Lethargy, failure to thrive Polydipsia and polyuria Bony deformities - renal rickets AKI Anaemia ```
144
Management of chronic renal disease? (8)
``` Nutrition Vitamin D, restrict phosphate Salt and fluids Bicarbonate supplements Erythropoietin Give growth hormone Dialysis Transplant ```
145
What is renal agenesis?
Absence of both kidneys causing reduced fetal urine and oligohydramnios = fetal compression - Potter syndrome
146
What is multicystic dysplastic kidney?
Failure of union of the ureteric bud with the nephrogenic mesenchyme, non functioning, large cysts, no connection with bladder
147
What are autosomal dominant and recessive polycystic kidneys?
Large cystic kidneys that maintain some function but predisposed to renal failure
148
What is a horseshoe kidney?
Pelvic kidney - abnormal caudal migration, in midline, may predispose to infection or obstruction
149
What are duplex kidneys?
Premature division of the ureteric bud, varies from a bifid renal pelvis to complete division with 2 ureters, often have abnormal drainage
150
Management of renal malformations? (4)
``` Prophylactic antibiotics at birth to prevent UTI USS Treat chronic kidney disease/AKI Dialysis Transplant ```
151
What is vesicoureteric reflux?
Developmental abnormality of the vesicoureteric junctions, ureters are laterally displaced and shortened and enter directly into bladder rather than angled
152
What does VUR cause?
Reflux into the renal system - can cause distended ureters and renal pelvis
153
Treatment of VUR? (3)
Many resolve on own with growth Treat infections Surgery - ureteric valve
154
What is Alport syndrome?
Familial nephritis, X linked recessive that progresses to end stage renal failure, associated with deafness and ocular defects
155
What is SLE?
Systemic lupus erythematosus, autoimmune disease, may present with haematuria or proteinuria
156
Effect of SLE on kidneys?
AKI, CKD from lupus nephritis | Immune complex deposition along the glomerular basement membrane
157
What is Henoch Schonlein purpura?
Inflammation of small blood vessels - vasculitis, possibly due to IgA and IgG complexes and complement activation Cause of nephritis
158
Symptoms of Henoch Schonlein purpura? (6)
May be preceded by URTI Purpuric palpable rash over buttocks, extensor surfaces of arms and legs, ankles - TRUNK SPARING Joint pain in knees and ankles Joint swelling Abdominal pain Glomerulonephritis - haematuria, possible proteinuria
159
Treatment of Henoch Schonlein purpura? (4)
``` Fluid and electrolyte balance Diuretics Possible steroids for abdo pain Monitor for deterioration after resolution Self limiting ```
160
What is impetigo?
Localised highly contagious staphylococcal and/or streptococcal skin infection, more common where there is pre-existing skin disease
161
Lesions in impetigo?
On the face, neck and hands and begin as erythematous macules which may become vesicular/pustular or blistered, drainage leaves honey-coloured crusted lesions
162
Treatment of impetigo?
Topical Abx for mild | Systemic flucloxacillin or co-amoxiclav for severe
163
What is scalded skin syndrome?
Caused by staphylococcus causing separation of the epidermal skin from underlying layers, widespread erythema and tenderness, treat with IV abx
164
What is whooping cough?
Highly contagious respiratory infection caused by Bordatella pertussis, epidemics every 3-4 years
165
Symptoms of whooping cough? (5)
Week of coryza, then development of characteristic paroxysmal cough followed by inspiratory whoop Cough worse at night, may vomit Red/blue in face when coughing, may have epistaxis Infants - apnoea instead of whoop May decrease but persist for months
166
Management of whooping cough?
If severe, isolated in hospital Erythromycin - only helps symptoms if started in coryzal phase Close contacts - erythromycin prophylaxis
167
Name 2 diseases eradicated by the vaccination programme?
Diptheria, polio
168
What are the major routes of HIV transmission to children?
Mother to child transmission - intrauterine, intrapartum, breastfeeding Infected blood products Rare - contaminated needles,sexual abuse
169
Diagnosis of HIV in children? (2)
Transplacental maternal IgG HIV detection if <18 months and mum has HIV shows exposure HIV DNA PCR positive shows infection
170
Features of HIV in children? (5)
Some progress rapidly to AIDS wihin a year Some asymptomatic for years before progressing Lymphadenopathy Recurrent bacterial infections or candidiasis Lymphocytic interstitial pneumonitis
171
Indications of severe HIV infection? (4)
Pneumocystis jiroveci (carinii) pneumonia (PCP) Severe failure to thrive Encephalopathy Malignancy
172
With what symptoms should you test for HIV? (6)
``` Persistent lymphadenopathy Hepatosplenomegaly Recurrent fever Parotid swelling Thrombocytopaenia Serious, persistent, unusual, recurrent infections ```
173
Treatment of HIV? (4)
Infants should start antiretroviral therapy after diagnosis Older children based on viral load and CD4 count Co-trimoxazole for PCP prophylaxis Up to date immunisations (not BCG as live)
174
What is encephalitis?
Inflammation of the brain, caused by viruses such as HSV, post-infectious encephalopathy after VZV, slow virus such as HIV
175
Symptoms of encephalitis? (4)
Fever Altered consciousness Seizures Behaviour change
176
Treatment of encephalitis?
High dose aciclovir IV
177
What does candida infection commonly cause in children?
Nappy rash - erythematous, involves the flexures, treat with topical antifungal
178
What is irritant dermatitis?
Nappy rash, affects convex surfaces of buttocks perineum abdomen and thighs but flexures are spared, use emollient or topical steroids
179
What is toxic shock syndrome?
Caused by staph aureus and group A strep, gives fever hypotension and a diffuse erythematous macular rash and can cause organ dysfunction
180
Treatment of toxic shock syndrome?
Surgical debridement of affected areas | Ceftriaxone and clindamycin
181
What is scarlet fever?
Caused by group A strep infection, can lead to rheumatic fever/post strep glomerulonephritis/reactive arthritis
182
Symptoms of scarlet fever? (6)
Sore throat, fever, headaches, swollen lymph nodes, and a characteristic rash - red and like sandpaper, starts on face can spread to body Strawberry tongue
183
Treatment of scarlet fever?
Antibiotics - penicillin/amoxicillin
184
What is coxsackie disease?
Hand foot and mouth disease - flu like symptoms, blisters
185
How is coxsackie treated?
Supportive | Watch for meningitis/encephalitis
186
Types of immune deficiency?
Primary - intrinsic defect, mostly inherited Secondary - caused by another disease/treatment such as infection, HIV, malignancy, immunosuppression, splenectomy, malnutrition
187
Presentation of immune deficiency? (6)
``` Recurrent bacterial infections Severe infections Atypically presenting infections Failure of treatment for infections Unexpected causative pathogen Extensive candidiasis Severe, Prolonged, Unusual or Recurrent ```
188
Management of immune deficiency? (5)
``` Antimicrobial prophylaxis Prompt treatment of infection, lower course abx Screen for end organ disease Ig replacement therapy Bone marrow transplantation - for SCID ```
189
What are febrile seizures?
Seizure accompanied by a fever in the absence of intracranial infection due to bacterial meningitis or viral encephalitis, occur in 3% between 6 months and 5 years
190
Features of febrile seizures?
Occur early into a viral infection when temp rises Usually brief generalised tonic clinic If focal, prolonged or repeated in same illness risk of epilepsy increased
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Management of febrile seizures? (3)
Infection screen If unconscious/unstable, abx If prolonged, rescue rectal diazepam
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What is premature sexual development?
Development of secondary sexual characteristics before 8 in girls and 9 in boys
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What is precocious puberty?
Premature sexual development either gonadotrophin-dependent with premature activation of HPG axis or gonadotrophin-independent from excess sex steroids
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Cause of precocious puberty?
Females - premature onset of puberty | Male - more often organic cause such as intracranial tumour, adrenal hyperplasia, gonadal tumour
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Management of precocious puberty?
``` Detect and treat underlying pathology Reduce rate of skeletal maturation Address psychological/behavioural issies Gonadotrophin-dependent: GnRH analogues Gonadotrophin independent: Androgen inhibitors medroxyprogesterone acetate ```
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What are thelarche and pubarche?
Thelarche - breast development | Pubarche - pubic hair development
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What is Prader-Willi syndrome?
Genetic disorder caused by loss of function of genes, mostly due to paternal partial deletion of chromosome 15 - imprinting disorder
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Features of Prader-Willi syndrome? (8)
``` Characteristic facial features Hypotonia Feeding problems FTT Obesity Hypogonadism Developmental delay Learning difficulties ```
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What is Noonan syndrome?
Mutation in one of several autosomal dominant genes
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Features of Noonan syndrome? (6)
``` Characteristic facial features Mild learning difficulty Short webbed neck, trident hair line Pectus excavatum Short stature Congenital heart disease ```
201
What is Angelman syndrome?
Loss of function of part of a maternal copy of chromosome 15 - imprinting disorder
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Features of Angelman syndrome?
Cognitive impairment Characteristic facial appearance Ataxia Epilepsy
203
What is muscular dystrophy?
Group of inherited disorders with muscle degeneration that is often progressive
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What is Duchenne muscular dystrophy?
Most common dystrophy, X linked recessive so affects males, deletion of a site of the short arm of the X chromosome that codes for dystrophin
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What does dystrophin do?
Connects the cytoskeleton of the muscle fibre to the surrounding extracellular matrix through the cell membrane - deficiency leads to myofibre necrosis
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Presentation of Duchenne muscular dystrophy? (7)
``` Waddling gait Language delay Difficulty running, with stairs Pseudohypertrophy of calves due to fat replacement Clumsiness Scoliosis Learning difficulty ```
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Complications of Duchenne muscular dystrophy?
Limited life due to respiratory failure or cardiomyopathy
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Management of Duchenne muscular dystrophy? (6)
Exercise and stretching Night splints to prevent contractures Orthoses and Achilles lengthening to help walk Brace for scoliosis/metal rod insertion Overnight CPAP Corticosteroids 10 days a month to preserve mobility
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What are causes of hypogonadotrophic hypogonadism? (4)
Systemic disease - cystic fibrosis, asthma, Crohns, anorexia, excess training Panhypopituitarism Isolated gonadotrophin deficiency Acquired hypothyroidism
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What are causes of hypergonadotrophic hypogonadism? (3)
Chromosomal abnormalities - Klinefelters, Turners Steroid hormone enzyme deficiencies Gonadal damage - surgery, chemo, trauma
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What is delayed puberty?
Absence of pubertal development by 14 in females 15 in males, more common in males due to constitutional delay
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Caused of delayed puberty? (3)
Constitutional Hypogonadotrophic hypogonadism - low gonadotrophic secretion, problem with pituitary/hypothalamus Hypergonadotrophic hypogonadism - high gonadotrophin secretion, problem with gonadal response to hormones
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Management of delayed puberty? (4)
Karyotyping Thyroid, sex hormone test Treatment not normally needed - if so, oxandrolone for boys (weak androgen steroid) or IM testosterone For girls, oestradiol
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How is childhood obesity defined?
BMI >98th centile for age and sex, >91st centile is overweight
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Complications of obesity?
Type 2 diabetes | CV disease
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2 exogenous causes of obesity?
Hypothyroidism | Cushings disease
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Management of obesity? (4)
Sustained changes in lifestyle - healthier eating, increased physical activity, reduction in inactivity Drug treatment if extreme obesity and over 12 or complications of obesity, after lifestyle changes - orlistat (lipase inhibitor), metformin (increases insulin sensitivity) Bariatric surgery e.g. gastric banding
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What is respiratory distress syndrome?
RDS - deficiency of surfactant which lowers surface tension in the lungs, more common with increasingly early birth
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Surfactant physiology?
Mixture of phospholipids and proteins Excreted by type II pneumocytes of the alveoli Deficiency leads to alveolar collapse and inadequate gas exchange
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Prevention of RDS?
Give mother glucocorticoids if preterm delivery anticipated
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Signs/symptoms of RDS? (4)
``` Tachypnoea >60bpm Laboured breathing, chest recession, nasal flaring Expiratory grunting Cyanosis Within 4hr of birth ```
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What is shown on X ray in RDS?
Bilateral pneumothorax
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Treatment of RDS? (2)
Oxygen - possibly CPAP, mechanical ventilation | Surfactant therapy into lung via tracheal tube
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What is bronchopulmonary dysplasia?
Infants who still have oxygen requirement at 36 weeks post gestation - chronic lung disease
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Cause of bronchopulmonary dysplasia? (3)
Pressure/volume trauma from artificial ventilation Oxygen toxicity Infection
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Treatment of bronchopulmonary dysplasia?
CPAP oxygen at home | Short course corticosteroids for earlier ventilation weaning
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What is meconium aspiration?
Meconium inhaled by the infant at birth, particularly if prolonged gestation or fetal hypoxia due to gasping
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Complications of meconium aspiration? (4)
Meconium is a lung irritant Results in mechanical obstruction and chemical pneumonitis Predisposes to infection High incidence of air leak - pneumothorax Persistent pulmonary hypertension of the newborn
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Management of meconium aspiration? (2)
Suction airways | Artificial ventilation
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What are the TORCH infections?
``` Toxoplasmosis Other - syphilis, VZV, parvovirus Rubella Cytomegalovirus Herpes ```
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What are the TORCH infections associated with?
Common viruses associated with congenital anomalies
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What is hypoxic-ischaemic encephalopathy?
Hypoxic-ischaemic injury to the brain due to perinatal asphyxia compromising gas exchange and cardiac output, causing brain damage or death
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Causes of HIE? (5)
Placental exchange failure - prolonged contractions, abruption, ruptured uterus Interruption of umbilical blood flow - shoulder dystocia, cord prolapse Maternal hypo/hypertension IUGR, fetal anaemia Failure of cardiorespiratory adaptation at birth
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Symptoms of HIE?
Mild - irritable, excessive response to stimulation, impaired feeding, hyperventilation Moderate - marked abnormalities of tone and movement, cannot feed Severe - seizures, no normal movements or response to pain, hypo and hypertonic limbs, multi organ failure
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Management of HIE? (6)
``` Respiratory support EEG Anticonvulsants Fluid restriction Treat electrolyte imbalances Cooling if within 6hr of birth ```
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Prognosis of HIE?
Complete recovery if mild If abnormalities persist over 2 weeks, full recovery unlikely Severe has mortality of 30-40% and 80% have disability such as cerebral palsy
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What is necrotising enterocolitis?
Mostly affects preterm infants, associated with bacterial invasion of ischaemic bowel wall, more likely if fed cows milk formula
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Symptoms of NEC? (6)
``` Infant stops tolerating feeds Milk aspiration from stomach Vomiting may be bile stained Distended abdomen Fresh blood in stool Shock ```
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What is shown on X ray in NEC? (4)
Distended loops of bowel Thickening of bowel wall with intramural gas Air in hepatic portal tract Air under diaphragm if bowel perforation
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Management of NEC? (4)
Stop feeding orally - parenteral Broad spectrum antibiotics Artificial ventilation, circulatory support Surgery if bowel perforation - resection
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How is newborn listeria monocytogenes transmitted?
To the mother from food such as unpasteurised milk, soft cheese, undercooked poultry
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Symptoms of listeria monocytogenes infection in the newborn? (8)
``` Spontaneous abortion Preterm delivery Sepsis Meconium stained liquor Widespread rash Pneumonia Meningitis ```
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When is hypoglycaemia in a newborn more likely? (7)
``` IUGR Preterm Mother has diabetes mellitus Large baby Hypothermic Polycythaemic Ill for any reason ```
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Why do diabetic mothers have hypoglycaemic babies?
Sufficient glycogen stores (as opposed to preterm/IUGR infants) but hyperplasia of pancreatic islet cells causes high insulin levels
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Symptoms of hypoglycaemia in the newborn? (6)
``` Jittery Irritable Apnoea Lethargy Drowsiness Seizures ```
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Management of newborn hypoglycaemia?
Early frequent milk feeding | IV glucose if very low
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What causes cleft lip or palate?
Cleft lip - failure of fusion of the frontonasal and maxillary processes Cleft palate - failure of fusion of the palatine processes and nasal septum
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Management of cleft lip/palate? (2)
Surgery in first few weeks/months | Special teats/feeding devices

3A REVISION (17 decks)

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