Paeds GASTRO Flashcards

1
Q

What is the cause of GOR in babies?

A

Inappropriate relaxation of the LOS (functional immaturity)

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2
Q

By when does GOR usuallly resolve?

A

12 months: if persistent, may be due to GORD

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3
Q

How is GOR diagnosed?

A

Clinical dx
24 hour LOS pH monitoring (it should remain > 4)
OGD

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4
Q

Recall the factors affecting choice to refer for GOR

A

Same day referral if haematemesis, melaena or dysphagia

Assess by paediatrician if there are:
1. Red flags (eg faltering growth)
2. Unexplained IDA
3. No improvement after 1 y/o
4. Feeding aversion
5. Suspected Sandifer’s syndrome

Refer if there are complications

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5
Q

Recall the management options for GOR

A
  1. Reassure: it’s v common!
  2. Must sleep on back
  3. If breast fed: assess breast-feeding, consider alginate for 1-2 weeks, if not: pharmacology

If formula-fed: review feeding hx, try a smaller, more frequent feed + thickened formula, if doesn’t work, try alginate

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6
Q

What safety net should you watch out for when assessing GORD?

A

Monitor vomit: if bloody or green seek medical attention

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7
Q

At what age does pyloric stenosis present?

A

2-8 weeks

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8
Q

Is pyloric stenosis more common in girls or boys?

A

Boys (4 x more common)

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9
Q

Recall a genetic association of pyloric stenosis

A

Turner’s syndrome

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10
Q

What is the main symptom of pyloric stenosis?

A

Projectile, non-billious vomiting

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11
Q

Recall 3 symptoms of pyloric stenosis other than vomiting

A

Weight loss
Depressed fontanelle from dehydration
Loss of interest in food

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12
Q

Recall some signs of pyloric stenosis

A

Palpable ‘olive’ mass
Visible peristalsis in upper abdomen

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13
Q

What will be the acid-base profile in pyloric stenosis?

A

Hypochloraemic, hypokalaemic metabolic alkalosis may progress to a dehydrated lactic acidosis (opposite biochemial picture)

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14
Q

What is the best investigation for pyloric stenosis?

A

USS: shows target lesion of >3mm thickness
Do ABG to guide management

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15
Q

How should pyloric stenosis be managed?

A
  1. IV slow fluid resuscitation + correct any disturbances:
    1.5 x maintenance rate
    5% dextrose
    0.45% saline
  2. Laparoscopic Ramstedt pyloromyotomy
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16
Q

What are the symptoms of colic?

A

Inconsolable crying + drawing up of the hands + feet: child remains distressed between episodes

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17
Q

What should be considered if the colic is persistent?

A

Cow’s milk protein allergy or reflux
Try:
2 week trial of hydrosylate formula followed by
2 week trial of anti-reflux tx

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18
Q

In what age group is appendicitis less common, and what is a more likely cause of similar symptoms in this age group?

A

Rare in under 3s, then it’s more likely to be faecolith (stony mass of impacted faeces)

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19
Q

Recall the management of appendicitis in children

A

GAME
G: group + save
A: Abx IV
M: MRSA screen
E: eat + drink NBM

Then laparoscopic appendectomy

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20
Q

What is intussusception?

A

Invagination of proximal bowel into distant component (telescoping distally)

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21
Q

What is the most common site of intussusception?

A

Ileum through to caecum through ileocaecal valve (ileo-colic) - 90% cases

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22
Q

Recall the appearance of stool in intussusception, and the pathophysiology of how this happens

A

Red-currant jelly (blood + mucus) due to venous obstruction + compression –> oedema + mucosal bleeding
This is a LATE sign

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23
Q

What are the causes of intussusception?

A

Idiopathic
Physiological lead point: Peyer’s patch
Pathological lead point: malignancy, Meckel’s diverticulum, Henoch-Schonlein purpura

Thought to have a seasonal occurrence with viral illness with inflammatory processes triggering abdominal inflammation.

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24
Q

What are the symptoms of intussusception?

A

Intermittent colicky pain
Vomit - depending on type: may be bile-stained or not
Bloody stools (may be a late sign)

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25
What are the signs of intussusception?
Abdominal distention with sausage shaped mass in RUQ Emptiness on palpation in RLQ (Dance's sign) Red-currant jelly stool is a late sign Signs of peritonitis if intussusception has been present a long time
26
What are the appropriate investigations for intussusception?
1. Abdo USS: may show donut sign (think: intUSSusception) 2. AXR (may be normal) 3. Barium/ gastrogaffin enema if have 1 of 3 Ps: Perforation, Peritonitis, Pale complexion
27
What would be a contraindication for an air contrast enema?
Signs of peritonitis or free fluid (perforation) on USS - requires urgent surgery HSP present
28
How should intussusception be managed?
It's an emergency If stable: - Fluid resuscitation - Enema: pneumatic - forces bowel to un-telescope - take x rays throughout If unstable: - Don't mess about with contrast, go in with open surgery - Remove any non-viable bowel
29
Is intussusception more commonly seen in males or females?
Males (2:1 ratio)
30
What age is intussusception most commonly seen?
Between 5 and 12 months
31
What should be done if there is recurrent intussusception?
Investigate for a lead point
32
When are intussusceptions with lead points commonly seen?
In patients with: HSP (intestinal wall haematoma) CF (hypertrophied intestinal mucosal glands) Lymphoma Peyer's patches (enlarged mesenteric lymph nodes) - seen in younger infants following respiratory or GI infection
33
What is Meckel's diverticulum?
Ileal remnant of vitello-intestinal duct on anti-mesenteric border containing ectopic gastric mucosa or pancreatic tissue
34
What is the rule used to remember all you need to know about Meckel's diverticulum?
Rule of twos 2 years old 2 x more common in boys 2 feet from ileocaecal valve 2 inches long 2 different mucosae (gastric + pancreatic)
35
What are the signs and symptoms of meckel's diverticulum?
Mostly asymptomatic Painless massive PR bleeding if it bleeds May show billious vomiting, dehydration + intractable constipation
36
How should meckel's diverticulum be investigated?
Technetium scan indicates increased uptake by gastric mucosa AXR or USS + laparoscopy
37
How should meckel's diverticulum be managed?
If asymptomatic, leave it alone! If symptomatic: Bleeding: excise diverticulum with blood transfusion Obstruction: excise diverticulum + lyse adhesions Perforation/ peritonitis: Excise with perioperative Abx
38
How may volvulus present?
1. At any age, after lying quiescent for ages 2. In first few days of life, with obstruction + possible compromised blood supply --> abdo pain, billious vomiting, peritonism etc
39
What is the main sign of volvulus on abdo examination?
Scaphoid abdomen
40
How should volvulus be investigated?
1. Upper GI contrast study (urgently) to assess patency if billious vomiting 2. USS
41
How should volvulus be managed?
Urgent laparotomy Untwist volvulus, mobilise the duodenum, place bowel in a good position + remove any necrotic bowel
42
What is the first thing to exclude in suspected IBS?
Coeliac
43
Recall the signs and symptoms of IBS
Abdo pain: often worse before or relieved by defaecation Explosive loose or mucus stools Bloating Tenesmus Constipation
44
Recall the 3 most common causes of paediatric gastroenteritis in decreasing prevalence
1. Rotavirus 2. Campylobacter 3. Shigella/ salmonella
45
If there is bloody diarrhoea in gastroenteritis, which microbes should be considered first?
CHESS organisms: Campylobacter Hemorrhagic E coli Entamoeba histolytica Salmonella Shigella
46
What investigations should be done in a case of gastroenteritis?
AXR to exclude other causes Stool sample analysis for viruses = stool electron microscopy for bacteria = stool culture
47
How should paediatric gastroeneteritis be managed?
Rehydration Learn maintenance fluid volumes: 0-10 kg = 100mls/kg 10-20kgs = 1000mls + 50ml/kg for each kg over 10kg 20+ kgs = 1500mls + 20 mls/kg for each kg over 20kgs If <5 use IV fluids + maintain with oral rehydration solution If >5, give 200mls after each
48
What is the safety netting for how long vomiting and diarrhoea should last?
Vomiting: usually 1-2 days, must stop within 3 days Diarrhoea: 5-7 days, must stop within 2 weeks
49
What is the most accurate marker of dehydration in children?
Weight loss
50
What is the threshold marker of dehydration for clinical dehydration and shock?
5-10% weight loss = clinical dehydration >10% weight loss = shock
51
Recall the symptoms of hypernatraemia
Mnemonic: f(ull) of salt Flushing Oedema Fever Seizures Agitation Low urine output Thirst
52
Recall the symptoms of hyponatraemia
SALT LOSS Stupor Anorexia Limp tone Tendon reflexes reduced Lethargy Orthostatic hypotension Seizures Stomach cramps
53
When are IV fluids (rather than ORS) indicated?
Shock Deterioration Persistent vomiting
54
What are the bolus fluids given in shock?
20mls/kg 0.9% saline over 15 mins (most situations) 10mls/kg 0.9% saline over 60 mins (trauma, fluid overload, heart failure)
55
Recall the day 1, 2, 3, 4, and 5 fluid resucitation requirements in neonates
Day 1: 50-60mls/kg/day Day 2: 70-80mls/kg/day Day 3: 80-100mls/kg/day Day 4: 100-120mls/kg/day Day 5: 120-150mls/kg/day
56
Which type of fluid should be used in fluid resus for term neonates?
Isotonic crystalloids with 10% dextrose
57
If giving IV fluids to a hypernatraemic child, what should be the biggest caution?
Take care with cerebral oedema Rapid reduction in plasma sodium concentration + osmolality will lead to a shift of water into cerebral cells May result in seizures + cerebral oedema
58
When should Abx be used in gastroenteritis?
Not even indicated when cause is bacterial Use when: - SEPSIS - salmonella < 6 months - C difficile with pseudomembranous colitis
59
What is the post-gastroenteritis syndrome and how can it be treated?
Introduction of a normal diet results in a return of watery diarrhoea Treat with oral rehydration therapy
60
What would be seen on biopsy in Crohn's?
Non-caseating epitheloid cell granulomata
61
Recall some important investigations to do for Crohn's disease
1. FBC including iron, folate and B12 2. Faecal calprotectin 3. Colonoscopy + biopsy (cobblestones)
62
How should Crohn's be treated?
Induce remission: Nutritional management Replace diet with whole protein modular diet: excessively liquid, for 6-8 weeks. The products are easily-digested and replace lost weight Pharmacological management: steroids (prednisolone)
63
What is the classical presentation of UC?
Rectal bleeding Diarrhoea Abdo pain
64
What are the appropriate investigations to do in ulcerative colitis?
Same as Crohn's 1. FBC including iron, folate + B12 2. Faecal calprotectin 3. Colonoscopy + biopsy
65
What does histology reveal in UC?
Mucosal inflammation/ ulceration Crypt damage
66
What scores can be used to score paediatric UC?
Paediatric UC Activity Index Truelove + Witts
67
What is one coexisting condition that it's important to be aware of in ulcerative colitis?
Depression
68
How should UC be managed?
1st line = oral aminosalicylates: may also be used to maintain remission 2nd line - oral corticosteroid 3rd line = oral tacrolimus Surgery in resistant disease
69
When does UC become an emergency?
In severe fulminating disease
70
What is the usual cause of toddler diarrhoea?
Underlying maturational delay in intestinal mobility
71
Recall some signs and symptoms of toddler diarrhoea
Varying consistency stools: well-formed to explosive + loose, may have bits of undigested vegetable Child will be well + thriving
72
How is toddler diarrhoea managed?
Increase fibre + fat in diet (whole milk, yoghurts, cheese) Avoid fruit juice + squash
73
What is the first-line management of constipation?
All first line: 1. Advise behavioural interventions (eg schedueled toileting, bowel habit diary, reward system) 2. Advise diet + lifestyle (adequate fluid intake) 3. Medication: step 1 = movicol paediatric plan (dose escalates for 2 weeks) Step 2: maintain for 6 months
74
Recall some important things to remember in PACES counselling for constipation
Explain movicol takes some time to work (dose increases over 2 weeks) Encourage child sitting on loo after mealtimes to use reflex Advise a star chart to aid motivation
75
What is Hirschprung's?
An absence of ganglion cells from the myenteric (Auerbach) + submucosal (Meissner’s) plexuses
76
Is there are gender predominance associated with Hirschprung's?
Male 4:1 ratio
77
Recall 2 risk factors for Hirschprung's
Down's Men2a
78
Recall some signs and symptoms of Hirschprung's
Failure to pass meconium in first 24 hours Explosive passage of liquid/ foul stools
79
If Hirschprung's doesn't present in first few days of life, what may happen?
May then present in a week or two with life-threatening Hirschprung's enterocolitis (C diff)
80
How should Hirschprung's be investigated?
1. AXR (if obstruction) 2. Contrast enema (showing dilated distal + narrowed proximal segments) 3. Definitive diagnosis is via suction-assisted full-thickness rectal biopsy showing absence of ganglion cells
81
When would an an enema be contraindicated in Hirschsprung's?
If there are signs of perforation or peritonitis
82
What is the aetiology of Hirschsprung's enterocolitis?
Proximal colonic dilatation secondary to obstruction (functional) with thinning of the colonic wall, bacterial overgrowth and translocation of the gut bacteria into the blood.
83
What is the management of Hirschprungs?
1st line: bowel irrigation Also 1st line: endorectal pullthrough (colostomy followed by anastomosing normally innervated bowel)
84
What is the management of Hirschsprung's enterocolitis?
Supportive fluid resuscitation Decompression with NG tube (and rectal tube if needed) Broad-spectrum Abx (with anaerobic coverage) Surgery - definitive treatment
85
Recall the principles of management for anal fissure
Ensure stools are soft + easy to pass (conservative) Increase dietary fibre + fluid intake Anal hygeine Safety net: seek further help if not healed within 2 weeks
86
Recall all the principles of management for threadworm
Single dose of an anti-helminth (mebendazole) for the whole household Advise rigorous hygeine for 2 weeks if on mebendazole, or 6 weeks if using hygeine measures alone Exclusion from school/ nursery is not required
87
What can cause a temporary lactase deficiency?
Gastroenteritis Crohn's Coeliac Alcoholism
88
What should be excluded in suspected lactose intolerance?
Gastroenteritis (stool sample) Crohn's (faecal calprotectin) Coeliac (anti-tTG/EMA)
89
How is a diagnosis of lactose intolerance made?
It's a clinical diagnosis trial a 2 week lactose-free diet + see how Sx are Breath hydrogen test: early rise in H2 following CHO ingestion
90
How is secondary lactose intolerance managed?
Cut out dairy to allow time to heal May need calcium + vit D supplements Digestive enzymes can be taken in a capsule before eating lactose until gut matures/ heals
91
Recall 2 genetic associations with Coeliac's?
HLA DQ2 (95%), DQ8 (80%)
92
Recall the symptoms of coeliac in children
Failure to thrive Abdo distention Bloating Irritability
93
When does coeliac disease first present in children?
8-24 months after introduction to wheat foods
94
How is coeliac disease diagnosed?
Most sensitive = IgA TTG Or (less sensitive) = IgA anti-EMA
95
What other investigations are useful in coeliac disease?
FBC + blood smear to look for anaemia In older children/ adults: OJD + biopsy can confirm dx In younger kids: no histopathological confirmation
96
How should coeliac disease be managed?
Cut out all wheat, rye + barley Dietician referral + annual review Support sources: Coeliac UK
97
What might be the consequences of non-adherence to diet in coeliac disease?
Micronutrient deficiency, osteoporosis, EATK, hyposplenism
98
What is mesenteric adenitis?
Swollen lymph glands that cause temporary abdo pain following infection
99
What are the signs and symptoms of mesenteric adenitis?
Abdo pain Nausea + diarrhoea, leading to reduced appetite Infectious picture Often preceded by UTI
100
How should mesenteric adenitis be diagnosed?
Definitive dx= laparoscopy showing large mesenteric lymph nodes + normal appendix More often dx of exclusion (exclude appendicitis with bloods, urine, MC+S)
101
How should mesenteric adenitis be managed?
Simple analgesia Maybe Abx (but not routine) Safety net for increased pain, deterioration
102
What is the pathophysiology of an indirect inguinal hernia?
Towards end of pregnancy the process vaginalis allows passage of testicles from abdomen to scrotum When this passage fails to close, abdo lining/ bowel can protrude through defect
103
Recall the signs and symptoms of hernia
Scrotal sac enlarged, contains palpable loops of bowel, fluid (does not always transilluminate) Swelling or bulge may be intermittent + can appear on crying or straining
104
How is hernia diagnosed?
Clinical diagnosis Examine supine + standing + try to reduce in order to determine type of hernia
105
Recall 3 risk factors for umbilical hernia
Afro-caribbean Down's Mucopolysaccharide diseases
106
How should hernia be managed?
Correct urgently 1. If < 6 weeks old, correct <2 days 2. If < 6 months old, correct <2 weeks 3. If <6 year old, correct <2 months
107
How does an umbilical granuloma appear?
Leaks + is watery
108
How is umbilical granuloma treated?
With salt
109
Where are femoral hernias located?
Beneath inguinal canal
110
What is femoral hernia most similar to?
Indirect inguinal hernia
111
What is gastroschisis?
Paraumbilical wall defect: abdominal contents outside body without a peritoneal covering Needs immediate surgery
112
What is omphalocele?
Bowel protruding out of the body with a peritoneal covering
113
How should omphalocele be managed?
Closure starting immediately, finishing at 6-12 months
114
What is encoparesis?
Soiling of underwear with stool in children who are past the age of toilet training
115
What is the usual cause of encoparesis?
Constipation with overflow
116
How should encopresis be managed?
Enquire about stressors, changes in medication, food intolerances etc
117
What are the 2 most likely causes of liver failure in children <2 y/o?
HSV infection Metabolic disease
118
What is the most likely cause of acute liver failure in children >2 y/o?
Paracetamol OD
119
What are the signs and symptoms of acute liver failure?
Jaundice Coagulopathy Hypoglycaemia Electrolyte disturbance Encephalopathy
120
How should Acute liver failure be managed?
Referral to a national paediatric liver centre To stabilise the child: - IV dextrose (due to hypoglycaemia) - broad spectrum Abx + anti-fungals to prevent sepsis - IV vit K + PPIs to prevent haemorrhage - Fluid restriction + mannitol
121
Recall some features of poor liver prognosis
Shrinking liver Falling transaminases Rising bilirubin Worsening coagulopathy
122
How should hepatic encephalopathy be managed?
Reduce nitrogen with lactulose
123
How should AI hepatitis be managed?
Prednisolone/ azothioprine
124
How should sclerosing cholangitis be managed?
Ursodeoxycholic acid (aids bile flow)
125
How should Wilson's disease be managed?
Zinc (blocks intestinal Cu resorption) Trientine/ penicillinamine (increases urinary Cu excretion) Pyridoxine (vit B6, prevents peripheral neuropathy) Symptomatic tx for tremor, dystonia + speech impediment
126
How is non-alcoholic fatty liver disease managed in children?
Weight loss Statins Treatment of diabetes Vit E + C Ursodeoxycholic acid to improve bile flow
127
How should paracetamol OD be managed?
<1 hour: activated charchoal, do paracetamol level 4 hours post ingestion, NAC if indicated >1 hour: do a paracetamol level, NAC if indicated
128
Is pyloric stenosis more common in girls or boys?
Boys (4 x more common)
129
What is the inheritance pattern of pyloric stenosis?
Polygenic
130
What is the incidence of pyloric stenosis?
0.3%
131
What is the race predilection of pyloric stenosis?
More common in Caucasian babies
132
What is a Ramstedt pyloromyotomy?
The procedure of choice for pyloric stenosis where the pyloric muscle is split longitudinally down to the mucosa. It is easily performed with minimal complications.
133
What is the classical triad of HUS?
Thrombocytopenia Microangiopathic haemolytic uraemia Acute renal failure
134
What is the most common cause of HUS in children?
E.coli 0157:H7 - causes bloody diarrhoea
135
What investigations should be done for HUS?
FBC Blood film Renal function and serum electrolytes Stool specimens for culture - as commonly caused by gastroenteritis in children
136
What are the 5 main causes of dysentry?
CHESS Y Campylobacter Haemolytic E.coli (0157:H7) Entamoeba (protozoa) Shigella Salmonella Yersinia enterocolitica
137
Give some reasons for physiological jaundice at birth
1. Hb release from RBCs as there is high [Hb] at birth 2. RBC lifespan being 70 days rather than 120 days 3. Breast milk jaundice (but not until after >24 hours) 4. BR metabolism being less efficient in first few days of life
138
What is the main danger of uBR buildup in neonates?
Kernicterus - a form of encephalopathy
139
What is kernicterus?
A form of encephalopathy caused by a deposit of uBR in the basal ganglia May develop into dyskinetic CP, LD + sensorineural deafness
140
How can uBR buildup in neonates be treated before any damage is done?
Phototherapy +/- IvIG and exchange transfusion
141
What would be the difference in clinical presentation between uBR buildup and cBR buildup?
uBR buildup --> kernicterus cBR buildup --> dark urine + pale stools
142
In what situations should phototherapy be stopped?
If bronzing occurs - means the child has a buildup of cBR, rather than uBR, which cannot be treated using phototherapy
143
How should investigations begin in neonatal jaundice?
1. Check transcutaneous or serum BR levels 2. Do a split BR to check uBR/cBR 3. May want to do a blood film analysis
144
Recall some pathological causes of neonatal jaundice in babies <24 hours old
GRAPHIC DOG - Gilbert's - Rhesus disease - ABO incompatability (--> haemolysis) - PK deficiency - Hereditary spherocytosis - Infection - Crigler-Najjar - Dubin-Johnson - Other... - G6PD deficiency
145
Recall 3 physiological causes of jaundice in a 2 day to 2 week old baby
Physiological jaundice - due to immature liver, peaks at 3-5 days Breastfeeding jaundice - less milk intake --> more enterohepatic recycling Breastmilk jaundice - Decreased UGT1A1 activity (occurs following physiological jaundice)
146
What haemolytic and metabolic causes for jaundice might present in a 2 day to 2 week old baby?
Metabolic: Gilbert's, Crigler-Najjar, Dubin-Johnson Haemolytic: G6PDD, PK deficiency, hereditary spherocytosis (less likely to be ABO at this point)
147
What may be the cause of jaundice in a 2 day to 2 week old baby that didn't present in first 24 hours?
Congenital hypothyroidism Dehydration Bruising (cephalohematoma) Polycythaemia
148
How should jaundice be investigated in a baby over 2 weeks old?
Direct and indirect serum BR
149
Which of the conditions that cause jaundice in a 2 day to 2 week old baby might continue to the >2 weeks stage?
All - but physiological + breastmilk is most common
150
Recall 2 further causes of a buildup of uBR in a baby over 2 weeks old
Pyloric stenosis (presents at 2-4 weeks) Congenital hypothyroidism*
151
Systematically recall the causes of a raised cBR in the neonate (>2w old)
Endocrine: Congenital hypothyroidism GI: Billiary atresia, ascending cholangitis (can be caused by lipids on TPN) Metabolic: Gal-1-PUT, A1AT deficiency, Tyrosinaemia type 1, peroxisomal disease Other: CF, idiopathic neonatal hepatitis
152
Recall some investigations you might do to find the underlying cause of jaundice in a baby
TC/ serum BR within 6 hours of presentation Haematocrit DAT/ Coombs (haemolysis?) G6PD levels (depending on ethnic origin) TSH (hypothyroid?) LFTs (ascending cholangitis? Biliary atresia?) Blood group of M and B (ABO incompatible? Rhesus?) Blood film + osmotic fragility (hereditary spherocytosis?) MC&S of urine/ CSF (if ? infection cause)
153
For how long does jaundice have to persist in order to be defined as 'prolonged'?
> 14 days if term
154
At what age should investigations include a split BR rather than just a total BR?
2 weeks
155
What is a worrying BR in a baby >37w gestation (red flag for kernicterus)?
>340
156
Recall the clinical features of kernicterus
Poor feeding Extreme lethargy Hypotonia High-pitched cry
157
How is treatment of neonatal jaundice guided?
There are thresholds at which phototherapy/ exchange transfusion are indicated
158
What are the options for treatment of neonatal jaundice?
Phototherapy +/- IV Ig Exchange transfusion + phototherapy +/- IV Ig
159
How does phototherapy work?
Converts uBR to a water-soluble pigment that is excreted in urine
160
What important checks should be done during/ after phototherapy?
During: temperature, BR levels every 4-6 hours (with regular feed breaks) After (12-18 hours post): check for a rebound hyperbilirubinaemia
161
When should intensive phototherapy be given?
1. Rapidly rising BR 2. Serum BR within 50mmol of exchange tranfusion threshold (after 72 hours life) 3. BR level doesn't respond after 6 hours of therapy
162
What are the 2 indications for exchange transfusion to treat neonatal jaundice?
1. BR threshold reached 2. Signs of kernicterus
163
What is an important thing to remember when giving an exchange transfusion?
Give folic acid afterwards to prevent anaemia