Mat Med Flashcards

(137 cards)

1
Q

DKA criteria

A

Ph <7.3 and/or Bicarbonate less than 15

Blood ketone >= to 3 or urine ketone more than 2+

Blood glucose more than 11 or known diabetes mellitus

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2
Q

At what level of spinal cord injury is associated with a risk of autonomic dysreflexia

A

T6

A spinal cord injury at the level of T6 or above repulses in loss of supraspinal control of the greater splanchnic sympathetic outflow.

AD results from disconnection of the sympathetic nervous system from supraspinal regulation, disabling the negative feedback loop. A noxious stimulus below the level of the spinal cord injury will result in an uncontrolled sympathetic outflow below the level of the lesion causes = high BP activating vagus nerve via baroreceptors then reset to fire at a lower BP since the spinal cord injury causing bradycardia

Summary- high BP, bradycardia, Nausea, difficulty breathing,

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3
Q

When to conceive post renal transplant?

A

1 year

Risk of acute rejection in the first year is approx 10-15% and is associated with recipients under 45

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4
Q

Treatment for high BP in autonomic dysreflexia - AD

A

Sublingual nifedipine 10mg

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5
Q

Postural tachycardia syndrome - symptoms and treatment
PoTS

A

You get tachy within 10 min of standing and no hypotension
Symptoms usually relieve when lying down

Treatment main- increased fluids 2-3L a day and salt intake up to 10-12g /day

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6
Q

Vaccines given at 8 weeks to babies

A

Diphtheria
Tetanus
Pertussis
Polio
Rotavirus

Avoid live vaccines like rota virus if mom on anti TNF or any other biological agents delay vaccine for first 6 months of like

It takes about 6 months to clear any maternal antibodies transferred transplacentally

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7
Q

What level of spinal cord injury is associated with risk of altered perception of fetal movements?

A

T10

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8
Q

Epilepsy women vs non epileptic moms increased risk of

A

APH
HTN disorders
PTB
PPH
Spont miscarriage
IOL
CS
RGR

No increase in GDM and perinatal death

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9
Q

Teratogenic meds

A

Mycophenolate mofetil - have a 3 months washout period pre pregnancy , make sure to use folate pre preg

ACE inhibitors

Stop statins

Methotrexate

Safe is; azathioprine, tacrolismus

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10
Q

Post renal transplant what obstetric complication is this woman at highest risk of

A

Not PET - but they are just less than the risk of…

It’s PRETERM birth <37w

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11
Q

Risk of relapse in pregnancy of IBD is

A

30%

2/3rds of patients with active disease at conception will have persistent flare-ups during preg.

Women with active disease during conception have an increased risk of 2 fold of having active disease in pregnancy

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12
Q

Post renal transplant and with proteinuria at booking woman in pregnancy need what meds …

A

Aspirin 150mg and LMWH prophylactic dose- start if PCR >300mg/mmol

Women who have proteinuria are in a hypercoagulable state of pregnancy and risk of VTE

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13
Q

Safest in epilepsy for congenital malformation

A

Lamotrigine and levetiracetam - no increase compared to general population

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14
Q

Increased risk of physical birth abnormality’s compared to general population in epilepsy meds

A

Mom with no epilepsy- 2.3/100

sodium valproate 10.7/100
Poly therapy 16.8 per 100

Carbamazepine 4-5/100
Phenobarbital 6-7 /100
Phenytoin 5/100
Topirimate 4 to 5 out of 100
Valproate 10/100

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15
Q

What meds cause transient B-cell depletion in the neonate

A

Belimumab & rituximab

Are immunoglobulins that cross the placenta from the 2nd trimester resulting in transient cytopenias and neonatal B-cell depletion that can persist up to 6 months

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16
Q

When can you restart biologics post pregnancy also if breastfeeding

A

Can be restarted immediatly

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17
Q

Types of diabetes insipidus - SDI

A

Symptoms of DI= polyurina, polydypsia, high osmolality in plasma, low osmolality in urine,

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18
Q

Meds in pregnancy and breastfeeding to treat IBD + risks

A
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19
Q

How to test for ulcerative colitis UC relapse in preg

A

Test for faecal calprotectin - a non invasive marker for intestinal inflammation

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20
Q

What to do if at high of seizures in pregnancy or increased seizures recently in preg

A

Increase current meds if possible and add clobazam

Clobazam may also be given if risk is high for increased seizures like risk of sleep deprivation or prev fits++ in labour

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21
Q

Treatment for suspected Stroke in pregnancy

A

Thrombolysis with intravenous alteplase

Avoided aspirin for the first 24h after thrombolysis as it increases the risk of a subsequent intracranial haemorrhage

Thrombectomy with IV thrombolysis should be offered within 6 hour of store symptom if confirmed occulsion of the proximal anterior circulation is seen on CTA 0 agniography or MRA

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22
Q

What is the incidence of pregnancy related stroke

A

30 in 100 000

Most strokes occur peripartum (90%) or in the first 6 weeks post delivery

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23
Q

What meds are associated with increased risk of haemorrhagic disease of the newborn

A

Enzyme inducing AEDs (

carbamazepine,
phenytoin,
phenobarbital,
primodone,
Oxcarbazepine,
topirimate
eslicarbazepine

All babies born to women with epilepsy taking Enzyme inducing meds should be offered 1mg of IM VITAMIN K. To prevent haemorrhagic disease of the newborn

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24
Q

MS in preg- what is associated with higher risk of relapse

A

Pre-pregnancy relapse

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25
Most common type of MS
85% - RRMS Relapsing remitting MS Breast feeding has beneficial effects on MS relapse Fewer relapses in the first 3-4 months of preg.
26
What is the risk of preterm birth after trachelectomy
25%
27
Investigations to do after a stroke ?
At the time of stroke/presentation do; ECG (will prob have ST changes) , then 24H Holter monitoring A prolonged cardiac monitor or implantable loop recorder should be considered for cryptogenic strokes potentially caused by paroxysmal atrial fibrillation Loop recorder is superior to a holter monitor for diagnosis in pregnancy Transthoracic echo is the BEST 1st line investigation - a BUBBLE TEST or contrast echo can be performed from the cardiac apex which involves the use of agitated saline and a salsalva manoeuvre to demonstrate an intra-atrial shunt Transoesophageal echo (TOE) can be performed if the transthoracis echo gives equivocal results TOE remains the optimum investigation to rule out aortic arch atheroma , left atrial appendage thrombus , PFO A thrombophilia screen shoul be done 6 weeks postanally as some components may not be accurate in the setting of pregnancy and postpartum
28
What is the risk of recurrent stroke in pregnancy
If woman has a concurrent thrombiphilia- 20% If PFO that is now closed - its 1% Outside of pregnancy is 0.5%
29
How to test for DI - diabetes insipidus
Paired serum and urine osmolality IN DI urine will be dilute with a U:P ratio of <2 In pregnancy normal plasma osmolality is approx 270 (normally >285 max 295 and urine <300 if non preggo ) , so if osmolality levels in pregnancy are normal like for a non preggp person that is abnormal In pregnancy plasma sodium is also reduced by 4-5 mmol/l
30
What are absolute contra indications to thrombolysis And relative contraindications
Intracerebral haemorrhage Suspected subarachnoid haemorrhage even if normal on CT Neurosurgery/head trauma in last 3 months BP>185, 105 Hx of intracereral haemorrhage ….
31
MS is associated with ?
Increased risk of FGR
32
What is the risk of 2nd trimester miscarriage after trachelectomy
7% Normal population rate is 4%
33
Rate of miscarriage in trachelectomy in 1st trimester
16% Vs 15-20 in normal ppl
34
How to take adalimumab in preg
Can take it till 28w then stop Very high levels in baby, long half life Anti TNG activity used to treat conditions such as RA, JIA, psoriasis, psoriatic arthritis, akylosing spon, IBD sarcoidosis It is a IGG1 monoclonal antibody
35
Best contraception post trachelectomy
Depo provera
36
How to delivery after abdominal cerclage after radical trachelectomy
Planned CS , risk of uterine rupture if in labour ad sever haemorrhage if contractions start
37
Can you restart adalimumab in the 3rd tri
Yes if flare up of chron;s occurs or ?IBD can start predinioslone and adalimumab but it takes 6 weeks for it to start working
38
Intracranial idiopathic hypertension IIH Symptoms and treatment
Headache worse at coughing associated with nausea blurred vision and diplopia , papilloedema , visual field defect with enlarged blind spot, reduced visual acuity, reduced colour vision and sixth nerve palsy Lumbar CSF opening pressure > than 250 mmH20 measured in the lateral decubitus position to diagnose IIH High BMI usually Main goals of treatment are symptom control and preservation of vision Tx options: Acetazolamide - analgesics for headache, diuretics to reduce CSF production, dietary modification to aid weight loss Surgical tx options: repeated lumbar puncture ,optic nerve sheath fenestration CSF diversion procedures
39
Criteria for IIH intracranial idiopathic hypertension
Modified dandy criteria used for diagnosis
40
First test for IIH
CT head
41
Which imaging for liver masses if solid in pregnancy
USS then MRI liver gadolinium contrast
42
Liver lesson with highest risk of bleeding in pregnancy
Hepatic adenoma - risk of haemorrhage is 27.2% and 15.8% of rupture Lesion greater than 10cm confer the highest risk and risk of rupture in pregnant women is highest in 3rd trimester
43
PRES - posterior reversible encephalopathy syndrome Symptoms
PRES is a clinical-neuroradiological entity associated with pre-eclampsia Headache, vision disturbances, nausea vomiting, seizures, altered metal state ON MRI- edema in the posterior circulation of the brain - parietal and occipital lobe contrivance and suboptimal signal changes are hyper tense on T2 and FLAIR sequences Tx: PET algorithm, delivery
44
How long to conceive after trachelectomy
6 months Confirmation of no early recurrence is with negative colposcopic assessment, vaginal vault and isthmic smear and pelvic MRI
45
How to teat seizure in pregnancy? And 2nd line as well?
1st) IV lorazepam if don’t work use …. 2n line) loading dose phenytoin 10-15mg/kg
46
What are the modified WHO mWHO classification of maternal cardiovascular risk?
2 photos
47
Treatment of gestational DI and neurogenic DI
Desmopressin - is an ADH analogue - DDAVP, has a different N terminal that renders it immune to metabolism from vaso[ressinase Can be take PO, SL , intranasally , SC, IM IV Can be taken in preg and breastfeeding In neurogenic DI in pregnancy DDAVP must be increased in pregnancy and decreased post partum In nephrogenic DI treatment is based around correcting hypercalcaemia and hypokalaemia
48
What is the highest association with antenatal stroke in pregnancy
gestational diabetes GDM
49
PPCM - perpartum cardiomyopathy
Signs and symptoms - productive cough, SOB when lying flat, bilateral ankle oedema , tachycardia, normal stats , raised JVP, fine bilateral crepitations at lung bases Definition: Left ventricular ejection fraction LVEF below 45% If left ventricular end diastolic diameter LVEDD is >6cm and LVEF <30% are indicative of a decreased prospect of spontaneous recovery and an increased likelihood of mechanical support, transplant and death
50
Best imaging for stroke in pregnancy
Non-enhanted CT head
51
Strokes in pregnancy cause cause and when do they occur
Cerebral infarction, cerebral vein thrombosis CVT, intracranial haemorrhage ICH, subarachnoid haemorrhage 90% of strokes occur peripartum pr in the 6 weeks post delivery In general population most strokes are ischaemic 80-85% In preg- ischaemia, haemorrhage and venous thrombosis have similar contribution to aetiology
52
Risk of relapse of PPCM
In subsequent pregnancy there is a risk of PPCM relapse, If persistent left ventricular dysfunction there is a 50% chance of further deterioration in LVF and increased morbidity and mortality rates - mortality may be as high as 20% If a woman has complete recovery of left ventricular function, prognosis appears to be better although 20% of women may have a further relapse If complete recovery of LVF long term outcome is unknown IF LVEF <25% at initial diagnosis or when the LVEF has not normalized over a course of time following adequate medical care, the woman should be counseled not to become pregnant
53
What is the mortality rate of PPCM peripartum cardiomyopathy
10% 50-80% of women diagnosed with PPCM achieve recovery in their LVEF >or equal to 50%. Mostly within the first 6 months after manifesting the disease PPCM assiciated with hypertension has been associated with a more successful recovery
54
How to treat new onset supraventricular tachycardia
Tx is vagal manoeuvres , if does not work then ADENOSINE Or beta-1-selective blockers- ie metoprolol or bisoprolol Tachyarrhythmias may present for the first time and become more frequent in pregnancy, particularly in older women and in women with congenital heart disease MOST FREQUENT arrhythmias: AF and paroxysmal supraventricular tachycardia (PSVT)
55
What do you treat cardioversion with
Any tachycardia with haemodynamic instability and for pre-excited atrial fibrillation
56
How to anticoagulate metallic heart valve in pregnancy if on warfarin
They are high risk mWHO class III Change from warfarin to therapeutic LMWH between week 6-12 then restart warfarin in second trimester Adjusted dose based on anti xa levels when on LMWH To stay on warfarin until get preggo- of dose is low can consider continuing it VKAs - vitamin K antagonists like warfarin have a small risk of embryopathy, fetal abnormalities and fetal loss but remain the most effective regimen to prevent valve thrombosis
57
AI Adrenal Insufficiency symptoms (Addison’s disease)
Can be primary, secondary and tertiary Primary= adrenocortical disease , both glucocorticoid and mineralocorticoid deficiency’s Autoimmune atrophy of adrena gland accounts for 70-90% of primary AI. - other causes, haemorrhage from sepsis, major burns, lymphoma, metastasis, infections TB Secondary & tertiary= are associated with ACTH and corticotropin-releasing hormone (CRH) secretion disorders, mainly from cortisol deficiency Symptoms: low BP, hyper K, hypo Na, hypoglycemia, increased pigmentation - mostly of mucous membrane, extensor surfaces and non exposed regions of body
58
Thalassaemia transfusions
Don’t need to transfuse if HB above 80 in an untrasfused patient at 36 weeks - can be post post delivery Monitor bloods post transfusion since HB can drop Do transfusion if worsening maternal anaemia or evidence of FGR- consider regular transfusions for ex. Every two weeks Top up of HB below 100, aim is to continue transfusions until reaches HB 120 Another aim is pre transfusion HB around 100
59
How to treat acute pancreatitis
IV antibiotics IV fluids to keep up with 3rd space loss and t maintain an adequate urine output. Increased vascular permeability means that 3rd space losses can be significant. Haematocrit can give an indication of these losses. In early severe actute pancreatitis fluid requirements of 150-300ml/hr would not be unusual- aim is for urine output of 0.5 ml/kg/hr Analgesia - will prob need opioids VTE prophylaxis Nutrition Consider critical care if organ support needed Antibiotics only if infection!!! Imaging - best done 5 days after onset of symptoms , CT or MRI Modification of risk factors to prevent more episode; cholecystectomy, manage lipids, alcohol - best cholecystectomy done with in 2 weeks of discharge , 12-24w gestation or after delivery Triglyceride levels will reduce by period of nil-by-mouth or use of a sliding scale Consider endocrine referral
60
Thalassaemia + splenectomy + Plts > 600
To take aspirin 75mg and LMWH low dose
61
Treatment of primary AI- adrenal insufficiency
Hydrocortisone 5mg TDS and fludrocortisone 0.1mg BD Hydrocortisone is the preferred glucocorticoid because it does not pass the placenta Hydrocortisone has an mineralocorticoid effect: 40mg of HC equals 0.1mg of fludrocortisone, So and increase in fludrocortisone is not essential However predispose does not have an MC effect and the fludrocortisone dose might be increased by 20-30%
62
Cushing’s syndrome symptoms
Generalized fatigue, increased weakness, High BP, high BMs, increase in weight Increased cortisol levels in the ACTH-dependent types and some adrenal concerns, elevated androgens. 60% of it accounts from adrenal adenoma in pregnancy , whereas pituitary dependent Cushing’s syndrome account for 70% of those outside of preg. Adrenal adenoma do not cause excess androgens secretion hence they are less likely to cause menstrual irregularities Adrenal hyperplasia and some adrenal carcinomas produce large amounts of androgens and spontaneous pregnancy is very unlikely
63
Define pregnancy Cushing’s syndrome
Onset occurring during gestation or with in 12 months of delivery or miscarriage - this may result from nodular hyperplasia of the adrenals stimulated by placentally produced ACTH or as a result of stimulation of ACTH receptors in a pre-existing but undiagnosed adrenal adenoma by ACTH from the placenta
64
Diagnosis of cushings in pregnancy
It’s hard due to high levels of cortisol in pregnancy already, low dose Dexamethasone suppression test don’t work
65
Differentiating features of cushings
Proximal myopathy Easy bruising Osteopenia/osteoperosis induced fractures Hirsutism from androgens Early onset hypertension in preg Reds or purple stria
66
What to educate women with primary AI on
Sick day rules - aimed to prevent the occurrence of adrenal crisis -always wear a medical alert bracelet/necklace -double the dose of oral glucocorticoid in cases of fever or illness requiring bed rest -provide intramuscular hydrocortisone for self-administration in cases of gastroenteritis or during fasting
67
What to do if primary AI and in labour or hyperemesis
Should receive IV HC typically between 100 and 200mg/day together with appropriate fluid resuscitation During labour delivery stress doses of GC should be administered -no studies on this
68
Signs of hyperaldosteronism
In primary aldosteronism (PA) - aldosterone production is inappropriately high for Na status, relatively autonomous of the major regulator of secretion (Angiotensin II and plasma potassium concentration) and non-suppressible by sodium loading The inappropriately elevated aldosterone leads to tissue damage suppression of plasma renin and hypokalaemia, sodium retention, hypertension, cardiovascular and renovascular diseases. Th most common causes of primary aldosteronism are **bilateral idiopathic hyperaldosteronism (60-70%) ** and **unilateral adrenal adenoma (30-40%)** . unilateral adrenal hyperplasia, familial hyperaldosteronism type 1 and pure aldosterone-producing adrenocortial carcinoma are rare causes. In the non pregnant state, PA was estimated to account for <1% of hypertension owing to the detection method that required the presence of hypokalaemia. Since hypokalaemia is no longer a prerequisite, PA is currently estimated to account for 3-15% of all pateints with hypertension. MAIN CLASSICAL PRESENTION IS HYPERTENSION AND MAY HAVE HYPOKALAEMIA and resistant hypertension - especially if before 20 w. The diagnosis of PA in pregnancy is based on suppressed renin and elevated aldosterone-to renin ratio. . Confirmatory testes is required if hypokalaemia but also if K+ normal because it could lead to critical volume expansion. MRI may be performed during the second or third trimesters to determine the subtype of PA. Whereas adrenal vein sampling is not performed because of high radiation exposure. If surgery is not considered as a treatment for PA during pregnancy, both MRi of adrenals and adrenal veins sampling which helps localize the site of the tumor for surgery should be postponed until after delivery.
69
Pheochromocytoma symptoms
Pheochromocytoma and paragangliomas (PPGL) are rare neuroendocrine tumors arising from neural crest-derived cells of the sympathetic and parasympathetic nervous systems. A Pheochromocytoma is a tumor causing excessive production of catecholamines, adrenaline, noradrenaline and dopamine, by the chromaffin cells of the adrenal medulla. A paraganglioma results from excessive production of catecholamines by the chromaffin cells located int he extra-adrenal sympathetic paraganglia, typically in the abdomen and pelvis. PPGL can be sporadic or hereditary such as von hipped Linus syndrome , MEN2, neurofibrimatosis type 1 - all Are autosomal dominant SYMPTOMS: Headache, sweating tachycardia - classic triad but is not common in preggo In preggo- orthostatic hypotension, arrhythmia, chest pains convulsions, syncope, blurring of vision, weight loss, papilloedema, insulin resistance, hyperglymaemia, high ESR, phsych disorders and erythrocytes is
70
Transient neonatal cutaneous lupus How common, signs
Is part of the neonatal lupus erythrematous spectrum - manifests as annular inflammatory lesions which appear much like lesions of subacute cutanous SLE -it occurs in 5% of infants corn to anti RO/La+ave mother - lesions usually on face and scalp and usually appear after UV exposure on the first 2 weeks of life but up tp 3-6 months post partum - rash lasts usually 6 months until baby clears maternal antibodies
71
CAH signs symptoms types
Congenital Adrenal hyperplasia - AR disorder , impaired cortisol synthesis secondary to enzyme deficiency 22 MC enzyme def. Is 21-hydroxylase def. (21 HD) Others: 11b-hydroxylase deficiency and 17a-hydroxyl are deficiency 21- HD- can classicary present at birth w, enhanced ACTh drives excess adrenal androgen production = clitoral enlargement, labial fusion, secular ambiguity at birth, even ininnapropate sex assignment 755 of patient of birth genders present at birth with sever hypo-Na caused by salt wasting as a result of severe aldosterone deficiency - non classical CAH secondary to 21 HD have features similar to PCOD including hirsutism primary or secondary amenorrhea or anuvulatory infertility
72
Lupus nephritis features
hypertension, proteinuria with or without haematuria and renal impairment Is caused by autoantibodies which produce immune complexes these are deposited in the kidneys and they activate the complement cascade causing a generalized inflammatory response The presence of haematuria or red cell casts as well as rise in anti-dsDNA titres or fall in complement levels help to distinguish this from PET The only tool to properly distinguish PET from lupus rephritis is a renal biopsy In cases of lupus nephritis that fail to respond to increasing dosages of steroids and azathioprine and where there is a deterioration of renal function and or hypertension other immunosuppressive drugs may be considered such as mycophenolate mofetil or tacrolismus
73
How to test for Sheehan’s syndrome
MIR pituitary will usually show avascular necrosis
74
What is the incidence of neurodevelpmental disorders (autism) in babies exposed to sodium valproate in utero
35% 30-40 out of the 100 children exposed to valproic acid in utero with have lifelong difficulties, learning, thinking
75
What is the risk of congenital heart block of the baby after a mom has a history of SLE and is positive anti-Ro and anti-LA antibodies .
2% Antibodies Ro and la cross the placenta and destroy baby purkinje system Usually presents as fixed fetal bradycardia of 60-80 beats per minutes on USS Recurrence rate of 16% in subsequent pregnancies Usually about half of infants need pacing by the first year of life The heart block develops between 18-28 weeks of gestation and fetal echo should be performed around this time to detect it Hydrops fetalis can occur in utero and is thought to be due to the degree of endomyocardial fibrosis and associated myocarditis
76
How to diagnose malaria
Microscopy, of thick and thin blood films for parasites and rapid diagnostic tests are the standard tools available Rapid detection tests may miss low parasitaemia which is more likely in pregnant women and rapid detection tests are relatively insensitive in P vivax malaria A positive rapid diagnostic tests should be followed by microscopy to quantify the number of infected red blood cells parasitaemia and to confirm the species and the stage of parasites
77
Asthma treatment
If PEF > 80% = good control of asthma Step 1- Mild intermittent asthma treatment = inhaled short acting reliever (B2 agonist) medication PRN Step 2 - If usage of reliever B2-agonist exceeds 3 times per weeks, regular inhaled anti-inflamatory meds with a steroid preventer needed eg beclomethasone inhaler 400nanog/day Step 3- either addition of a long acting reliever B2 agonist (LABA) eg. salmeterol or and increase int he dose if inhaled steroid (800nanog/day) Step 4- trial of additional therapies eg. Leukotriene receptor antagonist , slow release oral theophylline or oral B2 agonist . Alternatively the dose of inhaled steroid can be increased to 2000nanog/day Step 5- If these measures fail to achieve control then continuous or frequent use of oral steroids becomes necessary All patients with acute asthma attacks should get oral steroids
78
What are the benign malarias
P vivax Ovals Malarial They do not cause sequestration like in the more fatal one P falciparum where parasites are found sequestered in the placenta
79
How to check if baby got malaria
Vertical transmission occurs if parasites in the placenta All babies whose mom had malaria in preg. Need to be screened with standard microscopy of thick and thin blood films at birth and weekly blood films for 28 days.
80
What is the recommended chemoprophyaxis in 2/3rd trimester
Mefloquine is the recommended drug of choice for prophylaxis int he second and 3rd trimesters for chloroquine- resistant areas. With very few areas in the world free from chloroquine resistance , mefloquine is essentially the only drug considered safe for prophylaxis in pregnant travelers
81
Risk of contacting malaria in different part of world
82
Chemophrophylaxis how long to take it for when traveling
Chemoprophylaxis can be causal or suppressive Sausal prophylaxis is directed against liver schizont stage which takes approximately 7 days to develop so these drugs ex. Atovaquione-proguanil need to be continued for 7 days after leaving a malarias area Suppressive prophylaxis such as mefloquine is directed against liver against the red blood cell stages of the malaria parasite and so should be continued for 4 weeks after leaving a malaria area
83
Blood transfusion in preg
Needs to be CMV negative Kell typing, CDE compatibility
84
Which carrier is associated with a mild to moderate thalassemia disorder
HbC All others are serious haemogloninopathies
85
86
Acute anemia and sickle cell disease is at risk of what infection?
Parvovirus B19- erythrovirus infection - causes a red cell maturation arrest and an aplastic crisis characterized by a reticulocytopenia. Reticulocyte count should be requested in any woman presenting with an acute anaemia and if low may indicate infection with erythrovirus Treatment = blood transfusion and isolation for woman With erythrovirus infection there is risk of vertical transmission to the fetus which can results in hydrops fetalis
87
Complications of SCD Sickle cell disease
Acute painful cases - causes haemoytic anaemia and vaso-occlusion on the small blood vessels Stroke Pulmonary hypertension Renal dysfunction Retinal disease Leg ulcers Cholelithiasis A vascular necrosis- femoral head may need dip replacement
88
What does the assessment for chronic disease in sickle cell look like
Screening for pulmonary hypertension with echo Incidence of pulmonary hypertension in increased in patients with SCD . A tricuspid regurgitation jet velocity of >2.5m/s is associated with a height risk of pulmonary hypertension . Needs an echo if one not done in last year Blood pressure and urinalysis should be performed to identify women with hypertension and or proteinuria . Renal and liver function test should be done annually to indtify sickle nephropathy and or deranged hepatitic function Retinal screening. Proliferation retinopathy is common in patients with SCD, especially patients with HBSC. And can lead to vision loss. Recommended that women be screened pre conception Screening for iron overload in women who have had multiple transfusions in the past and how have a high ferritin lever. T2 cardiac magnetic resonance imaging may be helpful to assess body iron loading . Agressive iron chelation before conception is advisable in women who are significantly iron loaded Screening for red cell antibodies. Red cell antibodies may indicate an increased risk of haemolytic disease of the newborn
89
What is Haemoglicin S combines with normal haemoglibin A
Known as sickle trait (AS) It is asymptomatic except for a possible increased risk of UTId and microscopic haematuria HBSS = sickle cell anaemia HbSC HbSB thalassaemia
90
Reversible cerebral vasoconstriction syndrome (RCVS)
Is a cerebrovascular disorder associated with multifocal arterial constriction and dilation. It has a significant association with the postpartum period. RCVS is characterized by recurrent sudden onset and severe deadaches over 1-3 weeks. Often accompanies by nausea, vomiting, photophobia, confusion and blurred vision. Diagnosis requires the demonstration of diffuse arterial beading on **cerebral angiography** with resolution within 1-3 months. It is in the differenctial of a postpartum thunderclap headache often made after subarachnoid haemorrhage has been excluded but the headaches recur. Treatment is currently based on expert opinion including the use of calcium channel blockers, high-dose corticosteroids and magnesium sulphate.
91
Marfants syndrome
AD- 50 % risk of fetus affected by cagenital heart disease With aortic root dimension of 25mm there is a 10-20% risk of maternal cardiac event Aortic root dimension of 45mm there is a risk of 40-100% risk of maternal cardiac event
92
Risk of maternal cardiac event if VSD with good cardiac function EF>55%
2.5-5% with risk of fetus affected by congenital heart disease at 1%
93
Digeorge is what dominance
Autosomal dominant - CATCH22 C- cardiac defects A- Abnormal fancies T- thymi’s aphasia C- cleft palate H- Hypocalcaemia 22- Chromosome 22 deletion Cardiac defects may be - associated with tetralogy of fallot (pulmonary stenosis with ventricular septal defect and overriding aorta with right ventricular hypertrophy. - has a **5-10% risk of maternal cardiac event** - carrier of the 22Q11.2 deletion
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LONG QT syndrome
ECG = torsade de pointes LQTS is characterized by prolonged QT interval on the ECG secondary to a disorder of ventricular myocardial repolarisation , this can lead tp verntricuarl arrhythmias typically torsade de points and risk of sudden death There is a significant increase in the risk of cardiac events in the postpartum period especially if has type 2 LQTS , less in pregnancy. ,
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Hypertrophic cardiomyopathy genetic cause? And risk for mom and passing over
AD typically- autosomal dominant 10-20% risk of maternal cardiac event 50% risk of fetus affected
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Atrial fibrillation and atrial flutter
A fib and A flutter are uncommon in pregnancy and usually associated with cardiac pathology such as Mitral stenosis Metabolic and electrolyte abnormalities Fibrillation - atrial muscle bites contract independently in an irregular manner ; absence of P waves and irregular ventricular contraction. Flutter- atria beat regularly at a rate of 300 beats per minute with ventricular rate of 150 1:2 conduction ; sew tooth appreareane on the ECG secondary
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Mistrial stenosis increases risk of what?
Atrial fibrillation or flutter increases the risk of systemic embolism in an already pro-thrombotic state of pregnancy. If persistent atrial fibrillation should be anticoagulated-LMWH
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What to do if haemodynamic compromise is present With Atrial fibrillation or flutter
First line treatment is direct cardioversion Is stable can use pharmacological methods - IV Flecainide or butilide AV nodal blocking drugs are used as rate control in prophylaxis for paroxysmal episodes of if cardioversion cannot be achieved - ie beta blockers
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What is the most common non-benign arrhythmia in pregnancy
Supraventricular tachycardia SVT Most commonly paroxysmal SVT PSVT is caused by an atrioventricular nodal re-entrant tachycardia - in this arrhythmia a re-entry circuit occurs via the AV noted and surrounding perinodal atrial tissue Another common cause of PSVT is the presence of an overt or concealed accessory pathway which allows conduction to bypass the AV node Wolf parkinson white syndrome- delta wave
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SVT symptoms
Palpitation with abrupt onset and offset Symptoms of; syncope, chest pain, haemodynamic compromise are variable and in part influenced by whether structural heart disease is also present
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SVT treatment
Vagal manoeuvres ie valsalva manoeuvre or IC adenosine This terminated 90% of SVT in pregnancy Can also use verapamil, metoprolol , direct current cardioversion , particularly if there is haemodynamic compromise Prophylactics if know PSVT= beta blockers
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How to test for Cushing’s syndrome in pregnancy -
Night time serum cortisol level A midnight plasma cortisol level could be used as the preliminary screening test because the diurnal variation of cortisol is maintained during pregnancy and with a higher lowest point what’s in the non-pregnant population. The use of salivary cortisol at night in the combination with urinary free cortisol are reliable confirmatory diagnostic tools in preggo. Late night salivary cortisol and urinary free cortisol greater than 3 times the upper limit of normal are diagnostic of Cushing’s syndrome on pregnancy. The low dose dexamethasone (1mg) suppression test may lead to false positive results because of pregnancy induced hypercortsolism. But **failure to suppress cortisol following a high dose of dexamethasone (8Mg) is in keeping with a diagnosis of Cushing’s syndrome in pregnancy.** The usual post dexamethasone ACTH suppression in Cushing;s syndrome which is confirmatory of diagnosis is often not observed in pregnancy, probably because placental ACTH production is not suppressed by dexamethasone.
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What test results would patient who have Cushing’s syndrome have
Would not have cortisol suppression after high dose of dexamethasone and with a normal to low ACTH level are likely to have adrenal Cushing’s SYNDROME Normally - dexamethasone would suppress cortisol levels!
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What test results would patient who has cushings disease (pituitary dependent Cushing;s syndrome)
Would have cortisol suppression following dexamethasone (?high dose) but with a high ACTH MRI used for imaging modality for suspected pituitary lesions as well and adrenal masses
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How to test for Addison’s disease or primary adrenal insufficiency
Morning serum cortisol and plasma ACTH levels Recap of symptoms: high K+, low Na, low BP, hypoglycemia, weight loss, prolonged vomiting hyperpigmentation in skin folds Physiological changes in preggo include increased cortisol 2-3 times higher at term than normal ppl due to , Estrogen induced increased corticosteroid binding globulin , increased cortisol half life secondary to decrease in hepatic clearance and placental production of cortisol--releasing hormone , which induces adrenal hypertrophy and enhances its responsiveness to synthetic ACTH administration. In normal people with addisons you find morning cortisol < 140 in combination with an elevated ACTH concentration. >2x above the upper limit of the reference interval. DO a short syntactin test! - this is when IV or IM 250 mcg of synthetic ACTh given in the mane -a normal response to the SST is a rise in serum cortisol concentration after 30-6 min to >500 - 550 Pregnant women with highly suggestive clinical features but an indeterminate SST should be offered treatment during pregnancy and retested after delivery
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Conn’s syndrome - symptoms in preggo
PA, primary hyperaldosteronism Diagnosis is challenging in preggo because of the physiological changes that leak to extra renal stimulation of the renin angiotensin aldosterone system Usually in a woman with high BP that is resistant and low K+ especially if **before 20w of gestation*
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How to diagnose Conn’s syndrome
PA- primary hyperaldosteronism The diagnosis of PA in preggos based on the suppressed renin and elevated aldosterone to renin ratio Confirmatory test is not only needed in the presence of hypokalaemia but is also not recommended in normokalaemic subjects because saline induction could lead to critical volume expansion MRI may be performed during the second or third trimester to determine the subtype of PA, whereas adrenal vein sampling is not performed because of high radiation exposure If surgery is not considered as treatment for PA during pregnancy both MRI of adrenals and adrenal veins sampling which may help localize the site of the tumor for surgery, should be postponed until after delivery
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Summarize the renin angiotensin aldosterone system
Low BP/Na⁺ → Kidney → Renin → Renin + Angiotensinogen (from liver) → Angiotensin I → ACE (lungs) → Angiotensin II → 1. Vasoconstriction (↑ BP) 2. Aldosterone release → Na⁺/H₂O retention 3. ADH release → H₂O retention 4. ↑ Thirst → Final result: ↑ BP and blood volume
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Features of late onset of CAH- congenital adrenal hyperplasia
Older woman approx 25, recurrent miscarriage, early onset pubarche , irregular menstruation and hirsutism with acne 21 hydroxylase deficiency
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How to test for CAH 21Hydroxylase def
Measure serum 17alpha-hydroxyprojesterone in the morning and between day 3 and 5 of the menstrual cycle if not preggo to reduce the possibility for false positive results
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How to test for pheochromocytoma
Similar in preggo and non preggo 24 hour urine collection for plasma free metanephrines or urinary fractionated metanephrines Meds that might cause false positives: tricyclic antidepressants, resperine, phenoxybenzamines, clonidine, levodopa, amphetamines, ethanol, most antipsychotics, decongestants, and procholperazine Then CT of the abdominal and functional meta-iodobenzylguanidine (MIBG) scans delivery large doses of radiation hence MRI is the imaging mode of choice for the adrenal gland.
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How does a PE present on an ECG
T wave inversion S1Q3T3 pattern - S wave in lead 1, q wave in lead 3 inverted T wave in lead 3. RBBB
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MC compressive neuropathy in obs
lateral cutaneous nerve of the thigh
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lateral cutaneous nerve of thigh location and symptoms
L2, L3 Compression of the nerve as it exits the pelvis under the inguinal ligament can result in meralgia paraesthetica 2. symptoms, including numbness, pain, paraesthesia, hyperalgesia and hypersensitivity to heat. Specific to this condition, there are no motor symptoms, the sensory changes do not cross the midline of the thigh and the symptoms can be reproduced by pressure or Tinel’s test on the lateral inguinal ligament 3. 4. 5. Treatment relies on relieving nerve compression. Patients can be advised to lose excess weight and wear loose clothing In cases occurring in late gestation or labour, delivery of the fetus, the passage of time and simple analgesia should suffice Local corticosteroid injections, neuropathic pain medications (for example, amitriptylline, which is safe in breastfeeding) and, in severe cases, surgical decompression may also be needed
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Lumbosacral trunk location injury and symptoms
L4, L5 The lumbosacral trunk can be compressed between the sacrum and the fetal head or obstetric forceps. 2. The nerve fibres that are usually damaged contribute to the common peroneal nerve; therefore, lumbosacral trunk injuries cause foot drop and paraesthesia, or loss of sensation along the lateral calf and foot. 3. careful examination often shows mild weakness in other areas, such as knee flexion, hip abduction, extension and internal rotations. This enables lumbosacral trunk lesions to be distinguished from pure common peroneal nerve injuries 4. Treatment relies on physiotherapy and neuropathic pain medications.
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Femoral nerve location and symptoms
L2, L3, L4 The course it takes means that femoral nerve injuries are caused by stretching or compression at the inguinal ligament rather than compression between the fetal head and pelvis. 2. 3. Femoral neuropathy results in weakness of knee extension with or without hip flexion, and pain, paraesthesia, or loss of sensation in the anterior thigh and medial calf. Femoral nerve lesions can be bilateral in up to 25% of cases, although bilateral symptoms postpartum should always prompt urgent investigation with magnetic resonance imaging (MRI) to rule out a central lesion. 4. Careful neurological examination is therefore always required TO DIFFRENTIATE between Lesions to the nerve roots (radiculopathy) of the upper lumbar plexus (L2/L3), retroperitoneal lesions and peripheral lesions. 5. Simple analgesia, neuropathic pain medication or femoral nerve blocks. Muscle weakness resulting in the knee ‘giving way’ can be managed with physiotherapy and a knee brace for support. The prognosis is usually excellent.
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obturator nerve location and symptoms
L2, L3, L4 Because it enters the true pelvis, it can also be damaged by compression by the fetal head. The lithotomy position stretches the nerve as it exits the obturator foramen and therefore may also predispose to injury The obturator nerve supplies motor nerves to the hip adductors and sensory nerves to the medial portion of the thigh. Obturator neuropathy is bilateral in around 25% of cases and results in isolated weakness of hip adduction and loss of sensation over the medial thigh. Treatment involves physiotherapy and reassurance; the prognosis is usually excellent.
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Common Perineal nerve
The common peroneal nerve arises from the sciatic nerve in the posterior thigh and is vulnerable to compression (for example, from lithotomy supports) or traumatic injuries when it runs around the head of the fibula to reach the anterior calf causes foot drop and impaired sensation over the lateral and anterior calf and foot As noted above, pure common peroneal nerve injuries must be carefully distinguished from lumbosacral trunk injuries by examination and – possibly – by nerve conduction studies. 4. They should also be distinguished from injuries of the sciatic nerve, which will cause hamstring weakness and sensory/motor symptoms in the anterior/lateral/posterior lower leg, not just the anterolateral areas that are innervated by the common peroneal nerve. 5. Patients classically develop a high stepping gait to compensate for foot drop, but remain vulnerable to trips and falls. An ankle foot orthosis (AFO brace), supplied by a physiotherapist, will help reduce the risk.
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What is VWD Von Willebrand
An inherited bleeding disorder-common - THE MC one Results from a qualitative or quantitative deficiency of Von willebrand factor which is a large and complex glycoprotein essential for leveled dependant primary haemostasis and also for carriage of the factor 8 in the circulation Types: 1- partial quantitative 2- qualitative 3- severe quantitative All women with VWD should have VWF antigen levels and activity and factor 8 levels checked at booking in the third trimester and prior to any invasive procedures If Type 1 who have normal VWF levels can be safely managed in standard obs units in comb with haemophilia Centre staff
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Mode of action or TXA - tranexamic acid
Synthetic reversible competitive inhibitor to the lysine receptor found on plasminogen. The binding of this receptor prevents plasmin (activated form of plasminogen) from blinding to and ultimately stabilizing the firin matrix Antifibrinolytic
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When /how to treat VWD
TXA tranexamic acid should be considered in combination with treatment for all those with VWF activity less than 0.5iu/ml or as sole therapy for those with levels above 0.5iu/ml if clinically indicated This can be given orally or IV and can be started prior to delivery
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Haemophilia what is it
An X-linked condition associated with reduction or absence of clotting factor 8 (haemophilia A) or 9 (haemiphilia B) Known or potential female carriers may have low factor 8/9 levels. Levels need to be checked prior to invasive procedures or in association with bleeding symptoms . They are at risk of bleeding at procedures like terminations, miscarriage and at time of delivery All carriers of haemophilia with male fetuses should be offered third time steer amnicentesis if diagnostic investigations have not previously been performed to determine haemiphilia status to inform options for delivery
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How to treat haemophilia / when
Recombinant factor 9 is required to cover invasive or surgical procedures in women with factor levels less than 0.5iu/ml
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How to manage a surgical procedures with known factor II deficiency
Prothrombin complex concentrate
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What are factor 2,5 7,19,11,13
Rare bleeding disorders have been defined as monogenic bleeding disorders caused by deficiency of a soluble coag factor other than VWD and haemiphilia A or B They represent 3-5% of all inherited coagulation deficiencies and include inherited deficiencies of fibrinogen , all the factors mentioned above , combined factor 5+8 deficiencies and congenial deficiency of vitamin K dependent factors
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How to treat factors 2,5 7,19,11,13
If factor 2 if less than 0.2 is/ml and there is significant bleeding established labour or prior to CS , prothrombin complex concentrate 20-40iu/kg should be given to achieve factor II activity 0.2-0.4 iu/ml. further prothrombin complex concentrate 10-20 iu/kg at 48-hour intervals should be considered to maintain factor II activity moreover than 0.2 iu/ml for at least 3 days. Women already receiving prophylactic prothrombin complex concentrate can continue it throughout pregnancy
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What to do if a carrier with male fetus has a baseline factor of 8 of 0.3 iu/ml and is planning a late amino
Give desmopressin Desmopressin can be given to raise factor antenatally if factor 9 or 8 are under 0.5 iu/ml
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What is demographic urticaria
Widespread urticarial papules over the abdomen in a linear pattern matching the areas of excoriation It’s one of the most common types of urticaria’s affecting 2-5% of the population The condition manifests as an allergic like reaction causing a warm red wheal to appear on the skin, it is often the result of scratches involving contact which other materials , it can be confused with an allergic reaction when it fact it is the act of being scratched that causes a wheal to appear.
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Migraines
Women who have migraines are 2 times more likely to have PET 17 times more likely to have a stroke 4x more likely to have an MI Can treat with triptans in preggo and low does amitriptyline
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Most common site of CVT cerebral venous thrombosis
1. Thrombosis of the Sagittarius sinus with secondary extension Gino the orbital begins 2. Primary thrombosis of the cortical veins
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Best test for CVT
MRV - magnetic resonance imaging with T2 weighted imaging and magnetic resonance venography CT- only catch 30% of them Suspected to do FU MRC at 3-6 months post to see if recanalisation of the occluded sinuses
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CVT signs
Headache focal neurological sign - 6th cranial nerve palsy , worsening headache, diplopia, confusion ( unilateral failure of abduction of the eye) The greatest risk is on the 3rd trimester and first 4 weeks postpartum
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Postdural puncture headache
Worsening headache after epidural, which is fronto-occipital worse on standing and radiating to the neck Puncture of the dura occurs in 0.5-2.5% of epidurals and if there is accidental dural puncture with an epidural needle there is a 70-80% chance of psotdural puncture headache. Conservative management includes hydration and simple analgesics Untreated heache typically lasts for 7-10 days but can be up to 6 weeks Epidural blood patch has 60-90% cure rate
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