CK done!

I've had to pinch myself a few times in the last 2 days because it has hardly sunk in that USMLE step 2ck is over!

Day 0, I was 45mins flight away from home, totally psyched up, ready as I could be and knowing that my loved ones were back home praying for me. I got up early, prayed, exercised, got dressed, made sandwiches for my breaks,  read  1 or 2 pages of MTB to wake my brain cells and had a good proteinous breakfast. On my way out of the hotel, I sat down for about 10mins at the computer station and just scrolled through week 2 of this mental map (the week with the highest number of posts), nodding along. Those highlights bounced back and forth in my brain as I did the 10mins walk to the exam centre.

Generally, the exam went very well save for the intermittent drilling noise coming from outside the window at the prometric centre. It was distracting at first but when I talked to the proctor after block 1 and learnt that there was nothing they could do about it, (not even to change my seat to one farther from the window!), I told myself :" You can do this, the noise is now part of the exam and you just have to surmount it!" After that I wasn't as bothered by the noise. Nine hours went by quickly and before you knew it I was on my way back to my hotel room saying "thank you God" and telling Hubby (on the phone) how it went.

Well, the 4weeks wait is on...

I prepared for this USMLE step using U world, MTB, Kaplan Qbank, a few Kaplan videos and many online resources. One tool which was however extremely useful to me was this blog which I started about a month to the exam, when I was feeling like I needed some form of 'decongestion' or was it 'info organisation'? Whatever it was, trying to put down the most important facts about each condition or concept was really helpful in consolidating what I knew well and identifying what I didn't know  enough about. Also, the product of my mental mapping (this blog) was good for quick revision anywhere internet was available. I hope someone else will also find it useful.


To all those at the stage that I was when I started this blog, good luck!

PPD Interpretation

Classification of the Tuberculin Skin Test Reaction

An induration of 5 or more millimeters is considered positive in -HIV-infected persons -A recent contact of a person with TB disease -Persons with fibrotic changes on chest radiograph consistent with prior TB -Patients with organ transplants -Persons who are immunosuppressed for other reasons (e.g., taking the equivalent of >15 mg/day of prednisone for 1 month or longer, taking TNF-a antagonists)

An induration of 10 or more millimeters is considered positive in -Recent immigrants (< 5 years) from high-prevalence countries -Injection drug users -Residents and employees of high-risk congregate settings -Mycobacteriology laboratory personnel -Persons with clinical conditions that place them at high risk -Children < 4 years of age - Infants, children, and adolescents exposed to adults in high-risk categories

An induration of 15 or more millimeters is considered positive in any person, including persons with no known risk factors for TB. However, targeted skin testing programs should only be conducted among high-risk groups.

CDC (Center for Disease Control and Prevention)

OGTT

Normal values

75-gram 2 Hour oral glucose tolerance test

• Fasting    60 -100 mg/dL
• 1 hr:        less than 200 mg/dL
• 2 hrs:      less than 140 mg/dL. 

140 - 200 mg/dL = Impaired glucose tolerance
> 200 mg/dL = Diabetes




FOR GESTATIONAL DIABETES,
50-gram 1 hour oral glucose tolerance test

• 1 hour: < or =140 mg/dL

if >140, do
100-gram 3 hour oral glucose tolerance test

• Fasting: less than 95 mg/dL
• 1 hour: less than 180 mg/dL
• 2 hour: less than 155 mg/dL
• 3 hour: less than 140 mg/dL

2 or more abnormal values = GDM

Random facts (statistics)

Differential loss to follow up: Attrition. A type of selection bias found in cohort studies

Reliability: repeatability of a measure

Validity: absence of bias

Bias: systematic error

Selection bias: deferential access to study population (from selection process, respodents no longer available at the end, attrition etc)

Confounding: unfair comparison

Volunteer bias: type of selection bias, the tendency for one's health to influence decision to participate in a study

Information bias: occurs when the means of obtaining information from cases and controls differ

Hazards ratio, Relative risk

HR = Probability of event in treatment grp/Probability of event in control group

RR = Probability of event in treatment grp/Probability of event in control group

Exactly! same formular!

Hazard ratio is actually a type of Relative risk (a.k.a Risk ratio)

The difference?
HR is instantaneous while RR is culmulative.
RR can only be calculated at the end of the study while HR can be calculated at different points in time. Hazard ratios are calculated using the survival analysis technique. This keeps track of the number of subjects who haven't had the event (e.g death) at a particular time.

Again, HR is an estimate of RR

HR of 1 means the rate of occurrence of the event in treatment/intervention grp is same as in control/placebo grp

HR >1  means event occurs more in the intervention grp

HR <1  means event occurs less in the intervention grp

To convert a HR to probability,

Probability of event occurring = HR/1+ HR

So, HR of 1 is a probability of 50%. A 50:50 chance, i.e treatment and placebo grps are the same in terms of outcome

Regarding asthma treatment

Practice recommendations Every patient with persistent asthma, regardless of disease severity, should use a daily controller medication. Consider an inhaled corticosteroid (ICS) first when choosing controller medications for long-term treatment of mild, moderate, and severe persistent asthma in adults and children. Leukotriene modifiers, cromolyn, and nedocromil may be considered as alternative, not preferred, controller medications for patients with persistent asthma. Long-acting β2-adrenergic agonists should not be used as monotherapy. Long-term use of ICSs within labeled doses is safe for children in terms of growth, bone mineral density, and adrenal function; nonetheless, asthma should be monitored and ICS therapy stepped down to the lowest effective dose. Low-to medium-dose ICSs are not associated with the development of cataracts or glaucoma in children, but high cumulative lifetime doses may slightly increase the prevalence of cataracts in adults and elderly patients. ICSs are recommended for use in pregnant women with asthma; budesonide is the only ICS rated Pregnancy Category B.

 From:The Journal of Family Practice



Oral corticosteroids are used in acute exercerbations not for long term management.

Neonatal conjuctivitis

"Silver eyed on day 1

Gon home within 7 days

only to return after a week

with trachs on his chlam

and again in a month

when the herpes showed up!"

Day 1:Chemical conjuctivitis

Day 3-7: Gonococcal

After wk 1: Chlamydia

After 3wks: Herpes 

Countdown

7 days to go!

Wao! excited actually...

Kaplan q bank scores were initially lower than I expected but have improved now, I have a simulated exam to take when today dawns but first, I've got to catch some sleep!

Remember

In constrictive pericarditis and tamponade, catheterisation shows the same diastolic pressure in all 4 chambers!

Reye syndrome

Encephalopathy following an acute viral illness  in a child due to aspirin use

high level of ammonia

liver biopsy shows fatty changes

No treatment. supportive care: correct hypoglycemia, anti emetics, electrolytes etc

Rash involving hands and feet

Most rashes don't involve hands and feet. If an acute illness with rash involves hands and feet, think of

• Syphilis (secondary)
• Rocky mountain spotted fever (centrifugal rash)
• Coxsackie virus (hand, foot and mouth disease)

Remember

Pronator drift is a sign of upper motor neurone lesion

Patient is asked to hold out both arms at shoulder level, fully extended with forearms supine, and hold the position.

If the patient is unable to hold the position and the forearm pronates, then pronator drift is positive and the patient most likely has a contralateral pyramidal tract (UMN) lesion.

Closing the eyes accentuates it.

School phobia

School phobia is a seperation anxiety disorder.

It must be present for at least 4 weeks

Order of changes at Puberty

At puberty, it's time to TAG along!
Girls:
Thelarche - breast development (usually the first sign of Gonardache -onset of true central puberty)
Adrenarche: axillary hair and Pubarche- pubic hair (Adrenarche:can preceed true puberty)
Growth acceleration
Then Menarche


Boys:
Testicular growth (due to gonardache)
Adrenarche : axillary and pubic hair
Growth acceleration
Then Spermache

Remember

If u see migratory thrombophlebitis, look for visceral malignancies.

Remember

FeNa> 2% = intrinsic renal disease i.e acute tubular necrosis or acute interstititial nephritis

In prerenal azotemia e.g due to dehydration, FeNa is <1%, urine sodium is <20mEq/L, urine osmolality> 500mEq/L
Kidney reabsorbs Na and water to compensate for the hypovolaemia or hypotension.

Pneumococcal vaccine indications for adults

age >65yrs

Chronic diseases (hrt disease, lung disease, DM, cochlear implants, CSF leak, SCD, alcoholism)

Immunosuppressive diseases (HIV/AIDS, MM, Lymphomas,Leukemias, Chronic renal failure, organ transplant, nephrotic syndrome etc)

Immunosuppressive therapy (long term steroids, chemotherapy, radiotherapy)

Smokers

Asthmatics

Nursing home residents

Splenectomized individuals

TOF

Tetralogy of Fallot
Pulmonary stenosis+ VSD+overriding aorta+RVH

Cyanotic congenital heart disease (The 5 Ts. others are TGA, Truncus arteriosus, Total anomalous pulm venous return, Tricuspid atresia)

Right to left shunt leads to mixing

May not be cyanotic at birth

Difficulty feeding, polycythaemia, finger clubbing, dyspnoea, FTT, murmur

Tet spells
sudden, marked increase in cyanosis followed by syncope ,often precipitated by crying, feeding or exercise , may also occur on waking up.
It is due to an increase in rt to left shunting of unoxygenated blood following a decrease in systemic vascular resistance or increased pulmonary vascular resistance, favouring the "right to left to aorta shunt"
It can be relieved by squatting or placing child in knee chest position (This increases afterload thus allowing more blood to go thru pulmonary circulation rather than systemic)


Chest x ray shows boot shaped heart and decreased pulmonary vascular markings.

Tangier disease

Tangerines in the throat!


Aut recessive, rare

Deficiency of ABCA1 transporter needed to transport cholesterol out of cells

Low serum cholesterol, No cholesterol to pick up leading to low HDL

cholesterol accumulation in cells and tissues

Enlarged, yellow or orange tonsils, premature artherosclerosis, hepatosplenomegaly, corneal clouding

nursing-resource.com

TTP or HUS?

Both are microangiopathic hemolytic anemias
Both present with

1. Thrombocytopenia
2. hemolytic anemia (low hct, schistocytes, unconjugated hyperbilirubinemia, high LDH etc)
3. Acute renal insufficiency (high BUN)
4. Fever
5. Altered mental status

To differentiate however,

Altered mental status is more prominent in TTP
Acute anuric renal failure is more prominent in HUS

less specific is that HUS more in children while TTP more in adults

Colles fracture

Fall on outstretched hand,

Fracture of distal radius and ulna, dorsal angulation= dinnerfork deformity

Tmt: closed reduction and long arm cast involving wrist and elbow works well

de Quervain tenosynovitis

Tendons involved can be remembered by ALEB (ALL Exclusively Breastfeeding mothers!)

Abductor pollicis Longus and Extensor policis Brevis

Remember, it occurs commonly in new mothers due to the awkward way they support the baby's head with their hand e.g during breastfeeding. It can also be occupational (washer woman, carpenters, musicians, office workers)

The Finkelstein test is positive


© Cork Emergency Medicine 2013.



Bell's palsy

a post infectious demyelinating facial neuritis.

Associated infections include:

Herpes simplex
Varicella zoster
EBV
Lyme dx (Borrelia)

Selective Estrogen Receptor Modulators

Clomifene: @ hypothalamus: antagonist

Raloxifene:                              @breast :antagonist   @bone: agonist   @uterus: antagonist

Tamoxifene:                            @breast :antagonist   @bone: agonist   @uterus: agonist

Others include: femarelle, toremifene etc.


All reduce risk of breast cancer
All reduce risk of postmenopausal osteoporosis
Tamoxifene increases risk of endometrial cancer
SERMs increase risk of DVT

Clomifene used for treatment of infertility(anovulation)
Raloxifene used for treatment of Osteoporosis and breast cancer
Tamoxifene used for treatment of breast cancer

Dieulafoy lesion

Upper GI bleeding with no risk factors and normal endoscopy findings: suspect Dieulafoy lesion- a submucosal vessel that fails to divide into capillaries but bleeds intermittently and retracts under the mucosa during non bleeding periods.

Fanconi anemia

Autosomal recessive

Aplastic anemia


Pancytopenia

Hyperpigmentation

Short stature

microcephaly

hypogonadism

upper limb anomalies

radswiki.net

Cure is BM transplant

Glaucoma screening

 > 40yrs with risk factors: every year

>40 but <60yrs, no risk factors: every 3-5 yrs

 >60yrs , no risk factors: every 1-2 yrs

Risk factors for glaucoma include:
Black race
High IOP
DM
Family history
Severe eye injury
Myopia
Chronic corticosteroids
Hypothyroidism

Treatment of Primary pulmonary hypertension

Mgt:
IV vasodilator  challenge (prostacyclin, NO, adenosine) in order to decide definitive treatment

Definitive treatment:
If responsive to vasodilator challenge, start Calcium channel blockers. Try for at least 3-6months, if no response use prostacyclin

If no response to vasodilator challenge, other modalities including Transplant depending on the NYHA classification

Anticoagulation for all patients (Warfarin)

Remember

Neonate turns blue when feeding, turns pink when crying = choanal atresia

may be part of the CHARGE Syndrome

Coloboma
Heart defects
Atresia of the choanae
Retardation (mental)
Genitourinary anomalies
Ear anomalies

suspect if you can't pass an intranasal catheter, confirm with  contrast CT.

pseudoseizures

Psychogenic non epileptic seizure
Triggered by emotional stress
Normal EEG
Normal prolactin level following episode unlike in neurogenic seizure in which prolactin is elevated within 20 minutes of seizure.
There may be side to side head movement, pelvic thrusting, eyes may be closed, no post ictal confusion or incontinence
Very easy to be taken as malingearing.
Tmt is psychotherapy +/- antidepressants. Do not use anticonvulsants.

ITP or TTP?

Immune Thrombocytopenic Purpura:
Isolated thrombocytopenia, no hemolysis, normal red cell morphology
Tmt: first line is steroids


Thrombotic Thrombocytopenic purpura:
Microangiopathy due to narrowing of small vessels by thrombus
Thrombocytopenia, fragmented rbcs, schistocytes, neurological symptoms.
Tmt: plasmapheresis

Remember

Physiologic steatorrhoea can occur in neonates especially preterm neonates due to low amounts of bile acids.
Treatment is to replace long chain triglycerides in formular with medium chain trig (which do not require bile acids for digestion).

Do not confuse with CF which will show other features like meconium ileus, family history etc.

Remember

Patient with sudden onset cardiac decompensation with acute pulmonary edema should have an ECG done first before any medications, to rule out arrhythmia e.g atrial fibrillation which may require immediate synchronised cardioversion.

If no arrhythmia, then reduce preload with diuretics, nitrates and morphine.

Remember

For all left heart stenotic murmurs, reducing preload (e.g diuretics) prolongs devt of pulmonary congestion

For all left heart regurgitant murmurs, drug treatment is aimed at reducing afterload (vasodilators)

For mitral valve prolapse, drug treatment (if symptomatic), is aimed at increasing preload (propanolol)

For hypertrophic cardiomyopathy, increasing preload reduces the murmur( squatting increases VR, beta blockers and calcium channel blockers allow more ventricular filling)

note: Valsava manoeuvre increases afterload, reduces preload. It therefore increases intensity of murmur in aortic stenosis but reduces the intensity in HCM.

Remember

Elderly woman with new onset ascites, no Liver involvement: investigate for ovarian CA

Countdown

14 days to go!

Still pushing through Kaplan qbank. Results not what I expected, I guess Kaplan's style of questions is quite different and I see I still haven't mastered a few details.

2nd round MTB going well, quite fast actually.

Need to work on paediatrics, still.

Let's do this!

Remember

Bloody diarrhoea + leucocytosis post abdominal aortic aneurysm repair, think of ischemic colitis (desc and sigmoid)

Confirm with colonoscopy or flexible sigmoidoscopy

Do colonic resection + colostomy

Renal tubular acidosis

 common USMLE topic

Non anion gap metabolic acidosis


Type 1:
Distal RTA
Failure of H+ secretion by the α intercalated cells of distal tubes
There's hypokalaemia
Met. acidosis is severe, more alkaline urine >5.3
May be associated with urolithiasis , rickets

Type 2:
Proximal RTA.
e.g Fanconi syndrome (prox tubular cell dysfunction)
Failure of  HCO₃⁻ reabsorption from the urine by the proximal tubular cells
There's hypokalaemia. Also associated with rickets
Met acidosis is less severe because distal tubular function is normal. urine PH<5.3

Type 4:
No aldosterone (Addisson's disease,ACEIs,ARBs,DM nephropathy,Sickle cell nephropathy)
or
Na/K pump not responding to aldosterone (NSAID nephropathy,CKD,)
There's hyperkalaemia, hyponatremia, hyperchloraemia
urine PH<5.3. . Met. acidosis is mild

Remember

Fixed splitting of S2 = ASD

Remember

Most senstive test for subarachnoid haemorrhage, SAH, is Lumbar puncture: shows xanthochromia

Tuberous sclerosis

-a neurocutaneous syndrome

Tubers in the brain

Ash leaf hypopigmented macules on the skin, more visible under uv light (woods lamp), facial angiofibroma, forehead plaques, periungual fibroma,

Retinal harmatoma

Presents in infancy with infantile spasms (seizures)  which may be confused with colic. (may be described as head bobbing, doubling up etc) Treat with ACTH

Dental involement (pits), lung involvement etc

Cardiac harmatoma

There may be learning disabilities, mental retardation or epilepsy.

Bone lesion differentials

Osteoid osteoma:  radioluscent (lytic) centre,usually <1cm surrounded by sclerotic lesion. Benign

Brodie's abscess: form of osteomyelitis, remains subacute for years before converting to chronic (draining) osteo. radioluscent lesion >1cm surrounded by irregular margin of sclerosis. Benign

Osteosarcoma: hard irregular spiculated lesion (moth -eaten or sun burst appearance) with periosteal elevation- Codman's triangle, . Malignant. seen more commonly in Male adolescents. Most common primary bone cancer

Ewing's sarcoma: lytic lesion with periosteal reaction- onion skinning. Malignant. Highly metastatic.Typically seen  in Male adolescsent

Primary sclerosing cholangitis

Damage to both intra and extra hepatic bile ducts. ERCP or MRCP shows beading (segmental narrowing and dilatation)of both intra and extra hepatic ducts.

Anti smooth muscle antibodies (ASMA)(in 20%-50%), ANA, p-ANCA (not specific)

Young Man with itching, jaundice, malabsorption syndrome, signs of cirrhosis etc.

Biopsy may show periductal sclerosis (onion skinning)
Tmt: Ursodeoxycholic acid, cholestyramine, vitamin supplements



Note: ASMA and ANA + jaundice are also present in autoimmune hepatitis but Auto immune hepatitis is xterised by
female preponderance,
extrahepatic autoimmune diseases
hypergammaglobulinemia.

Treatment of autoimmune hepatitis is low dose steroids.

Primary biliary cirrhosis

interlobular bile duct destruction

itchy, yellow, middle aged woman with high alkaline phosphatase, normal transaminases, high GGT, high bilirubin and positive Anti mitochondrial antibodies, ANA

+signs of cirrhosis
Liver biopsy shows periductal mononuclear infilterate with bile duct destruction

Tmt: Ursodeoxycholic acid, cholestyramine (reduces itching)

no cure

Coronary artery bypass graft

Indicated in patients with CAD with angiography showing >70% stenosis of left main coronary artery or >70% stenosis of 3 or more vessels (2 or more vessels if diabetic)

Internal mammary artery graft preferred over saphenous vein graft (restenoses in abt 5 yrs)

Needle stick injury

Risk of infection:


HBV > HCV > HIV > Other organisms

Status Epilepticus

Seizure lasting 30mins or more
or
2 or more seizures occuring without interval recovery

TMT:
Benzodiazepine> phenytoin >more phenytoin >Phenobarb>more phenobarb>G.A + Intubate

Complications post MI

Sinus bradycardia : give atropine if symptomatic

3rd degree AV block:  Usually ffing Inf wall MI (ST elevation in leads II, III and avf) - bradycardia, there's independent contraction of RA and RV leading to Canon A waves (in Jugular). Treatment is pacemaker

Right Ventricle infarction: (also Inf Wall MI), RV dysfxn leads to reduced pulmonary bld flow, low preload, hypotension especially with nitrate use, tachycardia. Lungs are clear.Tmt is high volume fluid infusion. Avoid nitroglycerin
(vasodilator).

Ventricular fibrillation/tachycardia: loss of pulse, ECG findings. Tmt: defibrillation if no pulse

Reinfarction: recurrence of pain, new signs of pulmonary edema, new rise in CK-MB (Troponin is useless high cos level remains high for 10-14 days after initial infarction unlike CK-MB- 1-2 days) Tmt: As new MI

Free wall rupture: usually 2-8 days post MI when scars are forming but can occur as early as within the first 24hours, leads to cardiac tamponade, hypotension, JV distension, sudden loss of pulse. Do Echo. Tmt: emergency pericardiocentesis then surgical repair

Septal rupture: septal defect forms, new pansystolic (VSD) murmur hrd best at LLSB, pulmonary congestion, step up in oxygen saturation as you move from right atrium to right ventricle. Do Echo

Valve rupture: valvular insufficiency, mitral regurgitation, new systolic murmur heard best at apex radiating to axilla. Do Echo


edited: mechanical complications (rupture) can occur as early as the first day of MI although more common days and weeks after.

Snake bite

Rattle snakes are the leading cause of snake bites in the USA.
Bites are usually provoked, "intoxicated (drunk) young man who sees snake and still approaches it".

Symptoms
Local: swelling, severe pain, tingling.
Systemic: weakness, anxiety, nausea and vomiting, bleeding, perspiration,heart failure.
No local symptoms within 1 cm of fang marks after 1 hour - ? no or minimal envenomation 
No edema or erythema in the area of the bite after 6–8 hours = no envenomation

Treatment:
Local: splint or immobilise. DO NOT INCISE OR SUCTION BITE WOUND
Envenomation: Crotaline antivenom ("antivenin" )

Wegener granulomatosis

Upper resp disease + lower resp disease, + glomerulonephritis
Other signs of vasculitis on skin,eyes, joints etc

Best initial: c-ANCA
most accurate test: biopsy
(lung biopsy best)

Tmt: Prednisone +cyclophosphamide.



note: Good pasture has similar presentation but no upper resp involvement and anti GBM abs r present (Best initial test).
kidney biopsy shows linear deposits. No signs of systemic vasculitis unlike in Wegener's granulomatosis.

Treatment of inflammatory bowel disease

Acute exacerbations: steroids

Chronic maintenance: mesalamine
note: mesalamine is an active  metaboliteof sulfasalazine. Sulfasalazine was previously used but stopped due to side effects (agranulocytosis, hypospermia) from its other metabolite sulfapyridine.


Note:
For perianal disease, give ciprofloxacin+metronidazole
Colectomy can cure UC but is not routine tmt.
Crohn's usually recurs at surgery site





P.S :
IBD serology-  ASk Crohn ANd Call it !
ASCA- Crohn
ANCA- Colitis (ulcerative colitis)  specifically p-ANCA

pronounce the underlined as one sentence.

Most accurate test however is endoscopy

Treatment of heart block

First degree (prolonged PR) :
No treatment


Mobitz I (prolonged PR, longer PR, even longer PR then dropped beat, cycle repeats. could be 2:1,3:1, etc.):
Treat underlying cause


Mobitz II (Just dropped beats. Constant PR intervals,no prolongation of PR with subsequent beats as in in type I):
Pacemaker


3rd degree (No relationship between P and QRS waves and the P rate is faster than QRS rate):
Pacemaker

Atropine is used in first degree and Mobitz I if the bradycardia is severe or symptomatic.
IV Atropine is first line in any severe symptomatic bradycardia.

Treatment of stable angina

i.e angina precipitated by exertion, relieved by rest

Stable angina is as stable and simple as A, B, Cerine!

A- aspirin
B- beta blocker
C- nitroglycerine

Treatment of ST segment depression / Unstable angina

Aspirin (or clopidrogel or prasugrel): prevents plaque rupture

Heparin: prevents further growth of thrombus

then

Nitroglycerin
Morphine
Oxygen
Beta blockers
ACEi
GP2a/3b inhibitors


No mortality benefit from Oxygen, Morphine or Nitrates
No need for thrombolytics in non ST seg elevation event. Just prevent further elongation by giving heparin.

Treatment of STE Myocardial infarction (STEMI)

Aspirin (or if allergic, clopidrogel or prasugrel)

next

Angioplasty (door to balloon time 90mins) or if not available, thrombolytics (door to needle time: 30mins)

• Angioplasty (PCI) is preferable to thrombolytics however in the absence of PCI, give thrombolytics preferably within 30mins of infarction, but can still be given up to  12hrs post MI

then

Beta blockers (if contraindicated then use CCBs)
ACEi/ARB (especially with ejection fraction <40%)
Oxygen
Statins (especially with LDL >100mg/dl)



WARFARIN IS USELESS IN MI TREATMENT
Heparin can be administered for a short time post angioplasty to prevent restenosing 

Treatment of Pulmonary embolism

1. Anticoagulation: Heparin + Warfarin. Stop Heparin after 5-7 days when Warfarin may've kicked in. Target is INR of 2-3

2. IVC filter:
if heparin is contraindicated
if recurrent embolic event even while on heparin or therapeutic Warfarin (INR 2-3)
if there's severe disease with right ventricular dysfxn and enlargement. The next embolus could be fatal so put a filter


3. Thrombolytics (t-PA- tissue plasminogen activator): if patient is too unstable or if there's RV dysfxn

Aspirin is useless in PE

Note: watch out for HITT with heparin therapy. - monitor cbc
HITT:
Patient on heparin
Thrombocytopenia
5-10 days after start of treatment
New thrombus or expansion of old one
Mgt: stop heparin, give thrombin inhibitor: Argatroban or lepirudin

Do not transfuse platelets in HITT

Sarcoidosis

African American, Woman
Dyspnoea
Erythema nodosum on legs
Hilar adenopathy, fibrosis on Cxray
lymph node biopsy shows non caseating granuloma
Granuloma may be producing vitamin D causing hypercalcemia, hypercalciuria
Restrictive pattern on PFT
Cardiac invovement: restrictive cardiomyopathy- diastolic dysfxn
Other features: enlarged parotids, facial nerve palsy, uveitis, hepatomegaly, CNS involement.

Tmt: Steroids (if symptomatic only)

Empirical antibiotics treatment

compiled for the purpose of preparation for the USMLE step 2

Bacterial Meningitis: Ceftriaxone+vancomycin+steroids+/- Ampicillin

Though no culture results yet, you would have done LP (xcept if contraindicated) so u have an idea if bacterial (wbc in thousands)
If immunosuppressed or neonate or elderly or alcoholic and gram stain yields nothing , add Ampicillin (for Listeria monocytogenes which r gram positive rods). However, if gram stain already shows gram positive cocci, Ampicillin will be unneccessary.
Don't use ceftriaxone in neonates, use cefotaxime
Steroids lower mortality in pneumococcal meningitis
If confirmed Neisseria meningitis, give Rifampicin or ciprofloxacin to close contacts, isolate patient.
If culture results yield susceptible pneumococci, stop vancomycin.


Otitis Media: Amoxicillin


Sinusitis: Amoxicillin (or trim/sulfa or doxycycline) + decongestant


Pharyngitis: Amoxicillin
Rapid strep test done in the office diagnoses strep pharyngitis within minutes so the tmt is not really 'empirical' so to speak
(for penicillin allergic pt: clindamycin or clarithromycin Or if allergy is only a rash, use cephalexin)


Severe infectious diarrhoea: Ciprofloxacin + fluids
Severe if: hypotension, fever, bloody diarrhoea, abdo pain or acidosis -in this scenario, suspect bacterial etiology

if mild infectious diarrhoea, give fluids only


Urethritis: Ceftriaxone (or cefixime)  + doxycycline (or Azithromycin)


PID: exclude pregnancy first and give Ceftriaxone+doxycycline (outpatient)
or if inpatient, cefotetan + doxycycline

If pregnant, substitute doxycycline with azithromycin or amoxycillin or erythromycin


UTI: ciprofloxacin
or Trim/sulfa or cephalexin in cystitis
(Nitrofurantoin in pregnancy)
or Ampicillin+gentamicin for Pyelonephritis
(with PyNeph, suspect abscess if persistent fever after 5-7 days of antibiotics, do Sonography or CT and drain)





Acute Prostatitis: Ampicillin+gentamicin
(Chronic prostatitis:Trim/sulfa *8wks)





Endocarditis: Vancomycin + gentamicin



Community acquired pneumonia:
As outpatient:
Azithro or Clarithromycin or doxycycline  (if existing comorbidities/previous antibiotics, give Levofloxacin)
As inpatient:
Levo or moxifloxacin or Ceftriaxone+azithromycin



Hospital acquired pneumonia (after >48hrs in hospital):
(Antipseudomonals)
Cefepime
Ceftazidime
piperacillin+tazobactam
Carbapenems



Lung abscess: Clindamycin or Penicillin
usually due to aspiration thus cover for anaerobes



Impetigo: (staph and strep)
mild-    Topical mupirocin or bacitracin or Repatamulin
severe- Oral doxycycline or clindamycin or Trim/sulfa

(some say topical alone is not enough!)


Erysipelas: (strep > staph),Cellulitis: (staph>strep)
mild:    oral dicloxacillin or cephalexin or if allergic, macrolide or clindamycin
severe: iv oxacillin or nafcillin or cefazolin or if allergic, clindamycin


Postpartum Endometritis:
Clindamycin + gentamicin


Spontaneous bacterial peritonitis:  Cefotaxime


edited 8/06/12

Countdown

21 days to go!


Starting kaplan Q bank today.
Finished UWorld Q bank (1.5) times 1 wk ago


Doing 2nd round MTB by Conrad Fischer


Doing targeted viewing of some Kaplan videos.



God is on my side, I can do this!

2° aldosteronism

Due to reduced intravascular volume( e.g overdiuresis, edematous states, diarrhoea,surreptitious vomitting) or Barter's syndrome where there's defect in kidneys' ability to conserve sodium.

There's:
high aldosterone
hypokalaemia
muscle weakness
metabolic alkalosis
high renin (renin dependent hyperaldosteronism)

Unlike 1° aldosteronism,
There's no hypertension
There may be edema

Sensitivity and screening

A good screening test is very sensitive. This means it picks up most cases of the disease so a negative result tells you with good level of certainty that the disease is absent. Thus a good screening test "rules out".  It therefore also has high number of false positives.
It is usually not very specific, i.e if it is positive, it may be due to something other than the disease but it must be very sensitive i.e if it is negative, the disease is most likely absent.

Expl is the Overnight Dexamethasone suppression test for Cushing's syndrome
If cortisol drops : Cushing's is ruled out
However, if cortisol remains high, it may be Cushing's or Stress or something else i.e it's not specific

Also, ESR for temporal arteritis
If low, TA is ruled out
If high, there are several inflammatory diseases that could be responsible so it's not specific

Sensitivity rules out with its negative results, SnOut, specificity rules in with its positive results, SpIn

A confirmatory test is therefore highly specific for the particular disease or event.

Factitious hypoglycemia

patient in medical profession or with a relative taking insulin

Devs hypoglycemia

exogenous insulin injection

low C peptide (unlike in insulinoma)

Factitious disorder: no secondary gain, just to assume sick role, draw attention

Type 1 DM with morning hyperglycemia

Type 1 DM on insulin with high morning glucose, possible cause?

1. Dawn effect: not enough insulin
soln:
Increase dose or add a bedtime NPH

2. Somogyi phenomenon: Too much insulin leading to hypoglycemia leading to counterregulatory hormones secretion leading to hyperglycemia
soln:
Decrease the amount of night insulin

So, mgt approach:
Assume Somogyi effect until proven otherwise
DO NOT INCREASE INSULIN UNTIL SOMOGYI HAS BEEN RULED OUT. SEVERE NOCTURNAL HYPOGLYCEMIA MAY OCCUR


Best initial step is to Check sugar at 3am for some nights to differentiate.

Somogyi phenomenon:    There's 3am hypoglycemia
Dawn effect:                     No 3am hypoglycemia

DM diagnosis

Patient presenting with:

Fasting glucose > 125mg/dl done twice
or
DKA
or
HONK hyperglycemia

DM complications

The reversible complications are the microvascular complications.

Retinopathy
Neuropathy
Nephropathy

Tight control of glucose can reverse them, so look for them.

Type 1 DM : microvascular complications don't develop until about 5 yrs after onset
onset is usually close to diagnosis 'cos they can't live for long time without insulin

Type 2 DM: check for microvascular complications at diagnosis. They probably have had the disease undiagnosed for a long time.

Retinopathy:
Do yrly Ophthalmology visit
Non proliferative. (dilated veins,microaneurysms,edema, hard exudates, haemorrhage) Ensure tight sugar control
Proliferative- (neovascularisation,cotton wool spots, haemorrhage.) Do laser rx to prevent blindness

Neuropathy:
mononeuropathy esp occulomotor
peripheral neuropathy
autonomic neuropathy e.g gastroparesis (give metochlopromide), hypotension, erectile dysfxn

Nephropathy:
Earliest fxnal abnormality is hyperfilteration
Nephrotic range proteinuria (if proteinuria present,give ACEi)



Target :Hb A1c <7, fasting sugar<125mg/dl

Hypocalcemia

If there's low calcium:
check albumin- drop in albumin by 1 drops Ca by 0.8, so calculate the real Ca level first if albumin is low.

If corrected calcium is still low then look for the cause:

• Low PTH
• Renal failure- low Vit D
• Hypomagnesemia -Mg needed for PTH function
• Low Vit D absorption

Note: if PO4 is low, PTH must be high (PTH causes excretion of PO4).

Familial hypocalcuric hypercalcaemia

Family history
Asymptomatic
Hypercalcaemia
Hypocalcuria
Do not treat

Behçet syndrome

Palatal ulcers & Genital ulcers
Erythema nodosum-like lesions
Uveitis & Arthritis (just like with Ankylosing Spondylitis or IBD)
CNS symptoms

Treat with steroids

Low back pain; indications for imaging

Do imaging (initial: X ray, most accurate :MRI) if  there's:

point tenderness + compression symptoms


IV drug use (risk of epidural abscess) + compression symptoms


History of cancer with suspected metastases  + compression symptoms


Sciatica (disc herniation) + compression symptoms


Cauda Equina syndrome


Ankylosing spondylitis: (fused vertebrae)
young man with stiffness & low back pain worsened by rest! + Archilles tendon pain, AV block, Aortic insufficiency, uveitis, arthritis in other jts
(do Xray sacroiliac jt as initial test)




Cauda Equina syndrome is xterised by:

• Saddle anaesthesia
• Loss of sphincteric tone
• Incontinence
• leg weakness
• erectile dysfunction

Compression symptoms include:

• focal neurological deficit
• sensory loss
• hyperreflexia

(if suspected, give steroids immediately before investigation)

Pseudogout

looks like OA, involves large joints, wrists, but not DIP or PIP.

Calcium pyrophosphate deposition unlike uric acid in gout

Rhomboid shaped , +vely birefringent crystals unlike the needle shaped -vely birefringent crystals of gout

Suspect in patient with haemochromatosis or hyperparathyroidism

Arthritis

Osteoarthritis: Degenerative disease. Obese, athlete, weight bearing joints, DIP> PIP, Herbeden nodescrepitus, stiffness less than 15mins, normal labs, jt space narrowing, osteophytes, bone cysts, dense subchondral bone.

Rheumatoid arthritis: Autoimmune disease. Female, PIP, MCP, wrists,ankles, knees, morning stiffness longer than 30mins, nodules, systemic involvement, Baker cyst, Carpal tunnel syndrome,  anti-CCP (sensitive), raised ESR. Most common COD is CAD.

Gout: Metabolic. Male, sudden big toe swelling, pain and redness at night after binge drinking. Tophi, kidney stones, asymptomatic periods.

SLE: Autoimmune. Female. Other features of SLE, normal X ray, present in 90%


Reactive (Reiter's) arthritis: preceeded by an infection by chlamydia, Yersinia, shigella, salmonella or campylobacter. "Can't see, can't pee, can't climb a tree." Uveitis, non gonococcal urethritis, arthritis, archilles tendon pain, circinate balanitis, oral ulcers, nail changes etc Treat the preceeding infection + NSAIDS or Sulfasalazine if no response

Cervical cancer screening

Every woman, 21-65 yrs : Pap smear for cytology every 3yrs

For those above 30, alternative is Pap smear for cytology + HPV testing every 5yrs


If cytology yields abnormal results do further tests

Atypical squamous cells of unknown significance, ASC-US:
The "significance" is not clear so check for the HPV virus
If HPV +, then do colposcopy
If HPV -, then patient goes home and returns for repeat pap smear in 12 months (instead of the usual 3 yrs)


Atypical squamous cells, cannot exclude HSIL, ASC-H:
Still not clear and even more suspicious, HSIL is a possibility so go straight and do colposcopy


Low grade squamous intraepithelial lesion, LSIL:
For premenopausal women including pregnant women, do colposcopy

For postmenopausal women, 3 options:

• Do colposcopy
• Do HPV and then colposcopy if +
• Wait and repeat in 6months and 12months

High grade squamous intraepithelial lesion, HSIL:
Do colposcopy


20% of CA cervix may arise from glandular cells but the majority, 80%, are from squamous cells

Colposcopy is done for visual inspection + targeted biopsy + endocervical curretage (EC is contraindicated in pregnancy).

Findings:

If colposcopy done following abnormal cytology does not show any CIN, repeat cytology in 12 months (or 6 months if HPV+)

CIN 1: usually regresses spontaneously
CIN 2 & 3: Cautery, LEEP, Cone biopsy

histo, blasto, coccidiomycosis

This is a very common USMLE topic....

Coccidiomycosis: "cough and bumps" - pneumonia, bumps (nodules) on legs (aka Dessert bumps)
California, dessert areas

Blastomycosis: "cough and blasts"- pneumonia, ulcers and warts, may be disseminated to kidneys, liver, brain etc
Decomposing leaves, wooded areas,

Histoplasmosis: "gives no history" -Asymptomatic in many. (Serious in HIV),
Mississippi, Missouri,Ohio etc, Bat droppings, caves


All can present with granuloma in the lungs

A-a gradient

A- Alveolar pO2
a-Arterial pO2

A= 150-1.2(pCO2)

A-a gradient: (normal is 5 to 15)

What is the difference in the amount of oxygen getting into the lungs and that getting from the lungs into the blood?

Answer= A-a gradient

Higher if there's :

Impaired diffusion at alveolar level e.g pulmonary edema,  thickened alveolar membrane 

Abnormal ventilation/perfusion ratio :

• Decreased alveolar perfusion (shunt)-Pulmonary embolus
• Decreased alveolar ventilation (dead space)- obstructive diseases, ARDS (Atelectasis/alveolar collapse)

Right to left shunt (blood by passes the alveoli, goes straight back to left side of the heart without getting oxygenated)

Obstructive or Restrictive lung disease

Obstructive

Low FEV1, normal FVC

Low FEV1/FVC ratio

High Residual Vol

Restrictive

Low FEV1, Low FVC

High or normal FEV1/FVC ratio

Low Residual Vol

Thyroid CA

Most common : Papillary

Papillary: good prognosis, spread to nodes, slow growing, hx of exposure to radiation (head & neck), Tmt:  surgery , Give T3, T4 to suppress TSH

Follicular: distant spread (heamatogenous),

Medullary: Associated with familial syndromes e.g MEN 2a & 2b, produce calcitonin

Anaplastic: worst prognosis,  no metastases, Focal growth, palliative tmt



A non functioning (normal TSH) thyroid nodule is more likely to be malignant if:

• history of head & neck radiation
• Male gender
• Older person
• lymph nodes present
• cold nodule on thyroid scan

You MUST do a FNA for every euthyroid nodule:
if  malignant do surgery,
if benign, do nothing
if follicular, it may be benign or malignant, FNA can't differentiate so treat as malignant- do surgery.

MEN 2b

MuMP

Mucosal neuroma
Medullary CA of thyroid
Phaeochromocytoma



Marfanoid habitus, GI symptoms

MEN 2a

PaMP


Parathyroid hyperplasia
Medullary CA of thyroid
Phaeochromocytoma

MEN1

Pi, Pa, Pa

Pituitary
Parathyroid
Pancreatic


(PiPaPa, PaMP and MuMP: MEN1, 2a and 2b)

Hashimoto's thyroiditis

Auto immune, chronic thyroiditis

fibrosis

Anti microsomal Abs

Hypothyroidism (may have very mild hyperthyroidism initially)

Graves or subacute thyroiditis ?

Both have symptoms of hyperthyroidism
Both have elevated T4 T3, Low TSH


However,
Radioactive iodine uptake, RAIU:
high in Graves, Low in subacute (De Quervain) thyroiditis

Thyroid palpation:
Tenderness in subacute thyroiditis
no tenderness in Graves

Opthalmopathy:
Only in Graves

ESR:
high in subacute thyroiditis


Mgt decision:
Subacute thyroiditis is transient (can occur post partum), only supportive care- Aspirin
Graves requires definitive tmt.

Thyroid storm

Emergency

Triad of : Altered mental status +fever + high output heart failure in a patient with history of previous Graves disease (hyperthyroidism).

The sudden exacerbation is precipitated by stressful event e.g surgery, trauma, infection etc.

Can also occur due to sudden release of preformed thyroid hormones during radioiodine therapy.

Graves disease

Auto immune (TSH receptor antibodies)

Goitre- diffuse

Hyperthyroidism (most common cause is Graves dx)

Ophthamopathy(only in Graves)

hyperthyroid sympoms : wt loss , tremors, heat intolerance, diarrhoea, palpitations, menstrual irregularities, bruit over goitre, may have pretibial myxedema , proximal muscle weakness (can occur in both hypo and hyper) etc.

High T3, T4, low TSH (primary hyperthyroidism)

High radioiodine uptake (same for toxic nodule)

Tmt:
Acute phase

1. Propanolol or Atenolol
2. Propylthioracil or methimazole

continue until patient is euthyroid

Definitive treatment

• Radioactive iodine (kill the thyroid)

continue until patient is hypothyroid, then

• thyroid replacement therapy

Note:
proptosis may worsen with tmt
Agranulocytosis may occur with PTU or Methimazole, monitor wbc.
if patient c/o fever, sorethroat etc while on tmt, stop meds and check wbc count
The agranulocytosis is reversible

In pregnancy,
Acute phase: low dose propanolol + low dose PTU. Do not use Methimazole in pregnancy
Definitive: Surgery in 2nd trimester. Do not use radio iodine in pregnancy

Hyperprolactinemia

causes:

• Prolactinoma

woman with infertility, amenorrhoea, galactorrhoea (usually microadenomas in females so no pressure symptoms)

Man with visual field deficit, headaches etc (usually macroadenoma in males so there r pressure symptoms)

• Primary hypothyroidism

Increased TRH stimulates excess prolactin production

• Drugs:

Dopamine antagonists (antipsychotics), SSRIs, TCAs, methyldopa, metochlopromide
Dopamine inhibits prolactin secretion so its antagonists remove this inhibitory effect

Tmt:

First line for all patients: Bromocriptine

Men with prolactinomas(macroadenoma) : surgery or radio

Old women with microadenoma , not concerned about fertility, : no treatment reqrd.

SIADH

Syndrome of inappropriate ADH secretion.
Too much ADH
Too little urine (anti diuretic), highly concentrated urine, reabsorbing too much water, too dilute plasma
Hyponatremia

Causes:
Look out for CNS diseases (CNS tumour, trauma, stroke etc),chronic Lung diseases (Lung tumour, TB) or medications (vincristine, vinblastine)

Tmt:
Treat primary cause
Ristrict water

If no response, induce nephrogenic DI with Lithium or Demeclocycline.

Acromegaly

pituitary tumour secreting GH

GH stimulates prodxn of insulin-like GF (Sommatomedin C) in the liver

Organomegaly

Increase in shoe size, increase in cap size, "can no longer remove wedding ring", coarse facial features, large tongue, body odour, deeper voice, joint pains, snoring, erectile dysfunction (co-secretion of prolactin by tumour)

Remember medscape Alan with Acromegaly . google this.
carpal tunnel syndrome, Obstructive sleep apnoe,  DM/glucose intolerance, Hypertension, Congestive Heart Failure

Most common cause of death is CHF

Initial test: IGF level

Most accurate test: glucose suppression test (GH level post glucose ingestion is abnormally high)


Tmt:
Carbagoline, Bromocriptine,
Octreotide

most respond to surgery.

Radiotherapy if not responsive

Diabetes insipidus

polyuria, polydypsia, can't concentrate urine. Dehydrated, yet producing dilute urine.

Patients feel well until they lose access to water and then become dehydrated because the body is not responding appropriately.

There's hypernatremia with neurological symptoms such as  confusion, disorientation, seizures, lethargy, coma.

ADH can be low(central) or high(nephrogenic)

Do water deprivation test

Treatment:

Central: Give Desmopressin

Nephrogenic:
Treat underlying causes e.g Kidney disease, medications(stop Lithium)
If no improvement, try hydrochlorothiazide! or amilioride! (These diuretics will help lose some salts while patient continues to drink water)

Psychogenic polydypsia

Patient with polydypsia, polyuria and symptoms of hyponatremia- lethargy, confusion, seizures, psychosis, death

Water deprivation test shows an almost normal response. There's a little deviation from normal bcos kidney's concentrating ability is slightly impaired by hyperdilution of the renal medulla.

May be seen in Schizophrenics, children,

positive family history

Oral contraceptives and associated risks

OCPs increase risk of

cervical cancer 
breast cancer,
DVT
hypertension.


OCPs reduce risk of

ovarian cancer
endometrial cancer

Remember

Check TSH and T4 in every patient with new atrial fibrillation  
 (? HYPERTHYROIDISM)

Check TSH and T4 in every patient with hyperprolactinemia    
(? HYPOTHYROIDISM)

Check TSH and T4 in elderly patient with sudden dementia + new hypercholesterolaemia +decreased deep tendon reflexes , constipation, weight gain etc
(? HYPOTHYROIDISM)

Pancreatic cancer

Elderly

Painless jaundice (cancer head of pancreas, obstructing bile flow)

Upper abdo  pain, radiating to the back (cancer originating from the body or tail of pancreas)

weight loss

Abdo mass

steatorrhea

+/- pruritus



risk factors: Family history, Elderly, smoking, obesity, DM

Superior vena cava syndrome

Suspect in patient with history and findings suggestive of bronchogenic carcinoma, with

shortness of breath
face or arm swelling
headache
upper chest vein distension
neck vein distension


Due to compression of SVC by tumour

Ventricular tachycardia

Life threatening!

Tachcardia + wide QRS (diff from a specific SVT called wide QRS SVT, however better to consider and treat as VT until proven otherwise)

Causes include electrolyte abnormalities, MI, drugs, long QT syndrome etc

Tmt: Immediate cardioversion-

If stable: Chemical (Amiodarone , Lidocaine) or Sync DC cardioversion

If unstable: has pulse- Synchronised DC cardioversion
                   no pulse - defibrillation


Note:
 Torsades de pointes :

is a different type of VT. The morphology of QRS varies from one complex to the other thus it's refered to as polymorphic VT. 
Caused by anything that can prolong QT interval. culprits include Quinidine, Procainamide, Sotalol, Amiodarone (least likely) etc

Tmt includes Magnesium sulphate infusion first!

Paroxysmal supraventricular tachycardia

Sudden onset palpitation
Dizziness
Chest pain
Dyspnea
+/- LOC

Due to

• AV node re-entry or
• AV  re-entry  (av node + accessory pathway)- WPW syndrome

ECG : Tachycardia + narrow QRS, retrograde P wave -right after QRS


In WPW, there's the Delta wave- slurred upstroke of the QRS with a short PR interval bcos the signal evades the normal AV node delay and rather passes thru the accessory pathway.

Treatment:
Initial: vagal maneouvres (vasalva, carotid massage)

Drug Tmt :
Adenosine to block AV node except for Wolff-Parkinson-White (AV blockers  r contraindicated in WPW becos it'll only promote passage thru accessory pathway.) Amiodarone for WPW

2nd line drugs include verapamil, diltiazem, metoprolol, digoxin

Cardioversion for unstable patients or those not responsive to medications.

Conn Syndrome

Primary Hyperaldosteronism

Hypertension
Hypokalaemia
Muscle weakness

Metabolic alkalosis

Hypernatremia

No edema
Adrenal tumour

Diag: Do Aldosterone/ Renin ratio (>30)

Tmt: Spirinolactone (can cause gynecomastia)
         Eplerenone
         Surgery

Glucagonoma

Presents like Diabete mellitus with Polyuria, Polydypsia, weight loss, hyperglycaemia but with a necrotizing dermatitis-  necrolytic migratory erythema(blistering and swelling in areas subjected to friction).

Malignant Pancreatic tumour (alpha cells)

Tmt :surgery

Cystic fibrosis

Defective Chloride transport due to defective CFTR gene (usually a 3base pair deletion)
Aut recessive
Thick mucus secretions causing recurrent sinopulmonary infections, bronchiectasis, FTT
Psedomonas causes Pneumonia (Treat with 2 agents with antipseudomonad coverage)
Pancreatic insufficiency leading to Fat malabsorption with deficiency of vitamins A D E and K, Chronic diarrhoea
Meconium ileus (failure to pass meconium, bilous vomitting, history of polyhydramnios, ground glass appearance on X ray, intestinal perforation)

Positive sweat chloride test (done twice) is diagnostic.

Tmt: high calorie diet, pancreatic enzymes replacement, fat soluble vitamins supplementation. Only life saving tmt is bilateral lung transplant.

Median survival is 30yrs

Pyloric stenosis

Congenital
Infant 4-8 wks old with projectile vomitting which has become more frequent and more forceful
Vomittus is non bilous, occurs after feeding
Visible peristalsis in upper abdo
Failure to thrive
Olive shaped upper abdo mass palpable after vomitting (may however be absent)
Abdo USS  confirms diagnosis: shows thick hypoechogenic ring in pyloric area
Tmt: surgery

Intussusception

2yr old Child with abdo pain
presents with features of small bowel obstruction

red currant jelly stool

sausage shaped abdo mass

USS shows target sign (one tube inside another)

Air contast enema both diagnoses and reduces it in most cases

Paediatric Hip joint pain

Slipped capital femoral epiphysis : Obese adolescent male, referred pain to knee usually present, loss of abduction and internal rotation of hip. Tmt: surgical pinning promptly to avoid avascular necrosis.

Legg-Calve-Perthes disease:  child 4-10yrs, male, avascular necrosis of femoral head, idiopathic. Tmt- splint or surgery

Septic arthritis:  Emergency. Acute onset, warm swollen jt, hematogenous spread following URTI, staph & strep,
hip is externally rotated, X ray usually normal,  wbc count is high, ESR is high.
Do USS guided aspiration- synovial fluid leucocyte >100,000 is definitely SA, do culture.
Tmt : drain immediately, empirical antibiotics nafcillin (or vancomycin ) + cephalosporin

Avascular necrosis in sickle cell anemia: African descent, history suggestive of SS

Transient synovitis:  male child 3-10yrs, following trauma or viral infection.

Exclude septic arthritis. if at least 3 of the following are present, do further workup.

Fever>39°C,
WBC >12,000,
ESR >40 ,
 Refusal to bear weight

Kawasaki Disease

Vasculitis following infection
Child less than 5yrs
Hyperaemic buccal mucosa, strawberry tongue, Fissured lips
Diagnosis is clinical
Criteria: High fever (>39°C) for 5 days + 4 of ffing: unilateral large lymph nodes,
                                                                                     rash,
                                                                                    mouth changes,
                                                                                    limb desquamation/edema
                                                                                    conjuctivitis


Tmt: IVIG and Aspirin. (unlike other rashes in children where aspirin is contraindicated cos of risk of Reye's syndrome )

May be complicated by Coronary artery aneurysm with resultant risk of MI.

Young child with fever, red eyes, red tongue, red palms and rash

Guillain-Barré syndrome

Ascending paralysis

albumino-cytologic dissociation in CSF(high protein, normal wbc)

Recent Campylobacter jejuni  or other infection e.g respiratory
Investigations: nerve conduction studies, LP

Risk of death from respiratory failure
Monitor pulmonary function: Peak inspiratory pressure and FVC

Tmt:  IVIG or  Plasmapheresis

Gaucher's disease

Gauchers has a 3G phone in his wrinkled paper bag!

Lyosomal storage disease
Deficiency of acid beta Glucosidase (Glucocerebrosidase)
hepatosplenomegaly
Ashkenazi Jews
Aut recessive
wrinkled paper appearance of macrophages
Erlenmeyer flask deformity of distal femur (aseptic necrosis) - seen in other conditions e.g SS Dx
Grey-brown pigmentation of forehead, hands and pretibial area

Tay-Sachs disease

Lysosomal storage disease
Deficiency of Hexosaminidase A (TAY)
Cherry-red spot on Macula
Aut recessive
Ashkenazi Jews
Onion skin appearance of lysosomes


mnemonic: Sac of onions!

Niemman-Picks Disease

Lysosomal storage dx
Deficiency of Sphingomyelinase
Cherry-red spot on Macula
Hepatosplenomegaly
Aut recessive
Common in Ashkenazi Jews
5month old infant experiences regression in developmental milestones.

No Man Picks his nose with his Sphingers without Regressing!

Renal cell CA

Adult with gross painless hematuria, think of malignancy: bladder, renal, ureters
Do Contrast Abdo CT or IVU
If nothing is seen, do cystoscopy.

Note: Bladder  mass more common than renal cell CA

Renal cell CA:
hematuria, flank pain , abdo mass in 10%. Presence of lft sided varicocoele which doesn't empty when recumbent, is highly suggestive of RCC.

Rhesus incompartibility

Every woman @ first prenatal visit, do Rh screen and Rh Ab titre.

In Rh negative woman, consider as sensitized if Ab titre is >1:4

If Ab titre> 1:16, do amniocentesis @ 16-20 wks gestation, check fetal cells for rhesus factor
If not sensitized or <1:16, repeat Ab titre @28 wks

If fetal cells r rhesus positive and mother's Ab titre >1:16 then fetus is at risk of Erythroblastosis
If fetal cells r rhesus negative, then there's no risk for the fetus even though mother is sensitized, continue usual prenatal care.

If fetus is rh + and at risk, check if fetus is already hemolysing : serial amniocetensis for amiotic fluid bilirubin
if mild, repeat in 2-3wks, if moderate, repeat in 1-2 wks, if severe consider intervention:
       Is fetus anemic?
       How low is Hct?
       umbilical cord blood hematocrit <25% ----intrauterine transfusion or deliver if >34 wks G.A

Give anti D immunoglobulin to all Rh negative pregnant women who remain unsensitized at 28 wks. Repeat after delivery if baby is rh positive.
If already sensitized, Rhogam is of no value, focus on fetal monitoring and intervene as appropriate.

After an abruptio or other events with suspected fetomaternal haemorrhage,
Do a rosette test (qualitative) to confirm fetomaternal haemorrhage.
If positive, do a kleihauer - Betke test to quantify the haemorrhage.
For every 15mls of fetal blood in maternal circulation, give 300microgram of Rhogam up to a maximum of 1500microgam.

Drug Intoxication

Cocaine: Euphoria, anorexia, pupillary dilatation, illusions , hypertension, tachcardia tmt:BZDP, antipsychotics, antihypertensives

Marijuana : confusion, slow response time, red eyes, increased appetite

TCA: seizures, arrythmias, wide QRS, anti cholinergic effects (dry mouth, constipation, urinary retention etc) tmt:protect the heart with NaCO3

Aspirin: hyperventilating, tinnitus, anion gap metabolic acidosis + resp alkalosis, tmt: Alkalinization of urine with NaCO3

Opiates: miosis (may be absent if other drugs r also present), drowsy,slurred speech, coma tmt:Naloxone

Benzodiazepines: drowsy, confused, blurred vision, agitated, coma, resp depression , hypotension tmt: Flumazenil

PCP: violence, nystagmus, seizure, agitation tmt:BZDP, antipsychotics

Beta blocker: coma, seizures, hypotension, bradycardia tmt: ABC, glucagon

Digoxin: GI symptoms- anorexia, nausea, vomitting, abdo cramps, yellow green distortion, drowsiness, dyspnea, bradycardia, hypotension tmt: Atropine then gastric lavage, correct electrolytes.

Headaches

Migraine:  Female pt, Chronic, + aura, related to menses, N&V, may be pptatd by emotions, Acute tmt :ergotamine, triptans, DO NOT COMBINE ERGOT WITH TRIPTANS-risk of prolonging vasospasms.
Prevention: Propanolol, CCBs, TCAs, SSRIs, Botox


Cluster: Male pt, episodic, retro-orbital, wakes patient up at night, may not occur for months to yrs, red eye, Horner syndrome, Acute tmt: 100% Oxygen,  triptans, ergotamine, Lithium, Steroids . Prevention - Verapamil, Lithium, Steroids


Tension : diag of exclusion, tmt: NSAIDS


Raised ICP : Early morning headaches, worsened by tilting head forward, visual changes, papilledema


Pseudotumour cerebri: raised ICP + normal CT/MRI, normal CSF content, Obese female, OCPs, tmt: wt loss, acetazolamide, repeated LPs for acute reduction, VP shunt if no response..


Giant cell arteritis: visual disturbances, temporal area, systemic symptoms, jaw claudication. markedly elevated ESR. tmt: steroid. Start prednisone immediately without waiting for biopsy confirmation, to save vision.

Nephrotic Syndrome

Anarsaca
periorbital edema
hypoalbuminemia
hyperlipidemia
hyperproteinuria >3.5g/day,
hypertension
Thrombosis (due to loss of protein c  & s and antithrombin)
Infections (Ig loss)

Initial test : Urinalysis
Most accurate for cause : Biopsy
Most accurate measure of proteinuria: Urine albumin/creatinine ratio = 24hrs urinary protein

Children : think minimal change dx
Adults : most common is focal segmental (initially membranous glomerulonephritis)
AIDS: think focal segmental

Renal Biopy done in Adults
Biopsy not reqd in children with suspected NS, treat empirically with prednisone. + cyclophosphamide if resistant

Dementias

Xteristic features:

Alzheimers : memory loss first before onset of behavioural changes, confusion, gets lost in familiar neighbourhood , neurofibrillary tangles, enlarged ventricles, (Drug Tmt: Donepezil)

Lewy body : Parkinsonism,  Hallucinations, fluctuations in cognition and alertness

Picks  dx :  behavioural changes more prominent and preceeds memory loss, aphasia, frontotemporal atrophy

Pseudodementia : Depression

Normal pressure hydroceph : urinary incontinence, gait ataxia (large vol LP is both diagnostic and therapeutic)

Creutzfeldt Jacob dx  : prion dx, rapidly progressing dementia, ataxia, myoclonus, gait abnormalities, seizures, memory loss, personality changes, hallucination, sharp triphasic synchronous discharge on EEG. Mostly sporadic.

Huntington : Early onset dementia, Ataxia, Chorea, Family history, Aut Dominant.

Aortic Dissection

Severe, tearing chest pain, radiating to the back/inter-scapular area
Hypertension (or hypotension)
Asymmetrical BP
BP lower in rt arm if tear extends into brachiocephalic artery
BP lower in left arm if tear extends into left Subclavian
signs of ischemic stroke if tear extends into common carotid
New murmur if aortic regurgitation, due to aortic root involvement

Best initial test is CX-ray : widened mediastinum
TEE more sensitive
Most accurate test however is angiography (but is not usually necessary and carries higher risks)

Tmt of Ascending aortic dissection is emergent surgery
Tmt of Descending aortic dissection is BP control first! Beta blocker to reduce shearing force then Nitroprusside.
Don't give Nitroprusside before Beta blocker in aortic dissection.