1. A 35 year old man with complaints of ptosis, with difficulty in chewing and
occasionally swallowing. There is no history of diplopia and on examination
there is a symmetrical ptosis, lateral arm abduction time of 60 seconds.
Repetitive nerve stimulation test shows decremental response. EMG shows
myopathic response. Anti –Ach receptor antibodies are negative. Probable
diagnosis is:
a. Ocular M.Gravis
b. Generalised M.Gravis
c. Since Anti-ach receptor antibodies are negative consider other diagnosis
d. Chronic Progressive external opthalmoplegia
Reference: Harrison 18th edition chapter 386
http://emedicine.medscape.com/article/1215103-overview
This question looks intially as in favour of myaesthenia gravis but the
following points are against the diagnosis of Myasthenia gravis
1. Gender given as MALE at 35 years. While MG is seen in men in 50-60 years
of age.
2. Symetrical Ptosis without Diplopia. In MG we have asymetrical ptosis.
3. No diurnal variation in severity of weakness mentioned
4. For ocular M.G. symptoms must persist for 3 years
5. Anti- ach receptor antibodies are absent in 50% cases of ocular
myaesthenia gravis but they would be absent in CPEO as well as.
6. In CPEO- Ciliary and iris muscles are not involved and hence diplopia
is absent.
I have also compiled harrison and Emedicine data on these two close
answers in a tabular fashion
4. 3 yr old child is having barking cough with drooling of saliva. His RR is 36/min
and Xray neck lateral view shows “thumb sign”. The probable diagnosis is:
a. Epiglottis
b. Croup
c. Foreign body
d. Abductor vocal cord palsy.
Answer is A : epiglottitis
Reference: chapter 382.1 nelson textbook
EPIGLOTTITIS: A otherwise healthy child suddenly develops a sore throat
and fever. Within a matter of hours, the patient appears toxic, swallowing is
difficult, and breathing is labored. Drooling is usually present and the neck
is hyperextended in an attempt to maintain the airway. The child may assume
the tripod position, sitting upright and leaning forward with the chin up and
mouth open while bracing on the arms. A brief period of air hunger with
restlessness may be followed by rapidly increasing cyanosis and coma.
Stridor is a late finding and suggests near-complete airway obstruction.
Complete obstruction of the airway and death can ensue unless adequate
treatment is provided. The barking cough typical of croup is rare. Usually, no
other family members are ill with acute respiratory symptoms.The diagnosis
requires visualization of a large, “cherry red” swollen epiglottis by
laryngoscopy. Occasionally, the other supraglottic structures, especially the
aryepiglottic folds, are more involved than the epiglottis itself.. Anxietyprovoking
interventions such as phlebotomy, intravenous line placement,
placing the child supine, or direct inspection of the oral cavity should be
avoided until the airway is secure. If epiglottitis is thought to be possible but
not certain in a patient with acute upper airway obstruction, the patient can
undergo lateral radiographs of the upper airway first. Classic radiographs of
a child who has epiglottitis show the “thumb sign”.
CROUP : Croup is a clinical diagnosis and does not require a radiograph of
the neck. Radiographs of the neck may show the typical subglottic narrowing
or “steeple sign” of croup on the postero-anterior view.
5. 3 year old child is having sore throat with high grade fever. On examination he
has a inflamed phyranx and exudate on tonsils with tender lymphadenopathy.
Which of the following is a non suppurative complication of these patients?
a. Acute rheumatic fever
b. Acute glomerulonephritis
c. Acute rheumatic fever and acute Glomerulonephritis both
d. Scarlet fever
Answer is C: Acute rheumatic fever and acute Glomerulonephritis both
Reference: CMDT 2013 ,page no x
Group A β – hemolytic streptococci (Streptococcus pyogenes) are the most common
bacterial cause of pharyngitis. Transmission occurs by droplets of infected secretions.
Group A streptococci producing erythrogenic toxin may cause scarlet fever in
susceptible persons.
Clinical Findings
A. Symptoms and Sings
“Strep throat” is characterized by a sudden onset of fever, sore throat, pain
on swallowing, tender cervical adenopathy, malaise, and nausea. The
pharynx, soft palate, and tonsils are red and edematous. There may be a
purulent exudate. The centor clinical criteria for the diagnosis of
streptococcal pharyngitis are temperature > 38oC, tender anterior cervical
adenopathy, lack of a cough, and pharyngotosillar exudates.
The rash of scarlet fever is diffusely erythematous, resembling a sunburn, with
superimposed fine red papules, and is most intense in the groin and axillas. It
blanches on pressure, may become petechial, and fades in 2-5 days, leaving a
fine desquamation. The face is flushed, with circumoral pallor, and the tongue is
coated with enlarged red papillae (strawberry tongue).
B. Laboratory Findings
Leukocytosis with neutrophil predominance is common. Throat culture onto a
single blood agar plate has sensitivity of 80-90%. Rapid diagnostic tests based
on detection of streptococcal antigen are slightly less sensitive than culture.
Clinical criteria such as the centor criteria, are useful for identifying patients in
whom a rapid antigen test or throat culture is indicated. Patients who meet two
or more of these criteria merit further testing. When three of the four are
present, laboratory sensitivity of rapid antigen testing exceeds 90%. When only
one criterion is present, streptococcal pharyngitis is unlikely. In high prevalence
settings or if clinical suspicion for streptococcal pharyngitis or if clinical
suspicion for streptococcal paryngitis is high, a negative antigen test or culture
should be confirmed by a follow up culture.
Complications
Suppurative complications include sinusitis, otitis media, mastoiditis, peritonsillar
abscess and suppuration of cervical lymph nodes.
Nonsuppurative complications are rheumatic fever and glomerulonephritis.
Rheumatic fever may follow recurrent epidoses of pharyngitis beginning 1-4
weeks after the onset of symptoms. Glomerulonephritis follows a single
infections with a nephriotogenic strain of streptococcus group A (eg, types 4, 12,
2, 49 and 60), more commonly on the skin than in the throat, and begins 1 – 3
weeks after the onset of the infection.
6. 28 year old lady with history of recurrent abortions and repeated episodes of
pain the calves is most likely to have deficiency of which of the following
a. Plasmin
b. Thrombin
c. Protein C
d. Factor 13
Answer is C: protein C
Reference: table 58.3 Harrison 18th Edition and NELSON chapter 478
Factor 13 deficiency and thrombin deficiency causes bleeding manifestations.
Congenital plasminogen deficiency has been mentioned in texts but not
congenital plasmin deficiency. Hence by exclusion the diagnosis is protein C
deficiency.
Thrombohilia should be suspected in patients has a history of recurrent
venous thromboembolism, venous thrombosis in a person younger than
40 years, a familial history of venous thromboembolism, and thrombosis
in unusual sites (eg, mesenteric vein, renal vein, hepatic and cerebral
thrombosis).
Homozygous deficiency of protein C presents with purpura fulminans in the first
few hr of life. Fresh frozen plasma (FFP) is the only immediately available source
of protein C. Amelioration of symptoms usually requires 10–15 mL/kg of FFP
every 8–12 hr. Clinical trials are in progress using a plasma protein C
concentrate, which eliminates the need for large amounts of FFP. A recombinant
activated protein C concentrate (drotrecogin-α) has been approved for the
treatment of adult sepsis, but it has not been approved for treating hereditary
deficiency
Thrombophilic disorders are usually associated with venous thrombosis.
However, protein S, protein C, ATIII deficiencies, and lupus anticoagulants have
been associated with arterial thrombosis.
Patients with protein C and S deficiencies can develop warfarin-induced skin
necrosis when placed on warfarin since protein C and S are vitamin K–
dependent factors and, hence are suppressed.
Remember: Purpura fulminans in infancy could suggest protein C deficiency.
These antibodies occur in about 20% of patients with systemic lupus
erythematosus (SLE), but they are also associated with other autoimmune
diseases. Lupus anticoagulants may occur in patients taking phenothiazines,
phenytoin, phenytoin, hydralazine, quinine, amoxicillin, and oral contraceptives.
Also REMEMBER : Clinical criteria for indicating the presence of lupus
anticoagulants (Sapporo criteria for the antiphospholipid syndrome) are as
follows:
· One or more arterial, venous, or small vessel thrombosis, affecting any organ or
tissue
· Pregnancy morbidity: The risk for maternal and fetal morbidity increases after
the 10th week of pregnancy. Fetal mortality in pregnancy can include
spontaneous abortions, prematurity, and stillbirths.
· Three or more unexplained consecutive spontaneous abortions after the 10th
week of gestation
7. A lady is pregnant and is a known case of juvenile myoclonic epilepsy and is
receiving sodium valproate. Which of the following drugs is best suited after
valproate for management of this patient
a. phenytoin
b. carbamazepine
c. levetiracetam/lamotrigine
d. Lacosamide
Answer is C: lamotrigine
Reference: Harrison 18th edition table 368-9 and 369-9
60 year old patient with renal failure. He also has bone pain with X ray limb
showing lytic lesions in the long bones. Serum electrophoresis shows M spike.
Peripheral smear shows 35 % plasma cells. Bone marrow shows presence of
immature plasma cells. Diagnosis is
a. Plasma cell Leukemia
b. Multiple myeloma
c. Smoldering myeloma
d. Monoclonal Gammopathy of unknown significance.
Answer: A: Plasma cell leukemia
Reference: http://www.medscape.com/viewarticle/731900
The question mentions Peripheral smear showing plasma cells >35% and not in
the bone marrow. Hence the diagnosis goes in favour of plasma cell leukemia.
The diagnostic criteria for Plasma Cell Leukemia include the
Presence of a circulating clonal plasma cell count of more than 2000/μl (if the
total white blood cell count is >10,000/μl) OR
The presence of more than 20% CIRCULATING plasma cells.
Since in this question one of the critieria is satisfied hence diagnosis is
Plasma cell leukemia
· PCL can be secondary PCL (s PCL) if arising from a known a diagnosis of plasma
cell myeloma or primary PCL (p PCL) if no prior history of plasma cell myeloma
can be ascertained. sPCL was felt to arise late in the course of plasma cell
myeloma and those patients have been described to have a worse prognosis
than those with p PCL. However, a recent report indicates that the median time
to transformation to s PCL from plasma cell myeloma is approximately 21
months. The primary form accounts for 60% of all cases of PCL.
· Patients with PCL may present with signs and symptoms commonly found
in patients with plasma cell myeloma, such as bone pain, anemia, renal
dysfunction, Hypercalcemia and lytic bone lesions; or clinical manifestations
normally found in patients with leukemia, including leukocytosis, anemia,
thrombocytopenia, infections and hepato-splenomegaly. In addition, these
patients may also have elevated levels of LDH and β-2-microglobulin. Patients
with PCL are also more likely to present with advanced stage disease (stage III)
and have more significant extramedullary involvement that can include the CNS
· In contrast to most patients with plasma cell myeloma, the malignant cells in
PCL frequently lack CD56, CD9, CD117 and human leukocyte antigen DR
expression. It has been proposed that weak or absent CD56 expression
could explain the propensity of these clonal plasma cells to leukemic
transformation and also account for the lower osteolytic potential and
higher rates of extra-medullary involvement seen in patients with PCL
· The use of traditional combination chemotherapy, such as vincristine,
Adriamycin, dexamethasone (VAD) or high-dose cyclophosphamide-Etoposide is
associated with response rates of up to 59% and median overall survival of 15–
20 months. More aggressive regimens such as fractionated high-dose
cyclophosphamide and dexamethasone with infusional vincristine and
Adriamycin (hyper CVAD) have been used alone or in combination with
thalidomide and have revealed encouraging results including complete response
(CR).
8. A patient has Pc02 of 30 mmHg, P02 of 105 mmHg and pH of 7.45.
diagnosis is compensated
a. respiratory acidosis
b. respiratory alkalosis
c. metabolic acidosis
d. metabolic alkalosis
· Well if you look at values it indicates hyperventilation of the patient
due to C02 washout with increased 02 and normal pH due to the
process of compensation.
· If in same question pH>7.45 we shall call it uncompensated
respiratory alkalosis
Else follow this table:
· If direction of change of pH and pC02 is both in same direction ie
like both reduced or both increased then problem is metabolic
· If direction of change of pH and PC02 is opposite, then the primary
problem is respiratory.
· Else use a pen and mark arrows for increase or decrease in the table
below and u shall see a pattern emerge which will solve all MCQ in
books.
9. Bilateral Balbinski sign is seen in :
a. brain- stem stroke
b. thalamus and basal ganglia damage
c. cerebellum damage
d. mid pontine lesions
Reference: Harrison 18th edition figure 370-10 point no M
10. A 60 year old patient with 60 Packet per year history of smoking is having
a cancer which shows small cells with high mitotic index. Which of the following
is correct for this patient
a. aggressive pyscho-social behaviour
b. repeated blood transfusions
c. thin limbs with centripetal obesity
d. increased thirst/ version 2 of this choice was gynaecomastia
Reference: Harrison chapter 100.
Answer is A due to limbic encephalitis causing aggressive behaviour due to
involvement of limbic system. The closest choice increased thirst due to SIADH, is
negated as Harrison writes in the para-neoplastic syndrome (chapter 100) that most
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patient of ectopic ACTH secretion are asymptomatic. Read on the next text for a
comprehensive logic for the question.
The clinical diagnosis of small cell cancer/oat cell cancer of lung which has many
paraneoplastic manifestations:
1. Cushing syndrome is due to ECTOPIC ACTH release by lung cancer. However
these patients due to this aggressive cancer CANNOT HAVE presentation of
centripetal obesity but weight loss and hence choice number C is ruled out.
2. SIADH due to increase secretion of ADH leading to hyponatremia , Most
patients with ectopic vasopressin secretion are asymptomatic and are
identified because of the presence of hyponatremia on routine chemistry
testing. Symptoms may include weakness, lethargy, nausea, confusion,
depressed mental status, and seizures. The severity of symptoms reflects
the rapidity of onset as well as the extent of hyponatremia. Hyponatremia
usually develops slowly but may be exacerbated by the administration of
IV fluids or the institution of new medications.
3. Limbic encephalitis (anti –HU antibodies) due to SCLC can present with
agitation and psycho-social behaviour though the following 4 criteria must be
satisfied.
· Subacute onset (<12 weeks) of seizures, short-term memory loss, confusion, and
psychiatric symptoms
· Neuropathologic or radiologic evidence (MRI, SPECT, PET) of involvement of the
limbic system
· Exclusion of other possible aetiologies of limbic dysfunction
· Demonstration of a cancer within 5 years of the diagnosis of neurologic
symptoms, or the development of classic symptoms of limbic dysfunction in
association with a well-characterized paraneoplastic antibody (Hu, Ma2, CV2,
amphiphysin, Ri)
4. Paraneoplastic syndromes are common in patients with lung cancer, especially
those with SCLC, and may be the presenting finding or the first sign of recurrence.
Ectopic production of parathyroid hormone (PTH), or more commonly, PTHrelated
peptide, is the most common life-threatening metabolic complication of
malignancy, primarily occurring with squamous cell carcinomas of the lung.
Clinical symptoms include nausea, vomiting, abdominal pain, constipation,
polyuria, thirst, and altered mental status.
· Hyponatremia may be caused by the syndrome of inappropriate secretion
of antidiuretic hormone (SIADH) or possibly atrial natriuretic peptide
MEDICINE 2013
(ANP). SIADH resolves within 1–4 weeks of initiating chemotherapy in the
vast majority of cases. During this period, serum sodium can usually be
managed and maintained above 128 meq/L via fluid restriction. Demeclocycline
can be a useful adjunctive measure when fluid restriction alone is insufficient. Of
note, patients with ectopic ANP may have worsening hyponatremia if sodium
intake is not concomitantly increased. Accordingly, if hyponatremia fails to
improve or worsens after 3–4 days of adequate fluid restriction, plasma levels of
ANP should be measured to determine the causative syndrome.
· Ectopic secretion of ACTH by SCLC and pulmonary carcinoids usually
results in additional electrolyte disturbances, especially hypokalemia,
rather than the changes in body habitus that occur in Cushing's syndrome
from a pituitary adenoma. Treatment with standard medications, such as
metyrapone and ketoconazole, is largely ineffective due to extremely high
cortisol levels. The most effective strategy for management of Cushing's
syndrome is effective treatment of the underlying SCLC. Bilateral adrenalectomy
may be considered in extreme cases.
· Skeletal–connective tissue syndromes include clubbing in 30% of cases (usually
NSCLCs) and hypertrophic primary osteoarthropathy in 1–10% of cases (usually
adenocarcinomas). Patients may develop periostitis, causing pain, tenderness,
and swelling over the affected bones and a positive bone scan. Neurologic–
myopathic syndromes are seen in only 1% of patients but are dramatic and
include the myasthenic Eaton-Lambert syndrome and retinal blindness with
SCLC, while peripheral neuropathies, subacute cerebellar degeneration, cortical
degeneration, and polymyositis are seen with all lung cancer types. Many of
these are caused by autoimmune responses such as the development of anti–
voltage-gated calcium channel antibodies in Eaton-Lambert syndrome. Patients
with this disorder present with proximal muscle weakness, usually in the lower
extremities, occasional autonomic dysfunction, and rarely with cranial nerve
symptoms or involvement of the bulbar or respiratory muscles. Depressed deep
tendon reflexes are frequently present. In contrast to patients with myasthenia
gravis, strength improves with serial effort. Some patients who respond to
chemotherapy will have resolution of the neurologic abnormalities. Thus,
chemotherapy is the initial treatment of choice. Paraneoplastic
encephalomyelitis and sensory neuropathies, cerebellar degeneration, limbic
encephalitis, and brainstem encephalitis occur in SCLC in association with a
variety of antineuronal antibodies such as anti-Hu, anti-CRMP5, and ANNA-3.
Paraneoplastic cerebellar degeneration may be associated with anti-Hu, anti-Yo,
or P/Q calcium channel autoantibodies. Coagulation, thrombotic, or other
hematologic manifestations occur in 1–8% of patients and include migratory
venous thrombophlebitis (Trousseau's syndrome), nonbacterial thrombotic
(marantic) endocarditis with arterial emboli, and disseminated intravascular
coagulation with hemorrhage, anemia, granulocytosis, and
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leukoerythroblastosis. Thrombotic disease complicating cancer is usually a poor
prognostic sign. Cutaneous manifestations such as dermatomyositis and
acanthosis nigricans are uncommon (1%), as are the renal manifestations of
nephrotic syndrome and glomerulonephritis
GYNAECOMASTIA is seen with large cell carcinoma of lung.
occasionally swallowing. There is no history of diplopia and on examination
there is a symmetrical ptosis, lateral arm abduction time of 60 seconds.
Repetitive nerve stimulation test shows decremental response. EMG shows
myopathic response. Anti –Ach receptor antibodies are negative. Probable
diagnosis is:
a. Ocular M.Gravis
b. Generalised M.Gravis
c. Since Anti-ach receptor antibodies are negative consider other diagnosis
d. Chronic Progressive external opthalmoplegia
Reference: Harrison 18th edition chapter 386
http://emedicine.medscape.com/article/1215103-overview
This question looks intially as in favour of myaesthenia gravis but the
following points are against the diagnosis of Myasthenia gravis
1. Gender given as MALE at 35 years. While MG is seen in men in 50-60 years
of age.
2. Symetrical Ptosis without Diplopia. In MG we have asymetrical ptosis.
3. No diurnal variation in severity of weakness mentioned
4. For ocular M.G. symptoms must persist for 3 years
5. Anti- ach receptor antibodies are absent in 50% cases of ocular
myaesthenia gravis but they would be absent in CPEO as well as.
6. In CPEO- Ciliary and iris muscles are not involved and hence diplopia
is absent.
I have also compiled harrison and Emedicine data on these two close
answers in a tabular fashion
2. 18 yr old boy is asymptomatic. On ECG he has a short PR interval with delta
waves. Which of the following is not routinely required for these patients?
a. Holter monitoring
b. Treadmill test
c. Reassurance
d. Beta blockers
Answer is B : Treadmill test
Reference : Chapter 233 harrison 18th edition
o The clinical diagnosis of the patient is WOLF Parkinson SYNDROME.
These patients have a bypass tract that allows conduction of impulses
from atria to ventricles without having to be subjected to the decremental
property of AV node.
o Generally, no activity restrictions are recommended in patients with
ECG findings of pre-excitation in the absence of tachycardia. They
should be restricted from high-risk professions (eg, airline pilot) and
may be restricted from competitive sports.
o Patients presenting with tachycardias and accessory pathways
should avoid participating in competitive sports, because
MEDICINE 2013
catecholamines can decrease the refractoriness of the bypass tract and
facilitate tachyarrhythmias. Patients with hypertrophic cardiomyopathy,
or the Ebstein anomaly should also abstain from competitive sports.
Walking on Treadmill can precipitate due to sympathetic stimulation
an arryhtmia and would not be recommended for patient of W.P.W.
o The Holter monitor records electrical signals from the heart via a series of
electrodes attached to the chest. Electrodes are placed over bones to
minimize artifacts from muscular activity. The number and position of
electrodes varies by model, but most Holter monitors employ between
three and eight. The device is responsible for keeping a log of the heart's
electrical activity throughout the recording period.More modern units
record onto digital flash memory devices. The data are uploaded into a
computer which then automatically analyzes the input, counting ECG
complexes, calculating summary statistics such as average heart rate,
minimum and maximum heart rate, and finding candidate areas in the
recording worthy of further study by the technician. Thus Holter
monitoring in these patients can identify if patient is having a life
threatening event which on recording will help us know that these
patients would or would not require a RADIOFREQUENCY ABLATION
to get rid of the accessory pathway.
3. 8 yr old boy is having muscle weakness. On examination he has large muscles in
legs and his CPK levels are reducing. He also has a sub normal IQ. Probable
diagnosis is :
a. Hereditary sensory and motor neuropathy
b. Myelin deficiency
c. Dystrophin deficiency
d. Congenital myopathy
Answer is C : dystrophin deficiency
Reference: chapter 387 harrison 18th edition
o Duchenne's dystrophy is present at birth, but the disorder usually
becomes apparent between ages 3 and 5 years. The boys fall frequently
and have difficulty keeping up when playing. Running, jumping, and
hopping are invariably abnormal.
o GOWER sign is seen with PSEUDOHYPERTROPHY of the calf muscles.
MEDICINE 2013
o Contractures of the heel cords and iliotibial bands become apparent by
age 6 years, when toe walking is associated with a lordotic posture
o By age 12 years, most patients are wheelchair dependent. Contractures
become fixed, and a progressive scoliosis often develops that may be
associated with pain. The chest deformity with scoliosis impairs
pulmonary function, which is already diminished by muscle weakness. By
age 16–18 years, patients are predisposed to serious, sometimes fatal
pulmonary infections. Other causes of death include aspiration of food and
acute gastric dilation.
o A cardiac cause of death is uncommon despite the presence of a
cardiomyopathy in almost all patients
o Intellectual impairment in Duchenne's dystrophy is common; the
average intelligence quotient (IQ) is 1 SD below the mean. Impairment
of intellectual function appears to be nonprogressive and affects verbal
ability more than performance.
o Serum CK levels are invariably elevated to between 20 and 100 times
normal. The levels are abnormal at birth but decline late in the
disease because of inactivity and loss of muscle mass. EMG
demonstrates features typical of myopathy. The muscle biopsy shows
muscle fibers of varying size as well as small groups of necrotic and
regenerating fibers. Connective tissue and fat replace lost muscle fibers. A
definitive diagnosis of Duchenne's dystrophy can be established on the
basis of dystrophin deficiency in a biopsy of muscle tissue or mutation
analysis on peripheral blood leukocytes, as discussed below.
o A diagnosis of Duchenne's dystrophy can also be made by Western blot
analysis of muscle biopsy specimens, revealing abnormalities on the
quantity and molecular weight of dystrophin protein. In addition,
immunocytochemical staining of muscle with dystrophin antibodies can
be used to demonstrate absence or deficiency of dystrophin localizing to
the sarcolemmal membrane. Carriers of the disease may demonstrate a
mosaic pattern, but dystrophin analysis of muscle biopsy specimens for
carrier detection is not reliable.
4. 3 yr old child is having barking cough with drooling of saliva. His RR is 36/min
and Xray neck lateral view shows “thumb sign”. The probable diagnosis is:
a. Epiglottis
b. Croup
c. Foreign body
d. Abductor vocal cord palsy.
Answer is A : epiglottitis
Reference: chapter 382.1 nelson textbook
EPIGLOTTITIS: A otherwise healthy child suddenly develops a sore throat
and fever. Within a matter of hours, the patient appears toxic, swallowing is
difficult, and breathing is labored. Drooling is usually present and the neck
is hyperextended in an attempt to maintain the airway. The child may assume
the tripod position, sitting upright and leaning forward with the chin up and
mouth open while bracing on the arms. A brief period of air hunger with
restlessness may be followed by rapidly increasing cyanosis and coma.
Stridor is a late finding and suggests near-complete airway obstruction.
Complete obstruction of the airway and death can ensue unless adequate
treatment is provided. The barking cough typical of croup is rare. Usually, no
other family members are ill with acute respiratory symptoms.The diagnosis
requires visualization of a large, “cherry red” swollen epiglottis by
laryngoscopy. Occasionally, the other supraglottic structures, especially the
aryepiglottic folds, are more involved than the epiglottis itself.. Anxietyprovoking
interventions such as phlebotomy, intravenous line placement,
placing the child supine, or direct inspection of the oral cavity should be
avoided until the airway is secure. If epiglottitis is thought to be possible but
not certain in a patient with acute upper airway obstruction, the patient can
undergo lateral radiographs of the upper airway first. Classic radiographs of
a child who has epiglottitis show the “thumb sign”.
CROUP : Croup is a clinical diagnosis and does not require a radiograph of
the neck. Radiographs of the neck may show the typical subglottic narrowing
or “steeple sign” of croup on the postero-anterior view.
5. 3 year old child is having sore throat with high grade fever. On examination he
has a inflamed phyranx and exudate on tonsils with tender lymphadenopathy.
Which of the following is a non suppurative complication of these patients?
a. Acute rheumatic fever
b. Acute glomerulonephritis
c. Acute rheumatic fever and acute Glomerulonephritis both
d. Scarlet fever
Answer is C: Acute rheumatic fever and acute Glomerulonephritis both
Reference: CMDT 2013 ,page no x
Group A β – hemolytic streptococci (Streptococcus pyogenes) are the most common
bacterial cause of pharyngitis. Transmission occurs by droplets of infected secretions.
Group A streptococci producing erythrogenic toxin may cause scarlet fever in
susceptible persons.
Clinical Findings
A. Symptoms and Sings
“Strep throat” is characterized by a sudden onset of fever, sore throat, pain
on swallowing, tender cervical adenopathy, malaise, and nausea. The
pharynx, soft palate, and tonsils are red and edematous. There may be a
purulent exudate. The centor clinical criteria for the diagnosis of
streptococcal pharyngitis are temperature > 38oC, tender anterior cervical
adenopathy, lack of a cough, and pharyngotosillar exudates.
The rash of scarlet fever is diffusely erythematous, resembling a sunburn, with
superimposed fine red papules, and is most intense in the groin and axillas. It
blanches on pressure, may become petechial, and fades in 2-5 days, leaving a
fine desquamation. The face is flushed, with circumoral pallor, and the tongue is
coated with enlarged red papillae (strawberry tongue).
B. Laboratory Findings
Leukocytosis with neutrophil predominance is common. Throat culture onto a
single blood agar plate has sensitivity of 80-90%. Rapid diagnostic tests based
on detection of streptococcal antigen are slightly less sensitive than culture.
Clinical criteria such as the centor criteria, are useful for identifying patients in
whom a rapid antigen test or throat culture is indicated. Patients who meet two
or more of these criteria merit further testing. When three of the four are
present, laboratory sensitivity of rapid antigen testing exceeds 90%. When only
one criterion is present, streptococcal pharyngitis is unlikely. In high prevalence
settings or if clinical suspicion for streptococcal pharyngitis or if clinical
suspicion for streptococcal paryngitis is high, a negative antigen test or culture
should be confirmed by a follow up culture.
Complications
Suppurative complications include sinusitis, otitis media, mastoiditis, peritonsillar
abscess and suppuration of cervical lymph nodes.
Nonsuppurative complications are rheumatic fever and glomerulonephritis.
Rheumatic fever may follow recurrent epidoses of pharyngitis beginning 1-4
weeks after the onset of symptoms. Glomerulonephritis follows a single
infections with a nephriotogenic strain of streptococcus group A (eg, types 4, 12,
2, 49 and 60), more commonly on the skin than in the throat, and begins 1 – 3
weeks after the onset of the infection.
6. 28 year old lady with history of recurrent abortions and repeated episodes of
pain the calves is most likely to have deficiency of which of the following
a. Plasmin
b. Thrombin
c. Protein C
d. Factor 13
Answer is C: protein C
Reference: table 58.3 Harrison 18th Edition and NELSON chapter 478
Factor 13 deficiency and thrombin deficiency causes bleeding manifestations.
Congenital plasminogen deficiency has been mentioned in texts but not
congenital plasmin deficiency. Hence by exclusion the diagnosis is protein C
deficiency.
Thrombohilia should be suspected in patients has a history of recurrent
venous thromboembolism, venous thrombosis in a person younger than
40 years, a familial history of venous thromboembolism, and thrombosis
in unusual sites (eg, mesenteric vein, renal vein, hepatic and cerebral
thrombosis).
Homozygous deficiency of protein C presents with purpura fulminans in the first
few hr of life. Fresh frozen plasma (FFP) is the only immediately available source
of protein C. Amelioration of symptoms usually requires 10–15 mL/kg of FFP
every 8–12 hr. Clinical trials are in progress using a plasma protein C
concentrate, which eliminates the need for large amounts of FFP. A recombinant
activated protein C concentrate (drotrecogin-α) has been approved for the
treatment of adult sepsis, but it has not been approved for treating hereditary
deficiency
Thrombophilic disorders are usually associated with venous thrombosis.
However, protein S, protein C, ATIII deficiencies, and lupus anticoagulants have
been associated with arterial thrombosis.
Patients with protein C and S deficiencies can develop warfarin-induced skin
necrosis when placed on warfarin since protein C and S are vitamin K–
dependent factors and, hence are suppressed.
Remember: Purpura fulminans in infancy could suggest protein C deficiency.
These antibodies occur in about 20% of patients with systemic lupus
erythematosus (SLE), but they are also associated with other autoimmune
diseases. Lupus anticoagulants may occur in patients taking phenothiazines,
phenytoin, phenytoin, hydralazine, quinine, amoxicillin, and oral contraceptives.
Also REMEMBER : Clinical criteria for indicating the presence of lupus
anticoagulants (Sapporo criteria for the antiphospholipid syndrome) are as
follows:
· One or more arterial, venous, or small vessel thrombosis, affecting any organ or
tissue
· Pregnancy morbidity: The risk for maternal and fetal morbidity increases after
the 10th week of pregnancy. Fetal mortality in pregnancy can include
spontaneous abortions, prematurity, and stillbirths.
· Three or more unexplained consecutive spontaneous abortions after the 10th
week of gestation
7. A lady is pregnant and is a known case of juvenile myoclonic epilepsy and is
receiving sodium valproate. Which of the following drugs is best suited after
valproate for management of this patient
a. phenytoin
b. carbamazepine
c. levetiracetam/lamotrigine
d. Lacosamide
Answer is C: lamotrigine
Reference: Harrison 18th edition table 368-9 and 369-9
60 year old patient with renal failure. He also has bone pain with X ray limb
showing lytic lesions in the long bones. Serum electrophoresis shows M spike.
Peripheral smear shows 35 % plasma cells. Bone marrow shows presence of
immature plasma cells. Diagnosis is
a. Plasma cell Leukemia
b. Multiple myeloma
c. Smoldering myeloma
d. Monoclonal Gammopathy of unknown significance.
Answer: A: Plasma cell leukemia
Reference: http://www.medscape.com/viewarticle/731900
The question mentions Peripheral smear showing plasma cells >35% and not in
the bone marrow. Hence the diagnosis goes in favour of plasma cell leukemia.
The diagnostic criteria for Plasma Cell Leukemia include the
Presence of a circulating clonal plasma cell count of more than 2000/μl (if the
total white blood cell count is >10,000/μl) OR
The presence of more than 20% CIRCULATING plasma cells.
Since in this question one of the critieria is satisfied hence diagnosis is
Plasma cell leukemia
· PCL can be secondary PCL (s PCL) if arising from a known a diagnosis of plasma
cell myeloma or primary PCL (p PCL) if no prior history of plasma cell myeloma
can be ascertained. sPCL was felt to arise late in the course of plasma cell
myeloma and those patients have been described to have a worse prognosis
than those with p PCL. However, a recent report indicates that the median time
to transformation to s PCL from plasma cell myeloma is approximately 21
months. The primary form accounts for 60% of all cases of PCL.
· Patients with PCL may present with signs and symptoms commonly found
in patients with plasma cell myeloma, such as bone pain, anemia, renal
dysfunction, Hypercalcemia and lytic bone lesions; or clinical manifestations
normally found in patients with leukemia, including leukocytosis, anemia,
thrombocytopenia, infections and hepato-splenomegaly. In addition, these
patients may also have elevated levels of LDH and β-2-microglobulin. Patients
with PCL are also more likely to present with advanced stage disease (stage III)
and have more significant extramedullary involvement that can include the CNS
· In contrast to most patients with plasma cell myeloma, the malignant cells in
PCL frequently lack CD56, CD9, CD117 and human leukocyte antigen DR
expression. It has been proposed that weak or absent CD56 expression
could explain the propensity of these clonal plasma cells to leukemic
transformation and also account for the lower osteolytic potential and
higher rates of extra-medullary involvement seen in patients with PCL
· The use of traditional combination chemotherapy, such as vincristine,
Adriamycin, dexamethasone (VAD) or high-dose cyclophosphamide-Etoposide is
associated with response rates of up to 59% and median overall survival of 15–
20 months. More aggressive regimens such as fractionated high-dose
cyclophosphamide and dexamethasone with infusional vincristine and
Adriamycin (hyper CVAD) have been used alone or in combination with
thalidomide and have revealed encouraging results including complete response
(CR).
8. A patient has Pc02 of 30 mmHg, P02 of 105 mmHg and pH of 7.45.
diagnosis is compensated
a. respiratory acidosis
b. respiratory alkalosis
c. metabolic acidosis
d. metabolic alkalosis
· Well if you look at values it indicates hyperventilation of the patient
due to C02 washout with increased 02 and normal pH due to the
process of compensation.
· If in same question pH>7.45 we shall call it uncompensated
respiratory alkalosis
Else follow this table:
· If direction of change of pH and pC02 is both in same direction ie
like both reduced or both increased then problem is metabolic
· If direction of change of pH and PC02 is opposite, then the primary
problem is respiratory.
· Else use a pen and mark arrows for increase or decrease in the table
below and u shall see a pattern emerge which will solve all MCQ in
books.
9. Bilateral Balbinski sign is seen in :
a. brain- stem stroke
b. thalamus and basal ganglia damage
c. cerebellum damage
d. mid pontine lesions
Reference: Harrison 18th edition figure 370-10 point no M
10. A 60 year old patient with 60 Packet per year history of smoking is having
a cancer which shows small cells with high mitotic index. Which of the following
is correct for this patient
a. aggressive pyscho-social behaviour
b. repeated blood transfusions
c. thin limbs with centripetal obesity
d. increased thirst/ version 2 of this choice was gynaecomastia
Reference: Harrison chapter 100.
Answer is A due to limbic encephalitis causing aggressive behaviour due to
involvement of limbic system. The closest choice increased thirst due to SIADH, is
negated as Harrison writes in the para-neoplastic syndrome (chapter 100) that most
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patient of ectopic ACTH secretion are asymptomatic. Read on the next text for a
comprehensive logic for the question.
The clinical diagnosis of small cell cancer/oat cell cancer of lung which has many
paraneoplastic manifestations:
1. Cushing syndrome is due to ECTOPIC ACTH release by lung cancer. However
these patients due to this aggressive cancer CANNOT HAVE presentation of
centripetal obesity but weight loss and hence choice number C is ruled out.
2. SIADH due to increase secretion of ADH leading to hyponatremia , Most
patients with ectopic vasopressin secretion are asymptomatic and are
identified because of the presence of hyponatremia on routine chemistry
testing. Symptoms may include weakness, lethargy, nausea, confusion,
depressed mental status, and seizures. The severity of symptoms reflects
the rapidity of onset as well as the extent of hyponatremia. Hyponatremia
usually develops slowly but may be exacerbated by the administration of
IV fluids or the institution of new medications.
3. Limbic encephalitis (anti –HU antibodies) due to SCLC can present with
agitation and psycho-social behaviour though the following 4 criteria must be
satisfied.
· Subacute onset (<12 weeks) of seizures, short-term memory loss, confusion, and
psychiatric symptoms
· Neuropathologic or radiologic evidence (MRI, SPECT, PET) of involvement of the
limbic system
· Exclusion of other possible aetiologies of limbic dysfunction
· Demonstration of a cancer within 5 years of the diagnosis of neurologic
symptoms, or the development of classic symptoms of limbic dysfunction in
association with a well-characterized paraneoplastic antibody (Hu, Ma2, CV2,
amphiphysin, Ri)
4. Paraneoplastic syndromes are common in patients with lung cancer, especially
those with SCLC, and may be the presenting finding or the first sign of recurrence.
Ectopic production of parathyroid hormone (PTH), or more commonly, PTHrelated
peptide, is the most common life-threatening metabolic complication of
malignancy, primarily occurring with squamous cell carcinomas of the lung.
Clinical symptoms include nausea, vomiting, abdominal pain, constipation,
polyuria, thirst, and altered mental status.
· Hyponatremia may be caused by the syndrome of inappropriate secretion
of antidiuretic hormone (SIADH) or possibly atrial natriuretic peptide
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(ANP). SIADH resolves within 1–4 weeks of initiating chemotherapy in the
vast majority of cases. During this period, serum sodium can usually be
managed and maintained above 128 meq/L via fluid restriction. Demeclocycline
can be a useful adjunctive measure when fluid restriction alone is insufficient. Of
note, patients with ectopic ANP may have worsening hyponatremia if sodium
intake is not concomitantly increased. Accordingly, if hyponatremia fails to
improve or worsens after 3–4 days of adequate fluid restriction, plasma levels of
ANP should be measured to determine the causative syndrome.
· Ectopic secretion of ACTH by SCLC and pulmonary carcinoids usually
results in additional electrolyte disturbances, especially hypokalemia,
rather than the changes in body habitus that occur in Cushing's syndrome
from a pituitary adenoma. Treatment with standard medications, such as
metyrapone and ketoconazole, is largely ineffective due to extremely high
cortisol levels. The most effective strategy for management of Cushing's
syndrome is effective treatment of the underlying SCLC. Bilateral adrenalectomy
may be considered in extreme cases.
· Skeletal–connective tissue syndromes include clubbing in 30% of cases (usually
NSCLCs) and hypertrophic primary osteoarthropathy in 1–10% of cases (usually
adenocarcinomas). Patients may develop periostitis, causing pain, tenderness,
and swelling over the affected bones and a positive bone scan. Neurologic–
myopathic syndromes are seen in only 1% of patients but are dramatic and
include the myasthenic Eaton-Lambert syndrome and retinal blindness with
SCLC, while peripheral neuropathies, subacute cerebellar degeneration, cortical
degeneration, and polymyositis are seen with all lung cancer types. Many of
these are caused by autoimmune responses such as the development of anti–
voltage-gated calcium channel antibodies in Eaton-Lambert syndrome. Patients
with this disorder present with proximal muscle weakness, usually in the lower
extremities, occasional autonomic dysfunction, and rarely with cranial nerve
symptoms or involvement of the bulbar or respiratory muscles. Depressed deep
tendon reflexes are frequently present. In contrast to patients with myasthenia
gravis, strength improves with serial effort. Some patients who respond to
chemotherapy will have resolution of the neurologic abnormalities. Thus,
chemotherapy is the initial treatment of choice. Paraneoplastic
encephalomyelitis and sensory neuropathies, cerebellar degeneration, limbic
encephalitis, and brainstem encephalitis occur in SCLC in association with a
variety of antineuronal antibodies such as anti-Hu, anti-CRMP5, and ANNA-3.
Paraneoplastic cerebellar degeneration may be associated with anti-Hu, anti-Yo,
or P/Q calcium channel autoantibodies. Coagulation, thrombotic, or other
hematologic manifestations occur in 1–8% of patients and include migratory
venous thrombophlebitis (Trousseau's syndrome), nonbacterial thrombotic
(marantic) endocarditis with arterial emboli, and disseminated intravascular
coagulation with hemorrhage, anemia, granulocytosis, and
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leukoerythroblastosis. Thrombotic disease complicating cancer is usually a poor
prognostic sign. Cutaneous manifestations such as dermatomyositis and
acanthosis nigricans are uncommon (1%), as are the renal manifestations of
nephrotic syndrome and glomerulonephritis
GYNAECOMASTIA is seen with large cell carcinoma of lung.
