-SS DNA virus
- Human pathogen is parvo virus B-19
Clinical manifestations
- Erythema infectiosum
fifth disease
Slapped cheek appearance
- Arthropathy
- Transient aplastic crisis in chronic hemolytic disease patient
- In immunodeficient patient it causes chronic anemia with reticulocytopenia
- Fetal infection leading to nonimmunohydrops fetalis
- Hemophagocytic syndrome
Focal segmental glomerulosclerosis - HIV , HBV , Parvo virus , coxsackie
Membrane proliferative glomerulonephritis - HBV , HCV , HIV , CMV , EBV
Diffuse proliferative glomerulonephritis - coxsackie virus
Membranous nephropathy - HBV , HCV
Endocapillary proliferative glomerulonephritis - Measles , Dengue
Mesangioproliferative glomerulonephritis - parvo virus , Mumps
Hemoptysis: Bronchial artery
Duodenal ulcer: Gastroduodenal artery
Gastric ulcer: Left Gastric artery
SDH: Bridging veins
EDH: Middle meningeal artery
Tonsillectomy: Paratonsillar vein
Menstruation: Spiral arteries
K!ssing disease-Glandular fever/infectious mononucleosis
k!ssing ulcer-anterior and posteriorduodenal ulcers/vulval ulcer
k!ssing tonsils-Hypertrophdparenchymatous tonsils(grade 4)
Kissing virus-EBV
Kissing peptide-GnRH
Kissing lesion-Donovanosis
Kissing arthritis-TB knee
Kissing appearance on x-ray-Bladderpapilloma
Most Common Site of Distant mets from Lung Ca – Adrenal > Liver > Brain
Most Common Site of Distant mets from Breast Ca – Vertebrae (Bone)
Most Common Site of Distant mets from Urinary Bladder Ca – Lung
Most Common Site of Distant mets from Colorectal Ca – Liver
Most Common Site of Distant mets from Prostate Ca – Bones
Most Common Site of Distant mets from Testicular Ca – Lung
Most Common Site of Distant mets from Thyroid Ca – Bone > Lung
Most Common Site of Distant mets from Wilm’s Tumor – Lung
TRIAD OF ALPORT'S SYNDROME --
SENSORINEURAL DEAFNESS,
PROGRESSIVE RENAL FAILURE,
OCULAR ANOMALIES
TRIAD OF BEHCET'S SYNDROME --
RECURRENT ORAL ULCERS,
GENITAL ULCERS,
IRIDOCYCLITIS.
BECK'S TRIAD --
MUFFLED HEART SOUND,
DISTENDED NECK VEINS,
HYPOTENSION.
CHARCOT'S TRIAD --
PAIN
FEVER
JAUNDICE
GRADENIGO'S TRIAD -
SIXTH CRANIAL N. PALSY,
PERSISTANT EAR DISCHARGE,
DEEP SEATED RETRO-ORBITAL PAIN
Triad Of Hypernephroma --
pain
hematuria
renal Mass.
Hutchinson's Triad ---
Hutchison's Teeth,
Interstitial Keratitis,
Nerve Deafness.
Triad Of Kwashiorkar-
Growth Retardation,
Mental Changes,
Edema.
Saint's Triad ---
Gall Stones,
Diverticulosis,
Hiatus Hernia.
Trotter's Triad --
Conductive Deafness,
Immobility Of Homolateral Soft Palate,
Trigeminal Neuralgia.
VIRCHOW'S TRIAD --
STASIS
HYPERCOAGULABILTY
VESSEL INJURY.
Congenital Rubella syndrome--
PDA
deafness
cataract
SAMTER'S TRIAD --
ASPIRIN SENSITIVITY,
BRONCHIAL ASTHMA,
NASAL POLYP.
Grancher's triad--
lessened vesicular quality of breathing,
skodaic resonance,
increased vocal fremitus; seen in early pulmonary tuberculosis.
Osler's triad--
telangiectasis,
capillary fragility,
hereditary hemorrhagic diathesis seen in hereditary hemorrhagic telangiectasia.
BARTTER'S SYNDROME -
METABOLIC ALKALOSIS,
HYPOKALEMIA,
NORMAL OR DECREASED BP.
Weil's Disease--
Hepatorenal Damage,
Bleeding Diathesis,
Pyrexia.
Meniere's Disease --
Vertigo,
Tinnitus,
Sensorineural Hearing Loss.
Melkersson Rosenthal Syndrome--
recurrent Facial Palsy,
Plication Of Tongue,
Facial Edema.
Parkinsonism--
Rigidity,
Tremor
Hypokinasia
Cushing's traid --
Bradycardia,
hypertension
irregular respirations in increased intracranial pressure.
Kartagener’s Syndrome--
bronchiectasis,
Recurrent sinusitis,
Situs inversus.
Hemobilia(Triad of Sandblom)--
Malena,
Obstructive jaundice,
Biliary colic.
Murphy's triad(in order)-
Pain,
Vomitting,
Fever.
WHIPPLE'S TRIAD Of INSULINOMA--
HYPOGLYCAEMIA DURING ATTACKS,
S.GLUCOSE <40 mg%,
PROMPT RELIEF ON GLUCOSE ADMINISTRATION.
* Yellow cartilage - another name of elastic cartilage
* white papery tongue - it is due to fungal infection (yeast) THRUSH....in this white bump form on tounge.
* blue lip - cyanosis Reason in ENT for blue lip is laryngeal edema...
* flamingo red sign - Reddish Hue Seen Through The Tympanic Membrane +ve In Case Of Oto Sclerosis (active )n Glomus Tumor
Scurvy is a common nutritional disorder in developing countries due to high prevalence of malnutrition. Vitamin C performs various important functions in the body. It is required for synthesis of supporting tissues of mesenchymal origin like osteoid in bone, chondroid in cartilage, dentin in teeth and collagen in capillary walls. It enhances iron absorption from the gastrointestinal tract, acts as an anti-oxidant, required in the coagulation pathway, also in tyrosine metabolism and synthesis of corticosteroids. Amla, guava and citrus fruits are rich dietary sources of vitamin C. Human breast milk has five times more vitamin C than cow's milk, thus breast fed infants are less prone to develop scurvy. Delay in starting weaning foods, improper dietary habits and recurrent diarrhea predispose to scurvy.
The 5 Ps of lichen planus are used to describe the skin lesion.
They are:
planar
polygonal
pruritic
purple
papular
The 5 P's of paralytic ileus refers its common causes.
They are:
postoperative
peritonitis
potassium: low pelvic
and spinal fractures parturition
The 5 P's of cretinism refer to the features of an infant with congenital hypopituitarism.
They are:
pot belly
pallor
puffy face
protruding umbilicus
poked out tongue
The 5 P's of mercury poisoning refer to changes in the skin.
They are:
pink
puffy
painful
paraesthesia
peeling
Q 1. which one of the following diseases characteristically causes fatty change in liver?
A. Hepatitis
B virus infection
B. Wilson’s disease
C. Hepatitis C virus infection
D. Chronic alcoholism
ANSWER. D
Q 2. Under the WHO ‘Vision 2020‘ programme. the ‘SAFE‘ strategy is adopted for which of the following diseases?
A. Trachoma
B. Glaucoma
C. Diabetic retinopathy
D. Onchocerciasis
ANSWER A
Q 3. WHO defines adolescent age between:
A. 10-19 years of age
B. 10-14 years of age
C. 10-25 years of age
D. 9-14 years of age
ANSWER. A
Q 4. Which is not an autoimmune disease?
A. SLE
B. Grave’s disease
C. Myasthenia gravis
D. Sickle cell disease
ANSWER. D
Q 5. Most common nerve injured in supracondylar fracture humerus?
A. Median
B. Radial
C. Ulnar
D. Anterior interosseous nerve
ANSWER.
Q 6. Which of following is the feature of Y chromosome?
A. Acrocentric
B. Telocentric
C. Submetacentric
D. Metacentric
ANSWER. A
Q 7. Auto-Rikshaw ran over a child’s thigh, there is a mark of the tyre tracks, it is an
A. Contact bruise
B. Patterned bruise
C. Imprint abrasion
D. Ectopic bruise
ANSWER. C
Q 1. All of the following are included in basal ganglia except
a. Lenticular nucleus
b. Globus pallidus
c. Thalamus
d. Claustrum
ANSWER C
Q 2. BAL is useful in treating poisoning due to all except?
A. Lead
B. Organic mercury
C. Cadmium
D. Arsenic
ANSWER C
Q 3. A 30 year old pregnant woman developes tuberculosis. Which of the following antitubercular drugs should not be used:
A. INH
B. Rifampicin
C. Streptomycin
D. Ethambutol
ANSWER. C
Q 4. Morphine can be used in all the following conditions except:
A. Head injury
B. Asthma
C. Hypothyroidism
D. Diabetes
ANSWER. A
Q 5. Which among the following is a cardioprotective fatty acid?
A. Palmitic acid
B. Stearic acid
C. Oleic acid
D. Omega-3 fatty acids
ANSWER. D
Q 6. Orthotolidine test is used for detecting:
A. Chlorine
B. Nitrites
C. Nitrates
D. Ammonia
ANSWER. A
Q 7. Which features are seen with the Tetrology of Fallot?
A. Ventricular septal defect
B. Overriding of aorta
C. Right ventricular outflow obstruction
D. Right ventricular hypertrophy
E. All of the above
ANSWER E
Q 8. In a 25 year old male / female having generalized restriction of eye movement in all the directions, intermittent ptosis, proximal muscle weakness, fatiguability. The most useful test is ?
A) CPK
B) Edrophonium test
C) EMG
D) Muscle Biopsy
ANSWER. B
Q 9. Most common tumor of eyelid is ?
A) Sebaceous Ca
B) Basal cell Ca
C) Squamous cell Ca
D) Malignant melanoma
ANSWER. B
Q 10. An alcoholic with poor judgment and decreased in skilled motor movements. Blood alcohol level would be
A. 30-80 mg/dl
B. 80-200 mg/dl
C. 200-300 mg/dl
D. >300 mg/dl
ANSWER. B
IMPORTANT SIGNS IN OBSTETRICS AND GYNAECOLOGY:
Bagel sign:
Ultrasonographic sign. Gestational sac in the adnexa with hyperechoic ring
Ball Sign:
Radiological sign of intrauterine fetal death. X-ray shows crumpled up spine of the fetus..
Banana Sign:
Ultrasound sign in open spina bifida. Shows abnormal anterior curvature of cerebellum. Due to the associated Arnold-Chiari malformation.
Chadwick’s Sign:
Bluish hue to the vestibule and anterior vaginal wall. Seen in first trimester of pregnancy. Cause is increased blood flow to the pelvic organs. Also known as jacquemier’s Sign.
Cullen Sign:
Bluish discoloration of skin around umbilicus. Occurs due to intra peritonealhemorrhage. Seen in ruptured ectopic pregnancy.
Double Bubble Sign:.
Useful in prenatal diagnosis of duodenal atresia. Duodenal atresia usuallypresents with polyhydramnios and produces dilatation of stomach and first part of duodenum
Double decidual sac Sign:
Normal Ultrasonographic appearance of intrauterine gestational sac. Seen as twoconcentric echogenic rings separated by a hyperechoic space.
Goodell Sign:
Marked softening of the cervix in contrast to non pregnant state. Also due toincreased blood flow.
Hegar Sign:
An indication of Pregnancy. Softening of the lower parts of the uterus enablesapproximation of vaginal and abdominal fingers in bimanual pelvic examination. Vaginalfingers are placed in the posterior fornix and abdominal hand pressed down behind theuterus. Jacquemier’s sign: Refer Chadwick
SignKustner’s Sign:
Sign of placental separation. On pushing the uterus upwards does not move thecord with it due to the separation.
Ladin’s Sign:
Softening in the midline of the uterus anteriorly at the junction of the uterus andcervix. It occurs at about 6 weeks gestation
Lambda Sign:
Ultrasonographic sign seen in dichorionic pregnancies. Due to the chorionic tissuein between the two layers of the membrane between the twins.
Lemon Sign:
Ultrasound sign in open spina bifida. Shows abnormal anterior curvature of cerebellum. Due to the associated Arnold-Chiari malformation.
Osiander’s Sign:
Pulsations in the lateral fornix due to the increased vascularity.
Palmer’s Sign:
Regular rhythmic contractions of uterus felt as early as 6-8 weeks . It is a sign of pregnancy.
Piskacek’s Sign:
Asymmetric growth occurs to the uterus in initial stages of pregnancy due to thelateral implantation of the blastocyst. The area of implantation feels soft compared tothe other parts.
Robert’s Sign:
Radiological sign of intrauterine fetal death. X-ray shows presence of gas in thefetal great vessels. Earliest radiological sign of intrauterine fetal death
Schroder’s Sign:
A sign of placental separation. Uterus rises up when the separated placenta ispassed downwards.
Spalding Sign:
Sign of intrauterine fetal death. Overlapping of skull bones after fetal demise.Observed by ultrasonogrm.
Stallworthy’s Sign:
Slowing of fetal heart rate on pressing the head down I to the pelvis and promptrecovery on release of pressure is termed Stallworthy’s sign. This sign is suggestive of posterior placenta praevia.
Stuck Twin Sign:
Seen in twin to twin transfusion syndrome. Due to the severe oligohydramniossmaller twin is held in a fixed position along the uterine wall. This is called stuck twinsign.
‘T’ Sign:
Ultrasonographic sign seen in monochromic twins. As the intertwin membranedoes not have any chorionic tissue it gives rise to ‘T’ sign in ultrasound
CASE A 20-year-old college student is your next patient in the emergency room. When you walk into the room, he is lying on the examination table, on his side, with his arm covering his eyes. The light in the room is off. You look at his chart and see that the nurse recorded his temperature as 102.3°F, heart rate 110 bpm, and blood pressure 120/80 mm Hg. When you gently ask how he has been feeling, he says that for the past 3 days he has had fever, body aches, and a progressively worsening headache. The light hurts his eyes and he is nauseated, but he has not vomited. He has had some rhinorrhea, but no diarrhea, cough, or nasal congestion. He has no known ill contacts. On examination, he has no skin rash, but his pupils are difficult to assess because of photophobia. Ears and oropharynx are normal. Heart, lung, and abdomen examinations are normal. Neurologic examination reveals no focal neurologic deficits, but passive flexion of his neck worsens his headache, and he is unable to touch his chin to his chest.
#What condition are you concerned about?
#What diagnostic test would confirm the diagnosis?
Summary: A 20-year-old college student presents with a 3-day history of fever, headache, myalgias, and nausea. He has no respiratory or gastrointestinal symptoms, but now has developed photophobia. He is febrile to 102.3°F, tachycardic, and normotensive. His physical examination is generally unremarkable with a nonfocal neurologic examination but some neck stiffness, suggesting meningeal irritation. He has no skin lesions as might be seen in meningococcemia.
#Most likely condition: Meningitis.
#Diagnostic test to confirm diagnosis: Lumbar puncture (LP) for evaluation of the cerebrospinal fluid (CSF), possibly preceded by a computed tomographic (CT) scan of the head.
ANALYSIS
Considerations This 20-year-old college student has headache, nausea, photophobia, fever, and neck pain and stiffness—all suggestive of meningitis, which could be bacterial or viral. Prompt LP and analysis of CSF are essential to establish the diagnosis. In a patient without focal neurologic signs and a normal level of consciousness, CT scan may be unnecessary prior to performing an LP. If he had a purpuric skin rash, one would be suspicious of Neisseriameningitis, and appropriate antibiotics should be administered immediately. Dosing of antibiotics in suspected meningococcal infection should not await the performance of any diagnostic test because progression of the disease is rapid, and mortality and morbidity are extremely high even when antibiotics are given in a timely manner.
DEFINITIONS
MENINGITIS:A serious inflammation of the meninges, the thin, membranous covering of the brain and the spinal cord, which can be caused by bacteria, viruses, fungi, or protozoa.
PAPILLEDEMA: Swelling of the optic nerve, caused by an increased intracranial pressure. On fundoscopic examination, the optic disc margin appears hazy.
CLINICAL APPROACH
Bacterial meningitis is the most common pus-forming intracranial infection, with an incidence of 2.5 per 10,000 persons. The microbiology of the disease has changed somewhat since the introduction of the Haemophilus influenzae type B vaccine in the 1980s. Now Streptococcus pneumoniaeis the most common bacterial isolate, with Neisseria meningitidisa close second.Group B streptococcusorStreptococcus agalactiaeoccurs in approximately 10% of cases, more frequently in neonates or in patients older than 50 years or with chronic illnesses such as diabetes or liver disease. Listeria monocytogenesaccounts for approximately 10% of cases and must be considered in pregnant women, the elderly, or patients with impaired cell-mediated immunity such as AIDS (acquired immunodeficiency syndrome) patients. Haemophilus influenzaeis responsible for less than 10% of meningitis cases. Resistance to penicillin and some cephalosporins is now of great concern in the treatment of S pneumoniae.
Bacteria usually seed the meninges hematogenously after colonizing and invading the nasal or oropharyngeal mucosa. Occasionally, bacteria directly invade the intracranial space from a site of abscess formation in the middle ear or sinuses. The gravity and rapidity of progression of disease depend upon both host defense and organism virulence characteristics. For example, patients with defects in the complement cascade are more susceptible to invasive meningococcal disease. Patients with CSF rhinorrhea caused by trauma or postsurgical changes may also be more susceptible to bacterial invasion. Staphylococcus aureusandStaphylococcus epidermidisare common causes of meningitis in patients following neurologic proceduressuch as placement ofventriculoperitoneal shunts. The brisk host inflammatory response in the subarachnoid space may cause edema, vasculitis, and coagulation of vessels, leading to severe neurologic complications including seizures, increased intracranial pressure, and stroke. Acute bacterial meningitis can progress over hours to days. Typical symptoms include fever, neck stiffness, and headache.Patients may also complain of photophobia, nausea and vomiting, and more nonspecific constitutional symptoms. Approximately 75% of patients will experience some confusion or altered level of consciousness. Forty percent may experience seizures during the course of their illness.
Some physical examination findings may be useful in the evaluation of a patient with suspected meningitis. Nuchal rigidityis demonstrated when passive or active flexion of the neck results in an inability to touch the chin to the chest. Classic tests include Kernig and Brudzinski signs. Kernig signcan be elicited with the patient on his or her back. The hip and knees are flexed. The knee is then passively extended, and the test is positive if this maneuver elicits pain. Brudzinski signis positive if the supine patient flexes the knees and hips when the neck is passively flexed. Neither sign is very sensitive for the presence of meningeal irritation, but, if present, both are highly specific. Papilledema, if present, would indicate increased intracranial pressure, and focal neurologic signs or altered level of consciousness or seizures may reflect ischemia of the cerebral vasculature or focal suppuration.
Differential Diagnosis
The differential diagnosis of bacterial meningitis is fairly limited and can be narrowed depending upon the patient’s age, as discussed earlier, exposure history, and course of illness. Various viral infections may also cause meningitis. These include enteroviruses, which tend to be more common in the summer and fall, when patients may present with severe headache, accompanied by symptoms of gastroenteritis. The CSF white blood cell (WBC) count will be elevated, with a predominance of lymphocytes, and usually glucose and protein levels are normal (Table 29–1). Either herpes simplex virus (HSV)-1 or HSV-2 can cause herpes simplex meningitis. The CSF of these patients will also have a normal glucose level, whereas protein and WBC counts will be elevated with a predominance of lymphocytes. Typically, these patients have a high CSF red blood cell count, which is not seen in bacterial meningitis in the absence of a traumatic spinal tap. In a patient with human immunodeficiency virus (HIV) infection, fungal meningitis, specifically caused by Cryptococcus, should be considered. Tuberculous meningitis presents subacutely and is more common in older, debilitated patients, or in patients with HIV. Rickettsial disease, specifically Rocky Mountain spotted fever, may also present with meningitis. Intracranial empyema, or brain or epidural abscess, should be considered, especially if the patient has focal neurologic findings. The one nonsuppurative diagnosis in the differential is subarachnoid hemorrhage. These patients present with sudden onset of the “worst headache of their lives” in the absence of other symptoms of infection. They may have photophobia, and the CSF will be grossly bloody; the supernatant will be xanthochromic, reflecting the breakdown of blood into bilirubin.
The necessity of imaging of the head and brain prior to performing an LP is controversial. Studies show that in the patient with suspected meningitis who does not have papilledema, focal neurologic signs, or altered level of consciousness, an LP may be safely performed without preceding imaging. However, in instances in which performance of the LP may be delayed, antibiotics should be administered after blood cultures while awaiting the radiologic studies. Ideally, the CSF should be examined within 30 minutes of antibiotics, but it has been shown that if the LP is performed within 2 hours of antibiotic administration, it will not significantly alter the CSF protein, glucose, or WBC count, or Gram stain. If CSF is obtained, a culture and Gram stain should be sent. If enough fluid is available, it should also be sent for cell count and glucose and protein levels. Latex agglutination tests for S pneumoniae andH influenzaecan be useful in patients pretreated with antibiotics, and, although not very sensitive, if positive they can rule in disease (high specificity). Polymerase chain reaction (PCR) testing is available for some bacteria; however, it may be more useful in the diagnosis of herpes simplex, enteroviral, or tuberculous meningitis. In all, no more than 3.5 to 4 mL of CSF is necessary. The most critical issue in a patient with suspected bacterial meningitis, however, is the initiation of antibiotics. The CSF examination and imaging studies can be deferred in this medical emergency.
During the course of treatment, most patients will undergo some cerebral imaging study. Computed tomographic (CT) scans are most useful in the initial presentation to exclude intracranial mass or bleeding, or to evaluate for other signs of increased intracranial pressure. However, magnetic resonance imaging (MRI) is most helpful for demonstrating any focal ischemia or infarction caused by the disease. When HSV meningitisis suspected, MRI should demonstrateenhancement of the temporal lobes. In tuberculous meningitis, enhancement of the basal region may be seen. An electroencephalogram (EEG) may be helpful in patients suspected of HSV meningitis. Within 2 to 15 days of the start of the illness, periodic sharp and slow wave complexes originating within the temporal lobes can be demonstrated at 2- to 3-second intervals. When the purpuric skin lesions are present, skin biopsy may demonstrateN meningitidisand can be helpful in the diagnosis. Age may give a clue regarding etiology of meningitis.
Therapy
Treatment of meningitis often is empiric until specific culture data are available. Because of the growing incidence of resistant pneumococci as well as meningococci, the recommended empiric therapy in most areas is a high-dose third-generation cephalosporin given concurrently with vancomycin. In other areas, if the disease presentation is typical for meningococcus (with the typical rash) or the organism is identified quickly on Gram stain of the CSF, therapy with high-dose penicillin can be started if the meningococcus in that area is known to be sensitive. Ampicillin is added when there is a suspicion of listeriosis. Acyclovir should be started for suspicion of HSVor four-drug antituberculosis (TB) therapy started if the presentation is suspicious for tuberculous meningitis. The administration of glucocorticoidsto reduce CNS (central nervous system) inflammation is controversial. One study in adults demonstrated decreased mortality in patients with S pneumoniaemeningitis who were given glucocorticoids. There is stronger data for steroids for H influenzae andS pneumoniaemeningitis in children. There is also some evidence for benefit of steroids in severe tuberculous meningitis.
Prevention of meningitis can be achieved through the administration of vaccines and chemoprophylaxisof close contacts. Specific vaccinations are available forH influenzaetype B and some strains ofS pneumoniaeand are now routinely administered to children.Meningococcal vaccinationis recommended for those living in dormitory situations, such as college students and military recruits, but not for the general population. Rifampin given twice daily for 2 daysor a single dose of ciprofloxacin is recommended for household and close contactsof an index case of meningococcemia or meningococcal meningitis.
Case A 25-year-old African American man is admitted to your service with the diagnosis of a sickle cell pain episode. He was admitted to the hospital six times last year with the same diagnosis, and he was last discharged 2 months ago. Again he presented to the emergency room complaining of abdominal and bilateral lower extremity pain, his usual sites of pain. When you examine him, you note he is febrile to 101°F, respiratory rate 25 breaths per minute, normal blood pressure, and slight tachycardia of 100 bpm. Lung examination reveals bronchial breath sounds and egophony in the right lung base. His oxygen saturation on 2 L/min nasal cannula is 92%. Besides the usual abdominal and leg pain, he is now complaining of chest pain, which is worse on inspiration. Although he is tender on palpation of his extremities, the remainder of his examination is normal. His laboratory examinations reveal elevated white blood cell and reticulocyte counts, and a hemoglobin and hematocrit that are slightly lower than baseline. Sickle and target cells are seen on the peripheral smear
What is the most likely diagnosis?
What is your next step?
What are the potential complications of this condition
ANSWERS
Summary: A 25-year-old African American man with a history of numerous pain crises is admitted for abdominal and bilateral lower extremity pain. He is febrile to 101°F, respiratory rate 25 breaths per minute, and slight tachycardia of 100 bpm. Lung examination reveals bronchial breath sounds and egophony in the right lung base. His oxygen saturation on 2 L/min nasal cannula is 92%. He is now complaining of chest pain, which is worse on inspiration. He has a leukocytosis, an elevated reticulocyte count, and a hemoglobin and hematocrit that are slightly lower than baseline. Sickle and target cells are seen on the peripheral smear.
Most likely diagnosis:Acute chest syndrome.
Next step:Chest radiograph and empiric antibiotic therapy.
Potential complications:Respiratory failure, possible death
ANALYSIS
DEFINITIONS
SICKLE CELL ANEMIA:A congenital defect in hemoglobin formation such that both genes code for hemoglobin S, leading to hemolysis and an abnormal shape of the red blood cell. Affected individuals have numerous complications including pain crises.
ACUTE CHEST SYNDROME:A condition found in individuals with sickle cell disease characterized by fever, tachycardia, chest pain, leukocytosis, and pulmonary infiltrates
CLINICAL APPROACH
Pathophysiology
The molecular structure of a normal hemoglobin molecule consists of two alpha-globin chains and two beta-globin chains. Sickle cell anemia is an autosomal recessive disorder resulting from a substitution of valine for glutamine in the sixth amino acid position of the beta-globin chain. This substitution results in an alteration of the quaternary structure of the hemoglobin molecule. Individuals in whom only half of their beta chains are affected are heterozygous, a state referred to as sickle cell trait.When both beta chains are affected, the patient is homozygous and has sickle cell anemia. In patients with sickle cell disease, the altered quaternary structure of the hemoglobin molecule causes polymerization of the molecules under conditions of deoxygenation. These rigid polymers distort the red blood cell into a sickle shape, which is characteristic of the disease. Sicklingis promoted by hypoxia, acidosis, dehydration, or variations in body temperature.
Complications of Sickle Cell Disease
Acute painful episodes, also known as pain crisis,are a consequence of microvascular occlusion of bones by sickled cells. The most common sites are the long bones of the arms, legs, vertebral column, and sternum. Acute painful episodes are precipitated by infection, cold exposure, dehydration, venous stasis, or acidosis. They usually last 2 to 7 days.
Infectionsare another complication. Patients with sickle cell disease are at greater risk for infections, especially with encapsulated bacterial organisms. Autoinfarction of the spleenoccurs during early childhood secondary to microvascular obstruction by sickled red blood cells. The spleen gradually regresses in size and by age 4 years is no longer palpable. As a consequence of infarction and fibrosis, the immunologic capacity of the spleen is diminished. Patients with sickle cell disease are at greater risk for pneumonia, sepsis, and meningitis by encapsulated organisms such as Streptococcus pneumoniaand Haemophilus influenza.For the same reason, patients with sickle cell disease are at greater risk for osteomyelitis with Salmonellaspp. Acute chest syndromeis a vasoocclusive crisis within the lungs and is associated with infection or pulmonary infarction. Patients with acute chest syndrome present with hypoxia, dyspnea, fever, chest pain, and progressive pulmonary infiltrateson radiography. These episodes may be precipitated by pneumonia causing sickling in the infected lung segments, or, in the absence of infection, intrapulmonary sickling can occur as a primary event. It is virtually impossible to clinically distinguish whether or not infection is present; thus, empiric antibiotic therapy is used.
Aplastic crisisoccurs secondary to viral suppression of red blood cell precursors, most often by parvovirus B19. It occurs because of the very short half-life of sickled red blood cells and consequent need for brisk erythropoiesis. If red blood cell production is inhibited, even for a short time, profound anemia may result. The process is acute and usually reversible, with spontaneous recovery
Other complications of sickle cell disease include hemorrhagic or ischemic stroke as a result of thrombosis, pigmented gallstones, papillary necrosis of the kidney, priapism, and congestive heart failure.
Treatment
The mainstay of treatment of pain crisis is hydration and pain control with nonsteroidal anti-inflammatory agents and narcotics. It is important to also provide adequate oxygenation to reduce sickling. One must search diligently for any underlying infection, and antibiotics are often used empirically when infection is suspected. Acute chest syndromeis treated with oxygen, analgesia, and antibiotics. Sometimes exchange transfusions are necessary. In general, blood transfusions may be required for aplastic crisis, for severe hypoxia in acute chest syndrome, or to decrease viscosity and cerebral thrombosis in patients with stroke. Transfusion does not shorten the duration of pain crisis. To protect against encapsulated organisms, all patients with sickle cell disease should receive penicillin prophylaxisand a vaccination against pneumococcus. Hydroxyureais often used to reduce the occurrence of painful crisis by stimulating hemoglobin F production and thus decreasing hemoglobin S concentration, and should be considered in patients who have repeated episodes of acute chest syndrome, or frequent severe pain crises. The antineoplastic agent 5-deoxyazacytidine (decitabine) may also elevate levels of hemoglobin F without excessive side effects.
QUESTIONS Q 1. Which of the following therapies would most likely decrease the number of sickle cell crises?
A.Hydroxyurea
B.Folate supplementation
C.Prophylactic penicillin
D.Pneumococcal vaccination
ANSWER. A
Q. the syndrome to which it is commonly associated in persons with sickle cell anemia
Salmonella species = osteomylitis
Parvovirus B19 = Aplastic crisis
Q 1. You are evaluating a patient with a wide-complex tachycardia. The patient has a history of Wolff-Parkinson-White (WPW) syndrome. Which medication is the most effective for treating this patient’s tachycardia?
A.Adenosine
B. Digoxin
C.Diltiazem
D.Procainamide
E. Verapamil
ANSWER. D
Q 2. All of the following are electrocardiographic clues supporting the diagnosis of ventricular tachycardia except
A.capture beats
B. concordance of QRS complex in all precordial leads
C.fusion beats
D.QRS duration during tachycardia shorter than during sinus rhythm
E. RSR' pattern in V1
ANSWER. E
Q 3. A 68-year-old man with a history of myocardial infarction and congestive heart failure is comfortable at rest. However, when walking to his car, he develops dyspnea, fatigue, and sometimes palpitations. He must rest for several minutes before these symptoms resolve. His New York Heart Association classification is which of the following?
A.Class I
B. Class II
C.Class III
D.Class IV
ANSWER. C
Q 4. The husband of a 68-year-old woman with congestive heart failure is concerned because his wife appears to stop breathing for periods of time when she sleeps. He has noticed that she stops breathing for ~10 s and then follows this with a similar period of hyperventilation. This does not wake her from sleep. She does not snore. She feels well rested in the morning but is very dyspneic with even mild activity. What is your next step in management?
A.Electroencephalography
B. Maximize heart failure management
C.Nasal continuous positive airway pressure (CPAP) during sleep
D.Obtain a sleep study
E. Prescribe bronchodilators
ANSWER. B
Q 5. You are caring for a patient with heart rate-related angina. With minor elevations in heart rate, the patient has anginal symptoms that impact his quality of life. On review of a 24-h Holter monitor, it appears that the patient has sinus tachycardia at the time of his symptoms. What is the mechanism for this patient’s arrhythmia?
A.Delayed afterdepolarizations
B. Early afterdepolarizations
C.Increased automaticity
D.Reentry pathway
ANSWER. C
Q 6. Where are the most common drivers of atrial fibrillation anatomically located?
A.Left atrial appendage
B. Mitral annulus
C.Pulmonary vein orifice
D.Sinus venosus
E. Sinus node
ANSWER. C
Q 7. Symptoms of atrial fibrillation vary dramatically from patient to patient. A patient with which of the following clinical conditions will likely be the most symptomatic (e.g., short of breath) if they develop atrial fibrillation?
A.Acute alcohol intoxication
B. Hypertrophic cardiomyopathy
C.Hyperthyroidism
D.Hypothermia
E. Postoperative after thoracotomy
ANSWER. B
Q 8. When deciding whether to initiate anticoagulation for a patient with atrial fibrillation, which of the following factors is least important?
A.Age
B. History of diabetes
C.Mitral stenosis
D.Use of antiarrhythmic medications
E. Hypertension
ANSWER. D
Q 9. Which of the following electrocardiographic findings suggests a focal atrial tachycardia as opposed to an automatic atrial tachycardia (e.g., sinus tachycardia)?
A. Initiation of tachycardia with programmed stimulation
B. One P-wave morphology
C.Slow-onset and termination phase
D.Slowing of the rate with adenosine infusion
ANSWER. A
Q 10. You are seeing a return patient in clinic. The patient is a 76-year-old man with a history of hypertension, remote cerebrovascular accident, diet-controlled diabetes, and congestive heart failure with left ventricular systolic dysfunction (ejection fraction = 30%). The patient reports no new complaints and feels well. On physical examination, you palpate an irregular pulse, and an electrocardiogram verifies atrial fibrillation. The patient does not have a history of atrial fibrillation. You and the patient are interested in a trial of direct current cardioversion (DCCV). What is the appropriate management of anticoagulation for this patient?
A.Initiate warfarin (with goal INR 2.0–3.0) following DCCV only if cardioversion is unsuccessful.
B. Give full-dose aspirin (325 mg daily) 3 weeks prior to DCCV, perform transesophageal echocardiogram (TEE) and DCCV (if not contraindicated), then discontinue aspirin if DCCV is successful.
C.Initiate IV heparin and warfarin, perform transesophageal echocardiogram (TEE) and DCCV (if not contraindicated), then discontinue warfarin if DCCV is successful.
D.Initiate IV heparin, perform TEE and DCCV (if not contraindicated), then continue warfarin for at least 1 month.
ANSWER. D
Double bleb sign : Appearance of amnion and yolk sac at 5-6 weeks. Embryo lies between amnion and yolk sac
Double bubble sign : Sign of duodenal atresia and other forms of duodenal obstruction.
Double decidua sign : Sign of early normal intrauterine gestation.
Double density sign (cardiac) : Sign of left atrial enlargement when right side of the left atrium pushes into the adjacent lung.
Double density sign (Nucs) : Pattern of uptake that may be seen on bone scans in patients with osteoid osteomas.
Double duct sign : Simultaneous dilatation of the common bile and pancreatic ducts that is generally caused by a tumor in the pancreatic head.
Double PCL sign : MRI sign of a bucket-handle meniscal tear.
Double track sign : :Appearance of hypertrophic pyloric stenosis that may be seen on upper GI series.
Q 1. Which of the following conditions is not associated with sinus bradycardia?
A.Brucellosis
B. Leptospirosis
C.Hypothyroidism
D.Advanced liver disease
E. Typhoid fever
ANSWER. B
Q 2. All of the following are common consequences of congenital heart disease in the adult except
A.Eisenmenger syndrome
B. erythrocytosis
C.infective endocarditis
D.pulmonary hypertension
E. stroke
ANSWER. E
Q 3. Acute hyperkalemia is associated with which of the following electrocardiographic changes?
A.QRS widening
B. Prolongation of the ST segment
C.A decrease in the PR interval
D.Prominent U waves
E. T-wave flattening
ANSWER. A
Q 4. All of the following clinical findings are consistent with severe mitral stenosis except
A.atrial fibrillation
B. opening snap late after S2
C.pulmonary vascular congestion
D.pulsatile liver
E. right-ventricular heave
ANSWER. B
Q 5. A 37-year-old male with Wolff-Parkinson-White syndrome develops a broad-complex irregular tachycardia at a rate of 200 beats per minute. He appears comfortable and has little hemodynamic impairment. Useful treatment at this point might include
A.Digoxin
B. Amiodarone
C.Propranolol
D.Verapamil
E. Direct-current cardioversion
ANSWER. E
Q 6. In African Americans with New York Heart Association class II heart failure, which of the following drug combinations should be added to an angiotensinconverting enzyme inhibitor and beta blocker?
A.Hydralazine/angiotensin receptor blockers
B. Hydrazaline/digoxin
C.Isosorbide dinitrate/angiotensin receptor blockers
D.Isosorbide dinitrate/digoxin
E. Isosorbide dinitrate/hydralazine
ANSWER. E
Q 7. All of the following medications have been shown to worsen heart failure in patients with left ventricular systolic dysfunction except
A.angiotensin receptor blockers
B. calcium channel antagonists
C.nonsteroidal anti-inflammatory drugs (NSAIDs)
D.sotalol
E. thiazolidinediones
ANSWER. A
Q 8. Which of the following is true regarding dose escalation of angiotensin-converting enzyme (ACE) inhibitors and beta blockers in patients newly diagnosed with congestive heart failure?
A.ACE inhibitors should be escalated on a daily basis to maximal tolerated doses, while beta blockers should be gently increased in dose over weeks, as tolerated.
B. Beta blockers should be escalated on a daily basis to maximal tolerated doses, while ACE inhibitors should be gently increased in dose over weeks, as tolerated.
C.Both should be escalated rapidly to maximally tolerated doses.
D.Both should be escalated slowly to maximally tolerated doses.
E. Both should be initiated at full doses
ANSWER. A
Q 9. All of the following findings on echocardiographic assessment of patients with congestive heart failure with preserved ejection fraction are relevant except
A.atrial fibrillation
B. left atrial dilatation
C.left ventricular wall thickness
D.left ventricular diastolic filling as measured by tissue Doppler
E. systolic anterior motion of the mitral valve
ANSWER. E
Q 10. A 28-year-old man with long-standing cardiomyopathy presents with worsening dyspnea. Physical examination reveals a blood pressure of 85/50 mmHg, heat rate of 112 beats/min, elevated jugular venous pressure, positive hepatojugular reflex, quiet S1/S2, apical S3, no pulmonary rales, and 3+ lower extremity edema. Chest radiograph shows no pulmonary edema and a small left-sided pleural effusion. What information does the patient’s pulmonary examination give you in regards to his likely pulmonary capillary wedge pressure?
A.It is likely to be elevated.
B. It is likely to be normal.
C.It is likely to be decreased.
D.No information
ANSWER. D
