Mix2 Flashcards
Hearing impairment signs in children
Inattentiveness Poor language development Poor social skills development Difficulty following directions Refusal to listen Low self-esteem Social isolation
Inspiratory stridor, worse when supine
Laryngomalacia
Surgical debridement in Tx of malignant otitis externa
If fails to response to antibiotics
The most common middle ear pathology in patients with HIV
Serous otitis media
The most specific sign of eardrum inflammation
Bulging
Indication of observation in children with AOM
If >2y
Mild, unilateral symptoms
Normal immune system
Peritonsillar abscess Tx
Drainage + IV AB (GAS, respiratory anaerobes)
Clues to the diagnosis of peritonsillar abscess (quinsy)
Deviated uvula Muffled (hot potato) sound Unilateral cervical LAP Trismus Pooling of saliva Asymmetrically enlarged tonsills
Sialadenosis
Nontender enlargement of submandibular glands In pts with: Advanced liver disease (cirrhosis) Altered dietary pattern Malnutrition (DM, bulimia)
TMJ dysfunction
Nocturnal teeth grinding pain feels like coming from ear Pain worsened with chewing Audible click/crepitus with jaw movement Tx: coservative: nighttime bite gaurd Sometimes surgery necessary
Blunt abd trauma
Unstable pt
next step?
FAST
Blunt abd trauma
Unstable pt
Positive FAST
Next step?
Laparotomy
Blunt abd trauma
Unstable pt
Negative FAST
Next step?
Signs of extra-abdominal hemorrhage( pelvic/long-bone fx)?
Yes: stabilize, angio, splint
No: stabilize then CT of abd
Blunt abd trauma
Unstable pt
Inconclusive FAST
Next step?
DPL
If positive: Laparotomy
If negative: proceed as with negative FAST
Bronchial rupture features
Persistent pneumothorax despite chest tube
Pneumomediastinum
Subcutaneous emphysema
Mostly the right main bronchus is injured
Confirmation with: CT, bronchiscopy, surgical exploration
Tx: operative repair
The most important initial diagnostic study in all stabilized patients following blunt chest trauma
CXR
Esophageal rupture features
Mostly iatrogenic or following esophagitis
Pneumomediastinum
Pleural effusion
Disappearance of Babinski reflex in healthy child
Before 2 y of age
Disappearance of monro, grasp, tongue protrusion
By age 4 mo
Weight and height at 12 months of eight
Weight should triple and height should increase by 50%
Age of beginning and completing toilet training
2y
5y
Age of performing evaluations and interventions for children with urinary incontinence
5 y/o and higher
Contraindications to I/O access
Infection overlying the access site
Bone fracture
Previous I/O attempts in the chosen extremity
Bone fragility
Stemmer sign
Inability to lift the skin on the dorsum of the second toe
Highly specific for lymphedema
How much weight loss is normal after birth for Neonate?
<7% (during first 5 d)
Pink stains or brick dust in neonate’s diaper
Uric acid crystals
Common during the first week, or in later months with the morning void after infant begins to sleep through the night
When can infants have plain water?
After 6mo of age
Indications of laparotomy in penetrating abdominal injury
Hemodynamic instability
Peritonitis (rebound tenderness, guarding)
Evisceration
Blood from NGT or rectal exam
If no indications of laparotomy in penetrating abdominal injury
Local wound exploration
Extended ultrasound examination
Indication of DPL
Unstable patients with blunt abdominal trauma and no available CT or FAST or inconclusive GAST
Diaphragm location range
4th thoracic dermatome on the right and 5th on the left
Down to the 12th thoracic dermatome on both sides
Any penetrating injury at the thorax below the level of the nipples has potential to also involve the abdomen through the diaphragm, And is assumed to involve .both compartments until proven otherwise
Development of object permanence in an infant
6-12mo
Age of separation anxiety
9-18 mo
Contraindications to Nasotracheal intubation
Apneic/hypopneic pts
Basilar skull fracture
Diagnosis of coma is via:
Impaired brainstem activity
Motor dysfunction
Level of consciousness
ALL markers
Peroxidase -
PAS +
TdT + (preB, preT)
Anemia of chronic disease
Iron trapping within macrophages Low serum iron Low TIBC Low iron/TIBC Low retic count relative to anemia Nl/high ferritin Tx: treatment of underlying disorder If unresponsive, EPO If all failed transfusion
Tx of warfarin necrosis
Cessation of warfarin
Administration of protein C concentrate
How long in advance should warfarin be discontinued before measurement of protein S levels?
2 wk
Fanconi anemia
The most common congenital cause of aplastic anemia. Macrocytic
AR or X-linked
Pancytopenia presents between 4-12 years of age.
Initial manifestation of CBC: thrombocytopenia, then neutropenia, then anemia
Hyperpigmentations
Cafe au lait
Skeletal anomalies, microcephaly
Upper limb anomalies, absent thumbs
Short stature
Eye/eyelid changes, middle ear abnormalities
Hypogonadism
Diamond-Blackfan anemia
Presents within 3 mo of birth Pure red cell anemia Congenital anomalies: webbed neck, cleft lip, shielded chest, triphalangeal thumb Macrocytic anemia Elevated fetal Hb Nl chromosomes Tx: CS, transfusion
Defect in Fanconi anemia
DNA repair
Chromosomal breaks on genetic analysis
Tx: BMT
Infection prophylaxis for splenectomy
Vaccines against pneumococcus, meningococcus and hemophilis influenza B (several weeks before the procedure)
+
Daily oral penicillin for 3-5 years or until adulthood for children or AB available at home to begin with any significant fever
(After the procedure)
Howell-Jolly bodies
Single round blue RBC inclusion on Wright stain
Remnant of nucleus
Indicate physical/functional absence of spleen:
Splenectomy, autoimfarction, infiltration, congestion
Intravascular hemolysis lab
Markedly reduced serum haptoglobin (undetectable)
Elevated indirect Bil
Raised LDH levels
Extravascular hemolysis lab
Less intravascular Hb release
Nl/slightly low haptoglobin
Slightly elevated LDH
Elevated indirect Bil
Intravascular hemolysis DDx
MAHA (DIC...) Transfusion reaction Infection (clostridial sepsis...) PNH IV Rho(D) Ig infusion
Extravascular hemolysis DDx
RBC enzyme deficiencies Hemoglobinopathy Membrane defects Hypersplenism IVIg Warm/cold-agglutinin AI hemolytic anemia Bartonella Malaria
Indications of washed RBC
IgA deficiency
Complement-dependent AI hemolytic anemia
Allergic reaction with RBC despite antihistamine treatment
Indications for leukoreduced RBC
Chronically transfused pts
CMV seronegative at-risk pts (AIDS, transplant)
Potential transplant recipients
Previous febrile non-hemolytic transfusion reaction
Indications for irradiated RBC transfusion
BMT recipients
Acquired/congenital cellular immunodeficiency
Blood components donated by first or second degree relatives
Timing of transfusion reactions
Anaphylaxis: seconds to minutes Acute hemolytic: minutes to 1 h Febrile non-hemolytic: 1-6 h Transfusion-related acute lung injury: 1-6 h Urticaria/allergic: 2-3 h Delayed hemolytic: 2-10 d
Rx of febrile non-hemolytic transfusion reaction
Stop transfusion
Antipyretics
Use leukocyte depleted RBC
Tx of hypercalcemia due to malignancy
Bisphosphonate
Tx of hypercalcemia related to granulomatous diseases
CS
The most common sites of osteonecrosis in SCA patients
Humoral and femoral heads
Soap bubble appearance on bone xray
Giant cell tumor
Giant cell tumor of bone
Expansile eccentric lytic area Soap bubble appearance Benign Locally aggressive Young adults Pain, swelling, decreased range of motion at the involved site Pathologic Fx Epiphysial region of long bones Distal femur, proximal tibia MRI: Cystic and hemorrhagic regions Tx: surgical curettage +/- bone grafting
Osteitis fibrosa cystica
Due to hyperpara from parathyroid carcinoma
Brown tumor (fibrous tissue) of long bones
Osteolytic resorption of bones
Bone pain
Salt-and-pepper skull
Subperiosteal bone resorption on radial aspect of middle phalanges
Distal clavicle tapering
Osteosarcoma lab
Increased: ESR, ALP, LDH
No systemic symptoms
Soft tissue mass on examination
Parinaud (dorsal midbrain) syndrome
Limited upward gaze
Upper eyelid retraction (collier sign)
Pupils non-reactive to light but reactive to accomodation (light-near dissociation)
Features of pineal gland tumor
Pressure on pretectal region of the midbrain
Parinaud syndrome
Obstructive hydrocephalus
Neuroblastoma origin
Sympathetic nervous system
Intracranial neuroblastoma features
Opsoclonus myoclonus syndrome
Ab-mediated dancing eyes and feet
Trilateral neuroblastoma
Bilateral neuroblastoma (leukocoria) And pineal gland tumor
Symptoms of cerebellar vermis dysfunction
Truncal/gait instability
Symptoms if cerebellar hemispheres dysfunction
Dysmetria
Intention tremor
Dysdiadochokinesia
Tx of cancer-related anorexia/cachexia
Progesterone analogues (megestrol acetate) CS
If longer life expectancy, progesterone analogues preferred
Tx of advanced HIV cachexia
Synthetic cannabinoids (dronabinol)
Tx of chemotherapy-induced nausea
Serotonin 5HT3 receptor antagonist:
Ondansetron
+/- CS
Both for treatment and prophylaxis
Tx of nausea secondary to gastroparesis
Motilin receptor agonists: erythromycin
D2 antagonist. Dompridone
Tx if opioid induced constipation
Methylnaltrexone (GI-specific opioid antagonist)
A form of treatment for a disease when a standard treatment fails
Salvage therapy
Treatment given in addition to standard therapy
Adjuvant therapy
An initial dose of treatment to rapidly kill tumor cells and send a patient into remission (<5% tumor burden)
Induction therapy
A treatment given after induction therapy with multi drug regiment to further reduce tumor burden
Consolidation
A treatment given after induction and consolidation therapies to kill any residual tumor cells and keep the patient in remission
Maintenance
Treatment given before the standard therapy for a particular disease
Neoadjuvant
Indication of EPO in CRF (after ruling out iron deficiency)
Hct<30%
Pts on hemodialysis with symptoms attributed to anemia
Target: Hct:30-35%
Side-effects of EPO
HTN (IV>SC), the most common side effect.
Headache
Flu-like (IV>SC)
Red cell aplasia
Anemia in CRF vs IDA
CRF: normo, normo, hypoproliferative
IDA: micro, hypo
Must evaluate pts with CRF for iron deficiency before starting EPO
If iron deficiency concurrent with CRF anemia, therapeutic choice is: IV iron preparation
Worse prognosis of CLL with
Anemia
Thrombocytopenia
Multiple chain LAP
HSM
Classic presentation of hairy cell leukemia
Splenomegaly Pancytopenia B cell Age>50 Bone marrow fibrosis Typically no LAP, Hepatomegaly orB symptoms
Hodgkin usual presentation
Painless LAP and B symptoms
Nl CBC, PBS
CLL usual presentation
Dramatic lymphocytosis
LAP
HSM
thrombocytopenia, anemia
Dx of CLL
CBC, PBS, FCM (clonality of mature Bcells)
Elderly+ dramatic lymphocytosis+ mild anemia/thrombocytopenia + LAP + HSM = CLL
Bx usually not required (LN, BM…)
Leukocyte alkaline phosphatase score in CML vs leukemoid reaction
Low LAP in CML
Dx of CML
Dramatic granulocytosis Absolute basophilia Immature neutrophil precursors low ALP score (alkaline phosphatase) BCR-ABL Thrombocytosis Anemia Splenomegaly
Intracranial tumor with calcification
Craniopharyngioma
The earliest vaso-occlusion manifestation in SCA
Hand-foot syndrome or dactylitis
6mo-2y
Acute onset of pain and symmetrical swelling of hands and feet
+/- fever
vascular necrosis of metacarpals and metatarsals
There may be osteolytic lesions lesions
Best time for retesting G6PD after an episode of hemolysis
3 mo later
Pyruvate kinase deficiency manifestations
Chronic hemolysis
Pigmented gallstones
HSM
Skin ulcers
Pathophysiology of wiskott-Aldrich syndrome
Impaired cytoskeleton in plt and WBC
Hemochromatosis increases susceptibility to which infectious organisms?
Vibrio vulnificus
Listeria
Yersinia enterocolitica
Tx of HUS
Supportive
Dialysis
Blood transfusion
Fluid/electrolyte
HUS vs DIC
PT and PTT normal in HUS
How long after diarrhea does HUS develop ?
5-10 d
Hematuria in hemophilia
Common but no renal impairment.
Normal Cr
Hemophilic arthropathy
Hemosiderin deposition and arthritis and fibrosis
Chronic worsening pain and swelling
Contracture
Limited ROM
Early detection with MRI
Advanced disease visible on Xray
Early preventive treatment can significantly reduce the risk
Type 2 HIT
Plt reduction > 50% of baseline
Low risk of bleeding
But 50% risk if new arterial/venous thrombosis if untreated
Tx: D/C all heparin products. Start non-heparin medication (argatroban, fondaparinux)
Plt transfusion not required unless over bleeding
Normalization of plt count: 2-7 d after D/C
Dx: serotonin release assay (gold std), high-titer immunoassay
Warfarin in HIT
Started after another anticoagulant has been received and plt>150,000
PTT in HIT
Prolonged due to heparin!!
Type 1 vs Type 2 HIT
Type1: non immune mediated plt aggregation. Within 2d of heparin exposure. Plt rarely <100,000. No intervention required. No ill effect. Resolves w/o heparin cessation.
Type2: immunologic. More serious. Anti-PF4-Ab. Plt count usually 30-60,000. 5-10 d after heparin exposure (unless re-exposure). Limb ischemia, stroke.
Effect of enoxaparin on aPTT
None
Osler-Weber-Rendu
HHT
AVMs in: skin, mucous membranes, GI, Lung, liver
In lung, AVMs cause: chronic hypoxia, clubbing, polycytemia. Also massive hemoptysis
Hereditary spherocytosis
AD
Defect in ankyrin gene (spectrin scaffolding pr)
Increased MCHC (membrane loss, RBC dehydration)
Low MCV
Increased RDW
Splenomegaly
Increase osmotic fragility on acidified glycerol lysis test
Abn eosin-5-maleimide binding test
Tx: folic acid. Transfusion, splenectomy
Absent CD55
PNH
Increased HbF
Beta Thalassemia
SCA
Congenital aplastic anemia
Hereditary persistence of fetal Hb
Time of splenic autoinfarct in SCA
By 18-36 mo
Helmet cells
MAHA (HUS, DIC, TTP)
Effect of hydroxyurea in SCA
Decreases:
Pain crises
Acute chest syndrome
Need for transfusion
Risks of homocysteinuria
VTE
Atherosclerosis
Tx of homocysteinuria
B6
+ folate
+ B12 (if reduced levels)
Pica specific for iron deficiency
Pagophagia (ice)
The first finding of IDA on PBS
anisocytosis (increased RDW)
>20% in suggestive
Cow’s milk in infants
Not before 12 mo of age
Not mire than 24 oz daily
Target cells
Alpha/beta thalassemia
Hb electrophoresis in alpha and beta thalassemia
Alpha thalassemia: Nl
Beta thalassemia: increased HbA2
Tx of ITP in children
Only skin manifestations: observe
Bleeding: IVIg, CS
Tx of ITP in adults
Plt 30,000 or higher and no bleeding: observe
Plt<30,000 or bleeding, IVIg, CS
Tests required for ITP
CBC
PBS
HIV/HCV (if Hx suggestive)
Manifestations of acute lead poisoning
Abdominal pain
Constipation
Manifestations of chronic lead exposure
Fatigue Irritability Insomnia HTN motor/sensory neuropathies (paresis...) Neuropsychiatric disturbance Nephropathy (raised Cr)
Lead absorption route in children and adults
Children: GI
Adults: respiratory
Dx of lead toxicity
CBC, PBS Microcytic anemia Basophilic stippling Blood lead levels Xray fluorescence (measure bone lead concentration)
Occupations with lead exposure
Battery manufacturing Plumbing Mining painting paper hanging auto repair
Location of lead in the body
99% Bound to erythrocytes
Serum protein gap
Protein - albumin > 4
Indicates:
Poly/monoclonal gammopathy
Rouleaux formation indicates
Elevated serum protein
Eculizumab
PNH Tx
Inhibits complement activation
PNH Tx
Eculizumab
Folate
Iron
Age of presentation in PNH
4th decade of life
Abdominal pain + dark urine DDx
Hemolysis PNH Cholangitis Acute intermittent porphyria SCA
Prophylaxis in children with asplenia
Conjugated pneumococcal vaccine
Twice daily penicillin up to 5 y of age
Other routine vaccines
How long after reduced dietary intake does clinical folate deficiency appear?
4-5 mo
MDS vs Pernicious anemia
Both: pancytopenia and macrocytosis
Distinction requires careful examination of BMB and PBS for dysplastic cells
Transient visual disturbance DDx
TIA
MS
PV
CRVO
PV associations
Gout HTN erythromelalgia Aquagenic pruritus Thromboses Bleeding
Indication of hydroxyurea in PV
High risk of thrombosis
Reason for transient visual disturbance in PV
Hyper-viscosity.
It is not due to thrombosis, and anticoagulation is not effective
O2 saturation in PV
Nl
ESR in PV
Low
CO poisoning Dx
Carboxyhemoglobin level
ECG in all pts
Cardiac enzymes in: elderly, cardiac RF, signs of ischemia
Pulse oximetry does not detect CO-Hb. Measurement by arterial blood gas cooximetry
Tx: 100% O2 with non-rebreathing face mask
CO-Hb level in normal vs smoker person
<3% vs 10%
Neonatal polycytemia
Hct>65%
Causes: intrauterine hypoxia, IUGR, twin-twin transfusion, delayed cord clumping
Presentations: hypoglycemia, hypocalcemia, respiratory distress, ruddy skin, cyanosis, apnea, irritability, jitteriness, abdominal distention
Tx: symptomatic: partial exchange transfusion.
If asymptomatic: only hydration by feeding or parenteral fluids
Anemia of prematurity etiology
Impaired EPO production
Short RBC life span
iatrogenic blood sampling
Anemia of prematurity symptoms
Usually asymptomatic Tachycardia Apnea Poor weight gain Low Hb Low retic Normo-normo
Tx of anemia of prematurity
Minimize blood draws
Iron supplementation
Transfusion
The most common hereditary thrombophilia in whites
1st: Factor V Leiden (resistance to prC)
2nd: prothrombin mutation (increased levels)
Indications of hydroxyurea in SCA
Frequent acute painful episodes
Acute chest syndrome
Severe symptomatic anemia
Indication of periodic blood transfusion in SCA
Acute stroke Acute chest syndrome Acute multiorgan failure Acute symptomatic anemia Aplastic crisis Stroke prevention
Hypostenuria
Kidney’s inability to concentrate urine
e.g. SCA (disease and trait) red cell sickling in vasa recta of the inner medulla
Sickle cell trait features
Asymptomatic
At risk for renal issues
The most common: painless microscopic/gross hematuria
Others: isosthenuria, UTI
Splenic infarction uncommon but can occur in high altitudes
Anemia resulting from isoniazide
Sideroblastic
Decreased B6 -> decreased heme synthesis -> sideroblastic anemia
Dimorphic RBC population ( both normo and hypo)
Increased serum iron
Decreased TIBC
Spinal cord compression pain
Severe local back pain (pain is the first symptom, often present for 1-2 mo prior to additional symptoms)
Worse in recumbent position/ at night
Early signs: symmetric lower-extremity weakness, hypoactive/absent DTR
Late signs: Bilateral babinski reflex, decreased rectal sphincter tone, paraparesis/paraplegia, increased DTR, sensory loss
Spinal cord compression Tx
Emergency MRI
IV CS (given without delay)
Radiation/neurosurgery consultations
SVC syndrome etiologies
Malignancies (lung, Hodgkin)
Fibrosing mediastinitis (Histoplasmosis, TB)
Thrombosis due to CV line
Lab in APLS
Plt: mild decrease
PT: might be prolonged
PTT: prolonged (not corrected with dilution with normal plasma)
Microcytosis in IDA vs Thalassemia
IDA becomes microcytic when Hb<10
Thalassemia becomes microcytic with Hb>10
Mentzer index (MCV/RBC):
<13 in thalassemia
>13 in IDA
Screening for thalassemia trait in prenatal consultations
CBC
If reduced Hb and MCV, additional testing