Exam 2, deck 3 Flashcards
most common age groups for VTE
neonates and teenagers
most common precipitating factor in VTE
Central venous access device (CVAD)
Anticoagulation in symptomatic and asymptomatic deep vein thrombosis or pulmonary embolism
- The American Society of Hematology (ASH) guideline panel recommends using anticoagulationin pediatric patients with symptomatic deep vein thrombosis (DVT) or pulmonary embolism (PE) (
- The ASH guideline panel suggests either using anticoagulation or no anticoagulation in pediatric patients with asymptomatic DVT or PE
Thrombolysis, thrombectomy, and inferior vena cava filters in VTE
- against using thrombolysis followed by anticoagulation; rather, anticoagulation alone should be used in pediatric patients with DVT, submassive PE
2.The ASH guideline panel suggests using thrombolysis followed by anticoagulation, rather than anticoagulation alone, in pediatric patients with PE with hemodynamic compromise
3.suggests against using thrombectomy followed by anticoagulation; rather, anticoagulation alone should be used in pediatric patients with symptomatic DVT or PE
- suggests against using inferior vena cava (IVC) filter; rather anticoagulation alone should be used in pediatric patients with symptomatic DVT or PE
Antithrombin replacement therapy in VTE
- The ASH guideline panel suggests against using antithrombin (AT)-replacement therapy in addition to standard anticoagulation; rather, standard anticoagulation alone should be used in pediatric patients with DVT/cerebral sino venous thrombosis (CSVT)/PE
- ASH guideline panel suggests using AT replacement therapy in addition to standard anticoagulation rather than standard anticoagulation alone in pediatric patients with DVT/CSVT/PE who have failed to respond clinically to standard anticoagulation treatment and in whom subsequent measurement of AT concentrations reveals low AT levels based on age-appropriate reference ranges
CVAD-related thrombosis
- suggests no removal, rather than removal, of a functioning CVAD in pediatric patients with symptomatic CVAD-related thrombosis who continue to require venous access
2, removal, rather than no removal, of a nonfunctioning or unneeded CVAD in pediatric patients with symptomatic CVAD-related thrombosis
- suggests delayed removal of a CVAD until after initiation of anticoagulation (days), rather than immediate removal in pediatric patients with symptomatic central venous line–related thrombosis who no longer require venous access or in whom the CVAD is nonfunctioning
- ASH guideline panel suggests either removal or no removal of a functioning CVAD in pediatric patients who have symptomatic CVAD-related thrombosis with worsening signs or symptoms, despite anticoagulation and who continue to require venous access
Use of Low-molecular-weight heparin vs vitamin K antagonists in DTE
The ASH guideline panel suggests using either low-molecular-weight heparin or vitamin K antagonists in pediatric patients with symptomatic DVT or PE (conditional recommendation based on very low certainty in the evidence of effects
what happens in DIC
hemostasis is out of control
leading to coagulation all over the place
leads to organ ischemia
This uses up platelets and clotting factors
Other parts of body start to bleed even with slight damage of walls.
They have too much and too little clotting
Fibrin degradation products in circulation interferes with clot formation making hemostasis even more difficult
DIC is also called
consumption coagulopathy
Lab findings in DIC
decreased platelets
decreased fibrinogen
prolonged PT/PTT
elevated D Dimer (fibrin degradation product)
Treatment DIC
support organs with
-Ventilator
-Hemodynamic
-Transfusions
all if needed
fever/neutropenia def by megan harvey
A single temp of 38.3C (101) or
2 episodes of 38 (100.4) and above within a 24 hr period
or
Temp of 39C (100.4) persistent for one hour taken axillary orally or by tympanic probe
ANC formula by meg harvey
WBC x (%segs +%bands)
ANC <1500 neutropenia
ANC <1000 moderate neutropenia
ANC <500 severe neutropenia
ANC <100-200 profound neutropenia
When is ANC typically at lowest
7-14 days from start of round of chemo
common infection culprits in febrile neutropenia
Gram + Bacteria - coag neg staph. Staph aureus, strepto viridans and midas
Gram - Bacteria, enterobacter, pseudomonas
Anaerobic Bacteria - Clostr dificille, propionbacterum acnes
Fungus
Viruses- hsv varicella zoster, ebv, cmv, ect
others - pjp
what studies do you avoid in febrile neutropenia
lumbar puncture
tap shunt or reservoir
Abx in febrile neutropenia
Monotherapy
-Cefepime 50mg/kg IV q 8
-Meropenem 20-40 mg/kg IV q8
-Zosyn 80-100 mg/kg IV q8
Dual therapy
-Ceftazidime 33-50mg/kg IV q 8 and
Tobramycin 7.5 mg/kg/day IV q8 or 7-9mg/kg IV Q24
(monitor trough levels weekly with aminoglycoside administration due to nephron and ototoxicity)
Anaerobes
-Clindamycin 40mg/kg IV q6 (aspiration pneumonia)
-Flagyl 7.5mg/kg IV q6
-give flagyl if concern for abd infection, typhlitis, perianal or mucosal skin breakdown
Add Vanc if
-AmL receiving high dose Cytarabine (Ara-C) with risk of S.viridans due to interruption of mucosal integrity and risk of sepsis
-hypotensive or have signs of shock
-Mucositis
-Prior h/o alpha hemolytic strep bacteremia
-Concern for catheter site infection or skin breakdown
-Colonized with resistant organisms only sensitive to vancomycin
-Cardiac vegitations
(At risk for tox, monitor trough levels with goal of 10-15 for bacteremia, 15-20 for meningitis)
(at risk for impaired renal function, monitor renal function, electrolytes and fluid balance daily)
Vanc trough level goal for bacteremia and meningitis
10-15 for bacteremia
15-20 for meningitis
when should you cover for fungal in fever neutropenia
fever persists for >1 week
When you have a fever neutropenia pt who you are going to cover for fungal, what to order first
fungal blood culture
serum galactomannan
consider ENT consult for eval of sinuses with flexible scope
CT of sinuses, chest and/or abdomen to evaluate for fungal nodes
antifungal agents in fever neutropenia
Echinocandins - covers most candida and at least fungistatic to Aspergillus. Empiric fungal agents of choice.
-Micafungin
-Caspofungin
-Anidulafungin
Triazoles - Fungicidal to Aspergillus. If clinically worsening
-Fluconazole
-Voriconazole
-Posaconazole
Amphotericin B
-Significant toxicity potential (nephrotoxic)
-Lipid formulation (Ambisome) has less side effects, but is more expensive
HSV and VZV require treatment with
IV acyclovir
Valacyclovir
Famciclovir
If hemoc has VZV how does effect treatment
cessation of chemotherapy administration
labs to monitor when give Acyclovir
nephrotoxic - monitor renal function and fluid intake closely
CMV in hemonc pt, treat with
Ganciclovir
if persistent infection or resistant - transition to Foscarnet or Cidofovir
Hemonc with influenza requires treatment with
Oseltamivir
Most effective if given within 48 hrs of symptom onset
Can be given prophylaxis if exposed to virus
Considered an atypical fungus, causes severe pneumonitis in immunocompromised patients
Pneumocystis Jiroveci
Pneumocystis Jiroveci diagnosis
confirmed by testing induced sputum, lower resp culture
Bronchoalveolar lavage sample
percutaneous needle or open lung biopsy
Pneumocystis Jiroveci prophylaxis in fever neutropenia
Bactrim
Pentamidine
consider addition of corticosteroid
in fever neutropenia, continue appropriate antimicrobials until pt
remained afebrile for at least 24 hours, negative blood cultures from admission for 48 hours, has neg blood cultures providing clearance of infection and with rising ANC demonstrating appropriate bone marrow recovery
Bone marrow recovery - ANC of >200 and rising on 2 consecutive CBCs
Resolution of neutropenia is ANC >500
type A blood has what AB in Plasma and what antigens on RBCs
AB in plasma: Anti-B
Antigens: A antigen
type B blood has what AB in Plasma and what antigens on RBCs
AB in plasma: Anti-A
Antigens: B antigen
type AB blood has what AB in Plasma and what antigens on RBCs
AB in plasma: None
Antigens in RBC: A and B
type O blood has what AB in Plasma and what antigens on RBCs
AB in plasma: Anti-A and Anti-B
Antigens on RBC: none
life threatening disease of bone marrow failure resulting in peripheral pancytopenia and bone marrow aplasia
Aplastic anemia
causes of aplastic anemia
congenital in 20%
Acquired - exposure to drugs, chemicals, ionizing radiation or viruses
s/s of aplastic anemia
History: mucosal/gingival bleeding, headaches, fatigue, easy bruising, rash, fever, mucosal ulcerations, or recurrent viral infections. *
Symptoms: pallor, tachycardia, petechial rash, purpura, ecchymoses, or jaundice.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 785-786). Wolters Kluwer Health. Kindle Edition.
what do labs show in aplastic anemia
*Decrease in hemoglobin, white blood cell (WBC) count, and platelet count.
*Reduction in or absence of the absolute number of reticulocytes.
* Peripheral blood smear; no abnormal cells.
*Reduction or absence of hematopoietic elements from bone marrow aspirate.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
mgmt of aplastic anemia
- Transfusions of RBCs and platelets.
- Antibiotic therapy.
- Bone marrow transplantation (BMT).
*Immunosuppressive therapy if unable to receive a BMT.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
A rare congenital hypoplastic anemia resulting in constitutional bone marrow failure.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 786). Wolters Kluwer Health. Kindle Edition.
Diamond-Blackfan Anemia
Mutation for Diamond-Blackfan on chromosome
19 which encodes for a ribosomal protein known as RPS19
s/s Diamond Blackfan anemia
*Symptoms: pallor, fatigue, irritability, syncope, and dyspnea during feeding.
*Physical examination: irregular heartbeat, hypotonia, short stature, and evidence of failure to thrive.
*Associated with physical defects including craniofacial, hands, upper limbs, cardiac, or genitourinary.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
Diamond Blackfan Anemia is associated with what type of physical defects
craniofacial, hands, upper limbs, cardiac, or genitourinary.
what do labs look like in Diamond Blackfan Anemia
*Profound macrocytic anemia; WBCs and platelet count generally normal.
* Reticulocytopenia.
* Increased percentage of hemoglobin F for age.
* Elevated erythrocyte adenosine deaminase activity. *Decreased or absent erythroid precursors in bone marrow aspirate.
* Genetic screening; Diamond–Blackfan anemia—mutation in RPS19.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
management in Diamond Blackfan Anemia
*Corticosteroids, frequent blood transfusion, BMT in some cases.
* Hematology, BMT, and endocrinology team involvement.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 787). Wolters Kluwer Health. Kindle Edition.
An enzyme that is crucial in aerobic glycolysis
Glucose-6-phosphate Dehydrogenase Deficiency (G6PD)
Children who have G6PD-deficient RBCs are susceptible to
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
oxidative damage and hemolysis during certain conditions of stress, exposure to certain medications, foods, or chemicals. Occurs most often in males.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
G6PD deficiency is most common in what gender
males
which variant?
G6PD activity is less than 10% of normal, resulting in severe neonatal jaundice or congenital nonspherocytic hemolytic anemia.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
Variant 1
G6PD activity is typically less than 30% of the normal range, resulting in an asymptomatic steady state. Individuals who carry this mutation are at risk for neonatal jaundice, acute hemolytic anemia, and favism.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
variant 2
which variant?
G6PD Enzyme activity is greater than 85% of the normal reference range, resulting in no clinical manifestations. Considered the “wild type” disease.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
Variant 3
G6PD is a ___ linked inherited disease that affects primarily ___
X-linked inherited disease that affects primarily men.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 805). Wolters Kluwer Health. Kindle Edition.
G6PD is an enzyme required for the production of the reduced form of ____________ which __
Nicotinamide adenine dinucleotide Phosphate (NADPH) which is critical in preventing oxidative damage
In G6PD deficiency acute exacerbations may occur with ingestion of ____ and what else
fava beans (Favism)
infection
Oxidative drugs ( antimalarials, sulfa containing drugs, ASA, quinolones)
s/s G6PD deficiency
Symptoms: fever, nausea, abdominal pain, diarrhea, and occasionally vomiting within 24 to 48 hours after oxidative challenge. *Findings: Dark brown or black discoloration of the urine is present within 6 to 24 hours after exposure (result of hemolysis); jaundice, pallor, tachycardia, hypovolemic shock, and hepatosplenomegaly may develop.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 806). Wolters Kluwer Health. Kindle Edition.
lab findings for G6PD deficiency
severe anemia with marked variation in size of RBCs resulting in increase in RBC distribution width
WBC may be elevated
Hemoglobinuria may be present
*Large polychromatic cells with spherocytic morphology as well as markedly irregular-shaped cells known as poikilocytes on peripheral blood smear. *Increased reticulocyte count; may reach levels as high as 30%. *Heinz body stain: As the RBCs circulate through the spleen, Heinz bodies are removed, resulting in classic “bite cells.” Heinz bodies are identified with methyl violet staining and are denatured hemoglobin and a manifestation of the oxidative injury to the hemoglobin.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 806). Wolters Kluwer Health. Kindle Edition.
Mgmt G6PD def
*Blood transfusion is indicated if the child is hemodynamically unstable or the hemoglobin level declines to <7g/dL.
*If the hemoglobin is <9 g/dL with evidence of persistent brisk hemolysis with hemoglobinuria, blood transfusion may be indicated.
*Dialysis may be indicated for acute kidney failure.
*Neonatal jaundice related to G6PD deficiency is managed with observation for mild cases, phototherapy, and hydration for more significant cases, and exchange transfusion may be beneficial for severe cases.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 806-807). Wolters Kluwer Health. Kindle Edition.
An acute, systematic, immune complex mediated, small-vessel vasculitis, which is self-limiting and resolves within about 4 weeks, considered the most common vasculitis of childhood.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 801). Wolters Kluwer Health. Kindle Edition.
Henoch-Schonlein Purpura
most common vasculitis of childhood
Henoch-Schonlein Purpura
Peak age presentation in Henoch-Schonlein Purpura
2-8
what months is it more common to see Henoch-Schonlein Purpura
fall, winter and spring
most serious complication of Henoch-Schonlein Purpura
Renal impairment
Henoch-Schonlein Purpura is usually precipitated by
an URI, medication or environmental trigger
what infectious agents associated with Henoch-Schonlein Purpura
Group A strep
what happens in Henoch-Schonlein Purpura
IgA complexes are deposited in the small vessels of the renal glomeruli, skin and GI tract causing petechiae, purpura, GI bleeding and Glomerulonephritis
s/S Henoch-Schonlein Purpura
*Recent upper respiratory tract infection and prodrome of fever and fatigue.
* Commonly presents with tetrad of symptoms:
*Rash: nonpruritic, erythematous papules or wheals that progress to petechiae, and nonblanching, palpable, purpuric lesions >10 mm diameter; found in dependent areas of body that are subject to pressure and extensor surfaces of the extremities. Trunk is usually spared, and lesions fade over 10 to 12 days.
- Polyarthralgias: pain, swelling, decreased range of motion; Lower extremity joints most frequently involved.
- “Bowel angina” - diffuse, colicky abdominal pain with melena and vomiting; approximately 70% of patients.
- Renal symptoms with hematuria, proteinuria, and hypertension; approximately 20–60 % of patients weeks to months after initial presentation
- Mild renal impairment may progress to nephrotic syndrome and ARF.
- Renal biopsy consistent with focal and proliferative glomerulonephritis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 801-802). Wolters Kluwer Health. Kindle Edition.
lab findings in Henoch-Schonlein Purpura
*Based on clinical features and presenting symptoms; renal function should be evaluated at baseline. * Platelets normal or elevated. * BUN and creatinine may be elevated. * Normal coagulation studies. *Immune antibody panel—Presence of IgA antibodies in the blood, skin, or glomeruli may help to confirm diagnosis. * Urinalysis for evaluation of blood and protein.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 802). Wolters Kluwer Health. Kindle Edition.
management of Henoch-Schonlein Purpura
*Rest and activity limitations, with symptomatic management of systemic complications, NSAIDS.
*Oral prednisone; indicated for patients with kidney involvement.
*Henoch-Schönlein purpura resulting in severe kidney disease may require plasma exchange, high-dose IV immunoglobulin (IVIG), or immunosuppressant agents.
* Long-term management of hypertension may be required.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 802-803). Wolters Kluwer Health. Kindle Edition.
a disease of the microcirculation, is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure (ARF).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 791). Wolters Kluwer Health. Kindle Edition.
Hemolytic Uremic Syndrome (HUS)
Hemolytic Uremic Syndrome (HUS) occurs most frequently in what age
children <4 years
Most common cause of acute renal failure
Hemolytic Uremic Syndrome (HUS)
what causes Hemolytic Uremic Syndrome (HUS)
*Contamination of water, meat, fruits, and vegetables with infectious bacteria; peak incidence during summer.
*E. coli 0157:H7 is the most common etiology of postdiarrheal (D+) HUS.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 791). Wolters Kluwer Health. Kindle Edition.
types of HUS
*D+ HUS—Postdiarrheal or typical HUS; occurs in previously healthy children who have had recent gastroenteritis. Mortality rate is 3% to 5%; associated with renal failure in 50% to 70% of patients affected. Bacterial verotoxins, absorbed through intestinal mucosa, are produced by E. coli O157:H7 infection (Shiga toxin; most common cause), Shigella dysenteriae, Citrobacter freundii, and other subtypes of E. coli (also Shiga toxin).
*D−HUS—Atypical or sporadic HUS is less common and more severe than D+HUS with an approximately 25% mortality rate. It is associated with end-stage renal disease in approximately 50% of cases. More common in adulthood, atypical D−HUS infection may have a familial link and may also begin in the neonatal period; occurs year round with no gastrointestinal (GI) prodrome. Causative factors include Inherited factor H deficiency (10%–20%); inhibits complement activation, Membrane cofactor protein mutations, Streptococcus pneumoniae infection, medications including Cyclosporine and Tacrolimus.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 791-792). Wolters Kluwer Health. Kindle Edition.
what does ESR and CRP look like in Oligo, poly and systemic JIA
They can be normal in Oligo
Poly may be mildly elevated
systemic will be more elevated
racoon eyes, abd discomfort, no trauma…what should you be thinking
neuroblastoma
butterfly or malar rash, think
Systemic lupus erythematosus
s/s Systemic lupus erythematosus
Photosensitivity - rash in reaction to sunlight
oral ulcers - usually painless
Nonerosive arthritis
Serositis (pleuritis)
Renal disorder (persistent proteinuria)
Seizures
Anemia, leukopenia, lymphopenia
main worry after BMT
Graft vs host disease
if suspecting Lupus (SLE), but ANA comes back negative. How likely is it to still be Lupus
Very unlikely positive in 99%
Anti-dsDnA is used at time of diagnosis and beyond for Lupus (SLE), why?
to monitor disease activity
C3 and C4 in Lupus (SLE)
low or undetectable during a flair
Anti Smith antibody is highly specific fof
Lupus (SLE) positive in 50% . not used to monitor disease activity
SSA or SSb Ab in Neonatal lupus erythematosus causes destruction to what
conductive system of the heart. Typically leads to congenital heart block. 30-50% of these babies will need a pacemaker
what is the mother given during pregnancy in suspected neonatal Lupus erythematosus
Dexamethasone for Fetal bradycardia to prevent further destruction of conductive system of heart
After birth in neonatal lupus erythematosus, what is the prognosis
most will need a pacemaker
they may have rash or other lupus symptoms
usually all symptoms resolve except heart block approx 6 months later
Video source: lupus (SLE) medication management
Hydroxychloroquine
corticosteroids
Cyclophosphamide (Cytoxan)
Other immunosuppressive agents
NSAIDS
Calcium (due to corticosteroids)
vitamin D (due to corticosteroids)
inflammation of sacroiliac joints (SI) and the axial skeleton
Ankylosing Spondylitis (AS) and Spondyloarthropathy
Ankylosing Spondylitis (AS) and Spondyloarthropathy s/s
Low back pain that is worse in the morning and improves with exercise
expected lab findings in Ankylosing Spondylitis (AS) and Spondyloarthropathy
High ESR and CRP
Positive HLA B27 (helps support dx but not required)
Radiology findings for Ankylosing Spondylitis (AS) and Spondyloarthropathy
Sacroiliitis with sclerosis
Bamboo sign: Bridging syndesmophytes
Bamboo sign on x ray
Ankylosing Spondylitis (AS) and Spondyloarthropathy
clinical presentation of sjogren syndrome
recurrent parotitis
keratoconjunctivitis sicca (more common in adults)
Keratoconjunctivitis sicca is chronic, bilateral desiccation of the conjunctiva and cornea caused by too little tear production or accelerated tear evaporation. Typical symptoms include intermittent itching; burning; blurring, a gritty, pulling, or foreign body sensation; and photosensitivity.
Labs to pay attention to in Sjogren syndrome
Anti-Ro (SS-A) or Anti-La (SS-B)
management of sjogren syndrome
Artificial tears
Pilocarpine tablets
Antimalarial for skin rash and arthritis
sjogren syndrome is associated with r/o
lymphoma
infections associated with reactive arthritis
GI infections
-shigella
-salmonella
-yersinia
-Campylobacter
-C.Diff
GU infections
-UTIS, ect
reactive arthritis is developed _____-___ weeks after infection
2-4
reactive arthritis is usually in how many joints
more than one but can be only one
typical triad s/s of reactive arthritis
Noninfectious urethritis
Arthritis
Conjunctivitis
expected labs in reactive arthritis
elevated ESR and CRP
HLA-B27 positive in 65-96% (helps support case but doesnt rule in or rule out)
treatment for reactive arthritis
supportive care
JDM s/s
muscle pain and weakness
Skin rash
-photosensitivity
-Gottron papules
-Heliotrope rash
Calcinosis cutis
labs to look for in JDM
Elevated CK level
Treatment in JDM
Prednisone
Sun voidance
Hydroxychloroquine
s/s systemic scleroderma
pruritic skin
Raynaud’s phenomenon
difficulty swallowing
shortness of breath
Joint pain and limitation of movement
weakness
lab to look for in systemic scleroderma
Positive SCL 70 (topoisomerase) in 2/3 of pt
pruritic skin
Raynaud’s phenomenon
difficulty swallowing
shortness of breath
Joint pain and limitation of movement
weakness
systemic scleroderma
muscle pain and weakness
Skin rash
-photosensitivity
-Gottron papules
-Heliotrope rash
Calcinosis cutis
JDM
recurrent parotitis
keratoconjunctivitis sicca (more common in adults)
Sjogren syndrome
target sign on US
what can it be seen in?
(they have a rash)
intussusception - can be seen in HSP
s/s in localized scleroderma
Streak involve the face En coup de Sabre
seizure
Uveitis
Treatment in localized scleroderma
mainly supportive
physical therapy if joints resolve
Streak involve the face En coup de Sabre
seizure
Uveitis
localized scleroderma
Prognosis in localized scleroderma
resolve spontaneously within 3-4 years
Behcet’s disease diagnostic criteria
recurrent oral ulcers 3 times over 1 year
+ at least 2 of the following
-Recurrent genital ulceration
-eye inflammation
-Characteristic skin lesions
-positive pathergy test
Treatment for Behcet’s disease (video)
Azathioprine
or
Infliximab
test to confirm Behcet’s disease
Pathergy test
- Pathergy test, in which your doctor inserts a sterile needle into your skin and examines the area one to two days later. If the test is positive, a small red bump forms under your skin where the needle was inserted. This indicates your immune system is overreacting to a minor injury
most common vasculitis in children
Henoch-Schonlein purpura (HSP)
s/s in Henoch-Schonlein purpura (HSP)
purpura
arthritis
GI
-abd pain
-GI bleeding
Nephritis
subcutaneous edema
scrotal edema
purpura
arthritis
GI
-abd pain
-GI bleeding
Nephritis
subcutaneous edema
scrotal edema
Henoch-Schonlein purpura (HSP)
diagnosis for Henoch-Schonlein purpura (HSP)
clinical diagnosis
Platelet count may be normal or elevated (if low, suggests different diagnosis)
occult blood may positive
ESR can be elevated
Screen for blood or protein in urine
In Henoch-Schonlein purpura (HSP) what do you monitor and for how long
Monitor for at least 6 months even if initial was normal
proteinuria
BP for HTN
treatment for Henoch-Schonlein purpura (HSP)
Supportive (hydration , NSAIDS)
Corticosteroid (controversial) however consider in:
-persistent nephrotic syndrome
-severe abd pain
-severe scrotal edema
-neurologic system involvement
Immunosuppressive in complicated cases
d/c causative drug
diagnostic criteria for Kawasaki disease (video)
Fever lasts longer than 5 days plus
4/5 of following main clinical features
-changes in peripheral extremities (redness, edema of hands and feet, followed by desquamation)
-polymorphous rash
-oropharyngeal changes
-bilat, nonexudative, painless bulbar conjunctival injection (redness in both eyes without discharge)
-acute non-purulent cervical lymphadenopathy with lymph node diameter greater than 1.5cm, usually unilateral
common associated symptoms of KD
Hydrops of gallbladder
d/v
abd pain
irritability
vomiting alone
cough or rhinorrhea
decreased intake
weakness
joint pain
echo protocols in KD (video)
at time of dx
repeat in 2nd week
again 1 month after all labs have normalized
1 year if at 8 week no coronary involvement
Refer to pediatric cardiologist at anytime if echo is abnormal
labs suggestive of KD
no specific lab test to dx
CRP >= 3 or ESR >=40
WBC >= 15000
Normocytic, normochromic anemia
Pyuria: >= 10 wbc (clean catch instead of cath is better, can miss the pyuria if collected by cath)
Serum ALT >50
Serum albumin <= 3.0 g/DI
After 7 days of illness, platelet cell count >= 450,000
tx for KD (video)
IVIG (withing first 10 days of illness)
-2g/kg infusion over 12-14 hrs
-watch for anaphylaxis and aseptic meningitis
-relieves acute inflammation to decrease risk of C. aneurysm
Oral ASA
-80 -10mg/kg/day until fever resolves
-decrease to 3-5mg/kg/day if fever resolved and stop if no cardiac involvement after 6-8 weeks
IVIG and ASA have a synergistic effect and gives antiplatelet activity
you can also give corticosteroids
s/s growing pain
diagnosis of exclusion
believed to be a type of stress injury
bilat extremity pain
intermittent pain
pain occurs during evening
can wake from sleep
resolves by morning
does not limit during the day
occurs after days of significant activity
diagnosis of exclusion
believed to be a type of stress injury
bilat extremity pain
intermittent pain
pain occurs during evening
can wake from sleep
resolves by morning
does not limit during the day
occurs after days of significant activity
growing pain
treatment for growing pain
reassurance
Ice
heat
massage
NSAID
s/s reflex sympathetic dystrophy
chronic pain syndrome
pain affect one or more limb
often result of trauma or surgery
limb becomes swollen, red, mottled, warm, cold, sweaty (sympathetic reflex)
-pain is usually out of proportion of touch (hyperalgesia)
diagnosis of reflex sympathetic dystrophy
clinical dx
Affected area may show demineralization on x ray
less uptake on bone scan
treatment in reflex sympathetic dystrophy
aggressive physical therapy
Gabapentin or
Amitriptyline
s/s Familial Mediterranean Fever (MEFV)
periodic fever
severe abd pain
Pleuritis
Pericarditis
scrotal swelling
Erysipelas-like rash may appear around ankle
arthritis, arthralgia, myalgia
key points: periodic fever + pain +/- family history
Familial Mediterranean Fever (MEFV) is autosomal
recessive
Complications of Familial Mediterranean Fever (MEFV)
Amyloidosis leads to proteinuria and renal failure
dx of Familial Mediterranean Fever (MEFV)
clinical dx
can be supported by genetic testing but not excluded by it
ESR, CRP, WBC usually elevated during attack
Treatment for Familial Mediterranean Fever (MEFV)
Colchicine
PFAPA stands for
Periodic fever
Aphthous Stomatitis
Pharyngitis
Cervical Adenitis
Age for PFAPA
6 mos - 7 years
Periodic fevers in PFAPA usually last how many days
5-7 days
In PFAPA, fever cycles usually stops by what age
Teenage years
Treatment for PFAPA (video)
NSAID
single dose of steroids
tonsillectomy
Periodicity for PFAPA is usually how long
less than 4 weeks
painful muscle
weakness
facial rash
elevated ck
dermatomyositis
painful, swollen knee joints,
limited rom for 8 weeks
all other exams and labs are normal
ESR and CRP normal
Oligoarticular JIA
rash worse on sun exposure
joint pain
chest pain
fatigue
anemia
SLE
4 year old boy
bilat leg pain at night
no pain in the morning
physical exam is normal
very active
growing pain
recurrent pain oral ulcers for the last 2 years
deterioration of vision
skin rash
Behcet’s disease
Recurrent fever
abd pain
scrotal swelling
rash on both ankles
elevated ESR
in between attacks ESR is normal
Negative MEFV
FMF
encompasses a complex group of disorders comprising several clinical entities with the common feature of arthritis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1347). Wolters Kluwer Health. Kindle Edition.
Juvenile Idiopathic Arthritis
*JIA is a ________disorder, and the subtypes have varying clinical and laboratory features that may reflect distinct immunopathogenic processes.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1347). Wolters Kluwer Health. Kindle Edition.
heterogeneous
labs in JIA (lippincott)
antinuclear antibody (ANA), rheumatoid factor, and anti–cyclic citrullinated peptide; a positive ANA test does not confirm a diagnosis of JIA but may be prognostic for uveitis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1349). Wolters Kluwer Health. Kindle Edition.
what category of drugs are used in JIA
Anti-inflammatory drugs: corticosteroids—oral and intra-articular injections.
* Immunomodulatory therapy with disease-modifying antirheumatic drugs.
* Tumor necrosis factor α inhibitors.
* Interleukin (IL) inhibitors.
* T-cell- and B-cell-targeted therapy.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1349). Wolters Kluwer Health. Kindle Edition.
A small- to medium-vessel vasculitis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1350). Wolters Kluwer Health. Kindle Edition.
KD
Joint pain only
arthralgia
When you call something Arthritis you should be using objective findings such as
Joint effusion
Warmth
reduced range of motion
+/- pain
usually not erythema
(dr muscal talk)
inflammation of tendon
tendonitis
inflammation of the part of the tendon that connects into the bone
Enthesitis
gel phenomena
if they are in one position for a long time, stiff
Arthritis stiffness pattern
worse with rest, better with activity
% of kids with JIA have a pos ANA
only 50%
acutely swollen joint, particularly if painful with limited motion should be presumed to
be infected and evaluated urgently
arthrocentesis
WBC >100,000
% PMNs >75
Septic
Polymorphonuclear leukocytes (PMNs) are a type of white blood cell (WBC) that include neutrophils, eosinophils, basophils, and mast cells. PMNs are a subtype of leukocytes, which protect the body against infectious organisms. PMNs are also known as granulocytes
Arthrocentesis
WBC <75,000
% PMNs <50
Inflammatory
Polymorphonuclear leukocytes (PMNs) are a type of white blood cell (WBC) that include neutrophils, eosinophils, basophils, and mast cells. PMNs are a subtype of leukocytes, which protect the body against infectious organisms. PMNs are also known as granulocytes
Arthrocentesis
WBC <2000
% PMNs <25
Non-inflammatory
Polymorphonuclear leukocytes (PMNs) are a type of white blood cell (WBC) that include neutrophils, eosinophils, basophils, and mast cells. PMNs are a subtype of leukocytes, which protect the body against infectious organisms. PMNs are also known as granulocytes
Arthrocentesis
WBC <200
% PMNs <25
normal
Polymorphonuclear leukocytes (PMNs) are a type of white blood cell (WBC) that include neutrophils, eosinophils, basophils, and mast cells. PMNs are a subtype of leukocytes, which protect the body against infectious organisms. PMNs are also known as granulocytes
recent strep infection about 2-3 weeks prior
Positive ASO titer
+ Anti-DNAse B
asymmetric arthritis
Migratory arthralgias
doesn’t affect axial skeleton
Prompt response to NSAIDS/ASA
Short duration of arthritis
Pericarditis rare
Acute Rheumatic fever
Acute Rheumatic fever
s/s and labs
recent strep infection about 2-3 weeks prior
Positive ASO titer
+ Anti-DNAse B
asymmetric arthritis
Migratory arthralgias
doesn’t affect axial skeleton
Prompt response to NSAIDS/ASA
Short duration of arthritis
Pericarditis rare
can be symmetrical arthritis (not required)
not migratory
Can affect axial skeleton
slow response to NSAIDS/ASA
Prolonged duration
Can have pericarditis
30% + ASO titers
- Anti-DNAse B (no)
JIA
antecedent streptococcal infection about 2 weeks prior so + ASO titer
Arthritis symmetry
Not migratory
can affect axial skeleton
slow response to NSAIDS/ASA
prolonged duration
Pericarditis rare
+ Anti-DNAse B
Poststreptococcal arthritis (PSRA)
In Poststreptococcal arthritis (PSRA) how long do you need antibiotic prophylaxis
1 year
Other than KD what rheum disease is ASA used routinely to prevent carditis
Acute rheumatic fever
Meds mentioned in muscal lecture for Acute rheumatic fever
ASA 60-100mg/kg div qid
PCN prophylaxis (carditis?)
-PCN VK 250mg po BID
-Bicillin 1.2 million units q 4 weeks (>27kg)
Polyarticular JIA with ANA + will need Optho screening how often?
evaluate for?
3-4 months
Anterior uveitis
anterior uveitis involves what parts of eye
cornea and lens
In Spondyloarthritis or Enthesitis-related arthritis what lab marker is specific but not sensitive
HLA-B27
In Spondylarthritis or Enthesitis-related arthritis what lab marker is specific but not sensitive
HLA-B27
exam finding that suggests Enthesitis
Press on Achilles tendon, if positive they will jump off table in pain
other joints to check:
shoulders
SI joints
chest wall
hip joints where femur connects to hip
Is RF useful in diagnosing kids with JIA
useless
Meds in JIA (muscal lecture)
Daily chronic administration of NSAIDS
Corticosteroids - bridge therapy
Disease modifying or immunomodulating agents
-Methotrexate, tumor necrosis factor blockers
-Enbrel
-Humira
-Remicaide
rheum drug with side effect of retinopathy
hydroxychloroquine
which type of arthritis is associated with symptomatic uveitis
Enthesitis Related arthritis (ERA)
In poly and oligo its typically asymptomatic
IgA deficiency is highly associated with what type of allergy
anaphylaxis
what happens in the second exposure in anaphylaxis
mast cell activation occurs rapidly because the antigen-specific IgE has already bound to the mast cells via IgE receptors, making the cascade of inflammatory reactions rapid.
*Activation of mast cells and basophils triggers a rapid release of various inflammatory and newly formed mediators, including histamine, tryptase, leukotrienes, prostaglandins, platelet-activating factor, and various cytokines.
*Mediators affect various target organs, including the heart, lungs, vasculature, gastrointestinal tract, and skin.
*Heart rate, myocardial contractility, coronary blood flow, and electrical conduction through the sinoatrial and atrioventricular nodes are affected by the release of histamine and platelet-activating factor, causing decreased cardiac output and possible myocardial ischemia.
*Bronchospasm and increased mucus production in the lungs can occur as a result of mediator release.
*Vasodilation and increased vascular permeability lead to hypotension, distributive shock, and eventually impaired oxygen delivery.
*Increased vascular permeability of the airways and intestinal tract causes laryngeal edema and gastrointestinal upset.
*Mucocutaneous symptoms of flushing, angioedema, urticaria, and pruritus occur as a result of histamine release.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 1340-1341). Wolters Kluwer Health. Kindle Edition.
clinical presentation of Anaphylaxis
- Rapid onset: minutes to hours. *Hives, itching, abdominal pain, emesis, stridor, wheezing, shortness of breath, laryngeal edema, hypotension, and shock.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1341). Wolters Kluwer Health. Kindle Edition.
what labs are elevated in anaphylaxis
plasma histamine levels, serum total tryptase levels, and serum IgE levels, if measured during or following an anaphylactic episode, are all elevated.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1341). Wolters Kluwer Health. Kindle Edition.
Mild Anaphylaxis where you gave only one dose epi, how long to monitor after
4-6 hours of obs after epi
Moderate Anaphylaxis - slower to resolve and/or have asthma, how long do you obs after epi
6-8 hours
severe anaphylaxis - hypotension, upper/lower airway compromise, multiple doses of epi, how long do you obs after epi
24 hours
if pt has history of anaphylaxis, what other meds do you need to avoid
B blockers
Ace-Inhibitor
monoamine oxidase inhibitors
Tricyclic antidepressants
Patients taking ____________ may be resistant to EPI and can lead to unopposed a- adrenergic vasoconstrictor effects wiring blood pressure and perfusion
B-adrenergic blockers
If on B blockers first line of choice is still two doses of epi but if not responding may consider glucagon -> reverses refractory hypotension and bronchospasm by increasing cAMP levels through non- B- receptor mechanisms and can be used as adjunctive therapy for B- blocked patients not responding to EPI.
The bezold- Jarisch reflex
initial tachycardia followed by bradycardia
(anaphylaxis notes)
In anaphylaxis blood tryptase levels peak at _____ hrs and return to baseline by ____ hrs after onset of symptoms. Compare to baseline checked at —- hours after onset of symptoms
1-2
5-6
24
in anaphylaxis, Histamine lab is less clinically useful due to
short half life
In anaphylaxis, what is the urine test?
Histamine metabolite N-methylhistamine
-24 hour urine collections may be elevated
-Urine prostaglandin levels may also be elevated
allergy skin testing measures what
Skin testing or measurement of allergen specific IgE levels in the blood → can confirm allergy to clinically suspected triggers
treatment of anyphylaxis
Assess ABCs and mentation… monitor continuously until reaction improves
Place in recumbent position
Severe →35% of intravascular fluid may shift to extravascular space within 10 minutes → sudden death d/t “empty ventricle syndrome”
GIVE EPI
A- adrenergic
Vasoconstriction, increased peripheral vascular resistance, decrease mucosal edema
B- adrenergic
Increased inotropy/ chronotropy, bronchodilation, decreased mediator release
Immediate treatment is vital → delays have been associated with poor outcomes and death
ONE SHOULD NOT WAIT FOR THE DEVELOPMENT OF POTENTIALLY LIFE THREATENING SYMPTOMS (RESPIRATORY OR CARDIAC) BEFORE EPI IS ADMINISTERED.
Give epi → onset of milder symptoms such as pruritus, urticaria, and angioedema alone.
No absolute contraindication to the use of epi
All other treatment → depends on response to initial epi administration
Route of administration
IM
Young children → ½ inch needle
Obsese children ¾ inch needle
Lateral thigh → vastus lateralis muscle
1mg/mL (1:1,000) dose of 0.01mg/kg (0.01mL/kg) →max dose 0.3mg but may dose up to 0.5mg in large pt or severe non responsiveness
First dose asap → repeated every 5 minutes
IV dosing
0.1mg/ml (1:10,000)
0.1ml/kg to max of 3-5mL
If paradoxical bradycardia occurs → atropine can be used → only after failing to respond to epi
Refractory hypotension
IV EPI 0.05-3mcg/kg/min titrated to lowest effective dose
Consider IV dopamine if not effective
Other options → IV vasopressin or methylene blue
Dosing of AIE devices
0.15mg <20kg technically this is for <15kg but no dosing exists from 15-30kg?
0.3mg >20 kg technically >30kg
New dosing of 0.1mg for children 7.5mg-15kg
second line treatment in Anaphylaxis
After epi, fluids, assurance of adequate airway, breathing and circulation
H1 antihistamines → benadrly 1-2mg/kg body weight to max of 50
Only oral for mild cases 40-60% less bioavailability for PO benadryl although dosing is the same, may also give cetirizine
Glucocorticoid steroids
100% oral bioavailability
IV only for no PO intake possible
Oral dosing
1-2 mg/kg/dose max 60mg/dose
Can be given IM but no benefits over IV but complications IM
Atrophy at injection site
Increase risk of acute avascular necrosis of the hip joint if given in the thigh
Little evidence in tx of anaphylaxis
Inhaled SABA
Albuterol may be added after EPI for relief of wheezing especially with patient with pre existing asthma
H2 antihistamines (ranitidine)
Protect against stress and steroid induced gastritis
Data regarding efficacy is lacking
what meds/drugs sensitize the myocardium to effects of epi, increasing cardiac toxicity
cocaine and amphetamines
what does epi do in anaphylaxis
decreases laryngeal edema
treat hypotension and shock
cause bronchodilation
increase cardiac output by increasing heart rate and myocardial contraction
how often can you repeat epi in anaphylaxis
every 5-15 min
in anaphylaxis, how much NS is often required to treat hypotension
30-40ml/kg
medication used for mis-c to block cytokine
Anakinra -IL-1 blocker
Providers should consider mastocytosis or clonal mast cell disorder as an underlying diagnosis for idiopathic anaphylaxis.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1343). Wolters Kluwer Health. Kindle Edition.
IgG present in blood gives us what clues into MIS-C
post infectious
this appears in blood 4-6 weeks after
MIS-C criteria
< 21 yrs
present with fever
lab evidence of inflammation
requires hospitalization
>=2 organ systems
no other plausible diagnosis
+ or recent Sars-Cov 2 infection or Positive PCR within 4 weeks of symptoms
*A group of genetic disorders that affect components of innate and adaptive immune systems and ultimately lead to susceptibility to infection.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1343). Wolters Kluwer Health. Kindle Edition.
immunodeficiencies
s/s MIS-C
GI
mucocutaneous
neurological to include headache
others
resp sx
sore throat
myalgias
swollen hands/feed
lymphadenopathy
Other dx to consider in mis c picture
Typhus
-kids generally look well except with high fever
-Doxy 48-72 hours they will start to defervesce
-Antibody testing -neg early in disease
-Typhus doesnt have a troponin leak like typhus
-Typhus will not need ICU admission
labs to check in MIS C
SARS -Cov-2 RNA PCR
SARS-Cov-2 antibodies
CBC
Chem 10
Ferritin
Troponin
Albumin
Fibrinogen
BNP
LDH
D-Dimer
Procalcitonin
UA
ALT
AST
CRP
flu/rsv
Murine typhus
Urine, throat, wound cultures
abnormal lab findings in Misc
lymphopenia
neutrophilia
mild anemia
thrombocytopenia
CRP, ESR, Ddimer, fibrinogen, Ferritin Procal (PCT), IL-6
all inflammatory markers elevated
If fibrinogen is low, you may have a bleeding problem soon (think DIC picture)
Mild disease: increased AST/ALT, LDH, low albumin, elevated triglycerides
treatment for MISC
Path 1
If they have a complete kd (cKD-like) or incomplete kd (iKD-like) like picture
then
IVIG + steroids
plus
anakinra for shock or cardiac dysfunction
Path 2
does not meet iKD criteria but coronary dilated or other cardiac sign of KD (ie) valvulitis)
then
same as c/ikD like (path 1)
Path 3
If hypovolemic, cardiogenic
and/or distributive shock or
cardiac dysfunction
then
start steroids + anakinra ASAP
Box in corner
Cardiac indications for IVIG
(do not delay hyperinflammatory treatment)
troponin >1
or any two:
1. EF < 50%
2. Troponin >=0.1
3. Classic ECG changes (abnormal ST segments and/or low voltage QRS and/or AV conduction delay and/or ventricular arrhythmia
what cells are responsible for coronary dilation in hyperinflammatory states
Neutrophils (IVIG helps to get rid of these to prevent coronary dilation)
Primary warning signs of primary immunodeficiency
> =4 new ear infections within 1 year
Recurrent, deep skin or organ abscesses
> = 2 sinus infections within 1 year
persistent thrush in mouth or fungal infection on skin
> = 2 mos on antibiotics with little effect
Need for IV antibiotics to clear infections
> =2 deep seated infections including septicemia
> = 2 pneumonias within 1 year
Failure of an infant to gain weight or grow normally
A family history of primary immunodeficiency
3 guiding principles of ICU medicine
DO2 = CO x CaO2 (amount of oxygen dissolved in blood carrying capacity)
MAP=CO x SVR
Never ignore tachycardia
what monitoring/information do I need to look at in ICU to determine shock
physical exam:
are their peripheral extremities really cold
-is there a significant core to toe temp gradient
-Arterial line - is your lactate going up
acid/base status - base excesss?
CVL - mixed/central venous saturation, CVP
NIRS
why is your pt in shock?
cardiac output issue?
-ECHO
-Physical exam
Oxygen demand issue (VO2)?
Vasoplegia?
Whats my BP?
Do I have a SVR issue as well?
What am I going to do when pt is in shock
Is my function impaired?
Increase contractility
-Vasoactives
1) epinephrine
2) milrinone
3) dobutamine
Decrease afterload
1) Nipride/Cardene if BP can tolerate
2) Milrinone (If BP can tolerate) - to take full effect takes 4 hours
3) Positive pressure ventilation
If my oxygen demand (VO2) is excessive, reduce it!
-NIPPV (positive pressure ventilation
-Intubate
-Whiff of sedation
1) ativan
2) precedex - be careful bc it also has beta blockade. watch HR. Can effect cardiac output
The real clinical conundrum in MIS-C is the patient is _____ and has ____ _______
what do you need to do?
Hypotensive
depressed function
Increase cardiac output as much as possible
1) increase contractility (i.e. epinephrine)
2) Optimize fluid status - be careful not to fluid overload. 2.5-5ml/kg
3) Positive Pressure Ventilation - CPAP
BP/SVR support?
-Norepinephrine
or Vasopressin (not a inotrope. Just effects SVR….nothing to help heart squeeze)
Be careful with both
VA ECMO when all else fails
syndromes associated with primary immunodeficiency
22q11 deletion (DiGeorge syndrome)
Ataxia telangiectasia
sinopulmonary infections with encapsulated organisms
Humoral Immunodeficiency
failure to thrive, respiratory tract or gastrointestinal infections, candidal skin infections, Pneumocystis jiroveci pneumonia.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1345). Wolters Kluwer Health. Kindle Edition.
Combined SCIDs
(CGD): infection with catalase-positive organisms (Escherichia coli, Pseudomonas, Klebsiella, Serratia, Salmonella, Candida, and Aspergillus).
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1345). Wolters Kluwer Health. Kindle Edition.
Phagocytic (CGD) Immunodeficiency
- Early complement defects: sepsis.
- Late complement defects: Neisseria infections.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 1345). Wolters Kluwer Health. Kindle Edition.
Complement Immunodeficiency
what AB is the primary player in allergic rx
IgE
acute urticaria
anaphylaxis
oral allergy syndrome
is mediated by what AB?
IgE
Food protein induced enterocolitis syndrome is mediated by what AB
Non-IgE mediated
Atopic dermatitis
Eosinophilic Gastroenteritis
is mediated by what AB
Mixed IgE and non-IgE mediated
allergic contact dermatitis
is mediated by what AB
Cell mediated
Lactose intolerance is mediated by
Metabolic issue
Caffeine intolerance is mediated by
pharmacologic issue
Scromboid fish toxin is mediated by
Toxic issue
Sulfites intolerance is mediated by
idiopathic
An adverse health effect arising from a specific immune response that occurs reproducibly on exposure to a given food
food allergy
presence of IgE antibodies to a food, often in absence of clinical symptoms
Sensitivity
s/s IgE mediated allergic reactions
oropharynx:
oral pruritis
lip swelling
tongue swelling
throat tightening
GI:
Crampy abd pain
nausea
vomiting
diarrhea
Cutaneous
Urticaria
angioedema
Resp
SOB
Stridor
Cough
Wheezing
Cardiovascular
-Feeling of faintness
-Syncope
-Chest pain
-dysrhythmia
-Hypotension
Conjunctival erythema
Tearing
Aura of impending doom
Seizures
any food can induce anaphylaxis but the majority of the most severe reactions are triggered by
peanut
tree nuts
seafood
Pollen-food Syndrome (oral allergy syndrome)
rapid onset oral pruritis and mild angioedema caused by raw fruits and vegetables
Pollen allergens are the primary sensitizers and homologous proteins in plant-derived foods elicit symptoms
30-70% of people with allergic rhinitis have oral allergy syndrome.
Cross reactivity
Birch -> Apple and carrot family
Ragweed -> Melons, Banana
Grass -> Melon, Tomato, Potato, Orange and Swiss chard
Mugwort-> Carrot family, cabbage family, onion family, pepper
Pollen-food Syndrome (oral allergy syndrome)
Cross-reactivity foods associated with Birch allergy
Apple and carrot family
Pollen-food Syndrome (oral allergy syndrome)
Cross-reactivity foods associated with Ragweed
Melons
Banana
Pollen-food Syndrome (oral allergy syndrome)
Cross-reactivity foods associated with Grass
Melon
Tomato
Potato
orange
swiss chard
Pollen-food Syndrome (oral allergy syndrome)
Cross-reactivity foods associated with Mugwort
Carrot family
cabbage family
onion family
pepper
Labs for Immunodeficiency
CBC :
anemia
thrombocytopenia
lymphopenia
neutropenia
Quantitative immunoglobulins (IgG, IgA, IgM, IgE)
suggests immunoglobulin def:
Total protein - low
albumin - normal
Antibody titers to vaccines
Complement activity
-CH50
-C3
-C4
Nitroblue tetrazolium dye test: evaluate for CGD
Primary vs secondary immunodeficiency
Primary - genetic, rare
Acquired - more common, caused by something (HIV, malnutrition, malignancy, DM, splenectomy, ect)
treatment for cellular immunodeficiency
Bone marrow transplant depending on severity
tx for Combined AB and cellular immunodeficiency
Strict isolation
BMT
IVIG
Pneumocystis prophylaxis (Bactrim)
Tx for Phagocytic immunodeficiency
treat infection
Bactrim prophylaxis
Recombinant Gamma interferon
Tx for complement immunodeficiency
prevent infection with vaccines
prompt treatment of infection
How long do you have to remove a food from diet to see if linked to atopic dermatitis
7-14 days. Then reintroduce food. If you have an exacerbation, this pinpoints that food
35% of children with moderate-severe atopic dermatitis have food allergies as a trigger
Prevalence of food allergy is higher in those with
atopic dermatitis
pollen allergies
latex allergy
most common food allergen in children (chart of kids vs adult)
cows milk
egg
crustacean
adult - crustacian
80% of cow milk, soy, egg, wheat allergy remit by
teenage years
allergies to what are typically life long
peanuts
tree nuts
seeds
fish
shellfish
If your allergic to a legume (peanut), you may have a cross-reactivity risk to/ risk of
other legumes 5%
-peas
-lentils
-beans
If your allergic to a tree nut (walnuts), you may have a cross-reactivity risk to/ risk of
other tree nuts -37%
-brazil
-cashew
-Hazelnut
If your allergic to a fish (salmon), you may have a cross-reactivity risk to/ risk of
other fish -50%
-swordfish
-sole
If your allergic to shellfish (shrimp), you may have a cross-reactivity risk to/ risk of
other shellfish -75%
-crab
-lobster
If your allergic to a grain (wheat), you may have a cross-reactivity risk to/ risk of
other grains - 20%
-barley
-rye
If your allergic to cows milk, you may have a cross-reactivity risk to/ risk of
Beef - 10%
goats milk - 92%
Mare’s milk - 4%
If your allergic to peach, you may have a cross-reactivity risk to/ risk of
Other Rosaceae - 55%
apple
plum
pear
cherry
If your allergic to melon (cantaloupe), you may have a cross-reactivity risk to/ risk of
other fruits - 92%
watermelon
banana
avocado
If your allergic to latex, you may have a cross-reactivity risk to/ risk of
35%
kiwi
banana
avocado
If your allergic to kiwi, avocado, banana, you may have a cross-reactivity risk to/ risk of
Latex- 11%
larger skin tests/higher IgE response means
higher likelihood of allergy not more severe allergy
what type of immunodeficiency is r/t
gram negative MOs, viruses, protozoa, fungi, mycobacteria
Cellular immunodeficiency
What type of immunodeficiency is related to gram positive encapsulated MOs and mycoplasma species
Humoral immunodeficiency
What type of immunodeficiency is r/t staph and gram neg (Klebsiella, serratia)
Innate immunodeficiency
What type of immunodeficiency is r/t recurrent Neisseria
complement immunodeficiency
general precautions for immunodeficiency
leukoreduced, viral free blood transfusions
Post exposure prophylaxis for varicella
no live vaccines
education of schools/coaches ect regarding infection concerns
Prophylaxis in humoral immunodef
ABX
Prophylaxis in cellular and phagocytic immunodef
Abx
antifungal
Prophylaxis in complement immunodef
pneumococcal and meningococcal vaccines
stem cell transplant (HSCT) is only treatment option for
SCID
ADA (adenosine deaminase deficiency)
CGD (chronic granulomatous disease)
what immunodef can get live vaccines
complement
If antibody deficiency is present, then treatment with immunoglobulin therapy is required for how long
life - will need ongoing monitoring of IgG levels to ensure they’re adequately replaced
examples include HIV, DM, malignancies, splenectomy, immunosuppressive medications, malnutrition, trauma
secondary immunodeficiencies
is a retrovirus, whose infection occurs when the virus enters the body and binds to receptors on host T cells, fuses with the cell membrane, and then enters
HIV
Seroconversion to HIV antibody positive occurs
between 10-14 days to 3-4 weeks
HIV is lifelong because it infects what cells
long living memory T cells (CD4)
the end result of destruction of HIV Is
HIV is lifelong because it infects the long-living memory T cells (CD4)
· The end result of destruction is the failure of T cell production and eventual immune suppression of both the cellular and humoral parts of the immune system
How is HIV acquired
sex
contaminated blood exposure
perinatal transmission
acute viral syndrome of HIV s/s
fever
fatigue
headache
myalgia
arthralgia
pharyngitis
lymphadenopathy
oral/genital ulcers
nausea
diarrhea
rash
aseptic meningitis
weight loss
oral candidiasis
peripheral neuropathy
o Aphthous ulcers, oral candidiasis, CMV retinitis
o New nodes in patients on antiretroviral therapy may indicate that the disease has progressed, and that treatment failure has occurred
o A new single large node is suspicious for lymphoma
· Skin findings
o HIV dermatitis: erythematous, papular, found in 25% of kids with HIV
o Thrombocytopenia: bruising, bleeding on skin/mucous membranes
o Shingles: vesicles along dermatomes
o Candida dermatitis: unresponsive to other therapies
· Pulmonary: lung disease, LIP, OIs
· Abdominal: hepatomegaly, splenomegaly
acute viral syndrome for HIV generally occurs within
days, up to 10 weeks of initial infection
Opportunistic infections associated with HIV
Pneumocystis jurovecii, bacteremia, lymphocytis interstitial pneumonitis (LIP)
o Kaposi sarcoma, toxoplasmosis, cryptococcosis, CMV
In HIV what may be the first sign of infection during the asymptomatic phase
lymphadenopathy
CD4 count in HIV is used for
reliable indicator for current risk of opportunistic infections
serial counts trending is most helpful
surrogate marker of viral replication rate (rate of progression to AIDS and death is related to this)
viral load
In HIV, brain CT you would be looking for
white matter degeneration
In HIV, Abd us may show
calcifications in liver, spleen or kidneys
Prevention of Mycobacterium avium complex (MAC) in HIV
Azithromycin or
clarithromycin
Prevention of PJP in HIV
Cotrimoxazole
vaccines and HIV
if symptomatic - no live vaccines
all should receive MMR-V separately (M, M, R, V) because they are all live
should receive annual killed flu vaccine
Is HIV a reason to exclude kids from sports
no but consideration should be given for high impact sports like boxing
anaphylaxis s/s for infants
Symptoms often cannot be communicated
Can be confused for normal behavior → drooling, crying, postprandial drowsiness , emesis/ reflux from colic, respiratory distress can be confused for croup, asthma, bronchiolitis or unresponsiveness from seizures/ sepsis.
Anaphylaxis can occur the first time a child eats a food so difficult to know the cause
humoral is r/t
b cells
cellular is r/t
T cells (T lymphocytes)
Combined immunodeficiency is r/t what cells
B and T cells
The major hallmark of any primary immunodeficiency is
susceptibility to infection
immunodeficiency that is usually asymptomatic and does not generally carry increased risk of infection
IgA deficiency
infection history usually seen in Humoral Immunodef
sinopulmonary
otitis media
GI
cellulitis
meningitis
osteomyelitis
haemophilus
pneumococci
streptococci
Giardia lamblia
cryptosporidium
enterovirus
also GI problems to include malabsorption
infection history usually seen in Cellular immunodef
pulmonary
GI
Skin
candida
PJP
CMV, EBV, RSV, Parainfluenza, adenovirus
mycobacterium
also see FTT
oral candiddiasis
skin rashes
dermatitis
postvaccination diseases from live viral vaccines
infection history usually seen in phagocytic disorders
severe skin and visceral infections by common pathogens
staph aurus
pseudomonas species
serratia
klebsiella
candida
nocardia
aspergillus
also see
granuloma formulation include granulomatous enteritis
poor wound healing
abscesses
oral cavity infections
anorectal infections
infection history usually seen in complement disorders immunodef
meningitis septicemia
Neisseria infections:
meningococcal, pneumococcal
also Rheumatoid disorders lupus like syndrome, angioedema
HIV testing can occur in ages
> 18 mos
moms abs before
SCID is more commonly seen in what population
Navajo
Agammaglobulinemia
subtype
age of onset
lab findings
associated findings
Humoral
>6 mos
absent IgG, IgA and IgM
absent AB responses
Absent B cells
Absence of tonsils and lymph nodes
common variable immune deficiency
subtype
age of onset
lab findings
associated findings
Humoral
any age, peaks in second decade
low IgG
+/- IgA and IgM
Absent ab responses to vaccines
Normal to high numbers of B cells
+/- autoimmune cytopenias
autoimmunity
lymphoproliferative and granulomatous diseases
lymph nodes present to increased
hepatomegaly
splenomegaly
Hyper IgM
subtype
age of onset
lab findings
associated findings
Humoral
>6 mos
Very low IgG adn IgA
Increased IgM
B cells normal to increased
Neutropenia
Autoimmunity
Specific ab deficiency with normal IgG level and normal B cells
subtype
age of onset
lab findings
associated findings
Humoral
any age
Normal IgG
inability to produce AB to vaccines present
autoimmunity
lymph nodes normal to elevated
hepatomegaly
splenomegaly
increased association in syndromes
Transient Hypogammaglobulinemia of infancy
subtype
age of onset
lab findings
associated findings
Humoral
> 6 mos to 4-5 years
Low IgG and IgA
specific Ab to vaccines present
Normal lymphoid tissue
SCID
subtype
age of onset
lab findings
associated findings
Cellular
< 6 mos
decreased T cells
Increased or decreased B cells
decreased NK cells or normal
Low IgG, IgA, IgM
Absent lymphoid tissue
absent thymic shadow on x ray
Wiskott-Aldrich syndrome
subtype
age of onset
lab findings
associated findings
Cellular
birth to 1 yr
low T cells
B cells normal
low IgM +/- increased IgA and IgE
decreased polysaccharide
low small platelets
lymphopenia
eczema
lymphoma
autoimmunity
Humoral
>6 mos
absent IgG, IgA and IgM
absent AB responses
Absent B cells
Absence of tonsils and lymph nodes
Agammaglobulinemia
Humoral
>6 mos
Very low IgG adn IgA
Increased IgM
B cells normal to increased
Neutropenia
Autoimmunity
Hyper IgM
Humoral
any age
Normal IgG
inability to produce AB to vaccines present
autoimmunity
lymph nodes normal to elevated
hepatomegaly
splenomegaly
increased association in syndromes
Specific ab deficiency with normal IgG level and normal B cells
Humoral
> 6 mos to 4-5 years
Low IgG and IgA
specific Ab to vaccines present
Normal lymphoid tissue
Transient Hypogammaglobulinemia of infancy
Cellular
< 6 mos
decreased T cells
Increased or decreased B cells
decreased NK cells or normal
Low IgG, IgA, IgM
Absent lymphoid tissue
absent thymic shadow on x ray
SCID
Cellular
birth to 1 yr
low T cells
B cells normal
low IgM +/- increased IgA and IgE
decreased polysaccharide
low small platelets
lymphopenia
eczema
lymphoma
autoimmunity
Wiskott-Aldrich syndrome
Ataxia telangiectasia
subtype
age of onset
lab findings
associated findings
Cellular
<3 years
low IgA, IgE and IgG
Variable AB def
Increased alpha fetoprotein
Chromosomal instability
ataxia
telangiectasia
radiation sensitivity
increased frequency of malignancy
DiGeorge
subtype
age of onset
lab findings
associated findings
Cellular
birth to any age
T cell low or normal
B cell normal
Immunoglobulins normal or low
low calcium
low PTH
heart defects
abnormal facies
autoimmunity
IRAK4 deficiency
subtype
age of onset
lab findings
associated findings
Innate
infancy
lymphocytes and monocytes
toll and IL-1 receptor
signaling IRAK 4 pathway abnormal
Cellular
<3 years
low IgA, IgE and IgG
Variable AB def
Increased alpha fetoprotein
Chromosomal instability
ataxia
radiation sensitivity
increased frequency of malignancy
Ataxia telangiectasia
Cellular
birth to any age
T cell low or normal
B cell normal
Immunoglobulins normal or low
low calcium
low PTH
heart defects
abnormal facies
autoimmunity
DiGeorge
Innate
infancy
lymphocytes and monocytes
toll and IL-1 receptor
signaling IRAK 4 pathway abnormal
IRAK4 deficiency
Nemo
subtype
age of onset
lab findings
associated findings
innate
any age
lymphopenia
low igG
high IgM
conical teeth
colitis
ectodermal dysplasia
sparse hair
opportunistic infections
innate
any age
lymphopenia
low igG
high IgM
conical teeth
colitis
ectodermal dysplasia
sparse hair
opportunistic infections
Nemo
Hyper IgE (job syndrome)
subtype
age of onset
lab findings
associated findings
Other
any age
Normal T and B cells
increased IgE
staph aureus infections
thickened skin
pneumatoceles
eczema
nail candidiasis
broad nasal tip
delayed shedding of primary teeth
hypermobility of joints
Other
any age
Normal T and B cells
increased IgE
staph aureus infections
thickened skin
pneumatoceles
eczema
nail candidiasis
broad nasal tip
delayed shedding of primary teeth
hypermobility of joints
Hyper IgE (Job syndrome)
Chronic mucocutaneous candidiasis
subtype
age of onset
lab findings
associated findings
other
any age
T and B cell numbers normal
Impaired DTH to candida antigens
normal AB
skin and nail changes
autoimmunity
other
any age
T and B cell numbers normal
Impaired DTH to candida antigens
normal AB
skin and nail changes
autoimmunity
chronic mucocutaneous candidiasis
Chronic granulomatous disease
subtype
age of onset
lab findings
associated findings
other
> 6 months of age
Abnormal killing; faulty oxidative burst via NBT or flowcytometry testing
Granuloma on x ray
GI abnormalities
Abscesses
Complement deficiencies
subtype
age of onset
lab findings
associated findings
other
Any age
Absent CH50
+/- absent alternate pathway
Absent specific complement
absent specific component
Autoimmunity
angioedema
> 6 months of age
Abnormal killing; faulty oxidative burst via NBT or flowcytometry testing
Granuloma on x ray
GI abnormalities
Abscesses
Chronic granulomatous disease
Any age
Absent CH50
+/- absent alternate pathway
Absent specific complement
absent specific component
Autoimmunity
angioedema
Complement deficiencies
Treatment options in Humoral primary immunodeficiency
Abx
IVIG
Immunosuppressive medication and biologics
Treatment options in Cellular primary immunodeficiency
HSCT (stem cell transplant)
Gene therapy
Immunoglobulin
ABX prophylaxis
Antifungal prophylaxis
Immunosuppressive medication and biologics
Treatment options in Phagocytic primary immunodeficiency
Abx prophylaxis
antifungal prophylaxis
Gamma interferon
HSCT (stem cell tranplant)
Gene therapy
Treatment options in Complement primary immunodeficiency
Abx prophylaxis
pneumococcal and meningococcal vaccines
A disease of either a quantitative deficiency or qualitative defect of the von Willebrand protein.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 820). Wolters Kluwer Health. Kindle Edition.
Von Willebrand Disease
*Patients who are deficient or have a defect of VWF are at risk for
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 821). Wolters Kluwer Health. Kindle Edition.
prolonged bleeding particularly from the mucous membranes
why could repeated lab testing be needed to dx Von Willebrand disease
Von Willebrand protein levels may fluctuate
what med stimulates release of VWF and Factor VIII
Desmopressin Acetate (DDAVP)
*Used in managing recurrent bleeding in VWD, but do not stop active bleeding; slow the breakdown of clots to preventing rebleeding.
examples?
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 821). Wolters Kluwer Health. Kindle Edition.
Antifibronolytic agents
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 821-822). Wolters Kluwer Health. Kindle Edition.
Meds used in VWB disease
Desmopressin Acetate (DDAVP) -> stimulates release of VWF and Factor VIII
* Management of prolonged or refractory bleeding and prior to minor elective procedures.
Antifibronolytic agents
- Aminocaproic acid (Amicar); contraindicated with hematuria.
- Tranexamic acid (Lysteda): FDA-approved for menorrhagia.
VWF concentrates are derived from human blood donation
_______ has been demonstrated to have an impact on VWF levels, so knowledge of hormonal contraception or pregnancy is important when evaluating adolescents.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 822). Wolters Kluwer Health. Kindle Edition.
Estrogen
is extracted from whole blood and contains coagulation, fibrolytic, and complement systems that assist in the restoration of coagulation disorders such as DIC.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 823). Wolters Kluwer Health. Kindle Edition.
FFP
are responsible for hemostasis with resulting thrombus formation.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 823). Wolters Kluwer Health. Kindle Edition.
platelets
obtained by centrifuging plasma and removing the precipitate. It is not used as commonly as FFP, but can be used to replace low fibrinogen levels and when certain factors are not available for treatment of coagulation disorders.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 823). Wolters Kluwer Health. Kindle Edition.
Cryoprecipitate
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 823). Wolters Kluwer Health. Kindle Edition.
PRBC tranfused at ___ to ___ml/kg per transfusion
10-20ml/kg
Platelets are transfused at __ unit per ever ___kg
1 unit/10kg
FFP is transfused at
10-15ml/kg/transfusion
Cryoprecipitate is transfused at
expect rise of?
1 unit per every 10kg
expect rise of 60-100mg/dl
calculating prbc transfusion dose formula
Volume of PREC required (ml) = desired HCT - initial HCT x Total body volume. Divided by HCT
total body volume (infant - 100ml/kg
child-80ml/kg
adult - 65ml/kg)
____ is possible when children drink large volumes of milk
anemia
Stored PRBCs lack _____ content so children who receive any type of multiple transfusion requires what?
calcium
additional calcium
PRBC s stored > 5 days are associated with
higher potassium concentration
Based on illness severity, infants < ___ months will not require cross matching
4 months
estimating circulating blood volume is based on an average calculation of
80ml/kg
adults are 70mk/kg
Massive transfusion complications include
thrombocytopenia, hypocalcemia, coagulation factor depletion, hyperkalemia, and increased levels of lactic acid, leading to acid–base disorders, hypothermia, and altered or decreased oxygen delivery to tissues.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 824). Wolters Kluwer Health. Kindle Edition.
each unit of RBC contains _____ iron
200-250mg
Accumulation of iron leads to the formation of nontransferrin-bound iron, which is associated with
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 825). Wolters Kluwer Health. Kindle Edition.
progressive organ damage, primarily liver and heart disease.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 825). Wolters Kluwer Health. Kindle Edition.
*In children, iron accumulation in the anterior pituitary gland will produce
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 825). Wolters Kluwer Health. Kindle Edition.
systemic endocrine disturbances, including delayed sexual maturation and growth failure.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 825). Wolters Kluwer Health. Kindle Edition.
Congestive heart failure can be present in children as young as ___ years of age as a result of iron overload.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 825). Wolters Kluwer Health. Kindle Edition.
15
chelation agents for iron tox
Iron chelation therapy is often required. * Chelation therapies are utilized to treat iron overload by binding metal ions that are then excreted through feces. * Deferoxamine, IV chelation agent. * Deferasirox, oral chelation agent is administered once daily.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 825-826). Wolters Kluwer Health. Kindle Edition.
Administer blood products (RBC and platelets) through a _______ system to reduce the risk of febrile reactions, platelet alloimmunization, and CMV transmission.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (pp. 828-829). Wolters Kluwer Health. Kindle Edition.
a leukofiltration system
Irradiate blood products to prevent
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
Graft vs Host Disease
Use leukoreduction for blood products (except granulocytes) to decrease
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
decrease the risk of sensitization, the risk of CMV, and decrease the number of febrile nonhemolytic transfusion reactions. Administer CMV—negative blood.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
Washed products reduce the risk of
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
inflammatory markers
Administer single donor platelets to reduce the exposure to
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
multiple donors, platelet alloimmunization and septic reactions
For chronically transfused patients, administer units < __ days old.
Kline, Andrea M.; Haut, Catherine. Lippincott Certification Review: Pediatric Acute Care Nurse Practitioner (p. 829). Wolters Kluwer Health. Kindle Edition.
21
methotrexate is used in what rheum disorders
JIA
JDMS
SS (Sjogrens syndrome)
SLE
SE methotrexate
n/v
hepatotoxicity
Bone marrow suppression
What category of drug is Methotrexate
DMARD
Sulfasalazine is used in what rheum
JIA (ERA subtype)
Category of drug is Sulfasalazine
DMARD
Contraindications and SE of Sulfasalazine
Sulfa allergy
GI tox
monitor WBC count
Hydroxychloroquine is used in what rheum
category of drug
DMARD
SLE
SE of Hydroxychloroquine
Retinal hyperpigmentation
can cause retinal damage with vision loss
Thalidomide
what rheum used in
cat of drug
SE
SJIA
DMARD
can cause peripheral neuropathy
teratogenic
Mycophenolate mofetil (cellcept)
what rheum is used in
cat
se
Lupus nephritis class III/IV/V
JDMS
MCTD (mixed connective tissue disease)
Diarrhea
pancytopenia
decreases effectiveness of oral contraceptives
- A previously healthy 12-year-old female developed hives, shortness of breath, and dizziness during her fourth-
period class following lunch. For lunch the girl ate a peanut butter and grape jelly sandwich, an apple,
a chocolate chip cookie, and a container of milk. Which of the following food items is most likely to have
caused this reaction?
A. Apple.
B. Chocolate.
C. Cow Milk.
D. Grape Jelly.
E. Peanut Butter.
E. Peanut Butter
what allergies is a contraindication to propofol
soy and egg
which of the following are the most common food allergens for children living in the USA
peanuts
tree nut
egg
milk
soy
flatfish
wheat
another source shellfish
T/F
Specific IgE testing for food allergens is appropriate when clinical history supports a possible food allergy
True
or the girl in the previous question, the school nurse administered a dose of epinephrine. Shortly afterwards
the girl felt well. Twenty minutes later, she began to experience similar symptoms. Which of the following
actions is most appropriate to initiate now?
A. Diphenhydramine.
B. Epinephrine.
C. Glucocorticosteroid
D. Placement into the recumbent position.
E. Ranitidine.
B. Epinephrine
A 6-year-old male presented in late spring with large (3 cm by 3 cm) circular vesicular lesions with pinpoint
centers distributed in a linear fashion on both lower arms. This was the fourth incident of similar symptoms. He
has had no systemic symptoms. Which of the following is the most appropriate treatment for this child’s
lesions?
A. Diphenhydramine.
B. Epinephrine.
C. Leukotriene modifier.
D. Mid-potency topical steroid.
E. Ranititdine.
D. Mid-potency topical steroid.
Over a 24-hour period a 10-year-old girl developed swelling of her face, including lips and eyelids plus both
hands. She had no itching or visible rash. She had a history of two previous similar episodes. She was otherwise
well except for a recent tooth extraction in concert with placement of dental braces. Which of the following is
the most appropriate next step in management for this child?
A. C3 and C4 testing.
B. Diphenhydramine.
C. Epinephrine.
D. Third-generation cephalosporin.
E. Penicillin skin testing.
C. Epinephrine.
A 9-year-old boy developed nausea, abdominal cramping, vomiting, and diarrhea three hours after eating
supper. He had eaten fish sticks, potato salad, and milk. He had no rash, swelling or other cutaneous findings.
Which of the following treatments was most appropriate for this boy?
A. Epinephrine.
B. Diphenhydramine.
C. Fluids.
D. Glucocorticosteriods.
E. Oxygen.
B. Diphenhydramine.
stinging insects associated with anaphylaxis
honey bee
wasp
hornet
yellow jacket
fire ant
latex allergy examples
balloons
gloves
medical equiptment
things in vaccinations causing anaphylaxis
Gelatin
egg
yeast
neomycin
what food allergies are usually outgrown during first decade of life and which are usually lifelong
grown out
milk
egg
soy
wheat
lifelong
peanut
tree nut
fish
shellfish
2 most frequent culprit for med related anaphylaxis
B lactam abx and NSAIDS
also chemo drugs (Cisplatinum and carboplatinum and biologic agents and monoclonal ab such as omalizumab)
Latex allergy is caused by sensitization to
any of the antigens from the
Hevea brasiliensis tree
dosing from lecture for epi
10kg-24.9kg -> 0.15mg
25kg and above 0.3mg
Benadryl dosing
1.25mg/kg
if pt has severe eczema, egg allergy or both, when is the soonest you can introduce peanut
4-6 mos
