PNP-PC Part 3 Flashcards
Most common anemia in childhood
Iron-deficiency anemia
When is the routine screening of hemoglobin?
Between 9-12 months
What is the common age of Iron-deficiency anemia?
1-3 years of age
What is classified as mild Iron-deficiency anemia?
9.5-11
What is classified as severe Iron-deficiency anemia?
8-9.5
Poor weight gain Sclera or palmar pallor Splenomegaly Tachycardia Systolic flow murmurs with progression
Iron-deficiency anemia severe
What will RBC be for Iron-deficiency anemia?
Microcytic and hypochromic
What will MCV be for iron deficiency anemia?
Increased, decreased, normal
Decreased
What will serum ferritin be for iron deficiency anemia?
Increased, decreased, normal
Decreased
What will serum iron be for iron deficiency anemia?
Increased, decreased, normal
Decreased
What will total iron-binding capacity be for iron deficiency anemia?
Increased, decreased, normal
Increased
What will iron saturation be for iron deficiency anemia?
Increased, decreased, normal
Decreased
What nutrition changes should be changed for iron deficiency anemia?
reduce milk to no more than 16- 24 oz/day at 1 year
Increase intake of high-iron foods (dark green veggies, beans, whole cereals, pork, beef)
What is iron therapy for mild to moderate iron-deficiency anemia?
Ferrous sulfate
3-6 mg/kg/day in 2 divided doses
What is iron therapy for severe iron deficiency anemia?
4-6 mg/kg/day in 2-3 doses
How early can you start ferrous sulfate in premature infants?
2 months
How early can you start ferrous sulfate in term infants?
4-6 months
Inherited anemia that affects both males and females
Most often affects Italian, Greek, Middle Eastern, Asian, and African descent
Thalassemia
Pale, poor appetite, dark urine, jaundice, liver, spleen, and heart enlargement, bone problems, and failure to thrive
Severe Thalassemia
Hemoglobin electrophoresis testing
Thalassemia
Moderate/Severe Thalassemia management
Referral to hematolgoy
Regular blood transfusions, iron chelation therapy, folic acid, B vitamins help build healthy RBCs
Autosomal-recessive heme disorder
Sickle cell disorder
Pallor, jaundice, tachycardia, fatigue Vaso-occlusion Cerebrovascular accident Acute Chest Syndrome Priapism Ocular retinopathy Gallbladder disease Splenomegaly Cardiomegaly Failure to thrive Dactylitis
Sickle cell disorder
What are important education points for sickle cell anemia?
Adequate fluid intake
Fever (101F) is an emergency
Emergency admission for sickle cell disorder
Fever (1010)
Acute chest syndrome–pneumonia, chest pain
Sequestration crisis (left-sided abdominal pain, difficulty breathing, fever, pain, vomiting)
Aplastic crisis
Severe painful crisis
Unusual headache, visual disturbance
Priapism
How often do you check infant birth to 6 months with sickle cell disease?
CBC every 2 months
How often do you see infants 6 months to 2 years with sickle cell disease?
Every 3 months
CBC every 3-6 months
UA annually
Ferritin, TIBC, BUN, CREAT, LFTs once at 1-2 years
Hemolytic anemia that is characterized by deficiency or abnormality of RBC membrane protein which reduces RBC surface area; sequestered in the spleen due to shape
Seen in northern European ancestry
Hereditary Spherocytosis
Jaundice in the newborn period Splenomegaly after 2 years of age Chronic fatigue Malaise Abdominal pain
Hereditary Spherocytosis
Splenectomy is curable
Hereditary Spherocytosis
At what age should splenectomy be performed for severe Hereditary Spherocytosis?
6 years old
What vaccines must be given before splenectomy can be performed for Hereditary Spherocytosis?
Pneumococcal and Meningococcal
Headache, stomach ache, irritability, tiredness, poor appetite, poor attention span and memory, sleep disturbances, weight loss, muscle weakness, diminished DTRs, seizures, loss of visual-motor coordination
Lead Poisoning
Free erythrocyte protoporphyrin (FEP)
lead poisoning diagnosis
What blood level lead is required to refer?
All values >70
Thrombocytopenia (less than 150,000) in absence of other occurs; peak occurrence between 2-4 years
Most common after a febrile, viral illness
Idiopathic Thrombocytopenic Purpura (ITP)
Acute onset of petechiae, purpura, and bleeding in otherwise healthy child
Recent viral illness (1-4 weeks)
Hemorrhage of mucous membranes (gums, lips)
Epistaxis that is difficult to control
Menorrhagia
Liver, spleen, lymph nodes normal
Bone pain and pallor are rare
Idiopathic Thrombocytopenia Purpura (ITP)
Low platelet count (severe = <20,000)
Normal PT, aPTT
Normal WBC and RBC
Idiopathic Thrombocytopenia Purpura (ITP)
Labs:
PT & aPTT are elevated
Thrombocytopenia
DIC
Labs:
Prolonged PT and aPTT
Normal platelet
Coagulation factor deficiency
Sick, febrile child
Isolated thrombocytopenia
Petechia/Purpura
Meningococcemia
If platelet is >20,000 and no bleeding is observed what is the treatment for ITP?
Avoid contact sports, aspirin, and NSAIDs
If a child has an increased risk for serious bleeding with ITP, what is the treatment?
Corticosteroids
Most common form of systemic vasculitis in children
results from autoimmune reaction where body attacks its own tissues usually after respiratory infection, immunization, insect bite, or allergic reaction
Mean age: 6-7 years old
Henoch-Schonlein Purpura (HSP)
Purpura
Arthralgia/arthritis
Colicky abdominal pain
Henoch-Schonlein Purpura (HSP)
What should be performed on all patients with Henoch-Schonlein Purpura (HSP)?
Urinalysis to screen for renal involvement
What is management for Henoch-Schonlein Purpura (HSP)?
Most recover spontaneously Primarily supportive (adequate hydration, rest, relief of pain)
X-linked recessive hereditary disease
Most exclusively in males
Hemophilia A and B
Excessive bruising
Prolonged bleeding from mucous membranes after minor lacerations, circumcisions, or menstruation
Hemarthrosis charactered by pain and swelling in elbows, knee, and ankles
Hemophilia A and B
Which is most common between hemophilia A and B?
hemophilia A
What is the leading type of significant bleeding with hemophilia A and B?
Hemarthrosis
How do you treat hemarthrosis?
Apply ice and pressure to affected joints
What medications should patients with hemophilia A and B avoid?
Aspirin and NSAIDs
How should immunizations be given for hemophilia A and B?
Subcutaneous with 26G needle
IM with 23G needle
Hold pressure and ice for several minutes
Autosomal dominant
Most common inherited bleeding disorder
Occurs in both sexes
von Willebrand Disease
Epistaxis, menorrhagia
Easy bruising
Excessive posttraumatic or post-op bleeding
Hx of ecchymosis of trunk, upper arms, thighs
von Willebrand Disease
Labs: CBC w/ diff Normal platelet count PT, aPTT von Willebrand panel
von Willebrand Disease
What should workup include for unexplained splenomegaly?
H&P CBC with differential Reticulocyte count Peripheral blood smear LFTs EBV and CMV chest x-ray Abdominal US
URI is most common cause
Ill-appearing, febrile, progressive or persistent symptoms with CBC w/ diff, GAS, ESR, CRP, hepatic profile, blood culture, EBV, CMV, HIV, Chest-x-ray
Acute bilateral cervical lymphadenopathy
Usually caused by S. aureus Children with poor oral hygiene Blood culture if ill-appearing Throat culture Empiric therapy for moderate symptoms
Acute unilateral cervical lymphadenopathy
Unexplained weight loss Headache in the early morning Swelling/persistent pain in joints/back, or legs Lumps/masses Excessive bruising/bleeding Rash Recurrent infections Persistent N/V Fatigue Vision changes Recurrent fever without cause
Cancer
Most common form of childhood cancer
Leukemia
80% of childhood leukemia cases
Acute lymphoblastic leukemia (ALL)
Peak incidence of ALL
2-6 years
Anemic, pale, listless, irritable, or chronically tired
History repeat infections, fever, weight loss
Bleeding episodes
Lymphadenopathy
Hepatosplenomegaly
Bone/Joint Pain
Leukemia
Thrombocytopenia and anemia
WBC may be elevated, normal, or low
Malignant cells on peripheral smear
Bone marrow aspiration
leukemia
Solid tumors in lymphatic system; higher incidence in males; more common in 2nd decade of life
Non-Hodgkin Lymphoma
Acute abdomen pain, distention, fullness, and constipation
Nontender lymph nodes enlargement
Non-Hodgkin Lymphoma
Cervical nodes and spreads to other nodes; rare in children under 15
Hodgkin Lymphoma
Painless enlargement of lymph node (cervical)
Chronic cough (trachea compressed)
Fever
Decreased appetite
Weight loss of 10% or more within 6 months
Hodgkin Lymphoma
Anemia
Elevated ESR, CRP
Abnormal LFTs
UA-proteinuria
Hodgkin Lymphoma
most common primary site of neuroblastoma
adrenal gland
In advanced disease, appear ill on presentation with systemic signs and symptoms: **Abdominal mass Abdominal pain/constipation Proptosis Horner syndrome Localized back pain and weakness Scoliosis, bladder dysfunction Plapalte non-tender subcutaneous nodules Fever, weight loss Heterochromia iridis
Neuroblastoma
Presents with leukocoria (white reflex) in child <3
Strabismus, nystagmus, red inflamed eye
Retinoblastoma
2nd leading cause of cancer death in children
CNS tumor
headache early in the morning relieved by vomiting
CNS tumor
What diagnostic tools are used to diagnose CNS tumors?
MRI or CT
Diagnosis requires persistent arthritis for more than 6 weeks in patients less than 16 years
Juvenile Idiopathic Arthritis
Is juvenile idiopathic arthritis more common in males or females?
Females
Polyarthritis JIA
> 5 inflamed joints
Oligoarthritis JIA
<5 inflamed joints
Systemic-onset JIA
arthritis with characteristic fever
Pain aching Joint stiffness (worse in the morning and after rest) Swelling of joint with effusion Heat over inflamed joint Loss of ROM of affected joints
Juvenile Idiopathic Arthritis
Fever salmon-colored rashes leukocytosis lymphadenopathy rheumatoid nodules
Juvenile Idiopathic Arthritis
What is the common treatment for JIA?
NSAIDs
Corticosteroids
Antirheumatic drugs/Methotrexate
Physical Therapy
5 joints or more, symmetrical affected
Females more than males
Fever and rash are possible
Subcutaneous rheumatoid nodules
Polyarthritis JIA
What is the treatment for polyarthritis JIA?
NSAIDs and DMARDs (Methotrexate)
4 or fewer joints affected
Peak onset 1-2 years
Females > Males
Uveitis–must have slit-lamp eye exam every 3 months
Oligoarticular JIA
Onset anytime before 16 years
Female and Males equally affected
Fever, rash, arthritis
Inflammation in and around internal organs (carditis/pericarditis, pneumonitis/pleuritis)
Swollen lymph nodes, enlarged liver, and spleen
Elevated ESR, CRP, ferritin, WBC
Prolonged clotting time, low fibrinogen, elevated d-dimer
Systemic JIA
Ocular pain and redness Change in vision Photophobia headache Asymptomatic
Uveitis
JIA Reactive arthritis Kawasaki disease Bechet's HSP Wegener's Grandulomatosis Pars planitis HIV, EBV, Cat-srtach Herpes Lyme disease
all have which condition in common
Uveitis
What is the first line of treatment for uveitis?
Refer to ophthalmologist
Topical corticosteroids with mydriatics
Chronic, systemic disease characterized by altered immune regulation that can involve inflammation in multi-organ systems
Systemic Lupus Erythematosus (SLE)
What is the median age of onset for Systemic Lupus Erythematosus?
11-12 years
What is the hallmark for systemic lupus erythematosus?
Butterfly or malar erythematous facial rash which increases in intensity in sunlight
**Joint pain Low-grade fever Weight loss Painless mouth ulcerations Skin rashes Sun sensitivity Fatigue Proteinuria Hematologic disorders Pallor, petechiae Purpura Malar Mouth ulceration, gingivitis, serositis Cardiac friction rub (pericarditis) Pleural friction rub (pleurtic) Hepatosplenomegaly Lymphadenopathy
Systemic lupus erythematosus
What is common treatment for systemic lupus erythematosus?
Sunscreen
NSAIDs, oral steroids, antimalarial drugs
Vitamin D and calcium supplementation
Chronic, idiopathic pain syndrome
Females > Males
Mean onset of age is 12
Fibromyalgia Syndrome
If ANA is - what does this mean for systemic lupus erythematosus?
It is ruled out
Pain at multiple sites (muscles and soft tissues around joints) Pain may awaken from sleep and interfere with activities Fatigue and malaise Paresthesia and headache Insomnia or prolonged night wakening Depression Anxiety School absence due to pain
Fibromyalgia Syndrome
What is the management for fibromyalgia syndrome?
Physical therapy
Psychotherapy and relaxation
NSAIDs
Gabapentin
Diagnosis requires __ or 10 major criteria, more than __ of 18 tender points, and pain in at least ___ different areas for more than __ months with normal labs
3 out of 10
5 out of 18
3 different areas
more than 3 months
Genetic disorder in which both “arms (B and T cells)” of adaptive immune system are impaired
“bubble boy disease”
Severe Combined Immunodeficiency (SCID)
Why is there a delay in the detection of SCID?
Newborns carry their mother’s antibodies for the first few weeks of life so infants originally look normal/healthy
8 or more ear infections 2 or more cases of pneumonia infections that do not resolve with antibiotic treatment for 2+ months **Failure to thrive Infections that require IV antibiotic treatment Deep-seated infections Persistent thrust Family hx of immune deficiency
Evaluation for which disease?
Severe Combined Immunodeficiency (SCID)
What is a key characteristic of SCID?
Low antibody levels and lack of specific antibodies after vaccination or natural infection
Treatment for SCID
Enzyme therapy
Gene therapy
**bone marrow transplant
What is a significant risk factor for HSP?
Hypertension
Regulates growth, puberty, reproduction, homeostasis, and energy level
Endocrine system