Pediatrics Flashcards
Albuterol
Short-acting β2-adrenergic receptor agonist used for the relief of bronchospasm in conditions such as asthma and chronic obstructive pulmonary disease. It is marketed as Ventolin among other brand names.
Bulimia nervosa PE finding
enlarged parotid gland
Coombs Test
Test for autoimmune hemolytic anemia (is used to detect if antibodies or complement system factors have bound to RBC surface antigens in vivo)
Prevnar
Pneumococcal vaccine used to protect infants and young children against disease caused by the bacteria Streptococcus pneumoniae (pneumococcus).
Palivizumab/Synagis
monoclonal antibody for RSV
Tacrolimus
Immunosuppressive (IL2 antagonist) drug that is mainly used after allogeneic organ transplant to reduce the activity of the patient’s immune system and so lower the risk of organ rejection. Also treats atopic dermatitis (a skin rash and type of eczema).
Nystatin
An antifungal medication to which many molds and yeast infections are sensitive, including Candida.
Germinal matrix
A fragile portion of the brain that may be damaged leading to an intracranial hemorrhage, which is the most common cause of second trimester spontaneous abortions.
What are the TORCH infections?
Toxoplasmosis Other (syphilis) Rubella Cytomegalovirus (CMV) Herpes simplex virus (HSV)
5 “T”s of Cyanotic Congenital Heart Disease
- Tetralogy of Fallot (TOF)
- Transposition of the Great Arteries (TGA)
- Truncus Arteriosus
- Tricuspid Atresia
- Total Anomalous Pulmonary Venous Connection (TAPVC)
Normal Bilirubin Levels
Total 0.2-1.0
Conj. 0-0.2
Highest Risk Factor of Cerebral Palsy
prematurity (78% of CP pts)
Doxycycline treats…
IN CHILDREN OVER 8, it treats Lyme disease, chronic prostatitis, sinusitis, pelvic inflammatory disease, acne, rosacea, and rickettsial infections. It is contraindicated in children under 8 and pregnant women because it can cause permanent teeth stains.
Ewing’s Sarcoma
Highly malignant tumor found most often in the lower extremity, at the metaphysis and diaphysis of the femur, then tibia and humerus. Typically occurs in white males 0-20 yo. Has on “onion skin/lamellated” appearance.
Osteomyelitis
Presents with fever, joint pain, and swelling. Shows a central lytic bone defect with surrounding sclerosis.
Nursemaid’s Elbow
Also called subluxed radial head. Common elbow injury for 1-5 yo children, occurs when someone pulls upwards on their arm. They will present with pronated elbow and will refuse to move it.
Guillain-Barre Syndrome
An ascending polyneuropathy that usually presents one week after viral infections (like campylobacter jejuni!!!!) involving peripheral motor nerves, and sometimes sensory and autonomic nerves. Symptoms appear from the feet up and disappear in reverse order.
Treat with IVIG and steroids.
Scarlet Fever
Features erythematous SANDPAPER rash on neck, axillae, & groin initially, then spreads (BEGINS ON TRUNK). Blanchable. See Pastia’s Lines of petichiae. Desquamation of skin as rash resolves.
Fever, chills, tender lymphadenopathy.
Pharynx is red and covered in grey-white exudates. Desquamation occurs after a week. Caused by GABHS endotoxin, can follow strep pharyngitis (!!!) or skin infection. Treat with Penicillin or erythromycin.
Sequelae include: rheumatic fever and PANDAS/OCD/tics (preventable), post-strep GN and post-strep arthritis (nonpreventable).
Staphylococcal scalded skin syndrome
Skin condition characterized by superficial flaccid bullae followed by extensive exfoliation of skin. Common in infancy, rare after 5yo.
Herpangina
Throat infection caused by enteroviruses, especially Coxsackievirus, characterized by high fever and severe sore through that may result in an inability to swallow. Ulcerative lesions may be found on palate, tonsils, pharynx. Supportive treatment.
Most common causative organisms of otitis media:
Strep. pneumoniae (40%)
H. flu (25-30%)
Moraxella catarrhalis (10-15%)
About 30% caused by viruses (RSV & rhinovirus)
The rest (~5%) caused by S. aureus, gram-negative bacteria, Group A strep, found mostly in neonates.
Treatment: 10 days of amoxicillin.
Beckwith-Wiedmann Syndrome
Overgrowth Disorder
Path: Disregulation of imprinted gene expression in ch 11p15 (IGF2, insulin-like GF 2)
PE: Fetal macrosomia, rapid growth until late childhood, umbilical hernia, macroglossia, hemihyperplasia
Complications: Wilms Tumor, hepatoblastoma
Surveillance: serum a-fetoprotein, abdominal/renal ultrasound, monitor hypoglycemia in newborns only.
Infectious Mononucleosis
Most likely caused by EBV.
Meningitis neck restriction
Associated with flexion more than extension.
Normal total leukocyte count
4,500-11000 cells/mm3
Encapsulated bacteria
Haemophilus influenzae type b (Hib) Streptococcus pneumoniae (pneumococcus) Neisseria meningitidis (meningococcus) Group B streptococcus (GBS) Klebsiella pneumoniae Salmonella typhi
A popular mneumonic to remember most of the encapsulated bacteria is the SHiNS bacteria (S. pneumo, HiB, N. meningitidis, Salmonella).
Pediatric Strokes
Uncommon, so usually caused by a congenital abnormality, infection, or systemic illness. One of the most common causes is sickle cell anemia.
What does a measurement of serum antineutrophil cytoplasmic antibodies evaluate?
It evaluates for the presence of a vasculitis, a potential cause of stroke in children and young adults.
Lennox–Gastaut syndrome (LGS), also known as Lennox syndrome
A difficult-to-treat childhood-onset epilepsy .
appears between 2-6 yo frequent seizures different seizure types developmental delay psychological and behavioral problems
Niacin/B3 Deficiency
Deficiency of this causes pellagra, which is characterized by diarrhea, dermatitis (painful and looks like a sunburn), and dementia, as well as “Casal’s necklace” lesions on the lower neck, hyperpigmentation, thickening of the skin, inflammation of the mouth and tongue, digestive disturbances, amnesia, delirium, and eventually death, if left untreated. Common psychiatric symptoms include irritability, poor concentration, anxiety, fatigue, restlessness, apathy, and depression.
See in people with bowel disease or in cultures where they eat a lot of corn.
Thiamine/B1 Deficiency
beriberi/Wernike-Korsakoff Syndrome, characterized by neuro and psych symptoms, seen in alcoholics or patients who’ve had weight loss surgery.
Riboflavin/B2 Deficiency
Can lead to cheilosis, glossitis, seborrheic dermatitis (often affecting genital areas), pharyngitis, and edema/erythema of the mouth.
Cyanocobalamin/B12 Deficiency
Causes macrocytic anemia and peripheral neuropathy.
Normal Leukocyte Counts
The reference range for adults (males and females) is as follows:
Total leukocytes: 4.00-11.0 x 109/L Neutrophils: 2.5–7.5 x 109/L Lymphocytes: 1.5–3.5 x 109/L Monocytes: 0.2–0.8 x 109/L Eosinophils: 0.04-0.4 x 109/L Basophils: 0.01-0.1 x 109/L
Intususception
This GI disease may present in an infant or a young child with EPISODIC abdominal pain (pull legs up to chest), irritability, vomiting, red currant jelly stools, and an abdominal mass. Diagnose with a air or barium enema.
See “target sign” on ultrasound.
Common in kids with HSP.
Meckel’s Diverticulum
Typically presents with PAINLESS melena/tarry/maroon stools in 2-3 yo children. It is the most common anomaly of the GIT and it results from the failure of the vitelline duct to obliterate during fetal development. Gastric tissue may be present, which results in ulcerations and bleeding. Diagnosis made with technetium-99m pertechnetate scanning (uptake by heterotropic gastric mucosa).
Normal IQ Range
Mean for the population: 100.
2 SD below the mean: 70
Intellectual Disability:
Mild – from between 50 and 55 to approximately 70
Moderate – from between 35 and 40 to between 50 and 55
Severe – from between 20 and 25 to between 35 and 40
Profound – from <20 to 25
Fragile X Syndrome
Increased number of CGG trinucleotide repeats, mutation in the FMR1 gene. Characteristic appearance: large head, long face, prominent forehead and chin, large ears, large testicles.
Behavior: Hyperactivity, autism, temper tantrums. Most commonn inherited form of mental retardation.
Low to normal IQ, language disability.
Fetal Alcohol Syndrome
Mild to moderate mental retardation, hypo-plastic maxilla, long philtrum, microcephaly.
Reye Syndrome
A rare illness seen exclusively in children less than 15 yo who were treated with salicyclates/aspirin for a viral infection. Present with vomiting, agitation, irrational behavior, lethargy, stupor, restlessness, possibly convulsions.
ASPIRIN IN CONTRAINDICATED IN YOUNG CHILDREN WITH VIRAL INFECTIONS.
Labs: hyperammonia, slightly elevated bili and alk phos, prolonged PT time, hypoglycemia, and moderate to severe elevations in ALT, AST, and lactate dehyd. levels.
Biopsy of liver, kidneys and brain reveals microvesicular steatosis. Treatment is supportive.
Systemic Carnitine Deficiency (SCD)
Inherited defect in FA oxidation. Clinical features: hypoglycemia, hypoprothrombinemia, acute episodes of encephalopathy.
Clubbed Fingers
CF!
Gram Positive Diplococci
Streptococcus pneumoniae
Gram Positive Cocci
Staphylococcus spp.
Gram Negative Cocci
Neisseria
Gram Positive Rods
Listeria, Bacillus cereus and anthracis
Gram Negative Rods
Pseudomonas, Haemophilus, Klebsiella, Legionella
CMV pneumonia
Pneumonia that occurs in immunocompromised pts with CD4<50.
Pneumonia in immunocompromised hosts with CD4<200
Pneumocystis jiroveci, Cryptococcus neoformans, Histoplasma capsulatum, CMV (CD4<50)
3 most common causative organisms in CF pneumonia
Haemophilus influenza
Pseudomonas a.
Staphylococcus
Fancy word for “a scratch”
excoriation
Congenital CMV or Toxo Infection S&Sx
Developmental delay, IUGR (including microcephaly), cataracts, seizures!!!, HSM, prolonged neonatal jaundice, and purpura at birth. Calcified brain densities in periventricular area (CMV) or diffusely throughout cortex (Toxo)
Down Syndrome/Trisomy 21 features
Endocardial cushion defect or ASD, hypotonia with poor Moro reflex, flat face, slanted palpebral fissures, laxity of joints/flexible (esp. atlantoaxial instability), excessive skin on the back of neck, cardiac defects, duodenal atresia, extra space between first two toes
Edward’s Syndrome/Trisomy 18 features
Features: Clenched hand, with overlap Intrauterine growth retardation (IUGR) Rocker bottom feet Micrognathia, prominent occipital, micro-ophthalmia Low set ears Cardiac defects
More: Weak cry, single umbilical artery, SGA, low-set malformed ears, microcephaly, rocker-bottom feet, inguinal hernias, cleft lip/palate, micrognathia. Also clenched hands with overlapping digits, small palpebral fissures, prominent occiput, short sternum, cardiac defects (VSD, ASD, PDA, or aortic coarctation).
Patau Syndrome/Trisomy 13 features
Features:
Holoprosencephaly
Polydactyly
Microcephaly
Midline Cleft Lip/palate
Cutis aplasia ("punched out lesions" of the scalp)
More: SGA, dysmorphic, microcephaly, sloping forehead, deafness, cutis aplasia, polydactyly micropthalmia, omphalocele. Also holoprosencephaly, cleft lip, polydactyly, overlapping fingers, coloboma, omphalocele, and cardiac defects (VSD, ASD, PDA, dextrocardia).
Conditions associated with asthma
GERD, eczema, sleep-disordered breathing, allergic rhinitis, sinusitis
albuterol
Short-acting beta-2 agonist (bronchodilator)
Low birth weight (LBW)
birth weight < 2,500 g (5 lbs 8 oz)
Very low birth weight (VLBW)
birth weight < 1,500 g (3 lbs 5 oz)
Conditions associated with prematurity
hypoglycemia, intraventricular hemorrhage, respiratory distress syndrome, pneumonia,h transient tachypnea of the newborn, patent ductus arteriosus, persistent fetal circulation, necrotizing enterocolitis, hyperbilirubinemia, kernicterus, cerebral palsy
Conditions associated with high dose O2 given to neonates
pulmonary fibrosis, bronchopulmonary dysplasia, retrolental fibroplasia
Henoch Schonlein Purpura
A systemic vasculitis characterized by deposition of IgA immune complexes, exact etiology unknown.
Often preceded by URI
Palpable purpura on lower extremities and butt
joint and abdominal pain (*Can develop intussusception or GI hemorrhage!!)
renal disease (IgA deposition)
SCROTAL PAIN and SWELLING
Normal platelet count. Treatment is supportive.
technetium-99m pertechnetate
Uptake by heterotropic gastric mucosa to diagnose Meckel’s Diverticulum
Continuous flow murmur
Patent Ductus Arteriosus (high pressure aorta to low pressure pulm artery). Sounds like machinery.
Klumpke Palsy
“Claw hand”: Extended wrist, hyperextended MCP joints, flexed prox and distal interphalangial joints, absent grasp reflex. Also miosis and ptosis (Horner Syndrome).
Due to shoulder dystocia and subsequent traction of C8 and T1 nerves during delivery. Risks: LGA, maternal diabetes, maternal obesity.
Erb’s Palsy/Duchenne Palsy
Injury to C5-C6
Decreased moro and biceps reflexes on affected side, “Waiter’s tip” (extended elbow, pronated forearm, flexed wrist and finger), INTACT grasp reflex.
Enuresis treatment medication
DDAVP (desmopressin, a vasopressin mimetic) or imipramine
Haloperidol uses
Tourette syndrome
Schizophrenia
Clonidine drug use
Centrally acting alpha-agonist that is used to control blood pressure.
Friedreich Ataxia
Autosomal recessive condition characterized by excessive trinucleotide repeats resulting in an abnormal tocopherol transfer protein. Degeneration of nerve tissue in the spinal cord, in particular sensory neurons essential (through connections with the cerebellum) for directing muscle movement of the arms and legs.
Progressive disease with poor prognosis. Death at 30-35 years old. 90% develop myocarditis and die of this.
Parents need genetic counseling.
When are kids able to copy a cross, circle, square, rectangle, triangle and a diamond?
3 years: cross, circle
4 years: square, rectangle
5 years: triangle
6 years: diamond
Most common peds osteomyelitis causing organisms
Staphylococcus aureus (most common)
Others are GBS and E coli in infants, Strep pyo in children.
Osteomyelitis in kids with sickle cell is usually caused by…
Salmonella
Cervical Lymphadenitis etiologies
- Bacterial infection (S. aureus, S. pyogenes, TB, Myco. avium, B. henselae)
- Reactive lymphadenitis
- Viral Infections (EBV, CMV, HIV)
- Kawasaki Disease
- T. gondii
- Structural lesions on neck
Kawasaki Disease
An acute febrile illness of childhood or unknown origin. Most common cause of acquired heart disease in children in the US. M:F 3:2, most common in Asians, mean age is 18-24 mo.
Features: fever >5d, bilateral conjunctivitis with limbic sparing (no exudate), pharyngitis, strawberry tongue, red cracked swollen lips, unilateral cervical lymphadenitis, rash on trunk, edema of the hands and feet, (later) peeling around nailbeds.
Concern for coronary artery aneurisms (20%).
Labs: high ESR and CRP. LFTs can be elevated.
Treatmt: IVIG & aspirin within 10 days for start of fever.
T. gondii Infection
Intracellular parasite transmitted through direct contact with cat feces, undercooked meats, fruits or veggies, transplacental passage. Causes a mononucleosis like illness with cervical lymphadenopathy (malaise, fever, sore throat, myalgias, lymphadenopathy, rash, HSM).
Diagnose with antibody titers against protozoa.
Important pathogen of immunosuppressed people, like in HIV. Can present with focal seizures. Treat only immunocompromised, pregnant women, and newborns. Sulfadiazine and pyrimethamine.
Bartonella henselae
“Cat scratch disease” is caused by the gram-negative B. henselae.
Regional lympadenopathy (axillary, cervical or inguinal) after a cat/kitten bite/scratch. Results in a papule along the scratch and lympadenopathy 1-2 weeks later. Lymphnode is red, warm, and tender.
Diagnose with IgM ab. to B henselae. Treat with supportive care.
Parotitis (swollen parotid salivary gland) causes
- Mumps virus & other viruses (CMV, EBV, HIV, influenza) - bilateral involvement
- Bacterial infection (Staph. aureus, Strep. pyo/GABHS, TB). Uncommon in children. UNILATERAL involvement
Post-streptococcal Glomerulonephritis
Present with hypertension and cola-colored urine.
Seen after streptococcal pharyngitis, impetigo/SSSS, Scarlet fever
Antibiotic therapy DOES NOT prevent this complication.
PANDAS (Ped. Autoimmune Neuropsychiatric Disorders associated with Streptococcal infection)
Phenomenon in which patients develop the acute onset of OCD symptoms or a tic disorder after strep infection. Antibiotic therapy DOES prevent this complication.
Treat PANDAs with OCD therapy–Fluoxetine SSRI (Prozac)
IVIG
A blood product administered intravenously. It contains the pooled, polyvalent, IgG antibodies extracted from the plasma of over one thousand blood donors. IVIG’s effects last between 2 weeks and 3 months.
Used to treat: Allogeneic bone marrow transplant Chronic lymphocytic leukemia Many primary immunodeficiencies Idiopathic thrombocytopenic purpura Pediatric HIV Kawasaki disease Chronic inflammatory demyelinating polyneuropathy (CIDP)
Differential for bloody stools in a 1 month old:
Infectious colitis Diaper rash or anal fissure Sign of sepsis/infection Bleeding disorder Milk protein intolerance
Mid-Parental Height
Boys: [(Mom’s H + 13 cm) + Dad’s Height] /2
Girls: [(Dad’s H - 13 cm) + Mom Height] /2
13 cm = 5 in
How many calories in 1 oz of formula?
20 kcal
Still’s Murmur
Physiologic murmur heard in 30-50% of normal children aged 3-8 yo. Should disappear at puberty.
Peripheral Pulmonic Stenosis
Physiologic: turbulence in the pulmonic vasculature in newborns. Should disappear by 6-12 months of age. Murmur is louder in the back and axillae than in the precordium.
Pathologic: Can be caused by Williams syndrome (supravalvular aortic stenosis) or Alagille syndrome
Meningitis in a 6 year old: likely orgs, treatment
Orgs: pneumococcus, meningococcus (N. meningitides)
Abx: vancomycin, cefotaxime (3rd gen cef)
Pathogens causing URI then arthritis
S. aureus, Group A Strep, Neisseria if sexually active.
Start vanc or clinda
Another name for Strep pyogenes
Group A Strep, GABHS
Turner syndrome
45X. Appearance: Short stature, widely spaced nipples, webbed neck/excessive nuchal skin, swollen hands and feet.
Ovarian failure, coarction of the aorta, horseshoe kidney, recurrent acute otitis media, sensorineural hearing loss, HTN due to renal anomalies, autoimmune disease (thyroid, Celiac), dysgenesis of lymphatic system (causing edema), nail dysplasia.
When to suspect hypothyroidism in children:
GROWTH FAILURE!!!!!! (most important) Large tongue Umbilical hernia APATHY, Sluggishness, fatiguability Hypercholesterolemia Obesity
Diagnostic evaluation of precocious puberty
Check LH, FSH, androgens, progesterone, estradiol.
Bone age, Lupron (GnRH) stimulation test, pelvic ultrasound.
McCune-Albright Syndrome (MAS)
peripheral precocious puberty
café-au-lait skin pigmentation
fibrous dysplasia of bone (polyostotic fibrous dysplasia)
More common in girls than in boys. Affected girls overproduce estrogens, boys-androgens.
Endocrine DiffDx for Obesity
Cushing’s Disease
Hypothyroidism
Growth hormone deficiency
Endocrine etiologies
Lesch-Nyhan Syndrome
X-linked recessive disorder (so only males can get it) resulting from a deficiency in HPRT enzyme (hypoxanthine-guanine phosphoribosyl transferase) which is involved with purine metabolism. This leads to increased levels of uric acid. Presents around 6 months with hypotonia and persistent vomiting. Develop mental retardation, spasticity, dystonia, gouty arthritis and compulsive self injury, especially biting of the upper extremities.
Osteogenesis Imperfecta
Defect in type 1 collagen. Type II OI is the most severe form and characteristic findings include blue sclerae, multiple fractures causing limb deformity, and IUGR.
Is premature pubarche benign?
No, it is associated with a CNS disorder in 50% of cases and requires a thorough evaluation.
What is the most common sequelae associated with Kawasaki disease?
Coronary Artery Aneurism. Prevent this by administering aspirin and IVIG within 10 days of the start of symptoms. Cardiac follow up 2 weeks after disease.
Burkitt Lymphoma
Cancer of mature B cells, associated with EBV.
Acute Myelocytic Leukemia (AML)
Predominant cancer cells are of myeloid origin. Auer rods are specific for this.
Parinaud’s syndrome
Presents with paralysis of vertical gaze that may be associated with pupillary disturbances and eyelid retraction (Collier’s Sign). The lesion is most commonly germinomas and pinealomas at the rostral midbrain near the superior colliculus and CNIII. The endocrine syndrome results from interruption of hypothalamic inhibiting pathways and sometimes hCG analogue secretion → Leydig cell stimulation → precocious puberty.
Medulloblastoma arises from the ___ 90% of the time.
Cerebellar vermis
Fancy way of saying “clubfoot”
talipes equinovarus, equinus and varus of the calcaneum and talus
Nikolsky’s sign
Slight rubbing of the skin results in exfoliation of the outermost layer, forming a blister within minutes.
Sign is almost always present in toxic epidermal necrolysis (TEN), and is associated with pemphigus vulgaris and Scalded Skin Syndrome (caused by the exfoliative toxin of S. aureus).
Pharyngitis etiologies
All viruses associated with URIs
Coxackievirus, EBV, CMV
Bacterial causes: S. pyogenes/GABHS/Strep throat, diptheria
GABHS pharyngitis
Seen in school age children 5-15 yo in the winter and spring.
There is a LACK of other URI symptoms, exudates on the tonsils, petechiae on soft palate, strawberry tongue, enlarged cervical lymph nodes.
Impetigo
A superficial skin infection affecting the upper dermis, usually caused by S. aureus, but can also be caused by S. pyo. Features: honey-colored crust around the nares, fever absent, very contagious. Treat with topical mupirocin or oral -cillins, third gen. cephalosporins, or clindamycin.
Complications include: bacteremia, post-strep glomerulonephritis (treatment does not prevent this), and SSSS.
Erysipelas
Skin infection usually caused by GABHS (80%)
involves the dermal lymphatics
tender, shiny, erythematous skin with a distinct border
Commonly face and scalp
Treat with systemic abx against GABHS.
Complications include bacteremia, post-strep glomerularnephritis, and necrotizing fasciitis.
Cellulitis
A skin infection that occurs within the dermis, usually caused by S. aureus or GABHS. Infection is usually caused by a break in the skin barrier. Features: erythema, warmth and tenderness with an INDISTINCT border. Treatment with abx directed at the typical causative agents, with third gen cephalosporins or penicillins.
Buccal Cellulitis
Unilateral bluish discoloration on the cheek of a young unimmunized child caused by HIB. High rate of concomitant bacteremia and meningitis.
Perianal cellulitis & constipation caused by…
GABHS
Staphylococcal Scalded Skin Syndrome (SSSS)
Features: fever, tender skin, bullae, sheets of skin slough off days after beginning of illness. Caused by S. aureus species that produces an exfoliative toxin. Nikolsky sign present (extension of bullae when pressure is applied to the skin).
Treat with IV abx against S. aureus.
How do you remember how to diagnose Kawasaki Disease?
“Crash & burn”
- Conjunctivitis (bilateral, non purulent)
- Rash ( truncal, non-specific)
- Adenopathy (at least 1 cervical node > 1 cm)
- Strawberry tongue (or any change in oropharyngeal mucosa)
- Hand, feet swelling/desquamation.
Fever (burn) for > 5 days and four or five of the parameters above.
Rickettsial Infections (3)
Lyme Disease (Borrelia burgdorferi)
Rocky Mountain Spotted Fever (Rickettsia rickettsii, gram negative)
Ehrlichiosis (Ehrlichia chaffeensis)
Rocky Mountain Spotted Fever (RMSF)
Caused by Rickettsia rickettsii, a gram negative intracellular coccobacillus that is transmitted by tick bites, mostly in the SE United States in the spring and summer.
Features: fever, abdominal pain, petechial rash (begins on lower extremities and moves to hands and then trunk and head), palmar rash, myalgias, HSM & jaundice, CNS sx like headache!!!!, hypotension.
Labs: Thrombocytopenia, hyponatrimia, possible aseptic meningitis.
Treat with oral or IV doxycycline (or amoxicillin if under 8 yo) and supportive care.
Scrofula/Mycobacterial cervical lymphadenitis
A lymphadenitis of the cervical lymph nodes associated with tuberculosis as well as non tuberculous (atypical) mycobacteria. In immunocompetent children, scrofula is often caused by atypical mycobacteria (Mycobacterium scrofulaceum) and other nontuberculous mycobacteria (NTM). Unlike the adult cases, only 8% of cases in children are tuberculous.
Features: Chronic, painless neck mass that grows. No local color or warmth. Overlying skin acquires a violaceous (bluish-purple) color. As lesion progresses, skin becomes adhered to the mass and may rupture, forming a sinus and an open wound.
Treatment: Surgical excision without antimicrobial therapy. If refractory, treat with a macrolide (clarithromycin or azithromycin) in combination with another agent.
Wiskott-Aldrich Syndrome (WAS)
a rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhea (secondary to the thrombocytopenia). It is also sometimes called the eczema-thrombocytopenia-immunodeficiency syndrome. Initial manifestations at birth consist of petechiae, bruises, bleeding from circumcision or bloody stools.
Not enough platelet PRODUCTION, and the platelets are small.
Combined B and T cell disorder, recurrent infections with encapsulated organisms.
Chediak-Higashi Syndrome
A rare autosomal recessive disorder that arises from a mutation of a lysosomal trafficking regulator protein, which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections (usually S. aureus), partial albinism, peripheral neuropathy, and HSM. Findings of neutropenia and giant lysosomes in neutrophils will confirm the diagnosis. Treatment is with daily trimethoprim/sulfamethoxazole and daily ascorbic acid.
Leukocyte adhesion defect (LAD) syndromes
Syndromes that result from failure of innate host defenses against bacteria, fungi and other microorganisms due to defective tethering, adhesion, and targeting of myeloid leukocytes to sites of microbial invasion. The hallmark of the disease is neutrophilia WITHOUT polymorphs in the infected tissue or pus. Pts will typically have a history of delayed separation of the umbilical cord, recurrent bacterial infections, necrotic skin lesions, severe gingivitis, periodontitis, and alveolar bone loss leading to early loss of teeth.
Hyper-IgE Syndrome (Job’s)
Syndrome characterized by chronic pruritic dermatitis, recurrent staph infections of the skin and URT, markedly elevated serum IgE levels, eosinophilia and coarse facial features. Recurrent staph infections involving the skin, lungs, and joints, with other features such as distinctive facial appearance, dental abnormalities, and bone fractures are essential for the diagnosis.
What is the most common predisposing factor for acute bacterial sinusitis?
A viral upper respiratory infection
What is topical erythromycin opthalmic ointment good for prophylactically treating? What does it not work against?
Topical erythromycin (and silver nitrate solution) is only effective for gonococcal prophylaxis (GC shows up at 2-4 days of life). If needed, treat with IV cefotaxime.
Only prenatal maternal testing and treatment are effective for the prevention of neonatal chlamydial infections (4-10 days of life). Discharge may be blood stained. Treat affected infant with oral erythromycin.
Osteonecrosis (Avascular necrosis)
A common complication of sickle cell anemia secondary to red cell sickling, microinfarctions, and bone hyperplasia. The most common sites are the humeral and femoral heads. Treatment is pain management and limitation of weight bearing with surgical intervention if conservative management is unsuccessful.
Charcot arthritis
Neuropathic arthropathy. Progressive degeneration of a weight bearing joint, a process marked by bony destruction, bone resorption, and eventual deformity, resulting from deterioration of proprioception, pain sensation, and temperature sensation. Onset is usually insidious/gradual.
Neuroblastoma (NBL)
The most common pediatric cancer in the first year of life. The median age at diagnosis is 2 years. The tumor arises from neural crest cells from the sympathetic chain or the adrenal medulla. The tumor is usually firm and nodular, and it frequently crosses the midline with systemic symptoms. The levels of serum catecholamines and their metabolites (homovanillic acid and vanillylmandelic acid) are usually elevated. See calcifications and hemorrhages.
homovanillic acid and vanillylmandelic acid
Catecholamine metabolytes
Metanephros gives rise to…
Renal parenchyma (Wilms tumor can arise from this)
Mesonephros gives rise to…
Seminal vesicles, epididymis, ejaculatory ducts, and ductus deferens
Paramesonephron gives rise to…
Fallopian tubes, uterus, and part of the vagina.
Pyloric Stenosis
Usually presents between 3 and 12 weeks of age with nonbilious, nonbloody, projectile vomiting.
Treatment for absence seizures
Ethosuximide or valproic acid (valproate)
Treatment for partial seizures
Phenytoin or Gabapentin
Is Staph aureus resistant to penicillin?
Yes, >99% of S. aureus is resistant to penicillin. Use clindamycin or vancomycin.
Treatment for facial impetigo
Topical mupirocin (or oral erythromycin if it’s bad)
In what ways can diabetes evoke an immunodeficient state?
Diabetes Mellitus can evoke an immunodeficient state when you have lots of hyperglycemia. The hyperglycemia promotes neutrophil dysfunction, and circulatory insufficiency contributes to ineffective neutrophil chemotaxis during infection.
LAD/leukocyte adhesion deficiency
Disorder of leukocyte adherence
Recurrent sinopulmonary, oropharyngeal, and cutaneous infections
Delayed wound healing (delated umbilical detachment, gingivitis)
Neutrophilia, high WBC >50,000
ABSENCE of neutrophils from infection sites!
Life-threatening infection is possible with Staph spp., Enterobacteriaceae, and Candida spp.
Severe Combined Immunodeficiency (SCID)
An autosomal recessive or X linked disorder of both humoral and cellular immunity. Serum immunoglobulins and T cells are often markedly diminished or absent. Thymic dysgenesis is also seen. Recurrent cutaneous, GI, or pulmonary infections occur with opportunistic organisms like CMV and PCP. Death usually occurs in the first 2 years of life unless a curative bone marrow transplant is performed.
DiGeorge Syndrome
Syndrome caused by a 22q11 microdeletion, leading to immunodeficiency.
CATCH-22 mnemonic: Cardiac Abnormality (especially tetralogy of Fallot) Abnormal facies Thymic aplasia Cleft palate ***Hypocalcemia***/Hypoparathyroidism
Also: decreased T cell production (IMMUNODEFICIENCY) recurrent infection VSD and Tetralogy of Fallot Thymic or parathyroid dysgenesis hypocalcemia seizures Developmental and speech delay
What is the most common inherited form of mental retardation?
Fragile X Syndrome
Klinefelter Syndrome
XXY male. Features mild mental delay, gynecomastia, delayed puberty, sterility, increased rates of breast cancer, small testes and phallus.
Goat-milk diet: nutritional concerns
Can have folate or B12 deficiency leading to megaloblastic anemia. It must also be boiled before ingestion, as goats are susceptible to brucellosis.
Normal total bili (excluding newborns)
Less than 1.2 mg/dl
Choledochal cyst
A congenital abnormality of the biliary ducts characterized by the dilation of intra and/or extrahepatic biliary ducts or both. It presents in children with abdominal pain, jaundice and attacks of recurrent pancreatitis (may increase amylase and lipase levels). They will have epigastric RUQ pain. These cysts can degenerate into cholangiocarcinomas.
Cholesteatoma
a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. It can be congenital or acquired. You can note granulation tissue and skin debris within retraction pockets of the tympanic membrane. Should be suspected in any patient with continued ear drainage for several weeks despite appropriate antibiotic therapy. Complications include hearing loss, cranial nerve palsies, vertigo, and severe infections.
Normal ESR Ranges
Newborn: 0 to 2 mm/hr
Neonatal to puberty: 3 to 13 mm/hr
Normal CRP Value
<0.8 mg/dL)
Diagnostic Criteria for TSS
Causative Agents: S. aureus, less common is GABHS.
- Fever > 38.5 (101)
- Hypotension
- Diffuse macular erythroderma, like a sunburn
- Desquamation 10-14 days after illness onset.
- Multisystem involvement (>3 of the following: GI, myalgias, hyperemia of mucus membranes, sterile pyuria, thrombocytopenia, CNS altered consciousness)
- Negative cultures of blood, CSF, pharynx, OTHER than blood culture for S. aureus.
Viral diarrhea
Rotavirus (4-7 days, self limiting, supportive care) Norwalk virus (2-3 days, daycares/cruises/etc., supportive care)
Diarrhea causes what kind of metabolic derangement?
non-anion gap hyperchloremic metabolic acidosis, as a result of bicarbonate loss in the stool
Which organism causes hemolytic uremic syndrome?h
Enterohemorrhagic E. coli (EHEC), via endotoxin release. If HUS is present, DON’T use antibiotics.
Bloody diarrhea differential
Shigella, Salmonella, Campylobacter (most common)
At what age is a negative HIV-specific DNA PCR definitive for not having congenitally-acquired HIV?
4 months
Early symptoms of HIV infection
FTT thrombocytopenia recurrent infections (otitis media, pneumonia, and sinusitis) lymphadenopathy parotitis recurrent, difficult to treat Thrush loss of developmental milestones severe varicella infection or zoster
Which vaccines CAN’T an HIV positive child get and why?
They cannot get the varicella vaccine or the MMR vaccine (well, some can). This is because the vaccines are live.
Common infections of HIV+ persons
PCP
M. avium complex (MAC)
Fungal: Candida, cryptococcus, histoplasmosis, coccidioidomycosis, aspergillosis.
Viral: CMV, HSV, VZV
Parasites: Toxoplasmosis, cryptosporidium
Lymphoma (especially B cell, caused by EBV co-infection)
Major etiologic agent of mononucleosis
EBV, transmitted by saliva and infects the B lymphocyte.
EBV infectious mononucleosis symptoms
Fever, may last up to 2 weeks Malaise and fatigue Pharyngitis (typically exudative, resembles GABHS) Posterior cervical lymphadenopathy HSM (80%) macular or scarlatiniform rash
Diagnose with monospot test (same thing as heterophile antibody test)
Sx resolve in weeks to months
How do you test for EBV infectious mononucleosis?
Monospot in children >4 yo. Monospot measures the presence of heterophile antibody, with a sensitivity of 85%.
For children <4 yo., use EBV antibody titers.
EBV Infectious Mononucleosis treatment
Supportive
A patient you think has GABHS pharyngitis is administered amoxicillin and breaks out in a diffuse pruritic maculopapular rash. What has happened?
This patient has an EBV infection and was misdiagnosed. We don’t know why, but patients who have EBV infectious mononucleosis who are mistakenly administered amoxicillin break out in this rash. This is not an allergic reaction.
