CLIPP 5/4/17 Flashcards
Opsoclonus-myoclonus syndrome
paraneoplastic syndrome that occurs most often with neuroblastoma in a young child who presents with ataxia and jerking or erratic movements as well as jerky conjugate movements of the eyes.
Post-infectious cerebellitis
pres
path
typically presents in a younger child with ataxia, nystagmus, vomiting and sometimes dysarthria
believed to be an autoimmune response leading to demyelination of the cerebellum occurring several weeks after a viral infection such as varicella or coxsackie virus
. Headaches of new onset that do not go away and are worse in the mornings and associated with vomiting and personality changes would be most concerning for
brain tumor etiology
headache that should be responsive to nsaids
Tension-type headache
4yo child presents withCC fatigue, evidence of anemia (fatigue, tachycardia, pallor) and thrombocytopenia (unexplained bruising)
you should suspect…
ALL
Failure of two or more hematologic cell lines should always raise suspicion for malignant invasion of the marrow. Furthermore, the child’s chief complaint, fatigue, is the most common presenting symptom of acute leukemia. Finally, the incidence of ALL peaks at age 4 years.
HSP etiology peak age and range, gender signs and symptoms important to labs at follow-up
exact etiology of HSP is unknown, but it is believed to involve an IgA-immune-mediated vasculitis in response to infection or other triggers
peak at ages 4 to 6 years (range of 2 to 17 years)
M:F 2:1
characterized by a rash consisting of petechiae and palpable purpura. Other findings include a colicky diffuse or periumbilical abdominal pain, arthritis or arthralgia, and renal disease
UA, BP, BUN Cr: Given the incidence of renal disease, it is important to check the urine for signs of hematuria or proteinuria; sudden changes in blood pressure can potentially suggest a change in renal function. With abnormal findings, serum BUN and creatinine must be checked
platelet count in HSP
normal, hallmark rash is a NON-THROMBOCYTOPENIC PURPURA
Idiopathic thrombocytopenic purpura (ITP) is part of the differential for palpable purpura and petechiae. ITP is characterized by low platelet counts (usually < 20,000) with normal WBCs and hemoglobin. Should you find low platelets, you may want to reconsider the differential and think about ITP or possibly leukemia.
CBC in ITP
low platelet counts (usually < 20,000) with normal WBCs and hemoglobin
PT/PTT in HSP
normal
not a coag disorder, an IgA-immune-mediated vasculitis
but coag disorder on the ddx for palpable purpura and petechaie so get PT/PTT if dx uncertain
Peds leukemia
path
signs and symptoms
labs to get if suspected
bone marrow infiltration and cytopenia,
abnormalities in the different cell lines (WBC, RBCs and platelets)
petechia and purpura, bone pain, fever, fatigue, malaise, hepatosplenomegaly, and lymphadenopathy
When leukemia is suspected, it is important to measure WBCs, Hgb and platelets
intracranial hemorrhage is a potential but rare complication of which ddx for petechiae and palpable purpura
intracranial hemorrhage is a potential but rare complication of idiopathic thrombocytopenic purpura (ITP). The incidence of ICH in patients with ITP is 0.1 to 0.5% of cases
labs to get when working up palpable purpura and petechiae, according to ddx
HSP - BP, UA, BUN Cr if the first two abnormal, because of frequent renal involvement
ITP - CBC - low platelet counts (usually < 20,000) with normal WBCs and hemoglobin
Coag disorder - PT/INR
Leukemia - CBC - bone marrow infiltration and cytopenia
tf
hsp is characterized as a small vessel vasculitis
HSP is classified as a small vessel vasculitis. The exact mechanism of HSP is unknown; however, it is thought to be an IgA-mediated immune response affecting small vessels (skin, GI tract, joints, kidneys). Approximately 50% of cases follow viral or bacterial URIs. Biopsy of affected organs shows leukocytoclastic vasculitis with IgA deposition
biopsy of affected organs in HSP shows
leukocytoclastic vasculitis with IgA deposition
Approximately 50% of cases of HSP follow what kind of preceding illnesses
viral or bacterial URIs
UA in HSP
follow-up labs
UA in HSP will often reveal mild hematuria and/or proteinuria. If either are present, BUN and Cr should be ordered to further evaluate the extent of renal involvement.
HSP tx time to resolution recurrence rate give IVIG?
treatment of HSP is symptomatic and supportive (e.g., NSAIDs for joint pain). HSP usually resolves within four to six weeks and has a 30% recurrence rate. IVIG is not indicated in patients with HSP; it is, however, a treatment option for ITP
what % HSP pts have elevated serum IgA
50%
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HSP pts have GI bleeding
T
Since IgA immune complexes in HSP also attack the GI vessels, around 67% of patients have GI bleeding, either occult blood or grossly bloody stool
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HSP pts have abdominal pain
T
Abdominal pain occurs in 50 to 75% of HSP patients, as a result of the immune attack on the GI vessels, as described above
tf
HSP pts have arthralgias
T
Arthralgias, mainly of the knees and ankles, are seen in about 75% of children with HSP
___ are often the delineating finding between HSP and ITP
Decreased platelets (ITP)
hallmark rash in HSP is a NON-THROMBOCYTOPENIC PURPURA
rash description in HSP
erythematous, slightly raised, non-blanching, maculopapular rash over the legs, buttocks, and posterior portion of the elbows
aka petechiae and palpable purpura
tf
observation is appropriate in this patient: 8yo, joint pain, erythematous, slightly raised, non-blanching, maculopapular rash over the legs, buttocks, and posterior portion of the elbows, normal CBC, Skin biopsy shows leukocytoclastic vasculitis with IgA deposition
T
treatment of HSP is symptomatic and supportive (e.g., NSAIDs for joint pain). HSP usually resolves within four to six weeks and has a 30% recurrence rate
fitz-hugh-curtis syndrome
define
symptoms
rare complication of pelvic inflammatory disease (PID) involving liver capsule inflammation leading to the creation of adhesions. abdominal pain would be felt in the right upper quadrant and may be referred to the right shoulder if the peritoneum becomes irritated
___ is the most sensitive and specific lab test to diagnose pancreatitis.
Lipase
fever and vomiting in the setting of a uti makes you think
pyelonephritis
tf
pelvic inflammatory disease requires inpatient treatment
depends
may be managed with abx outpatient if low risk… but if eg pregnant manage inpatient, or homeless and higher risk of not completing abx course, manage inpatient
is ovarian torsion more common pre or post menopausal
Ovarian torsion is more common in the post-menopausal population, though it can present in any age group
things that would make you lean ovarian torsion instead of appendicitis on history and physical
pain is intermittent
palpable ovarian mass (inc risk of torsion)
postmenopausal (more common than premenopausal)
temporality of pelvic inflammatory disease pain
often post-coital and also first occurs during or immediately following menstruation
cervical motion tenderness think
pelvic inflammatory disease
tf
pelvic ultrasound to dx PID
F for initial dx
can initiate treatment based on H&P without further diagnostic workup
but can consider pelvic ultrasound if ovarian torsions were suspected, or if her symptoms were refractory to treatment, suggesting a tubo-ovarian abscess that could be detected by ultrasound
Cervical discharge in PID should be tested for
should be tested for gonorrhea and chlamydia and sent for culture
tf
infant bloody stools and poor weight gain put milk protein allergy on the ddx
T
but
most common symptoms include diarrhea, vomiting, abdominal pain, and allergic reactions ranging from urticaria to anaphylaxis
gastroenteritis or intussusception, or other intestinal anomaly on ddx for bloody stool too
milk protein allergy more common in breast milk or formula?
more common with formula
less common with breast milk, but still occurs
what signs and symptoms suggest CHF as cause of failure to thrive
tiring with feeds, sweating, or tachypnea, tachycardia, or hepatomegaly
most common cause of failure to thrive
inadequate caloric intake
most common symptoms of milk protein allergy
most common symptoms include diarrhea, vomiting, abdominal pain, and allergic reactions ranging from urticaria to anaphylaxis
Stool culture with Wright stain for WBC’s could be useful in a patient with diarrhea if you suspect
an infectious cause of the diarrhea
TF
9wk old baby with steatorrhea and poor weight gain but normal newborn screen at birth should have CF on the ddx
T
newborn screens not completely standardized across states, CF may have been missed
inheritance of CF
autosomal recessive
tf
most peope with CF have a positive family history
f
autosomal recessive inheritance
tf
gene therapy is a current treatment option for CF
f
not yet
Current treatment includes a multidisciplinary approach - pancreatic enzyme and vitamin replacement, mucus clearing, possibly transplant, and more
kernicterus due to hyperbilirubinemia is associated with what type of cerebral palsy
kernicetrus is associated with dyskinetic CP (basal ganglia pathology on imaging)
dyskinetic cerebral palsy is associated with what newborn issues, what neural structures, what symoptoms
perinatal asphyxia involving the thalamus and cerebellum
kernicterus from unconjugated hyperbilirubinemia affecting the basal ganglia
motor abnormalities throughout the body
spastic diplegia
assoc w what newborn issue,
what neural structures, what
symptoms
spastic diplegia is classically associated with premature birth and specific MRI findings of periventricular white matter abnormalities. Patients present with motor involvement that is more prominent in the legs than the arms
spastic quadriplegia
symptoms’
neural abnormalities
spasticity, clonus, and exaggerated tendon jerks throughout their bodies
Imaging would show global brain abnormalities
spastic hemiplegia
typical presentation
after a stroke damaging a unilateral upper motor neuron tract
spasticity of the contralateral arm and leg
ataxic cerebral palsy
what neural abnormality
cerebellar abnormalities
18 month old not walking. physical exam reveals spasticity, exaggerated deep tendon reflexes, and clonus in both of his lower extremities. An MRI of the brain is ordered, and the radiologist reports findings of periventricular leukomalacia. What is the most likely diagnosis?
spastic diplegia
Neimann-Pick disease is a _________ disease that can present in children between the ages of _ months and _ years. It causes _____ delays as well as regression of _______. Other signs and symptoms include __________, __________, and __________
Neimann-Pick disease is a neurodegenerative disease that can present in children between the ages of 6 months and 2 years. It causes global delays as well as regression of milestones. Other signs and symptoms include hepatosplenomegaly, interstitial lung disease, and a macular cherry red spot
Spastic diplegia is a form of __________, a non-progressive static __________ characterized by delays in __________. It may be associated with ________________ abnormalities that are thought to be due to _____. These changes can be visualized on ___. In spastic diplegia, the motor abnormalities are often greater in the ___ than in the ___
Spastic diplegia is a form of cerebral palsy, a non-progressive static encephalopathy characterized by delays in motor development. It may be associated with periventricular white matter abnormalities that are thought to be due to ischemia. These changes can be visualized on MRI. In spastic diplegia, the motor abnormalities are often greater in the legs than in the arms
Athetoid cerebral palsy involves motor deficits of ________. It is often caused by perinatal _____ and _____, both of which damage the ________, ________, and/or ______
Athetoid cerebral palsy involves motor deficits of the entire body. It is often caused by perinatal asphyxia and kernicterus, both of which damage the basal ganglia, cerebellum, and/or thalamus
A ___ age child should be able to scribble, use a cup, and/or stack 2 blocks. His word count should be three to six words at this stage of development
A 15-month-old child should be able to scribble, use a cup, and/or stack 2 blocks. His word count should be three to six words at this stage of development
a premie born via emergency c-section now 18 mos old can pull to stand but not walking yet, increased tone and hyperreflexia of lower extremiteis on exam
most likely dx
cerebral palsy
Children with cerebral palsy often present with defects in motor development and are often found to have abnormal neuromuscular exams, including increased tone and reflexes. Other areas of development are less consistently impacted. The cerebral palsy most likely was the result of an hypoxic injury during the perinatal period
3-year-old male presents to clinic with an annular, well-circumscribed, scaly plaque with a raised erythematous border and central hypopigmentation on the left thigh. The mother reports that the lesion is highly pruritic and that the patient has been exposed to other children with a similar rash at day care. Upon further examination, a similar lesion with boggy borders is also found on the posterior aspect of his scalp
treat
Although topical antifungal therapy (TOPICAL CLOTRIMITAZOLE) would be appropriate for the lesion on the leg (tinea corporis), involvement of the scalp (tinea capitis) necessitates systemic antifungals. Topical antifungals are not usually successful in treating tinea capitis, because the infected hair follicles are deep within the scalp. SYSTEMIC GRISEOFULVIN is the first choice for the treatment of tinea capitis.
SELENIUM SULFIDE SHAMPOO may decrease the likelihood of transmission of the infection to others by decreasing the number of spores shed, but does not treat the primary infection
Cellulitis in children is usually caused by _____ or _____ . Treatment with a ___________ (such as _____ ) or a __________ with a __________ (such as __________) would be approppriate of there is no concern for ____. _____ or __________ would be appropriate treatment for cellulitis caused by ____.
Cellulitis in children is usually caused by S. pyogenes or S. aureus. Treatment with a first-generation cephalosporin (such as cefalexin) or a semi-sythenthetic penicillin with a beta-lactamase inhibitor (amoxicillin-clavulonate) would be approppriate of there is no concern for MRSA. Clindamycin or trimethoprin/sulfamethoxazole would be appropriate treatment for cellulitis caused by MRSA.
Changing detergents would be an appropriate intervention for what skin pathology
where does it usually appear in children
is it more or less pruritic than atopic dermatitis
Changing detergents would be an appropriate for irritant dermatitis
diaper area, face, and extensor surfaces on children
typically less pruritic than atopic dermatitis
Treatment with topical clotrimazole would be appropriate for ________
Treatment with topical clotrimazole would be appropriate for tinea corporis
dry, itchy skin in addition to erythema, scaling, vesicles, or lichenification in skin flexures
dx
tx
Atopic dermatitis
Treatment consists of emollients and topical corticosteroids
Treatment with permethrin cream would be appropriate for ____ infection
Treatment with permethrin cream would be appropriate for scabies infection
when do scabiesoften itch more
at night
where are the common lesion locations for scabies
wrists, ankles, palms, soles, interdigital spaces, axilla, waist, and groin are among the most common locations for lesions
10-year-old boy, history of hypopigmented non-pruritic “dots,” mostly located on his face and neck. worse during the summer when plays outside. On exam, they are slightly scaly, hypopigmented lesions approximately 0.5 cm in diameter. What is the most likely etiology of his rash
pityriasis alba
Decreased number of active melanocytes and decreased number and size of melanosomes
Pityriasis alba
age
appearance
etiology
common in children 3 to 16 years of age
presents as hypopigmented macules. most often on the face, neck, trunk, and extremities. irregular borders, can vary in size, and may have a slight scale. may become more noticeable after sun exposure because of tanning of the surrounding skin.
etiology is unknown, but ultrastructural examination of epidermal cells reveal decreased number of active melanocytes as well as decreased number and size of melanosomes
