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immediate management and long term treatment of SCD vaso-occlusive pain crisis

-IVF and possible narcotics (morphine or hydromorphone)
-Administration of hydroxyurea will increase the concentration of fetal hemoglobin, thus reducing the frequency of sickle cell crisis episodes.


risk factors and triggers for sickle cell pain crisis

increasing age and high baseline hemoglobin level
infection, stress, cold temperatures, or high altitude, often no trigger can be found


most common sites of sickle cell pain crisis

extremities or back


vaso-occlusive crisis

an episode of severe pain caused by increased sickling of RBCs, which leads to bone marrow ischemia and infarction.


acute chest syndrome (ACS)

A new pulmonary infiltrate on chest x-ray (CXR) in addition to one of the following signs: fever, chest pain, shortness of breath, tachypnea, or low oxygen saturations.
-empiric antibiotics, supplemental oxygen, pain medications, and IVF


aplastic crises: etiology?

Infection with parvovirus B19 (most commonly), which leads to temporary cessation of RBC formation. Reduced lifespan of RBCs in SCD coupled with reduced production may result in profound anemia.


why is a pt with SCD + fever greater than 38.5°C an emergency?
how to dx/tx

SCD causes functional asplenia and predisposes patients to invasive encapsulated organisms (typically pneumococcal disease)
get CBC and blood culture
initiate empiric abx if no source found (i.e. ceftriaxone)


a child with SCD who has abdominal pain, distension, or acute enlargement of the spleen likely has acute splenic sequestration and requires hospitalization, possibly in the intensive care unit, to observe for ?
management may require ?

cardiovascular collapse
Blood transfusions

-spleen typically auto-infarcts with age


10% of kids with SCD have acute strokes, so routine well-child care may involve this screening and treatment?

transcranial Doppler (TCD) ultrasonography to identify those with increased flow velocity in the large cerebral blood vessels, and thus are at high-risk for developing a first stroke
-Routine chronic transfusion


child with SCD who presents with a significant increase in pallor, fatigue, or lethargy may be exhibiting signs of ?

aplastic crisis
hgb level below their normal baseline and a low reticulocyte count
observe for CV collapse, may need transfusion


management of boy with SCD who has a priapism episode persisting for more than 3 to 4 hours

IVF, pain control, don't use ice, urologist may need to aspirate and irrigate the corpora cavernosa to achieve detumescence, Failure of 3 or 4 aspirations in the outpt setting requires more extensive inpt care, including exchange blood transfusions, further pain control, and sx intervention.


prophylactic care for kids with SCD

prophylactic penicillin and folate, pneumococcal PCV13 series at 2, 4, 6, and 12 to 15 months, pneumococcal and meningococcal vaccines starting at 2 years of age


routine care for kids with SCD

Frequent CBCs are performed, and renal, liver, and lung function are monitored annually beginning at 1 year of age, Routine spleen palpation should be performed at home


Children with SCD who have ? (many conditions) must be evaluated urgently

fever (risk of sepsis), pallor (aplastic crisis), abdominal pain or distension (splenic sequestration), pain crisis, evidence of lower respiratory disease (acute chest syndrome) priapism, new neurologic findings (stroke), or dehydration


hyperammonemia and elevated urine orotic acid are diagnostic of ?

OTC deficiency, an x-linked condition, the most common urea cycle disorder.


Lesions within the vermis (midline cerebellum) cause ?

dysarthria, truncal ataxia, and gait abnormalities


Cerebellar hemispheric lesions cause ?

ipsilateral limb abnormalities, nystagmus, tremor/dysmetria and tend to spare speech.
-fall towards the side of the lesion and have worse nystagmus when they look towards the side of the lesion.


Lesions of the deep cerebellar nuclei cause ?

resting tremor, myoclonus, and opsoclonus such as that seen in children with a neuroblastoma.


Infratentorial lesions usually present with ?

cerebellar signs and signs of raised intracranial pressure (ICP).


Cerebellar hemispheric lesions can cause changes in ?

muscle tone and DTRs, usually hypotonia and hyporeflexia.


Supratentorial lesions more commonly lead to ?

focal motor and sensory abnormalities on the side opposite to the lesion.


Brain stem tumors will often be associated with ?

cranial nerve and gaze palsies.


Post-infectious cerebellitis

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.


(A, E, I, O, U) and TIPS is often helpful in remembering the causes of altered mental status:

Alcohol, ingested toxins
Epilepsy, encephalitis, endocrine, electrolytes
Infection, insulin
Overdose, opiates, oxygen deprived
Uremia (renal failure)
Trauma, temperature
Insulin, infection
Stroke, shock, space occupying lesions


Immediate treatment to address hypoglycemia, hypotension, and tachycardia would include ?

giving boluses of 20cc/kg normal saline and 25% dextrose. These can be followed by administration of fluids with 10% dextrose at maintenance.


possible antidote for hypoglycemia caused by antiglycemic OD

Octreotide: a somatostatin analog—inhibits insulin release and may be indicated as an antidote in dextrose-refractory sulfonylurea overdose


kid vital signs



by 12 mos how should weight and height change from birth?

weight should triple and height increase by 50%


transposition of the great vessels presents how?

presents in 1st few hours with cyanosis and single loud S2
most common congenital cyanotic heart defect in neonatal period
initiate prostaglandins to keep PDA open to facilitate blood mixing


first-line management of epiglottis with severe respiratory distress

endotracheal intubation
then consider abx and lateral neck films
steroids and racemic epi are for croup


Diamond-Blackfan Syndrome (DBS)

congenital hypoplastic anemia
macrocytic anemia with NO hyperseg. neutros, low retic, congenital anomalies (short, web neck, cleft lip, shield chest, triphalangeal thumb), pallor in neonate, elevated Hgb F
tx: corticosteroids, transfusion therapy


refeeding syndrome

increased insulin results in increase in intracellular uptake of phosphorus, K+, Mg+, thiamine, can result in low serum levels leading to arrhythmia, CHF, seizures, Wernicke's
-always supplement refeeding with electrolytes


cerebral palsy

A disorder of nonprogressive movement and posture that results from an insult to or anomaly of the developing central nervous system (CNS)


how common is CP? comorbidities?

CP is the most common childhood movement disorder. about 1/3 also have seizures, about 60% of affected children are mentally retarded
-Deafness, visual impairments, swallowing difficulty with concomitant aspiration, limb and sensory impairments, and behavioral disturbances


proposed causes of CP

antenatal insults, and subsequent difficulties during the pregnancy, delivery, and perinatal period increase insult
-10% have family history (chromosomal or metabolic disorder)


Acquired conditions that carry a risk for CP are

infection (cytomegalovirus, group B Streptococcus, herpes simplex virus), intraventricular hemorrhage, especially if it extends into the white matter and causes cystic periventricular leukomalacia (PVL), acute bilirubin encephalopathy (kernicterus), stroke, and brain trauma.


why is diagnosis of CP nearly impossible in a neonate and difficult in infants younger than 6 months

Immaturity of the CNS at birth, primitive reflexes may persist until 6 months


imaging for CP

MRI, may show periventricular leukomalacia, atrophy, or focal infarctions


concerning developmental findings in CP

A stepping response after the age of 3 months
A Moro reflex beyond 6 months
An asymmetrical tonic neck reflex beyond 6 months


definitions hemiplegia, diplegia, spastic quadriplegia (topographic classification)

Hemiplegia: single lateral side of the body, UE worse than LEs
Diplegia: 4 limbs, LE worse than UE
Spastic quadriplegia: 4 limbs typically equal but LE may be worse than UE


Physiologic classification of CP identify the major motor abnormality and are divided into ?

pyramidal (spastic) and extrapyramidal (nonspastic) categories. Extrapyramidal types can be subdivided further into choreoathetoid, ataxic, dystonic, or rigid types.


functional classification of CP relies on a "motor quotient"

A motor quotient of 75 to 100 represents minimal impairment, 55 to 70 is mild impairment, 40 to 55 is moderate impairment, and lesser quotients indicate severe impairment.


Identification of comorbid conditions with CP includes ?

cognitive testing for mental retardation and electroencephalography (EEG) for seizures, vision and hearing test


cystic fibrosis

The major cause of chronic debilitating pulmonary disease and pancreatic exocrine deficiency in the first three decades of life. It is characterized by the triad of chronic obstructive pulmonary disease, pancreatic exocrine deficiency, and abnormally high sweat electrolyte concentrations. Characteristic pancreatic changes give the disease its name.


most common mutation causing the abnormal CFTR (cystic fibrosis transmembrane conductance regulator) protein is known as CFTR (cystic fibrosis transmembrane conductance regulator) protein ?
which allows loss of ?

delta 508
altered CFTR protein allows excess loss of sodium and chloride. The loss from the eccrine glands of the skin causes a hyponatremic, hypochloremic alkalosis


CF may result in fat-soluble vitamin deficiencies manifesting as ?

peripheral neuropathy and hemolytic anemia (vitamin E), night blindness (vitamin A), or mucosal bleeding (vitamin K)


The diagnosis of CF requires

two positive sweat tests (60 mEq/L or greater) in conjunction with any of the characteristic respiratory, gastrointestinal, or genitourinary symptoms.
then DNA testing is then employed to detect the presence of mutations known to cause CF to confirm, even if sweat Cl- is negative (may be falsely neg)


Routine screening for CF is performed on all newborns in the US with what test?

detection of pancreatic enzyme, immunoreactive trypsinogen (IRT), which is elevated in infants with CF.
if +, undergo sweat chloride testing for definitive diagnosis


management of CF

Bronchodilators, inhaled corticosteroids, antibiotics, and inhaled recombinant human DNAse are used to minimize airway reactivity, infections, and secretions.
Optimal nutrition is dependent on pancreatic enzyme replacement and vitamin supplements.


Extrapulmonary signs and symptoms, such as ? are clues to the diagnosis of CF.

digital clubbing, recurrent sinusitis, failure to thrive, fat malabsorption, bronchiectasis, and a history of meconium ileus