18. Recognize that children with hemihypertrophy and somatic overgrowth syndromes should be periodically evaluated for the development of embryonal tumors
42. In the rest of childhood it keeps up with growth, gradually increasing; then in adolescence it further increases so that male > female hgb (due to androgen)
61. More advanced tests: ferritin (total body iron stores) may be low, but in cases of acute illness it may be elevated as it is an acute phase reactant; TIBC may me elevated; transferring saturation is low
62. Mentzer index = MCV/RBC; if it is >13 it is indicative of Fe deficiency, if <13 it is indicative of a thalassemia (but this is only a screen)
63. Know that tx for Fe deficiency with Fe sulfate should continue after the hgb concentration has returned to normal
64. Should see increases in MCV, hgb and retic counts in about 1-4 weeks, and these should be checked in that timeframe to see if there is a result of the Fe tx
65. Usual dose is 6mg/kg/day of elemental Fe and it can be in divided doses. Giving it with vitamin C can help improve absorption
66. Reason for continuing the tx beyond the initial improvement is to replenish iron stores and prevent repeat problems
67. Know that IM injxns or erythrocyte infusions are not appropriate for a child with routine nutritional Fe deficiency
75. Document the dx of B12 or folate deficiency with specific measurement of serum B12 concentration or serum or erythrocyte folic acid concentrations before beginning replacement tx
76. Regarding folate, the RBC / erythrocyte folate level is the one to check because the serum folate level is variable and may not acutely reflect that folate status
77. Know that B12 deficiency may occur following small bowel resection or as a result of a maternal vegan diet in a child who is exclusively breastfed
78. If there are gut probs in the patient, think B12
79. Remember intrinsic factor (made in the parital cells) required to absorb the B12 in the ileum. Also requires transcobalamin II within the cells to be converted to active form
89. Should also be considered in any newborn who manifests jaundice in the first 24 horus after borth, is jaundce depiste being exclusively formula fed, or has a fam hx of RBC disorders
90. Know that causes of hemolysis in pts with G6PD and manage appropriately
101. All pts with SCD who develop fver >101 is mandated and administration os a 3rd generation cephalosporin (ceftriaxone, cefotaxime) plus clinda or vanco for severe infections is the current practice (Per PIR article, 2007)
102. The spleen is the first organ to experience major damage in SCD; therefore infection w encapsulated orgaisms (strep penumo) is especially concerning
103. Recognize the findings of an enlarged spleen and worsening anemia as indicative of sequestration crisis in SCD
104. Understand that immediate intervention with IVFs and/or blood is the tx for acute sequestration crises
114. Tx = broad spectrum abx, including a cephalosporin and a macrolide, as well as oxygen, hydration, incentive spriometry and early intervention with simple transfusion tx for associated hypoxia or hct <18%
116. Frequent admissions for pain crises (>6/year) are a known risk factor for early death in SCD
117. Management should include aggressive pain management with age appropriate patient controlled analgesia (morphine or hydromorphone), NSAIDs (ibup or ketorolac), hydration, PT, and ancillary therapies like guided therapy/relaxation, etc
118. Know that thalassemia major usually presents as a severe hypochromic, microcytic anemia with enlargement of the liver and/or spleen
120. 42% of patients with Hb-SS have cholelithiasis by dolescence.
121. Cholecystectomy is only indicated if they hav suffer abd pain or cholestatic jaundice due to common duct obstruction; if they get cholecystitis then they are txed with abx and supportive care and have elective cholcystectomy weeks after the acute event
149. Occur in about 0.2% of transfusions and are likely due to cytokine release from the donor blood WBCs; can be minimized with leukocyte reduced blood
167. It might be helpful for those with CKD who would have reduced levels of epo due to their kidney probs, but what the actual role and benefit can be is currently being questioned (my editorialization)
174. Dx can be difficult b/c by the time they present they may have a nrmal or near normal WBC count; therefore if it suspected, they will need to have their counts checked 2-3 times a week for 4-6 weeks in order to determine if there is a cycle
175. Does not appear to be a risk factor for myelodysplasia or AML
194. Know that TCP in a newborn infant may be a sign of bacterial sepsis and, in an ill child, should lead to appropriate culture and antibiotic therapy
195. Usually have TCP due to platelet destruction caused by vasculitis, endotoxin, immune complexes or DIC
196. Plt transfusions are recommended when the count is <10, whenthere is difficult to control bleeding or when an invasive procedure is planned
197. Recognize that a hx of medications should be part of the eval of a child with tcp
198. Valproic acid and other anticonvulsanrs are notable for causing TCP
199. Recognize that the presence of TCP in a newborn infant with microcepahly a=or other congenital abnormalities may be due to a congenital viral infection such as CMV
207. Note: the TCP is not responsive to steroids or IVIg, it is actually due to a hyporegenerative marrow; don’t need to do a bone marrow bx
208. Know that ITP is characterized by a low plt count and normal or increased production of plts in the marrow
209. This is the most common cause of isolated TCP in otherwise well children
210. Bone marrow bx isn’t part of the work up, and should only be considered with referral to a hematologist
211. Understand that most children with ITP will recover in less than one year without tx
212. Tx is available, but do not alter the natural hx of acute ITP, neither preventing its progression to chronicity or accelerating disappearance of the offending antibody
213. Know that the common presenting sx of ITP is increased bruising
214. Usually a sudden onset of petechiae and bruising in a previously healthy child; may also have some mucosal bleeding
215. Know that corticosteroids and IVIg usually increase the plt count in children with ITP but do not alter the natural course (e.g. length of the dz)
216. Steroids help raise the count faster than no tx, and IVIg helps raise the count faster than steroids
217. No data show that drug treatment helps prevent serious bleeding, including ICH
218. Note, British panels recommend observation, American panels recommend intervention; overall more study is needed per the PIR article
219. Understand that ASA or other drugs that interfere with plt fxn should not be given to children w ITP or other qualitative or quantitative plt d/os
227. If they do have bleeding issues then IVIg is the tx of choice, and the TCP usually resolves after several months (the maternal ab has to be catabolized by the infant’s system before they can fully recover)
244. Know that a child born to the daughter of a person with hemophilia should be tested for that particular bleeding disorder
245. A child whose maternal grandparent has hemophilia needs to be checked
246. Carrier females have a 50% chance of passing the affected chromosome to male offspring
247. Know that HA is an important sx of intracranial bleeding and requires early assessment and tx
248. Understand that serious head trauma in a person with hemophilia requires careful assessment and early replacement tx even in the absence of neurologic abnormalities
249. Recognize bleeding in the forearm of a person with hemophilia as an emergency because of the danger of nerve compression
250. Know that femoral or jugular venipunctures should be avoided in the person with hemophilia who has not received replacement tx
251. Know that some kids with hemophilia have a negative family hx for bleeding disorders
252. Recognize that partial thromboplastin time is often normal in patients with vWF disease, but the bleeding time is commonly prolonged
253. Recognize that a manifestation of vWF in girls may be heavy menstrual bleeding
254. Recognize that a strong fam hx of PE or DVT may suggest a congenital hypercoagulable d/o
264. Peripheral smear will show evidence of intravascular hemolysis, like fragmented, burr, or helmet shaped cells
265. Tx of DIC = find and treat the sause of the shock; acidosis and hypoxemia worsen DIC; may need to give platelets, cryoprecipitate or FFP to stop hemorrhaging.
283. ID the CXR as an important part of the initial eval of the pt with unexplained LAD
284. Know that overwhelming sepsis is a serious complication in patients with Hodgkin dz who have undergone splenectomy, and know that such patients should be evaluated thoroughly if fever develops
286. Understand that a neuroblastoma usually presents as a nontender abd mass
287. Solid, often fixed, painful or painless, irregularly shaped masses that cross the midline
288. Understand that urinary catecholamine excretion is increased in most patients with nboma and that tests for urinary VMA and HVA are appropriate screening tests for this kind of tumor
289. Know that Wilms tumor is associated with hemihypertrophy and/or aniridia
297. Confined to sella tucica: usually clinical manifestations due to pituitary-hypothalamic involvement (short stature due to GH deficiency) and visual field defects due to pressure on optic chiasm,may also have other si of pituitary dysfxn like DI, hypothyroid, precocious puberty
298. If extands through the diaphragm sella, it may compress the third ventricle and cause sx of increased ICP like HA, vomiting and papilledma
299. Other sx can include alterations in personality, lethargy, hyperactivity, irritability, forgetfulness
300. Recognize the presenting si of brain tumor (eg, headache, deteriorating school performance, ataxia, emesis)
301. School age children: declining academic performance, fatigue, behavioral changes and vague HA, especially in the occipital or frontal region; over time the HA may be worse in the AM and lethargy and vomiting may develop; also horizontal diplopia and papilledema
302. Infants: irritability, anorexia, FTT, developmental regression can be signs. Macrocephaly, splitting of cranial sutures, bulging anterior fontanelle
303. “Setting sun” sign can be seen with attendant hydrocephalus (downward deviation of eyes)
317. Differentiate the clinical manifestations of spinal cord compression (eg, from a tumor) from those of other myelopathies and eval appropriately
318. Identify varicella as a life threatening illness in a pt receiving hemotx, and know that varicella Ig should be given immediately after exposure to varicella
319. Recognize the need for immediate eval of a febrile child who is neutropenic as a result of chemotx