CPHON Exam 48 Questions with Verified Answers
Thalassemia - CORRECT ANSWER Inherited defect in production of hemoglobin chain. Destruction of RBC.
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Treatment: frequent RBC transfusions
Sickle Cell Disease - CORRECT ANSWER inherited defect of hemoglobin causing sickling. destruction of RBC
hemophilia - CORRECT ANSWER Congenital deficiency in clotting factor. prolonged bleeding.
von Willebrand Disease (vWB) - CORRECT ANSWER inherited deficiency or defective clotting protein.
mucous membrane bleeding
Immune Thrombocytopenia (ITP) - CORRECT ANSWER ACQUIRED. disorder of excessive platelet destruction
Aplastic Anemia - CORRECT ANSWER suppression of production of formed elements in blood. Congenital or acquired.
Red Cell Aplasia - CORRECT ANSWER lack of erythoid cell. Congential = Diamond blackfan. Can be acquired
What does mutatin result in? - CORRECT ANSWER 1. lack of differentiation
2. loss of contact inhibition
3. unregulated growth
4. cellular immortality
what are genetic predispositions to cancer? - CORRECT ANSWER 1. down syndrome
2. Li-Fraumeni Syndrome
3. Retinoblastoma gene
What is part of the ectoderm? - CORRECT ANSWER CNS, brain and spinal cord, meninges
What cancers are common in infancy and early childhood? - CORRECT ANSWER 1. neuroblastoma
2. Wilm's Tumor
3. Retinoblastoma
What cancers are common in adolsence? - CORRECT ANSWER 1. Hodgkin Lymphoma
2. Osteosarcoma
What does the supratentorial part of the brain control? - CORRECT ANSWER 1. Personality
2. Speech
3. Auditory/ vision
4. motor/ sensory
5. Area of seizure foci
What does the infratentorial part of the brain control? - CORRECT ANSWER 1. balance and coordination
2. autonomic functions
3. conduit for nerve transmission
What is part of the posterior fossa? - CORRECT ANSWER cerebellum, pons, medulla oblongata, brain stem
*brain stem controls involuntary function*
think: posterior fossa syndrome, S.S.
What commonly causes increased ICP? - CORRECT ANSWER 1. head trauma
2. hemorrhage
3. neoplasm
4. epilepsy
5. CNS infections
6. medications
7. F&E imbalance
symptoms of increased ICP include - CORRECT ANSWER 1. increasing headaches
2. decrease in consciousness
3. restlessness and irritability
4. bradycardia
5. hypertension
6. Pailledema
Why would we place a shunt for ICP? - CORRECT ANSWER 1. increased ICP
2. hydrocephalus
3. risk for death
Types of shunts - CORRECT ANSWER 1. ventriculoperitoneal
2. ventriculostomy
What are some complications associated with an LP? - CORRECT ANSWER 1. increased ICP with an LP = upward or downward herniation and compression of brain stem and spine
2. RAPID death, neurological destruction
What are the demographics of brain tumors? - CORRECT ANSWER 1. more common in developing countries
2. white children more than Asian or African
3. males more than females have malignant tumors
4. females more than males get benign tumors
What is the most common malignant pediatric brain tumor? - CORRECT ANSWER PNET
Where do more pediatric brain tumors occur? - CORRECT ANSWER infratentorial (65%)
supratentorial (35%)
What are the known causes of pediatric brain tumors? - CORRECT ANSWER 1. genetic disorders
2. Exposure to radiation
What is the gene associated with Fi-Fraumeni Syndrome? - CORRECT ANSWER p53 gene. cuases multiple tumors, changes treatment plan completely
What is neurofibromatosis 1 associated with? - CORRECT ANSWER 1. optic glioma
What is retinoblastoma assicated with? - CORRECT ANSWER pineoblastoma
What is a common workup for a brain tumor? - CORRECT ANSWER 1. neuro exam, history
2. CT/ MRI
3. CSF cytology
What are common symptoms of pediatric brain tumors? - CORRECT ANSWER 1. flu-like
2. morning headache and vomiting
3. ataxia
4. cranial nerve deficits
5. impaired vision
6. mental status change
7. delay or loss in developmental milestones
8. increased head circumference
9. seizures
why are seizures uncommon symptoms in brain tumors? - CORRECT ANSWER the location of the tumor plays a big role. Infratentorial tumors are more common and that is not where seizure foci is located (supratentorial)
Astrocytoma (aka glioma) - CORRECT ANSWER high grade = supratentorial
low grade = optic glioma, juvenile pilocytic astrocytoma
Treatment of astrocytoma/ glioma - CORRECT ANSWER 1. high grade = surgery, radiation, chemo, BMT < 3 years
2. low grade = surgery, radiation and chemo are RARE
What is juvenile pilocytic astrocytoma - CORRECT ANSWER occurs in cerebellum. age 6-7. male and female ratio the same.
what is the treatment plan for juvenile pilocytic astrocytoma? - CORRECT ANSWER surgery, radiation
What are the presenting symptoms of optic Glioma? - CORRECT ANSWER age 1-5 years. semi uncommon. NF 1 association.
presents with: change in vision is MOST common, nystagmus, DI/ SIADH
Why is optic glioma associated with DI/ SIADH - CORRECT ANSWER it is so close to hypothalamus
What is Medulloblastoma? (PNET) - CORRECT ANSWER most common malignant brain tumor in kids
age 4-7 Is most common. more common in MALES. Very malignant.
what is the treatment for PNET/ Medulloblastoma? - CORRECT ANSWER 1. resection to determine prognosis
2. radiation (brania dn spinal cord) dose reduced
3. chemotherapy
4. BMT if <3 years or high risk
what is the goal of treatment for PNET? - CORRECT ANSWER total resection
Can you get radiation if you're < 3 years old? - CORRECT ANSWER no.
how many transplants do some medulloblastoma/ PNET patients get? - CORRECT ANSWER up to 3 transplants
What is a brain stem glioma? DIPG - CORRECT ANSWER peak age 8-10 years, significant neurological defects.
chemo doesn't work because of BBB
<5% survival
What is an Ependymoma? - CORRECT ANSWER fairly uncommon, usually low grade, anaplastic
age 2-6
occurs in intracranial 4th ventricle
adolescence get them in spinal cord
what is the treatment for Ependymoma - CORRECT ANSWER surgery, radiation, chemotherapy.
about 50% survival
What is the gold standard for spinal cord tumor? - CORRECT ANSWER surgery
what do you do for localized high grade spinal cord tumors? - CORRECT ANSWER radiation
where are deficits most common in spinal cord tumors? - CORRECT ANSWER below the level of the tumor (i.e. if tumor is mid spine, lower spine and legs would be affected)
Is proton therapy more specific or less specific than traditional radiation? - CORRECT ANSWER it is very specific. [Show Less]