Nokea
A 3-year-old boy is referred to you for evaluation of right leukocoria. Funduscopic examination under anesthesia reveals a large amelanotic mass occupying more than two-thirds of the vitreous space in his right eye, with massive retinal detachment, consistent with group E retinoblastoma. The left eye is normal. An MRI confirms the funduscopic findings and shows no extraocular disease. What is the most appropriate next step in the management of this child's disease?
- A. Enucleation
- B. Systemic chemotherapy
- C. Brachytherapy
- D. Needle biopsy
Correct Answer: A
Rationale: The correct answer is A: Enucleation. In this case, the child has a group E retinoblastoma in the right eye, with massive retinal detachment. Enucleation, or surgical removal of the eye, is the most appropriate next step as the tumor is extensive and poses a risk of metastasis. Enucleation helps prevent spread of the cancer to other parts of the body and provides the best chance for cure. Systemic chemotherapy (B) and brachytherapy (C) are not ideal for such advanced cases due to the high risk of metastasis. Needle biopsy (D) is not recommended as the diagnosis is already confirmed by funduscopic examination and MRI.
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A patient with a bleeding disorder is prescribed an infusion of plasma. What should the nurse explain as being the purpose of this infusion?
- A. Contains clotting factors
- B. Carries oxygen to the tissues
- C. Supports cellular metabolism
- D. Removes waste products from cells
Correct Answer: A
Rationale: The correct answer is A: Contains clotting factors. Plasma contains essential clotting factors like fibrinogen, Factor VIII, and others that are necessary for blood clotting in patients with bleeding disorders. This infusion helps improve the patient's ability to form blood clots and control bleeding.
B: Plasma does not carry oxygen to tissues; that is the role of red blood cells.
C: Plasma does not directly support cellular metabolism; that is the function of nutrients.
D: Plasma does not remove waste products from cells; that is the role of the kidneys and liver.
A 30 year old patient had presented with fever and posterior cervical lymphadenopathy over the past two years. Which is the most probably causative organism?
- A. Trypanosoma cruzi
- B. Trypanosoma brucei rhodesiense
- C. Trypanosoma brucei gambiense
- D. Leishmania donovani
Correct Answer: C
Rationale: The correct answer is C: Trypanosoma brucei gambiense. This is the most probable causative organism because the patient's presentation of fever and posterior cervical lymphadenopathy over a prolonged period aligns with the chronic infection caused by Trypanosoma brucei gambiense, which leads to African Trypanosomiasis (sleeping sickness). Trypanosoma cruzi (choice A) causes Chagas disease, which typically presents with acute symptoms such as fever and swelling at the site of infection. Trypanosoma brucei rhodesiense (choice B) causes acute African Trypanosomiasis with a more rapid progression compared to gambiense. Leishmania donovani (choice D) causes visceral leishmaniasis, which presents with prolonged fever, splenomegaly, and pancytopenia, but not typically with cervical lymphadenopathy.
A 19-year old male patient with a history of acute lymphoblastic leukemia, currently 13 years from completion of therapy, presents for a fertility consultation. He is interested in his risk for infertility. Which of the following statements is true?
- A. A semen analysis at this point would provide accurate information about future fertility.
- B. Males can maintain gonadal function at higher cumulative alkylator dosages compared with females.
- C. He should have been offered sperm cryopreservation at diagnosis.
- D. His risk for testosterone deficiency is greater than his risk for infertility.
Correct Answer: A
Rationale: Step 1: Semen analysis is the gold standard for assessing male fertility.
Step 2: The patient's history of ALL and completion of therapy make semen analysis relevant.
Step 3: Long-term survivors of ALL are at risk for infertility due to treatment effects.
Step 4: Semen analysis can provide valuable information on sperm count, motility, and morphology.
Summary:
- Option A is correct as semen analysis is crucial for assessing male fertility.
- Option B is incorrect as alkylator dosages affect both males and females.
- Option C is incorrect as sperm cryopreservation should ideally be offered at diagnosis.
- Option D is incorrect as infertility risk may be higher than testosterone deficiency in this case.
Which of the following is the most suitable anti malarial drug for pregnant women?
- A. Atovaquone and proguanil
- B. Quinine and doxycycline
- C. Quinine and clindamycin
- D. Primaquine
Correct Answer: C
Rationale: The correct answer is C: Quinine and clindamycin. Quinine is considered safe for use in pregnancy to treat malaria, as it has been used for many years without significant adverse effects. Clindamycin is also safe for use in pregnancy and can be used in combination with quinine to treat malaria. Atovaquone and proguanil (choice A) are not recommended for use in pregnancy due to limited safety data. Quinine and doxycycline (choice B) is not suitable for pregnant women as doxycycline is contraindicated during pregnancy. Primaquine (choice D) is not recommended for use during pregnancy as it can cause harm to the fetus.
The patient is a 2-month-old boy who presented with a skin abscess and is febrile. On exam, he is noted to have silvery hair and hypopigmented skin. A CBC shows a leukocyte count of 3.4 K/mcL with 10% neutrophils. What does the abnormality on the peripheral smear suggest?
- A. Abnormal lysosomal biogenesis
- B. Abnormal ribosome function
- C. Abnormal phagocytosis of opsonized particles
- D. Abnormal mitochondrial activity
Correct Answer: A
Rationale: The correct answer is A: Abnormal lysosomal biogenesis. The patient's presentation of silvery hair and hypopigmented skin suggests a lysosomal storage disorder like Chediak-Higashi syndrome. The peripheral smear showing 10% neutrophils with a low leukocyte count indicates impaired neutrophil function due to abnormal lysosomal biogenesis. This affects phagocytosis and intracellular killing of pathogens, leading to recurrent infections and abscess formation.
Incorrect choices:
B: Abnormal ribosome function - not related to the patient's presentation.
C: Abnormal phagocytosis of opsonized particles - the primary issue is with lysosomal biogenesis, not phagocytosis.
D: Abnormal mitochondrial activity - does not explain the silvery hair and hypopigmented skin seen in Chediak-Higashi syndrome.