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WorksheetsChapters 123-137
Total questions: 20
Worksheet time: 11mins
What is the most frequently earliest indicator of acute GVHD?
Liver
Skin
GI
Hematologic
Among patients with GHVD, the following systemic risk factors portend a poor prognosis EXCEPT:
history of progressive involvement from acute to chronic GHVD
hyperbilirubinemia
thrombocytopenia
lack of response to therapy at 12 months
Which of the following syndromes share the same ocular abnormalities with Focal Dermal Hypoplasia?
Shopf-Shulz Passarge Syndrome
Conradi-Hünermann Syndrome
microphthalmia, dermal dysplasia, sclerocornea syndrome
incontinentia pigmenti
odonto-onycho-dermal dysplasia
Which of the following would present with this tooth?
Witkop Syndrome
Tricho-dento-osseous syndrome
Tooth and nail syndrome
Oculo-dento-digital dysplasia syndrome
5-year old male with dysarthria, drooling and recurrent pneumonia. Which of the following is most likely elevated in this patient?
PSA
AFP
CEA
IL-6
The following statements about the two conditions represented by these two images are correct EXCEPT:
Both patients (B & C) may have “accelerated phase” of hemophagocytic lymphohistiocytosis that is usually triggered by EBVinfection.
Both patients (B & C) are most likely born to consanguineous families.
Both patients would show pigment clumping in hair shaft but patient B would have blood smears that show no leukocyte granules.
The pigmentary dilution in patient C is often limited to the hair, characterized by a silver-gray sheen with a few cases of skin involvement.
An infant with strict cow's milk diet presents with hypopigmentation of hair and skin. Which of the following is TRUE about the infant's condition?
Erythrocyte glutathione reductase activity can be used as a screening test.
Serum alkaline phosphatase may be low-normal.
Neutropenia is the earliest and most common sign and is a sensitive measure of treatment adequacy.
Plasma pyridoxal-5-phosphate is low.
Which of the following etiologic agents have recently infected this patient?
Plasmodium falciparum
cytomegalovirus
measles
Toxoplasma gondii
Which of the following statement is NOT TRUE about this patient?
All are true.
Pink to brownish staining of the diapers may be noted soon after birth.
This patient may have Gunther disease.
This patient may not have porphyria.
What is the defective gene involved in the most common porphyria in children?
Uroporphyrinogen decarboxylase (UROD)
Ferrochelatase (FECH)
Uroporphyrinogen synthase (UROS)
mutation of both UROD alleles, homozygous form of familial (type 2) PCT
In which of the following tumors would it be normal to see collections of amyloids?
BCC
Bowen disease
seborrheic keratosis
actinic keratosis
squamous cell carcinoma
Cutaneous lichen amyloidosis may define which of the following syndromes?
MEN1
MEN2A
MEN2B
Von Hippel Lindau
Which of the following diet plans may reduce total cholesterol and LDL-C levels?
Ketogenic diet
Paleo diet
Mediterranean diet
Atkins diet
Which of the following is most commonly associated with malignancy?
planar xanthoma
all of the above
xanthelasma
tendinous xanthoma
Which of the following drug may result to storage of similar inclusions in many of the same cells as Fabry disease and may result in similar clinical manifestations, including the development of angiokeratoma.
Chlorthalidone
Chloroquine
Hydroxychloroquine
Hydrochlorothiazide
Which of the following is true about Fabry disease?
There is an increased prevalence of factor V Leiden among Fabry patients who have suffered stroke.
Stroke, involving the anterior circulation, is often reported as the presenting feature of Fabry disease.
Definitive diagnosis of Fabry disease is delayed and it cannot be diagnosed in utero.
All are true.
Transforming growth factor-α is implicated in the pathophysiology of which of the following paraneoplastic conditions?
Tripe palm
Acanthosis nigricans
Leser Trelat Sign
Pityriasis rotunda
Which of the following micronutrients are hypothesized to be low in Bazex syndrome patients' serum?
Vitamin A
Vitamin C
Copper
Zinc
What is the most common malignancy seen in patients with Rothmund-Thomson Syndrome?
osteosarcoma
basal cell carcinoma
squamous cell carcinoma
Ewing's sarcoma
Which of the following is TRUE about neurofibromatosis?
The period of greatest risk for the development of symptomatic OPTs in NF-1 is during the first 10 years of life.
As many as 40% of children who have chiasmal tumors develop precocious puberty. Accelerated linear growth will be the first sign of precocious puberty.
Dysplasia of a long bone, characterized by congenital thinning and bowing, affects approximately 2% of children with NF-1. The femur is most commonly affected.
All are true.
