1. All of the following is true about Proteus syndrome:  

a. It is a disorder which consists of skeletal, hamartomatous and other mesodermal malformations.

b. The manifestations are variable and features may be present at birth but become more apparent with time.

c. Several tumors are occasionally associated with Proteus syndrome including testicular tumors, mesothelioma, central nervous tumors and bilateral ovarian serous cystadenomas.

d. The associated anomalies include ocular manifestations and mental deficiency.

e. All of the above

2. The following are clinical characteristics of hereditary lymphedema, except:

a. The lower limb lymphedema is often associated with other anomalies

b. Lymphedema is painless, non-pitting.

c. There are no associated varicosities.

d. There is no tendency to ulceration

e. It is a lifelong disease that does not affect longevity

3. Hereditary lymphedema:  

a. has a male to female ratio is 1:1

b. occurs approximately in 1-2:10,000 births

c. is an autosomal recessive disorder with reduced penetrance

d. can be suggested in fetus with lymphedema and a abnormal karyotype

e. does not persists throughout life

4. The diagnosis of hereditary lymphedema includes:

a. Distal subcutaneous edema of the extremities

b. Persistent or transient pleural effusion

c. Ascites

d. Genital edema

e. All of the above

5. All of the following are true about the Klippel-Trenaunay-Weber syndrome except:

a. The syndrome is characterized by the presence of multiple skin hemangiomas, asymmetric limb hypertrophy and arteriovenous fistulas.

b. The pattern of inheritance is an autosomal recessive disorder.

c. The differential diagnosis includes proteus syndrome and cystic hygroma.

d. The ultrasound diagnosis is based upon the association of cutaneous multicystic lesions and marked enlargement of the soft tissues of a limb or other part of the fetal body.

e. The prognosis depends on the location and size of the hemangioma.

6. All of the following are important in the differential diagnosis of hereditary lymphedema except:

a. Turner syndrome

b. Noonan syndrome

c. Hydrops

d. Down syndrome

e. Lymphedema and ptosis syndrome

7. All of the following are associated anomalies of hereditary lymphedema, except:

a. Distichiasis

b. Hydroceles

c. Diaphragmatic hernia

d. Atrial septal defect

e. Characteristic facial changes

8. Which one of the following is true in the genetics of hereditary lymphedema?:

a. It is an autosomal dominant disorder with incomplete penetrance

b. The gene mutation has been found near the most telomeric region of 5q35.32

c. There is also some evidence of 2 different sub-mutations or variants depending on the nucleotide substituted

d. Variation in age at onset, both within and between families, may be related to a digenic inheritance

e. All of the above

9. Which one of the following is true about the hereditary lymphedema?:

a. The pathogenesis is based on a congenital dysgenesis of lymphatic micro vessels

b. The prenatal diagnosis is not feasible

c. Anasarca is very often present in the birth

d. Inadequate postnatal growth support this condition

e. All of the above

10. All of the following are possible complications of hereditary lymphedema, except:

a. Intestinal lymphangiectasia

b. Ventriculomegaly

c. Recurrent septic arthritis

d. Angiosarcoma

e. Bacterial infections of dorsal aspects of feet and toes