CompTIA
Checkpoint
ISC
HP
Dell
EC-Council
EXIN
Fortinet
ITIL®
Juniper
PMI
Microsoft
VMware
Avaya
Google
Salesforce
Amazon
Palo Alto Networks
Certiport
ISC2
All Vendors
  2. Home
  3. Exams
  4. USMLE
  5. STEP3

Free STEP3 Exam Braindumps (page: 94)

Page 93 of 202
PDF with 804 Questions

A 4-year-old child is brought to your office because of a sudden onset of irritability, weakness, and pallor. The mother tells you that both of her children have been experiencing episodes of vomiting and diarrhea. Your physical examination reveals a blood pressure of 115/80, dry mucus membranes, petechiae, and diffuse abdominal pain. The following laboratory work is obtained:
Urinalysis: microscopic hematuria and proteinuria Blood urea nitrogen (BUN)/creatinine (Cr):
20/1.0 mg/dL
Hemoglobin: 7 g/dL
Peripheral blood smear: fragmented RBCs
Prothrombin time (PT), partial thromboplastin time (PTT): normal Coombs' test: negative

What is the most likely diagnosis?

  1. ITP
  2. HSP
  3. Evans syndrome
  4. meningococcemia
  5. HUS

Answer(s): E

Explanation:

HUS is, as the name implies, the combination of a microangiopathic hemolytic anemia and acute renal failure. It is commonly associated with E. coli O157/H7 gastroenteritis. HUS is one of the most common causes of acquired renal failure in children.



A mildly mentally retarded 9-year-old girl is brought to your office for acne. On examination, she does not actually have acne but has small flesh colored papules along her nasolabial fold. Her past history is significant for having had a first (afebrile) seizure last year. The mother reminds you that she has a faint birthmark on her hip that is pale and becomes more prominent in the summer, when the child's skin tans. Examination of this area reveals a 5-cm oval patch that is hypopigmented. Which condition would she most likely have?

  1. Sturge-Weber syndrome
  2. neurofibromatosis, type 1 (von Recklinghausen disease)
  3. tuberous sclerosis
  4. CHARGE association
  5. Beckwith-Wiedemann syndrome

Answer(s): C

Explanation:

Children with tuberous sclerosis (tuberous sclerosis complex, TSC) can develop nasolabial fold angiofibromas (commonly referred to adenoma sebaceum). These can be mistaken for acne in an adolescent and is seen in over 80% of adolescents with TSC. Other cutaneous findings include peri- and subungual fibromas, ash leaf spots (hypomelanocytic macules), and shagreen patches. Skin findings are seen in 75% of cases of tuberous sclerosis. Tuberous sclerosis can also have CNS cortical defects and "tubers," which may be foci of seizure activity. The "tubers" have a classic periventricular distribution. Mental retardation is very common in varying degrees. Sturge-Weber syndrome (also known as leptomeningeal angiomatosis) is a port wine stain, typically with V1 or V2 distribution, with an underlying leptomeningeal blood vessel dysplasia. These dysplastic vessels will typically result in an underlying cortical atrophy. This cortical atrophy can be a nidus for seizures and can be seen on a CT scan of the head. Neurofibromatosis, type 1 is polymorphous neurocutaneous syndrome. The most common findings are café au lait spots, which may be present at birth and worsen during puberty. Beckwith-Wiedemann syndrome and CHARGE association are discussed further in explanations 44 and 45.



A mildly mentally retarded 9-year-old girl is brought to your office for acne. On examination, she does not actually have acne but has small flesh colored papules along her nasolabial fold. Her past history is significant for having had a first (afebrile) seizure last year. The mother reminds you that she has a faint birthmark on her hip that is pale and becomes more prominent in the summer, when the child's skin tans. Examination of this area reveals a 5-cm oval patch that is hypopigmented. Which condition would she most likely have?

In evaluating her first seizure, a head CT was performed. Which finding would be most consistent with her diagnosis?

  1. frontal cortical atrophy with dysplastic vessels
  2. periventricular "tubers"
  3. diffuse white matter calcifications
  4. hydrocephalus from aqueductal stenosis
  5. poor gray-white differentiation

Answer(s): B

Explanation:

Children with tuberous sclerosis (tuberous sclerosis complex, TSC) can develop nasolabial fold angiofibromas (commonly referred to adenoma sebaceum). These can be mistaken for acne in an adolescent and is seen in over 80% of adolescents with TSC. Other cutaneous findings include peri- and subungual fibromas, ash leaf spots (hypomelanocytic macules), and shagreen patches. Skin findings are seen in 75% of cases of tuberous sclerosis. Tuberous sclerosis can also have CNS cortical defects and "tubers," which may be foci of seizure activity. The "tubers" have a classic periventricular distribution. Mental retardation is very common in varying degrees. Sturge-Weber syndrome (also known as leptomeningeal angiomatosis) is a port wine stain, typically with V1 or V2 distribution, with an underlying leptomeningeal blood vessel dysplasia. These dysplastic vessels will typically result in an underlying cortical atrophy. This cortical atrophy can be a nidus for seizures and can be seen on a CT scan of the head. Neurofibromatosis, type 1 is polymorphous neurocutaneous syndrome. The most common findings are café au lait spots, which may be present at birth and worsen during puberty. Beckwith-Wiedemann syndrome and CHARGE association are discussed further in explanations 44 and 45



Which of the following conditions usually causes hypoglycemia at birth?

  1. Sturge-Weber syndrome
  2. neurofibromatosis, type 1 (von Recklinghausen disease)
  3. tuberous sclerosis
  4. CHARGE association
  5. Beckwith-Wiedemann syndrome

Answer(s): E

Explanation:

Of the listed syndromes, only Beckwith Wiedemann syndrome has neonatal hypoglycemia as part of its clinical spectrum. The constellation of macroglossia, hypoglycemia, and visceral organomegaly (hepatosplenomegaly) is a common finding in children with Beckwith-Wiedemann syndrome. The presence of an omphalocele in a newborn would also be concerning for Beckwith- Wiedemann syndrome.






Post your Comments and Discuss USMLE STEP3 exam with other Community members:

STEP3 Exam Discussions & Posts