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Special Pops- Peds > Nephrology > Flashcards

Nephrology Flashcards

1
Q

Congenital anomalies- pediatric nephrology

A

Antenatal hydronephrosis

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2
Q

Hematuria & Glomerular Disease (4)

A

o Post strep glomerulonephritis
o Hemolytic uremic syndrome o Henoch Scholein purprua
o Benign familial hematuria

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3
Q

Diseases of Renal Vessels

A

o Renal artery stenosis/thrombosis

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4
Q

Antenatal Hydronephrosis

A 
o Urinary tract infections (UTIs)
o Abdominal mass
o Hematuria, urinary stone, poor urinary stream, incontinence o Failure to thrive
o Renal insufficiency or failure
Potters Syndrome
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5
Q

Potters Syndrome is?

A

flattened nose, recessed chin, prominent epicanthal folds, low-set ears

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6
Q

Hemolytic Uremic Syndrome (HUS)

A
  • Progressive renal failure associated with microangiopathic (nonimmune, Coombs-negative)
  • Hemolytic anemia
  • Thrombocytopenia
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7
Q

Hemolytic Uremic Syndrome (HUS) Presentation

A 
Prodromal gastroenteritis (83%)
– Fever (56%
– Bloody diarrhea (50%) for 2-7 days before the onset of renal failure
• Irritability, lethargy
• Seizures (20%)
• Acute renal failure (97%)
 • Anuria
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8
Q

Henoch Scholein Purpura Presentation

A

Rash (95-100% of cases), especially involving the legs; this is the hallmark of the disease
• Abdominal pain and vomiting (35-85%)
• Joint pain (60-84%), especially involving the knees and ankles
• Subcutaneous edema (20- 50%)
• Scrotal edema (2-35%)
• Bloody stools

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9
Q

3 Prodome for Henoch Scholein Purpura

A
  • Headache
  • Anorexia
  • Fever
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10
Q

5 types of seizures

A 
• Neonatal
• Febrile
• Infantile Spasms
(shalom seizures)
• Petit mal
• Generalized
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11
Q

What are some warning signs of a brain tumor in children?

A 
o Headaches (usually worse in the
morning or at night)
o Nausea or vomiting
o Changes in speech, vision, or
hearing
o Problems balancing or walking o Changes in mood, personality or
ability to concentrate
o Problems with memory
o Muscle jerking or twitching
(seizures or convulsions)
o Numbness or tingling in the arms or
leg
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12
Q

Pseudotumor Cerebri

A

Idiopathic intracranial hypertension (IIH) - unknown etiology
o Exposure to or withdrawal from certain exogenous substances (eg, drugs)
o Systemic diseases (including Lyme disease)
o Disruption of cerebral venous flow
o Certain endocrine or metabolic disorders
o Predominantly affects obese women of childbearing age

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13
Q

Symptoms of increased ICP or papillodema

A

o Headaches, typically nonspecific and varying in type, location, and frequency
o Diplopia, usually horizontal but rarely vertical
o Pulsatile tinnitus
o Radicular pain, usually in the
arms

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14
Q

Symptoms of papillodema

A

o Transient visual obscurations, often predominantly or uniformly orthostatic
o Progressive loss of peripheral vision in one or both eyes
Fredenburg Fall 2014
o Blurring and distortion (ie, metamorphopsia) of central vision
o Sudden visual loss

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15
Q

Treatment of pseudotumor cerebri

A 
o Pharmacologic
• Acetazolamide and
furosemide
• Headache prophylaxis 
• Corticosteroids
o Optic nerve sheath fenestration (decompression)
o Cerebrospinal fluid (CSF) diversion
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16
Q

Epidemiology of Primary Nocturnal Enuresis

A

o 4 years old, 25% of children
frequently wet the bed
o 7 years old, only 5-10% still wet the bed

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17
Q

Primary Nocturnal Enuresis treatment

A 
UA should be ordered
o Behavioral, including limiting
fluids prior to bedtime
o Alarm therapy
o DDAVP, anticholinergics, tricyclics
o ENT if signs of obstructive
18
Q

Neurofibromatosis type 1 & 2

A

Multisystem genetic disorder that commonly is associated with cutaneous, neurologic, and orthopedic manifestations

19
Q

NF- 1

A

common cutaneous findings
• Complications include visual loss 2nd to optic nerve gliomas, spinal cord tumors, scoliosis, vascular lesions, long bone abnormalities
Axillary or inguinal freckles
• Two or more typical neurofibromas or one
plexiform neurofibroma
• Optic nerve glioma
• Prognosis better with lower frequency of CNS tumors

20
Q

NF-2(central NF)

A

Paucity of cutaneous findings

Relative high incidence of meningiomas and acoustic neuromas

21
Q

Von Hippel-Lindau disease (retinocerebellar angiomatosis)

A

autosomal dominent
symptoms begin in the 2nd and 3rd decades of life
visions issues, blindness, H/A, retinal hemorrhage/detachment, glaucoma

22
Q

Tuberous sclerosis (Borneville’s disease)

A

autosomal dominent
– Cutaneous – ash leaf spots, Shagreen patch, angiofibromas
– Neurologic – seizures, intracranial calcifications
– Retinal, renal, pulmonary, musculoskeletal manifestations

23
Q

What is often the 1st presenting sign of tuberous sclerosis?

A

seizures

24
Q

Sturge-Weber syndrome

A

Characterized by congenital facial port-wine stains and leptomeningeal vascular angiomatosis, with malformations most commonly occur in the leptomeninges, facial capillaries, and ocular vessels. Leptomeningeal angiomatosis can present clinically as epilepsy, mental retardation, and hemiplegia

25
Q

Ataxia-Telangiectasia (Louis-Bar disease)

A

autosomal recessive
progressive cerebellar ataxia, oculocutaneous telangiectasia, abnormalities in cellular and humoral immunity, and recurrent viral and bacterial infections

26
Q

Telangiectasias develop when?

A

aged 3-6 years and are noted first on the bulbar conjunctiva and ears
• Later, they appear on the flexor surface of the arms, eyelids, malar area of the face, and upper chest.

27
Q

Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia - HHT)

A

‘Rare’ autosomal dominant disorder involving mucous membranes and results in a tendency for bleeding

28
Q

Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia - HHT) presentation

A 
- spontaneous recurrent epistaxis
– Skin telangiectases-
– Hepaticorpulmonary involvement (AVMs) 
– GI bleeding
– CNS lesions 
- multi pulmonary nodes of CXR
29
Q

Spastic gait might be due to lesions to?

A

Upper motor neuron lesions, such as brain anoxia and cerebral palsy, may be detected in the spastic gait (toe walking and/or scissoring) of an afflicted child

30
Q

Slapping gait is the destruction of?

A

Destruction of the dorsal columns may result from neurosyphilis or spinal column space-occupying lesions, leading to loss of proprioception and subsequent slapping gait

31
Q

What can cause steppage gait

A

Peripheral nerve palsies, such as hereditary motor sensory neuropathy (including Charcot-Marie-Tooth disease) or posttraumatic peroneal nerve palsy, can result in a steppage gait

32
Q

What can often lead to antalgic gait

A

Musculoskeletal pathology (traumatic, infectious, inflammatory or rheumatologic) of the lower extremity and back most often lead to an antalgic gait

33
Q

What can cause trendelenburg gait?

A

Joint pathology, such as idiopathic avascular necrosis of the hip (Legg-Calve- Perthes disease), may cause a Trendelenburg gait as described below.

34
Q

Birth defects are classified into 3 sequences

A

malformation sequence
deformation sequence
disruption sequence

35
Q

malformation sequence

A

in which an intrinsic malformation exists in the embryo, resulting in certain other abnormalities

36
Q

deformation sequence

A

no intrinsic defect is found in the embryo; rather, an abnormal external mechanical or structural force results in secondary distortion or deformation

37
Q

disruption sequence

A

the healthy embryo is subjected to tissue breakdown or injury

38
Q

Vascular/ Avascular Bone Cysts

A

benign osteoblastic tumor
o Single bone or several bone
o Occur in the cortex of the shafts of long bones in 80-90% of cases
NSAIDs, ASA and surgery are the treatment

39
Q

Unicameral Bone Cyst

A

Common, benign, fluid-filled lesion found almost exclusively in children (etiology “not elucidated)
MC after having a fracture

40
Q

Epidemiology and Prognosis of JRA

A

300,000 children have arthritis in U.S
Incidence rate for JIA range from 4-14 cases per 100,000 children annually
o Almost all children with JIA lead productive lives
o Many patients, particularly those with polyarticular disease, may have problems with active disease throughout adulthood

41
Q

Signs and symptoms of congenital hypothyroidism

A 
o Decreased activity
o Large anterior fontanelle
o Poor feeding and weight gain
o Small stature or poor growth
o Jaundice
o Decreased stooling or
constipation o Hypotonia
o Hoarse cry
42
Q

Mucopolysaccharidosis

A

Defective activity of the lysosomal enzymes

• Blocks degradation of mucopolysaccharides

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