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Flashcards in Nephrology Deck (42)
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1

Congenital anomalies- pediatric nephrology

Antenatal hydronephrosis

2

Hematuria & Glomerular Disease (4)

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

3

Diseases of Renal Vessels

o Renal artery stenosis/thrombosis

4

Antenatal Hydronephrosis

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

5

Potters Syndrome is?

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

6

Hemolytic Uremic Syndrome (HUS)

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

7

Hemolytic Uremic Syndrome (HUS) Presentation

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

8

Henoch Scholein Purpura Presentation

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

9

3 Prodome for Henoch Scholein Purpura

• Headache
• Anorexia
• Fever

10

5 types of seizures

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

11

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

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

12

Pseudotumor Cerebri

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

13

Symptoms of increased ICP or papillodema

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

14

Symptoms of papillodema

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

15

Treatment of pseudotumor cerebri

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

16

Epidemiology of Primary Nocturnal Enuresis

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

17

Primary Nocturnal Enuresis treatment

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

Neurofibromatosis type 1 & 2

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

19

NF- 1

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

NF-2(central NF)

Paucity of cutaneous findings
Relative high incidence of meningiomas and acoustic neuromas

21

Von Hippel-Lindau disease (retinocerebellar angiomatosis)

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

22

Tuberous sclerosis (Borneville’s disease)

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

23

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

seizures

24

Sturge-Weber syndrome

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

Ataxia-Telangiectasia (Louis-Bar disease)

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

26

Telangiectasias develop when?

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

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

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

28

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

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

29

Spastic gait might be due to lesions to?

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

Slapping gait is the destruction of?

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