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Flashcards in Nephrology Deck (42)
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Congenital anomalies- pediatric nephrology

Antenatal hydronephrosis


Hematuria & Glomerular Disease (4)

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


Diseases of Renal Vessels

o Renal artery stenosis/thrombosis


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


Potters Syndrome is?

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


Hemolytic Uremic Syndrome (HUS)

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


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


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


3 Prodome for Henoch Scholein Purpura

• Headache
• Anorexia
• Fever


5 types of seizures

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


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
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


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


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


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


Treatment of pseudotumor cerebri

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


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


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


Neurofibromatosis type 1 & 2

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


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


NF-2(central NF)

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


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


Tuberous sclerosis (Borneville’s disease)

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


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



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


Ataxia-Telangiectasia (Louis-Bar disease)

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


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.


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

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


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


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


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