Nephrology Flashcards
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 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
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
arms
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 furosemide • 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?
seizures
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
