Rosen & Barkin's 5-Minute Emergency Medicine Consult (333 page)

TREATMENT

Stabilize as clinically indicated

• IV fluids for shock
  
• Packed RBCs for massive GI hemorrhage
  

• Emergent intervention for life-threatening conditions, if any
  
• NSAIDs (ibuprofen):
  
  • Arthralgia
    
• Prednisone:
  
  • Severe abdominal pain once life-threatening pathology excluded
    
  • Painful SC edema or arthritis
    
  • Renal disease (high-dose pulse therapy required with methylprednisolone)
    
  • CNS involvement
    
• Immunosuppressant drugs:
  
  • Severe, life-threatening disease with HSP nephritis
    
• Polyclonal immunoglobulin therapy:
  
  • Severe, life-threatening disease (controversial)
    

Ibuprofen: 600 mg (peds: 5–10 mg/kg per dose) PO q6h

Prednisone: 60 mg (peds: 1–2 mg/kg/24 h) PO daily for 5–7 days

FOLLOW-UP

• Severe abdominal pain
  
• CNS findings
  
• GI bleeding
  
• Intussusception
  
• Evidence of renal failure
  

• Normal platelet count
  
• Normal renal function
  
• Minimal or no abdominal pain
  
• If steroids started, follow up within 24 hr.
  

• GI:
  
  • Severe abdominal pain
    
• Renal:
  
  • Evidence of renal failure or insufficiency
    

Primary care physician:

• Close monitor of BP. Recheck CBC, urinalysis as clinically indicated (recommended for at least 6 mo in children)
  

• Exclude life-threatening causes
  
• NSAIDs are usually adequate
  
• Most patients do not require systemic corticosteroids as it has not been shown to affect the prognosis of HSP nephritis
  

• Chang WL, Yang YH, Wang LC, et al. Renal manifestations in Henoch-Schonlein purpura: A 10-year clinical study.
  Pediatr Nephrol
  . 2005;20:1269–1272.
  
• Chartapisak W, Opastiraku S, Hodson EM, et al. Interventions for preventing and treating kidney disease in Henoch Schonlein purpura: A systemic review.
  Cochrane Database Syst Rev.
  2009;(3):CD005128.
  
• McCarthy HJ, Tizard EJ. Clinical practice: Diagnosis and management of Henoch-Schonlein purpura.
  Eur J Pediatrics
  . 2010;169(6):643–650.
  
• Saulsbury FT. Clinical update: Henoch-Schonlein purpura.
  Lancet
  . 2007;369:976–978.
  
• Trapani S, Mariotti P, Resti M, et al. Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: Three pediatric cases and review of literature.
  Rheumatol Int
  . 2010;30(10):1355–1359.
  
• Zaffanello M, Brugnara M, Franchini M. Therapy for children with Henoch Schonlein purpura nephritis: A systematic review.
  ScientificWorldJournal
  . 2007;7:20–30.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Intussusception
  
• Rash, Pediatric
  

CODES

287.0 Allergic purpura

BASICS

Hepatic encephalopathy (HE) is characterized by changes in behavior, consciousness, and motor disturbances, associated with hepatic insufficiency and the accumulation of substances normally metabolized by the liver. HE may result from a combination of:

• Accumulation of ammonia (NH
  ₃
  ) from:
  
  • Protein degradation by colonic bacteria
    
  • Deamination of glutamine in small bowel, kidney, and muscle.
    
  • Accumulated NH
    ₃
    crosses the blood–brain barrier. Astrocytes uptake NH
    ₃
    and metabolize it into glutamine which causes cellular swelling. Ultimately leads to cerebral edema and cerebral mitochondrial dysfunction.
    
• Accumulation of other neurotoxins:
  
  • Short-chain fatty acids
    
  • Manganese toxicity
    
  • Neurosteroids
    
  • Phenols
    
  • Mercaptans
    
  • Amino acids such as tryptophan
    
• Increased levels of inhibitory neurotransmitters:
  
  • Benzodiazepines
    
  • γ-aminobutyric acid (GABA)
    
  • Serotonin
    
• Decreased levels of excitatory neurotransmitters:
  
  • Glutamate
    
  • Dopamine
    
  • Aspartate
    
  • Catecholamines
    
• Other contributing factors to HE:
  
  • Decreased cerebral blood flow and oxygen
    
  • Increased glucose consumption and possible hypoglycemia
    
  • Zinc deficiency
    
• Genetics:
  
  • Inherited errors of the urea cycle
    

• Classification based on the 11th World Congress of Gastroenterology:
  
  • Type A: HE associated with acute liver injury and fulminant hepatic failure (FHF).
    
  • Type B: HE associated with portosystemic bypass and no intrinsic liver disease.
    
  • Type C: HE associated with cirrhosis and portal hypertension.
    
• Precipitating events:
  
  • GI bleeding (more common in elderly)
    
  • Hypokalemia and hyponatremia.
    
  • Alkalosis decreases renal NH
    ₄
    excretion.
    
  • Sepsis (e.g., spontaneous bacterial peritonitis [SBP])
    
  • Constipation
    
  • Noncompliance with treatment regimen in chronic liver failure
    
  • High-protein diet
    
  • Hypoglycemia
    
  • Hypovolemia (e.g., post large-volume paracentesis)
    
  • Azotemia (e.g., diuretic or diarrhea induced)
    
  • Narcotics or sedatives, including alcohol
    
  • Zinc deficiency as multiple urea cycle enzymes are zinc dependent
    
  • Hepatocellular injury
    
  • Viral- or drug-induced hepatitis
    
  • Post portosystemic shunt placement
    
  • Recurrent encephalopathy can occur without precipitating factors
    

DIAGNOSIS

• Type A: Rapidly progresses to seizures, decerebrate rigidity, coma, and frequently death from cerebral edema.
  
• Type B and C are chronic conditions that may manifest as minimal or overt HE. Overt HE may be classified as episodic or persistent:
  
  • Minimal HE: Characterized by impaired psychomotor speed, visual perception, and attention and concentration; slow mental processing; and memory loss. Only detected by psychometric testing.
    
  • Overt HE: Slow monotonous speech pattern, loss of fine motor skills, hyperreflexia, clonus, hyperventilation, extrapyramidal type movement disorder, seizures, confusion, coma, decerebrate/decorticate posturing.
    
  • Overt HE episodic: Characterized by short period of changes in consciousness over hours to days that usually return to a normal mental state with treatment. In persistent HE, patients do not return to a normal mental state.
    
• Grading (West Haven criteria):
  
  • Stage 0:
    
    • No apparent clinical changes
      
    • Abnormal neurophysiologic and neuropsychological tests
      
    • No asterixis
      
  • Stage I:
    
    • Personality changes, irritability, depression, or euphoria
      
    • Reversal of normal sleep pattern
      
    • Impairment of writing, drawing, addition, subtraction
      
    • Asterixis may be present
      
  • Stage II:
    
    • Significant behavioral changes, often inappropriate
      
    • Lethargy
      
    • Slow responses
      
    • Asterixis
      
    • Slurred speech
      
    • Ataxia
      
  • Stage III:
    
    • Disorientation to time and place
      
    • Amnesia
      
    • Paranoia
      
    • Nystagmus
      
    • Hypoactive reflexes
      
    • Positive Babinski reflex
      
    • Semistupor to stupor
      
  • Stage IV:
    
    • Dilated pupils
      
    • Stupor or coma