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Differential Diagnosis Cover Desktop

Differential Diagnosis

Is it sepsis or SCPCD?

Ruling out sepsis as a cause of purpura fulminans (PF)

One of the difficulties in diagnosing the cause of PF in neonates is that disseminated intravascular coagulation (DIC) is associated with both sepsis and Severe Congenital Protein C Deficiency (SCPCD).1

For proper management of PF, diagnosing the underlying cause is crucial.1,2

 

Sepsis vs. SCPCD: Differences in clinical and laboratory FINDINGS

PF due to
SEPSIS?

LESIONS THAT:1

  • Develop in distal extremities and progress proximally
  • Appear as a generalised or diffuse rash affecting entire body surface

UNDETECTABLE PROTEIN C LEVELS1*

ISOLATION OF CAUSATIVE AGENT1

e.g. Neisseria meningitides, streptococcus pneumoniae, Group A and B streptococci, etc.1

LABORATORY EVIDENCE OF A SEVERE ACUTE PHASE REACTION1

RESPONSE TO ANTIMICROBIAL THERAPY1

PF due to
SCPCD?

SKINS LESIONS THAT:1

  • Develop on the lower limbs and male genitalia
  • Form at pressure points, such as the heels and buttocks

UNDETECTABLE PROTEIN C LEVELS2,3*

  • In carriers of a protein C mutation, protein S levels are higher than non-carriers5

CEREBRAL VENOUS THROMBOSIS1

Blindness arising from vitreal bleeding, retinal vein, artery or vitreal vein thrombosis with retinal detachment in the form of leukocoria or ischaemic optic atrophy1

CONSANGUINEOUS PARENTS AND A POTENTIAL HISTORY OF MISCARRIAGES1

*However, DIC, which can also indirectly result from sepsis, is associated with reduced Protein C and S levels because of consumption.1

Differential diagnosis can be PERFORMED by:

PROTEIN C (PC) ACTIVITY ASSAY1,3

Protein S assay1 (low PC levels but normal Protein S levels are suggestive of SCPCD)4

PC antigen5

Full blood count (Haemoglobin, White blood cells, Platelets)5,6

Fibrinogen2,5

D-dimer5

Prothrombin Time (PT)2,5

Activated partial prothrombin time2,5

Genetic analysis2

To rule out sepsis:

Blood culture7 and procalcitonin levels8 (to test for bacterial infections including Meningococcal, Streptococcus, Haemophilus and Staphylococcus sepsis)1

PC level testing in parents1

PC activity assay and PC antigen

MRI (to check for cerebral venous thrombosis)1

References:

  1. Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.

  2. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

  3. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  4. Libourel, EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. British Journal of Haematology. 2002;118:615–618.

  5. Khor, B, et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440–442.

  6. Tairaku S, et al. Prenatal genetic testing for familial severe congenital protein C deficiency. Hum Genome Var. 2015;2:15017.

  7. Rhodes A, et al. Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016. Intensive Care Med. 2017;43(3):304-377.

  8. Wacker C, et al. Procalcitonin as a diagnostic marker for sepsis: a systematic review and meta-analysis. Lancet Infect Dis. 2013;13(5):426-435.

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