Table 6.

Hepatic Veno-Occlusive Disease with Immunodeficiency: Recommended Surveillance

System/ConcernEvaluationFrequency
General Assessment of growthEvery 3-6 mos
Immunodeficiency Measurement of Ig concentrations
  • Bronchoalveolar lavage to diagnose Pneumocystis jirovecii infection
  • Viral & bacterial cultures/PCR
As needed
Liver disease
  • Serum aminotransferases, bilirubin, & albumin
  • Complete blood count incl platelet count to assess for anemia & thrombocytopenia
Every 3-6 mos
Respiratory disease Pulmonary function studiesAnnually once able to perform reliably
Neurologic manifestations Cerebrospinal imaging to identify leukodystrophy or other CNS pathologyWhen clinically indicated

CNS = central nervous system; Ig = immunoglobulin; PCR = polymerase chain reaction

From: Hepatic Veno-Occlusive Disease with Immunodeficiency

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