Hem1 deficient mouse models exhibited remarkable clinical and pathological similarities to their human counterparts including increased susceptibility to bacterial and viral infections, hyperinflammation in multiple tissues and organs (hepatitis, enteritis, colitis, otitis, endocarditis, bronchiolitis, pneumonia etc), atopic diseases (eczema, etc), hepatosplenomegaly, thrombosis, autoimmunity (Lupus like disease, glomerulonephrosis, increased autoantibodies, etc), and failure to thrive overall. The gene discussed is NCKAP1L; the disease is pneumonia.