PMC7711070_01
Timeline. Graphic display of the clinical course of the disease, complications, and the treatment used in the A-T patient with systemic granulomatosis.
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The replacement therapy with monthly intravenous infusions of polyvalent immunoglobulins (IVIg) was then initiated and the child was followed up systematically (Figure 1). Due to the chronic liver insufficiency with portal hypertension, propranolol, spironolactone, and ursodeoxycholic acid were given on a long-term basis, and blood-derived preparations, somatostatin, terlipressin, tranexamic acid, and vitamin K interim (Figure 1).
Magnetic resonance imaging of the abdominal cavity. Markedly enlarged spleen (19.1 x 8.2 cm) with irregular, nodular reconstruction, with hypointense foci in T1 and T2 dependent images, 5-60 mm large, modeling on vessels, and signal loss flow void foci in the central part of the spleen. The liver enlarged, the right lobe 18 cm, left lobe 14 cm, both lobes showing irregular nodular reconstruction, in T1 image hyperintense, in T2 image hypointense. All images show different phases of one MRI examination in the patient aged 17. (A) Transverse view, T1-weighted gradient-echo HASTE (Turbo spin-echo) sequence, oppose-phase imaging. (B) Transverse view, 3D starVIBE T1-weighted gradient-echo sequence FS (fat-suppressed), contrast protocol. (C) Transverse view, T2 image with SPC pulse sequence. (D) Coronal view, T2 image HASTE FS sequence.
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The abdominal MRI imaging showed features of hepatosplenomegaly, with nodular regenerative hyperplasia of the liver and multiple granulomas in the spleen (Figure 2).