Graft-versus-host disease after pediatric liver transplantation: A diagnostic challenge.

BACKGROUND: Graft-versus-host disease (GVHD) is a rare but serious complication after pediatric liver transplantation (LTx). Early diagnosis is difficult due to nonspecific presenting symptoms and non-pathognomonic skin histopathological features. The aim of this article was to describe a case of pediatric GVHD after LTx and to review available data on pediatric GVHD highlighting the diagnostic difficulty. We also propose a diagnostic algorithm to improve the diagnostic capability and increase clinical awareness about this potentially fatal condition. METHODS: We did a comprehensive literatures review on studies on GvHD following pediatric LTx between 1990 and February 2021, chimerism study by short tandem repeat (STR), HLA typing by sequence-specific oligonucleotide (SSO) method, and flowcytometry crossmatch. RESULTS: Our search yielded 23 case reports. The most common clinical manifestations were fever and rash (91%) followed by diarrhea. Mortality rate was 36.8% mainly due to sepsis and organ failure. Diagnosis was challenging and chimerism study to confirm donor engraftment was performed on only half of the cases. Prevalence of "donor dominant HLA one-way matching" typically occurs in homozygous parents-to-child transplantation was 75% in cases with HLA testing. CONCLUSION: So far, there are no available standard diagnostic criteria for GVHD following pediatric LTx. Recognition of multiple risk factors through proper laboratory assessment can predict the occurrence, and early chimerism study can confirm suggestive clinical manifestation. The strong likelihood of developing GVHD in "donor one-way HLA match" and the severe problems imposed by this complication may justify avoidance of HLA homozygous parent's donation.

Controlled attenuation parameter: A measure of hepatic steatosis in patients with cystic fibrosis.

BACKGROUND: Hepatic steatosis is a common manifestation of CF-related liver disease(CFLD). Controlled attenuation parameter(CAP) measurement during transient elastography(TE) semiquantifies liver steatosis. We examined the relationship between CAP and CFLD severity, clinical factors and liver stiffness measurements(LSM). METHODS: This is a cross-sectional study of CF patients seen for outpatient care between January 2013-March 2014. CFLD severity was categorized as no CFLD, CFLD without portal hypertension(PHTN) and CFLD with PHTN, based on published criteria. RESULTS: 129 patients (median 18.4y; 57% male) had valid CAP. 70(54%) had no CFLD, 44(34%) CFLD without PHTN, and 15(12%) CFLD with PHTN. The median CAP was 210 dB/m (IQR 181-239). Steatosis(CAP ≥230 dB/m) was seen in 27% of subjects without CFLD, 48% in CFLD but no PHTN, and 20% in with CFLD and PHTN(P = .04). CAP was higher for subjects with CFLD without PHTN (P < .05). There was no CAP difference between subjects with no CFLD and those with CFLD and PHTN (P ≥ .65). LSM was not different between no CFLD and CFLD without PHTN (P = .07), but each of these groups had lower LSM compared to subjects with CFLD and PHTN(P < .001 for each). Except for direct bilirubin, CAP was not associated with clinical markers of liver disease. CONCLUSION: CAP was normal in 86(67%) of patients with CF and was not associated with standard clinical markers of liver disease. CAP was higher in patients with liver disease, which could possibly reflect the loss of steatosis observed with progression to cirrhosis and portal hypertension.