A Rare Case of "Periportal Cuffing" as an Incidental Finding on 18F-FDG PET/CT.

A 7-year-old boy with known diagnosis of hereditary spherocytosis and ulcerative colitis was referred for 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography after detection of a 28 mm lesion suspicious for malignancy in spleen on upper abdomen magnetic resonance imaging (MRI). As an incidental finding, a moderately increased uptake of 18F-FDG was observed in periportal region with no definable mass. MRI revealed compatible findings with "periportal cuffing" as described on ultrasonography.

Turkish Validity-Reliability Study of the Celiac Disease-Specific Pediatric Quality of Life Scale.

BACKGROUND: Celiac disease is an autoimmune enteropathy triggered by the presence of gluten. There are Celiac Disease Dutch-Child Quality of Life Scale, Celiac-Specific Pediatric Quality of Life Scale for children/adolescents patients to measure the quality of life. In this study, due to lack of quality of life scales for children with celiac in Turkey, we aimed at Turkish adaptation of the Celiac-Specific Pediatric Quality of Life Scale. METHODS: This methodological study was conducted in Ankara University Faculty of Medicine, Cebeci Hospital Hospital between July 2019 and July 2020. A total of 192 children were included. Reliability was demonstrated by the Cronbach's alpha coefficient. Structural validity was evaluated using explanatory factor analysis and confirmatory factor analysis. The Statistical Package for Social Sciences (SPSS) 22.0 and Amos were used in analyses. RESULTS: In 8-12 age groups; Cronbach's alpha was 0.92 in negative emotions dimension, 0.88 in school dimension, and 0.74 in enjoyment dimension. In explanatory factor analysis, Kaiser-Meyer-Olkin measure of sampling adequacy value was 0.698, Bartlett's test of sphericity was significant (P < .001). Variance explained was 75.8%. In confirmatory factor analysis, X2/df was 3.26, root mean square error of approximation value was 0.07, comparative fit index value was 0.96. In 13-18 age groups; Cronbach's alpha was 0.87 in social dimension, 0.84 in uncertainty dimension, 0.78 in isolation dimension, and 0.83 in limitations dimension. In explanatory factor analysis, Kaiser-Meyer-Olkin measure of sampling adequacy was 0.684, Bartlett's test of sphericity was significant (P < .001). Variance explained was 68.6%. In confirmatory factor analysis, X2/sd value was 3.78, root mean square error of approximation value was 0.061, and comparative fit index value was 0.961. CONCLUSION: Cronbach's alpha values of the groups were found to be above 0.70. Kaiser-Meyer-Olkin values were above 0.5 in terms of sample size, Bartlett's tests for sphericity were significant in terms of correlations between variables, root mean square error of approximation values were below 0.08, comparative fit index and goodness of fit index values were above 0.95 in terms of model fit. If the scales have been found to be valid and reliable, it is recommended for use in Turkey.

Allogeneic hematopoietic stem cell and liver transplantation in a young girl with dedicator of cytokinesis 8 protein deficiency.

DOCK8 deficiency is a rare inherited combined immunodeficiency, caused by mutations in the DOCK8 gene. We describe a case with DOCK8 deficiency associated with severe CLD in whom orthotopic LT was performed successfully after allogeneic HSCT. A 5 year-old girl with DOCK8 deficiency presented with mild direct hyperbilirubinemia and abnormal GGT level and without a previous history of jaundice. She had severe growth retardation, hepatosplenomegaly and generalized eczema. Progressive worsening of CLD was observed within 4 months. Investigations for etiology of liver disease were negative. Liver biopsy showed bridging necrosis, cholestasis and, cirrhosis. Recurrent immune hemolytic crisis and several viral infections developed in follow-up. She underwent whole cadaveric LT for end-stage liver disease (ESLD) 1 year after allogenic HSCT from a full matched related donor. The postoperative course was uneventful. The patient is alive with normal liver function and moderate skin graft versus host disease for 36 months after LT. In conclusion DOCK8 deficiency can be associated with severe CLD. Successful LT following HSCT is possible in patients with ESLD in DOCK8 deficiency. The timing of LT is challenging in patients requiring both HSCT and LT since conditioning regimens for HSCT can be highly hepatotoxic and the patients with suboptimal liver function can become decompensated during HSCT.

Bone mineral density and growth in children with coeliac disease on a gluten free-diet.

BACKGROUND/AIM: To evaluate changes in growth and bone metabolism during consumption of a gluten-free diet (GFD) in children with coeliac disease (CD). MATERIALS AND METHODS: Thirty-seven children with CD (mean age of 8.8 ± 4.6 years, 21 girls) were enrolled. Anthropometric measurements, bone mineral density (BMD) in lumbar 2-4 vertebrae, and serum alkaline phosphatase, calcium, and phosphorus levels at diagnosis and at follow-up were recorded. RESULTS: The mean follow-up period was 3.5 ± 2.3 years. The BMD of patients was significantly lower than that of control subjects at the time of diagnosis but not after 1 year of the GFD. Incidence of low BMD with respect to z-scores for chronological age (CA) was significantly higher than z-scores for height age (HA) (P = 0.006). At the first year of GFD, BMD, BMD z-score, height-for-age z-scores, and weight-for-age z-scores were significantly increased compared with the baseline, but not after 1 year of the GFD. CONCLUSION: In CD, the first year of GFD is important in weight gain, linear growth, and improvement of BMD. A considerable relation of low BMD in children with CD, with respect to z-scores for CA, may be a result of misinterpretation of low BMD due to short stature.