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Purpose: To assess dry eye disease (DED) in thalassemic adolescents by evaluating corneal epithelial thickness (CET) and various dry eye clinical tests and correlate them to tissue iron overload. Methods: The study included 120 Beta-thalassemia patients (11 to 18 years) and 120 matched controls. CET maps were captured using anterior segment optical coherence tomography. OSDI questionnaire was completed. Dry eye tests included Schirmer test, tear film breakup time ‎‎ (TBUT), and ocular surface staining (OSS) with fluorescein and lissamine green. We recorded serum ferritin ‎level, and liver iron concentration (LIC) measured by magnetic resonance imaging. Results: Superior and inferior CET was thinner, while map standard deviation (MSD) was higher in thalassemics compared to controls (all P‎< 0.001‎). Thalassemic group also showed higher OSDI scores‎ (P‎< 0.001), shorter TBUT ‎‎(P‎< 0.001‎), and higher OSS grades (P‎< 0.001‎). Both superior and inferior CET was correlated positively with TBUT, and negatively with OSS (all P < 0.001). Serum ferritin and LIC showed negative correlations with CET (superior and inferior, both P< 0.001), positive correlations with MSD, P< 0.001, as well as with TBUT (P< 0.001), OSS ‎(P< 0.001), and OSDI scores (P< 0.001). Conclusion: Thalassemic adolescents had thinner CET with higher thickness' variability, shorter TBUT and more marked OSS‎ than controls. Correlation of higher serum ferritin and hepatic iron overload with irregular epithelial thinning and more affected dry eye tests results supports our hypothesis that high tissue iron levels could play a pivotal role in DED pathogenesis in thalassemic patients. [ABSTRACT FROM AUTHOR]

Background Long-term survivors after hematopoietic stem cell transplant (HSCT) may encounter issues that lower their quality of life. Data on the psychological effects of HSCT in childhood are scarce. Morever, the effect of HSCT on cognitive function is regarded as a key issue. Objectives Assess survivors of HSCT for cognitive or psychological affection and explore their quality of life after HSCT. Patients and methods A cross-sectional Study was conducted on 25 long-term survivors of HSCT. The children psychological aspects were assessed using Pediatric Symptom Checklist (PSC), Self-esteem score of Rosenberg, Children Depression Inventory, Children anxiety scale, Pediatric Quality of Life (QOL),Wechsler III for IQ. Results The psychological evaluation scores of 14 males and 11 females showed that 28% of survivors had mild anxiety, 40% had a moderate anxiety and 32% had sever anxiety, according to PSC, 36% only reported emotional and behavioral problems. Regarding depression scale, 36% had mild depression and 12% had moderate depression and only 8% had severe depression. There was significant positive correlation between the survival period after transplantation and IQ (P value 0.008). There was statistically significant association between post-transplant infections and lower QOL (P value 0.047). Another significant association between chronic GVHD and lower self-esteem score (P value 0.031). Conclusion These findings brought to light ongoing issues with the cognitive and psychological evaluation of pediatric long-term survivors of HSCT, as well as the impact of chronic GVHD and post-transplant infection on their psychological evaluation, particularly on self-esteem score and QOL, respectively. Therefore, it is crucial to create focused treatments for pediatric HSCT care in Egypt. Psychologists must be a part of the transplantation care team. [ABSTRACT FROM AUTHOR]

Background: Transfusion is critical in the management of sickle cell disease (SCD) complications. The resultant alloimmunization to RBC group antigens is a major complication of allogeneic blood transfusion and generally presents significant challenges in the management of SCD patients. Aim: To measure the frequency of the occurrence of the alloimmune markers in sickle cell disease and to investigate its predicators. Subjects and methods: This cross-sectional study included 50 children and young adults with SCD, all patients were subjected to detailed medical history thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis and serum ferritin. The D, C, c, E, e, M, NJKa and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s and Anti Lua antigens were typed by commercially prepared polyclonal anti- human sera. Antibody screening was performed using Ortho screening panel. Results: The study Sickle SS was the most common type of SCD representing more than half of the recruited patients. Frequency of antibody positive screening among SCD was 16%. Comparison between SCD patients with positive and those with negative antibody screening showed that higher frequency of positive antibody screening in those with SBþ than those SS and SB0. Patient with positive antibodies screening were significantly older when they received their first blood transfusion and had lower transfusion index (p=0.037) than those with negative antibodies screening (p=0.013). There were no significant difference between patients with positive and those with negative antibodies screening as regards age, gender, transfusion frequency and as regards comorbidities; painful crisis (p=0.117), stroke (p=0.398), ACS (p=0.363). Conclusion: The distribution of the blood group antigens in patients with SCD has important clinical significance especially those who often require regular blood transfusion and who may have developed multiple antibodies. The resultant alloimmunization is serious necessitates early recognition and management. [ABSTRACT FROM AUTHOR]

Background: Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim: To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method: This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results: In our study, there were 30 patients with mean age (14.1 ± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient had abnormal visual acuity A 29 years old girl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion: we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy. [ABSTRACT FROM AUTHOR]

Background: Occult hemorrhage can occur in any internal organ in ITP patients. Four sites of occult hemorrhage deserve special attention including microscopic hematuria, fecal occult blood, retinal hemorrhage, and silent intracranial hemorrhage. Aim: The aim of this study was to investigate for the frequency of subclinical bleeding in Egyptian children with ITP and its relation to different clinical and laboratory parameters of the disease. Methods: This cross-sectional study included 40 ITP patients recruited from the Pediatric Hematology& Oncology unit, Children Hospital, Ain Shams University. Occult blood in stools and urine analysis, fundus examination, and non-contrast brain MRI, for brain microbleed, were done. Results: The total number of patients with occult bleeds was eleven. Two patients had occult blood in stool, five had microscopic hematuria, one had retinal bleeds and three patients had brain microbleeds. Nine patients with occult bleeding were chronic, one persistent and one acute ITP patients. Their mean age was 10.23 6 4.18 and their mean initial bleeding score was 2.55 6 0.82.There were no significant differences between patients with occult bleeding and those without as regards the platelet counts & hemoglobin level, as well as the mean platelet counts & mean hemoglobin level over the disease duration. Conclusion: Our results suggest that subclinical bleeding is a potential risk in children with ITP, more commonly chronic ITP patients. We could not demonstrate a significant relation of occult bleeding to the laboratory findings; nevertheless, the significance of the routine assessment of occult bleeding in ITP and the identification of high-risk patients require additional studies. [ABSTRACT FROM AUTHOR]