Prevalence of COVID-19 in Egyptian Children With Hemoglobinopathies and Inherited Anemias.

Since the World Health Organization (WHO) announced coronavirus disease-2019 (COVID-19) to be a pandemic, children's COVID-19 cases were generally less severe than adults. The aim of the study was to determine the prevalence of COVID-19 cases among children with hemoglobinopathies and other inherited anemias living in El-Minya Governorate, Egypt, who are at high risk of exposure to infection. This cross-sectional study evaluated data from 258 children with hemoglobinopathies and inherited anemias. A questionnaire was used to collect data about COVID-19 symptoms coupled with appropriate investigations (complete blood count, d-dimer, anti-COVID antibodies, chest computed tomography scans, and polymerase chain reaction). We found 38 of 258 (14.7%) children had mild to moderate COVID-19, while there were no cases with severe form of COVID-19. COVID-19 cases were significantly older (8.63±3.37 vs. 6.71±3.56 y, P =0.01), noncompliant to iron chelators (63.2% vs. 11.8%, P =0.01), had higher serum ferritin (2639.47±835.06 vs. 1038.95±629.87 ng/mL, P <0.0001) and serum iron levels (803.68±261.36 vs. 374.18±156.15 µg/dL, P <0.0001) and more frequently had undergone splenectomy (78.9% vs. 25.5%; P <0.0001) than non-COVID-19 cases. In conclusion, only 14.7% of children with hemoglobinopathies and inherited anemias were recorded to have contracted mild to moderate COVID-19, with no reported severe cases.

Diagnostic value of monocyte chemoattractant Protein-1, soluble mannose receptor, Presepsin, and Procalcitonin in critically ill children admitted with suspected sepsis.

INTRODUCTION: The differentiation between systemic inflammatory response syndrome and sepsis is very important as it determines essential treatment decisions, such as selection, initiation, and duration of antibiotic therapy. OBJECTIVES: We aimed to investigate the diagnostic value of Procalcitonin, Monocyte Chemoattractant Protein-1, soluble Mannose Receptor, Presepsin as early biomarkers of pediatric sepsis in comparison to systemic inflammatory response syndrome in severely ill children. PATIENTS AND METHODS: This study included 58 children diagnosed as sepsis (group 1), 24 children with systemic inflammatory response syndrome without infection (group 2), and 50 healthy children as controls (group 3). All the plasma levels of the studied biomarkers were measured and ROC curves were created for all the tested parameters to discriminate between sepsis and SIRS. RESULTS: The area under the curve for Monocyte Chemoattractant Protein-1 was 0.926 (0.846-0.927) with sensitivity 100% and specificity 62.5%. The soluble Mannose Receptor had the highest sensitivity (100%), with AUC equals 1(.0.956-1.0) and specificity of 100%. The cut-off values for Procalcitonin, Presepsin, soluble Mannose Receptor, and Monocyte Chemoattractant Protein-1 and were: 0.62 ng/ml, 100 pg/ml, 13 ng/ml and 90 pg/ml, respectively. In septic cases, both soluble Mannose Receptor and Procalcitonin have positive correlations with the severity of sepsis, low Glasgow Coma Scale, ventilatory support, use of inotropic drugs and mortality rate (r = 0.950, 0.812, 0.795, 0.732 and 0.861respectively) for soluble Mannose Receptor and (0.536, 0.473, 0.422, 0.305 and 0.474 respectively) for Procalcitonin. CONCLUSION: Soluble Mannose Receptor, Presepsin, and Monocyte Chemoattractant Protein-1 can be used to differentiate between sepsis and SIRS in critically ill children.

The effect of zinc deficiency and iron overload on endocrine and exocrine pancreatic function in children with transfusion-dependent thalassemia: a cross-sectional study.

BACKGROUND: Children with transfusion-dependent thalassemia (TDT) suffer from secondary hemosiderosis and the delirious effects this iron overload has on their different body organs, including the pancreas. They are also more prone to develop zinc deficiency than the general pediatric population. This study aimed to determine the effect of zinc deficiency and iron overload on the endocrine and exocrine pancreas in TDT children. METHODS: Eighty children, already diagnosed with TDT, were included in this study. We assessed the following in the participant children: serum ferritin, serum zinc, endocrine pancreatic function (oral glucose tolerance test (OGTT), fasting insulin level and from them, HOMA-IR was calculated), and exocrine pancreatic function (serum lipase and serum amylase). RESULTS: Forty-four TDT children had a subnormal zinc level, while 36 of them had a normal serum zinc level. TDT children with low serum zinc had significantly more impaired endocrine pancreatic function and an abnormally high serum lipase than children with normal serum zinc, p < 0.05 in all. Serum zinc was significantly lower in TDT children with serum ferritin above the ferritin threshold (≥2500 ng/ml) than those below (59.1 ± 20.2 vs. 77.5 ± 28.13), p = 0.02. TDT children, having a serum ferritin ≥2500 ng/ml, had significantly more frequently impaired endocrine pancreatic function and abnormally high serum lipase than TDT children below the ferritin threshold, p < 0.05 in all. CONCLUSION: In children with transfusion-dependent thalassemia, zinc deficiency aggravates iron-induced pancreatic exocrine and endocrine dysfunction.

Admission platelet count and indices as predictors of outcome in children with severe Sepsis: a prospective hospital-based study.

BACKGROUND: Sepsis is still one of the main causes of infants and children mortality especially in developing, economically challenged countries with limited resources. Our objective in this study was to determine, the prognostic value of platelet count, mean platelet volume (MPV), platelet distribution width (PDW) and plateletcrit (PCT) in critically ill infants and children with severe sepsis, as they are readily available biomarkers, that can guide clinicians during managing of severe sepsis. METHODS: Sixty children were included; they were diagnosed with severe sepsis according to the international pediatric sepsis consensus conference criteria. At admission to Pediatric intensive care unit, complete blood count with platelet count and parameters (MPV, PDW and PCT) and C-reactive protein (CRP) level were determined for all children. Also, assessment of the Pediatric Risk of Mortality (PRISM III) score was done to all. These children were followed up till discharge from hospital or death. Accordingly, they were grouped into: (1) Survivor group: included 41 children. (2) Non-survivor group: included 19 children. RESULTS: Platelet count and PCT were significantly lower (p < 0.001) and MPV was significantly higher in non-survivor than survivors (p = 0.004). MPV/PLT, MPV/PCT, PDW/PLT, PDW/PCT ratios were found to be significantly higher in the non-survivors than survivor (p < 0.001 in all). PCT with sensitivity = 94.74%, was the most sensitive platelet parameter for prediction of death, while MPV/PCT was the most sensitive ratio (sensitivity = 94.7%). CONCLUSION: Thrombocytopenia, platelet indices and their ratios, especially plateletcrit and MPV/PCT, are readily available, sensitive, prognostic markers, that can identify the severe sepsis patients with poorest outcome.

Evaluation of serum soluble urokinase plasminogen activator receptor as a marker for steroid-responsiveness in children with primary nephrotic syndrome.

Nephrotic syndrome (NS) is the most common primary glomerular disease among children. It runs a relapsing course involving prolonged periods of treatment with corticosteroids and other immunosuppressive medications. Soluble urokinase plasminogen activator receptor (suPAR) has been regarded as an inflammatory as well as a permeability factor. The aim of our study was to evaluate serum suPAR levels in children with NS and its relation to steroid responsiveness. Our study was carried out on 75 children who were already diagnosed as having NS; they were classified into three groups (steroid sensitive, steroid dependent, and steroid resistant). Furthermore, 40 apparently healthy children, age and sex matched with the NS patients, were enrolled as controls. All children had undergone assessment of serum suPAR, renal function tests (urea and creatinine), serum albumin, C-reactive protein, and 24-h protein in urine. The study found that suPAR level was significantly different between the studied groups (P <0.05), being highest in steroid-resistant NS (66.52 ± 9.7 ng/mL), followed by steroid dependent (56.82 ± 11.09 ng/mL), and steroid-sensitive patients (26.22 ± 3.86 ng/mL), and lowest in the control group (20.29 ± 0.69 ng/mL). When receiver operating characteristics curves were plotted, suPAR had high sensitivities and specificities in predicting steroid responsiveness, [area under the curve (AUC) = 0.99, 95% confidence interval (CI) = 0.911-1.000, P <0.001], steroid dependence (AUC = 1.00, 95% CI = 0.929-1.000, P <0.001), and predicting steroid resistance. Our study indicates that suPAR is significantly higher in children with primary NS and varies according to their response to steroid therapy. It may act as a marker for steroid responsiveness in these children.

Health-related quality of life in pediatric and adolescent patients with transfusion-dependent ß-thalassemia in upper Egypt (single center study).

BACKGROUND: Thalassemia is a major health problem that disturbs the lifestyle of the affected patient. The aim of this work is to detect the impact of thalassemia on the quality of life regarding physical, social, emotional, psychological scored assessment. METHODS: A case-control survey was conducted in Minia University children's hospital on 64 patients recruiting pediatric hematology outpatient clinic from July 2014 to February 2017. PedsQL™ 4.0 Generic Core Scale (Arabic version) was used to assess HRQOL in 64 thalassemia patients between 8 and 18 years of ages. Other related clinical data of the involved patients were collected from the pediatric hematology records. RESULTS: Mean physical, emotional, social, school performance, psychological and total scores (- 36.9 ± 20.9, 49.4 ± 17, 47.2 ± 21.3, 38.5 ± 15.5, 45.3 ± 13.8, 47.9 ± 38.8 respectively) were significantly decreased compared with control (p = 0.001 for all). The younger age group had better scores regarding social, emotional, psychological and total scores compared to older ones (p = 0.01, 0.03, 0.01 and 0.009 respectively). Older age of starting transfusion was statistically significant protecting factor from poor physical QOL in thalassemia patients (OR = 0.96, p = 0.03). The presence of hepatomegaly was a statistically significant predictor for poor physical QOL (OR = 8.5, p = 0.02). Household income was the statistically significant predictor for poor emotional QOL (OR = 5.03, p = 0.04). High serum ferritin was the statistically significant predictor for poor social QOL (OR = 1.1, CI 95%=, p = 0.04). Regarding poor psychological QOL (OR = 0.94, p = 0.01) and total QOL (OR = 0.94, p = 0.01) scores, older age of starting transfusion was the statistically significant protecting factor. CONCLUSION: Scheduled programs giving psychosocial help and a network connecting between the patients, school officials, thalassemia caregivers and the physician is required especially in developing countries where the health services are not integrated with social organizations. Special school services for thalassemia patients are required to deal with the repeated absence and anemia induced low mental performance of thalassemia children.

The effect of anti-thyroid antibodies positivity on children with primary immune thrombocytopenia.

Primary immune thrombocytopenia (ITP) is the most common cause of acquired thrombocytopenia in children. Anti-thyroid antibodies (aTA) have previously been found to be present in various autoimmune diseases. Our aim was to study the effect of aTA positivity (which are anti-thyroid peroxidase (aTPo) and/or anti-thyroglobulin (aTg)) on children with primary immune thrombocytopenia and their relation to treatment response. Sixty-one children with primary ITP were enrolled in the present study. They were further subdivided into: ND&P group (newly diagnosed and persistent) and chronic ITP group. Seventy-five apparently healthy children were enrolled as control group. aTPo and aTg antibodies were significantly higher and more frequently positive in all children with ITP and in each ITP group than the control group (P <.05 in all). But, there were no statistically significant differences between the two ITP subgroups (P >.05). aTA positive children with ITP had significantly lower platelet count: at the start of treatment (P =.009), after receiving methylprednisolone or intravenous immunoglobulin (P =.02) and at one month follow-up (P =.003) than aTA negative children with ITP. Lastly, aTA positive children had more relapses (P =.03), continued more frequently to have relapses after one year in the ND&P group (P =.02) and required immunosuppressive therapy more frequently in the chronic ITP group (P =.005).