Egyptian Pediatric Guidelines for the Management of Children with Isolated Thrombocytopenia Using the Adapted ADAPTE Methodology-A Limited-Resource Country Perspective.

BACKGROUND: Thrombocytopenia is a prevalent presentation in childhood with a broad spectrum of etiologies, associated findings, and clinical outcomes. Establishing the cause of thrombocytopenia and its proper management have obvious clinical repercussions but may be challenging. This article provides an adaptation of the high-quality Clinical Practice Guidelines (CPGs) of pediatric thrombocytopenia management to suit Egypt's health care context. METHODS: The Adapted ADAPTE methodology was used to identify the high-quality CPGs published between 2010 and 2020. An expert panel screened, assessed and reviewed the CPGs and formulated the adapted consensus recommendations based on the best available evidence. DISCUSSION: The final CPG document provides consensus recommendations and implementation tools on the management of isolated thrombocytopenia in children and adolescents in Egypt. There is a scarcity of evidence to support recommendations for various management protocols. In general, complete clinical assessment, full blood count, and expert analysis of the peripheral blood smear are indicated at initial diagnosis to confirm a bleeding disorder, exclude secondary causes of thrombocytopenia and choose the type of work up required. The International Society of Hemostasis and thrombosis-Bleeding assessment tool (ISTH-SCC BAT) could be used for initial screening of bleeding manifestations. The diagnosis of immune thrombocytopenic purpura (ITP) is based principally on the exclusion of other causes of isolated thrombocytopenia. Future research should report the outcome of this adapted guideline and include cost-analysis evaluations.

Assessment of peripheral blood lymphocyte subsets in children with iron deficiency anemia.

BACKGROUND: Iron plays an important role in body defense and essential for normal immune system development where its deficiency may result in an inadequate immune response. We aimed to assess the lymphocyte subsets in childhood iron deficiency anemia (IDA) with their laboratory correlations. METHODS: Fifty IDA (< 18 years) and 25 age and sex-matched healthy children were enrolled and a complete history was obtained and clinical examination was performed. Complete blood count, serum iron, total iron binding capacity and serum ferritin, were performed. Flow cytometric determination of peripheral blood CD3+, CD4+, CD8+ T-lymphocytes and CD19+ B-lymphocytes and CD4/CD8 ratio were done. RESULTS: Patients had significantly lower hemoglobin, Serum iron, ferritin levels and higher lymphocytic count in patients compared with controls (p = 0.001, 0.03, 0.001, 0.001 respectively). CD3 count and percentage were significantly lower in IDA patients compared to controls (p = 0.007 and 0.005 respectively). There was a Significant reduction in the CD4 count, percentage and CD4/CD8 ratio in patients compared with controls (p = 0.001, 0.001 and 0.005 respectively) while there was no significant difference regarding CD8 count and percentage. No significant difference between the two studied groups regarding either CD19 count or percentage (p = 0.28 and 0.18 respectively) were found. CONCLUSIONS: IDA is associated with impaired cell-mediated immune response specifically T-cell mediated immunity.

Detection of Occult Acute Kidney Injury in Glucose-6-Phosphate Dehydrogenase Deficiency Anemia.

BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency anemia is associated with intravascular hemolysis. The freely filtered hemoglobin can damage the kidney. We aimed to assess any subclinical renal injury in G6PD children. METHODS: Sixty children were included. Thirty G6PD deficiency anemia children were enrolled during the acute hemolytic crisis and after the hemolytic episode had elapsed. Another thirty healthy children were included as controls. Serum cystatin C, creatinine levels, and urinary albumin/creatinine (A/C) ratio were measured, and the glomerular filtration rate (GFR) was calculated. RESULTS: Significantly higher urinary A/C ratio (p=0.001,0.002 respectively) and lower GFR (p=0.001 for both) were found during hemolysis and after the hemolytic episode compared to the controls. Also, significant higher serum cystatin C (p=0.001), creatinine (p=0.05) and A/C (p= 0.001) ratio and insignificant lower GFR (p=0.3) during acute hemolytic crisis compared to the same children after the hemolytic episode subsided. CONCLUSIONS: G6PD deficiency anemia is associated with a variable degree of acute renal injury during acute hemolytic episodes which may persist after elapsing of the hemolytic crises.

Assessment of Vitamin D status in a group of Egyptian children with non alcoholic fatty liver disease (multicenter study).

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is one of the health problems with great burden on the liver that may end with liver cirrhosis and hepatocellular carcinoma. The aim of this work was to assess serum vitamin D level in nonalcoholic fatty liver disease children. METHODS: This cross sectional case control study involved 47 patients with nonalcoholic fatty liver disease selected while recruiting the pediatric hepatology clinics. Their ages ranged from 5-15 years and were compared with 23 healthy age and sex matched children. All involved patients were subjected to careful history taking, clinical examination and for patients and control, anthropometric measures for body mass index (BMI) calculation (plotted on WHO percentile growth charts), aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma glutamyl transferase (GGT), bilirubin (total and direct), serum albumin, creatinine, triglycerides, cholesterol, high density lipoprotein (HDL),low density lipoprotein (LDL), fasting blood glucose and fasting insulin (for calculation of insulin resistance), C reactive protein and serum vitamin D all were assayed. NAFLD was detected by ultrasonography and graded as absent, mild, moderate and severe. RESULTS: Ninety-three percent of NAFLD patients were obese. Significant differences were found between patients and control regarding AST, ALT, ALP, GGT, total and direct bilirubin, serum albumin, creatinine, triglycerides, cholesterol, HDL, fasting blood glucose, fasting insulin, the homeostatic model assessment for insulin resistance (HOMA-IR) and serum vitamin D levels. Significant negative correlation was found between serum vitamin D level and grades of steatosis. CONCLUSIONS: Serum vitamin D level decreases in children with NAFLD. This low serum vitamin D level is associated with higher stages of steatosis but not with BMI.

Assessment of Subclinical Pancreatitis in Epileptic Children With Different Treatment Modalities.

Acute pancreatitis differ in pediatrics and adults. Drug-induced pancreatitis is one of the common causes of pancreatitis in children. This case-control study aimed to assess subclinical pancreatitis in patients with epilepsy treated with different drug regimens. Eighty known patients with epilepsy were enrolled. Forty patients were treated with monotherapy (group I) and 40 were treated with multitherapy (group II) regimens. Twenty age- and sex-matched healthy children were enrolled as control (group III). Serum lipase and amylase were assayed in all included children. Significant differences were found between groups I and III and between groups II and III regarding serum amylase and lipase (P < .001 for all). Significant difference were found between groups I and II (P = .024) and between groups II and III (P = .01) regarding pancreatic duct and body diameters. Significant difference were found between patients with controlled and uncontrolled fits regarding serum amylase (P = .008). In conclusion, subclinical pancreatitis can complicate the treatment with different antiepileptic regimens.

Adiponectin, leptin and insulin levels at birth and in early postnatal life in neonates with hypoxic ischemic encephalopathy.

BACKGROUND: Hypoxic ischemic encephalopathy (HIE) occurs in one to three per 1000 live full-term births. Fifteen to twenty percent will die in the postnatal period, and an additional 25 % will develop severe and permanent neuropsychological sequalae. The control of growth and nutritional status in the fetus and neonate is a complex mechanism, in which also hormones produced by adipose tissue, such as adiponectin and leptin are involved. The aim of this study was to measure the levels of adiponectin, leptin and insulin in neonates with HIE at birth and in early postnatal life and comparing them with normal healthy AGA and SGA neonates. METHODS: This study carried out on 80 full-term neonates born in Minia university hospital during the period from May 2013 to December 2014. They were divided into group I included 25 neonates with HIE and group II included 55 normal healthy neonates (30 appropriate for gestational age (AGA) and 25 small for gestational age (SGA)). Weight, length, head circumference, body mass index (BMI), glucose, adiponectin, leptin and insulin levels were measured for all neonates. Adiponectin, leptin and insulin levels were compared between neonates with HIE and normal healthy neonates as well as between AGA and SGA neonates at birth, 2nd and 6th days of life. RESULTS: Adiponectin and leptin levels were significantly higher at birth then began to decrease during the first postnatal week in all neonates while insulin level increased during the same period. Serum adiponectin levels were significantly lower while serum leptin and insulin levels were significantly higher in neonates with HIE than healthy neonates. In all neonates, the serum adiponectin level was positively correlated at birth with weight, length, BMI and leptin levels but not with insulin level. In neonates with HIE, serum adiponectin level was not correlated with weight, BMI, leptin level or insulin level. In all neonates, the serum leptin level was positively correlated at birth with body weight, height and BMI. In neonates with HIE serum leptin levels were not correlated with weight, BMI or insulin level after birth. There were no correlations between either leptin or adiponectin serum levels or any of the studied parameters in neonates with HIE. CONCLUSIONS: Neonates who are suffering from HIE had lower serum levels of adiponectin and higher serum levels of leptin and insulin than normal healthy neonates at birth and during the early postnatal period. The decline of leptin and increased the insulin levels after birth in all neonates may be important for the stimulation of feeding behavior and the acquisition of energy homeostasis during the early postnatal life. Positive significant correlations between adiponectin, leptin, body weight and body mass indices were present in normal healthy neonates but not in neonates with HIE reflecting the effect of hypoxia on the regulatory mechanisms controlling the adipose tissue functions.

The Role of the Carotid Doppler Examination in the Evaluation of Atherosclerotic Changes in ß-Thalassemia Patients.

BACKGROUND: Iron overload in patients with beta-thalassemia major (BTM) lead to alterations in the arterial structures and the thickness of the carotid arteries. Doppler ultrasound scanning of extra-cranial internal carotid arteries is non-invasive and relatively quick to perform and may identify children at increased risk of stroke that would otherwise be missed. Increased carotid artery intima media thickness (CIMT) is a structural marker for early atherosclerosis and correlates with the vascular risk factors and to the severity and extent of coronary artery disease. OBJECTIVE: To evaluate the role of carotid Doppler examination and CIMT measurement as a predictor of atherosclerotic changes in BTM children with iron overload. PATIENTS AND METHODS: Sixty two children with BTM and, thirty age and sex matched normal controls were included. Complete blood count, ferritin, serum cholesterol were done, as well as carotid Doppler ultrasonography to measure the CIMT in both patients and controls. RESULTS: CIMT of thalassemic patients was significantly increased compared to controls (p=0.001). There was a significant positive correlation between CIMT and patient's age, the duration from first blood transfusion, serum cholesterol and, iron overload parameters as serum ferritin, frequency of blood transfusion, iron chelation. The length of the transfusion period was the highest risk factor, and an inadequate iron chelation was a further risk factor. Significant negative correlation was found between CIMT and hematocrit value while no significant correlation was found between CIMT and weight, height, BMI centiles and Hb level. CONCLUSION: Carotid Doppler is very useful in measurement of CIMT that increased in thalassemic patients that shows a strong relationship with features of iron overload. Routine Doppler measurement of CIMT in these patients is recommended to predict early atherosclerotic changes as well as in follow-up.

Interleukin 1beta level in human colostrum in relation to neonatal hyperbilirubinemia.

Breast-fed infants have higher bilirubin level than formula-fed infants which is of undetermined etiology. The cholestatic effect of cytokines (e.g. IL-1beta, IL-6) is believed to result from the repression of genes that normally mediated the hepatic uptake, metabolism, and biliary excretion of bile salts and bilirubin. The present study aimed to assess the level of interleukin 1beta (IL1beta) in early milk of nursing mothers and its relation to neonatal jaundice. Sixty full term neonates and their mothers were included. They were classified into two groups; group I included Forty neonates patients with physiological jaundice and group 11 included twenty age and sex matched controls neonates without jaundice. Milk samples were collected from mothers for estimation of serum IL-1beta level by ELISA, and blood samples were collected from the neonates for measuring total serum bilirubin (TSB) level. A significant difference (P < 0.01) in the level (pg/dl) of IL-1beta was found in early milk between group I (10.25 +/- 4.23) and group II (3.75 +/- 2.07). The total serum bilirubin level was higher in group I than group II (10.91 +/- 3.25 mg/dl versus 3.88 +/- 0.78 mg/dl) (P < 0.01). A significant positive correlation was found between of IL-1beta in breast milk and total serum bilirubin levels (r = 0.494, P < 0.001). It is concluded that Elevated levels of IL-1beta in the colostrum provide additional data to understand one of path physiologic mechanisms of breast milk jaundice.