Emergency Department Utilization by Pediatric Patients With Sickle Cell Disease in Basrah, Iraq.

Background Patients with sickle cell disease (SCD) often present in the Emergency Department (ED) with acute and debilitating pain and other SCD-related complications. Objectives The objective is to analyze the causes of ED visits of pediatric patients with SCD, assess the burden of ED admission due to SCD in relation to other pediatric diseases, the treatment given, and the outcomes. Methods A prospective analytical study was conducted on children and adolescents with SCD, 1-14 years old who had been admitted to the ED at Basrah Maternity and Children Hospital over a six-month period. Patient's sociodemographic and clinical data, drug history, length of ED stay, complications, outcome, and readmissions were recorded.  Results A total of 422 patients with SCD were admitted to ED during the study period representing 4.10% of the total admitted cases; 276(65.40%) of them were recruited in this study, and their mean age was 7.84 ±3.47 years. The main cause for ED admission was pain (73.91%), followed by infection (10.14%) and hemolytic crisis (6.15%). The mean duration of stay at ED was 6.11±1.87 hours. All admitted SCD patients had received analgesia; non-steroidal anti-inflammatory drugs (NSAIDs) were the commonest (80.4%), followed by acetaminophen (39.5%), and opioid narcotic (18.5%). Readmission within 30-days was reported in 82(29.71%) patients and was associated with the number of ED visits/last year (B=0.151, P=0.023), length of stay at ED (B=0.140, P=0.034) and severe disease (B=0.253, P<0.001). Conclusions Acute painful episodes were the main cause of ED admission. Although most patients with pain did receive NSAIDs, only a small percentage of them did receive opioids. About one-third of patients have been readmitted within 30 days, and readmission was associated with the number of ED visits/last year, disease severity, and length of ED stay. These findings can help in establishing local guidelines for managing such patients in the ED especially pain management.

The Role of Hyposthenuria in Enuresis Among Paediatric Patients With Sickle Cell Disease.

Objectives: Enuresis is common among children with sickle cell disease (SCD). Many risk factors have been postulated, but its relation to hyposthenuria is debatable. This study aimed to determine the prevalence of enuresis in children with SCD in Basrah, Iraq, and to examine its relation with hyposthenuria. Methods: A cross-sectional epidemiological study was performed on children with SCD who met the inclusion criteria at the Basrah Center for Hereditary Blood Diseases from December 2020 to May 2021. A questionnaire was used to collect relevant data. Blood samples were tested for haemoglobin genotype, certain blood indices and serum haemoglobin. Urine was tested for albumin and creatinine, and the specific gravity was measured using urine dipsticks. The relationships between enuresis and various sociodemographic and clinical variables were assessed. Binary logistic regression analysis was done to examine the independent risk factors of enuresis. Results: A total of 161 out of 200 eligible children were included in this study (response rate: 80.5%). The majority of participants (60.9%) were males. The mean age of the participants was 10.9 ± 2.9 years. Enuresis was reported in 50 (31.1%) patients. The independent risk factors for enuresis included family history of enuresis (adjusted odds ratio [OR] = 5.94, 95% confidence interval [CI]: 2.54-13.89; P <0.001), hyposthenuria (OR = 3.76, 95% CI: 1.25-11.30; P = 0.018) and sleep disorders (OR = 2.90, 95% CI: 1.19-7.06; P = 0.019. Conclusion: Enuresis is common among children with SCD in Basrah, Iraq. Hyposthenuria was significantly associated with enuresis. Family history of enuresis and sleep disorders were also found to be significantly related to enuresis.

Analysis of Macrophage Migration Inhibitory Factor Genotype in Hemophilia a Patients.

BACKGROUND: Hemophilia A, an X-linked bleeding disorder, is caused by a complete or partial deficiency in factor VIII. Multiple factors engage in the development and progression of bleeding episodes in hemophilia patients, especially arthropathy. OBJECTIVES: Detection of macrophage migration inhibitory factor (MIF)-173 G/C polymorphism in people with hemophilia A (PWH) and the possible associations between the type of MIF gene polymorphism and selected disease-related variables. PATIENTS AND METHODS: This case-control study included 95 male patients with hemophilia A and 95 non-hemophiliac subjects, all aged from 2 months to 63 years. An allele-specific polymerase chain reaction (AS-PCR) with a multiplex technique was used to detect MIF polymorphisms. RESULTS: A significantly higher frequency of GG polymorphism was reported in the control group (81, 85.3%) compared to PWH (64, 67.4%), while a significantly higher frequency of GC polymorphism was found in PWH (21, 22.1%) than that in healthy subjects (10, 10.5%), P < 0.05. The G allele polymorphism was detected in 90.0% of the control group compared to 78.4% of PWH (149 subjects), while the C allele frequency was higher in PWH (41, 21.6%) compared to that in healthy individuals (18, 10.0%), P < 0.05. The frequencies of varied MIF-173 polymorphisms did not show significant differences among patients with different clinical presentations or in relation to presence of inhibitors, P > 0.05. CONCLUSIONS: MIF-173 GC polymorphism is seen in PWH more than that in healthy individuals. Further studies are required to detect additional SNPs through sequencing of the MIF gene and to detect MIF serum levels during bleeding episodes.

Renal Dysfunction in Pediatric Patients in Iraq With ß-Thalassemia Major and Intermedia.

Background With optimum transfusion and chelation therapy, the survival of ß-thalassemia patients and the incidence of various complications, including renal complications, have improved. Objectives To investigate renal involvement in ß-thalassemia patients using serum and urinary biochemical markers of glomerular and tubular dysfunction. Methods This case-control study included 69 ß-thalassemia major (ß-TM) patients, 23 ß-thalassemia intermedia (ß-TI) patients, and 100 healthy controls, all ranging from 1 to 16 years in age. Blood urea nitrogen (BUN), serum ferritin, serum and urinary levels of creatinine (Cr), uric acid (UA), calcium (Ca), phosphorus (Ph), magnesium (Mg), sodium (Na), and potassium (K), and the urinary albumin/creatinine ratio were evaluated. Results The BUN level and the urinary Na/Cr, K/Cr, Ca/Cr, Mg/Cr, Ph/Cr, albumin/Cr, and UA/Cr ratios were significantly higher in the ß-thalassemia patients than in the controls. In contrast, the serum Na, K, Ca, and Mg levels were significantly lower in the patients (P<0.05). An elevated urinary UA/Cr ratio was found in 61.9% of ß-thalassemia patients, and an elevated urinary Ca/Cr, and urinary albumin/Cr ratio was found in 53.2%. An elevated Na/Cr ratio was found in 41.3%. The serum and urinary renal markers showed no significant differences between patients with ß-TM and ß-TI, except for microscopic hematuria, which was significantly higher in ß-TI patients (34.8%) than in ß-TM patients (13%), P>0.02. At an older age, high serum ferritin levels and deferoxamine therapy were associated with significant tubular and glomerular dysfunction in ß-thalassemia patients. Conclusions Pediatric patients with ß-thalassemia have significantly abnormal tubular and glomerular functions, necessitating early detection and monitoring to prevent/reverse renal function deterioration.

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq.

Objectives. Despite improvements in the management of sickle cell disease (SCD), many patients still experience disease-related complications requiring hospitalizations. The objectives of this study were to identify causes of hospitalization among these patients and factors associated with the length of hospital stay (LOS) and readmission. Methods. Data from 160 patients (<14 years old) with SCD who were admitted to the Basra Maternity and Children's Hospital from the first of January 2012 through July 2012 were analyzed. Results. The main causes of hospitalization were acute painful crises (73.84%), infections (9.28%), acute chest syndrome (8.02%), and acute splenic sequestration crisis (6.32%). The mean LOS was 4.34 ± 2.85 days. The LOS for patients on hydroxyurea (3.41 ± 2.64 days) was shorter than that for patients who were not (4.59 ± 2.86 days), P < 0.05. The readmission rate (23.1%) was significantly higher among patients with frequent hospitalizations in the previous year (OR 9.352, 95% CI 2.011-43.49), asthma symptoms (OR 4.225, 95% CI 1.125-15.862), and opioid use (OR 6.588, 95% CI 1.104-30.336). Patients on hydroxyurea were less likely to be readmitted (OR 0.082, 95% CI 0.10-0.663). Conclusions. There is a relatively high readmission rate among patients with SCD in Basra. The use of hydroxyurea significantly decreases the LOS and readmission rate.

Health-related quality of life in children and adolescents with hemophilia in Basra, Southern Iraq.

OBJECTIVES: The aim of this study was to assess health-related quality of life in hemophilic children and adolescents, and describe the impact of health status on quality of life. METHODS: The study included 45 patients with hemophilia A and B, their ages ranged from 4 to 16 years. Health-related quality of life was assessed by Hemophilia Quality of Life Questionnaire (Haemo-QOL) US-English long version for 3 age groups (I: 4 to 7; II: 8 to 12; and III: 13 to 16 y). RESULTS: The study did not reveal a significant difference in the Hemophilia Quality of Life total score in relation to age of patients. However, young children are mainly impaired in the dimension family; children aged 8 to 12 years are mainly impaired in the dimension sport and adolescents in perceived support. Severity of hemophilia adversely affects the quality of life; patients with severe hemophilia have the highest total score of 58.51 ± 3.62, followed by moderate type 37.55 ± 5.37, and then mild hemophilia 35.47 ± 4.19, P<0.05. Among children with severe hemophilia, young children who had ≥ 5 joint bleeds during the last year have significant impairment in the total score and in several dimensions including physical health, feeling, and treatment compared with children who had <5 joint bleeds, P<0.05. CONCLUSIONS: Severity of hemophilia adversely affects the quality of life, and clinical severity significantly affects quality of life among patients with severe hemophilia. Thus, the importance of prophylaxis is emphasized in improving the quality of life of our children with hemophilia.