Pediatric Sciences Journal最新文献

{"title":"Zinc Supplementation Improves Nocturnal Asthma Symptoms","authors":"Iman Abdelaziz, M. Kotb, N. Yassin, W. Rabie, A. Alsayed, Dina H. Hamed","doi":"10.21608/cupsj.2021.107314.1038","DOIUrl":"https://doi.org/10.21608/cupsj.2021.107314.1038","url":null,"abstract":"Background: Treatment of bronchial asthma aims to control the symptoms of bronchoconstriction and disease modification. Zinc influences bradykinin that promotes inflammation and promotes contraction of smooth muscle in the bronchus. Aim of the work: To test the effect of zinc supplementation on improvement of both clinical symptoms and lung functions in children with bronchial asthma. Methods: Asthma symptoms, severity and pulmonary function tests were performed to 50 children with bronchial asthma before and after zinc supplementation (1mg/kg/ day) as adjuvant therapy. Serum zinc levels were assessed at initial enrollment for all 50 children. Results: The studied cohort comprised 28 boys and 22 girls with age range of 6-13 years and mean age +/SD of 8.88 +/2.24 years. Of them 48 %, 30 % and 22% were diagnosed: moderate persistent asthma, mild persistent and severe persistent asthma respectively. Initial serum zinc level was low in 34% of patients (mean 47.2 ±10.8 μg/dl) and 66% of patients had a normal initial serum zinc level (mean 100.6 ± 27.2 μg/dl). After 8 weeks of zinc supplementation there was a significant improvement in decreasing frequency of attacks (p=0.036), nocturnal symptoms (p˂ 0.001), clinical control of asthma symptoms (p˂ 0.001) and pulmonary functions showed significant improvement in forced expiratory volume in 1 second (FEV1) (p˂0.001) and forced vital capacity (FVC) (p=0.002) but there was no significant difference in the frequency of daytime symptoms (p˃0.05), limitation of activities (p˃0.05) or need for reliever medications (p˃0.05). Conclusion: Zinc is an effective adjuvant in controlling nocturnal symptoms and significantly improves pulmonary functions even if the initial serum zinc level was within the normal range. Level of Evidence of Study: IIB (1).","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"56 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114859752","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Effect of Nutritional Counseling via Printed Recipe Cards Versus Verbal on Growth of a Cohort of Egyptian Children with Cystic Fibrosis","authors":"Dina H. Hamed, Hala Hamdi, A. Saeed, Shrouk Moataz Abdallah","doi":"10.21608/cupsj.2021.94919.1028","DOIUrl":"https://doi.org/10.21608/cupsj.2021.94919.1028","url":null,"abstract":"Background: Nutritional management in cystic fibrosis children has a dramatic effect on growth and survival. Aim of the work: to assess the effect of structured recipe cards nutritional protocol versus verbal nutritional counselling on growth of children cystic fibrosis (CF). Methods: We prospectively studied a cohort study of 40 children recruited from the CF Clinic and following at Nutrition Clinic, Children`s hospital, Cairo University whose caretakers were counselled and were provided with ready-made multiple calculated recipe cards that allowed preparation of specific meals to ensure adequate salt supplementation, high calorie, high fat diet and pancreatic enzyme dose of (1000IU lipase per 1g fat). Anthropometric parameters were recorded at the beginning, after 3 months and 6 months and compared to those of a historical cohort of 13 children with CF who received verbal nutritional counselling only. Results: Malnutrition was highly prevalent with 64% of patients underweight and 70% stunted. Weight z-score, height z-scores, body mass index z score and middle upper arm circumference improved from -2.64, -2-77, -1.41 to -1.9, -2.22, -1 (p<0.001, p= 0.008, p<0.001) respectively after the intervention. Statistically significant elevation of hemoglobin, albumin and sodium from 11.49gm/dl, 3.85g/dl, 136.6mEq/l to 12.3, 4.44, 139 (p<0.001, p<0.001, p=0.04) respectively and statistically significant reduction of total leucocytic count (TLC), C-reactive protein and CRP/ albumin ratio were detected after the intervention. There was no difference between achieved weight, height and BMI z-scores between both groups (p=0.485, p=0.499 and p=0.227 respectively). Mean care taker nutritional counseling session time was 17.9 ±3.8 min for those who received the recipe cards, which was significantly shorter than the mean (48.2±7.3 min) of the other group (p<0.001). Conclusion: Optimization of nutritional status in children with CF patients resulted in significant improvement of anthropometric, laboratory and inflammatory parameters. Providing care takers with recipe cards allowed shorter counselling session time, and achieved same positive effect on growth as those verbally instructed without recipe cards. Level of Evidence of Study: IIB (1).","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"12 2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116776336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Detoxification Genomics in Children with β-Thalassemia Major: Pilot Study of Glutathione S Transferase M1, Pi & Methyltetrahydrofolate Reductase Gene Polymorphisms Combinations in β- Thalassemia Major","authors":"M. Kotb, M. Hamdy, K. Eid, M. Aziz, M. Salam, Hend Abd El Baky, Nabil Lotfi, Niveen Salama","doi":"10.21608/cupsj.2021.55630.1011","DOIUrl":"https://doi.org/10.21608/cupsj.2021.55630.1011","url":null,"abstract":"Background: Children with β-thalassemia major differ regarding age at presentation, transfusion requirements and unpredictable timing, rate and severity of hemolytic crisis. The blood transfusions are associated with iron overload.  Glutathione S transferase M1 (GSTM1) null mutation was reported to be associated with myocardial iron overload with low body iron.  Aim of the Work: To investigate if children with β-thalassemia major have more than a detoxification enzyme defect. Materials and Methods: GSTM1, glutathione S transferase Pi (GSTPi) and methyltetrahydrofolate reductase (MTHFR) polymorphism were studied among 97 children with β-thalassemia major in a cross-sectional study. Results: The studied cohort comprised 24 (24.7%) girls and 73 (75.3%) boys. Mean hemoglobin was 5.9+/- 0.7gm%, serum iron was 145.69 +/- 58.6 mcg% and total iron binding capacity was 222.58 +/-50.66 mcg%. Of them, 68 (70.1%) demonstrated single or multiple polymorphisms (43 had GSTM1, 20 GSTPi and 32 with MTHFR polymorphisms respectively), while 29 (29.2%) did not demonstrate any polymorphism. There was no correlation between type, number of polymorphisms and clinical phenotype. Sample size and cross- sectional nature of our study did not allow genotype-phenotype correlation. Most of studied children express GSTM1, GSTPi and MTHFR gene polymorphism which was not consistent among them.  Conclusion: Children with β-thalassemia major may have one or more than a detoxification/ regeneration potential enzyme gene GSTM1, GSTPi and MTHFR polymorphism. Every child with β-thalassemia major has unique genetic detoxification and regeneration abilities. The detected detoxification defects might explain the lack of predictability of occurrence of hemolytic attacks and their severity. More studies are needed to highlight impact of detoxification and regeneration genomics in β-thalassemia.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"52 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128973590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nasopharyngeal Microbiome Composition is Different Among Children with Bronchial Asthma","authors":"Christine William Shaker Basanti, M. Kotb, A. Sayed, M. AbdElfattah, M. Ghanem, Azza Abdelmegeid","doi":"10.21608/CUPSJ.2021.75415.1019","DOIUrl":"https://doi.org/10.21608/CUPSJ.2021.75415.1019","url":null,"abstract":"Background: The microbiome composition is as unique and diverse as humans are; it may vary substantially over time. A stable microbiome is essential for immune development, while dysbiosis in the microbiome has been associated with an increased risk for asthma and allergies. Knowledge about nasopharyngeal microbiome among Egyptian children with asthma is lacking.  Aim of the work: To study the nasopharyngeal microbiome in a group of Egyptian asthmatic children, and to compare it to a group of healthy controls of similar age and sex distribution. Subjects and Methods: A cross-sectional case-control study was performed on 60 Egyptian children aged 1-13 years. Of those, 30 children suffered un-exacerbated asthma, and 30 children were healthy controls. Nasopharyngeal swabs were obtained from all participants and the nasopharyngeal microbiome was identified using the VITEK 2 system after primary organism isolation by routine cultures.  Results: A statistically significant difference was found for the overall microbiome composition between asthmatic and control children (p=0.007). The most common combination of organisms in asthmatics was Staphylococcus aureus and Streptococcus pneumoniae, and was statistically higher than in controls (0.038). Normal controls had a higher incidence of presence of coagulase negative staphylococci (30%) than the asthmatic group (3.3%) (p-0.009). Kocuria rosea, although present more in asthmatics, the difference did not reach statistical significance. However, within the asthmatic group, the presence of K. rosea was noted more in patients with more severe asthma (p=0.02). Conclusion: The microbiome composition in a group of asthmatic Egyptian children was different from that of healthy controls. Whether this difference has a causative relation to asthma development /severity, or is due to upregulation of certain species to combat certain environmental hazards was beyond the scope of this study and awaits further research.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"212 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132790092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Extrapulmonary Tuberculosis is the Leading Presentation among Hospitalized Children: A Single-Center Experience in a Decade","authors":"Christine William Shaker Basanti, Sonia Al-Saiedi, M. Moawad, Azza Abdelmegeid","doi":"10.21608/cupsj.2021.77577.1021","DOIUrl":"https://doi.org/10.21608/cupsj.2021.77577.1021","url":null,"abstract":"Background: With an incidence of twelve tuberculosis (TB) cases for every 100,000 persons in 2019, Egypt is considered a medium-burden country. Nevertheless, guidelines and studies for admission of pediatric TB cases are lacking in developing countries. Aim of the Work: To describe the clinical presentations of hospitalized pediatric TB cases. Materials and Methods: A retrospective analytical study of data collected from the inpatient medical files of children with confirmed TB, from the archives of Giza Chest Hospital (Omraneya), Egypt was implemented during January 2009 to the end of 2018.  Results: Of the 76 children hospitalized with TB, 42 were males with a male to female ratio of 1.2:1, 61 (80.3%) patients were from rural areas, and 65 (85.5%) were above six years of age. Extra-pulmonary TB was more common (45 patients; 59.2%) than pulmonary TB (31 patients; 40.8%). Tuberculous LN affection was (N=20) 26.2% of total patients, followed by pleural affection (N=17), then affection of bones (N=4). CNS involvement was among least common presentations (N=4; 5.3%), but was the only type of TB to occur more in preschool children. Moreover, there was an annual rate of change of -2.714 in the recorded numbers of pediatric TB inpatients with no cases in the last two years. Conclusion: Extra-pulmonary TB was more common than pulmonary TB among the hospitalized children. Lymph nodes were the most common extra-pulmonary site of affection. School-aged children were a majority as well as rural residents. These findings warrant evoking awareness among physicians about the various presentations of pediatric TB.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"16 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123909472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital Heart Disease Does Not Increase Risk of Bacteremia In Children With Pneumonia","authors":"Azza Abdelmegeid, F. Mostafa, S. Ragab, M. AbdElfattah, H. ElGindy","doi":"10.21608/cupsj.2021.77422.1020","DOIUrl":"https://doi.org/10.21608/cupsj.2021.77422.1020","url":null,"abstract":"Background: Pneumonia is a major cause of childhood morbidity and mortality worldwide. It can be complicated by bacteremia. Congenital heart diseases (CHD) that cause increased pulmonary blood flow are a common predisposing factor for pneumonia in children. Aim of the Work: We aimed to identify if children with pneumonia suffer from bacteremia and to identify  the most common organisms causing bacteremia in cases of pneumonia with and without congenital heart disease and to study role of CHD in influencing type of bacteremia in pneumonia. Patients and Methods: We conducted a pilot prospective study at Pediatric Hospital, Cairo University on 55 children with pneumonia (group 1) and 55 children with pneumonia and CHD (group 2). They all underwent complete blood counts, blood cultures and chest x-ray.  Results: The frequency of bacteremia was 56.4% in children with pneumonia and no CHD and 38.2% in those with pneumonia and CHD. In group with pneumonia and no CHD blood culture results showed no growth in 24 cases (43.6%), Klebsiella spp. growth 11 cases (20%), Coagulase negative staphylococci (CONS) in 6 cases (10.9%), Methicillin Resistant Staphylococcus aureus in 4 cases (7.3%), Candida albicans in 2 cases (3.6%) and Pseudomonas spp. in 2 cases (3.6%). While the blood cultures in the group with pneumonia and CHD showed no growth in 34 cases (61.8%), Klebsiella spp. in 10 cases (18.2%), CONS in 5 cases (9.1%) and Candida albicans in 3 cases (5.5%) and no statistically significant difference was found.  Conclusion: Frequency of bacteremia in children with pneumonia with and without congenital heart disease were not significantly different, the most common organism causing bacteremia in cases of pneumonia with or without congenital heart disease is Klebsiella spp . and the most sensitive antibiotic is polymyxin.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"34 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127780639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effectiveness and Dose of Carvedilol Therapy In Children with Dilated Cardiomyopathy: A Prospective Randomized Double-Blinded Trial","authors":"Zeinab Selim, S. El-Saiedi, R. Ismail, Rania El Kaffas, M. Meabed, B. Hanna","doi":"10.21608/cupsj.2021.64625.1015","DOIUrl":"https://doi.org/10.21608/cupsj.2021.64625.1015","url":null,"abstract":"Background: The efficacy and safety of the beta blocker carvedilol in pediatric patients with chronic heart failure (CHF) has not been fully established till now. Aim of the Work: To assess the role of carvedilol in the treatment of children with dilated cardiomyopathy, to demonstrate its efficacy, tolerability and determine the least effective dose. Patients and Methods: We conducted a double-blind, placebo-controlled study of 53 children with dilated cardiomyopathy. Patients were randomly assigned to receive either placebo (26 patients) or carvedilol (27 patients) added to the standard CHF therapy that consists of angiotensin-converting-enzyme inhibitor and diuretics. The carvedilol group was further subdivided into 2 groups according to the received dose of carvedilol. Subgroup I (11 cases) received carvedilol dose of 0.01-0.09 mg/kg/day and subgroup II (16 cases) received carvedilol with dose increments every 2 weeks from 0.01-0.2 mg/kg/day. All patients were followed up for four months after reaching the target dose. Results: The end results of the study showed that there was a significant improvement in Ross heart failure class, fractional shortening (FS), left ventricle end systolic dimension (LVESD) and left ventricle end diastolic dimension (LVEDD) in patients of the study group which was not achieved in the control group. On the other hand there was no statistically significant difference between sub groups I and II regarding the end point. Carvidolol was well tolerated, with no side effects or drug interaction reported. Conclusion: Addition of carvedilol to standard anti-failure therapy is of beneficial effect and is well tolerated in children with CHF on top of DCM.  There may be no need for carvedilol dose up- titration at least for the first months of treatment.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"501 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132177390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unusual Presentation of Atypical Kawasaki Disease in a Child with Thrombocytopenia and Spontaneously Femoral Artery Leak: A Case Report","authors":"M. Eid, A. Badr, H. Hamza, Y. Sedky","doi":"10.21608/cupsj.2021.61910.1013","DOIUrl":"https://doi.org/10.21608/cupsj.2021.61910.1013","url":null,"abstract":"Kawasaki disease (KD) is an acute systemic vascular disease that affects mostly medium sized and small vessels. The most serious of which is coronary artery disease. Vasculitis involves proliferative granulomatous inflammation and resolves by cicatrization if not treated. It is generally a self-limited disease and its highest incidence is in children under five years.  The diagnostic criteria include the presence of fever for at least 5 days along with four of five other clinical features (rash, mucositis, conjunctival injection, cervical lymphadenopathy or extremity changes). Atypical Kawasaki disease includes patients who meet only 2 or 3 of the 5 criteria for diagnosis. We here report a 4- year old male patient who presented with fever, mucositis, perineal rash and evidence of de novo femoral vasculitis leading to spontaneously leaking femoral arteritis. His complete blood count revealed thrombocytopenia. The patient fulfilled the criteria for the diagnosis of atypical Kawasaki Disease.  He received intravenous immunoglobulins and aspirin in anti-inflammatory doses and his condition improved. We report this unusual presentation of Kawasaki disease with spontaneous femoral artery leak, rash and thrombocytopenia. Diagnosis of atypical Kawasaki disease is very challenging but essential as initiation of specific management is life-saving.","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"133 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116418093","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Echocardiographic Parameters of Severity in Isolated Neonatal Patent Ductus Arteriosus","authors":"B. Hanna, Sandra Nashed, M. Tadros, Zahraa E. Osman","doi":"10.21608/cupsj.2021.79032.1022","DOIUrl":"https://doi.org/10.21608/cupsj.2021.79032.1022","url":null,"abstract":"Background: A hemodynamically-significant patent ductus arteriosus (hsPDA) compromises the early neonatal transition. There is no general agreement on echocardiographic indicators of hsPDA that can predict clinical decompensation. Aim of the Work: We aimed to assess echocardiographic parameters that are associated with the isolated PDA effects on hemodynamics, which could help in subsequent management decision making. Materials and Methods: We conducted a prospective observational analytical study on 50 neonates with isolated PDA and 20 controls. They underwent clinical and echocardiographic assessment at 48 hours of age, after another 48-72 hours and prior to discharge. Results: No correlation was found between PDA diameter and weight (p=0.72), length (p=0.11), Body surface area (BSA) (p=0.33), gestational age (p=0.13). A strong association of PDA-related hemodynamic instability was found with pulmonary hypertension (p=<0.01 & 0.05 for initial and latter studies). Left atrium diameter (LA) Z-score was higher among cases, correlated with PDA size in the 3 echocardiographic studies (p=0.001, 0.001 and 0.007 respectively), and correlated with hemodynamic instability in the initial study (p=0.03). Diameter of descending aorta at level of diaphragm and pulmonary flow/systemic flow ratio (Qp:Qs) correlated with PDA diameter in the latter 2 studies (p=0.001). Main pulmonary artery and left pulmonary artery (LPA) Z-scores were correlated with PDA size at the initial and follow-up studies as expected (p=0.001, 0.047 & 0.047; and p=0.004, 0.018 & 0.032, respectively). LPA Z-score correlated with hemodynamic instability at the follow-up study (p=0.005), which was not sustained at the subsequent study. Conclusion: Pulmonary hypertension, larger LA Z-score and LPA Z-scores are important early (at 48 hours) associations of a hsPDA and hemodynamic instability. Level of Evidence of Study: IIA. (1)","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"36 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127474890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autosomal Recessive Polycystic Kidney Disease in a Child Complicated by Autoimmune Hemolytic Anemia: A Case Report","authors":"M. Kotb, Hend Abd El Baky, Shaimaa Sayed, Mohammed Al Komy, Marwa Onsy, A. Aly, A. Tamer, Esraa Mohamed","doi":"10.21608/cupsj.2022.126454.1046","DOIUrl":"https://doi.org/10.21608/cupsj.2022.126454.1046","url":null,"abstract":": Autosomal recessive polycystic kidney disease (ARPKD) is a rare genetic disorder that presents as an isolated polycystic renal disease in childhood, or associated with congenital hepatic fibrosis and/or Caroli disease. The spectrum of complications of ARPKD include end stage kidney disease, systemic hypertension and liver disease. Anemia in ARPKD is commonly due to be reduced erythropoietin or iron deficiency. We present a 2-year old male patient with ARPKD who presented to the emergency room by striking pallor and dark urine. Initial lab work revealed Hemoglobin level 2g/dL. Blood transfusion was challenging due to difficult typing and frequent mismatch. Other labs showed elevated urea and creatinine, positive direct Coomb’s anticoagulant test and positive urine culture. Imaging was consistent with ARPKD. The patient was resuscitated and after stabilization, he received pulsed methylprednisolone at 30 mg/day for 5 days followed by prednisone 2mg/kg/day for 4 weeks that was tapered over 2 months with marked improvement. Herein we report autoimmune hemolytic anemia as another and rare cause for anemia associated with ARPKD. Level of Evidence of Study: IV ( 1 ) .","PeriodicalId":153483,"journal":{"name":"Pediatric Sciences Journal","volume":"41 7","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"120978934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}