Sickle hepatopathy is the hepatobiliary dysfunction associated with sickle cell disease. It has a varied spectrum ranging from asymptomatic transaminasemia to gallstones or fulminant liver failure. Hepatobiliary manifestations may be the initial presentation in children with undiagnosed sickle cell disease as seen in our three index cases. This may mimic a primary liver disease, delaying definite diagnosis and management. We describe three cases. The first case was a 9-year-old girl child with cholecystitis with choledocholithiasis, the second case was a 15-year-old boy with acute hepatitis of unidentified aetiology, and the third case was a 3-month-old infant with neonatal cholestasis in absence of common structural or metabolic cause. All three cases had underlying haemolytic anaemia with splenomegaly and belonged to the sickle belt of the region. The final diagnosis in all three index cases was sickle cell disease with hepatopathy. The clinical syndrome of hepatitis or cholestasis with or without cholangitis in the background of splenomegaly and haemolytic anaemia should prompt screening for sickle cell disease.
{"title":"Paediatric sickle cell disease presenting with hepatobiliary symptoms—a case presentation and brief literature review","authors":"Aditi Kumar, Rashmi Ranjan Behera, Samarendra Mahapatro, Ranjan Patel, Hemanta Nayak, Amit Kumar Satapathy","doi":"10.1186/s43054-024-00288-w","DOIUrl":"https://doi.org/10.1186/s43054-024-00288-w","url":null,"abstract":"Sickle hepatopathy is the hepatobiliary dysfunction associated with sickle cell disease. It has a varied spectrum ranging from asymptomatic transaminasemia to gallstones or fulminant liver failure. Hepatobiliary manifestations may be the initial presentation in children with undiagnosed sickle cell disease as seen in our three index cases. This may mimic a primary liver disease, delaying definite diagnosis and management. We describe three cases. The first case was a 9-year-old girl child with cholecystitis with choledocholithiasis, the second case was a 15-year-old boy with acute hepatitis of unidentified aetiology, and the third case was a 3-month-old infant with neonatal cholestasis in absence of common structural or metabolic cause. All three cases had underlying haemolytic anaemia with splenomegaly and belonged to the sickle belt of the region. The final diagnosis in all three index cases was sickle cell disease with hepatopathy. The clinical syndrome of hepatitis or cholestasis with or without cholangitis in the background of splenomegaly and haemolytic anaemia should prompt screening for sickle cell disease.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"31 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141507717","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}
Childhood and adolescence are critical periods for physical and mental development. For that, sounding the alarm for the warning signs and red flags of children’s mental health disorders is important to promote good health and mental wellness throughout the lifespan. The aim of the study was to assess children’s and adolescents’ mental health in relation to parenting styles. This study used a descriptive cross-sectional design. From early May 2022 until late October 2022, Zagazig University in Egypt hosted this investigation. Subjects: For this study, 400 parents of Zagazig University staff, employees, and workers who agreed to engage in the current study were gathered as a convenience sample. Tools: In order to get the required data, three tools were utilized. Tool I: A questionnaire for interviews to gather demographic information about the participating parents and their children. Tool II: Adapted Ontario Child Health Study Emotional Behavioural Scale: Parent Version (for children 4–17 years). Tool III: Parenting style scale. It was found that criteria for conduct disorder constituted the highest followed by criteria for attention deficit hyperactivity disorder and major depression disorder with a mean and standard deviation of 15.10 ± 3.7, 12.83 ± 3.4, and 11.9 ± 2.8. Also, 66% of the participating parents practiced a permissive parenting style while 18% of them practiced the authoritative style and 16% for the authoritarian style. It was determined that criteria of conduct disorder were the most prevalent, followed by criteria of attention deficit hyperactivity disorder and major depressive disorder. Additionally, there was high statistical significance between mental health disorders, parenting styles, and parental educational level. In order to protect children’s and adolescents’ mental health, this study recommended alerting parents and teachers about red flags and warning signs of mental health disorders for early detection and management. Additionally, educate parents about effective parenting methods and how to behave correctly with their children. Psychological counseling centers for seeking help should be available everywhere and announced.
{"title":"Sounding the alarm regarding mental health of children and adolescents in relation to parenting style","authors":"Nesreen Mosbah Elsayed Mohamed, Fawzia Nabeel Mohammad Abd-Elmageed, Rasha Abdelateef Abdelaziz Ramadan","doi":"10.1186/s43054-024-00285-z","DOIUrl":"https://doi.org/10.1186/s43054-024-00285-z","url":null,"abstract":"Childhood and adolescence are critical periods for physical and mental development. For that, sounding the alarm for the warning signs and red flags of children’s mental health disorders is important to promote good health and mental wellness throughout the lifespan. The aim of the study was to assess children’s and adolescents’ mental health in relation to parenting styles. This study used a descriptive cross-sectional design. From early May 2022 until late October 2022, Zagazig University in Egypt hosted this investigation. Subjects: For this study, 400 parents of Zagazig University staff, employees, and workers who agreed to engage in the current study were gathered as a convenience sample. Tools: In order to get the required data, three tools were utilized. Tool I: A questionnaire for interviews to gather demographic information about the participating parents and their children. Tool II: Adapted Ontario Child Health Study Emotional Behavioural Scale: Parent Version (for children 4–17 years). Tool III: Parenting style scale. It was found that criteria for conduct disorder constituted the highest followed by criteria for attention deficit hyperactivity disorder and major depression disorder with a mean and standard deviation of 15.10 ± 3.7, 12.83 ± 3.4, and 11.9 ± 2.8. Also, 66% of the participating parents practiced a permissive parenting style while 18% of them practiced the authoritative style and 16% for the authoritarian style. It was determined that criteria of conduct disorder were the most prevalent, followed by criteria of attention deficit hyperactivity disorder and major depressive disorder. Additionally, there was high statistical significance between mental health disorders, parenting styles, and parental educational level. In order to protect children’s and adolescents’ mental health, this study recommended alerting parents and teachers about red flags and warning signs of mental health disorders for early detection and management. Additionally, educate parents about effective parenting methods and how to behave correctly with their children. Psychological counseling centers for seeking help should be available everywhere and announced.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"47 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141529674","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}
Pub Date : 2024-06-24DOI: 10.1186/s43054-024-00284-0
Yi Wu, Weiming Gong, Zhenni Wang, Mengjie Luo
The clinical diagnosis of neonatal sepsis remains difficult because of various challenges, such as culturing the bacteria and avoiding contamination. Therefore, this study aimed to identify bacterial pathogens in patients with clinically diagnosed neonatal sepsis by next-generation sequencing (NGS). High-throughput NGS and traditional culture identification were performed by comparing samples from newborns with neonatal sepsis with healthy control infants. All blood samples were separately inoculated into anaerobic and aerobic bottles and incubated for 7 days at 37 °C, the positive specimens were then identified. Novel bacteria identified through high-throughput NGS were analysed using polymerase chain reaction (PCR), PCR products were verified by Sanger sequencing. Wilcoxon rank-sum and chi-square tests were performed to assess the significance of differences in species abundance between groups. Subjects were clinically diagnosed and hospitalized at the Pediatrics Department of Shenzhen Seventh People’s Hospital and Pediatrics Department of the Longhua Branch of Shenzhen People’s Hospital. Experiments were performed at the Shenzhen Seventh People’s Hospital. The experimental group comprised 45 newborns clinically diagnosed with neonatal sepsis (age: 0–28 days; 28 males, 17 females). Fifteen normal newborns aged 0–28 days (7 males, 8 females) were included as the control group. High-throughput NGS showed a positivity rate of 44% (20/45) for bacteria in patients clinically diagnosed with neonatal sepsis, whereas traditional bacterial culture identification showed a positivity rate of 0% (0/45). The four main bacterial species identified were Anoxybacillus kestanbolensis, Geobacillus vulcani, Klebsiella oxytoca, and Acinetobacter guillouiae. A. kestanbolensis, G. vulcani, K. oxytoca, and A. guillouiae, newly discovered bacteria in patients with neonatal sepsis, were identified with high-throughput NGS. Which may result from maternal intrauterine infection or birth-canal infection and have a high clinical-cure rate. Owing to a lack of methods to culture these bacteria, their role in neonatal sepsis remains unclear. A definite diagnosis cannot rely solely on bacterial culture identification for patients with a suspected diagnosis and clinical diagnosis of neonatal sepsis and should involve other effective diagnostic techniques.
{"title":"Identification of novel bacterial species in the blood of patients with neonatal sepsis","authors":"Yi Wu, Weiming Gong, Zhenni Wang, Mengjie Luo","doi":"10.1186/s43054-024-00284-0","DOIUrl":"https://doi.org/10.1186/s43054-024-00284-0","url":null,"abstract":"The clinical diagnosis of neonatal sepsis remains difficult because of various challenges, such as culturing the bacteria and avoiding contamination. Therefore, this study aimed to identify bacterial pathogens in patients with clinically diagnosed neonatal sepsis by next-generation sequencing (NGS). High-throughput NGS and traditional culture identification were performed by comparing samples from newborns with neonatal sepsis with healthy control infants. All blood samples were separately inoculated into anaerobic and aerobic bottles and incubated for 7 days at 37 °C, the positive specimens were then identified. Novel bacteria identified through high-throughput NGS were analysed using polymerase chain reaction (PCR), PCR products were verified by Sanger sequencing. Wilcoxon rank-sum and chi-square tests were performed to assess the significance of differences in species abundance between groups. Subjects were clinically diagnosed and hospitalized at the Pediatrics Department of Shenzhen Seventh People’s Hospital and Pediatrics Department of the Longhua Branch of Shenzhen People’s Hospital. Experiments were performed at the Shenzhen Seventh People’s Hospital. The experimental group comprised 45 newborns clinically diagnosed with neonatal sepsis (age: 0–28 days; 28 males, 17 females). Fifteen normal newborns aged 0–28 days (7 males, 8 females) were included as the control group. High-throughput NGS showed a positivity rate of 44% (20/45) for bacteria in patients clinically diagnosed with neonatal sepsis, whereas traditional bacterial culture identification showed a positivity rate of 0% (0/45). The four main bacterial species identified were Anoxybacillus kestanbolensis, Geobacillus vulcani, Klebsiella oxytoca, and Acinetobacter guillouiae. A. kestanbolensis, G. vulcani, K. oxytoca, and A. guillouiae, newly discovered bacteria in patients with neonatal sepsis, were identified with high-throughput NGS. Which may result from maternal intrauterine infection or birth-canal infection and have a high clinical-cure rate. Owing to a lack of methods to culture these bacteria, their role in neonatal sepsis remains unclear. A definite diagnosis cannot rely solely on bacterial culture identification for patients with a suspected diagnosis and clinical diagnosis of neonatal sepsis and should involve other effective diagnostic techniques.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"50 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141507718","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}
Pub Date : 2024-06-19DOI: 10.1186/s43054-024-00283-1
Mona Hamdy, Iman Shaheen, Hadi Ramadan, Fatma Abdel Wahab Abdel Maksoud, Yasmin Mohamed Ramadan
Sickle cell nephropathy is a complication of sickle cell disease characterized by functional abnormalities of the kidney and glomeruli. Our study aimed to investigate the single-nucleotide genetic variants in TGF-β-1-related genes as an early predictor of sickle cell nephropathy (SCN) risk. Two hundred participants, 100 patients with SCD, and 100 age and sex-matched control. The study included full history taking, clinical examination, and laboratory evaluation. Renal function tests (serum urea and creatinine, microalbuminuria, albumin/ creatinine ratio, and e-GFR). Genotyping for TGF-β1 genetic variants rs1800469 and rs1800471. Twenty-one percent of patients had glomerular hyperfiltration, while 31% had reduced e-GFR. Microalbuminuria was present in 14%, and none had macroalbuminuria or edema. TGF-β1 genotyping revealed a statistically significant difference in the rs 1800471 C allele, which was more common in the control group (p 0.028). No significant correlation between the result of TGF‐ β genotyping and the albumin-to-creatinine ratio, creatinine, and e-GFR. TGF-β1 rs1800469 and rs1800471 genetic variants were not associated with the risk of sickle nephropathy in children with sickle cell disease.
{"title":"Transforming growth factor-β1: relation between its single-nucleotide genetic variants and sickle cell nephropathy","authors":"Mona Hamdy, Iman Shaheen, Hadi Ramadan, Fatma Abdel Wahab Abdel Maksoud, Yasmin Mohamed Ramadan","doi":"10.1186/s43054-024-00283-1","DOIUrl":"https://doi.org/10.1186/s43054-024-00283-1","url":null,"abstract":"Sickle cell nephropathy is a complication of sickle cell disease characterized by functional abnormalities of the kidney and glomeruli. Our study aimed to investigate the single-nucleotide genetic variants in TGF-β-1-related genes as an early predictor of sickle cell nephropathy (SCN) risk. Two hundred participants, 100 patients with SCD, and 100 age and sex-matched control. The study included full history taking, clinical examination, and laboratory evaluation. Renal function tests (serum urea and creatinine, microalbuminuria, albumin/ creatinine ratio, and e-GFR). Genotyping for TGF-β1 genetic variants rs1800469 and rs1800471. Twenty-one percent of patients had glomerular hyperfiltration, while 31% had reduced e-GFR. Microalbuminuria was present in 14%, and none had macroalbuminuria or edema. TGF-β1 genotyping revealed a statistically significant difference in the rs 1800471 C allele, which was more common in the control group (p 0.028). No significant correlation between the result of TGF‐ β genotyping and the albumin-to-creatinine ratio, creatinine, and e-GFR. TGF-β1 rs1800469 and rs1800471 genetic variants were not associated with the risk of sickle nephropathy in children with sickle cell disease.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"27 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141507722","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}
Neonatal intestinal obstruction is a complex condition that poses challenge in management. Prompt recognition and appropriate management are crucial to prevent complications. We present a case of a term neonate with intermittent bilious vomiting. The baby underwent routinely performed investigations, including abdominal radiography and ultrasound, which provided inconclusive results. However, contrast-enhanced computed tomography revealed a web at duodenojejunal flexure. Exploratory laparotomy confirmed the diagnosis, and surgical intervention was performed. The baby’s postoperative course was prolonged with a gradual transition from parenteral nutrition to enteral feeds. This case highlights a rare cause of intestinal obstruction, the unusual location of duodenal web, the importance of maintaining a high index of suspicion for intestinal obstruction, even in the absence of classical signs, and the value of utilizing contrast-enhanced computed tomography when initial investigations are inconclusive.
{"title":"A case of intermittent bilious emesis in a term neonate: a case report and brief review of literature","authors":"Asha Mudugere, Antaryami Pradhan, Vijay Kumar, Debasish Nanda","doi":"10.1186/s43054-024-00274-2","DOIUrl":"https://doi.org/10.1186/s43054-024-00274-2","url":null,"abstract":"Neonatal intestinal obstruction is a complex condition that poses challenge in management. Prompt recognition and appropriate management are crucial to prevent complications. We present a case of a term neonate with intermittent bilious vomiting. The baby underwent routinely performed investigations, including abdominal radiography and ultrasound, which provided inconclusive results. However, contrast-enhanced computed tomography revealed a web at duodenojejunal flexure. Exploratory laparotomy confirmed the diagnosis, and surgical intervention was performed. The baby’s postoperative course was prolonged with a gradual transition from parenteral nutrition to enteral feeds. This case highlights a rare cause of intestinal obstruction, the unusual location of duodenal web, the importance of maintaining a high index of suspicion for intestinal obstruction, even in the absence of classical signs, and the value of utilizing contrast-enhanced computed tomography when initial investigations are inconclusive.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"20 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141254649","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}
Renal salt wasting (RSW) is primarily seen with central nervous disorders and is characterized by hyponatremia, elevated urinary sodium excretion, increased urine output, and hypovolemia. Although there have been reports of RSW in children, it has not been reported in newborns. A term (38 weeks 2 days) female weighing 2060 g, born via normal vaginal delivery, to a primi-gravida mother, was admitted to our neonatal intensive care unit. At birth, the baby was non-vigorous, had aspirated meconium, and required bag-and-mask ventilation following which the baby cried. The child was put on CPAP and inotropes for respiratory distress and shock, respectively, along with first-line antibiotics. Over the next few days, the child was weaned off CPAP, inotropes were stopped, and feeds were started. On postnatal day (PND) 8, the baby developed repeated episodes of seizures requiring two-antiepileptics followed by midazolam infusion, had shock requiring inotropes, and also had a tense anterior fontanelle with altered sensorium and tone changes, for which mannitol and 3% saline were given. Antibiotics were upgraded, and neuroimaging (MRI) revealed left basal-ganglia bleed with intraventricular extension and cerebral venous thrombosis. The baby showed gradual clinical improvement with the above measures and was restarted on feeds. However, from PND-19 onwards the baby had repeated episodes of hyponatremia requiring 3% saline infusions, progressive weight loss, and polyuria. Considering RSW, urine sodium was done which was high (110 mmol/L). For polyuria and weight loss, feed volume was increased, whereas, for hyponatremia, table salt was added to the expressed breast milk (1 gm × tds). Subsequently, all the serum sodium values remained within normal limits, and the baby started gaining weight and was discharged on similar advice (PND 38). On further follow-up, table salt was gradually decreased in the feeds and was stopped after 1 month of discharge with normal serum sodium values. Management of recurrent hyponatremia in a neonate is challenging. Despite its rarity, RSW in newborns should be considered a differential.
{"title":"Recurrent hyponatremia in neonate: a case of renal salt wasting syndrome","authors":"Innama Maryam, Rufaida Mazahir, Afreen Khanam, Uzma Firdaus, Syed Manazir Ali","doi":"10.1186/s43054-024-00275-1","DOIUrl":"https://doi.org/10.1186/s43054-024-00275-1","url":null,"abstract":"Renal salt wasting (RSW) is primarily seen with central nervous disorders and is characterized by hyponatremia, elevated urinary sodium excretion, increased urine output, and hypovolemia. Although there have been reports of RSW in children, it has not been reported in newborns. A term (38 weeks 2 days) female weighing 2060 g, born via normal vaginal delivery, to a primi-gravida mother, was admitted to our neonatal intensive care unit. At birth, the baby was non-vigorous, had aspirated meconium, and required bag-and-mask ventilation following which the baby cried. The child was put on CPAP and inotropes for respiratory distress and shock, respectively, along with first-line antibiotics. Over the next few days, the child was weaned off CPAP, inotropes were stopped, and feeds were started. On postnatal day (PND) 8, the baby developed repeated episodes of seizures requiring two-antiepileptics followed by midazolam infusion, had shock requiring inotropes, and also had a tense anterior fontanelle with altered sensorium and tone changes, for which mannitol and 3% saline were given. Antibiotics were upgraded, and neuroimaging (MRI) revealed left basal-ganglia bleed with intraventricular extension and cerebral venous thrombosis. The baby showed gradual clinical improvement with the above measures and was restarted on feeds. However, from PND-19 onwards the baby had repeated episodes of hyponatremia requiring 3% saline infusions, progressive weight loss, and polyuria. Considering RSW, urine sodium was done which was high (110 mmol/L). For polyuria and weight loss, feed volume was increased, whereas, for hyponatremia, table salt was added to the expressed breast milk (1 gm × tds). Subsequently, all the serum sodium values remained within normal limits, and the baby started gaining weight and was discharged on similar advice (PND 38). On further follow-up, table salt was gradually decreased in the feeds and was stopped after 1 month of discharge with normal serum sodium values. Management of recurrent hyponatremia in a neonate is challenging. Despite its rarity, RSW in newborns should be considered a differential.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"22 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141188774","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}
Pub Date : 2024-05-29DOI: 10.1186/s43054-024-00270-6
Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan
Autoimmune hemolytic anemia (AIHA) is a rare disease in children, sometimes associated with acute, life-threatening, rapidly progressive course requiring prompt management. The aim of our study is to describe the role and outcome of plasma exchange in the acute management of pediatric patients with AIHA requiring transfusion and refractory to high doses of corticosteroids. This was a descriptive retrospective report of all patients admitted to the pediatric intensive care unit (PICU) of Children’s University Hospital who received PE for acute intractable AIHA resistant to management with pulse steroids starting from June 2017 to June 2022. The demographic data, vitals, and laboratory investigations at PICU admission and upon discharge were gathered. The number of PE sessions needed for each patient, volume used for exchange, type of replacement, IV access used, complications, and outcome were reported. This series included 19 patients, 10 males, and 9 females, with a median age and weight of 76 months (IQR 18–121), and 20.9 kg. Improvement of the mean hemoglobin was observed from 5.3 ± 1.8 to 9.9 ± 2.6 g/dl at discharge. The average number of PE sessions was 2.4 sessions with no adverse effects encountered. The mean PICU stay was 16.6 days. Mortality occurred in 2 patients (10.5%) due to their primary illness, while 7 patients (36.8%) were in need of further immunotherapy, and 5 patients (28%) showed relapse. PE may be used as a safe and successful therapy in children with severe acute life-threatening AIHA not responding to steroids, or if well-matched PRBCs are unavailable for transfusion.
{"title":"Plasma exchange is the hope for critically ill children with life-threatening autoimmune hemolytic anemia","authors":"Noha El-Anwar, Hafez Bazaraa, Fatma Abdel Maksoud, Yasmin Ramadan","doi":"10.1186/s43054-024-00270-6","DOIUrl":"https://doi.org/10.1186/s43054-024-00270-6","url":null,"abstract":"Autoimmune hemolytic anemia (AIHA) is a rare disease in children, sometimes associated with acute, life-threatening, rapidly progressive course requiring prompt management. The aim of our study is to describe the role and outcome of plasma exchange in the acute management of pediatric patients with AIHA requiring transfusion and refractory to high doses of corticosteroids. This was a descriptive retrospective report of all patients admitted to the pediatric intensive care unit (PICU) of Children’s University Hospital who received PE for acute intractable AIHA resistant to management with pulse steroids starting from June 2017 to June 2022. The demographic data, vitals, and laboratory investigations at PICU admission and upon discharge were gathered. The number of PE sessions needed for each patient, volume used for exchange, type of replacement, IV access used, complications, and outcome were reported. This series included 19 patients, 10 males, and 9 females, with a median age and weight of 76 months (IQR 18–121), and 20.9 kg. Improvement of the mean hemoglobin was observed from 5.3 ± 1.8 to 9.9 ± 2.6 g/dl at discharge. The average number of PE sessions was 2.4 sessions with no adverse effects encountered. The mean PICU stay was 16.6 days. Mortality occurred in 2 patients (10.5%) due to their primary illness, while 7 patients (36.8%) were in need of further immunotherapy, and 5 patients (28%) showed relapse. PE may be used as a safe and successful therapy in children with severe acute life-threatening AIHA not responding to steroids, or if well-matched PRBCs are unavailable for transfusion.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"25 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141172746","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}
Pub Date : 2024-05-27DOI: 10.1186/s43054-024-00256-4
Mohamed Sayed Abd El-Monsif, Noha Arafa, Mahmoud Marei Marei, Gamal Eltagy, Ahmed M. K. Wishahy
The diagnosis of male differences of sex development is a challenging multidisciplinary team task, that requires external genital evaluation, karyotyping, hormonal profiling, radiological work up and frequently diagnostic laparoscopy and biopsy, for evaluation of internal duct system and nature of gonads. The debate still persists regarding the best diagnostic modality for accurate visualization of Müllerian duct remnants (MDRs) in those patients. The aim of the study was to compare between laparoscopy (L) and ultrasonography (US) regarding the diagnostic accuracy in detection of Müllerian duct remnants, in addition to describing their anatomical nature and relations with the male duct system, in patients with male DSD, with various karyotypes. We prospectively included 20 patients with male DSD, mostly due to 46 XY DSD or chromosomal DSD, over 2 years. The medical and radiological data were collected and analyzed. The age at the first diagnostic intervention ranged from 8 to 24 months (mean: 17 months). There were 14 patients with 46XY DSD with variable diagnoses (3 ovotesticular DSD, 3 partial gonadal dysgenesis, 6 persistent Müllerian duct remnants syndrome and 2 mixed gonadal dysgenesis). Two patients with 46XX DSD were included (one XX male, and one patient with ovotesticular DSD). One patient with chimerism (46XY/46XX) and three patients with 46XY/45XO mixed gonadal dysgenesis were also recruited. MDRs were evident in all cases (100%) by laparoscopy, only 25% (n = 5) were visualized by US. There was a statistically significant difference between laparoscopy and US regarding gonadal and MDR visualization, being higher with laparoscopy (p values, 0.0180 and 0.001). Ultrasonography failed to visualize Müllerian remnants in 75% of patients with complex DSD. On the other hand, laparoscopy provided optimum visualization of MDRs and gonads in those children.
{"title":"Laparoscopy versus ultrasonography for the evaluation of Müllerian duct remnants in male patients with disorder of sex differentiation","authors":"Mohamed Sayed Abd El-Monsif, Noha Arafa, Mahmoud Marei Marei, Gamal Eltagy, Ahmed M. K. Wishahy","doi":"10.1186/s43054-024-00256-4","DOIUrl":"https://doi.org/10.1186/s43054-024-00256-4","url":null,"abstract":"The diagnosis of male differences of sex development is a challenging multidisciplinary team task, that requires external genital evaluation, karyotyping, hormonal profiling, radiological work up and frequently diagnostic laparoscopy and biopsy, for evaluation of internal duct system and nature of gonads. The debate still persists regarding the best diagnostic modality for accurate visualization of Müllerian duct remnants (MDRs) in those patients. The aim of the study was to compare between laparoscopy (L) and ultrasonography (US) regarding the diagnostic accuracy in detection of Müllerian duct remnants, in addition to describing their anatomical nature and relations with the male duct system, in patients with male DSD, with various karyotypes. We prospectively included 20 patients with male DSD, mostly due to 46 XY DSD or chromosomal DSD, over 2 years. The medical and radiological data were collected and analyzed. The age at the first diagnostic intervention ranged from 8 to 24 months (mean: 17 months). There were 14 patients with 46XY DSD with variable diagnoses (3 ovotesticular DSD, 3 partial gonadal dysgenesis, 6 persistent Müllerian duct remnants syndrome and 2 mixed gonadal dysgenesis). Two patients with 46XX DSD were included (one XX male, and one patient with ovotesticular DSD). One patient with chimerism (46XY/46XX) and three patients with 46XY/45XO mixed gonadal dysgenesis were also recruited. MDRs were evident in all cases (100%) by laparoscopy, only 25% (n = 5) were visualized by US. There was a statistically significant difference between laparoscopy and US regarding gonadal and MDR visualization, being higher with laparoscopy (p values, 0.0180 and 0.001). Ultrasonography failed to visualize Müllerian remnants in 75% of patients with complex DSD. On the other hand, laparoscopy provided optimum visualization of MDRs and gonads in those children.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"18 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141172854","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}
The study aims to evaluate the usefulness of preoperative ultrasonography (US) at the internal inguinal ring level in predicting contralateral patent processus vaginalis (CPPV). This is a prospective study of patients who presented with unilateral inguinal hernia and underwent laparoscopic hernia repair and contralateral side exploration. The gender, age, initial presenting side, and the preoperative width of the low echoic region at the internal ring (WLIR) of the contralateral side that was determined using US and laparoscopic findings were recorded. The preoperative diagnosis of CPPV was considered if the WLIR is > 4 mm. We compared laparoscopic with US findings. This study included 30 patients with a median age of 3.5 years (range, 25 days to 10 years), with 3 females and 27 males. The preoperative US and laparoscopic diagnoses of the contralateral side were concordant in 19 (63.3%) and discordant in 11 (36.6%) patients. US showed a sensitivity of 50.00%, specificity of 72.22%, and accuracy of 63.3% in diagnosing CPPV. Measuring the WLIR by US was inadequate for an accurate CPPV diagnosis in our study. Therefore, more distinctive US criteria are required for a proper CPPV diagnosis.
{"title":"Accuracy of ultrasonography in predicting contralateral patent processus vaginalis compared with laparoscopic findings in children","authors":"Abeer Aboalazayem, Hadeel Seif, Sherif Kaddah, Mohamed Elbarbary, Ahmed MK Wishahy","doi":"10.1186/s43054-024-00258-2","DOIUrl":"https://doi.org/10.1186/s43054-024-00258-2","url":null,"abstract":"The study aims to evaluate the usefulness of preoperative ultrasonography (US) at the internal inguinal ring level in predicting contralateral patent processus vaginalis (CPPV). This is a prospective study of patients who presented with unilateral inguinal hernia and underwent laparoscopic hernia repair and contralateral side exploration. The gender, age, initial presenting side, and the preoperative width of the low echoic region at the internal ring (WLIR) of the contralateral side that was determined using US and laparoscopic findings were recorded. The preoperative diagnosis of CPPV was considered if the WLIR is > 4 mm. We compared laparoscopic with US findings. This study included 30 patients with a median age of 3.5 years (range, 25 days to 10 years), with 3 females and 27 males. The preoperative US and laparoscopic diagnoses of the contralateral side were concordant in 19 (63.3%) and discordant in 11 (36.6%) patients. US showed a sensitivity of 50.00%, specificity of 72.22%, and accuracy of 63.3% in diagnosing CPPV. Measuring the WLIR by US was inadequate for an accurate CPPV diagnosis in our study. Therefore, more distinctive US criteria are required for a proper CPPV diagnosis.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"43 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140937642","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}
Pub Date : 2024-05-06DOI: 10.1186/s43054-024-00280-4
Jose Luis Gonzalez Chavez, Pedro Salvador Jiménez Urueta, Edgar Melo Camacho, Sofia Brenes Guzmán
Pediatric ureteral injuries can arise from trauma or surgical procedures, and urinary-enteral fistulas, although rare, are both challenging to manage and repair. More information is needed regarding ureteral-enteral fistulas in the general population, and successful treatment options are limited. This case report introduces an innovative approach, treatment, and successful follow-up featuring a novel repair technique designed for pediatric patients. As a result of complicated appendicitis, a 2-year-old male developed a uretero-enteral fistula. A right ureteral-enteral fistula was detected, and because of the persistent metabolic acidosis and deteriorating malnourishment, a percutaneous nephrostomy tube was inserted with the closure of the fistula through a colonoscopy clip. Three months later, with a better patient’s condition, corrective ureteral surgery was performed with total resection of the fistulized ileocolonic segment (an anastomosis previously made because of the ileocecal valve resection secondary to appendiceal process) and total reconstruction of the urinary tract using a Boari vesical flap. The postoperative course was satisfactory. Managing the ureteral-enteral fistulas is a surgical challenge due to their diverse etiology and presentation. An effective surgical management plan requires a comprehensive understanding of ureteral injuries and associated conditions and an examination of the patient's urological anatomy and function to tailor the best treatment for each case.
{"title":"Management of uretero-colonic fistulas and ureteral injuries: a comprehensive surgical and endoscopic approach","authors":"Jose Luis Gonzalez Chavez, Pedro Salvador Jiménez Urueta, Edgar Melo Camacho, Sofia Brenes Guzmán","doi":"10.1186/s43054-024-00280-4","DOIUrl":"https://doi.org/10.1186/s43054-024-00280-4","url":null,"abstract":"Pediatric ureteral injuries can arise from trauma or surgical procedures, and urinary-enteral fistulas, although rare, are both challenging to manage and repair. More information is needed regarding ureteral-enteral fistulas in the general population, and successful treatment options are limited. This case report introduces an innovative approach, treatment, and successful follow-up featuring a novel repair technique designed for pediatric patients. As a result of complicated appendicitis, a 2-year-old male developed a uretero-enteral fistula. A right ureteral-enteral fistula was detected, and because of the persistent metabolic acidosis and deteriorating malnourishment, a percutaneous nephrostomy tube was inserted with the closure of the fistula through a colonoscopy clip. Three months later, with a better patient’s condition, corrective ureteral surgery was performed with total resection of the fistulized ileocolonic segment (an anastomosis previously made because of the ileocecal valve resection secondary to appendiceal process) and total reconstruction of the urinary tract using a Boari vesical flap. The postoperative course was satisfactory. Managing the ureteral-enteral fistulas is a surgical challenge due to their diverse etiology and presentation. An effective surgical management plan requires a comprehensive understanding of ureteral injuries and associated conditions and an examination of the patient's urological anatomy and function to tailor the best treatment for each case.","PeriodicalId":43064,"journal":{"name":"Egyptian Pediatric Association Gazette","volume":"33 1","pages":""},"PeriodicalIF":0.8,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140886812","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}
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