Pub Date : 2020-06-19DOI: 10.31031/rpn.2020.04.000592
Farrag S
Talking is one of the most important visible developmental achievements of early childhood [1]. Given that ability to make intelligible speech and language is an important correlate of child’s overall development and intellect, the diagnosis of speech delay is made when a child does not achieve this developmental milestone at the expected age [2,3]. Globally, the prevalence of speech and language delay in children ranges from 5-15% [4]. In the United States and Canada, 8-12% of pre-school and 12% of school-age children experienced speech delay [5]. In Egypt and many countries in the Gulf regions; paucity of data exists on the burden of this important early childhood disorder. Nevertheless, empirical evidence showed that the quality of language learning during these early years of life does not only impact significantly on the development of oral language skills, but also on later success in literacy [6,7]. Language problems may result from mental retardation, physical handicaps, hearing loss, neurological problems, or environmental deprivations [7]. It has serious sequelae that may persist into adulthood in terms of educational, social, and emotional development [8]. When children fail to develop normal speech at appropriate time, it is therefore not surprising that they develop Crimson Publishers Wings to the Research Research Article
{"title":"Effect of Parents’ Involvement Interventions in Speech Language Delay among Late Talking Toddlers in Egypt: A Quasi-Experimental Study","authors":"Farrag S","doi":"10.31031/rpn.2020.04.000592","DOIUrl":"https://doi.org/10.31031/rpn.2020.04.000592","url":null,"abstract":"Talking is one of the most important visible developmental achievements of early childhood [1]. Given that ability to make intelligible speech and language is an important correlate of child’s overall development and intellect, the diagnosis of speech delay is made when a child does not achieve this developmental milestone at the expected age [2,3]. Globally, the prevalence of speech and language delay in children ranges from 5-15% [4]. In the United States and Canada, 8-12% of pre-school and 12% of school-age children experienced speech delay [5]. In Egypt and many countries in the Gulf regions; paucity of data exists on the burden of this important early childhood disorder. Nevertheless, empirical evidence showed that the quality of language learning during these early years of life does not only impact significantly on the development of oral language skills, but also on later success in literacy [6,7]. Language problems may result from mental retardation, physical handicaps, hearing loss, neurological problems, or environmental deprivations [7]. It has serious sequelae that may persist into adulthood in terms of educational, social, and emotional development [8]. When children fail to develop normal speech at appropriate time, it is therefore not surprising that they develop Crimson Publishers Wings to the Research Research Article","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"15 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127272158","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}
Introduction: Perinatal care has undergone many changes over time. Therefore, women’s feelings and experiences will differ depending on the perinatal care provided at the time of childbirth. Time of childbirth and the perinatal care received are the main determinants in this process. However, one thing remains unchanged over time. Childbirth is considered one of the most notable events in the life of every woman. The aim of the study was a comparative analysis of experiences and feelings shared by females giving birth in the past and the present in Poland. Material and methods: A questionnaire was designed specifically for this research project. It was a set of multiple choice (single answer) questions concerning childbirth conditions and perinatal care. Results were analyzed with a chi square test. Data was collected in 2016 in Poland. The questionnaire was distributed both in paper and electronic form. Results: The study group comprised of 671 females divided into three groups: childbirth before 2000, between 2001–2012, and after 2013. This time frame was associated with significant changes in perinatal care in Poland over the years. Changes in the delivery rooms have raised the comfort of childbirth, but progression of obstetrics resulted in greater medicalization of childbirth.
{"title":"Childbirth Yesterday and Today: A Comparative Analysis of Women’s Experiences and Feelings Through the Ages to The Present","authors":"G. Bączek, J. Jasińska","doi":"10.14738/abr.88.8663","DOIUrl":"https://doi.org/10.14738/abr.88.8663","url":null,"abstract":"Introduction: Perinatal care has undergone many changes over time. Therefore, women’s feelings and experiences will differ depending on the perinatal care provided at the time of childbirth. Time of childbirth and the perinatal care received are the main determinants in this process. However, one thing remains unchanged over time. Childbirth is considered one of the most notable events in the life of every woman. The aim of the study was a comparative analysis of experiences and feelings shared by females giving birth in the past and the present in Poland. \u0000Material and methods: A questionnaire was designed specifically for this research project. It was a set of multiple choice (single answer) questions concerning childbirth conditions and perinatal care. Results were analyzed with a chi square test. Data was collected in 2016 in Poland. The questionnaire was distributed both in paper and electronic form. \u0000Results: The study group comprised of 671 females divided into three groups: childbirth before 2000, between 2001–2012, and after 2013. This time frame was associated with significant changes in perinatal care in Poland over the years. Changes in the delivery rooms have raised the comfort of childbirth, but progression of obstetrics resulted in greater medicalization of childbirth.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125142650","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 : 2020-03-16DOI: 10.31031/rpn.2020.04.000589
R. Chakrabarti
support. Abstract Development of pneumo-pericardium in neonate is a rare event. If it occurs, it presents as a complication of respiratory distress syndrome. Commonly it requires some intervention to be cured, but we experienced a case which was totally resolved by conservative management. The baby was a late preterm and developed pneumopericardium at the age of 4 hours, he was managed conservatively. After 48 hours the pneumopericardium completely resolved.
{"title":"Successful Conservative Management of Neonatal Pneumopericardium","authors":"R. Chakrabarti","doi":"10.31031/rpn.2020.04.000589","DOIUrl":"https://doi.org/10.31031/rpn.2020.04.000589","url":null,"abstract":"support. Abstract Development of pneumo-pericardium in neonate is a rare event. If it occurs, it presents as a complication of respiratory distress syndrome. Commonly it requires some intervention to be cured, but we experienced a case which was totally resolved by conservative management. The baby was a late preterm and developed pneumopericardium at the age of 4 hours, he was managed conservatively. After 48 hours the pneumopericardium completely resolved.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"9 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125116723","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 : 2020-02-25DOI: 10.31031/rpn.2020.04.000587
E. Belousova, N. Matinyan, T. Ushakova, V. Polyakov
Introduction. Retinoblastoma (RB) is a malignant tumor of the embryonic nerve retina. Purpose. To determine the effectiveness of retrobulbar blockade (RbB) with ropivacaine 0.5% for intra- and postoperative analgesia, as well as for the prevention of oculocardial reflex (OCR), postoperative nausea and vomiting (PONV) during enucleation. Material and methods. A prospective, randomized, controlled clinical trial was performed. Eighty patients aged 0 to 10 years were included, who were randomly assigned to the RbB group (retrobulbar blockade with ropivacaine 0.5% with general anesthesia) N = 40 and the GA control group (general anesthesia) N = 40. Results. There were no complications in the RbB group caused by the methodology. In the intraoperative period, the average dose of fentanyl in the RbB group was 4.7 ± 0.7 μg / kg, which is significantly lower than in the OA group of 10.1 ± 1.9 μg / kg (P <0.05). OCR in the RbB group was observed in 5% of cases versus 100% in the GA group (P = 0.002). The average VAS score was 1.8 vs. 3.7 60 minutes after surgery (P <0.001). For the first time 12 hours after surgery, PONV was not observed in the RBB group, and in the control group it was observed in 45% of patients. Conclusions. The study revealed that intraoperative retrobulbar blockade with 0.5% ropivacaine solution in children with RB is effective and safe. Provides stable intraoperative hemodynamics and reduces the need for opioids. Promotes the prevention of OCD and PONV, as well as the improvement of postoperative analgesia during the operation of enucleation of the eyeball in pediatric. Key words: ropivacaine, retrobulbar block, oculocardial reflex, pain, retinoblastoma, enucleation, pediatric patients.
{"title":"Efficacy and Safety of Retrobulbar Blockade of 0.5% Ropivacaine During Eyeball Enucleation Surgery in Children with Retinoblastoma","authors":"E. Belousova, N. Matinyan, T. Ushakova, V. Polyakov","doi":"10.31031/rpn.2020.04.000587","DOIUrl":"https://doi.org/10.31031/rpn.2020.04.000587","url":null,"abstract":"Introduction. Retinoblastoma (RB) is a malignant tumor of the embryonic nerve retina. Purpose. To determine the effectiveness of retrobulbar blockade (RbB) with ropivacaine 0.5% for intra- and postoperative analgesia, as well as for the prevention of oculocardial reflex (OCR), postoperative nausea and vomiting (PONV) during enucleation. Material and methods. A prospective, randomized, controlled clinical trial was performed. Eighty patients aged 0 to 10 years were included, who were randomly assigned to the RbB group (retrobulbar blockade with ropivacaine 0.5% with general anesthesia) N = 40 and the GA control group (general anesthesia) N = 40. Results. There were no complications in the RbB group caused by the methodology. In the intraoperative period, the average dose of fentanyl in the RbB group was 4.7 ± 0.7 μg / kg, which is significantly lower than in the OA group of 10.1 ± 1.9 μg / kg (P <0.05). OCR in the RbB group was observed in 5% of cases versus 100% in the GA group (P = 0.002). The average VAS score was 1.8 vs. 3.7 60 minutes after surgery (P <0.001). For the first time 12 hours after surgery, PONV was not observed in the RBB group, and in the control group it was observed in 45% of patients. Conclusions. The study revealed that intraoperative retrobulbar blockade with 0.5% ropivacaine solution in children with RB is effective and safe. Provides stable intraoperative hemodynamics and reduces the need for opioids. Promotes the prevention of OCD and PONV, as well as the improvement of postoperative analgesia during the operation of enucleation of the eyeball in pediatric. Key words: ropivacaine, retrobulbar block, oculocardial reflex, pain, retinoblastoma, enucleation, pediatric patients.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127943807","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 : 2020-02-25DOI: 10.31031/rpn.2020.04.000586
S. Asadi
{"title":"The Role of Mutations on Genes AKT1, PTEN, GPC3, PIK3CA, In Proteus Syndrome","authors":"S. Asadi","doi":"10.31031/rpn.2020.04.000586","DOIUrl":"https://doi.org/10.31031/rpn.2020.04.000586","url":null,"abstract":"","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"275 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129964624","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 : 2020-01-28DOI: 10.31031/RPN.2020.04.000585
Halyna Bulak, Yaryna Kozak
reflexes, cold, mottled skin, acrocyanosis; jaundice ; pallor of the mucous ; watery eye discharge, including light yellow mucus component. Stigmas of dysembriogenesis-phenotype features of Down syndrome. Pulmonary auscultation was normal. Cardiovascular auscultation revealed sonorous, rhythmic heart tones, a systolic murmur radiating in the back. Liver palpating up to 2.0cm below the costal arch. The urogenital system was developed in male type, testicles were in scrotum. Urinary output was spontaneous. Stool formed without Abstract Congenital hypothyroidism is one of the most common congenital pathology of endocrine system in newborns. Among other diseases causing nervous system disorders in children, congenital hypothyroidism provides most promising treatment results for further child development. The earlier replacement therapy is started, the better results can be expected. This in its turn can only be possible with early diagnostics, mostly on subclinical stages, by screening newborns for this pathology. This article represents a clinical case of congenital hypothyroidism diagnosed on its early stage. jaundice, congenital hydrocephaly, Down syndrome, conjunctivitis, congenital hypothyroidism, congenital heart defect arterial duct and patent foramen ovale).
{"title":"Congenital Hypothyroidism in Child with Down syndrome - A Case Report","authors":"Halyna Bulak, Yaryna Kozak","doi":"10.31031/RPN.2020.04.000585","DOIUrl":"https://doi.org/10.31031/RPN.2020.04.000585","url":null,"abstract":"reflexes, cold, mottled skin, acrocyanosis; jaundice ; pallor of the mucous ; watery eye discharge, including light yellow mucus component. Stigmas of dysembriogenesis-phenotype features of Down syndrome. Pulmonary auscultation was normal. Cardiovascular auscultation revealed sonorous, rhythmic heart tones, a systolic murmur radiating in the back. Liver palpating up to 2.0cm below the costal arch. The urogenital system was developed in male type, testicles were in scrotum. Urinary output was spontaneous. Stool formed without Abstract Congenital hypothyroidism is one of the most common congenital pathology of endocrine system in newborns. Among other diseases causing nervous system disorders in children, congenital hypothyroidism provides most promising treatment results for further child development. The earlier replacement therapy is started, the better results can be expected. This in its turn can only be possible with early diagnostics, mostly on subclinical stages, by screening newborns for this pathology. This article represents a clinical case of congenital hypothyroidism diagnosed on its early stage. jaundice, congenital hydrocephaly, Down syndrome, conjunctivitis, congenital hypothyroidism, congenital heart defect arterial duct and patent foramen ovale).","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"60 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116711952","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 : 2020-01-17DOI: 10.31031/RPN.2020.04.000584
Adhikari Sb, M. SaifulIslam
Inflammatory myofibroblastic tumor, a rare benign tumor, occurs at any site in the body, is more common in pediatric age group than adult. Among them, inflammatory myofibroblastic tumors arising from stomach and mesentery are rare entity. We report two cases Barium swallow examination lower gastro-esophageal junction with bird-beak Endoscopy esophagus. During operation, the mass was found to be in fundus of stomach obstructing gastro-esophageal junction and total radical gastrectomy was done and histopathology report traced inflammatory myofibroblastic tumor. 7 years old boy presented with recurrent (two times operated) ill-defined, periumbilical abdominal mass. Previous tissue biopsy was non-conclusive. Abdominal Contrast CT Scan revealed heterogenous mass in epigastric and umbilical region encasing Superior Mesenteric Artery (SMA), which could not be separated from small bowel loops. During operation, the tumor was found to be arising from root of mesentery, encasing SMA & Head of Pancreas and adhere with almost middle ½ of small intestine. Debulking of mass along with resection anastomosis of small intestine was realized and histopathology report traced inflammatory myofibroblastic tumour. Keywords:
{"title":"Inflammatory Myofibroblastic Tumor: Encounter with A Rare Tumour in Pediatric Age Group","authors":"Adhikari Sb, M. SaifulIslam","doi":"10.31031/RPN.2020.04.000584","DOIUrl":"https://doi.org/10.31031/RPN.2020.04.000584","url":null,"abstract":"Inflammatory myofibroblastic tumor, a rare benign tumor, occurs at any site in the body, is more common in pediatric age group than adult. Among them, inflammatory myofibroblastic tumors arising from stomach and mesentery are rare entity. We report two cases Barium swallow examination lower gastro-esophageal junction with bird-beak Endoscopy esophagus. During operation, the mass was found to be in fundus of stomach obstructing gastro-esophageal junction and total radical gastrectomy was done and histopathology report traced inflammatory myofibroblastic tumor. 7 years old boy presented with recurrent (two times operated) ill-defined, periumbilical abdominal mass. Previous tissue biopsy was non-conclusive. Abdominal Contrast CT Scan revealed heterogenous mass in epigastric and umbilical region encasing Superior Mesenteric Artery (SMA), which could not be separated from small bowel loops. During operation, the tumor was found to be arising from root of mesentery, encasing SMA & Head of Pancreas and adhere with almost middle ½ of small intestine. Debulking of mass along with resection anastomosis of small intestine was realized and histopathology report traced inflammatory myofibroblastic tumour. Keywords:","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125583275","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 : 2020-01-06DOI: 10.31031/RPN.2020.04.000581
Keerthidarshini k, S. Kalyanshettar, B. Lakhkar, R. Gobbur, A. Akki, M. Patil, Patil Sv
Children are curious and explore their world with all their senses, including taste. As a result, the home and its surroundings can be a dangerous place when poisonous substances are inadvertently ingested. Poisoning in pediatric patients is a common and preventable cause of morbidity and mortality. Incidence of childhood poisoning in India is 0.23-3.3%. 50-90% of all childhood poisoning cases are between 1-5yrs age group and mortality rate is 3-5% and males outnumber females and mainly are due to accidental consumption. Suicidal poisoning is common after 13years of age and is due to household chemicals and drugs. Profile and outcome of poisoned patients in a given region is influenced by the social, economic and cultural practices prevalent and also by the availability and the quality of the medical facilities. With this background we conducted this study in the department of Pediatrics of Shri B. M. Patil Medical College hospital and research Centre, Vijayapura.
孩子们很好奇，用他们所有的感官探索他们的世界，包括味觉。因此，当无意中摄入有毒物质时，家庭及其周围环境可能是一个危险的地方。儿科患者中毒是一种常见的可预防的发病和死亡原因。印度儿童中毒发生率为0.23-3.3%。50-90%的儿童中毒病例发生在1-5岁年龄组，死亡率为3-5%，男性多于女性，主要是由于意外食用。自杀性中毒在13岁以后很常见，是由于家用化学品和药物引起的。在某一地区，中毒病人的情况和结果受到普遍存在的社会、经济和文化习俗以及医疗设施的可用性和质量的影响。在此背景下，我们在Vijayapura的Shri B. M. Patil医学院医院和研究中心的儿科进行了这项研究。
{"title":"Acute Poisoning in Children: Data of a Tertiary Care Unit","authors":"Keerthidarshini k, S. Kalyanshettar, B. Lakhkar, R. Gobbur, A. Akki, M. Patil, Patil Sv","doi":"10.31031/RPN.2020.04.000581","DOIUrl":"https://doi.org/10.31031/RPN.2020.04.000581","url":null,"abstract":"Children are curious and explore their world with all their senses, including taste. As a result, the home and its surroundings can be a dangerous place when poisonous substances are inadvertently ingested. Poisoning in pediatric patients is a common and preventable cause of morbidity and mortality. Incidence of childhood poisoning in India is 0.23-3.3%. 50-90% of all childhood poisoning cases are between 1-5yrs age group and mortality rate is 3-5% and males outnumber females and mainly are due to accidental consumption. Suicidal poisoning is common after 13years of age and is due to household chemicals and drugs. Profile and outcome of poisoned patients in a given region is influenced by the social, economic and cultural practices prevalent and also by the availability and the quality of the medical facilities. With this background we conducted this study in the department of Pediatrics of Shri B. M. Patil Medical College hospital and research Centre, Vijayapura.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"223 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133153624","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 : 2019-12-02DOI: 10.31031/RPN.2019.04.000579
A. Harroche, D. Lasne, C. Bally, T. Pascreau, R. Rothschild, D. Borgel
Acquired Hemophilia A is a very rare disease in children but may be life-threatening. We report the case of a 5-year-old child, without any bleeding history. He presented a severe bleeding episode after a circumcision. The blood loss was important, with a hemoglobin rate at 54g/L, requiring a transfusion. The first biological assessment of hemostasis lead to the suspected diagnosis of congenital hemophilia (FVIII=4%), but factor VIII infusions were inefficient. Identification of a specific inhibitor against factor VIII rectified the diagnosis: it was an acquired hemophilia A. No underlying infectious, autoimmune or neoplastic disease was found. Interestingly, he has family history of immune disease: his brother has a juvenile arthritis and his father a Sjogren syndrome. favorable after treatment by recombinant activated factor VII (Novoseven®). Afterwards, he has two bleeding episodes: a large post-venous puncture hematoma, and a post-traumatic hemarthrosis of the knee, efficiently treated by Novoseven®. An immunosuppressive treatment started then: he received a first line therapy of corticoids without any effect on the inhibitor level. A second line therapy with rituximab was attempted, with a greater efficacy. The inhibitor level is now below 1 BU/ml, and the factor VIII level is 120%. This case highlights the difficulties of diagnosing acquired hemophilia A in childhood. Due to its rarity, this condition was first not recognized. Concerning immunosuppressive therapy, this case is the second case of the pediatric literature in this indication that proves efficacy of rituximab.
{"title":"Diagnosis of Acquired Hemophilia A Must be Considered in Childhood: A Case Report","authors":"A. Harroche, D. Lasne, C. Bally, T. Pascreau, R. Rothschild, D. Borgel","doi":"10.31031/RPN.2019.04.000579","DOIUrl":"https://doi.org/10.31031/RPN.2019.04.000579","url":null,"abstract":"Acquired Hemophilia A is a very rare disease in children but may be life-threatening. We report the case of a 5-year-old child, without any bleeding history. He presented a severe bleeding episode after a circumcision. The blood loss was important, with a hemoglobin rate at 54g/L, requiring a transfusion. The first biological assessment of hemostasis lead to the suspected diagnosis of congenital hemophilia (FVIII=4%), but factor VIII infusions were inefficient. Identification of a specific inhibitor against factor VIII rectified the diagnosis: it was an acquired hemophilia A. No underlying infectious, autoimmune or neoplastic disease was found. Interestingly, he has family history of immune disease: his brother has a juvenile arthritis and his father a Sjogren syndrome. favorable after treatment by recombinant activated factor VII (Novoseven®). Afterwards, he has two bleeding episodes: a large post-venous puncture hematoma, and a post-traumatic hemarthrosis of the knee, efficiently treated by Novoseven®. An immunosuppressive treatment started then: he received a first line therapy of corticoids without any effect on the inhibitor level. A second line therapy with rituximab was attempted, with a greater efficacy. The inhibitor level is now below 1 BU/ml, and the factor VIII level is 120%. This case highlights the difficulties of diagnosing acquired hemophilia A in childhood. Due to its rarity, this condition was first not recognized. Concerning immunosuppressive therapy, this case is the second case of the pediatric literature in this indication that proves efficacy of rituximab.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"135 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121367401","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 : 2019-11-26DOI: 10.31031/RPN.2019.04.000578
D. F. Crudo, Lin Jm, Sebesta Mr
Alkaline Phosphatase (ALP) is a group of four isoenzymes originating in various tissues such as bone, liver, intestine, kidney, and placenta. Serum levels vary throughout life with levels higher in childhood than in adult, especially during the pubertal bone growth spurt. Hyperphosphatasemia can result from physiologic conditions such as puberty and pregnancy, in pathological conditions of bone and the hepatobiliary tract, and in an idiopathic condition known as benign transient hyperphosphatasemia (BTH). The diagnosis of BTH was first described by Bach et al. [1] and in 1985 Kraut et al. [2]defined the diagnostic criteria for this entity as: age of presentation less than five years; no other evidence for bone or liver disease on physical examination or laboratory findings; elevation in both bone and liver ALP isoenzymes; and a return to normal serum ALP values within four months. BTH is accepted as a harmless self-limiting biochemical disorder without sequelae. The aim of this paper is to report a series of BTH cases and to call attention to this condition and the proposed evaluation guidelines to avoid unnecessary costly testing.
{"title":"Benign Transient Hyperphosphatasemia","authors":"D. F. Crudo, Lin Jm, Sebesta Mr","doi":"10.31031/RPN.2019.04.000578","DOIUrl":"https://doi.org/10.31031/RPN.2019.04.000578","url":null,"abstract":"Alkaline Phosphatase (ALP) is a group of four isoenzymes originating in various tissues such as bone, liver, intestine, kidney, and placenta. Serum levels vary throughout life with levels higher in childhood than in adult, especially during the pubertal bone growth spurt. Hyperphosphatasemia can result from physiologic conditions such as puberty and pregnancy, in pathological conditions of bone and the hepatobiliary tract, and in an idiopathic condition known as benign transient hyperphosphatasemia (BTH). The diagnosis of BTH was first described by Bach et al. [1] and in 1985 Kraut et al. [2]defined the diagnostic criteria for this entity as: age of presentation less than five years; no other evidence for bone or liver disease on physical examination or laboratory findings; elevation in both bone and liver ALP isoenzymes; and a return to normal serum ALP values within four months. BTH is accepted as a harmless self-limiting biochemical disorder without sequelae. The aim of this paper is to report a series of BTH cases and to call attention to this condition and the proposed evaluation guidelines to avoid unnecessary costly testing.","PeriodicalId":153075,"journal":{"name":"Research in Pediatrics & Neonatology","volume":"12 8","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132274542","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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