cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.14282515.v1
title: ATTACHMENT STYLE IN CHILDREN WITH CHRONIC DISEASES: A COMPREHENSIVE REVIEW

description: ABSTRACT Objective: To investigate how attachment style has been studied in children with chronic disease in the scientific literature, and what repercussions this attachment has on this population. Data sources: An integrative review of the literature was carried out from a survey in the LILACS, MEDLINE and SciELO databases, including original national and international publications in Portuguese, Spanish and English from 2007 to 2018, using the descriptors “apego” and “criança” in the Health Sciences Descriptors (DeCS), and “attachment” and “children” for the Medical Subject Headings (MeSH). Sixteen (16 articles) were obtained for the sample analysis. Data synthesis: The chronic diseases found in the research were neurobehavioral disorders such as attention deficit hyperactivity disorder (ADHD) and autism, childhood obesity, and chronic migraine. The predominant attachment style was insecurity, which could compromise the biopsychosocial development of the child. Conclusions: The type of attachment between child and primary caregiver may be considered a protective or risk factor for child development. Considering this premise, it is important to equip/inform families based on dialogic educational practices, in which professionals create opportunities and means for families to develop their skills and competencies, and acquire resources which meet the child’s needs. It is important that this professional helps the family to build secure bases for their child with chronic disease, understanding that the main focus for promoting child development is in the family unit.
DOI: 10.6084/m9.figshare.14282516.v1
title: SNAPPE II: ANALYSIS OF ACCURACY AND DETERMINATION OF THE CUTOFF POINT AS A DEATH PREDICTOR IN A BRAZILIAN NEONATAL INTENSIVE CARE UNIT

description: ABSTRACT Objective: To analyze the accuracy of the Score for Neonatal Acute Physiology Perinatal Extension (SNAPPE II) as a death predictor, to determine the cutoff point for mortality, and to analyze the association of independent variables with death. Methods: Prospective, longitudinal, hospital-based study on newborns admitted to the Neonatal Intensive Care Unit (NICU) for the first time from November 1, 2016 to April 30, 2017. Newborns with less than 12 hours of length of stay at the NICU, out-of-hospital births, major congenital malformations, and inter-hospital transfer were excluded. Variables were grouped according to hierarchical framework, related to maternal characteristics (distal level), prenatal and childbirth care (intermediate level), and birth conditions (proximal level). Descriptive analyses of SNAPPE II score ranges, Receiver Operating Characteristics Curve (ROC curve) to define the cutoff point for mortality, and bivariate analysis by the Wald test and multiple logistic regression were conducted. Results: After selection, the sample consisted of 247 newborns. In this study, the SNAPPE II cutoff point for mortality was 27, with sensitivity of 84.1% and specificity of 82.4%. 61% of those with a score ≥27 died. Multiple logistic regression showed an association between death and proximal-level variables: sepsis (Odds Ratio [OR] 10.68; 95% confidence interval [95%CI] 2.82–40.48; p&lt;0.001); SNAPPE II ≥27 (OR 5.85; 95%CI 1.90–18.05; p=0.002); birth weight 750–999 g (OR 4.15; 95%CI 1.06–16.14; p=0.040); and nonuse of surfactant (OR 0.159; 95%CI 0.04–0.53; p=0.003). Conclusions: Neonatal mortality was directly proportional to increase in SNAPPE II. Score≥27 increased the odds of dying by six times compared with neonates with lower scores. The proximal variables related to health conditions and neonatal care were associated with death.
DOI: 10.6084/m9.figshare.11609853.v1
title: SYSTEMATIC REVIEW OF THE LITERATURE ON SURGICAL TREATMENT OF CHRONIC RHINOSINUSITIS IN CHILDREN: WHAT IS THE BEST APPROACH?

description: ABSTRACT Objective: To carry out a systematic literature review on the surgical treatment of chronic rhinosinusitis in the pediatric population. Data sources: A bibliographic review methodology was used, based on data from National Library of Medicine (Medline), PubMed, Latin American and Caribbean Health Sciences Literature (LILACS) and Scientific Electronic Library Online (SciELO), of the indexed works from 2006 to 2016, including the pediatric population from zero to 13 years of age. The search keywords according to Medical Subject Heading (MESH) and Health Sciences Descriptors (DeCS) were: child, surgery, sinusitis and chronic disease. A total of 318 articles were collected, five of which met the inclusion criteria and were used as a basis for this review. All articles were prospective cohort studies, level of evidence 2B, according to the criterion used by evidence-based medicine. Data synthesis: The literature agreed that the next step for the cases refractory to drug treatment in chronic rhinosinusitis in childhood would be surgery. Adenoidectomy would be the initial method, for the safety of the procedure and improvement in about 50% of the cases, although more significant results were found in patients who associated this procedure with facial sinus surgery. Conclusions: Surgical treatment should be indicated for chronic rhinosinusitis in childhood after treatment failure. The results pointed out that adenoidectomy, when associated with some type of approach to the facial sinus, present better results.
DOI: 10.6084/m9.figshare.11609505
title: SCHISTOSOMAL MYELORADICULOPATHY IN A NON-ENDEMIC AREA

description: ABSTRACT Objective: To report a schistosomal myeloradiculopathy case in a non-endemic area. Case description: A previously healthy 11-year-old boy, stricken by an acute loss of strength on his lower limbs, followed by a loss of strength on his upper limbs and upper body, associated with altered sensitivity of the vesical globe formation. The patient’s cerebrospinal fluid analysis showed eosinophilic meningitis, in addition to peripheral eosinophilia. The investigation resulted in a positive serology for Schistosoma mansoni. The treatment included steroids and praziquantel 60mg/kg, with a new dose after a month, as well as physical therapy for rehabilitation. The patient evolved with clinical improvement in the neurological exam, with a medullary section initially at C6, but now at T6. The patient is kept at prednisolone use (30mg/day) and longterm urinary catheter dependence. Comments: The schistosomiasis is endemic in many regions of Brazil; however, it has low incidence in the south of the country. Among its main manifestations, the schistosomal myeloradiculopathy is the most severe ectopic form of the disease, and should be suspected in patients with low back pain, strength and/or sensibility disorder of the lower limbs or urinary tract’s disturbance. Early diagnosis and treatment should be done in order to reduce severe neurological sequelae. Treatment includes schistosomiasis drugs, corticosteroids and/or surgery.
DOI: 10.6084/m9.figshare.14282516
title: SNAPPE II: ANALYSIS OF ACCURACY AND DETERMINATION OF THE CUTOFF POINT AS A DEATH PREDICTOR IN A BRAZILIAN NEONATAL INTENSIVE CARE UNIT

description: ABSTRACT Objective: To analyze the accuracy of the Score for Neonatal Acute Physiology Perinatal Extension (SNAPPE II) as a death predictor, to determine the cutoff point for mortality, and to analyze the association of independent variables with death. Methods: Prospective, longitudinal, hospital-based study on newborns admitted to the Neonatal Intensive Care Unit (NICU) for the first time from November 1, 2016 to April 30, 2017. Newborns with less than 12 hours of length of stay at the NICU, out-of-hospital births, major congenital malformations, and inter-hospital transfer were excluded. Variables were grouped according to hierarchical framework, related to maternal characteristics (distal level), prenatal and childbirth care (intermediate level), and birth conditions (proximal level). Descriptive analyses of SNAPPE II score ranges, Receiver Operating Characteristics Curve (ROC curve) to define the cutoff point for mortality, and bivariate analysis by the Wald test and multiple logistic regression were conducted. Results: After selection, the sample consisted of 247 newborns. In this study, the SNAPPE II cutoff point for mortality was 27, with sensitivity of 84.1% and specificity of 82.4%. 61% of those with a score ≥27 died. Multiple logistic regression showed an association between death and proximal-level variables: sepsis (Odds Ratio [OR] 10.68; 95% confidence interval [95%CI] 2.82–40.48; p&lt;0.001); SNAPPE II ≥27 (OR 5.85; 95%CI 1.90–18.05; p=0.002); birth weight 750–999 g (OR 4.15; 95%CI 1.06–16.14; p=0.040); and nonuse of surfactant (OR 0.159; 95%CI 0.04–0.53; p=0.003). Conclusions: Neonatal mortality was directly proportional to increase in SNAPPE II. Score≥27 increased the odds of dying by six times compared with neonates with lower scores. The proximal variables related to health conditions and neonatal care were associated with death.
DOI: 10.6084/m9.figshare.11609505.v1
title: SCHISTOSOMAL MYELORADICULOPATHY IN A NON-ENDEMIC AREA

description: ABSTRACT Objective: To report a schistosomal myeloradiculopathy case in a non-endemic area. Case description: A previously healthy 11-year-old boy, stricken by an acute loss of strength on his lower limbs, followed by a loss of strength on his upper limbs and upper body, associated with altered sensitivity of the vesical globe formation. The patient’s cerebrospinal fluid analysis showed eosinophilic meningitis, in addition to peripheral eosinophilia. The investigation resulted in a positive serology for Schistosoma mansoni. The treatment included steroids and praziquantel 60mg/kg, with a new dose after a month, as well as physical therapy for rehabilitation. The patient evolved with clinical improvement in the neurological exam, with a medullary section initially at C6, but now at T6. The patient is kept at prednisolone use (30mg/day) and longterm urinary catheter dependence. Comments: The schistosomiasis is endemic in many regions of Brazil; however, it has low incidence in the south of the country. Among its main manifestations, the schistosomal myeloradiculopathy is the most severe ectopic form of the disease, and should be suspected in patients with low back pain, strength and/or sensibility disorder of the lower limbs or urinary tract’s disturbance. Early diagnosis and treatment should be done in order to reduce severe neurological sequelae. Treatment includes schistosomiasis drugs, corticosteroids and/or surgery.
DOI: 10.6084/m9.figshare.11266949.v1
title: HEMOLYTIC UREMIC SYNDROME ASSOCIATED WITH STREPTOCOCCUS PNEUMONIAE IN PEDIATRICS: A CASE SERIES

description: ABSTRACT Objective: To describe a case series of four (4) patients with hemolytic uremic syndrome due to Streptococcus pneumoniae in a level four complexity institution in the city of Bogotá, D.C., Colombia. Cases description: We describe cases of four patients who presented respiratory symptoms and fever. All four patients were in regular conditions on hospital admission, after which they required intensive care and ventilatory support. Upon admission, three cases showed evidence of pleuropulmonary complication. Penicillin-sensitive Streptococcus pneumoniae was isolated in all cases. All patients presented anemia, severe thrombocytopenia, schistocytes on peripheral blood smear, and hyperazotemia. They required blood transfusion and renal replacement therapy during their hospitalization. The patients were diagnosed with hemolytic uremic syndrome due to S. pneumoniae. Three of the four patients had a progressive recovery of the renal function and were discharged after an average of 36 days of hospital stay. The remaining patient had two amputations in the extremities due to thrombotic vascular complications and was discharged after 99 days of hospital stay, requiring hemodialysis every other day. Comments: Hemolytic uremic syndrome due to Streptococcus pneumoniae is a rare but severe complication of invasive pneumococcal disease. Complicated pneumonia is the main condition associated with this entity. It is noteworthy the short period in which these cases were presented, considering the low annual incidence of the disease.
DOI: 10.6084/m9.figshare.11869287
title: PREPUBERTAL GYNECOMASTIA: A RARE MANIFESTATION OF MYOTONIC DYSTROPHY TYPE 1

description: ABSTRACT Objective: To present a case of bilateral gynecomastia in a prepubertal boy with autism spectrum disorder, diagnosed with myotonic dystrophy type 1. Case description: A 12-year-old boy with autism spectrum disorder presented at a follow-up visit with bilateral breast growth. There was a family history of gynecomastia, cataracts at a young age, puberty delay, and myotonic dystrophy type 1. The physical examination showed that he had bilateral gynecomastia with external genitalia Tanner stage 1. Neurologic examination was regular, without demonstrable myotonia. The analytical study revealed increased estradiol levels and estradiol/testosterone ratio. After excluding endocrine diseases, the molecular study of the dystrophia myotonica protein kinase gene confirmed the diagnosis of myotonic dystrophy type 1. Comments: A diagnosis of prepubertal gynecomastia should include an investigation for possible underlying diseases. This case report highlights the importance of considering the diagnosis of myotonic dystrophy type 1 in the presence of endocrine and neurodevelopmental manifestations.
DOI: 10.6084/m9.figshare.11266949
title: HEMOLYTIC UREMIC SYNDROME ASSOCIATED WITH STREPTOCOCCUS PNEUMONIAE IN PEDIATRICS: A CASE SERIES

description: ABSTRACT Objective: To describe a case series of four (4) patients with hemolytic uremic syndrome due to Streptococcus pneumoniae in a level four complexity institution in the city of Bogotá, D.C., Colombia. Cases description: We describe cases of four patients who presented respiratory symptoms and fever. All four patients were in regular conditions on hospital admission, after which they required intensive care and ventilatory support. Upon admission, three cases showed evidence of pleuropulmonary complication. Penicillin-sensitive Streptococcus pneumoniae was isolated in all cases. All patients presented anemia, severe thrombocytopenia, schistocytes on peripheral blood smear, and hyperazotemia. They required blood transfusion and renal replacement therapy during their hospitalization. The patients were diagnosed with hemolytic uremic syndrome due to S. pneumoniae. Three of the four patients had a progressive recovery of the renal function and were discharged after an average of 36 days of hospital stay. The remaining patient had two amputations in the extremities due to thrombotic vascular complications and was discharged after 99 days of hospital stay, requiring hemodialysis every other day. Comments: Hemolytic uremic syndrome due to Streptococcus pneumoniae is a rare but severe complication of invasive pneumococcal disease. Complicated pneumonia is the main condition associated with this entity. It is noteworthy the short period in which these cases were presented, considering the low annual incidence of the disease.
DOI: 10.6084/m9.figshare.11869287.v1
title: PREPUBERTAL GYNECOMASTIA: A RARE MANIFESTATION OF MYOTONIC DYSTROPHY TYPE 1

description: ABSTRACT Objective: To present a case of bilateral gynecomastia in a prepubertal boy with autism spectrum disorder, diagnosed with myotonic dystrophy type 1. Case description: A 12-year-old boy with autism spectrum disorder presented at a follow-up visit with bilateral breast growth. There was a family history of gynecomastia, cataracts at a young age, puberty delay, and myotonic dystrophy type 1. The physical examination showed that he had bilateral gynecomastia with external genitalia Tanner stage 1. Neurologic examination was regular, without demonstrable myotonia. The analytical study revealed increased estradiol levels and estradiol/testosterone ratio. After excluding endocrine diseases, the molecular study of the dystrophia myotonica protein kinase gene confirmed the diagnosis of myotonic dystrophy type 1. Comments: A diagnosis of prepubertal gynecomastia should include an investigation for possible underlying diseases. This case report highlights the importance of considering the diagnosis of myotonic dystrophy type 1 in the presence of endocrine and neurodevelopmental manifestations.
DOI: 10.6084/m9.figshare.11966106
title: COMPLICATED PNEUMONIA WITH EMPYEMA CAUSED BY STREPTOCOCCUS ANGINOSUS IN A CHILD

description: ABSTRACT Objective: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. Case description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. Comments: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.
DOI: 10.6084/m9.figshare.11966106.v1
title: COMPLICATED PNEUMONIA WITH EMPYEMA CAUSED BY STREPTOCOCCUS ANGINOSUS IN A CHILD

description: ABSTRACT Objective: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. Case description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. Comments: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.
DOI: 10.6084/m9.figshare.14282524
title: INVERSE KLIPPEL-TRENAUNAY SYNDROME

description: ABSTRACT Objective: To report a rare case of inverse Kipplel-Trenaunay. Case description: A 16-year-old girl with a grayish-depressed plaque on her left thigh. Angioresonance showed a vascular malformation affecting the skin and subcutaneous tissue. Comments: Inverse Klippel-Trenaunay is a Klippel-Trenaunay syndrome variation in which there are capillary and venous malformations associated to hypotrophy or shortening of the affected limb. Modifications on the limb’s length or width result from alterations in bones, muscles, or subcutaneous tissues. It has few described cases. Further clinical and molecular studies must be performed for a proper understanding.
DOI: 10.6084/m9.figshare.14282524.v1
title: INVERSE KLIPPEL-TRENAUNAY SYNDROME

description: ABSTRACT Objective: To report a rare case of inverse Kipplel-Trenaunay. Case description: A 16-year-old girl with a grayish-depressed plaque on her left thigh. Angioresonance showed a vascular malformation affecting the skin and subcutaneous tissue. Comments: Inverse Klippel-Trenaunay is a Klippel-Trenaunay syndrome variation in which there are capillary and venous malformations associated to hypotrophy or shortening of the affected limb. Modifications on the limb’s length or width result from alterations in bones, muscles, or subcutaneous tissues. It has few described cases. Further clinical and molecular studies must be performed for a proper understanding.
DOI: 10.6084/m9.figshare.14282521
title: TYROSINEMIA TYPE III: A CASE REPORT OF SIBLINGS AND LITERATURE REVIEW

description: ABSTRACT Objective: Tyrosinemia type III (HT III) is the rarest form of tyrosinemia, and the full clinical spectrum of this disorder is still unknown. The neurological involvement varies, including intellectual impairment and attention deficit disorder with hyperactivity (ADHD). We report the case of two siblings diagnosed with HT III at different ages. Case description: The index case was diagnosed by newborn screening for endocrine and metabolic disorders, starting a low-protein diet immediately, with a consistent decrease in tyrosine levels. By the age of three, the child displayed a hyperactive behavior, starting treatment for ADHD two years later. At seven years of age, he shows a slight improvement in terms of behavior and attention span and has a cognitive performance slightly lower than his peers, despite maintaining acceptable tyrosine levels. His sister, who had a history of ADHD since age five, was diagnosed with HT III after family screening at the age of eight. Despite initiating a dietetic treatment, her behavior did not improve, and she has a mild intellectual impairment. Comments: This is the first case report describing siblings with HT III who underwent nutritional treatment with a low-protein diet in different phases of life, with a better neurological and behavioral evaluation in the patient who started treatment earlier.