Dolor abdominal y parálisis flácida, una aproximación a las porfirias: serie de casos y revisión de la literatura / Flaccid paralysis and abdominal pain, an approach to porphyrias: a literature review and case series

RESUMEN Las porfirias son trastornos metabólicos hereditarios causados por deficiencias enzimáticas de la biosíntesis del grupo HEM. Con presentación en distintos grupos de edades, más común en infancia y tercera a cuarta década de la vida, se caracterizan por elevación de porfirinas, y manifestaciones variadas cutáneas y neuro viscerales. Describimos una serie de 3 casos de pacientes femeninas en tercera década de la vida con dolor abdominal severo e inespecífico y una amplia gama de manifestaciones clínicas con sus complicaciones a corto y largo plazo en quienes se diagnosticó porfiria aguda intermitente (PAI). Se hará revisión en la literatura para aportar al reconocimiento temprano de estas condiciones e instaurar de forma temprana el manejo específico e impactar en desenlaces irreversibles.

ABSTRACT Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

[Flaccid paralysis and abdominal pain, an approach to porphyrias: a literature review and case series]. / Dolor abdominal y parálisis flácida, una aproximación a las porfirias: serie de casos y revisión de la literatura.

Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

Classrooms in Peace Within Violent Contexts: Field Evaluation of Aulas en Paz in Colombia.

Classrooms in Peace (Aulas en Paz) is an elementary school-based multicomponent program for prevention of aggression and promotion of peaceful relationships. Inspired by international programs and socio-emotional research, it includes (1) a classroom universal curriculum, (2) parent workshops and home visits to parents of the 10% most aggressive children, and (3) extracurricular peer groups of two aggressive and four prosocial children. Activities seek to promote socio-emotional competencies such as empathy, anger management, creative generation of alternatives, and assertiveness. A 2-year quasi-experimental evaluation was conducted with 1154 students from 55 classrooms of seven public schools located in neighborhoods with the presence of youth gangs, drug cartels, and high levels of community violence in two Colombian cities. Despite several implementation (e.g., about half of the activities were not implemented) and evaluation (e.g., randomization problems, large number of missing data, and changes between treatment and control groups) challenges, positive results were found in prosocial behavior and in reduction of aggressive behavior, according to teacher reports, and in assertiveness and reduction of verbal victimization, according to student reports. Furthermore, implementation cost (25 US dollars per student per year) was very low compared to other programs in developed countries. This study shows that the Classrooms in Peace program has an important potential to generate positive results and highlights the challenges of implementing and evaluating prevention programs in highly violent environments.

School-Based Interventions to Promote Empathy-Related Responding in Children and Adolescents: A Developmental Analysis.

Empathy has been identified as a core component of social and emotional functioning across development. Various prevention and intervention programs have utilized components of empathy-related responding to promote the development of children's and adolescents' social-emotional functioning and impede their aggression in school contexts. In this article, we assess the effectiveness of select school-based empathy interventions and the extent to which they align with developmental theory and research. First, we review current conceptualizations of empathy-related responding, identify its components, outline its normative development, and describe the need for developmentally tailored interventions. We then identify and assess the effectiveness and developmental sensitivity of 19 school-based programs with strong empirical support that target empathy-related responding across childhood and adolescence. Although the majority of these programs showed some degree of developmental differentiation between grades, none considered developmental differences within grades. Commencing interventions earlier in development and targeting higher numbers of empathy-related constructs were, in part, associated with larger effects. We discuss how future research can bridge the gap between basic developmental research and the design of developmentally tailored interventions to promote empathy-related responding.

Parent and adolescent intentions to disclose and links to positive social behavior.

Children's disclosure to parents as a buffer against antisocial behavior, and its parenting antecedents, have been extensively studied in recent years. The influence of parents' own disclosure on children's disclosure and positive social behavior has received little attention, however. We assessed mothers' (n = 149), fathers' (n= 105), and 12- to 14-year-old early adolescents' (n = 127) intentions to disclose about distress-related events and rule transgressions, their reasons for disclosing, and links to positive social behavior (assessed by mothers' ratings of willing compliance and by teachers' [n = 114] ratings of prosocial behavior). Parents reported that they disclosed to teach lessons or to encourage their children to disclose, whereas adolescents said they disclosed for comfort and advice. Child intention to disclose about distressing events mediated between mother (but not father) intention to disclose about these same issues and children's willing compliance, but not prosocial behavior in the classroom. Parent and child disclosure about rule transgressions were not related.

Early sympathy and social acceptance predict the development of sharing in children.

Sharing is a fascinating activity of the human species and an important basis for the development of fairness, care, and cooperation in human social interaction. Economic research has proposed that sharing, or the willingness to sacrifice own resources for others, has its roots in social emotions such as sympathy. However, only few cross-sectional experiments have investigated children's other-regarding preferences, and the question how social-emotional skills influence the willingness to share valuable resources has not been tested. In the present longitudinal-experimental study, a sample of 175 6-year-old children, their primary caregivers, and their teachers is examined over a 3-year period of time. Data are analyzed by means of growth curve modeling. The findings show that sharing valuable resources strongly increases in children from 6 to 9 years of age. Increases in sharing behavior are associated with the early-developing ability to sympathize with anonymous others. Sharing at 7 years of age is predicted by feelings of social acceptance at 6 years of age. These findings hold after controlling for children's IQ and SES. Girls share more equally than boys at 6 and 7 years of age, however, this gender difference disappears at the age of 9 years. These results indicate that human sharing strongly increases in middle childhood and, that this increase is associated with sympathy towards anonymous others and with feelings of social acceptance. Additionally, sharing develops earlier in girls than in boys. This developmental perspective contributes to new evidence on change in sharing and its social-emotional roots. A better understanding of the factors underlying differences in the development of sharing and pro-social orientations should also provide insights into the development of atypical, anti-social orientations which exhibit social-emotional differences such as aggression and bullying behavior.