RESUMO
The early management of neonates with meconium ileus (MI) and cystic fibrosis (CF) is highly variable across countries and is not standardized. We conducted a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. The protocol was registered in PROSPERO (CRD42024522838). Studies from three providers of academic search engines were checked for inclusion criteria, using the following search terms: meconium ileus AND cystic fibrosis OR mucoviscidosis. Regarding the patient population studied, the inclusion criteria were defined using our predefined PICOT framework: studies on neonates with simple or complicated meconium which were confirmed to have cystic fibrosis and were conservatively managed or surgically treated. Results: A total of 566 publications from the last 10 years were verified by the authors of this review to find the most recent and relevant data, and only 8 met the inclusion criteria. Prenatally diagnosed meconium pseudocysts, bowel dilation, and ascites on ultrasound are predictors of neonatal surgery and risk factor for negative 12-month clinical outcomes in MI-CF newborns. For simple MI, conservative treatment with hypertonic solutions enemas can be effective in more than 25% of cases. If repeated enemas fail to disimpact the bowels, the Bishop-Koop stoma is a safe option. No comprehensive research has been conducted so far to determine the ideal surgical protocol for complicated MI. We only found three studies that reported the types of stomas performed and another study comparing the outcomes of patients depending on the surgical management; the conclusions are contradictory especially since the number of cases analyzed in each study was small. Between 18% and 38% of patients with complicated MI will require reoperation for various complications and the mortality rate varies between 0% and 8%. Conclusion: This study reveals a lack of strong data to support management decisions, unequivocally shows that the care of infants with MI is not standardized, and suggests a great need for international collaborative studies.
RESUMO
Hidradenitis suppurativa (HS) is a recurrent chronic inflammatory condition of the skin with a debilitating potential, especially in adolescents who may experience psychosocial disorders and impaired quality of life. Recognition of typical, recurrent lesions, history of the disease and family investigation establishes the diagnosis. The rarity of the disease in males and the appearance of this condition in children, which is also rare, in two of our adolescent patients, respectively, are the reasons for publishing this article. We reviewed the literature and found that variation exists across international treatment for this HS, and much collaboration is needed to put forth unified and updated recommendations. It is of major importance that the early recognition of the condition and the establishment of treatment in the multidisciplinary team. Psychotherapy, health education, the adoption of a healthy lifestyle, the application of local prevention measures, and long-term medical treatment are components of a correct management. Extensive surgical treatment should be restricted only to severe cases because it may lead to complications, extend the hospitalization period and increase psychological problems in adolescents. The treatment will be personalized, performed according to the stage of evolution, and the approach will be a multidisciplinary one.