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OBJECTIVE: To test the hypothesis that allergic proctocolitis, a cause of self-limiting rectal bleeding in infants, can predispose to the development of functional gastrointestinal disorders (FGIDs) later in childhood. STUDY DESIGN: We studied a cohort of 80 consecutive patients diagnosed with allergic proctocolitis. Their sibling or matched children presenting to the same hospital for minor trauma served as controls. Parents of the patients with allergic proctocolitis and controls participated in a telephone interview every 12 months until the child was at least 4 years old. At that time, they were asked to complete the parental Questionnaire on Pediatric Gastrointestinal Symptoms, Rome III version. RESULTS: Sixteen of the 160 subjects (10.0%) included in the study met the Rome III criteria for FGIDs. Among the 80 patients with allergic proctocolitis, 12 (15.0%) reported FGIDs, compared with 4 of 80 (5.0%) controls (P = .035). After adjustment for age and sex, the OR for FGIDs in allergic proctocolitis group was 4.39 (95% CI, 1.03-18.68). FGIDs were significantly associated with iron deficiency anemia, duration of hematochezia, and younger age at presentation. In a multivariate analysis, only the duration of hematochezia was significantly associated with the development of FGIDs (OR, 3.14; 95% CI,1.72-5.74). CONCLUSIONS: We have identified allergic proctocolitis as a new risk factor for the development of FGIDs in children. Our data suggest that not only infection, but also a transient early-life allergic inflammatory trigger may induce persistent digestive symptoms, supporting the existence of "postinflammatory" FGIDs.
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Hipersensibilidad a los Alimentos/complicaciones , Enfermedades Gastrointestinales/etiología , Proctocolitis/complicaciones , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Mucosa Intestinal/patología , Masculino , Proctocolitis/clasificación , Estudios Prospectivos , Factores de RiesgoRESUMEN
The advent of new energy sources for hemostasis has greatly facilitated advanced laparoscopic procedures. We describe a straightforward technique of laparoscopic splenectomy (LS) accomplished using the THUNDERBEAT™ system (TS) (Olympus Medical Systems Corp., Tokyo, Japan) as the sole means of tissue dissection and hemostasis in two patients aged 19 and 6 years, respectively. The specimens were removed intact via a Pfannenstiel incision. Total operative time was 165 and 150 min, and length of hospital stay was three and 4 d, respectively. The TS is an appealing and reliable alternative to currently available energy devices, allowing fast dissection and secure hemostasis during laparoscopic splenectomy.
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Disección/instrumentación , Hemostasis Quirúrgica/instrumentación , Laparoscopía/instrumentación , Esplenectomía/instrumentación , Pérdida de Sangre Quirúrgica/prevención & control , Niño , Disección/métodos , Humanos , Laparoscopía/métodos , Tempo Operativo , Esplenectomía/métodos , Adulto JovenRESUMEN
BACKGROUND: Little is known about the prevalence and clinical relevance of hypersensitivity to the plant panallergen profilin in children. OBJECTIVES: The present study aimed to investigate prevalence, risk factors and clinical relevance of profilin sensitization in a large cohort of Italian children of different ages living in different geographic areas. METHODS: Children with pollen allergy enrolled by 16 pediatric outpatient clinics sited in three main geographic areas of Italy were studied. SPT were carried out with commercial pollen extracts and a commercial purified date palm pollen profilin. IgE specific for allergenic pollen molecules, Phl p 12 (grass profilin) and Pru p 3 (peach lipid transfer protein) were tested by ImmunoCAP FEIA. RESULTS: IgE to Phl p 12 (≥0.35 kU/l) was observed in 296 of the 1,271 participants (23%), including 17 of the 108 (16%) preschool children. Profilin SPT was positive (≥3 mm) in 320/1,271 (25%) participants. The two diagnostic methods were concordant in 1,151 (91%, p < 0.0001) cases. Phl p 12 IgE prevalence declined from northern to southern Italy and was directly associated with IgE to Phl p 1 and/or Phl p 5 and Ole e 1. Among children with IgE to Phl p 12, OAS was provoked by kiwi, melon, watermelon, banana, apricot and cucumber. CONCLUSIONS: Profilin sensitization is very frequent among pollen-allergic children, occurs at a very young age and contributes to the development of childhood OAS with a typical pattern of offending foods. Pediatricians should always consider IgE sensitization to profilin while examining pollen-allergic children, even if they are at preschool age.
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Alérgenos/inmunología , Antígenos de Plantas/inmunología , Hipersensibilidad/epidemiología , Hipersensibilidad/inmunología , Inmunoglobulina E/inmunología , Polen/inmunología , Profilinas/inmunología , Proteínas Portadoras/inmunología , Niño , Reacciones Cruzadas/inmunología , Cucumis sativus/inmunología , Femenino , Frutas/inmunología , Humanos , Italia , Masculino , Poaceae/inmunología , Prevalencia , Rinitis Alérgica Estacional/inmunología , Factores de Riesgo , Pruebas Cutáneas/métodosRESUMEN
BACKGROUND: Lymphoid nodular hyperplasia (LNH) of the lower gastrointestinal tract is a common finding during paediatric colonoscopies, and its clinical significance has not yet been clearly established. OBJECTIVE: The purpose of this prospective, parallel multi-arm, randomized clinical trial was to study relationship between food allergy and LNH. METHODS: We recruited 268 children who had undergone a diagnostic colonoscopy between 1 January 2009 and 30 September 2011. The inclusion criteria were the following: (i) demonstration of LNH; (ii) no concomitant inflammatory or immune disease; (iii) no treatment since the clinical onset. The patients were assigned 1:1:1 to elimination diet (Group A), mesalamine (Group B) or symptomatic treatment with antispasmodics or antidiarrhoeal drugs (Group C) for an 8-wk period. Patients were followed for 24 months. RESULTS: We enrolled 72 of 97 children with intestinal LNH who were referred for haematochezia (76%), recurrent abdominal pain (68%) and/or chronic diarrhoea (32%). Clinical improvement was observed in 75%, 83% and 79% of the patients in Groups A, B and C, respectively. The difference in the response to treatment among the groups and the association to the clinical, endoscopic and allergic features of the patients were not statistically significant. CONCLUSIONS: Intestinal LNH should be considered a benign finding in children without red flags, because the symptoms are largely self-limiting. The use of mesalamine or elimination diet does not modify the clinical outcome compared to symptomatic therapy. The presence of food allergy was not predicted by allergy skin testing and was found in a minority of patients.
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Enfermedad de Castleman/epidemiología , Hipersensibilidad a los Alimentos/epidemiología , Mesalamina/administración & dosificación , Adolescente , Antiinflamatorios no Esteroideos/administración & dosificación , Antiinflamatorios no Esteroideos/efectos adversos , Antidiarreicos/administración & dosificación , Antidiarreicos/efectos adversos , Enfermedad de Castleman/terapia , Niño , Preescolar , Colonoscopía , Dietoterapia , Femenino , Estudios de Seguimiento , Hipersensibilidad a los Alimentos/terapia , Humanos , Italia , Masculino , Mesalamina/efectos adversos , Parasimpatolíticos/administración & dosificación , Parasimpatolíticos/efectos adversos , Estudios Prospectivos , Pruebas CutáneasRESUMEN
We describe a 10-week-old hemophilic infant who developed a large-sized pseudoaneurysm of the radial artery after arterial puncture. Based on our previous experience, compression bandage was elected as first-line treatment. The clinical course was complicated by skin ulceration. However, compression bandage was continued, and complete thrombosis of the pseudoaneurysm occurred after 4 weeks of treatment, preserving arterial patency. Bedside clot evacuation and enzymatic debridement promoted rapid wound healing with negligible scar formation. Compression bandage is a safe and valuable measure for repair of radial artery pseudoaneurysm, even in patients with clotting disorders. Professionals are encouraged to consider the benefit of such an artery-sparing treatment option.
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Aneurisma Falso/terapia , Vendajes de Compresión , Arteria Radial , Aneurisma Falso/diagnóstico por imagen , Estudios de Seguimiento , Humanos , Lactante , Masculino , UltrasonografíaRESUMEN
Introduction: One of the most prevalent congenital wall abnormalities in children, umbilical hernias are often linked to premature or small-for-gestational-age babies. In cases of intestinal malrotation or if the cecum is very movable, generalized peritonitis may facilitate the imprisonment of these hernias. Case report: We described a case of a 4-month-old baby who had a prior reducible umbilical hernia with a history of fever, vomiting, poor appetite, and constipation for around 48â h. The patient experienced significant intestinal bloating, vomiting, irreducibility of the umbilical hernia, skin pigmentation, and erythema at the umbilical site within 2 days after hospitalization. When there was no free abdominal air, a direct abdominal x-ray revealed evidence of hydro-gas stasis and various hydro-aerial levels that were pertinent to the ileum. In order to reduce the hernia, the patient had an emergency surgical treatment where the hernia sac was isolated and released from the ileal loop, which was securely attached to a fibrin plate. When the herniary sac was opened, a gangrenous and perforated appendix was found inside. On the seventh postoperative day, the patient was released from the hospital after an uncomplicated postoperative stay. Conclusion: Our patient's clinical presentation is similar to that of only one other case report involving a 25-day-old male patient. Our case presented with a variant of the clinical symptoms of the previously described umbilical hernia, which became unfixable and strangulated as a result of appendix inflammation. The appendix was discovered inside the hernia sac during surgery.
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BACKGROUND: No age-specific pediatric guidelines exist for the management of spontaneous pneumothorax (SP) in children and adolescents. Treatment remains heterogeneous and center dependent. The role of computed tomography (CT) has yet to be defined. AIMS: Review the management of SP in children and adolescents, with emphasis on conservative management and role of CT. METHODS: Retrospective analysis of 61 consecutive patients with SP at single tertiary center. Clinical, radiological, surgical data, follow-up, and outcomes were revised. RESULTS: First-line management was conservative for 32 (53%) patients and operative for 29 (47%). Asymptomatic/paucisymptomatic patients managed conservatively experienced less first-line treatment failure. Furthermore, the patients needing at least a chest drain or surgery during the follow-up were significantly lower in the conservative group. Conservative and operative patients showed no significant differences regarding ipsilateral recurrences or contralateral occurrences. Of the 61 overall CTs performed, 14 (23%) had an impact on management. Forty-three (70%) patients had at least a CT, in 22 (51%) the CT was positive for blebs. For 10 of these patients (45%) the presence of blebs had an impact on management. Patients with and without blebs showed no differences regarding ipsilateral recurrence, contralateral occurrences, or the need for at least a chest drain or surgery during the follow-up. CONCLUSIONS: First-line conservative management had a significantly shorter hospitalization and better outcome, with a similar incidence of recurrences. The presence of blebs at CT does not predict the risk of recurrence. The CT scan should be reserved for a small number of selected patients who have post-VATS refractory or recurrent pneumothorax.
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Neumotórax , Humanos , Adolescente , Niño , Neumotórax/diagnóstico por imagen , Neumotórax/terapia , Neumotórax/etiología , Estudios Retrospectivos , Cirugía Torácica Asistida por Video/métodos , Recurrencia , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
Introduction: Midgut volvulus and intussusception are prevalent paediatric abdominal emergencies. To the best of our knowledge, this is the first reported case of a connection between intestinal volvulus and a massive intussusception. Case report: An 8-month-old male infant was brought to the emergency room with a history of abdominal pain and vomiting for <24â h. On physical examination, the child appeared restless and was found to have a circumferential hard mass of approximately 4â cm in diameter in the epigastric region. Upon admission, laboratory results showed a C-reactive protein level of 0.4â mg/dl, LDH level of 351â U/L, mild leukocytosis with a white blood cell count of 12 × 103â /µl, and 67% neutrophils. A physical exam was significant for abdominal distention, hyperresonance in percussion, and a palpable, painful epigastric mass. The findings of the operation included a dilated and ischemic intestinal loop, approximately 25â cm from the ileocecal valve, twisted upon itself for three turns. After de-rotation, an extensive occluding ileo-ileal invagination with an ischemic intestinal loop was identified, and a length of approximately 55-60â cm of the distal ileum, including the ischemic segment, was resected. Discussion: This is the first reported case of a connection between intestinal volvulus and a massive intussusception. Currently, only two reported cases describe the connection between volvulus and intussusception, which are insufficient to establish a direct link between the two clinical conditions.
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Introduction: Pectus excavatum, also known as "sunken chest" or "funnel chest," is a congenital condition where the sternum caves inward, creating a noticeable depression in the chest. This deformity can range from mild to severe cases, and can affect appearance and lung and heart function. Treatment options vary depending on the severity of the condition and associated symptoms. A case study was conducted on three patients suffering from severe forms of pectus excavatum using modified taulinoplasty with two Pectus Up bars. Case description: The patients were males, with an age of 15 years. Preoperatively, they underwent spirometry, an echocardiogram, and allergy tests. The procedure involved inserting two Pectus Up bars into the chest wall at the major sternal defect, allowing the implant to remain completely invisible. The procedure involved placing the sternal plate at the deepest point and anchored to the sternum with five self-tapping screws. The chest plate was then fixed to the bar using two screws. Discussion: The use of Pectus Up was first reported in 2016 and has been a subject of scientific discussion and research. The double Pectus Up technique offers improved correction, increased stability, and reduced complications. However, it also presents challenges such as increased technical complexity and potential for prolonged operative times. Patient outcomes showed promising results in terms of short-term correction and long-term stability. The use of a double bar technique in the modified Taulinoplasty procedure is a key area of ongoing clinical research and innovation in pectus excavatum repair. Further studies will be needed, including more institutions that use this technique to validate our initial experience.
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Introduction: International research suggests that poor patient compliance is the main cause of tutor failures in the context of potential novel orthopedic bivalve braces for conservative treatment of pectus carinatum. Our entire experimental study is based on the hypothesis that a rigid bivalve brace that patients can accept could solve the main problem associated with the conservative approach-poor compliance. The hypothesis was to reduce the thickness and weight of the classic bivalve brace to ensure concealment and make it sustainable enough to be worn several hours a day without compromising its therapeutic efficacy. Materials and method: The research was conducted from January 2020 to December 2022 to ensure follow-up of all participants for at least 6 months. In 36 months, 140 patients with pectus carinatum were assessed and conservatively treated with the studied guardian to analyze the therapeutic efficacy of the bivalve brace and patient compliance. From the initial visit, the parents and patient were informed that this is a 2-year therapeutic course during which the bivalve brace should be worn at least 23â h a day (with 1â h of abstinence per day for routine personal hygiene practices). Compliance is the key to therapy success, and the duration of treatment depends on patient adherence. Results: The exceptional effectiveness of the experimental brace was confirmed by both the questionnaire from the patients (with an average satisfaction rate of 8.9/10) and an assessment of the therapy's results by a properly selected medical committee (with a VAS scale satisfaction of 7.2/10 for symmetric forms and 7.1/10 for asymmetric ones). Conclusion: In conclusion, the analyzed data confirmed the research hypotheses. First, none of the 140 patients had cardiovascular diseases directly related to their condition, confirming that pectus carinatum is a pathology of a purely aesthetic nature. Second, a cheap, lightweight, and easily obscured brace significantly improved patient compliance. Along with this, the social relevance of the aesthetic aspect today may be an important factor in motivating the study cohort to adhere to therapy. In the past, esthetics and appearance were less relevant at the social level, which may have contributed to the high abandonment and reduced compliance rates of the many studies in the literature.
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BACKGROUND: Laparoscopic appendectomy followed by postoperative intravenous (IV) antibiotics is the standard of care for acute appendicitis and postoperative prevention of intra-abdominal abscesses. The aim of or study was to determine if intraperitoneal irrigation with antibiotics could help prevent intra-abdominal abscess formation after laparoscopic appendectomy for complicated appendicitis in pediatric patients. METHODS: A retrospective study was conducted on consecutive pediatric patients with acute appendicitis who had appendectomy in our Pediatric Surgery Department between August 2020 and February 2022. We compared two groups with similar age and symptoms. The first group (A) was treated with the normal standard of care, i.e., laparoscopic appendectomy and postoperative IV antibiotic therapy. For the second group (B) intraperitoneal cefazoline irrigation was added at the end of the laparoscopic procedure. Postoperative intra-abdominal abscess was diagnosed with ultrasound examination, performed after clinical suspicion/abnormal blood test results. RESULTS: One hundred sixty patients (males:females 109:51; median age 10.5 years [range 3-17 years]) who had laparosopic appendectomy for complicated appendicitis were included, 82 in group A and 78 in group B. In the first 7 days after surgery, 18 patients in group and 5 in group B developed an intra-abdominal abscess (p < 0.005). Drains were positioned in 38 patients in group A vs. 9 in group B. One patient in group A had a different complication which was infection of the surgical incision. CONCLUSIONS: Intraperitoneal cefazoline irrigation at the end of the laparoscopic appendectomy in pediatric patients significantly reduces the formation of intra-abdominal abscesses.
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Absceso Abdominal , Antibacterianos , Apendicectomía , Apendicitis , Laparoscopía , Complicaciones Posoperatorias , Humanos , Apendicectomía/efectos adversos , Niño , Estudios Retrospectivos , Absceso Abdominal/prevención & control , Absceso Abdominal/etiología , Masculino , Femenino , Preescolar , Adolescente , Apendicitis/cirugía , Complicaciones Posoperatorias/prevención & control , Antibacterianos/administración & dosificación , Antibacterianos/uso terapéutico , Cefazolina/administración & dosificación , Cefazolina/uso terapéutico , Lavado Peritoneal/métodosRESUMEN
PURPOSE: Femoral hernia (FH) is a rare and often misdiagnosed pathology in pediatric population. The aim of our study was to describe the experience of a Tertiary Center in children with FH, underlying diagnostic and surgical details that could improve its management. METHODS: A retrospective study of pediatric patients who underwent FH repair from January 2010 to June 2023 at our Institution was performed. RESULTS: In the analyzed period, 31 patients underwent surgical procedure for FH at our institution, of whom 16 (51.6%) were female. The mean age at time of surgery was 5.8 years (range 0.5-17.1 years). The rate of pre-operative misdiagnosis was 35.5% and open approach was adopted in all cases (16.1% with mesh application). Only two patients (6.4%) experienced surgical complications: one recurrence (repaired six months later) and one post-operative hematoma (treated successfully with conservative method). CONCLUSION: Due to the high rate of misdiagnosis, the variety of surgical approaches proposed, and the potential for intraoperative complications, FH poses a challenge for pediatric surgeons and urologists, as confirmed by the literature. It is essential to underline the importance of a proper clinical examination in order to correctly diagnose FH, make the best surgical plan for the patient and prevent post-operative complications.
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Hernia Femoral , Hernia Inguinal , Laparoscopía , Humanos , Niño , Femenino , Lactante , Preescolar , Adolescente , Masculino , Hernia Femoral/diagnóstico , Hernia Femoral/cirugía , Estudios Retrospectivos , Laparoscopía/métodos , Herniorrafia/efectos adversos , Herniorrafia/métodos , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Hernia Inguinal/cirugía , Mallas Quirúrgicas , RecurrenciaRESUMEN
BACKGROUND: Mild-to-moderate renal function loss may be an independent risk factor for cardiovascular disease and overall mortality. As in adults with renal carcinoma nephrectomy is associated with an high risk for moderate renal function loss, we aimed to assess the renal function adaptation over a long period of time in children with unilateral renal tumor (URT). PROCEDURE: Seventy-two children who underwent surgery for URT were enrolled in this study. Glomerular filtration rate was estimated (eGFR) with the Modification of Diet in Renal Study or the Schwartz equation, as appropriate for the age. RESULTS: Twelve patients treated by nephron-sparing surgery (Group A) and 42 treated by nephrectomy (Group B) had an age between 2 and 30 years; 18 patients treated by nephrectomy had an age between 33 and 51 years (Group C). At cross-sectional follow-up 8% patients of Group A, 42% of Group B and 78% of Group C presented a mild-to-moderate renal function. The longitudinal data stratified by post-operative intervals showed that patients of Group C presented a significant progressive decrease in mean ± standard deviation eGFR (88.1 ± 22.6 during the third decade after surgery vs. 66.6 ± 15.6 ml/min/1.73 m(2) during the fifth decade after surgery; P = 0.02). The longitudinal data stratified by age showed that patients with an age between 45 and 54 years presented a mean eGFR significantly lower than that expected for the physiological renal function decline with aging (P = 0.001). CONCLUSION: Aging is associated with a mild-to-moderate renal function loss in many adult patients following nephrectomy during childhood for URT.
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Envejecimiento , Tasa de Filtración Glomerular , Neoplasias Renales/fisiopatología , Neoplasias Renales/cirugía , Riñón/fisiopatología , Riñón/cirugía , Adulto , Factores de Edad , Niño , Preescolar , Estudios Transversales , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Introduction: The prevalence of life-threatening complications (LTCs) related to the minimally invasive repair of pectus excavatum (MIRPE) is unknown and underreported. The aim of this study is to contribute to the real prevalence of these rare but dramatic complications and show what went wrong in order to prevent it in the future. Case presentation: A 15-year-old boy affected by pectus excavatum with severe asymmetric deformity of the chest wall was evaluated for elective corrective surgery. Preoperative computed tomography showed a Haller index of 5.7 and a correction index of 0.40. MIRPE was performed under right video-assisted thoracoscopy. Cardiac arrhythmias occurred after placement of the bar introducer. The introducer was removed, and massive bleeding was noted. Emergency Clamshell thoracotomy was performed, and cardiac surgeon was alerted immediately. A first pulmonary wound was found and controlled. Two cardiac lacerations were found: on the interventricular wall and on the right atrium. Under cardiopulmonary bypass, cardiac lacerations were sutured and other three pulmonary wounds were repaired. An urgent fasciotomy was performed for compartmental syndrome of the right lower art after femoral cannulation. Pulmonary distress occurred; the patient was admitted on ECMO (ExtraCorporeal Membrane Oxygenation) in intensive care unit. Right lower lobectomy was carried out on the fifth postoperative day due to massive pulmonary bleeding requiring temporary tracheostomy. The patient was discharged to rehabilitation after 3 months with no brain injuries, minor hearing loss, and tracheostomy. Conclusion: We want to maintain the high alertness required for this procedure. Reporting these scaring complications contributes to the real prevalence of LTCs. We suggest the use of bilateral thoracoscopy and crane elevator in severe sternal defects. We also suggest to have a cardiac surgeon available in the hospital owing to cardiac perforation.
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Introduction: The digital chest drainage monitoring system (Medela Thopaz+), unlike analogical systems, reliably regulates the pressure applied to the patient's chest and digitally and silently monitors critical therapeutic indicators (volume of fluid and/or drained air). Its use in adulthood has been widely described, but there is still little experience in the pediatric field. The aim of this study is to test this new device in the pediatric population. Materials and methods: We conducted a retrospective study of 160 patients undergoing chest surgery at our Hospital. These patients were divided into 82 treated with the Thopaz system in the period from January 2021 to April 2023 and 78 in whom Pleurevac, had been used in the time period from January 2020 to April 2023. Results: The average age of patients was 10.45 years (range: 3.1-17.2) for the Thopaz Group and 10.71 years for Pleurevac Group. The groups were homogeneus also by weight and type of intervention. The device was held in place for 10.64 days (mean) for Thopaz Group, compared to 16.87 days (mean) for Pleurevac Group (p < 0.05). The median number of postoperative x-rays before the closure of the chest tube was 4.29 in the digital drainage group compared to 8.41 in the traditional draining group (p < 0.05). Conclusions: The digital chest monitoring device provides objective measurement, allows for rapid patient mobilization (with good pain control and increased compliance). In addition, the use of Thopaz in the paediatric population seems to be safe (there is no statistically significant difference in terms of complications such as prolonged air leaks and pneumothorax after the chest tube closure) and potentially beneficial.
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Background: Described for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS. Material and methods: A retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed. Results: We presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves. Conclusions: We obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings.
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Background and objectives: Marfan syndrome (MS) is a systemic disease of connective tissues consisting of a variable combination of anomalies. These patients have an increased risk of spontaneous pneumothorax (SP). However, there is a scarcity of pediatric literature on management, and no specific guidelines exist. Our aim was to analyze the management of spontaneous pneumothorax in children and adolescents with Marfan syndrome, comparing syndromic and non-syndromic patients. Methods: Retrospective analysis of pediatric patients (18 years) with SP diagnosed at our tertiary pediatric hospital (January 10-June 22), with special emphasis on diagnosis, treatment, and follow-up (FU). Results: Sixty-six patients with SP were identified, with nine (13%) having MS. In terms of baseline, there were no significant differences between the groups (age, sex, asthma, symptoms, and side, first-line treatment and hospitalization length). Overall, Marfan patients had significantly more first-line treatment failures requiring additional surgery, as well as more contralateral occurrences and the need for surgery/chest drain during the follow-up. Instead, conservative management resulted in significantly more ipsilateral recurrences and the need for surgery/chest drain in Marfan patients than controls during the follow-up. Conclusions: Treatment failure, contralateral occurrence, ipsilateral recurrence, and the need for surgery/chest drain during follow-up make management of patients with Marfan syndrome and spontaneous pneumothorax more difficult. In patients with a diagnosed MS a more aggressive first-line management should be considered, bearing in mind the higher risks of this population.
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Introduction: About 95% of congenital chest wall deformities are pectus abnormalities, with pectus excavatum (PE) being the most common. The purpose of this work is to offer a modified Taulinoplasty Technique based on 35 consecutive PE patients' 1-year single-center experience in 2022. Technique: One minimally invasive procedure for PE is taulinoplasty. In order to prevent invasion of the mediastinum or pleural cavity, it is considered that external traction can be used to raise the sternum. Our experience indicates that the most common surgical consequences of this procedure-which involves creating a submuscular and subcutaneous tunnel to install the metal device-are post-operative seroma and wound dehiscence. We modified the conventional method to achieve more aesthetically pleasing results. Discussion: Taulinoplasty seems to be a safe technique, easier and more feasible than standard Taulinoplasty, with better outcomes in terms of surgical complications, although further experience is necessary to confirm our preliminary data.
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PURPOSE: In patients who have undergone nephrectomy lower stage chronic kidney disease may develop, which is an independent risk factor for cardiovascular disease and overall mortality. We investigated whether the prevalence of lower stage chronic kidney disease is related to the amount of renal parenchyma excised in children with unilateral renal tumor. MATERIALS AND METHODS: A total of 15 patients treated with nephrectomy and 10 treated with nephron sparing surgery were enrolled at a single academic center. The Kidney Disease Outcomes Quality Initiative guidelines were used to classify patients by chronic kidney disease stage based on estimated glomerular filtration rate values. The Modification of Diet in Renal Disease study equation and Schwartz equation were used in patients older and younger than 17 years, respectively. RESULTS: At a mean followup of more than 12 years 8 patients who had undergone nephrectomy and 1 treated with bilateral nephron sparing surgery presented with stage II chronic kidney disease (estimated glomerular filtration rate 60 to 89 ml/min/1.73 m(2)). Sequential measurements from diagnosis to 12 to 17 years postoperatively showed that stage II chronic kidney disease in patients who had undergone nephrectomy manifested as a negligible postoperative increase in mean ± SD estimated glomerular filtration rate (75.7 ± 25.5 vs 79.4 ± 3.9 ml/min/1.73 m(2), p = 0.6). Five of the 8 patients presented with stage II chronic kidney disease even before nephrectomy. The other 7 patients who had undergone nephrectomy and those treated with nephron sparing surgery presented with a significant postoperative increase in mean ± SD estimated glomerular filtration rate (81.1 ± 24 vs 102.3 ± 3 ml/min/1.73 m(2), p = 0.02, and 88.7 ± 2 vs 107.4 ± 14 ml/min/1.73 m(2), p = 0.005, respectively). CONCLUSIONS: A subset of children with unilateral renal tumor presents before and/or after nephrectomy, and not after nephron sparing surgery, with stage II chronic kidney disease, probably due to a reduced renal reserve capacity. Whether patients with preoperative renal dysfunction may benefit from nephron sparing surgery should be studied in a cooperative clinical trial setting.
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Carcinoma de Células Renales/epidemiología , Neoplasias Renales/epidemiología , Neoplasias Renales/cirugía , Nefrectomía , Insuficiencia Renal Crónica/epidemiología , Quimioterapia Adyuvante , Niño , Estudios Transversales , Tasa de Filtración Glomerular , Humanos , Neoplasias Renales/tratamiento farmacológico , Nefrectomía/métodos , Prevalencia , Insuficiencia Renal Crónica/fisiopatología , Tumor de Wilms/epidemiología , Tumor de Wilms/secundarioRESUMEN
BACKGROUND: The use of pneumatic dilation (PD) is well established in adults with achalasia; however, it is less commonly used in children. OBJECTIVE: To evaluate the efficacy of PD in pediatric achalasia and to define predictive factors for its treatment failure. DESIGN: Single-center, prospective cohort study. SETTING: Academic tertiary referral center. PATIENTS: Twenty-four patients with achalasia were enrolled from January 2004 to November 2009 and were followed for a median of 6 years. INTERVENTION: PD was performed with the patients under general anesthesia. MAIN OUTCOME MEASUREMENTS: Efficacy and safety of PD. Follow-up was performed by using the Eckardt score, barium swallow contrast studies, and esophageal manometry at baseline; 1, 3, and 6 months after dilation; and every year thereafter. A Cox regression model was used to identify independent predictors of failure after the first PD. RESULTS: The PD success rate was 67%. In 8 patients, the first PD failed, but the parents of one patient refused a second PD and requested surgery. Of the 7 patients who underwent repeated treatment, the second PD failed in 3 (43%). Overall, only 3 of the 24 patients underwent surgery (overall success rate after a maximum of 3 PDs was 87%). Multivariate analysis showed that only older age was independently associated with a higher probability of the procedure success (hazard ratio [HR] 0.66; 95% CI, 0.45-0.97). LIMITATIONS: Small sample size, single-center study. CONCLUSIONS: PD is a safe and effective technique in the management of pediatric achalasia. Young age is an independent negative predictive factor for successful clinical outcome.