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1.
Takaharu Oue Tsugumichi Koshinaga Tetsuya Takimoto Hajime Okita Yukichi Tanaka Miwako Nozaki Masayuki Haruta Yasuhiko Kaneko Masahiro Fukuzawa Renal Tumor Committee of the Japanese Children’s Cancer Group 《Pediatric surgery international》2016,32(9):851-855
Purpose
To evaluate the clinical features and treatment results of anaplastic histology (AH) Wilms’ tumor (WT) patients registered in the Japan Wilms’ Tumor Study (JWiTS) group to elucidate the clinical characteristics of AH in the Japanese population.Patients and methods
Of 344 WT patients who were enrolled in JWiTS between 1995 and 2013, 17 had AH. Treatment using the JWiTS protocols was similar to the fifth National Wilms’ Tumor Study 5 (NWTS-5) protocols. Clinical characteristics and mutation status of TP53 gene were evaluated and compared with those in NWST-5 study.Results
AH incidences in JWiTS were 4.9 %, lower than that in NWTS-5. Seven tumors had focal AH and 10 had diffuse AH. Clinical stages of AH patients were stage I in seven, stage II in three, stage III in five, stage IV in one and unknown in one. Four-year event-free survival and overall survival rates were 90.9 and 86.7 %, respectively. Two patients with diffuse AH and none with focal AH had TP53 mutation.Conclusion
Japanese patients presented with higher incidence, earlier stages and may have better outcomes than American patients, indicating a possible biological heterogeneity of AH WT. Further analysis is necessary to elucidate the different characteristic of AH WT between Japanese and American populations.2.
Objectives
To study the etiology and burden of comorbidities across stages of chronic kidney disease (CKD).Methods
Children, 2–16 y of age with CKD Stages II- IV were recruited over 12 mo. The etiology, clinical presentation and severity of complications were studied.Results
Among 78 children [Stage II (n = 21), Stage III (n = 26), Stage IV (n = 31)], congenital anomalies of the kidney and urinary tract (CAKUT) was the commonest etiology and 28 were newly diagnosed in Stage III /IV. High prevalence of comorbidities was observed with growth retardation (65%), hypertension (59%), hyperphosphatemia (32%), vitamin D deficiency (92%), dyslipidemia (64%), left ventricular hypertrophy (45%) and hyperparathyroidism (56%). While most comorbidities correlated with the estimated glomerular filtration rate and severity of CKD, hypertension, vitamin D deficiency and cardiovascular morbidity were prevalent even in early stages.Conclusions
CAKUT was the commonest cause of CKD. Late detection and high prevalence of comorbidities even in early stages of CKD were observed.3.
Purpose
To describe our clinical experience with nephron sparing surgery (NSS) for bilateral Wilms tumor and evaluate the outcomes of patients treated at one of the largest pediatric medical centers in China.Methods
Medical records of children with bilateral Wilms tumor undergoing NSS in the Children’s Hospital of Chongqing Medical University during a 15-year period were retrospectively analyzed. Data collected were composed of age at surgery, tumor response, tumor rupture during resection, final pathologic margins, use of radiation therapy, pathology reports, renal function, and patient survival.Results
A total of 18 eligible patients (10 males, 8 females) with bilateral Wilms tumor at a mean age of 2.28?±?1.12 years were identified. The administration of preoperative chemotherapy did not result in universally successful outcomes. All children underwent successfully unilateral or bilateral NSS, of which one had positive pathologic margins and five received radiation therapy postoperatively. The rates of tumor rupture and positive lymph nodes involvement were 11.1 and 19.4%, respectively. The pathological study showed favorable histology and unfavorable histology in 32 and 4 kidneys, respectively. The 4-year event-free survival and overall survival rates were 68.18 and 85.56%. In univariable analysis, tumor histology (p?=?0.0028) and disease stage (p?=?0.0303) appeared significantly associated with overall survival. After a median follow-up period of 41.5 months (range 10–89), three of the surviving patients were diagnosed with hypertension and one had renal insufficiency.Conclusions
Our experience suggests that NSS has become a feasible and effective option with good oncologic outcomes. Further research, ideally in a multicenter randomized manner, is warranted to better assess the role of NSS in this challenging clinical scenario.4.
Purpose
To evaluate the epidemiologic, demographic, and clinical characteristics, as well as prognostic factors and long-term outcomes of mediastinal germ cell tumors (MGCT) in children.Patients and methods
A retrospective study of pediatric patients diagnosed with a primary MGCT between January 1963 and August of 2014 was performed.Results
Twenty-five patients were identified. Six children with teratomas were treated with resection alone (median age 7.8 years, range newborn to 15 years) and were cured without recurrence or progression. Nineteen children were treated for a malignant MGCT (median age 11.7 years, range 7 months–18 years); 5 year overall survival (OS) was 0.39 ± 0.12. For malignant non-seminomatous mediastinal germ cell tumors, platinum-based chemotherapy regimen (OS 0.56 vs 0.14, p = 0.03), complete surgical resection with negative margins (OS 0.73 vs 0.11, p = 0.03); and localized disease (OS 0.76 vs 0.0, p = 0.004) demonstrated a survival advantage.Conclusions
Initial surgical resection is appropriate for teratomas. Localized disease, complete resection, and platinum-based chemotherapy are associated with improved survival in malignant non-seminomatous mediastinal germ cell tumors. Neoadjuvant, platinum-based three drug regimens followed by delayed surgical resection is the appropriate treatment modality for malignant mediastinal germ cell tumors.5.
Background
Hypertension is a prevalent cardiovascular disease risk factor among blacks and adolescent hypertension can progress into adulthood.Objective
To determine the prevalence of hypertension and prehypertension among secondary school adolescents in Enugu South East Nigeria.Methodology
A study of 2694 adolescents aged 10-18 years in Enugu metropolis was carried out. Socio-demographic profile anthropometric and blood pressure readings were obtained. Derived measurements such as Prehypertension, hypertension and BMI were obtained.Results
The results showed that the mean systolic blood pressure and diastolic blood pressure for males were 106.66+ 11.80 mmHg and 70.25 + 7.34 mmHg respectively. The mean SBP and DBP for females were 109.83+ 11.66 mmHg and 72.23 + 8.26 mmHg respectively (p < 0.01). Blood pressure was found to increase with age. Prevalence of hypertension and prehypertension was 5.4% and 17.3% respectively with a higher rate in females (6.9%) than males (3.8%). Prevalence of prehypertension among males and females were 14.3% and 20.1% respectively. The prevalence of obesity was 1.9%.Conclusion
Modifiable risk factors exist among adolescents. Early lifestyle modification and a strengthened school health are recommended.6.
Justin T. Huntington Laura A. Boomer Victoria K. Pepper Karen A. Diefenbach Jennifer L. Dotson Benedict C. Nwomeh 《Pediatric surgery international》2016,32(5):459-464
Purpose
Single-incision laparoscopic surgery (SILS) has been described in adults with Crohn’s disease, but its use in pediatric Crohn’s patients has been limited. The purpose of this study was to review our experience with SILS in pediatric patients with Crohn’s disease.Methods
A retrospective review was performed for patients diagnosed with Crohn’s disease who underwent small bowel resection or ileocecectomy at a freestanding children’s hospital from 2006 to 2014. Data collected included demographic data, interval from diagnosis to surgery, operative time, length of stay, and postoperative outcomes.Results
Analysis identified 19 patients who underwent open surgery (OS) and 41 patients who underwent SILS. One patient (2.4 %) within the SILS group required conversion to OS. Demographic characteristics were similar between the 2 cohorts. The most common indication for surgery was stricture/obstruction (SILS 70.7 % vs. OS 68.4 %, p = 0.86), and ileocecectomy was the most common primary procedure performed (SILS 90.2 % vs. OS 100 % OS). Operative times were longer for SILS (135 ± 50 vs. 105 ± 37 min, p = 0.02). However, when the last 20 SILS cases were compared to all OS cases, the difference was no longer statistically significant (SILS 123.3 ± 34.2 vs. OS 105 ± 36.5, p = 0.12). No difference was noted in postoperative length of stay (SILS 6.5 ± 2.2 days vs. OS 7.4 ± 2.2 days, p = 0.16) or overall complication rate (SILS 24.4 % vs. OS 26.3 %, p = 0.16).Conclusion
SILS ileocecectomy is feasible in pediatric patients with Crohn’s disease, achieving outcomes similar to OS. As experience increased, operative times also became comparable.7.
Jian-Fang Zhou Zhi-Yong Chen Shan-Ming Yang Jia-Zhen Chen Ling-Ye Zhou Ya-Fen Wang Gang Wang Xia-Jian Yu Wen-Hong Zhang 《Indian journal of pediatrics》2017,84(2):124-127
Objective
To investigate the changes in lymphocyte subsets that are caused by infection with different pathogens in children with hand, foot, and mouth disease.Methods
T lymphocyte subsets were measured in the patients’ peripheral blood, and serum, throat swab, and fecal samples were tested for enterovirus.Results
Fecal and throat swab samples exhibited similar positive detection rates, and were significantly more likely to be positive, compared to serum samples (P < 0.01). The EV71-positive group exhibited significantly lower CD4 + TM cell counts (QR: 1.058), compared to the CD4 + TM cell counts in the CoxA16-positive group (QR: 1.391; P < 0.05).Conclusions
Throat swab and fecal samples exhibited significantly higher positive detection rates, compared to serum samples. In addition, EV71-infected children exhibited significantly lower CD4+ T-cell counts, compared to CoxA16-infected children, which suggests that EV71 infection may be associated with a poorer prognosis.8.
Carlos Giuseppucci Aixa Reusmann Verónica Giubergia Carolina Barrias Anahi Krüger Mónica Siminovich Hugo Botto Martin Cadario Mariano Boglione Julieta Strambach Marcelo Barrenechea 《Pediatric surgery international》2016,32(5):451-457
Purpose
Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years.Methods
A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990–2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome.Results
Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91–16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology.Conclusion
Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.9.
Maria G. Sacco Casamassima Colin D. Gause Seth D. Goldstein Fizan Abdullah Avner Meoded Jeffrey R. Lukish Christopher L. Wolfgang John Cameron David J. Hackam Ralph H. Hruban Paul M. Colombani 《Pediatric surgery international》2016,32(8):779-788
Purpose
Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies.Methods
We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015.Results
Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5–18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7–10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma.Conclusions
Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.10.
Valentina Rossi Cinzia Mazzola Lorenzo Leonelli Paolo Gandullia Serena Arrigo Marina Pedemonte Maria Cristina Schiaffino Margherita Mancardi Oliviero Sacco Nicola Massimo Disma Clelia Zanaboni Giovanni Montobbio Arrigo Barabino Girolamo Mattioli 《Pediatric surgery international》2016,32(3):277-283
Background
Fundoplication is considered a mainstay in the treatment of gastro-esophageal reflux. However, the literature reports significant recurrences and limited data on long-term outcome.Aims
To evaluate our long-term outcomes of antireflux surgery in children and to assess the results of redo surgery.Methods
We retrospectively analyzed all patients who underwent Nissen fundoplication in 8 consecutive years. Reiterative surgery was indicated only in case of symptoms and anatomical alterations. A follow-up study was carried out to analyzed outcome and patients’ Visick score assessed parents’ perspective.Results
Overall 162 children were included for 179 procedures in total. Median age at first intervention was 43 months. Comorbidities were 119 (73 %), particularly neurological impairments (73 %). Redo surgery is equal to 14 % (25/179). Comorbidities were risk factors to Nissen failure (p = 0.04), especially children suffering neurological impairment with seizures (p = 0.034). Follow-up datasets were obtained for 111/162 = 69 % (median time: 51 months). Parents’ perspectives were excellent or good in 85 %.Conclusions
A significant positive impact of redo Nissen intervention on the patient’s outcome was highlighted; antireflux surgery is useful and advantageous in children and their caregivers. Children with neurological impairment affected by seizures represent significant risk factors.11.
Background
Sacrococcygeal teratoma (SCT) is a rare childhood malignancy. Although overall survival is favorable, recurrent tumors are associated with poor outcomes. As most recurrences occur within 3 years of presentation, the utility of long-term surveillance is uncertain.Methods
Patients with SCTs evaluated and managed by our pediatric surgery department between 1986 and 2013 were included. Details pertaining to laboratory values, operative findings, tumor histology, management, recurrence, and outcomes were recorded and analyzed.Results
During the study period, 40 children with the diagnosis of SCT were managed by our practice. Five (13%) developed a recurrence. The median age at the initial resection was day of life two (range day of life 0–2.5 years). The median time to recurrence was 5 years (range 5 months–15 years). Among those with recurrences, mature teratoma was the most common histological type on the initial resection (n = 3), with yolk sac and immature teratomas comprising the other two. At the time of recurrence, three patients had mature teratomas, and all are alive and well following resection. Two patients had yolk sac tumors at the time of recurrence and both died.Conclusion
SCT can recur many years after the initial resection. Our findings suggest that all patients with SCT should be closely followed into adulthood.12.
Purpose
To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus.Methods
A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee.Results
From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free.Conclusion
A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.13.
Purpose
Fifty years ago, Bolande described Congenital Mesoblastic Nephroma (CMN) as a benign lesion. Unexpected aggressive clinical behaviors prompted a sub-classification based on histology. Recent molecular genetic evidence has identified the aggressive cellular variant to be the renal manifestation of congenital infantile fibrosarcoma. We submit a reappraisal and analysis of the available literature on recurrent and metastatic CMN.Methods
An electronic search of PubMed, MEDLINE, EMBASE, and Scopus yielded 38 children with local recurrence and/or metastases.Results
Of the 38 children with local recurrence and/or metastasis, 59% were girls. Median time to recurrence was 6 months (range 1–12 months). The commonest sites of metastases were the lung (39%) and liver (29%). Fifty percent of these children died of disease. The outcome of additional chemotherapy (p = 0.5) did not differ from that of surgery alone. The choice of chemotherapy did not influence the outcome (p = 0.6).Conclusions
Recurrence and metastasis in cellular CMN are much more common than described earlier and carry a high mortality. Children with cellular and mixed CMN require close clinical and radiological follow-up for a minimum of 12 months after primary surgery. Surgery is the mainstay of the treatment of recurrent and metastatic lesions. Neoadjuvant chemotherapy is recommended only if the lesion is inoperable. Targeted therapy may be an option in treatment of refractory cases.14.
Purpose
Tracheal cartilage reconstruction is an essential approach for the treatment of tracheal congenital abnormalities or injury. Here, we evaluated the use of allogeneic decellularized tracheas as novel support scaffolds.Methods
Six weaned pigs (4-week-old domestic males) were transplanted with allogeneic tracheal graft patches (three decellularized and three fresh tracheal scaffolds) onto artificial defects (approximately 15 × 15 mm). After 11 weeks, the tracheas were evaluated by bronchoscopy and histological studies.Results
No pigs displayed airway symptoms during the observation period. Tracheal lumen restored by fresh graft patches showed more advanced narrowing than that treated with decellularized grafts by bronchoscopy. Histologically, fresh grafts induced typical cellular rejection; this was decreased with decellularized grafts. In addition, immunohistochemistry demonstrated regenerating foci of recipient cartilage along the adjacent surface of decellularized tracheal grafts.Conclusion
Decellularized allogeneic tracheal scaffolds could be effective materials for restoring impaired trachea.15.
Objective
To quantitatively summarize the evidence from observational studies on the relation between pediatric asthma and food allergy.Methods
A literature search was conducted in Medline and EMBASE (August 2016). Two independent reviewers appraised the studies and extracted the estimates of interest. Methodological quality of the included studies was assessed using National Heart Lung and Blood Institute (NHLBI) Quality Assessment Tools. Data were pooled using random-effects meta-analysis.Results
A total of 32 relevant studies were identified but only 8 studies met the inclusion criteria. Using random-effect model, food allergy showed strong association with asthma in children (OR = 2.87 [95% CI: 2.05–4.00]; P < 0.0001).Conclusions
This study suggested that food allergy is associated with an increased risk of asthma in children.16.
Background
The effects and potential hazards of brain magnetic resonance imaging (MRI) at 3 T in newborns are debated.Objective
Assess the impact of 3-T MRI in newborns on body temperature and physiological parameters.Material and methods
Forty-nine newborns, born preterm and at term, underwent 3-T brain MRI at term-corrected age. Rectal and skin temperature, oxygen saturation and heart rate were recorded before, during and after the scan.Results
A statistically significant increase in skin temperature of 0.6 °C was observed at the end of the MRI scan (P<0.01). There was no significant changes in rectal temperature, heart rate or oxygen saturation.Conclusion
Core temperature, heart rate and oxygen saturation in newborns were not affected by 3-T brain MR scanning.17.
Background
Acute hemorrhages in neonates and young children can be compensated for a long period of time until a decrease in blood pressure and manifest shock occur.Objective
To determine the characteristics of the pathophysiology of the circulation in children and to recognize critical signs and symptoms of hemorrhage.Material and methods
Pathophysiology and discussion of the literatureResults
Shock in neonates and children can be present long before a decrease in blood pressure occurs. It is characterized by tachycardia, tachy(dys)pnea, prolonged capillary refilling time, disorders of consciousness (apathy), disturbance of temperature regulation and reduced urine output. Laboratory markers are metabolic acidosis and elevated lactate and a normocytic anemia earlier than in adults.Conclusion
In contrast to adults a decrease in blood pressure in neonates and young children is a late sign of shock, whereas normocytic anemia occurs earlier than in adults.18.
Xin-Yu Li Xin Sun Jing Chen Mao-Quan Qin Zuo Luan Yi-Ping Zhu Jian-Pei Fang 《World journal of pediatrics : WJP》2018,14(1):92-99
Background
β-Thalassemia major (β-TM) has become a public health problem in mainland China. Hematopoietic stem cell transplantation (HSCT) has remained the only cure for β-TM in mainland China since 1998.Methods
This multicenter retrospective study provides a comprehensive review of the outcomes of 50 pediatric patients with β-TM who received HSCT between 1998 and 2009 at five centers in mainland China. Both related (n = 35) and unrelated donors (n = 15) with complete human leukocyte antigen matches were included. The stem cell sources included bone marrow (BM), peripheral blood stem cells, umbilical cord blood (UCB) and a combination of BM and UCB or a combination of BM and peripheral blood stem cells from a single sibling donor.Results
The probabilities of 5-year overall survival (OS) and thalassemia-free survival (TFS) after the first HSCT were 83.1 and 67.3%, respectively. Graft failure (GF) occurred in 17 patients. Univariate analyses showed that umbilical cord blood transplantation (UCBT) was one of the potential risk factors for decreased OS (P = 0.051), and that UCBT (P = 0.002) was potentially related to TFS. GF incidence was distinct between the UCBT and non-UCBT groups (P = 0.004). Four cases of UCB-BM combined transplantation led to decreased risks of mortality and recurrence. In the UCBT group, related donor transplantation produced more favorable results than unrelated donor transplantation in OS (P = 0.009) but not in TFS (P = 0.217).Conclusions
GF was the primary cause of UCBT failure. Though UCBT from related donors was not favorable, the combined transplantation of UCB and BM could improve the prognosis of UCBT.19.