Diagnosis and outcome of neutropenic enterocolitis: experience in a single tertiary pediatric surgical center in China

Background/purpose  

Neutropenic enterocolitis (NE) is clinically defined by the triad of neutropenia, abdominal pain and fever. This retrospective study is to review 24 cases of NE in a single Chinese tertiary center, to elucidate clinical feature, treatments and outcome for this dangerous gastrointestinal complication of neutropenia.     

Risk of hearing impairment in pediatric liver transplant recipients: a single center study

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs. We conducted a review of 74 children who had undergone liver transplantation between December 1996 and September 2000 at Cincinnati Children's Hospital Medical Center. Hearing was assessed at discharge by an audiologist using age and developmentally appropriate techniques. The principal outcome measure was sensorineural hearing impairment. Independent variables were age at transplantation, United Network for Organ Sharing (UNOS) status at transplantation, primary diagnosis, post-transplant length of hospital stay, days of treatment with aminoglycosides, and days of treatment with loop diuretics. Eleven of 74 children (15%) had sensorineural hearing loss, of whom four had severe to profound hearing loss. Multivariate analyses showed that the adjusted relative risk for hearing loss in patients with hepatoblastoma was 66 and that there was a 5% increase risk for hearing loss for each additional day of hospitalization. Age at transplantation, UNOS status, and days of treatment with loop diuretics or aminoglycosides did not achieve significance in the model. Sensorineural hearing impairment occurs in a subset of pediatric patients following liver transplantation. Patients with hepatoblastoma or those who experience prolonged hospitalization after transplantation are at increased risk. Our observations are of particular importance for pediatric liver transplant recipients since the median age at transplantation is 12-18 months, a critical period for language acquisition.     

Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9?±?68.7?months (range: 2–202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3?days (1–67?days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1?=?2.3[1.48–3.43]) and etoposide usage (OR2?=?1.22 [1.14–1.89]). The patient’s 30-day survival was inferior among patients whose albumin level was 2?g/dL or less compared to those over 2?g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.     

Analysis of the pediatric surgery match: factors predicting outcome

Introduction  

Applicants in the NRMP for pediatric surgery have little objective data available regarding factors predicting successful matching. We analyzed data from applicants at our institution to attempt to identify parameters correlated with three outcomes: successfully matching, or attaining either a top ten or top three ranking in our final submitted match list.     

Mycophenolate mofetil in pediatric renal transplantation: a single center experience

We assessed our long-term experience with regards to the safety and efficacy of MMF in our pediatric renal transplant population and compared it retrospectively to our previous non-MMF immunosuppressive regimen. Forty-seven pediatric renal transplants received MMF as part of their immunosuppressive protocol in the period from January 1997 till October 2006 (MMF group). A previously reported non-MMF group of 59 pediatric renal transplants was included for comparative analysis (non-MMF group). The MMF group comprised 29 boys and 18 girls, whereas the non-MMF group comprised 34 boys and 25 girls. Mean age was 11.7 and 12 yr in the MMF and non-MMF groups, respectively. The incidence of acute rejection episodes was 11 (23.4%) and 14 (24%) in the MMF and non-MMF group, respectively. Two (3.3%) grafts were lost in the non-MMF group compared with one (2.1%) in the MMF group. Twenty-one (44.68%) patients in the MMF group developed post-transplant infections compared with 12 (20.33%) in the non-MMF group (p < 0.0001). In conclusion, the use of MMF in pediatric renal transplantation was not associated with a lower rejection rate or immunological graft loss. It did, however, result in a significantly higher rate of viral infections.     

Sonography of pediatric male breast masses: gynecomastia and beyond

Background: Palpable breast masses are rare in the pediatric male population. Prior reports have described the ultrasound findings of the normal pediatric breast, masses seen primarily in female pediatric patients, and masses seen in adult males. Objective: To describe and illustrate the sonographic findings in gynecomastia and other causes of breast masses in a group of pediatric males. Materials and methods: We reviewed the ultrasound database of a large tertiary-care childrens hospital for male pediatric patients presenting with breast masses from 1994 to 2000. The findings were correlated with additional imaging and pathology results. Results: Twenty-five pediatric patients ranging in age from 1 month to 18 years were referred for breast ultrasound. Eighteen patients (72%) had gynecomastia. Two patients each (8%) had galactocele(s) or had postoperative hematoma. One patient had ductal ectasia, which resolved. One patient had periductal hemangioma. One patient with neurofibromatosis and a solid mass was lost to follow-up. Conclusion: Palpable breast masses are rare in the male pediatric patient and sonography is the primary imaging modality. The overwhelming majority of these cases are adolescent boys presenting with gynecomastia. Other diagnoses such as galactocele(s), hemangiomas, and ductal ectasia should be considered when young male patients present with a palpable mass. Malignant breast lesions are rare and are likely to be metastatic or primary tumors of nonbreast tissue origin.     

Surgical treatment of pediatric and adolescent papillary thyroid cancer: a retrospective study of 54 patients in a single center

ObjectiveIn 2015, American Thyroid Association (ATA) issued the first version of Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer. The purpose of this study is to evaluate whether the ATA pediatric guidelines recommended surgical approach for the patient can be applied to surgical treatment of pediatric PTC in China.MethodFrom April 2012 to December 2020, clinical data of children (≤18 years) with PTC consecutively admitted and treated with initial surgery in the study's department were retrospectively reviewed.ResultsThe authors found that the central lymph node metastasis (CLNM) rate was significantly higher than that in the lateral neck (83.33 % vs 62.96%, χ² = 5.704, p = 0.017) .The lymph node metastasis rate was significantly lower in cN1b (-) patients than in cN1b (+) patient (55.00% vs 100.00%, χ² = 15.263, p = 0.000); Meanwhile, the CLNM and LLNM rates of ipsilateral were significantly higher than those of contralateral central compartment (83.33?vs 57.41?%, χ² = 8.704, p = 0.003). Lymph nodes of 51 lateral lymph node dissection (LND) were analyzed, which revealed the LNM rate of cN1b (-) patients was significantly lower than that of cN1b (+) patients (55.00% vs. 100.00%, χ² = 15.263, p = 0.000).ConclusionChildren and adolescents have a higher rate of lymph node metastasis at the time of diagnosis. TT should be conducted in the majority of children with PTC. CND should be routinely performed; therapeutic LND is recommended for children with cN1b (+).     

Neurological complications of liver transplantation in pediatric patients: a single center experience

Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 +/- 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilson's disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilson's disease had a higher incidence of NCs (60%) than did patients without Wilson's disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.     

Incidence of iatrogenic withdrawal syndrome and associated factors in surgical pediatric intensive care

BackgroundIatrogenic withdrawal syndrome (IWS) is a complication of prolonged sedation/analgesia in pediatric intensive care unit (PICU) patients. The epidemiology of IWS is poorly understood, as validated diagnostic tools are rarely used. The main objective of our study was to use the WAT-1 score to assess the incidence of IWS in our unit. The secondary objectives were to evaluate the consequences of IWS, associated factors, and management modalities.Material and methodsFrom July 2018 to January 2019, 48 children receiving endotracheal ventilation and sedation/analgesia by continuous infusion (>48 h) of benzodiazepines and/or opioids were included. As soon as sedation/analgesia was decreased and until 72 h after its complete discontinuation, the WAT-1 score was determined every 12 h. Substitution therapy was used for 98% of patients upon opioid and/or benzodiazepine withdrawal. IWS was defined as a WAT-1 score ≥3. Factors associated with IWS were assessed by univariate analysis.ResultsIWS occurred in 25 (52%) patients. IWS was associated with a higher number of ventilator-associated pneumonia episodes (17 [68%] vs. one [4%]) and a longer PICU stay (13 [7; 25] vs. 9.0 [5.0; 10.5]) (p<0.001). Overall, 11 patients developed IWS after less than 5 days of sedation/analgesia. Severe head injury was associated with IWS (p = 0.03). Neither sedation discontinuation nor IWS prevention was standardized.ConclusionThe high incidence and adverse consequences of IWS require improved prevention. Risk groups should be defined and a standardized withdrawal protocol established. The occurrence of IWS should be monitored routinely using a validated score.     

Rapid steroid discontinuation for pediatric renal transplantation: a single center experience

To determine the outcomes of pediatric renal transplant recipients who received immunosuppression consisting of early withdrawal of corticosteroids at a single Northern California center. Protocols using minimal steroid exposure have been recently reported in adult transplant recipients with successful results. We examined the outcomes of pediatric renal transplant recipients who were managed at our center using a protocol with very early discontinuation of steroids after renal transplantation. We retrospectively studied the medical records of all renal transplant recipients followed at the Children's Hospital at the University of California, Davis Medical Center from 01/2004 to 12/2005. All patients were less than 18 yr of age at the time of transplantation. The immunosuppressive protocol included three tapering daily doses of methylprednisolone, together with five doses of thymoglobulin followed by maintenance therapy with tacrolimus and MMF. Eight patients with equal numbers of males and females were transplanted during this time period. There were equal numbers of Caucasians, African-Americans, Hispanics, and Asians. A total of 37.5% (3/8) of the subjects received preemptive transplantation, 25% (2/8) received peritoneal, and 37.5% (3/8) received hemodialysis before transplantation. The median (range) age at transplantation was 12.3 (3.1-16.0) year with a follow-up of 1.7 (0.9-2.8) year. At one yr post-transplantation, 57% (4/7) of patients still required anti-hypertensives. Three children required erythropoietin supplementation after transplantation. The mean delta height standard deviation score at 12 months was 0.20 +/- 0.56. There were no episodes of clinical acute rejection. One patient switched from tacrolimus to sirolimus due to biopsy-proven CAN. No patient became diabetic or required hypoglycemic agents. Surveillance biopsies showed no subclinical acute rejection in any patient. Steroid-free immunosuppression is safe in children after renal transplantation. Larger number of patients and longer follow-up are required to further confirm the effectiveness and safety of immunosuppression with rapid steroid discontinuation.     

隐睾患儿手术年龄变化趋势及相关影响因素分析

目的 本研究拟分析隐睾患儿临床特点及手术年龄的变化趋势,同时探索影响隐睾手术时机的相关因素.方法 回顾性分析上海交通大学医学院附属新华医院2007年1月至2015年10月间接受手术治疗的隐睾患儿.收集患儿的人口统计学数据、合并阴茎发育异常情况、手术时间及其他影响因素等资料.运用t检验及多元线性回归分析对影响隐睾手术时机相关因素进行分析,P＜0.05为差异有统计学意义.结果 共有1 607例隐睾患儿被纳入本研究,其中单侧隐睾1449例(90.17％),合并阴茎发育异常的患儿112例(7％),平均手术年龄为39个月,有81％的患儿于1岁后接受手术治疗.民族、医疗保险类型、家庭收入、城乡差异以及隐睾侧别对手术年龄没有影响.多因素分析结果提示:腹腔内型隐睾(t=3.021,P=0.000)、合并阴茎畸形(t=3.916,P=0.000)、父母文化程度(t=3.563,P=0.000)、等待手术时间(t=2.657,P＜0.05)以及获得专业小儿外科医师治疗意见的时间(t=7.751,P=0.000)明显影响手术时机,结果有统计学意义.结论 在所研究的隐睾患儿中,大多数患儿的手术年龄在1岁以后.对患儿家长以及非专科医务人员进行隐睾知识的健康教育可能会提前隐睾的手术时机.     

Long‐term outcome of pediatric renal transplantation: A single center study in Japan

Little is known about the risk factors for long‐term poor outcome in pediatric renal transplantation. Between 1973 and 2010, 111 renal transplants (92 living donations) were performed in 104 children (56 males, mean age, 12.5 yr) at the Social Insurance Chukyo Hospital, and followed‐up for a mean period of 13.6 yr. The patient survival at 1, 5, 10, 15, 20 (living‐ and deceased‐donor transplants), and 30 yr (living‐donor transplants only) was 98.1%, 92.8%, 87.8%, 84.9%, 82.6%, and 79.3%. The graft survival at 1, 5, 10, 15, 20, and 30 yr was 92.0%, 77.3%, 58.4%, 50.8%, 38.5%, and 33.3%. The most common cause of graft loss was CAI, AR, death with functioning, recurrent primary disease, ATN, and malignancy. Donor gender, ATN, malignancy/cardiovascular events, and eras affected patient survival. AR and CAI were the risk factors for graft loss. The evolved immunosuppression protocols improved the outcome by reducing AR episodes and ATN but not CAI, suggesting CAI as the major risk factor for graft loss. CAI was correlated with AR episodes, CMV infection, and post‐transplant hypertension. Strategies for preventing the risk factors for malignancy/cardiovascular events and CAI, including hypertension/infection, are crucial for better outcomes.     

Incidence and risk factors for cardiovascular events and death in pediatric renal transplant patients: A single center long‐term outcome study

Abstract: There are considerable mortality data associated with renal transplantation in children; however, morbidity data, especially related to CV disease, are scarce. The objectives of this study were to determine incidence of non‐fatal and fatal CV events and all‐cause mortality in PRTx and evaluate risk factors for these conditions. Using a population‐based retrospective cohort design, 274 PRTx with or without a functioning graft was followed until death or date of last contact (median follow‐up 11.9 yr). Primary outcomes (time to first fatal or non‐fatal CV event and all‐cause mortality after first transplant) were ascertained from chart review and linkage with administrative databases of a universal health care system. During 3073 patient‐years, there were 46 deaths; 13 were because of CV disease. Twenty patients had CV events that did not result in death. Post‐transplant diabetes mellitus (10.5%) was associated with increased risk of death (HR: 2.79, 95% CI: 1.04–7.44) and CV events (HR: 3.90, 95% CI: 1.31–11.59). Low estimated glomerular filtration rate at one yr post‐transplant was also associated with increased risk of death. The rates of developing CV disease and dying prematurely are extraordinarily high in PRTx, underscoring the need for early and aggressive intervention to reduce the burden of suffering in this patient population.     

Medical and surgical treatment of neonatal hemochromatosis: single center experience

NH is a rare disorder of iron storage in newborns resulting in rapid liver failure. Outcomes are dismal with 20-30% survival. We report our experience in eight children with NH. Assessment of liver function included admission PT and serum levels of FV and FVII. Medical treatment (antioxidant cocktail) was started in all patients, with chelation therapy in six. Of these six, three survived with medical treatment alone. The other three underwent liver transplant. One died 158 days after transplant to sepsis: two are well more than five yr after transplant. The two neonates who did not receive chelation therapy, died to multi-organ failure and sepsis. In summary, five children (62.5%) survived long-term. In the three transplanted, one- and five-yr-survival was 66%. Older children with compromised synthetic liver function (FVII levels < or = 15%) required liver replacement for survival. Early referral to a tertiary care center is essential to increase survival of these children with a rare and otherwise fatal disease. Single center experience of children with NH is here presented. Potentials for survival improvement with of medical and surgical treatment are examined.     

Once-daily intravenous busulfan for 47 pediatric patients undergoing autologous hematopoietic stem cell transplantation: a single center study

We report our results using once-daily intravenous (IV) busulfan (Bu)-based conditioning adjusted by weight without drug monitoring as myeloablative conditioning in 47 pediatric patients undergoing first autologous hematopoietic transplantation from 2006 to 2010. Median age was 4 years (range, 1 to 22 y). There were 35 boys and 12 girls. Conditioning was Bu-based in all patients. In medulloblastoma patients (n=12), IV thiotepa at 5 mg/kg/d×2 days was associated with Bu. In solid tumors other than medulloblastoma, IV melphalan was used at 140 mg/m/d×1 day. In lymphoma patients (n=9), IV cyclophosphamide was used at 60 mg/kg/d×2 days. Median number of CD34 cells infused was 4.65×10/kg (range, 1.7 to 58.7×10/kg). Median times to neutrophil and platelet recovery (>20×10/L) were 11 days (range, 8 to 16 d) and 12 days (range, 7 to 135 d), respectively. Median hospitalization time was 14 days (range, 9 to 92 d). Sixteen patients developed grades III and IV mucositis. Only 1 patient developed mild sinusoidal obstruction syndrome. No patient developed seizures or severe neurological complications. With a median follow-up of 18 months, probability of transplantation-related mortality and disease-free survival were 0% and 70%±8%, respectively. In conclusion, in our experience, once-daily IV Bu-based conditioning without drug monitoring for autologous hematopoietic transplantation in pediatric patients is a safe and effective regimen with no mortality.