AIM:To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease(WD)patients.METHODS:We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent mu...AIM:To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease(WD)patients.METHODS:We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent multiple follow-up liver biopsies.Demographic,clinical and laboratory data were gathered and all patients underwent an initial biopsy and at least one repeat biopsy.RESULTS:Time to repeat biopsy ranged from 2 to 12 years.Six patients(non-progressors)showed stable hepatic histology or improvement.In one case,we observed improvement of fibrosis from stage 2 to 0.Six patients(progressors)had worsening of fibrosis.There was no significant correlation between the histological findings and serum aminotransferases or copper me-tabolism parameters.The hepatic copper concentration reached normal levels in only two patients:one from the non-progressors and one from the progressors group.The estimated rate of progression of hepatic fibrosis in the entire group was 0 units per year in the time frame between the first and the second liver biopsy(4 years),and 0.25 between the second and the third(3 years).In the progressors group,the rate of progression of liver fibrosis was estimated at 0.11 fibrosis units per year between the first and second biopsy and,0.6 fibrosis units between the second and third biopsy.CONCLUSION:The inability of clinical tools to detect fibrosis progression in WD suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered.展开更多
Biliary rhabdomyosarcoma (BRMS) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMS, in whom endoscopic retrograde cholangiopancreatography (ERCP) was...Biliary rhabdomyosarcoma (BRMS) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMS, in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used both diagnostically and therapeutically, thus obviating the need for surgery and its attendant risks of morbidity and mortality. We conclude that ERCP is an effective alternative to surgery for BRMS in some patients.展开更多
BACKGROUND Tillaux fractures occur primarily in adolescents due to the pattern of physeal closure and are classified as Salter-Harris type III physeal fractures.Operative management with screw fixation is recommended ...BACKGROUND Tillaux fractures occur primarily in adolescents due to the pattern of physeal closure and are classified as Salter-Harris type III physeal fractures.Operative management with screw fixation is recommended for more than 2 mm of displacement or more than 1 mm of translation.However,the efficacy and complications of trans-physeal vs all-physeal screw fixation have not been investigated extensively.AIM To compare the clinical and functional outcomes of trans-physeal(oblique)and all-epiphyseal(parallel)screw fixation in management of Tillaux fractures among pediatric patients.METHODS This was an ethics board approved retrospective review of pediatric patients who presented to our tertiary children’s care facility with Tillaux fractures.We included patients who had surgical fixation of a Tillaux fracture over a 10 year period.Data analysis included demographics,mode of injury,management protocols,and functional outcomes.The patients were divided into group 1(oblique fixation)and group 2(parallel fixation).Baseline patient characteristics and functional outcomes were compared between groups.Statistical tests to evaluate differences included Fisher’s Exact or Chi-squared and independent samples t or Mann Whitney tests for categorical and continuous variables,respectively.RESULTS A total of 42 patients(28 females and 14 males)were included.There were no significant differences in body mass index,sex,age,or time to surgery between the groups[IK2].Sports injuries accounted for 61.9%of the cases,particularly non-contact(57.1%)and skating(28.6%)injuries.Computed Tomography(CT)scan was ordered for 28 patients(66.7%),leading to diagnosis confirmation in 17 patients and change in management plan in 11 patients.[GRC3]Groups 1 and 2 consisted of 17 and 25 patients,respectively.For mid to long-term functional outcomes,there were 14 and 10 patients in groups 1 and 2,respectively.Statistical analysis revealed no significant differences in the functional outcomes,pain scores,or satisfaction between groups.No infections,non-unions,physeal arrest,or post-operative ankle deformities were reported.Two(4.8%)patients had difficulty returning to sports post-surgery due to pain.One was a dancer,and the other patient had pain while running,which led to hardware removal.Both patients had parallel fixation.Hardware removal for groups 1 and 2 were 4(23.5%)and 5(20.0%)patients,respectively.The reasons for removal was pain in 2 patients,and parental preference in the remaining.CONCLUSION This is the largest reported series of pediatric patients with Tillaux fractures comparing functional outcomes of different methods of screw fixation orientation to the physis,which showed no difference regarding functional outcomes.展开更多
Maternal nutrition is found to be the key factor that determines fetal health in utero and metabolic health during adulthood.Metabolic diseases have been primarily attributed to impaired maternal nutrition during preg...Maternal nutrition is found to be the key factor that determines fetal health in utero and metabolic health during adulthood.Metabolic diseases have been primarily attributed to impaired maternal nutrition during pregnancy,and impaired nutrition has been an immense issue across the globe.In recent years,type 2 diabetes(T2D)has reached epidemic proportion and is a severe public health problem in many countries.Although plenty of research has already been conducted to tackle T2D which is associated with obesity,little is known regarding the etiology and pathophysiology of lean T2D,a variant of T2D.Recent studies have focused on the effects of epigenetic variation on the contribution of in utero origins of lean T2D,although other mechanisms might also contribute to the pathology.Observational studies in humans and experiments in animals strongly suggest an association between maternal low protein diet and lean T2D phenotype.In addition,clear sex-specific disease prevalence was observed in different studies.Consequently,more research is essential for the understanding of the etiology and pathophysiology of lean T2D,which might help to develop better disease prevention and treatment strategies.This review examines the role of protein insufficiency in the maternal diet as the central driver of the developmental programming of lean T2D.展开更多
Background/Purpose: The aim of this study was to determine the presentation, treatment, and outcome of children requiring surgery for peptic ulcer disease (PUD) in the post-histamine2-blocker era. Methods: The charts ...Background/Purpose: The aim of this study was to determine the presentation, treatment, and outcome of children requiring surgery for peptic ulcer disease (PUD) in the post-histamine2-blocker era. Methods: The charts of all children undergoing surgery for PUD in our institution since 1980 were retrospectively reviewed. Data were collected regarding clinical presentation, operative details, postoperative course, and outcome. Results: Twenty-nine children (7.2 ±7.5 years) required surgery for complications of PUD. Indications for operation were bleeding (n = 11), pneumoperitoneum(n = 13), peritonitis (n = 3), and gastric outlet obstruction refractory to medical therapy (n = 2). For those children with bleeding, 8 had simple oversew of the bleeding ulcer(s), 2 had oversew with vagotomy and pyloroplasty, and 1 required vagotomy and antrectomy. All patients with perforation (n = 16) were treated with simple closure with or without omental patch. One child with gastric outlet obstruction underwent vagotomy and antrectomy and 1 had vagotomy and pyloroplasty. Preoperative risk factors or comorbidities were present in 27 of 29 patients and included steroid or nonsteroidal antiinflammatory drug medications in 13 children (only 3 of whom were receiving antiulcer prophylaxis). Postoperative complications occurred in 11 of 29 patients. Three (10%) children required reoperation for persistent or recurrent ulcer disease and 4 children died. Conclusions: PUD remains a highly morbid and mortal condition in children despite the availability of effective acid-reducing medications. Effective prophylaxis of children receiving steroids and nonsteroidal antiinflammatory drugs may play a role in decreasing the risk of PUD.展开更多
Background/Purpose:Because severe dysplasia and carcinoma in children with familial adenomatous polyposis (FAP) younger than 18 years is rare,earlier surgical intervention is not common. The purpose of this study is t...Background/Purpose:Because severe dysplasia and carcinoma in children with familial adenomatous polyposis (FAP) younger than 18 years is rare,earlier surgical intervention is not common. The purpose of this study is to report the prevalence of dysplasia and carcinoma among children with FAP in our institution. Methods:From 1998 through 2004,children 18 years or younger with FAP that underwent total proctocolectomy at a large children’s hospital were retrospectively reviewed. Results:Eleven children underwent surgery for FAP. The mean age at surgery was 13 ± 3.2 years. Approximately half of the patients who underwent preoperative endoscopy had evidence of dysplasia. Nine (82% ) patients had dysplasia on preoperative biopsy and/or operative specimen,and 3 (27% ) of these patients had severe dysplasia or carcinoma in situ. Four of the 5 patients without symptoms had evidence of dysplasia. No patients had invasive carcinoma. Conclusion:We observed a higher incidence of severe colonic dysplasia in young children with FAP compared with the current literature. A significant number of patients with dysplasia at surgery had no symptoms and no evidence of dysplasia on preoperative endoscopic biopsies. These data suggest that earlier intervention in children with FAP may be beneficial and should be investigated further.展开更多
Objective. More than 70%of the community-acquired (CA)-staphylococcal infections treated at Texas Children’s Hospital are caused by methicillin-resistant Staphylococcus aureus (MRSA). Since September 2002, an increas...Objective. More than 70%of the community-acquired (CA)-staphylococcal infections treated at Texas Children’s Hospital are caused by methicillin-resistant Staphylococcus aureus (MRSA). Since September 2002, an increase in the number of severely ill patients with S aureus infections has occurred. This study provides a clinical description of severely ill adolescent patients and an analysis of their isolates using molecular methods. Methods. We identified adolescent patients meeting criteria for severe sepsis requiring admission to the PICU. Patient records were reviewed, and isolates were obtained for susceptibility testing and DNA extraction. Isolates were tested for the presence of virulence genes (cna, tst, lukS-PV, and lukFPV) and enterotoxin genes (sea, seb, sec, sed, seh, and sej) by polymerase chain reaction. Genomic fingerprints were determined by repetitive-element polymorphism polymerase chain reaction and pulse-field gel electrophoresis. SCCmec cassette type was determined. Results. Fourteen adolescents with severe CA S aureus infections were identified between August 2002 and January 2004. All were admitted to the PICU with sepsis and coagulopathy. Twelve patients had CA-MRSA infections; 2 had CA methicillin-susceptible Staphylococcus aureus (MSSA) infections. The mean age was 12.9 years (range: 10-15 years). Thirteen patients had pulmonary involvement and/or bone and joint infection; 10 patients had ≥2 bones or joints infected (range: 2-10); 4 patients developed vascular complications (deep venous thrombosis);and 3 patients died. All isolates were identical or closely related to the previously reported predominant clone in Houston, Texas (multilocus sequence type 8, USA300), and carried lukS-PV and lukF-PV genes as well as the SCCmec type IVa cassette (12 MRSA isolates) but did not contain cna or tst. Only 1 strain carried enterotoxin genes (sed and sej). Conclusions. Severe staphylococcal infections in previously healthy adolescents without predisposing risk factors have presented more frequently at Texas Children’s Hospital since September 2002. CA MRSA and clonally related CA MSSA characterized as USA300 and sequence type 8 have been isolated from these patients.展开更多
Background/Purpose: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several case...Background/Purpose: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome. Methods: We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome. Results: Ten children (6 boys and 4 girls) were treated for PPB a t a mean age of 3.2 ±4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow- up (mean, 7.7 ±11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease. Conclusions: PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.展开更多
Purpose: To evaluate the long-term visual acuity (VA) and refractive error responses to excimer laser photorefractive keratectomy (PRK) for treatment of anisometropic amblyopia in children. Design: Prospective interve...Purpose: To evaluate the long-term visual acuity (VA) and refractive error responses to excimer laser photorefractive keratectomy (PRK) for treatment of anisometropic amblyopia in children. Design: Prospective interventional case-control study. Participants: Eleven children,2 to 11 years old,with anisometropic amblyopia who were noncompliant with conventional therapy with glasses or contact lenses and occlusion therapy were treated with PRK. A cohort derived retrospectively of 13 compliant and 10 noncompliant children with refractive errors similar to those of the PRK group who were treated with traditional anisometropic amblyopia therapy served as control groups. Intervention: Photorefractive keratectomy for the eye with the higher refractive error. Main Outcome Measures: (1) Refractive error reduction and stability in the treated eye,(2) cycloplegic refraction,(3) VA,(4) stereoacuity,and (5) corneal haze up to 3 years after PRK. Compliant and noncompliant children with anisometropia amblyopiawere analyzed as controls for refractive error and VA. Results: Preoperative refractive errors were-13.70 diopters (D) (± 3.77) for themyopic group and + 4.75 D (± 0.50) for the hyperopic group. Mean postoperative refractive errors at last follow-up (mean,31 months) were-3.55 D (± 2.25) and + 1.41 D (± 1.07) for the myopic and hyperopic groups,respectively. At last follow-up,cycloplegic refractions in 4 (50% ) of 8 myopes and all hyperopes (100% ) were within 3 D of that of the fellow eye. Five (63% ) of 8 myopic children achieved a refraction within 2 D of the target refraction. Two (67% ) of 3 hyperopic patients maintained their refractions within 2 D of the target. Refractive regressions (from 1 year after surgery to last followup) were 0.50± 1.41D (myopes) and 0.60± 0.57D (hyperopes). Seven children (77% ) were able to perform psychophysicalVA testing preoperatively and postoperatively. Five (71% ) of the 7 children had uncorrected VA improvement of at least 2 lines,and 4 (57% ) of 7 had best spectacle-corrected VA improvement of at least 2 lines,with 1 improving 7 lines. Five (55% ) of 9 children had improvement of their stereoacuity at last followup. Subepithelial corneal haze remained negligible. The mean final VA of the PRK group was significantly better than that of the noncompliant control group (P=0.003). The mean final refractive error for both myopic and hyperopic groups was also significantly better that that of the control groups (P=0.007 and P<0.0001,respectively). Conclusions: Photorefractive keratectomy for severe anisometropic amblyopia in children resulted in long-term stable reduction in refractive error and improvement in VA and stereopsis,with negligible persistent corneal haze.展开更多
Objective: To review clinical, laboratory, and outcome characteristics of chi ldren diagnosed with pulmonary capillaritis (PC), a small- vessel vasculitis, p resenting as diffuse alveolar hemorrhage (DAH), and to comp...Objective: To review clinical, laboratory, and outcome characteristics of chi ldren diagnosed with pulmonary capillaritis (PC), a small- vessel vasculitis, p resenting as diffuse alveolar hemorrhage (DAH), and to compare these findings wi th those for children with other alveolar hemorrhage syndromes. Study design: A retrospective chart review of patients who underwent a lung biopsy because of a clinical suggestion of pulmonary hemorrhage. Results: PC was identified in 8 of 23 patients. In these patients, cough, crackles, and hypoxia were common. Alveol ar infiltrates on radiography and anemia were present in 7 of 8 cases. Serologic evidence of a systemic vasculitis was present in 50% of patients. High- dose corticosteroids proved effective in controlling alveolar hemorrhage in all cases . There were no presenting signs or symptoms that could differentiate patients w ith PC from those with non- immunemediated alveolar hemorrhage. In general, pat ients with PC had a lower hematocrit and higher erythrocyte sedimentation rate ( ESR). Conclusion: Children presenting with lower respiratory tract symptoms, che st x- ray abnormalities, and anemia should undergo evaluation for PC, as early initiation of immunosuppression can be lifesaving and organ sparing. No clinical signs to differentiate immune and non- immune- mediated alveolar hemorrhage w ere evident in this study.展开更多
Although the incidence of caustic ingestion is declining, the management of caustic esophageal strictures remains a challenge. Mitomycin C (MMC) inhibits fibroblast proliferation and is effective in reducing scar in a...Although the incidence of caustic ingestion is declining, the management of caustic esophageal strictures remains a challenge. Mitomycin C (MMC) inhibits fibroblast proliferation and is effective in reducing scar in animal experiments. We report the case of a child with a distal esophageal stricture from lye ingestion managed with MMC. Despite repeated dilatations, at 1 year post injury, the stricture was 20% of esophageal diameter. Mitomycin C (4 μ g/mL)was applied topically and circumferentially by endoscopy and repeated 4 months later. At 20 months follow- up, the child eats normally, and esophagram showed decreased stenosis (stricture was 50% of esophageal diameter). No complications were observed. Although controlled trials are required to confirm its efficacy, MMC should be considered as an adjunct in the management of caustic esophageal strictures in children.展开更多
Objective To compare wound complication rates between orthopedic closure(OC)and plastic multilayered closure(PMC)in patients undergoing primary posterior spinal fusion for neuromuscular scoliosis(NMS).We hypothesize t...Objective To compare wound complication rates between orthopedic closure(OC)and plastic multilayered closure(PMC)in patients undergoing primary posterior spinal fusion for neuromuscular scoliosis(NMS).We hypothesize that multilayered closure will be associated with better postoperative outcomes.Methods We collected data on pediatric patients diagnosed with NMS who underwent first time spinal instrumentation between 1 January 2018 and 31 May 2021.Patient demographics,length of surgery,spinal levels fused and operative variables,wound complication rate,treatments,and need for wound washout were reviewed in depth and recorded.Results In total,86 patients were reviewed:46 with OC and 40 with PMC.There was a significant increase in operating room(OR)time with PMC compared with OC(6.7±1.2 vs 7.3±1.3,p=0.016).There was no difference in complication rate,mean postoperative day of complication or unplanned return to the OR for OC and PMC,respectively.There was a slightly significant increase in the number of patients going home with a drain in the PMC cohort compared with the OC cohort(2.1%vs 15%,p=0.046).Conclusions PMC demonstrated longer OR times than OC and did not demonstrate a statistically significant reduction in wound complications or unplanned returns to the OR.However,other studies have demonstrated statistical and clinical significance with these variables.Surgical programs should review internal patient volumes and outcomes for spinal fusion in NMS patients and consider if PMC after spinal fusions in pediatric patients with NMS or other scoliosis subtypes is an appropriate option in their institution to minimize postoperative wound complications.展开更多
Langerhans cell histiocytosis(LCH)is a rare disease most commonly presenting in the pediatric population and characterized by neoplastic clonal proliferation of Langerhans dendritic cells with accumulation in various ...Langerhans cell histiocytosis(LCH)is a rare disease most commonly presenting in the pediatric population and characterized by neoplastic clonal proliferation of Langerhans dendritic cells with accumulation in various sites,including skeletal and visceral lesions.1 There are three levels of classification per the Histiocyte Society:single-system single-site(SS-s),single-system multiple-site(SS-m),and multisystem(MS).2 SS-s predominantly carries a better prognosis with more conservative treatment while MS requires a more aggressive treatment that is more likely to have an inferior outcome.展开更多
Objectives:To examine the volume,topics,and reporting trends in the published literature of randomized clinical trials for pharmacologic pain management of pediatric tonsillectomy and adenotonsillectomy and to identif...Objectives:To examine the volume,topics,and reporting trends in the published literature of randomized clinical trials for pharmacologic pain management of pediatric tonsillectomy and adenotonsillectomy and to identify areas requiring further research.Data Sources:PubMed(National Library of Medicine and National Institutes of Health),Scopus(Elsevier),CINAHL(EBSCO),and Cochrane Library(Wiley).Methods:A systematic search of four databases was conducted.Only randomized controlled or comparison trials examining pain improvement with a pharmacologic intervention in pediatric tonsillectomy or adenotonsillectomy were included.Data collected included demographics,pain-related outcomes,sedation scores,nausea/vomiting,postoperative bleeding,types of drug comparisons,modes of administration,timing of administration,and identities of the investigated drugs.Results:One hundred and eighty-nine studies were included for analysis.Most studies included validated pain scales,with the majority using visual-assisted scales(49.21%).Fewer studies examined pain beyond 24 h postoperation(24.87%),and few studies included a validated sedation scale(12.17%).Studies have compared several different dimensions of pharmacologic treatment,including different drugs,timing of administration,modes of administration,and dosages.Only 23(12.17%)studies examined medications administered postoperatively,and only 29(15.34%)studies examined oral medications.Acetaminophen only had four self-comparisons.Conclusion:Our work provides the first scoping review of pain and pediatric tonsillectomy.With drug safety profiles considered,the literature does not have enough data to determine which treatment regimen provides superior pain control in pediatric tonsillectomy.Even common drugs like acetaminophen and ibuprofen require further research for optimizing the treatment of posttonsillectomy pain.The heterogeneity in study design and comparisons weakens the conclusions of potential systematic reviews and meta-analyses.Future directions include more noninferiority studies of unique comparisons and more studies examining oral medications given postoperatively.展开更多
Before the founding of the People's Republic of China 70 years ago,both extreme poverty and parasitic infections and other neglected tropical diseases were highly prevalent.Owing to social development,particularly...Before the founding of the People's Republic of China 70 years ago,both extreme poverty and parasitic infections and other neglected tropical diseases were highly prevalent.Owing to social development,particularly economic reforms since the 1980s,poverty has since been dramatically reduced,and China became increasingly urbanized and industrialized.In parallel,China's economic transformation translated into similar and remarkable reductions in neglected tropical diseases.Qian and colleagues report in their review published in Infectious Diseases of Poverty,the elimination or near elimination as a public health problem of lymphatic filariasis,trachoma,soil-transmitted helminth infections,schistosomiasis and other neglected tropical diseases.Of note,neglected tropical disease control and poverty reduction each appear to reinforce the other.China's formula for success in parasitic and neglected tropical disease control might translate to other parts of the world,such as in sub-Saharan Africa through China's new Belt and Road Initiative.展开更多
文摘AIM:To investigate the progression of hepatic histopathology in serial liver biopsies from Wilson disease(WD)patients.METHODS:We report a group of 12 WD patients treated with zinc and/or penicillamine who underwent multiple follow-up liver biopsies.Demographic,clinical and laboratory data were gathered and all patients underwent an initial biopsy and at least one repeat biopsy.RESULTS:Time to repeat biopsy ranged from 2 to 12 years.Six patients(non-progressors)showed stable hepatic histology or improvement.In one case,we observed improvement of fibrosis from stage 2 to 0.Six patients(progressors)had worsening of fibrosis.There was no significant correlation between the histological findings and serum aminotransferases or copper me-tabolism parameters.The hepatic copper concentration reached normal levels in only two patients:one from the non-progressors and one from the progressors group.The estimated rate of progression of hepatic fibrosis in the entire group was 0 units per year in the time frame between the first and the second liver biopsy(4 years),and 0.25 between the second and the third(3 years).In the progressors group,the rate of progression of liver fibrosis was estimated at 0.11 fibrosis units per year between the first and second biopsy and,0.6 fibrosis units between the second and third biopsy.CONCLUSION:The inability of clinical tools to detect fibrosis progression in WD suggests that a liver biopsy with hepatic copper quantification every 3 years should be considered.
文摘Biliary rhabdomyosarcoma (BRMS) is an uncommon childhood malignancy which has been managed surgically. We present a case of a 3-year-old boy with BRMS, in whom endoscopic retrograde cholangiopancreatography (ERCP) was successfully used both diagnostically and therapeutically, thus obviating the need for surgery and its attendant risks of morbidity and mortality. We conclude that ERCP is an effective alternative to surgery for BRMS in some patients.
文摘BACKGROUND Tillaux fractures occur primarily in adolescents due to the pattern of physeal closure and are classified as Salter-Harris type III physeal fractures.Operative management with screw fixation is recommended for more than 2 mm of displacement or more than 1 mm of translation.However,the efficacy and complications of trans-physeal vs all-physeal screw fixation have not been investigated extensively.AIM To compare the clinical and functional outcomes of trans-physeal(oblique)and all-epiphyseal(parallel)screw fixation in management of Tillaux fractures among pediatric patients.METHODS This was an ethics board approved retrospective review of pediatric patients who presented to our tertiary children’s care facility with Tillaux fractures.We included patients who had surgical fixation of a Tillaux fracture over a 10 year period.Data analysis included demographics,mode of injury,management protocols,and functional outcomes.The patients were divided into group 1(oblique fixation)and group 2(parallel fixation).Baseline patient characteristics and functional outcomes were compared between groups.Statistical tests to evaluate differences included Fisher’s Exact or Chi-squared and independent samples t or Mann Whitney tests for categorical and continuous variables,respectively.RESULTS A total of 42 patients(28 females and 14 males)were included.There were no significant differences in body mass index,sex,age,or time to surgery between the groups[IK2].Sports injuries accounted for 61.9%of the cases,particularly non-contact(57.1%)and skating(28.6%)injuries.Computed Tomography(CT)scan was ordered for 28 patients(66.7%),leading to diagnosis confirmation in 17 patients and change in management plan in 11 patients.[GRC3]Groups 1 and 2 consisted of 17 and 25 patients,respectively.For mid to long-term functional outcomes,there were 14 and 10 patients in groups 1 and 2,respectively.Statistical analysis revealed no significant differences in the functional outcomes,pain scores,or satisfaction between groups.No infections,non-unions,physeal arrest,or post-operative ankle deformities were reported.Two(4.8%)patients had difficulty returning to sports post-surgery due to pain.One was a dancer,and the other patient had pain while running,which led to hardware removal.Both patients had parallel fixation.Hardware removal for groups 1 and 2 were 4(23.5%)and 5(20.0%)patients,respectively.The reasons for removal was pain in 2 patients,and parental preference in the remaining.CONCLUSION This is the largest reported series of pediatric patients with Tillaux fractures comparing functional outcomes of different methods of screw fixation orientation to the physis,which showed no difference regarding functional outcomes.
基金Supported by the National Institutes of Health Grants,No. HL102866, HL58144 and DK114689
文摘Maternal nutrition is found to be the key factor that determines fetal health in utero and metabolic health during adulthood.Metabolic diseases have been primarily attributed to impaired maternal nutrition during pregnancy,and impaired nutrition has been an immense issue across the globe.In recent years,type 2 diabetes(T2D)has reached epidemic proportion and is a severe public health problem in many countries.Although plenty of research has already been conducted to tackle T2D which is associated with obesity,little is known regarding the etiology and pathophysiology of lean T2D,a variant of T2D.Recent studies have focused on the effects of epigenetic variation on the contribution of in utero origins of lean T2D,although other mechanisms might also contribute to the pathology.Observational studies in humans and experiments in animals strongly suggest an association between maternal low protein diet and lean T2D phenotype.In addition,clear sex-specific disease prevalence was observed in different studies.Consequently,more research is essential for the understanding of the etiology and pathophysiology of lean T2D,which might help to develop better disease prevention and treatment strategies.This review examines the role of protein insufficiency in the maternal diet as the central driver of the developmental programming of lean T2D.
文摘Background/Purpose: The aim of this study was to determine the presentation, treatment, and outcome of children requiring surgery for peptic ulcer disease (PUD) in the post-histamine2-blocker era. Methods: The charts of all children undergoing surgery for PUD in our institution since 1980 were retrospectively reviewed. Data were collected regarding clinical presentation, operative details, postoperative course, and outcome. Results: Twenty-nine children (7.2 ±7.5 years) required surgery for complications of PUD. Indications for operation were bleeding (n = 11), pneumoperitoneum(n = 13), peritonitis (n = 3), and gastric outlet obstruction refractory to medical therapy (n = 2). For those children with bleeding, 8 had simple oversew of the bleeding ulcer(s), 2 had oversew with vagotomy and pyloroplasty, and 1 required vagotomy and antrectomy. All patients with perforation (n = 16) were treated with simple closure with or without omental patch. One child with gastric outlet obstruction underwent vagotomy and antrectomy and 1 had vagotomy and pyloroplasty. Preoperative risk factors or comorbidities were present in 27 of 29 patients and included steroid or nonsteroidal antiinflammatory drug medications in 13 children (only 3 of whom were receiving antiulcer prophylaxis). Postoperative complications occurred in 11 of 29 patients. Three (10%) children required reoperation for persistent or recurrent ulcer disease and 4 children died. Conclusions: PUD remains a highly morbid and mortal condition in children despite the availability of effective acid-reducing medications. Effective prophylaxis of children receiving steroids and nonsteroidal antiinflammatory drugs may play a role in decreasing the risk of PUD.
文摘Background/Purpose:Because severe dysplasia and carcinoma in children with familial adenomatous polyposis (FAP) younger than 18 years is rare,earlier surgical intervention is not common. The purpose of this study is to report the prevalence of dysplasia and carcinoma among children with FAP in our institution. Methods:From 1998 through 2004,children 18 years or younger with FAP that underwent total proctocolectomy at a large children’s hospital were retrospectively reviewed. Results:Eleven children underwent surgery for FAP. The mean age at surgery was 13 ± 3.2 years. Approximately half of the patients who underwent preoperative endoscopy had evidence of dysplasia. Nine (82% ) patients had dysplasia on preoperative biopsy and/or operative specimen,and 3 (27% ) of these patients had severe dysplasia or carcinoma in situ. Four of the 5 patients without symptoms had evidence of dysplasia. No patients had invasive carcinoma. Conclusion:We observed a higher incidence of severe colonic dysplasia in young children with FAP compared with the current literature. A significant number of patients with dysplasia at surgery had no symptoms and no evidence of dysplasia on preoperative endoscopic biopsies. These data suggest that earlier intervention in children with FAP may be beneficial and should be investigated further.
文摘Objective. More than 70%of the community-acquired (CA)-staphylococcal infections treated at Texas Children’s Hospital are caused by methicillin-resistant Staphylococcus aureus (MRSA). Since September 2002, an increase in the number of severely ill patients with S aureus infections has occurred. This study provides a clinical description of severely ill adolescent patients and an analysis of their isolates using molecular methods. Methods. We identified adolescent patients meeting criteria for severe sepsis requiring admission to the PICU. Patient records were reviewed, and isolates were obtained for susceptibility testing and DNA extraction. Isolates were tested for the presence of virulence genes (cna, tst, lukS-PV, and lukFPV) and enterotoxin genes (sea, seb, sec, sed, seh, and sej) by polymerase chain reaction. Genomic fingerprints were determined by repetitive-element polymorphism polymerase chain reaction and pulse-field gel electrophoresis. SCCmec cassette type was determined. Results. Fourteen adolescents with severe CA S aureus infections were identified between August 2002 and January 2004. All were admitted to the PICU with sepsis and coagulopathy. Twelve patients had CA-MRSA infections; 2 had CA methicillin-susceptible Staphylococcus aureus (MSSA) infections. The mean age was 12.9 years (range: 10-15 years). Thirteen patients had pulmonary involvement and/or bone and joint infection; 10 patients had ≥2 bones or joints infected (range: 2-10); 4 patients developed vascular complications (deep venous thrombosis);and 3 patients died. All isolates were identical or closely related to the previously reported predominant clone in Houston, Texas (multilocus sequence type 8, USA300), and carried lukS-PV and lukF-PV genes as well as the SCCmec type IVa cassette (12 MRSA isolates) but did not contain cna or tst. Only 1 strain carried enterotoxin genes (sed and sej). Conclusions. Severe staphylococcal infections in previously healthy adolescents without predisposing risk factors have presented more frequently at Texas Children’s Hospital since September 2002. CA MRSA and clonally related CA MSSA characterized as USA300 and sequence type 8 have been isolated from these patients.
文摘Background/Purpose: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome. Methods: We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome. Results: Ten children (6 boys and 4 girls) were treated for PPB a t a mean age of 3.2 ±4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow- up (mean, 7.7 ±11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease. Conclusions: PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.
文摘Purpose: To evaluate the long-term visual acuity (VA) and refractive error responses to excimer laser photorefractive keratectomy (PRK) for treatment of anisometropic amblyopia in children. Design: Prospective interventional case-control study. Participants: Eleven children,2 to 11 years old,with anisometropic amblyopia who were noncompliant with conventional therapy with glasses or contact lenses and occlusion therapy were treated with PRK. A cohort derived retrospectively of 13 compliant and 10 noncompliant children with refractive errors similar to those of the PRK group who were treated with traditional anisometropic amblyopia therapy served as control groups. Intervention: Photorefractive keratectomy for the eye with the higher refractive error. Main Outcome Measures: (1) Refractive error reduction and stability in the treated eye,(2) cycloplegic refraction,(3) VA,(4) stereoacuity,and (5) corneal haze up to 3 years after PRK. Compliant and noncompliant children with anisometropia amblyopiawere analyzed as controls for refractive error and VA. Results: Preoperative refractive errors were-13.70 diopters (D) (± 3.77) for themyopic group and + 4.75 D (± 0.50) for the hyperopic group. Mean postoperative refractive errors at last follow-up (mean,31 months) were-3.55 D (± 2.25) and + 1.41 D (± 1.07) for the myopic and hyperopic groups,respectively. At last follow-up,cycloplegic refractions in 4 (50% ) of 8 myopes and all hyperopes (100% ) were within 3 D of that of the fellow eye. Five (63% ) of 8 myopic children achieved a refraction within 2 D of the target refraction. Two (67% ) of 3 hyperopic patients maintained their refractions within 2 D of the target. Refractive regressions (from 1 year after surgery to last followup) were 0.50± 1.41D (myopes) and 0.60± 0.57D (hyperopes). Seven children (77% ) were able to perform psychophysicalVA testing preoperatively and postoperatively. Five (71% ) of the 7 children had uncorrected VA improvement of at least 2 lines,and 4 (57% ) of 7 had best spectacle-corrected VA improvement of at least 2 lines,with 1 improving 7 lines. Five (55% ) of 9 children had improvement of their stereoacuity at last followup. Subepithelial corneal haze remained negligible. The mean final VA of the PRK group was significantly better than that of the noncompliant control group (P=0.003). The mean final refractive error for both myopic and hyperopic groups was also significantly better that that of the control groups (P=0.007 and P<0.0001,respectively). Conclusions: Photorefractive keratectomy for severe anisometropic amblyopia in children resulted in long-term stable reduction in refractive error and improvement in VA and stereopsis,with negligible persistent corneal haze.
文摘Objective: To review clinical, laboratory, and outcome characteristics of chi ldren diagnosed with pulmonary capillaritis (PC), a small- vessel vasculitis, p resenting as diffuse alveolar hemorrhage (DAH), and to compare these findings wi th those for children with other alveolar hemorrhage syndromes. Study design: A retrospective chart review of patients who underwent a lung biopsy because of a clinical suggestion of pulmonary hemorrhage. Results: PC was identified in 8 of 23 patients. In these patients, cough, crackles, and hypoxia were common. Alveol ar infiltrates on radiography and anemia were present in 7 of 8 cases. Serologic evidence of a systemic vasculitis was present in 50% of patients. High- dose corticosteroids proved effective in controlling alveolar hemorrhage in all cases . There were no presenting signs or symptoms that could differentiate patients w ith PC from those with non- immunemediated alveolar hemorrhage. In general, pat ients with PC had a lower hematocrit and higher erythrocyte sedimentation rate ( ESR). Conclusion: Children presenting with lower respiratory tract symptoms, che st x- ray abnormalities, and anemia should undergo evaluation for PC, as early initiation of immunosuppression can be lifesaving and organ sparing. No clinical signs to differentiate immune and non- immune- mediated alveolar hemorrhage w ere evident in this study.
文摘Although the incidence of caustic ingestion is declining, the management of caustic esophageal strictures remains a challenge. Mitomycin C (MMC) inhibits fibroblast proliferation and is effective in reducing scar in animal experiments. We report the case of a child with a distal esophageal stricture from lye ingestion managed with MMC. Despite repeated dilatations, at 1 year post injury, the stricture was 20% of esophageal diameter. Mitomycin C (4 μ g/mL)was applied topically and circumferentially by endoscopy and repeated 4 months later. At 20 months follow- up, the child eats normally, and esophagram showed decreased stenosis (stricture was 50% of esophageal diameter). No complications were observed. Although controlled trials are required to confirm its efficacy, MMC should be considered as an adjunct in the management of caustic esophageal strictures in children.
文摘Objective To compare wound complication rates between orthopedic closure(OC)and plastic multilayered closure(PMC)in patients undergoing primary posterior spinal fusion for neuromuscular scoliosis(NMS).We hypothesize that multilayered closure will be associated with better postoperative outcomes.Methods We collected data on pediatric patients diagnosed with NMS who underwent first time spinal instrumentation between 1 January 2018 and 31 May 2021.Patient demographics,length of surgery,spinal levels fused and operative variables,wound complication rate,treatments,and need for wound washout were reviewed in depth and recorded.Results In total,86 patients were reviewed:46 with OC and 40 with PMC.There was a significant increase in operating room(OR)time with PMC compared with OC(6.7±1.2 vs 7.3±1.3,p=0.016).There was no difference in complication rate,mean postoperative day of complication or unplanned return to the OR for OC and PMC,respectively.There was a slightly significant increase in the number of patients going home with a drain in the PMC cohort compared with the OC cohort(2.1%vs 15%,p=0.046).Conclusions PMC demonstrated longer OR times than OC and did not demonstrate a statistically significant reduction in wound complications or unplanned returns to the OR.However,other studies have demonstrated statistical and clinical significance with these variables.Surgical programs should review internal patient volumes and outcomes for spinal fusion in NMS patients and consider if PMC after spinal fusions in pediatric patients with NMS or other scoliosis subtypes is an appropriate option in their institution to minimize postoperative wound complications.
文摘Langerhans cell histiocytosis(LCH)is a rare disease most commonly presenting in the pediatric population and characterized by neoplastic clonal proliferation of Langerhans dendritic cells with accumulation in various sites,including skeletal and visceral lesions.1 There are three levels of classification per the Histiocyte Society:single-system single-site(SS-s),single-system multiple-site(SS-m),and multisystem(MS).2 SS-s predominantly carries a better prognosis with more conservative treatment while MS requires a more aggressive treatment that is more likely to have an inferior outcome.
文摘Objectives:To examine the volume,topics,and reporting trends in the published literature of randomized clinical trials for pharmacologic pain management of pediatric tonsillectomy and adenotonsillectomy and to identify areas requiring further research.Data Sources:PubMed(National Library of Medicine and National Institutes of Health),Scopus(Elsevier),CINAHL(EBSCO),and Cochrane Library(Wiley).Methods:A systematic search of four databases was conducted.Only randomized controlled or comparison trials examining pain improvement with a pharmacologic intervention in pediatric tonsillectomy or adenotonsillectomy were included.Data collected included demographics,pain-related outcomes,sedation scores,nausea/vomiting,postoperative bleeding,types of drug comparisons,modes of administration,timing of administration,and identities of the investigated drugs.Results:One hundred and eighty-nine studies were included for analysis.Most studies included validated pain scales,with the majority using visual-assisted scales(49.21%).Fewer studies examined pain beyond 24 h postoperation(24.87%),and few studies included a validated sedation scale(12.17%).Studies have compared several different dimensions of pharmacologic treatment,including different drugs,timing of administration,modes of administration,and dosages.Only 23(12.17%)studies examined medications administered postoperatively,and only 29(15.34%)studies examined oral medications.Acetaminophen only had four self-comparisons.Conclusion:Our work provides the first scoping review of pain and pediatric tonsillectomy.With drug safety profiles considered,the literature does not have enough data to determine which treatment regimen provides superior pain control in pediatric tonsillectomy.Even common drugs like acetaminophen and ibuprofen require further research for optimizing the treatment of posttonsillectomy pain.The heterogeneity in study design and comparisons weakens the conclusions of potential systematic reviews and meta-analyses.Future directions include more noninferiority studies of unique comparisons and more studies examining oral medications given postoperatively.
文摘Before the founding of the People's Republic of China 70 years ago,both extreme poverty and parasitic infections and other neglected tropical diseases were highly prevalent.Owing to social development,particularly economic reforms since the 1980s,poverty has since been dramatically reduced,and China became increasingly urbanized and industrialized.In parallel,China's economic transformation translated into similar and remarkable reductions in neglected tropical diseases.Qian and colleagues report in their review published in Infectious Diseases of Poverty,the elimination or near elimination as a public health problem of lymphatic filariasis,trachoma,soil-transmitted helminth infections,schistosomiasis and other neglected tropical diseases.Of note,neglected tropical disease control and poverty reduction each appear to reinforce the other.China's formula for success in parasitic and neglected tropical disease control might translate to other parts of the world,such as in sub-Saharan Africa through China's new Belt and Road Initiative.
