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BACKGROUND: Our study aimed to compare the clinical outcomes and cost-efficiency of antibiotic management versus laparoscopic appendectomy for acute uncomplicated appendicitis (AUA) in children during the COVID-19 pandemic when resources were limited and transmission risks uncertain. METHOD: In this prospective comparative cohort study, we analyzed the data of 139 children diagnosed with AUA meeting the following inclusion criteria: 5-18 years of age, symptoms duration of ≤ 48 h, appendix diameter ≤ 11 mm and no appendicolith. Treatment outcomes between non-operative management group (78/139) and upfront laparoscopic appendectomy group (61/139) were compared. Antibiotic regimes were intravenous ceftriaxone/metronidazole or amoxicillin/clavulanic acid for 48 h, followed by oral antibiotics to complete total 10-days course. RESULTS: 8/78 (10.3%) children had early failure (within 48 h) requiring appendectomy. 17/70 (24.3%) patients experienced late recurrence within mean follow-up time of 16.2 ± 4.7 months. There were no statistical differences in peri-operative complications, negative appendicectomy rate, and incidence of perforation and hospitalization duration between antibiotic and surgical treatment groups. Cost per patient in upfront surgical group was significantly higher ($6208.5 ± 5284.0) than antibiotic group ($3588.6 ± 3829.8; p = 0.001). CONCLUSION: Despite 24.3% risk of recurrence of appendicitis in 16.2 ± 4.7 months, antibiotic therapy for AUA appears to be a safe and cost-effective alternative to upfront appendectomy.
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Apendicitis , COVID-19 , Humanos , Niño , Antibacterianos/uso terapéutico , Apendicitis/tratamiento farmacológico , Apendicitis/cirugía , Apendicitis/diagnóstico , Estudios de Cohortes , Estudios Prospectivos , Pandemias , Resultado del Tratamiento , Apendicectomía , Enfermedad Aguda , Tratamiento ConservadorRESUMEN
We report a case of junctional epidermolysis bullosa with pyloric atresia (JEB-PA) with minimal skin involvement but severe protein-losing enteropathy and airway involvement. Genetic analysis revealed heterozygous mutations in the ITGB4 gene encoding integrin ß4 protein. Parental testing confirmed inheritance of frameshift variant (c.794dupC) as maternal and splice site variant (c.1608C>T/p.Cys536Cys) as paternal. Immunofluorescence mapping of her skin revealed a subepidermal blister with decreased and frayed integrin ß4 at both the floor and the roof of the blister, while the intestinal mucosa showed complete absence of integrin ß4. We review the literature and discuss the differential expression of integrins in the skin and gastrointestinal tract, as well as the role of chronic inflammation in the pathogenesis of EB.
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Displasia Ectodérmica , Epidermólisis Ampollosa , Epidermólisis Ampollosa/diagnóstico , Epidermólisis Ampollosa/genética , Femenino , Humanos , Integrina beta4/genética , Mutación , PíloroRESUMEN
PURPOSE: Assays of salivary biomarkers for diagnosis are gaining popularity in pediatric diseases due to their non-invasive nature. Our pilot project aims to evaluate the utility of salivary leucine-rich-alpha-2-glycoprotein (LRG) in the diagnosis of pediatric acute appendicitis (AA). METHODS: We prospectively recruited 34 patients, aged between 4 and 16 years, admitted with acute abdominal pain suspicious of appendicitis. The patients' demography, clinical characteristics, laboratory investigations, imaging examination results, operative findings, and discharge diagnoses were recorded. We compared the diagnostic performance of the patients' total white counts, neutrophil percentages, C-reactive protein, and saliva LRG levels. Saliva samples were obtained using the SalivaBio Children's Swab and LRG levels were quantified using a commercially available LRG enzyme-linked immunosorbent assay (ELISA) kit. IRB approval was obtained. RESULTS: Seventeen patients had a confirmed diagnosis of appendicitis on histology. Another 17 were confirmed not to have appendicitis after a minimum of 24 h of hospitalization, with further verification via telephone interview 2 weeks later. The median levels of saliva LRG were elevated in patients with AA as compared to those without (P = 0.008). At a cutoff of LRG 0.33 ng/µg, we obtained a diagnostic specificity of 100% and sensitivity of 35.3%. CONCLUSION: Our proof-of-concept study demonstrated the diagnostic potential of saliva LRG for appendicitis in children. The distinct advantage of saliva LRG assays is that the procedure is simple, pain-free, and requires no specialized skill. Further study with a larger cohort is needed to verify our results.
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Apendicitis/metabolismo , Saliva/metabolismo , Enfermedad Aguda , Adolescente , Apendicitis/diagnóstico , Biomarcadores/metabolismo , Proteína C-Reactiva/metabolismo , Estudios de Casos y Controles , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Masculino , Proyectos Piloto , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
PURPOSE: We aim to evaluate the diagnostic value and time course response of the triple inflammatory markers: white blood cell count (WBC), neutrophil percentage (Neu), and C-reactive protein (CRP) in pediatric acute appendicitis. METHODS: A retrospective review of clinical data pertaining to 1391 patients admitted with suspicion for pediatric appendicitis from 2012 to 2017 was conducted. Triple inflammatory markers were acquired upon admission. Appendicitis was confirmed histologically post-appendectomy. The diagnostic value and time course response of these markers was trended in relation to the duration of abdominal pain on admission. RESULTS: 718 patients had histologically confirmed appendicitis. WBC and Neu demonstrate high sensitivity for early appendicitis at 94.6% and 80.0% at Day 1, while CRP demonstrates highest sensitivity of 97.9% at Day 4. The triple markers had poor overall diagnostic value when interpreted individually, however, had a high combined sensitivity of 99.7% and negative predictive value of 98.7% regardless of duration of disease. Overall negative appendectomy rate was 6.7% (n = 52). Among 19 patients with triple markers negative who underwent appendectomy, 17 (89.5%) were histologically normal. CONCLUSIONS: The triple inflammatory markers have limited diagnostic value when interpreted individually, but are strong discriminators of pediatric appendicitis when combined. Their high sensitivity and negative predictive value could potentially help patients avoid unnecessary admissions or costly imaging studies, and reduce negative appendectomy rates. In addition, their objective nature confers an advantage over existing clinical scoring systems which comprise subjective elements.
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Apendicectomía/métodos , Apendicitis/sangre , Proteína C-Reactiva/metabolismo , Hospitalización/tendencias , Inflamación/sangre , Enfermedad Aguda , Apendicitis/cirugía , Biomarcadores/sangre , Niño , Femenino , Estudios de Seguimiento , Humanos , Recuento de Leucocitos , Masculino , Estudios Retrospectivos , Factores de TiempoRESUMEN
AIM: Studies report that most boys with undescended testis(UDT) are referred and operated beyond the recommended age of 1 year, possibly due to lack of awareness of treatment guidelines. We investigate the level of knowledge of UDT among potential referring health-care providers. METHOD: We devised a survey on the clinical features and appropriate management of UDT. Using convenience sampling, we approached health-care professionals with regular contact with paediatric patients and final year medical students. Respondents were allowed to remain anonymous. They were categorised according to specialty and level of experience/training. RESULTS: Of 1179 approached, 203 responded. Thirty-six (24%) of 149 qualified doctors had never seen a case of UDT. Median score was 6 (range 1-9). There was no significant difference in scores when comparing specialty. Mean scores decreased significantly in trend according to level of experience. When questioned regarding timings of referral and orchidopexy, 24% of qualified doctors would not refer until 9 months of age, and 66% thought orchidopexy should be done after 1 year old. Half would stop examining for UDT after 2 years old. CONCLUSIONS: Inexperience with UDT and outdated knowledge may contribute to delays in referral for UDT. Many would stop examining for UDT at 2 years old, placing undue reliance on accurate physical examination in early childhood and indicating lack of awareness of the ascending testis. Community health initiatives must emphasise recent changes in guidelines for management of UDT.
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Criptorquidismo/diagnóstico , Conocimientos, Actitudes y Práctica en Salud , Personal de Salud/educación , Pautas de la Práctica en Medicina/estadística & datos numéricos , Adolescente , Factores de Edad , Niño , Preescolar , Criptorquidismo/cirugía , Humanos , Lactante , Masculino , Orquidopexia , Estudios Prospectivos , Derivación y Consulta/estadística & datos numéricos , Encuestas y Cuestionarios , Testículo/cirugíaRESUMEN
INTRODUCTION: This study aims to find out the optimal timing for herniotomy for premature infants with inguinal hernia (IH): early during hospitalisation or delayed after hospital discharge. METHOD: A retrospective cohort study was conducted on premature infants diagnosed with IH during their initial hospitalization between 2015 and 2020. Demographic data and clinical outcomes were compared between infants undergoing herniotomy before discharge ("early") and those who were discharged without herniotomy ("delayed"). Student's t-test or Mann-Whitney U test and Fisher's exact test were used for statistical analysis. RESULTS: Of 219 premature infants, 189 (86.3%) underwent early herniotomy, while 30 were discharged with unoperated IH. In the delayed group, 15 (50%) underwent planned delayed herniotomy, and the remaining 15 experienced spontaneous resolution (absence of inguinal bulge over at least 1-year follow-up). The gestational age and birth weight of both groups were similar. At surgery, the delayed group median (interquartile range) was significantly older (42.1[38-49] vs 37.7 [36-40] weeks, p < 0.001) and heavier (3.27 [2.21-4.60] vs 2.22 [2.00-2.70] kg, p < 0.001). Two infants (1%) in the early group presented with incarcerated IH requiring urgent operation. In the delayed group, no infant developed incarcerated IH while awaiting elective operation (time from diagnosis to operation 44 [21-85] days). There was no statistically significant difference in respiratory and surgical complications between the two groups, although the delayed group had lesser surgical complications (0% vs 9.5%). CONCLUSION: Deferring herniotomy after discharge for premature infants is safe with close monitoring and associated with a chance of spontaneous resolution. LEVEL OF EVIDENCE: Level III, treatment study.
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Hernia Inguinal , Enfermedades del Prematuro , Humanos , Recién Nacido , Hernia Inguinal/cirugía , Herniorrafia , Hospitales , Recien Nacido Prematuro , Enfermedades del Prematuro/epidemiología , Enfermedades del Prematuro/cirugía , Alta del Paciente , Estudios Retrospectivos , LactanteRESUMEN
We describe a case of a premature 24 weeks gestation infant who presented with clinical lability and abdominal distention with initial concerns of necrotising enterocolitis. On further examination, a right inguinal hernia was noted and serial abdominal X-rays showed bowel loop dilatation with intramural air and no perforation. However, the hernia was recurrent and later found to be not reducible. He underwent right groin exploration. Intraoperatively, distal ileal perforation was noted and he was found to have an additional five sites of perforation. He had a stoma sited at the left iliac fossa as well as primary anastomosis at the site of the second to fifth perforations. He had a stormy postoperative period but is currently doing well. Although obstructed hernias are rare in the initial course of an extreme preterm infant, it should not be missed as a cause of intestinal obstruction and early surgical opinion should be sought.
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Enfermedades del Sistema Nervioso Autónomo , Hernia Inguinal , Enfermedades del Recién Nacido , Obstrucción Intestinal , Perforación Intestinal , Masculino , Lactante , Recién Nacido , Humanos , Recien Nacido Prematuro , Obstrucción Intestinal/diagnóstico por imagen , Obstrucción Intestinal/etiología , Obstrucción Intestinal/cirugía , Hernia Inguinal/complicaciones , Hernia Inguinal/diagnóstico por imagen , Hernia Inguinal/cirugía , Edad Gestacional , Perforación Intestinal/diagnóstico por imagen , Perforación Intestinal/etiología , Perforación Intestinal/cirugíaRESUMEN
Introduction: Doppler scrotal ultrasonography (US) is the modality of choice in diagnosing testicular torsion. We aimed to evaluate the performance of scrotal US in diagnosing testicular torsion over the past 18 years in our institution and determine the factors contributing to the length of wait times for it. Methods: A retrospective review was conducted of boys who presented with acute scrotal pain from 2014 to 2015. US reports, operative findings, final diagnosis and key time points of the patients' journey (time to emergency department consultation, time to admission, time to US and time to operating theatre [OT]) were collected. US performance results were compared with those observed in a historical cohort from 1998 to 2004. Wait times were compared between operated and non-operated patients. Results: Data from 519 boys with a mean age of 9.15 years was collected. Of these, 438 (84.4%) boys had undergone initial scrotal US; of these scrotal US cases, 28 were surgically explored, with 23 confirmed to have torsion. Another five cases were explored without prior US, and all were confirmed to have torsion. Performance analysis of US showed a sensitivity of 100% and a specificity of 98.8%. There was no significant difference between wait times of operated and non-operated patients. Time to US (P < 0.0001, r = 0.96) and time to OT (P < 0.0001, r = 0.64) correlated significantly with the total time from presentation to surgery. Conclusion: There has been an improvement in the diagnostic performance of scrotal US for testicular torsion over the past 18 years. Quality improvement programmes targeted at reducing wait times for patients presenting with acute scrotum should target time to US and time to OT.
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Dolor Agudo , Torsión del Cordón Espermático , Masculino , Niño , Humanos , Femenino , Escroto/diagnóstico por imagen , Escroto/cirugía , Torsión del Cordón Espermático/diagnóstico por imagen , Torsión del Cordón Espermático/cirugía , Dolor Agudo/diagnóstico por imagen , Ultrasonografía , Estudios RetrospectivosRESUMEN
BACKGROUND: During the Coronavirus disease 2019 (COVID-19) pandemic, a protocol was adopted by our department on antibiotics treatment for Acute Uncomplicated Appendicitis (AUA). Our study aimed to determine the feasibility and safety of non-operative treatment (NOT), compared to upfront laparoscopic appendectomy (LA), for AUA in children during the pandemic. METHOD: Our prospective comparative study was conducted from May 1, 2020 to January 31, 2021. Patient selection criteria included: age ≥5 years, abdominal pain duration ≤48 h, ultrasound (US)/Computered Tomography scan confirmation of AUA, US appendiceal diameter 6-11 mm with no features of perforation/abscess collection and no faecolith. For NOT patients, intravenous antibiotics were administered for 24-48 h followed by oral for 10-day course. Comparison was performed between patients whose parents preferred NOT to those who opted for up-front appendectomy. Primary outcomes were NOT success at index admission, early and late NOT failure rates till 27 months. Secondary outcomes were differences in complication rate, hospital length of stay (LOS) and cost between groups. RESULTS: 77 patients were recruited: 43 (55.8%) underwent NOT while 34 (44.2%) patients opted for LA. Success of NOT at index admission was 90.7% (39/43). Overall, NOT failure rate at 27 months' follow-up was 37.2% (16/43). Of the NOT failures, 1 appendix was normal on histology while only 1 was perforated. There were no significant differences in secondary outcomes between both groups except for LOS of late NOT failure. Cost for upfront LA was nearly thrice that of NOT. CONCLUSION: Our stringent COVID protocol together with shared decision-making with parents is a safe and feasible treatment option during a crisis situation. LEVEL OF EVIDENCE: Treatment study, Level II.
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Apendicitis , COVID-19 , Laparoscopía , Niño , Humanos , Preescolar , Antibacterianos/uso terapéutico , Apendicitis/tratamiento farmacológico , Apendicitis/cirugía , Pandemias , Estudios Prospectivos , Laparoscopía/métodos , Apendicectomía/métodos , Resultado del Tratamiento , Enfermedad AgudaRESUMEN
INTRODUCTION: Posterior urethral valve (PUV) is the most common congenital cause of bladder outflow obstruction in male infants. Despite timely treatment, renal damage can still occur in the long-term leading to chronic kidney disease (CKD). METHODS: A retrospective review of all PUV patients in a single tertiary institution between April 1998 and July 2019 was conducted to analyze their presentations, management and outcomes. Long-term renal function, radiologic scans and somatic growth were evaluated. RESULTS: A total of 16 patients were included in this study. Two patients who defaulted all follow-ups were excluded. Seven patients (43.7%) presented in the antenatal period; four patients (25%) presented in the neonatal period and five patients (31.3%) presented in the post-neonatal period. Primary transurethral fulguration of valves was done in 13 patients, while three had vesicostomies as the primary procedure. Three patients had associated anterior urethral valves (AUV), which were treated endoscopically. Nine boys had additional procedures for diversion and undiversion, VUR, non-functioning kidney and clean intermittent catheterization. Ten patients had urodynamic studies performed, of which eight patients received anticholinergic therapy. Eleven patients had DMSA scans, of which three patients had a normal study and eight patients showed unilateral reduced function. Four patients were diagnosed with CKD on long-term follow-up duration over 5 years. All patients were shown to have good somatic growth. CONCLUSION: Patients with PUV can suffer from complications despite primary treatment. In our small cohort, a quarter of our patients developed CKD on follow-up. Thus, patients need long-term follow-up to optimize bladder and renal function.
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Insuficiencia Renal Crónica , Uretra , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Embarazo , Estudios Retrospectivos , Singapur/epidemiología , Centros de Atención Terciaria , Uretra/cirugía , Vejiga UrinariaRESUMEN
Purpose: This study aims to compare the outcomes of extravesical (EVUR) and intravesical (IVUR) ureteric reimplantation for primary vesicoureteral reflux (VUR) via systematic review and meta-analysis. Methods: Literature review from Medline, Embase, and Cochrane since inception to March 2022 was performed. Meta-analysis was conducted on eligible randomized controlled trials (RCT) and observational cohort studies (OCS) comparing outcomes between EVUR and IVUR. Results: Twelve studies were included, comprising 577 patients (778 ureters) operated by EVUR and 395 patients (635 ureters) by IVUR. Pre-operative VUR grade, postoperative VUR persistence and hydronephrosis was not statistically significant. EVUR had shorter operative time [mean differences (MD) -22.91 min; 95% confidence interval (CI), -44.53 to -1.30, P = 0.04] and hospital stay (MD -2.09 days; 95% CI, -2.82 to -1.36, P < 0.00001) compared to IVUR. Bilateral EVUR had higher risk of postoperative acute urinary retention (ARU) (8.1%) compared to bilateral IVUR (1.7%) (OR = 4.40; 95% CI, 1.33-14.58, P = 0.02). No patient undergoing unilateral EVUR or IVUR experienced ARU. Conclusion: Both EVUR and IVUR are equally effective in correcting primary VUR. Operative time and hospital stay are shorter after EVUR compared to IVUR. However, bilateral EVUR is associated with higher risk of postoperative ARU.
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Hematuria is not uncommonly seen among children. We describe the case of a 13-year-old boy who was diagnosed with urothelial carcinoma after presenting with persistent gross hematuria for 2 weeks. We highlight the importance of adequate workup for gross hematuria as it is often associated with an underlying pathology that could lead to significant morbidity if left undiagnosed.
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Carcinoma de Células Transicionales , Neoplasias de la Vejiga Urinaria , Adolescente , Carcinoma de Células Transicionales/complicaciones , Carcinoma de Células Transicionales/diagnóstico , Niño , Cistoscopía , Familia , Hematuria/etiología , Humanos , Masculino , Neoplasias de la Vejiga Urinaria/complicaciones , Neoplasias de la Vejiga Urinaria/diagnósticoRESUMEN
INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. METHODS: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. RESULTS: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. CONCLUSION: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. STUDY DESIGN: Case series, level IV evidence.
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Riñón/anomalías , Conductos Paramesonéfricos/anomalías , Anomalías Urogenitales , Adolescente , Adulto , Niño , Preescolar , Errores Diagnósticos , Femenino , Hematocolpos/etiología , Hematocolpos/cirugía , Humanos , Lactante , Estudios Retrospectivos , Síndrome , Anomalías Urogenitales/complicaciones , Anomalías Urogenitales/diagnóstico , Anomalías Urogenitales/terapia , Vagina/anomalías , Vagina/cirugía , Adulto JovenRESUMEN
Diabetes insipidus is a syndrome characterised by the inability to conserve water or concentrate urine, leading to excessive excretion of urine. In congenital nephrogenic diabetes insipidus (CNDI), common presentations include failure to thrive, polydipsia, polyuria and dehydration. The long trajectory of the disease, coupled with psycho-behavioural changes as a child grows, can precipitate a period of non-adherence despite initial optimal control, especially in the adolescent age group. Social inconvenience of repeated voiding and nocturnal disturbances can lead to adapted urine holding behaviour, also known as non-neurogenic neurogenic bladder (Hinman syndrome). Anatomical changes in the urinary system, such as bladder trabeculation and hydroureteronephrosis, can subsequently give rise to functional renal impairment. We present a case of CNDI with concomitant Hinman syndrome, resulting in acute renal impairment and hypertensive emergency. We aim to raise awareness of the association between these two entities.
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Lesión Renal Aguda/etiología , Conducta Infantil/psicología , Diabetes Insípida Nefrogénica/fisiopatología , Hipertensión/etiología , Cooperación del Paciente/psicología , Vejiga Urinaria Neurogénica/etiología , Lesión Renal Aguda/fisiopatología , Lesión Renal Aguda/terapia , Niño , Diabetes Insípida Nefrogénica/tratamiento farmacológico , Diabetes Insípida Nefrogénica/psicología , Consejo Dirigido , Humanos , Hipertensión/fisiopatología , Hipertensión/terapia , Masculino , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vejiga Urinaria Neurogénica/fisiopatologíaRESUMEN
PURPOSE: The aim of our study was to develop an appendicitis score incorporating a urine biomarker, Leucine rich alpha-2-glycoprotein (LRG), for evaluation of children with abdominal pain. METHODS: From January to August 2017 we prospectively enrolled children aged 4-16â¯years old admitted for suspected appendicitis. Urine samples for LRG analysis were obtained preoperatively and quantified by enzyme-linked immunosorbent assay (ELISA) after correction for patient hydration status. The diagnosis of appendicitis was based on operative findings and histology. Logistic regression was used to identify prospective predictors. RESULTS: A total of 148 patients were recruited, of which 42(28.4%) were confirmed appendicitis. Our Appendicitis Urinary Biomarker (AuB) model incorporated urine LRG with 3 clinical predictors: 'constant pain', 'right iliac fossa tenderness', 'pain on percussion'. Area under the ROC curve for AuB was 0.82 versus 0.78 for the Pediatric Appendicitis Score (PAS) on the same cohort of patients. A model-calculated risk score of <0.15 is interpreted as low risk of appendicitis. Sensitivity for the AuB at this cutoff was 97.6%, specificity 37.7%, negative predictive value 97.6%, positive predictive value 38.3%, and negative likelihood ratio 0.06. CONCLUSION: The noninvasive AuB score appears promising as a diagnostic tool for excluding appendicitis in children without the need for blood sampling. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level III.
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Apendicitis/orina , Biomarcadores/orina , Glicoproteínas/orina , Dolor Abdominal/diagnóstico , Adolescente , Apendicitis/diagnóstico , Niño , Preescolar , Estudios de Cohortes , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Masculino , Estudios Prospectivos , Sensibilidad y EspecificidadRESUMEN
Acute appendicitis in the immunosuppressed patients are often difficult to diagnose and are associated with increased morbidity and mortality. Recent debates on primary appendicitis and typhlitis-associated appendicitis have contributed to the diagnostic and therapeutic dilemma. We retrospectively reviewed all our immunosuppressed patients with appendicitis. The most frequent presenting symptom was fever followed by non-specific intermittent abdominal pain. The median delay in diagnosis was 4 days. CT scan was useful in making the diagnosis, but the presence of adjacent bowel thickening has contributed to the dilemma in diagnosis and treatment.
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Apendicitis/diagnóstico , Terapia de Inmunosupresión , Adolescente , Apendicectomía , Apendicitis/cirugía , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Humanos , MasculinoRESUMEN
BACKGROUND: Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires. METHODOLOGY: A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation. Age-matched and gender-matched controls were enrolled from our patients with minor outpatient complaints. All participants completed the following PedsQL™ scales (maximum score 100): 4.0 Generic Core Scales, 3.0 General Well-Being (GWB) Scale and 2.0 Family Impact (FI) Module. All were also scored on bowel function (BFS), with a maximum score 20. Appropriate statistical analysis was performed, with significance level <0.05. RESULTS: There were 193 participants: 87 controls, 62 ARM, 44 HD. When comparing Core, GWB and FI scores, there were no significant differences between groups although controls had best scores indicating best QOL and general wellbeing, with least impact of the child's health on the family. BFS was significantly different with controls having best and ARM worst scores. There were no significant differences in scores between parent and child indicating intradyad consistency. There was significant positive correlation between BFS and Core (p<0.0001), and between BFS and GWB scores (p<0.005); and significant negative correlation between BFS and FI scores (p<0.0001). CONCLUSIONS: Bowel function impacts quality of life. Those with ARM and HD can achieve good quality of life comparable to controls, based on patient and caregiver self-reported outcomes. TYPE OF STUDY: Prospective comparative study LEVEL OF EVIDENCE: Level II.
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Malformaciones Anorrectales/fisiopatología , Enfermedad de Hirschsprung/fisiopatología , Calidad de Vida , Adolescente , Adulto , Malformaciones Anorrectales/psicología , Malformaciones Anorrectales/cirugía , Niño , Preescolar , Estudios Transversales , Femenino , Enfermedad de Hirschsprung/psicología , Enfermedad de Hirschsprung/cirugía , Humanos , Masculino , Padres , Estudios Prospectivos , Autoinforme , Encuestas y Cuestionarios , Adulto JovenRESUMEN
INTRODUCTION: Congenital pelviureteric junction obstruction (PUJO) is one of the most frequent causes of neonatal hydronephrosis. Obstruction at the PUJ has potential severe adverse outcomes, such as renal damage. While pyeloplasty has been established as the definitive treatment, the exact pathophysiology of congenital PUJO remains unknown. Recent research has proposed neuronal innervation defects as an etiological factor in congenital PUJO. We aim to study the expression of various neuronal markers in PUJO specimens compared with controls, and evaluate whether severity of renal disease or dysfunction pre-operatively is related to expression of neuronal markers in resected PUJO specimens. MATERIALS AND METHODS: All consecutive patients who underwent dismembered pyeloplasty at KK Women's and Children's Hospital, Singapore, for intrinsic PUJO from 2008 to 2012 were included. Patients with other co-occurring renal pathologies were excluded. Controls were obtained from nephrectomy patients with Wilm's tumor or other benign renal conditions during the same period. Specimens were stained immunohistochemically with neuronal markers protein gene product 9.5 (PGP9.5), synaptophysin, and S-100, and with CD-117, a marker for interstitial cells of Cajal (Table). Levels of expression of the markers were assessed semiquantitatively (decreased, increased or no change) in comparison with controls by two independent observers. Pre-operative data of patients' renal anatomical (ultrasonography measurements of renal pelvis size) and functional parameters (differential renal function measured using MAG-3 renal scans) were obtained. DISCUSSION: Thirty-eight PUJO specimens (38 renal units) and 20 controls were studied. Mean patient age at pyeloplasty was 25.3 months (2.9-167.6 months). Median pre-operative pelvic size was 25.0 mm (17.0-50.0 mm). Both PUJO specimens and controls showed great heterogeneity in distribution of innervation. All four immunohistochemical markers were not predictive of significant pre-operative renal pelvis dilation or pre-operative diminished renal function of the operated kidney. CONCLUSIONS: There exists marked variability in expression of neuronal markers synaptophysin, PGP9.5, and S-100, and CD-117 in PUJO specimens compared with controls. Our results show no clinical significance of the expression of neuronal markers in predicting degree of pre-operative renal pelvis dilation or differential renal function. The heterogeneity of expression of neuronal markers in PUJO specimens and controls in our population is at variance with prior studies. The etiology of PUJO is likely to be complex and multifactorial.
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Hidronefrosis/congénito , Pelvis Renal/cirugía , Riñón Displástico Multiquístico/etiología , Riñón Displástico Multiquístico/patología , Riñón Displástico Multiquístico/cirugía , Ubiquitina Tiolesterasa/metabolismo , Obstrucción Ureteral/etiología , Obstrucción Ureteral/patología , Obstrucción Ureteral/cirugía , Biomarcadores/metabolismo , Biopsia con Aguja , Estudios de Casos y Controles , Preescolar , Femenino , Humanos , Hidronefrosis/diagnóstico por imagen , Hidronefrosis/etiología , Hidronefrosis/patología , Hidronefrosis/cirugía , Inmunohistoquímica , Lactante , Laparoscopía/métodos , Masculino , Riñón Displástico Multiquístico/diagnóstico por imagen , Conducción Nerviosa , Valores de Referencia , Proteínas S100/metabolismo , Índice de Severidad de la Enfermedad , Sinaptofisina/metabolismo , Resultado del Tratamiento , Ultrasonografía Doppler , Obstrucción Ureteral/diagnóstico por imagenRESUMEN
In this paper, we report a case of bleeding solitary jejunal Dieulafoy pseudopolyp that was detected on capsule endoscopy and treated with a laparoscopic-assisted transumbilical polypectomy procedure. This case illustrates an innovative, tailored application of minimal invasive techniques in the management of a relatively uncommon lesion. To our knowledge, this is the first case report of the combination of capsule endoscopy and laparoscopic-assisted transumbilical resection for a bleeding jejunal Dieulafoy pseudopolyp in children.