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PURPOSE: Hepatocellular carcinoma (HCC), the second most common pediatric malignant liver tumor after hepatoblastoma, represents 1% of all pediatric tumors. METHODS: A retrospective study was conducted on children with HCC treated at our center from March 2002 to October 2022, excluding those with inadequate follow-up or records. Demographic data, initial complaints, alpha-fetoprotein (AFP) values, underlying disease, size and histopathological features of the masses, chemotherapy, and long-term outcomes were analyzed. RESULTS: Fifteen patients (8 boys, 7 girls) with a mean age of 11.4 ± 4.1 years (0.8-16.4 years) were analyzed. The majority presented with abdominal pain, with a median AFP of 3.9 ng/mL. Hepatitis B cirrhosis in one patient (6.6%) and metabolic disease (tyrosinemia type 1) in two patients (13.3%) were the underlying diseases. Histopathological diagnoses were fibrolamellar HCC (n:8; 53.3%), HCC (n:6; 40%). Four of the 15 patients underwent liver transplantation, and 9 underwent surgical resection. Due to late diagnosis, two patients were considered inoperable (13.3%). The survival rate for the four patients who underwent liver transplantation was found to be 75%. CONCLUSION: Surgical treatment of various variants of HCC can be safely performed in experienced centers with a multidisciplinary approach, and outcomes are better than in adults.
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Carcinoma Hepatocelular , Neoplasias Hepáticas , Trasplante de Hígado , Humanos , Masculino , Neoplasias Hepáticas/cirugía , Carcinoma Hepatocelular/cirugía , Femenino , Estudios Retrospectivos , Niño , Adolescente , Preescolar , Lactante , Resultado del Tratamiento , Hepatectomía/métodos , Tasa de Supervivencia , Estudios de SeguimientoRESUMEN
AIM: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed. METHODS: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated. RESULTS: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period. CONCLUSION: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases.
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Hernia Diafragmática , Vólvulo Intestinal , Trasplante de Hígado , Humanos , Niño , Trasplante de Hígado/efectos adversos , Hernia Diafragmática/cirugía , Hernia Diafragmática/complicaciones , Tomografía Computarizada por Rayos X/efectos adversos , Dolor Abdominal/complicacionesRESUMEN
AIM: Hesitations concerning the long-term results of transanal endorectal pull-through (TEPT) due to prolonged anal stretching and resultant stricture and continence problems has been started to be questioned. This meta-analysis intended to compare long-term results between TEPT and transabdominal (TAB) pull-through techniques in the surgical management of Hirschsprung's disease. METHODS: All publications between the years 1998-2021 in the PubMed, Medline, Google Scholar, Cochrane databases were reviewed. Retrospective and prospective comparative studies for TEPT, TAB as well as Laparoscopic-assisted TEPT (LTEPT) were included. Data included age at operation, postoperative constipation, enterocolitis, incontinence, stricture, and soiling rates. RESULTS: Eighteen publications met the inclusion criteria for TAB and TEPT, and six for TEPT and LTEPT. Patients who underwent TEPT had significantly younger operation age than patients with TAB (SMD - 1.02, 95%Cl - 1.85 to - 0.18, p: 0.0168). Postoperative constipation (OR 0.39, 95% Cl 0.25-0.61 p < 0.0001) and enterocolitis (OR 0.65, 95% Cl 0.46-0.90, p: 0.0108) rates were significantly lower in TEPT groups. Postoperative incontinence (OR 1.06, 95% Cl 0.56-2.01, p: 0.8468), stricture (OR 1.97, 95% Cl 0.81-4.80, p: 0.1352) and soiling rates were similar between the two groups. Furthermore, when TEPT and LTEPT results were compared, incidence of incontinence (OR 7.01, 95% Cl 0.75-65.33, p: 0.0871), constipation (OR 1.95, 95% Cl 0.70-5.37, p: 0.199), enterocolitis (OR 3.16, 95% Cl 0.34-29.55 p: 0.3137), stricture (OR 1.33, 95% Cl 0.29-6.15, p: 0.7188) and soiling (OR 1.57, 95% Cl 0.57-4.31, p: 0.3778) were similar for both techniques. DISCUSSION: TEPT is superior to TAB in terms of constipation and enterocolitis. Contrary to concerns, postoperative incontinence rates are not statistically different. However, further publications about long-term LTEPT results are necessary for more reliable conclusions.
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Procedimientos Quirúrgicos del Sistema Digestivo , Enterocolitis , Enfermedad de Hirschsprung , Humanos , Lactante , Enfermedad de Hirschsprung/cirugía , Enfermedad de Hirschsprung/complicaciones , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Resultado del Tratamiento , Estudios Retrospectivos , Estudios Prospectivos , Constricción Patológica/complicaciones , Estreñimiento/etiología , Estreñimiento/complicaciones , Enterocolitis/etiología , Complicaciones Posoperatorias/epidemiologíaRESUMEN
PURPOSE: The management of pediatric ovarian neoplasms (ON) is based on finding a balance between adequate surgical treatment and future reproductive capacity. We aimed to evaluate long-term results of patients who underwent surgery for ON. METHODS: A retrospective cohort study design was used. Medical records of patients with ON were reviewed. They were invited to participate in a telephone-based survey assessing complaints, menstrual status, and post-surgical recurrence. RESULTS: Eighty-five patients were operated for ON between 1995 and 2015. Median age at surgery was 14.7 years. 62.4% of patients had ovary-sparing surgery (OSS). Median tumor size in oophorectomy group was significantly larger than OSS group (p = 0.029). Median length of follow-up was 5.1 years. Recurrent/metachronous disease was not significantly different between OSS and oophorectomy groups (p = 1.000). In OSS group, irregular menses (p = 0.004) and painful menses (p = 0.002) were significantly higher than oophorectomy group. CONCLUSION: The main goal of treatment in pediatric ON is to find the right balance between adequate and appropriate tumor resection and maximal effort for fertility preservation. Our results showed no difference between oophorectomy and OSS in the terms of recurrence. Although irregular and painful menses were found to be significantly higher in the OSS group, longer follow-up and prospective studies are needed to clarify this issue.
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Neoplasias Ováricas , Niño , Humanos , Femenino , Adolescente , Estudios Retrospectivos , Neoplasias Ováricas/cirugía , Neoplasias Ováricas/patología , Ovariectomía/métodos , Resultado del Tratamiento , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/cirugía , Estadificación de NeoplasiasRESUMEN
INTRODUCTION: Neurogenic bladder with anorectal malformations (ARM) is a well-known yet understudied topic. Diffusion tensor imaging (DTI) is a special usage of MRI that can evaluate peripheric nerves. The aim of this study is to evaluate the lumbosacral plexuses of patients with ARM using DTI. PATIENTS AND METHODS: Patients with ARM willing to participate were included. Patient files were reviewed, a questionnaire was made, and DTI was performed. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were computed for the right and left lumbosacral plexuses. RESULTS: There were 18 patients and 12 controls. Groups were similar in terms of sex (p = 0.232) and age (p = 0.853). There was a significant difference only for ADC of the left plexus. There was visible asymmetry in tractographies and DTI parameters of two patients with severely deformed pelvises (image 1) but also in another patient with a normal sacrum. In addition, despite asymmetry, FA and ADC were similar to controls also in these patients. CONCLUSION: This is the first study presenting quantitative data about the lumbosacral plexus in ARM. We think the most interesting finding of this study was observing the normal values even in the patients with severely malformed sacrum which is yet to be validated with further studies.
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Malformaciones Anorrectales , Imagen de Difusión Tensora , Malformaciones Anorrectales/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética , Imagen de Difusión Tensora/métodos , Humanos , Plexo Lumbosacro/diagnóstico por imagen , Proyectos PilotoRESUMEN
STUDY OBJECTIVE: Antenatal ovarian torsion (AOT) is rare and requires differentiating from other congenital cystic masses of the abdomen and pelvis in neonates. In this study, it was aimed to evaluate the prenatal characteristics and postnatal outcomes of AOT. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Hospital records of patients (n = 28) with diagnosis of AOT between 2004 and 2020 were reviewed and their prenatal characteristics, postnatal examination, imaging, operative, and histopathological findings were evaluated. RESULTS: There were 28 patients during the study period. In most of the patients (25/28; 89.3%) AOTs were detected prenatally. All were term babies with a mean birth weight of 3010 ± 466.6 g. Mean maternal age was 25.75 ± 3.65 years. Prenatal history was event-free in all and none had additional anomalies. Physical examination revealed mobile intra-abdominal cystic lesions in 16 (57.1%) patients. AOTs mimicked other pathologies as intestinal duplication cyst or mesenteric cyst (n = 7), complex ovarian cyst (n = 3), mature cystic teratoma (n = 3), simple renal cyst (n = 1), and ectopic kidney (n = 1) in 15 (53.6%) patients in postnatal ultrasonography. Elective laparoscopic surgery was performed in 26 (92.8%) patients. The other 2 patients required emergency open surgery because of acute symptoms. Ovaries were autoamputated in 17 (60.7%) patients. Histopathological examination revealed necrosis and dystrophic calcification in all specimens. There was neoplastic involvement in 2 patients (serous cystadenoma and gonadoblastoma). No complication occurred in the early follow-up period (mean: 73.9 ± 46.8 months). CONCLUSION: AOTs can be detected easily during the antenatal period. Neoplastic involvement with AOTs is rare but possible. Although AOTs might mimic other cystic pathologies, it should be one of the first diagnoses to be considered, in the presence of a palpable intra-abdominal mass and complex cystic lesion in infant girls. It can be efficiently and safely managed using minimally invasive techniques.
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Quiste Dermoide , Neoplasias Ováricas , Adulto , Femenino , Humanos , Lactante , Recién Nacido , Torsión Ovárica , Embarazo , Estudios Retrospectivos , Ultrasonografía , Adulto JovenRESUMEN
PURPOSE: Wnt/Beta-catenin pathway plays an essential role in liver development and regeneration. Abnormal activation in this pathway leads to development of hepatoblastoma (HB). Although its importance has invoked attention, its prognostic role is debatable. We aimed to evaluate the significance of intracellular localization of beta-catenin (BC) expression in the outcome of hepatoblastoma patients. METHODS: Medical records of HB patients between 2004 and 2018 were reviewed. Patients were grouped according to intracellular localization of BC expression by immunohistochemistry as being cytoplasmic or nuclear. Demographics, radiological images, PRETEXT classifications, vascular involvement, risk groups, chemotherapy responses, and survival rates were analyzed and compared between groups. RESULTS: There were 41 patients. Thirteen patients were excluded for unavailability of records in four, negative/unclear BC expressions in seven. Cytoplasmic expression of BC was observed in 17 patients whereas 13 patients displayed nuclear expression. Demographics were similar in both groups. Cytoplasmic BC expression was associated with poor chemotherapy response (p = 0.001) and increased vascular involvement (p = 0.0162) requiring more extensive surgeries (p = 0.039). CONCLUSION: Although the numbers are limited in our series, the intracellular localization of BC expression has been found to be a promising determining factor for hepatoblastoma prognosis. With larger patient series, more reliable results can be achieved.
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Hepatoblastoma/tratamiento farmacológico , Hepatoblastoma/genética , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/genética , beta Catenina/genética , Femenino , Hepatoblastoma/metabolismo , Humanos , Inmunohistoquímica , Lactante , Neoplasias Hepáticas/metabolismo , Masculino , Pronóstico , Tasa de Supervivencia , Resultado del Tratamiento , beta Catenina/metabolismoRESUMEN
The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40 years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals.
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Canal Anal/cirugía , Malformaciones Anorrectales/cirugía , Procedimientos Quirúrgicos del Sistema Digestivo/efectos adversos , Procedimientos de Cirugía Plástica/efectos adversos , Complicaciones Posoperatorias , Recto/cirugía , Canal Anal/anomalías , Niño , Humanos , Recto/anomalíasRESUMEN
BACKGROUND/AIMS: Despite surgical advances in liver transplantation and effective prophylactic strategies, posttransplant infections are the most important cause of morbidity and mortality. Diagnosis and management of infections because of developing immunosuppression is difficult and adversely affects mortality. This study aimed to review bacterial and fungal infections in patients after liver transplantation and to reveal the resistance rates. MATERIALS AND METHODS: A total of 107 patients who underwent liver transplantation between January 2017 and February 2018 were evaluated retrospectively with regard to demographic characteristics, causes of transplantation, conditions that may lead to infection, postoperative infections, pathogens, and resistance patterns. RESULTS: Of the 107 patients who underwent liver transplantation, 48 (44.8%) had an infection. Bacterial infections were detected in 41% of the patients, and fungal infections were found in 13%. When we compared living and cadaveric transplants in terms of infection development, these rates were found to be 53% and 33%, respectively (p=0.034). No statistically significant results could be obtained when evaluating conditions such as sex, presence of underlying primary disease, Model for End-Stage Liver Disease MELD score, diabetes status, total parenteral nutrition, and risk factors for infection. CONCLUSION: After liver transplantation, infections are often seen in the first month of the postoperative period. Knowing the most common pathogens and resistance states in this process reduces infection-related deaths by providing appropriate treatment regimens at the right time.
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Infecciones Bacterianas/inmunología , Terapia de Inmunosupresión/efectos adversos , Trasplante de Hígado/efectos adversos , Micosis/inmunología , Complicaciones Posoperatorias/mortalidad , Adolescente , Adulto , Anciano , Enfermedad Hepática en Estado Terminal/inmunología , Enfermedad Hepática en Estado Terminal/cirugía , Femenino , Humanos , Trasplante de Hígado/métodos , Donadores Vivos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/inmunología , Complicaciones Posoperatorias/microbiología , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
Tekin A, Yagmur I, Ergün O, Ayik MF, Atay Y, Ulman I, Avanoglu A. Excision of the atrial Wilms` tumor thrombus without sternotomy, atriotomy and cardiovascular By-pass. Turk J Pediatr 2019; 61: 436-439. The treatment of atrial-extention Wilms` tumor thrombus is surgical excision after chemotherapy. Atriotomy with cardiovascular by-pass is the one of the most common method for this procedure. Herein, we aimed to present a case of Wilms` tumor with a tumor thrombus extending into the right atrium totally excised with retrohepatic cavatomy. A 3.5 year-old girl was admitted with the symptom of dysuria. The examinations revealed a mass consistent with Wilms` tumor in the middle and lower poles of the left kidney. Doppler ultrasound and Echocardiographic examinations showed a tumor thrombus extending into the right atrium and some pulmonary nodules which were interpreted to be metastasis. Wilms` tumor was histopathologically diagnosed by an open biopsy. After three courses of chemotherapy imaging studies revealed that the atrial axtention of the tumor thrombus persisted. The tumor thrombus was found to be fibrotic on the magnetic resonance imaging scan of the patient. Therefore, nephroureterectomy along with the excision of the tumor thrombus from the inferior vena cava was done with intraoperative continuous trans-esophageal echocardiography (TEE). The suprarenal and retrohepatic vena cava were exposed by dissecting and ligating all short hepatic veins and completely mobilizing the right lobe of the liver. The thrombus was dissected out via Vertical cavatomy at the retrohepatic level. TEE confirmed complete removal of the thrombus from the atrium; Vena cava was then repaired. There was no need for a blood transfusion, or cardiovascular by-pass (CPB) during the operation. Total exposure of the retrohepatic and subdiaphragmatic vena cava using transplantation techniques is an effective method for the excision of a tumor thrombus without sternotomy, atriotomy and CPB, avoiding possible intra- and postoperative complications in selected cases of Wilms` tumor with intraatrial thrombus extension. The case emphasises the importance of multidisciplinary communication and collaboration.
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Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías/cirugía , Neoplasias Renales/complicaciones , Trombosis/cirugía , Tumor de Wilms/complicaciones , Preescolar , Ecocardiografía , Femenino , Atrios Cardíacos , Cardiopatías/diagnóstico , Cardiopatías/etiología , Humanos , Neoplasias Renales/diagnóstico , Neoplasias Renales/cirugía , Imagen por Resonancia Cinemagnética , Esternotomía , Trombosis/diagnóstico , Trombosis/etiología , Tumor de Wilms/diagnóstico , Tumor de Wilms/cirugíaRESUMEN
BACKGROUND/AIMS: Liver transplantation (LT) is now the standard of care for most end-stage liver diseases. Over the next 30 years, advances in medicine and technology will greatly improve the survival rates of patients after this procedure. The aim of the present study was to analyze retrospectively the results of 1001 patients withLT. MATERIALS AND METHODS: Medical reports of 989 patients were analyzed retrospectively. Data were obtained from the patient's data chart. Descriptive statistics were used to describe continuous variables (mean, median, and standard deviation). RESULTS: A total of 1001 LTs for 989 recipients were performed at Ege University Organ Transplantation and Research Center between 1994 and 2017. Therewere 639 male and 350 female recipients. Among 1001 LTs, there were 438 deceased donors and 563 living donors. The age interval of the patients was 4 months to 71 years old. The median Model for End-Stage Liver Disease score was 20. There were 12 deceased liver donors using the split method. There were 12 cases subject to retransplantation. In living donor LT grafts, 423 right lobes, 46 left lobes, and 94 left lateral sectors were used. In the first monitoring,the total annual mortality rate was 130 cases (13%). The mortality rate in retransplantation was found to be 66%. A 1-year survival rate of 87% was generally stablished. CONCLUSION: LThas been improving consistently over the last two decades. Ege University is one of the biggest liver transplant centers in Turkey for both technical and educational perspective.
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Enfermedad Hepática en Estado Terminal/cirugía , Trasplante de Hígado/mortalidad , Adolescente , Adulto , Anciano , Niño , Preescolar , Enfermedad Hepática en Estado Terminal/etiología , Enfermedad Hepática en Estado Terminal/mortalidad , Femenino , Supervivencia de Injerto , Hospitales Universitarios/estadística & datos numéricos , Humanos , Lactante , Trasplante de Hígado/estadística & datos numéricos , Donadores Vivos/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Resultado del Tratamiento , Turquía , Adulto JovenRESUMEN
BACKGROUND: Decision-making for management may sometimes be difficult in acute appendicitis (AA). Various diagnostic scoring systems exist, but their sensitivity and specificity rates are far from ideal. In this study, the determination of the predictors and the effect of radiological data and developing a new scoring system were aimed. METHODS: Medical records of patients who were hospitalized for AA between February 2012 and October 2016 were retrospectively reviewed. All data were compared between patients with and without appendicitis. The multivariate analysis was performed to define significant variables and to examine the sensitivity and specificity of each group of predictors including radiological data. A new scoring system (NSS) was formed and was compared with two existing scoring systems: pediatric appendicitis score (PAS) and Alvarado scoring system (ASS) by using reclassification method. RESULTS: Negative appendectomy rate was 11.3%. Statistical analysis identified 21 independently significant variables. The heel drop test had the highest odds ratio. Sensitivity and specificity rates of clinical predictors were 84.6% and 94.8%, respectively. Radiological predictors increased the sensitivity rate to 86.9%. Sensitivity and specificity rates for PAS, ASS, and NSS were 86.8% and 83.9%, 84.7% and 81.6%, and 96.8% and 95.6%, respectively. The "re-assessed negative appendectomy rate" was 6.2% and false positive results were remarkably more common in patients with duration of symptoms less than 24 hours. CONCLUSION: Radiological data improves the accuracy of diagnosis. Containing detailed clinical and radiological data, NSS performs superiorly to PAS and ASS, regarding sensitivity and specificity without any age limitation. The efficiency of NSS may be enhanced by determining different predictors for different phases of the inflammatory process.
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Background/aim: The aim of this study was to combine some easy and economical defined methods and constitute a comprehensive cell culture model to use in the bowel diseases characterized by inflammation. Materials and methods: Induction of inflammation was performed using lipopolysaccharide (LPS) or a cytokine mixture (TNF-α: 10 ng/mL; IFN-γ: 100 ng/mL; IL-1ß: 1 ng/mL). IEC-6 cells were grown to confluence and serum-starved, wounds were constituted, and progress of cell migration into the wounds was photographed at 0, 2, 4, 6, 8, 10, 12, and 24 h in the presence or absence of an inflammatory environment. Cells were then grown and multiple scratches were performed to replicate the conditions of migration assay. Nitric oxide synthase-2 (iNOS) and cyclooxygenase-2 (COX-2) protein expressions were assessed. Results: Cells covered 88% of the initial wound at 24 h in the control group, 54% in the LPS group, and 35% in the cytokine mixture group. LPS and the cytokine mixture were also found to independently increase iNOS and COX-2 expressions. Conclusion: Our study, being inexpensive and practical, describes a model that integrates some methods to constitute a basic model for bowel diseases characterized by inflammation. It can be integrated as a preliminary experiment for etiopathogenesis and drug research studies.
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Symptoms of infantile inflammatory bowel disease (I-IBD) can be life-threatening and associated with poor prognosis. The presence of Mediterranean fever (MEFV) gene mutations play an important role in treatment of I-IBD. In this article, we describe a case of I-IBD with a resistant fistula, in which remission occurred following colchicine therapy. The patient was a six-month-girl with complaints of bloody diarrhea and a perianal abscess of three months duration. Laboratory tests revealed elevated inflammatory parameters, hypoalbuminemia, and anemia. Results of repeated viral, bacterial and parasitic analyses were negative. Endoscopic and histopathological examinations confirmed a diagnosis of I-IBD. Although diarrhea episodes decreased following intensive conventional treatment with immunosuppressive therapy and anti-tumor necrosis factor, the perianal abscess and fistula did not resolve. Molecular genetic analysis to identify causes of infantile disease revealed the MEFV gene mutation. Thus, colchicine was added to the treatment regimen. Following treatment with colchicine, defecation returned to normal, and the fistula resolved. The MEFV gene mutation should be investigated in children with infantile colitis and resistant fistulas, particularly in Mediterranean countries. In patients with infantile colitis who have the MEFV gene mutation, colchicine treatment may be an alternative to intensive immunosuppressive therapy.
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INTRODUCTION: Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. CASE REPORT: The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). CONCLUSION: Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases.How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166.
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BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively. Patients were evaluated for birth weight; age at jaundice onset; age at alcoholic stool detection; age at the time of Kasai portoenterostomy (KPE), if performed; age at admission to our center; age at liver transplantation; duration between KPE and transplantation; pediatric end-stage liver disease (PELD) scores during transplantation; and growth and developmental status. The periods 1990-2002 and 2003-2015 were defined as phases I and II, respectively. RESULTS: The median age of the patients at presentation to our hospital was 149 (range: 20-730) days during phase I and 61 (range: 28-720) days during phase II. The median age at jaundice onset was 7 days, and the median age at alcoholic stool detection was 15 days. There was no significant difference between phases I and II in terms of age at jaundice onset, age at alcoholic stool detection, or birth weight. Twenty-five (92.5%) of the 27 patients in the phase I group were admitted to our center after undergoing KPE. Forty-four (61.1%) of the 72 patients in the phase II group (median age at the time of KPE: 47 days) were operated at our center. Median ages of the patients at the time of KPE at our center were 67.5 (range: 25-220) and 47 (range: 28-139) days during phases I and II, respectively. The median age of the 28 patients who were transferred from another center was 70 (range: 45-105) days during phase II. Liver transplantation was performed in 55 of 99 patients (55.5%). Significant differences were observed in the age at transplantation, duration between KPE and transplantation, and PELD scores between patients with BA who underwent KPE at our center and who underwent KPE at other institutes from other institutes. CONCLUSION: These findings demonstrate the importance of a timely diagnosis of BA and undergoing KPE before malnutrition and/or cirrhosis deteriorate the patient's health. Furthermore, follow-up of patients with BA at a liver transplantation center increased the success of KPE and improved survival rates.
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Atresia Biliar/mortalidad , Trasplante de Hígado/mortalidad , Portoenterostomía Hepática/mortalidad , Factores de Edad , Atresia Biliar/cirugía , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Trasplante de Hígado/métodos , Masculino , Estado Nutricional , Portoenterostomía Hepática/métodos , Estudios Retrospectivos , Facultades de Medicina , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Factores de Tiempo , Tiempo de Tratamiento , Resultado del Tratamiento , UniversidadesRESUMEN
BACKGROUND: Primary ovarian fibrosarcomas are rare and usually observed in perimenopausal and postmenopausal women. To our knowledge, to date, there are only 3 reports of ovarian fibrosarcoma in childhood and adolescence in the English-language literature. In this report, we aimed to present to our knowledge, the first pediatric case with advanced stage primary ovarian fibrosarcoma and to compare with previous cases. CASE: A 14-year-old teenage girl was admitted because of a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass. Salpingo-oophorectomy along with staging procedures were performed. Pathologic examination confirmed grade III ovarian fibrosarcoma with severe nuclear atypia and high mitotic count. Because of pulmonary metastasis, she received 4 courses of adjuvant combination chemotherapy (Ewing protocol). The patient withstood the treatment well and is disease-free at 2 years of follow-up. CONCLUSION: Fibrosarcoma of the ovary is an uncommon neoplasm with poor prognosis. To our knowledge, the presented patient is the first pediatric case with advanced staged primary ovarian fibrosarcoma to be treated with surgery and adjuvant chemotherapy.
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Fibrosarcoma/terapia , Neoplasias Ováricas/patología , Salpingooforectomía/métodos , Adolescente , Quimioterapia Adyuvante , Femenino , Humanos , Neoplasias Ováricas/terapiaRESUMEN
OBJECTIVE: The management of childhood thyroid nodules is still a big challenge for clinicians. In this study, we aimed to present our surgical and endocrinological experience in more than one hundred pediatric cases. METHODS: A retrospective analysis of patients admitted with a thyroid nodule between 2006 and 2014 was performed. Detailed ultrasonography and fine-needle aspiration biopsy (FNAB) were the cornerstones of the diagnostic approach. RESULTS: One hundred-three children (72 female, 31 male) with a mean age of 13.1±3.6 years (3-18 years) were admitted to our center. Management strategy was surgery in 58 patients and follow-up in 45 patients. Mean nodule size was 17±12.7 mm (2-45 mm). The diagnoses were listed as benign solitary nodule (48 patients), thyroid carcinoma (26 patients), multinodular goiter (23 patients), Hashimoto thyroiditis (4 patients), and Graves' disease (2 patients). Surgical procedures were nodulectomy/lobectomy (32 patients), total thyroidectomy (TT) (13 patients), or TT+ neck dissection (13 patients). The rate of malignancy was 25% in the total group and 44% in the surgery group. The malignancy rate was higher in patients younger than 12 years compared to older children (41% vs. 17%, p=0.040). Metastasis was seen in 38% of the malignant nodules. Postoperative complications were transient hypocalcemia (8%), permanent hypocalcemia (1.7%), and unilateral vocal cord paralysis (1.7%). Recurrence or mortality was not encountered in the 5.4±1.2-year follow-up period. CONCLUSION: Thyroid nodule in a child requires an aggressive diagnostic approach due to increased risk of malignancy and metastasis. Intraoperative frozen section examination must be done as a useful adjunct to determine the surgical strategy. Incidence of complications is small in thyroid surgery when performed by experienced surgeons.