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1.
Pediatr Transplant ; 28(3): e14749, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38623878

RESUMO

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.


Assuntos
Hérnia Diafragmática , Volvo Intestinal , Transplante de Fígado , Humanos , Criança , Transplante de Fígado/efeitos adversos , Hérnia Diafragmática/cirurgia , Hérnia Diafragmática/complicações , Tomografia Computadorizada por Raios X/efeitos adversos , Dor Abdominal/complicações
3.
Pediatr Surg Int ; 39(1): 89, 2023 Jan 24.
Artigo em Inglês | MEDLINE | ID: mdl-36692536

RESUMO

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.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Enterocolite , Doença de Hirschsprung , Humanos , Lactente , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/complicações , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Resultado do Tratamento , Estudos Retrospectivos , Estudos Prospectivos , Constrição Patológica/complicações , Constipação Intestinal/etiologia , Constipação Intestinal/complicações , Enterocolite/etiologia , Complicações Pós-Operatórias/epidemiologia
4.
Pediatr Surg Int ; 38(12): 2035-2044, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36169670

RESUMO

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.


Assuntos
Neoplasias Ovarianas , Criança , Humanos , Feminino , Adolescente , Estudos Retrospectivos , Neoplasias Ovarianas/cirurgia , Neoplasias Ovarianas/patologia , Ovariectomia/métodos , Resultado do Tratamento , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/cirurgia , Estadiamento de Neoplasias
5.
Pediatr Surg Int ; 38(4): 609-616, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35129658

RESUMO

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.


Assuntos
Malformações Anorretais , Imagem de Tensor de Difusão , Malformações Anorretais/diagnóstico por imagem , Imagem de Difusão por Ressonância Magnética , Imagem de Tensor de Difusão/métodos , Humanos , Plexo Lombossacral/diagnóstico por imagem , Projetos Piloto
6.
J Pediatr Adolesc Gynecol ; 35(1): 18-22, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34454073

RESUMO

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.


Assuntos
Cisto Dermoide , Neoplasias Ovarianas , Adulto , Feminino , Humanos , Lactente , Recém-Nascido , Torção Ovariana , Gravidez , Estudos Retrospectivos , Ultrassonografia , Adulto Jovem
7.
Pediatr Surg Int ; 36(7): 817-825, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32436062

RESUMO

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.


Assuntos
Hepatoblastoma/tratamento farmacológico , Hepatoblastoma/genética , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/genética , beta Catenina/genética , Feminino , Hepatoblastoma/metabolismo , Humanos , Imuno-Histoquímica , Lactente , Neoplasias Hepáticas/metabolismo , Masculino , Prognóstico , Taxa de Sobrevida , Resultado do Tratamento , beta Catenina/metabolismo
8.
Pediatr Surg Int ; 36(4): 431-445, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32086570

RESUMO

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.


Assuntos
Canal Anal/cirurgia , Malformações Anorretais/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Procedimentos de Cirurgia Plástica/efeitos adversos , Complicações Pós-Operatórias , Reto/cirurgia , Canal Anal/anormalidades , Criança , Humanos , Reto/anormalidades
9.
Turk J Gastroenterol ; 31(12): 894-901, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33626002

RESUMO

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.


Assuntos
Infecções Bacterianas/imunologia , Terapia de Imunossupressão/efeitos adversos , Transplante de Fígado/efeitos adversos , Micoses/imunologia , Complicações Pós-Operatórias/mortalidade , Adolescente , Adulto , Idoso , Doença Hepática Terminal/imunologia , Doença Hepática Terminal/cirurgia , Feminino , Humanos , Transplante de Fígado/métodos , Doadores Vivos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/imunologia , Complicações Pós-Operatórias/microbiologia , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Adulto Jovem
10.
Turk J Pediatr ; 61(3): 436-439, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31916725

RESUMO

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.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias/cirurgia , Neoplasias Renais/complicações , Trombose/cirurgia , Tumor de Wilms/complicações , Pré-Escolar , Ecocardiografia , Feminino , Átrios do Coração , Cardiopatias/diagnóstico , Cardiopatias/etiologia , Humanos , Neoplasias Renais/diagnóstico , Neoplasias Renais/cirurgia , Imagem Cinética por Ressonância Magnética , Esternotomia , Trombose/diagnóstico , Trombose/etiologia , Tumor de Wilms/diagnóstico , Tumor de Wilms/cirurgia
11.
Turk J Gastroenterol ; 29(6): 664-668, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30381274

RESUMO

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.


Assuntos
Doença Hepática Terminal/cirurgia , Transplante de Fígado/mortalidade , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Doença Hepática Terminal/etiologia , Doença Hepática Terminal/mortalidade , Feminino , Sobrevivência de Enxerto , Hospitais Universitários/estatística & dados numéricos , Humanos , Lactente , Transplante de Fígado/estatística & dados numéricos , Doadores Vivos/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Turquia , Adulto Jovem
12.
Emerg Med Int ; 2018: 4810730, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30275991

RESUMO

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.

14.
Turk J Med Sci ; 48(2): 435-440, 2018 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-29714467

RESUMO

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.

15.
Arch Rheumatol ; 33(4): 473-477, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30874249

RESUMO

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.

16.
Euroasian J Hepatogastroenterol ; 8(2): 163-166, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30828559

RESUMO

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.

17.
Turk J Gastroenterol ; 28(4): 298-302, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28699603

RESUMO

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.


Assuntos
Atresia Biliar/mortalidade , Transplante de Fígado/mortalidade , Portoenterostomia Hepática/mortalidade , Fatores Etários , Atresia Biliar/cirurgia , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Transplante de Fígado/métodos , Masculino , Estado Nutricional , Portoenterostomia Hepática/métodos , Estudos Retrospectivos , Faculdades de Medicina , Índice de Gravidade de Doença , Taxa de Sobrevida , Fatores de Tempo , Tempo para o Tratamento , Resultado do Tratamento , Universidades
18.
J Pediatr Adolesc Gynecol ; 30(6): 655-658, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28502829

RESUMO

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.


Assuntos
Fibrossarcoma/terapia , Neoplasias Ovarianas/patologia , Salpingo-Ooforectomia/métodos , Adolescente , Quimioterapia Adjuvante , Feminino , Humanos , Neoplasias Ovarianas/terapia
19.
J Clin Res Pediatr Endocrinol ; 9(3): 222-228, 2017 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-28387647

RESUMO

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.


Assuntos
Nódulo da Glândula Tireoide/diagnóstico , Nódulo da Glândula Tireoide/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
20.
Turk J Gastroenterol ; 28(3): 214-218, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28336499

RESUMO

BACKGROUND/AIMS: Pancreatic solid pseudopapillary tumor (SPT) is a rare neoplasm in children. In this study, we aimed to present our surgical strategy based on minimal resection by enucleation or limited resection in localized pancreatic SPT. MATERIALS AND METHODS: We retrospectively analyzed the medical records of children who underwent surgical resection between October 2011 and September 2016. RESULTS: Five female patients with a median age of 15 years (range, 14-17 years) were operated. Tumors were located in the pancreatic head (n=4) or tail (n=1). The median greatest tumor diameter was 9 cm (range, 5-13 cm). All the patients were investigated with MRI before the resection to demonstrate the relationship between the tumor and the main pancreatic duct. Patients underwent enucleation (n=4) for head localization or local distal resection without splenectomy (n=1) at the pancreatic tail. At postoperative follow-up, major pancreatic leakage was observed in two patients and endoscopically treated. Surgical margins were negative in all patients. The median follow-up period was 44 months (range, 2-59 months) and no local recurrence or distant metastasis was observed in the postoperative period. CONCLUSION: An optimal surgical strategy is still controversial in pancreatic SPT in children. Radical resections such as pancreaticoduodenoctomy or distal pancreatectomy with splenectomy result in loss of pancreatic tissue for endocrine and exocrine functions. Minimal resections such as enucleation or limited pancreatic resection with negative surgical margins should be performed in selected patients with no invasion to the main pancreatic duct or adjacent organs.


Assuntos
Carcinoma Papilar/cirurgia , Pancreatectomia/métodos , Neoplasias Pancreáticas/cirurgia , Adolescente , Carcinoma Papilar/patologia , Feminino , Seguimentos , Humanos , Pâncreas/patologia , Pâncreas/cirurgia , Neoplasias Pancreáticas/patologia , Pancreaticoduodenectomia/métodos , Estudos Retrospectivos , Esplenectomia/métodos , Resultado do Tratamento , Carga Tumoral , Conduta Expectante
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