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1.
Biomed Res Int ; 2023: 7624875, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37124932

RESUMO

Objectives: The current study is aimed at evaluating epidemiological characteristics and spatiotemporal distribution of cleft lip and/or palate (CL/P) in the south of Iran. Methods: Data were extracted from the 1840 medical records of patients who were referred to the Cleft Lip and Palate Center of Shiraz University of Medical Sciences, from January 1, 2011, to September 1, 2022. The collected variables included demographic data (gender, birth date and season, place, birth order, and weight), cleft types and the subtypes, parental information (health status, education level, marital status, and age during the pregnancy), and other basic parameters. The chi-square test at a significance level of 0.05 was used to analyze collected data. The geographic information system (GIS) analysis was also used for analyzing the spatial distribution of CL/P patients. Results: Based on our inclusion criteria, 1281 nonsyndromic patients were included in this study. The most common type was cleft lip and palate (CLP) with 48.32%, whereas cleft palate (CP) and cleft lip (CL) accounted for 40.75% and 10.93% of the patients, respectively. There was a progressive increase in the frequency of all types of clefts, and most of them were male (P ≤ 0.001). The urban population outnumbered the rural ones in all provinces. Parents were mostly healthy (>80%) with low educational status (47.5%). Most born CL/P patients were from consanguineous marriages (58.9%), especially between first-degree relatives. A majority of CL/P patients (73.1%) were born in the first two gestations with a birth weight of 2500-4000 g (77.4%). Most infants with CL/P (84.3%) were born from mothers who had at least one of the predisposing factors. Conclusion: In this study, the frequency of cleft types and subtypes was similar to the existing literature. However, high rate of consanguineous marriage, especially between first-degree relatives, was the most notable feature of this population.


Assuntos
Fenda Labial , Fissura Palatina , Fenda Labial/epidemiologia , Fissura Palatina/epidemiologia , Irã (Geográfico)/epidemiologia , Prevalência , Humanos , Estudos Retrospectivos , Análise Espaço-Temporal , Estudos Transversais , Masculino , Feminino , Lactente
2.
Am J Trop Med Hyg ; 105(5): 1222-1226, 2021 09 07.
Artigo em Inglês | MEDLINE | ID: mdl-34491224

RESUMO

Gastrointestinal basidiobolomycosis (GIB) is a rare, life-threatening fungal infection affecting immunocompetent individuals in tropical and subtropical regions. A diverse presentation of GIB has been reported, but no report has yet been published on intussusception. We describe a 23-month-old immunocompetent boy from a subtropical area in Iran who presented with intussusception. Prolonged fever, an abdominal mass, hepatomegaly, high erythrocyte sedimentation rate, and peripheral eosinophilia strongly suggested GIB. Accordingly, GIB was diagnosed based on the characteristic histopathology (the Splendore-Hoeppli phenomenon) detected in a liver sample taken via biopsy. Exploratory laparotomy showed several organs, including the colon, gall bladder, liver, and abdominal wall, were involved. Antifungal therapy with trimethoprim/sulfamethoxazole, liposomal amphotericin B, a saturated solution of potassium iodide, and surgical resection of involved tissues were used with improved outcome. The presence of non-septate fungal hyphal elements in the colonic mucosa led to the thickening of the bowel wall, leading to secondary intussusception.


Assuntos
Antifúngicos/uso terapêutico , Gastroenteropatias/tratamento farmacológico , Gastroenteropatias/cirurgia , Intussuscepção/tratamento farmacológico , Mucormicose/tratamento farmacológico , Mucormicose/cirurgia , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Gastroenteropatias/diagnóstico , Gastroenteropatias/microbiologia , Humanos , Lactente , Intussuscepção/diagnóstico , Intussuscepção/microbiologia , Intussuscepção/cirurgia , Irã (Geográfico) , Masculino , Mucorales/isolamento & purificação , Mucormicose/complicações , Mucormicose/diagnóstico , Resultado do Tratamento
3.
Diagn Pathol ; 16(1): 55, 2021 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-34162402

RESUMO

BACKGROUND: Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. METHODS: In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected. RESULTS: A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH. CONCLUSIONS: In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.


Assuntos
Hamartoma/patologia , Neoplasias Hepáticas/patologia , Neoplasias Embrionárias de Células Germinativas/patologia , Sarcoma/patologia , Adolescente , Biomarcadores Tumorais/metabolismo , Criança , Pré-Escolar , Humanos , Imuno-Histoquímica , Lactente , Fígado/patologia , Masculino , Neoplasias de Tecidos Moles/patologia
4.
World J Surg ; 45(6): 1812-1817, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33655360

RESUMO

BACKGROUND: The aim of this study was to correlate the connection between self-management behaviors and anthropometric indices after Roux-en-Y gastric bypass (RYGB). METHODS: A sample of 180 patients suffering from obesity were treated with RYGB in 2019; 6 and 18 months after surgery, anthropometric indices, including weight, waist circumference, waist- to-height ratio, body mass index (BMI), and waist-to-hip ratio, were gauged, and participants completed the post-bariatric surgery self-management behaviors questionnaire. RESULTS: Correlation matrix results showed that all anthropometric indices had a significant positive relationship with self-management behaviors after RYGB; the results of the 18-month post-surgical follow-up exhibited no significant difference between anthropometric indices and self-management behaviors, 6 and 18 months after surgery. CONCLUSION: Therefore, the more self-management behaviors increase, the greater decrease in anthropometric indices will happen after RYGB.


Assuntos
Cirurgia Bariátrica , Derivação Gástrica , Obesidade Mórbida , Autogestão , Índice de Massa Corporal , Humanos , Obesidade Mórbida/cirurgia , Redução de Peso
6.
Pediatr Surg Int ; 36(5): 603-610, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32206891

RESUMO

PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease characterized by cholestasis, which may cause jaundice, severe pruritus, and cirrhosis in the later stages. By the invention of biliary diversion methods, these patients were prevented from undergoing liver transplant. Using biliary diversion techniques, the entero-hepatic cycle was interrupted. This lowers the bile acid pool and resolves the pruritus. Herein, we report 44 cases of PFIC who underwent partial internal biliary diversion (PIBD) and long-term follow-up of these children. This comprises the largest case series of PIBD. METHODS: All patients were diagnosed by liver biopsy as PFIC before the operation. All underwent cholecysto colic bypass by jejunal interposition due to severe pruritus unresponsive to medication. Laboratory blood tests, sonography, and physical exam were done before and after the operation once every 3 months. Besides, a questionnaire was designed to ask the patients about the symptoms after the operation, and a pruritus score was measured using the 5D-itch scale. RESULTS: 44 children (25 boys, 19 girls), between 1.75 and 27.5 years (at the time of this study) were followed for a median period of 54 months. Age at operation ranged from 2 months to 18 years, with a median of 29 months. Of these children, 14 were lost to follow up. Results showed a significant decrease in pruritus and sleep disturbance after the surgery (p < 0.001). Also, jaundice decreased from 82.1 before to 7.1% following the surgery. 50% of the patients became medication-free at follow-up. CONCLUSION: PIBD is a safe procedure which helps non-cirrhotic children preserve their liver function. Therefore, PIBD prevents them from undergoing liver transplant. Effective results were achieved in terms of severe pruritus and jaundice, and children were able to regain their sleep patterns. It also avoided external stoma, which is more convenient from the patient's point of view.


Assuntos
Procedimentos Cirúrgicos do Sistema Biliar/métodos , Colestase Intra-Hepática/cirurgia , Vesícula Biliar/cirurgia , Adolescente , Adulto , Anastomose Cirúrgica/efeitos adversos , Biópsia , Criança , Pré-Escolar , Colestase Intra-Hepática/diagnóstico , Feminino , Seguimentos , Humanos , Lactente , Masculino , Inquéritos e Questionários , Fatores de Tempo , Resultado do Tratamento , Ultrassonografia , Adulto Jovem
7.
World J Plast Surg ; 8(1): 85-92, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30873367

RESUMO

BACKGROUND: Adherence has been defined as the degree to which a patient's voluntary behavior corresponds with the clinical recommendations of health care providers. The aim of this study was to predict self-management behaviors in obese patients undergoing surgery based on general and specific adherence scales. METHODS: All obese patients who underwent bariatric surgery in Ghadir Mother and Child Hospital, Shiraz, Iran from April 2017 to September 2017 were enrolled. By using available sampling method, 201 patients with BMI above 35 (36.43±35.11) and in the age range of18-65 years (13.38±80/5) were selected. A questionnaire containing general adherence scale (GAS), specific adherence scale (SAS) and post-surgery self-management behaviors questionnaire (BSSQ) was completed by all patients. RESULTS: The general and specific adherence scales were significant predictors for self-management behaviors after surgery, and positive relation was found for general and specific adherence scales with self-management behaviors. CONCLUSION: Self-management behaviors regarding eating behaviors, supplements, fruits, vegetables, grains, protein and fluid intake, physical activity, dumping syndrome management have significant relationship with general and specific adherence scales. So increasing knowledge, skills, motivation, self-confidence, self-efficacy and self-monitoring of obese patients after surgery seem necessary.

8.
Iran J Microbiol ; 8(5): 326-330, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28149493

RESUMO

BACKGROUND AND OBJECTIVES: Pneumocystis jiroveci is an important causative agent of Pneumocystis pneumonia. During childhood, exposure to Pneumocystis occurs and antibody was built in early childhood. The aim of this study was to describe the molecular epidemiology of P. jiroveci in children without any respiratory syndrome and survey the distribution of different mitochondrial large subunit, ribosomal ribonucleic acid (mtLSU- rRNA) genotypes. MATERIALS AND METHODS: Mini-bronchoalveolar lavage (mini-BAL) fluids from pediatric patients with no history of lung disorders were obtained during a 14-month period. P. jiroveci colonization was confirmed by immunofluorescence staining, nested PCR and sequencing. Genotypic characterization at the mitochondrial large subunit rRNA gene was performed by direct sequencing. RESULTS: Of 172 BAL specimens from patients, with mean age of 4.9 years, the prevalence of P. jiroveci colonization was 3.5% (6 samples). The results of sequencing revealed the two polymorphisms; 85/A; 248/C in 3 cases, and 85/T; 248/C in other cases. One sample also showed a mutation replacement at position 258 (T-to-C change), which was not reported previously. CONCLUSION: Colonized person as an environmental reservoir might play an important role in the progression of infection in immunocompromised patients. Diagnosis of the reservoir and genotyping can be essential for the prevention of nosocomial infections.

9.
Surg Endosc ; 30(7): 3107-13, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-26487229

RESUMO

BACKGROUND: The use of transanal laparoscopic access to completely avoid abdominal wall incisions represents the most current evolution in minimally invasive surgery. The combination of single-site surgery and natural orifice transluminal endoscopic surgery (NOTES™) can be used for totally transanal laparoendoscopic pull-through colectomy with J-pouch creation (TLPC-J). The aim of the present study was to provide evidence for the feasibility of TLPC-J in adult human cadavers. METHODS: TLPC-J was performed in six fresh adult human cadavers. The procedure involved endorectal submucosal dissection from 1 cm above the dentate line to a point above the peritoneal reflection, where the rectal muscle was divided circumferentially. The edge of the mucosal cuff was closed distally in order to prevent fecal contamination and the endorectal tube was placed back into the abdomen. A Triport+™ or QuadPort+™ system was introduced transanally, and it served as a multiport device (MD). Resection of the entire colon, mobilization of the distal ileal segment, and extracorporeal suture of the ileal J-loop were performed via the transanal approach. The J-pouch was created using Endo GIA™. After removal of the MD, the J-pouch was sutured to the rectal wall. RESULTS: TLPC-J was performed in all cadavers, with a mean operation duration of 236 ± 22 min. Conversion to either transabdominal laparoscopy or laparotomy was not required in any of the cadavers. No bowel perforation or damage to other organs was observed. The use of a curved endoscope greatly facilitated visualization during transanal laparoscopic dissection for partial and total colectomy, making the procedure feasible. All specimens were retrieved through the anus, eliminating the need for additional transabdominal incisions. CONCLUSIONS: TLPC-J was technically feasible in adult human cadavers, and abdominal wall incisions were not required. However, clinical studies are needed to determine its feasibility in living adults.


Assuntos
Traumatismos Abdominais/prevenção & controle , Colectomia/métodos , Laparoscopia , Cirurgia Endoscópica por Orifício Natural/métodos , Cavidade Abdominal , Parede Abdominal/cirurgia , Adulto , Cadáver , Feminino , Humanos , Doença Iatrogênica/prevenção & controle , Perfuração Intestinal/prevenção & controle , Masculino
10.
Arch Iran Med ; 19(1): 57-63, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26702750

RESUMO

PURPOSE: To evaluate the outcome of laparoendoscopic single-site (LESS-A) through one transumbilical port vs. 3-port laparoscopic (3TA) appendectomy in children. METHODS: We reviewed the records of 309 children (65 LESS-A, 244 3TA) operated on between 2008 and 2012. One hundered forty-nine patients had acute catarrhalis (CA), 133 phlegmonous (PLA), and 27 perforated appendicitis (PA). We compared the duration of operation (DO) the incidence of abdominal abscesses (AA) and wound infections (WI), as well as the degree of appendiceal inflammation (DI) among surgeons with and without board certification. RESULTS: For all DI, LESS-A resulted in a shorter DO than 3TA (CA 57.9 ± 22.8 vs. 68.5 ± 23.2, P = 0.014; PLA 51.5±16.5 vs. 68.4±33.0, P = 0.006; PA 66.0 ± 29.0 vs. 97.3 ± 41.8, P = 0.039). LESS-A was not used for less complicated cases when compared to 3TA (CA 50.8% vs. 47.5%; PLA 33.8% vs. 45.5%; PA 15.4% vs. 7.0%; CA vs. PLA, P = 0.292; CA vs. PA, P = 0.142; PLA vs. PA, P = 0.031). Surgeons without board certification were assigned to a similar percentage to perform both techniques for any DI (CA 30.3% vs. 37.1%, P = 0.541; PLA 31.8% vs. 40.5%, P= 0.484; PA 40% vs. 35.3%, P = 1.0). We found no significant differences concerning AA (1.5% vs. 1.2%, P = 1.0) and WI (3.1% vs. 1.6%, P = 0.61). CONCLUSIONS: LESS-A can be done by surgeons with and without board certification for all DI, with shorter DO and similar complication rates as compared to 3TA.


Assuntos
Apendicectomia/métodos , Apendicite/cirurgia , Laparoscopia/métodos , Duração da Cirurgia , Complicações Pós-Operatórias/epidemiologia , Cirurgiões/educação , Abscesso Abdominal/epidemiologia , Adolescente , Antibacterianos/administração & dosagem , Apendicite/tratamento farmacológico , Cefuroxima/administração & dosagem , Criança , Pré-Escolar , Feminino , Alemanha , Humanos , Lactente , Masculino , Metronidazol/administração & dosagem , Estudos Retrospectivos , Infecção da Ferida Cirúrgica/epidemiologia
11.
Iran J Med Sci ; 40(5): 425-9, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26379349

RESUMO

BACKGROUND: The natural orifice transluminal endoscopic surgery (NOTES) has become a commonly considered novel approach in the surgical field. The NOTES provide possibility of operation through the natural orifice and decreases the intentional puncture of the systemic organ and subsequent complications. Totally transanal laparo-endoscopic single-site proctoColectomy-Ileoanal J-Pouch (TLPC-J) is a novel method in minimally invasive surgery for total colectomy. The main goal of this study is to perform this new method on an animal model, to assess probable complication and to resolve probable issues by using patients that are candidate for total colectomy. METHOD: Five dogs were prepared in lithotomy position. The TLPC-I procedure consists of endorectal technique with full thickness rectal dissection starting 1 cm orally from the dentate line above the peritoneal reflection and the proximal bowel was replaced into the abdominal cavity. Afterwards, the TriPort system was inserted in the anal canal and mesentrial resection of the total colon, mobilization of a distal ileal segment and intracorporeal suture of an ileal J-loop was accomplished by this system. An incision in the J-loop was conducted transanally. The J-pouch was created with an Endo-GIA® and sutured to the rectal wall. RESULTS: All animals survived and passed stool with clear post operation situation. There was no infection in site of anastomosis. CONCLUSION: The TLPC-I provides the possibility of surgery without abdominal wall incision and decreases post operation complication such as pain, abdominal wound infection and wound dehiscence. This technique increases the quality of life and surgeons can discharge the patients early.

12.
Iran J Pediatr ; 25(2): e367, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26196001

RESUMO

BACKGROUND: Hirschsprung's disease (HD) is a congenital intestinal motility disorder with absence of ganglion cells in the colonic wall. Diagnosis of the disease is mainly based on the identification of the lack of ganglion cells in the pathology sections of the colon which is very difficult and time consuming and also needs several serial cut sections. There are many proposed markers in this field in the literature but none of them has been satisfactory. Calretinin immunohistochemistry (IHC) has been introduced as a new diagnostic marker to overcome the problems in diagnosis of this disease about 5 years ago. However there are few studies regarding the benefits and pitfalls of this marker. OBJECTIVES: The aim of this study is to determine the diagnostic value of calretinin IHC in detecting aganglionosis (HD). PATIENTS AND METHODS: 27 HD patients and 28 non-Hirschsprung's disease (NHD) patients were collected in a prospective study and calretinin IHC was performed on 31 aganglionic and 51 normoganglionic full wall thickness sections of colectomies (some of the cases had more than 1 section). The IHC slides were evaluated by two pathologists and the diagnostic value was calculated in comparison with gold standard which is the presence or absence of ganglion cells in serial Hematoxylin and Eosin (HE) stained sections of the colectomies. RESULTS: There was great concordance between the final diagnosis of both pathologists and gold standard (k > 0.9). Calretinin immunostaining showed 100% specificity and positive predictive value and more than 90% sensitivity and negative predictive value. High agreement was present between the two pathologists (k > 0.9). CONCLUSIONS: Calretinin IHC is a very convenient, useful and valuable method to demonstrate aganglionosis in HD patients. Loss of calretinin immunostaining in lamina propria and submucosa is characteristic of HD.

13.
J Laparoendosc Adv Surg Tech A ; 23(3): 276-80, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23402288

RESUMO

UNLABELLED: Abstract Introduction: Minimally invasive surgery in children with long-segment intestinal aganglionosis aims to reduce the number of abdominal wall incisions. Conventional laparoscopic and laparoendoscopic single-site (LESS) surgeries fulfill this goal. In children, natural orifice translumenal endoscopic surgery (NOTES™; American Society for Gastrointestinal Endoscopy [Oak Brook, IL] and Society for American Gastrointestinal and Endoscopic Surgeons [Los Angeles, CA]) has been limited because of fear of access site complications. We present a novel technique of totally transanal LESS pull-through colectomy (TLPC), avoiding abdominal wall incision, which combines LESS technology and the NOTES approach. SUBJECTS AND METHODS: Two boys and one girl (2.5 months, 6 months, and 5 years of age, respectively) with sigmoid and transverse colon aganglionosis underwent surgery. The TLPC procedure consisted of an endorectal technique with submucosal dissection starting 1 cm orally from the dentate line to above the peritoneal reflection, where the rectal muscle was divided circumferentially. After ligation of the rectal mucosa, the proximal bowel was replaced into the abdominal cavity, and a TriPort(®) (Olympus Surgical Technologies Europe, Hamburg, Germany) was introduced transanally. Mesenterial resection of the aganglionic bowel was accomplished via transanal LESS until the normoganglionic colon segment was reached and pulled down to the site of anastomosis. After removal of the port, a conventional pull-through procedure was performed. RESULTS: All children displayed normal bowel movements and were complication-free during the follow-up period of up to 7 months. CONCLUSIONS: TLPC combines the minimally invasive LESS surgery with the scarless concept of NOTES and allows resection of long-segment aganglionosis without abdominal incision. TLPC is a safe, effective, and feasible surgical procedure in children with long-segment intestinal aganglionosis.


Assuntos
Colectomia/métodos , Doença de Hirschsprung/cirurgia , Laparoscopia , Cirurgia Endoscópica por Orifício Natural , Canal Anal , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
14.
Histol Histopathol ; 28(3): 345-51, 2013 03.
Artigo em Inglês | MEDLINE | ID: mdl-23348388

RESUMO

BACKGROUND: Hirschsprung's disease (HD) is one of the most common motility disorders in pediatric age groups and it is very important that it be differentiated from other types of motility disorders, especially intestinal neuronal dysplasia B (IND B). Although many studies regarding the differences between the two disorders by immunohistochemical studies exist, there is as yet no consistent result. The purpose of this research was to study the immunohistochemical findings of enteric nervous system in these two motility disorders in comparison with colectomies without motility disorder. METHODS: Full wall thickness specimens of three groups of patients (HD, IND B and non motility disorders) were included in the study to be evaluated by immunohistochemistry (IHC). Markers were specific for neuronal cells and pace maker cells composed of PGP 9.5, c-kit, synaptophysin, S100 and CD56. The number of cells was evaluated in the muscularis properia, and myenteric plexus. RESULTS: The number of all the IHC markers i.e. PGP9.5, c-kit, synaptophysin, S100 and CD56 was completely different in HD from the two other groups, while IND B was quite similar to control group. CONCLUSION: Our finding suggests that there is a marked and significant difference between HD and IND B by IHC markers, which can be used as an additional test for the diagnosis of HD with more accuracy. Further multicenter studies with a greater number of cases would be necessary to find a cut-off point for every IHC marker to differentiate HD and IND B.


Assuntos
Doença de Hirschsprung/diagnóstico , Enteropatias/diagnóstico , Plexo Mientérico/patologia , Doenças do Sistema Nervoso/diagnóstico , Neurônios/patologia , Adolescente , Biomarcadores/metabolismo , Antígeno CD56/metabolismo , Criança , Pré-Escolar , Constipação Intestinal/diagnóstico , Constipação Intestinal/etiologia , Constipação Intestinal/metabolismo , Diagnóstico Diferencial , Feminino , Doença de Hirschsprung/complicações , Doença de Hirschsprung/metabolismo , Humanos , Imuno-Histoquímica/métodos , Lactente , Recém-Nascido , Células Intersticiais de Cajal/metabolismo , Células Intersticiais de Cajal/patologia , Enteropatias/complicações , Enteropatias/metabolismo , Masculino , Plexo Mientérico/metabolismo , Doenças do Sistema Nervoso/complicações , Doenças do Sistema Nervoso/metabolismo , Neurônios/metabolismo , Proteínas Proto-Oncogênicas c-kit/metabolismo , Proteínas S100/metabolismo , Sinaptofisina/metabolismo , Ubiquitina Tiolesterase/metabolismo
16.
Iran J Med Sci ; 36(1): 54-6, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23365480

RESUMO

Surgical glues have been used in pediatric surgery because of the fragility of tissue, and to prevent major surgeries. The present report describes our experience with using a new cyanoacrylate Glubran 2 (Viareggio, Italy) in the treatment of five cases of tracheoesophageal atresia with fistula (one fistula protection, three recurrent fistula, and one unstable patients), two cases of hypospadias, one case of vesicutanouse fistula after bladder extrophy, and one case of cloacal extrophy from January-December 2008. Three cases of recurrent tracheoesophageal atresia with fistula were treated by bronchoscpic glue injection. The other two cases benefited from glue through its ability to plug the fistula and to act as a protecting layer on anastomosis. In two cases with hypospadias excessive use of the glue caused skin necrosis, which was repaired. The wounds of cloacal extrophy were protected from nearby colostomy contamination and infection, and the vesicocutanouse fistula was closed by deepithelialization and sealing with glue. Based on the outcomes of the cases, it may be possible to suggest that Glubran 2 may be used safely in Pediatric Surgery as a sealant for the prevention and treatment of fistulas.

17.
Rare Tumors ; 3(4): e51, 2011 Oct 21.
Artigo em Inglês | MEDLINE | ID: mdl-22355506

RESUMO

Lipoblastoma is a rare tumor of infancy. It originates from the white fetal fat in soft tissue. The most common location of this rare tumor is extremity and to best of our knowledge less than 10 cases of intrathoracic and mediastinal lipoblastoma has been reported in the English literature. Herein we present our experience with a 15-month-old boy infant who presented with severe dyspnea. Imaging studies showed a mass in the thoracic cavity and mediastinum which was diagnosed as lipoblastoma after pathologic examination of the resected mass. Lipoblastoma has been considered as a tumor of soft tissue, but it should also be considered as a rare cause of intrathoracic masses of young children.

18.
Indian J Pathol Microbiol ; 53(3): 422-6, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20699496

RESUMO

BACKGROUND: Pediatric hepatic malignancies are rare, accounting for 1-4% of all solid childhood tumors. The histopathology of childhood hepatic tumors guides the treatment and prognosis, and is the cornerstone for precise diagnosis. Until now, there has been no documented study on pediatric liver tumor cases from this center; in this report, we show our experience about the common types of childhood hepatic tumors during five years (2002-2007) and compare them with other studies. MATERIALS AND METHODS: During five years (2002-2007), all the hepatic tumors of childhood (under 18 year-old) from the pathology file of Namazi Hospital of Shiraz University of Medical Sciences are recorded. This includes both resected specimens and biopsies. All the slides were reviewed and the pathologic diagnosis was confirmed. RESULTS: We detected 53 liver tumor cases in children (below 18 years of age). Among these tumors, 36 (67.9%) were malignant. Male to female ratio was 1.5 to 1. Hepatoblastoma was the most common liver tumor in this age group accounting for 22 patients (41.5%). The second most common primary tumor was hepatocellular carcinoma (HCC), with five patients. Another malignant tumor was embryonal sarcoma. Benign tumors included adenoma, mesenchymal hamartoma, vascular tumors, focal nodular hyperplasia, and inflammatory pseudo tumor. There were also seven metastatic tumors during these five years. CONCLUSIONS: The spectrum of hepatic tumors in children is different from that found in the older age group (adults) and also different in different populations.


Assuntos
Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/patologia , Adenoma/epidemiologia , Adenoma/patologia , Adolescente , Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/patologia , Criança , Pré-Escolar , Feminino , Hiperplasia Nodular Focal do Fígado/epidemiologia , Hiperplasia Nodular Focal do Fígado/patologia , Granuloma de Células Plasmáticas/epidemiologia , Granuloma de Células Plasmáticas/patologia , Hamartoma/epidemiologia , Hamartoma/patologia , Hepatoblastoma/epidemiologia , Hepatoblastoma/patologia , Humanos , Lactente , Irã (Geográfico)/epidemiologia , Neoplasias Hepáticas/secundário , Masculino , Prevalência , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/patologia , Distribuição por Sexo , Neoplasias Vasculares/epidemiologia , Neoplasias Vasculares/patologia
19.
Indian J Gastroenterol ; 27(3): 99-102, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18787278

RESUMO

OBJECTIVE: Our aim was to correlate the pathological results and clinical response in patients who underwent botulinum toxin (BT) injection for obstructive symptoms (OS) after a pullthrough operation for Hirschsprung's disease (HD). METHODS: Between August 2002 and February 2006, 16 of 107 HD patients (15%) were referred with persistent OS after pull-through (PT) operation in this center. They underwent rectal biopsy and BT injection in the internal sphincter. Their responses to BT injection were evaluated by the constipation score before, and at 1, 3 and 8 months after the injection, and anorectal manometry (ARM) before and at 2 weeks, and 1 and 8 months after the injection. The association between response to BT and acetylcholinesterase (AChE) staining of rectal biopsy was also assessed. RESULTS: Fourteen of 16 patients (87%) had improvement in bowel function after 2 weeks, and two patients did not respond at all. Six of the 14 patients with early response had recurrence of symptoms after 2-3 months. Eight patients with normal ganglia and negative AChE had good response with no recurrence on follow-up. However, 4 of 6 recurrences were neurogenic dysfunctions and 2 were intestinal neuronal dysplasia (2-4+AChE). Two patients with no response had an aganglionic segment (4+AChE). Four of 6 patients with recurrence showed improvement with BT re-injection and only 2 did not improve. CONCLUSION: A higher degree of AChE staining is associated with lack of response to BT injection. This is also a test for predicting the severity of neurogenic dysfunction in the intestinal wall.


Assuntos
Toxinas Botulínicas Tipo A/uso terapêutico , Doença de Hirschsprung/patologia , Obstrução Intestinal/prevenção & controle , Fármacos Neuromusculares/uso terapêutico , Complicações Pós-Operatórias , Reto/patologia , Acetilcolinesterase/metabolismo , Canal Anal , Biópsia , Criança , Pré-Escolar , Estudos de Coortes , Constipação Intestinal/etiologia , Constipação Intestinal/prevenção & controle , Feminino , Doença de Hirschsprung/enzimologia , Doença de Hirschsprung/cirurgia , Humanos , Injeções Intramusculares , Obstrução Intestinal/etiologia , Obstrução Intestinal/patologia , Masculino , Valor Preditivo dos Testes , Resultado do Tratamento
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