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PURPOSE: We explored factors affecting gastric emptying in neurologically impaired (NI) patients using the 13C-acetate breath test. METHODS: Twenty-four NI patients were classified by the presence of gastroesophageal reflux disease (GERD), which was treated by fundoplication plus gastrostomy, or the absence of GERD, which was treated by gastrostomy alone, along with gastric malposition involving cascade stomach and organoaxial gastric volvulus (OGV). Gastric emptying parameters (GEPs), which were the emptying half time (T 1/2 , minute), the lag phase time (T lag , minute), and the gastric emptying coefficient (GEC), were measured before and after surgery. We evaluated the relationship between GEPs and GERD, gastric malposition, and surgical intervention. All data were expressed as the median (interquartile range). RESULTS: The T1/2 and GEC of patients with OGV were significantly worse than in those without OGV before surgery (T1/2 with OGV: 241.3 [154.9, 314.3] vs. T1/2 without OGV: 113.7 [105.2, 151.4], p = 0.01, GEC with OGV: 3.19 [2.46, 3.28] vs. GEC without OGV: 3.65 [3.24, 3.90], p = 0.02). GERD and cascade stomach were not associ ated with GEPs. The GEPs of all NI patients showed no significant difference between before and after surgery. The surgical change in T1/2 (ΔT 1/2 ) in the patients with OGV was significantly lower than in those without OGV (ΔT1/2 with OGV: -47.1 [-142.7, -22.1] vs. ΔT1/2 without OGV: -3.78 [-26.6, 12.0], p = 0.03). CONCLUSION: Stomach malposition, such as OGV, seems to affect gastric emptying and may be improved by surgi cal intervention.
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Testes Respiratórios , Esvaziamento Gástrico , Refluxo Gastroesofágico , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Refluxo Gastroesofágico/fisiopatologia , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/cirurgia , Idoso , Isótopos de Carbono , Volvo Gástrico/fisiopatologia , Volvo Gástrico/cirurgia , Volvo Gástrico/diagnóstico , Acetatos , Estômago/fisiopatologia , Estômago/cirurgia , Gastrostomia , Fundoplicatura , Adulto , Doenças do Sistema Nervoso/fisiopatologia , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/complicaçõesRESUMO
BACKGROUND: Duplication of the alimentary tract can occur in any of its parts. For duodenal duplication, complete resection is particularly difficult when the ampulla of Vater is on the duplicated lumen and a deliberate management is necessary. CASE PRESENTATION: A 0-day female baby was referred to our department due to abdominal distention. The X-ray examination showed dextrocardia and opacity of the liver on the left side and abdominal ultrasonogram revealed remarkable intestinal dilatation. Therefore, urgent laparotomy was performed on the day of birth. Complete situs inversus of the abdominal organs was revealed, and the origin of the jejunum was on the left side and was accompanied by tubular intestinal duplication. The origin of the duplicated intestine was at the pancreatic head's dorsal area. There were two points of type Шa atresia on the ileum. Therefore, we spared the duplicated intestine with a length of 3 cm to secure the passage of the biliary and pancreatic juices by a functional-side-to-side anastomosis with a 45-mm Endo-GIA™ camel load (Medtronic, Minneapolis, MN, USA). The ileum was transected at the distal side of the atresia point, and end-to-end jejunoileostomy was performed. Postoperative gastrointestinal series revealed remnants of the duplicated alimentary tract on the dorsal area of the stomach. CONCLUSIONS: Identifying the position of the ampulla of Vater is crucial in the surgery of alimentary tract duplication with duodenal involvement. However, in the present case, it was difficult to identify whether the ampulla of Vater was on the true or duplicated lumen, and we had to spare the duplicated duodenum. Stapler anastomosis could be performed safely even in neonatal cases.
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BACKGROUND: A questionnaire survey was conducted to clarify whether surgical treatments performed by a pediatric surgeon improve the quality of life (QOL) of caregivers (medical doctors, nurses, physical therapists, and nursery teachers) supporting neurologically impaired (NI) patients who work at specialized institutions. METHODS: The questionnaire survey was conducted with caregivers who cared for NI patients who underwent an operation at the Department of Pediatric Surgery, Kurume University Hospital. Quality-of-life related responses concerning the change of care after surgical procedures in NI patients were collected in addition to the background data related to caregivers and the surgical procedures performed for them. Degrees of difficulty in caring for NI patients and of the caregivers' own QOL postoperatively were also compared. RESULTS: There were 192 valid responses. The majority of responses were from females and nurses aged in their 40s and 50s. The surgical procedure performed most often in the NI patients was gastrostomy. Of the respondents, 77% answered that the postoperative care became easier. The degrees of difficulty in caring for NI patients and the caregivers' own QOL were significantly higher and lower, respectively, among physical therapists compared with caregivers in other occupations. Moreover, the QOL in respondents with factors related to a longstanding relationship with NI patients was significantly higher compared with the respondents without them. CONCLUSION: This survey indicated that surgical procedures for NI patients might contribute to improved QOL for the caregiver by reducing the difficulty of caring for NI patients.
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Cuidadores , Qualidade de Vida , Feminino , Criança , Humanos , Idoso , Inquéritos e QuestionáriosRESUMO
BACKGROUND Difficult tracheal intubation (DTI) is common in patients with rheumatoid arthritis (RA) because of the subluxation of atlas and axis, the fusion of the cervical spine as a result of arthritis. We report a case of negative-pressure pulmonary edema (NPPE) caused by DTI in a patient with juvenile RA (JRA) who underwent surgery for spigelian hernia. CASE REPORT A 35-year-old man was referred to our department for repeated abdominal pain and a left-lower quadrant mass. Spigelian hernia was diagnosed with abdominal computed tomography (CT), and surgery was scheduled. Despite careful preoperative preparation and intubation strategy, fiber-optic intubation and laryngeal mask ventilation failed; nasal fiber-optic tracheal intubation was subsequently successfully performed. During induction, upper airway obstruction caused NPPE. CT findings showed bilateral infiltration and diffuse ground-glass opacity suggestive of pulmonary edema. Surgery for the spigelian hernia was canceled as the patient required intensive care as a result of NPPE. After 48 h of initiating treatment, the patient's respiratory condition gradually improved. Seven days after DTI, he underwent laparoscopy-assisted surgery for the spigelian hernia. The patient was discharged after 2 weeks of hospitalization. Four years have passed since the surgical procedure; the outcome has remained favorable and there has been no recurrence. CONCLUSIONS Normal ventilation may be the most important factor for preventing NPPE. It is vital that patients with RA receive treatment in an environment with advanced airway equipment and staff fully trained in its use. Similarly, the necessary staff and equipment for emergency cricothyroidotomies should also be readily available.
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Artrite Juvenil , Hérnia Ventral , Edema Pulmonar , Adulto , Artrite Juvenil/complicações , Vértebras Cervicais , Hérnia Ventral/cirurgia , Humanos , Intubação Intratraqueal , Masculino , Edema Pulmonar/etiologiaRESUMO
PURPOSE: A nationwide survey was conducted to investigate the current status of pediatric gastroesophageal reflux disease (GERD) in Japan, with special reference to the characteristics of intractable GERD. METHODS: Data were collected using a questionnaire from facilities specializing in the treatment of pediatric GERD in Japan. Intractable GERD was defined as follows: I, no symptomatic improvement after 8 weeks of optimal medical treatment (OMT) plus fundoplication; II, no symptomatic improvement after 8 weeks of OMT and with no indications for fundoplication; and III, no symptomatic improvement after surgery without OMT. RESULTS: We collected data from 3,463 pediatric patients with GERD from 91 institutions, and 81 satisfied the definition of intractable GERD. Additional clinical information was obtained from 56 patients, and 41 represented cases of definite intractable GERD. The main underlying disorders included neurological impairment (NI), esophageal atresia (EA), and congenital heart disease (CHD), which altogether accounted for 85% of patients. Of the 41 patients, 33 received fundoplication, and the remaining 8 received medical treatment alone because surgery was considered unsuitable. CONCLUSIONS: The nationwide survey revealed that pediatric intractable GERD is rare in Japan. Three main underlying disorders-NI, EA, and CHD-were implicated in the majority of cases of intractable GERD.
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Atresia Esofágica , Refluxo Gastroesofágico , Cardiopatias Congênitas , Laparoscopia , Criança , Atresia Esofágica/cirurgia , Fundoplicatura , Refluxo Gastroesofágico/cirurgia , Refluxo Gastroesofágico/terapia , Cardiopatias Congênitas/cirurgia , Humanos , Japão/epidemiologia , Inquéritos e Questionários , Resultado do TratamentoRESUMO
BACKGROUND: Excessive belching is frequently reported in adult patients with gastro-oesophageal reflux disease (GORD) and dyspepsia. Although postprandial gastric belching (GB) is considered a physiological mechanism for gastric venting, supra-gastric belching (SGB) is considered a distinct behavioural disorder. We aimed to define the prevalence of different types of belching and its association with reflux disease in paediatric patients. METHODS: We retrospectively analysed reflux monitoring studies from 287 patients (median age: 7.0 years; interquartile range 3.0-11.3 years) with a suspicion of GORD. Based on oesophageal acid exposure time (AET) patients were divided in 3 groups: (a) physiological AET, (b) borderline AET and (c) pathological AET. MII-pH studies were manually edited and reflux disease parameters were measured together with quantification of GB and SGB. KEY RESULTS: Two hundred one children (70.0%) had physiological AET (median: 0.8; interquartile range 0.3-1.6), 52 (18.1%) had borderline AET (median: 4.2; interquartile range 3.4-5.3) and 34 (11.9%) had pathologic AET (median: 10.7; interquartile range 9.1-14.2). Gastric belching was observed in all studies. Gastric belching related reflux was more frequently observed in patients with borderline and pathological AET (p < 0.001). This was more common in older children. SGB were observed in only 7 (2.4%) children (age range: 8-17years) in our population and all patients had Physiological AET. Only 3 (1%) patients had pathological number of SGB (>13/24 h). CONCLUSIONS AND INFERENCES: Gastric belching related reflux is observed in children with increased AET. SGB is very rare in the paediatric population.
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Eructação/epidemiologia , Refluxo Gastroesofágico/epidemiologia , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Masculino , PrevalênciaRESUMO
BACKGROUND: Development of gastroesophageal reflux disease (GERD) after gastrostomy remains debatable. Therefore, this study aimed to evaluate whether laparoscopy-aided gastrostomy (LAG) influence on the occurrence of GERD in neurologically impaired (NI) patients. Furthermore, we investigated whether preoperatively excluding NI patients with GERD can reduce the number of patients requiring subsequent anti-reflux surgery (ARS) after LAG. METHODS: This retrospective study included 35 NI patients (median age: 11.0; interquartile range 5.0-23.5 years) who underwent LAG according to our criterion from October 2012 to June 2020 and MII-pH before and after LAG. MII-pH parameters were compared in all patients and among three age groups between before and after LAG. RESULTS: There were no significant differences in MII-pH parameters before and 1 year after LAG in all patients, and no patient underwent subsequent ARS. Only one paediatric patient with 64 number of reflux episodes before LAG required subsequent ARS 3 years after LAG. CONCLUSIONS: Generally, LAG did not influence the postoperative GERD at 1 year after LAG, and our criteria could reduce the number of patients requiring subsequent ARS. However, paediatric NI patients with higher number of reflux episodes in preoperative MII-pH study may need careful long-term follow-up after LAG.
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Refluxo Gastroesofágico , Laparoscopia , Adolescente , Adulto , Criança , Pré-Escolar , Impedância Elétrica , Monitoramento do pH Esofágico , Refluxo Gastroesofágico/complicações , Refluxo Gastroesofágico/cirurgia , Gastrostomia/efeitos adversos , Humanos , Concentração de Íons de Hidrogênio , Laparoscopia/efeitos adversos , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Apart from Kasai's procedure, liver transplantation (LTx) has dramatically improved the outcome of children with biliary atresia (BA). However, de novo malignancy has been reported to be one of the major causes of late mortality after LTx among adults. We report a rare case of de novo gastric cancer developing after LTx for BA received during childhood. CASE PRESENTATION: A 21-year-old male patient who had undergone LTx for BA at age 2 years occasionally visited our outpatient clinic due to symptoms of epigastric pain and dysphagia. Endoscopic examination and computed tomography revealed advanced gastric cancer at the gastroesophageal junction with multiple liver metastases. Despite systemic chemotherapy, the disease progressed, resulting in patient's death 2 years after the diagnosis. CONCLUSIONS: De novo malignancy in the absence of post-transplant lymphoproliferative disease is rare in pediatric patients who received LTx. To the best of our knowledge, no report has been available on the development of gastric cancer after LTx for BA during childhood. Primary physicians should therefore establish a follow-up plan for patients receiving LTx for BA considering the potential for the development of de novo malignancy, including gastric cancer, despite its rarity.
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BACKGROUND/AIMS: This study aims to evaluate the presence of laryngopharyngeal reflux (LPR) and to investigate the use of hypopharyngeal baseline impedance (BI) for assessing swallowing dysfunction and gastroesophageal reflux disease (GERD) using hypopharyngeal multichannel intraluminal impedance and pH (HMII-pH) monitoring in neurologically impaired patients (NIPs). METHODS: The study population in this retrospective study comprised 20 NIPs (mean age, 36.1 ± 15.0 years; age range, 13-64 years) who underwent multichannel intraluminal impedance and pH (MII-pH), HMII-pH, and laryngoscopy using the Hyodo scoring method from December 2016 to April 2019. The MII-pH and HMM-pH parameters were compared in the NIPs, whereas hypopharyngeal BI values were compared between NIPs with ≥ 5 and < 5 in Hyodo scores. Correlations between the hypopharyngeal BI values and the Hyodo score were analyzed using Spearman's correlation coefficient. A receiver operator characteristic curve was created to determine the optimum cut-off of hypopharyngeal BI value to discriminate SD. RESULTS: Three NIPs were diagnosed with pathological LPR and GERD by the HMII-pH monitoring. No significant differences in parameters were observed between MII-pH and HMII-pH monitoring. The correlation analysis demonstrated a significant negative correlation between the hypopharyngeal BI values and Hyodo scores. The optimal cutoff value for hypopharyngeal BI was 1552 Ω. CONCLUSIONS: This study demonstrated the usefulness of HMII-pH monitoring in identifying NIP with pathological LPR. Considering the difficulties in performing examinations in NIPs, HMII-pH monitoring may be a potentially useful technique for the simultaneous evaluation of swallowing dysfunction, LPR, and GERD in NIP.
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A 7-year-old girl was referred to our hospital with a suspected right-sided indirect inguinal hernia. An egg-sized elastic, non-painful mass was palpated in the right inguinal region. Preoperative CT demonstrated a 30-mm simple cystic mass in the right internal inguinal canal, which we diagnosed as an abdominal cyst of the canal of Nuck. Laparoscopy revealed that the abdominal cystic component was ruptured, so we performed Pott's procedure. However, the patient's right groin swelled on postoperative day 3, necessitating re-operation. The patient's symptoms recurred again after 3 months, despite having had two surgical repairs. In the third operation, a right-sided femoral hernia was confirmed and repaired via external and laparoscopic approaches. To the best of our knowledge, there have been no reports on the combination of femoral hernia and cyst of the canal of Nuck in children. A laparoscopic approach was useful for obtaining a definitive diagnosis and conducting a safe and secure surgical repair.
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Cistos , Hérnia Femoral , Laparoscopia , Doenças Peritoneais , Criança , Cistos/complicações , Cistos/diagnóstico , Cistos/cirurgia , Feminino , Hérnia Femoral/complicações , Hérnia Femoral/diagnóstico , Hérnia Femoral/cirurgia , Humanos , Canal Inguinal/anormalidades , Canal Inguinal/cirurgia , Doenças Peritoneais/complicações , Doenças Peritoneais/diagnóstico , Doenças Peritoneais/cirurgia , Recidiva , Reoperação , Ruptura EspontâneaRESUMO
Oral nutritional supplements (ONS) are multi-nutrient products used to increase the energy and nutrient intakes of patients. The aim of this study was to examine whether or not the adherence of patients varies according to their receiving prescription or over-the-counter ONS. Data were obtained from an online cross-sectional survey conducted with patients in Japan. A total of 107 patients who matched the inclusion criteria for the prescription ONS group and 148 who matched the criteria for the over-the-counter ONS group were further analyzed. In the prescription and over-the-counter ONS groups, the main medical reason for ONS consumption were "malnutrition" (48 patients [44.9%] vs. 63 patients [42.6%] p = 0.798], "frailty" (29 patients [27.1%] vs. 36 patients [24.3%] p = 0.663) and "aging" (25 patients [23.4%] vs. 30 patients [20.3%] p = 0.644). The proportion of "No particular disease" for prescription ONS consumption was significantly lower than that for over-the-counter ONS (6 patients [5.6%] vs. 24 patients [16.2%] p = 0.001). The body mass index of the prescription ONS group was significantly higher than that of the over-the-counter ONS group (21.1±4.38 kg/m2 vs. 19.9±3.75 kg/m2, p = 0.0161). In the prescription ONS group, all patients were given medical advice by doctors or registered dietitians. In contrast, in the over-the-counter ONS group, only 46 patients (31.1%) were given advice by doctors or registered dietitians (p<0.001). In the prescription ONS group, ONS was taken significantly more times and for a longer duration than in the over-the-counter ONS group (p<0.0001). However, among patients given advice by doctors or registered dietitians, there were no significant differences between the groups. Greater support by the medical team is still needed in order to maximize adherence to supplementation, especially concerning the calories, timing and period, so that benefits can be achieved and sustained.
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Suplementos Nutricionais/estatística & dados numéricos , Desnutrição/tratamento farmacológico , Pacientes Ambulatoriais/estatística & dados numéricos , Cooperação do Paciente/estatística & dados numéricos , Administração Oral , Idoso , Estudos Transversais , Prescrições de Medicamentos/estatística & dados numéricos , Feminino , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Medicamentos sem Prescrição/administração & dosagem , Medicamentos sob Prescrição/administração & dosagem , Inquéritos e Questionários/estatística & dados numéricosRESUMO
BACKGROUND: Biliary atresia (BA) cases are generally not associated with congenital abnormalities. However, accurate diagnosis of BA is often challenging because the histopathological features of BA overlap with those of other pediatric liver diseases and rarely overlap with those of other genetic disorders. We experienced a rare case of BA with the histopathological finding of bile duct paucity, a gene mutation in KDM6A, and KS-like phenotypes. CASE PRESENTATION: A male baby was diagnosed with biliary atresia by intraoperative cholangiography at 4 days of age, and histological examination following a liver biopsy revealed a paucity of bile ducts and several typical clinical findings of Alagille syndrome. However, Alagille syndrome was ruled out after neither JAG1 nor NOTCH2 gene mutations were identified. Whole-exome sequencing on DNA from his parents was additionally performed to examine other possible syndromic disorders, and a mutation was identified in KDM6A. However, Kabuki syndrome was not diagnosed as a result. The histological finding of interlobular bile duct paucity and the genetic mutation in KDM6A, as well as several clinical findings consistent with Alagille syndrome or Kabuki syndrome, made it difficult to confirm the diagnosis of BA. CONCLUSIONS: Based on the interesting findings of the present case, we hypothesized that KDM6A is associated with hepatic malformations via a connection with the Notch signaling pathway.
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BACKGROUND: The present study aimed to evaluate whether the detailed observation of pH/MII waveforms and the analysis of baseline impedance (BI) values could detect esophageal dysmotility in pediatric patients with esophageal disorders. PATIENTS AND METHODS: Eleven patients with congenital esophageal disorder in whom pH/MII was conducted from April 2011 to June 2015, were enrolled in this study. The diagnoses of the patients were as follows: postoperative esophageal atresia (EA), n = 6; esophageal achalasia (EAch), n = 4; and congenital esophageal stenosis (CES), n = 1. The characteristics of the pH/MII waveform, pathological GERD, BI value, and the average BI value of the 2 distal channels (distal BI; DBI) were analyzed in each disorder. RESULTS: Two EA (33%) patients and one EAch (25%) patient were diagnosed with GERD. The mean DBI values of the EA, EAch and CES patients was 912 ± 550, 2153 ± 915 and 1392 Ω, respectively. The EA patients showed consistently low DBI values. One CES patient and two infantile EAch patients showed postprandial prolonged low DBI values. Whereas, the pH/MII waveforms of the adolescent EAch patients were difficult to interpret due to their extremely low BI values. CONCLUSIONS: The present study demonstrated that the detailed observation of the pH/MII waveforms in all channels and the analysis of BI were useful for evaluating esophageal motility in children with congenital esophageal disorders. In particular, infantile patients with EAch showed DBI findings that were distinct from those of adult EAch patients. Considering the difficulty of performing esophageal manometry in young children, the detailed observation of the pH/MII waveform may help in the diagnosis of esophageal dysmotility in children.
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Transtornos da Motilidade Esofágica/diagnóstico , Adolescente , Criança , Pré-Escolar , Impedância Elétrica , Atresia Esofágica/diagnóstico , Atresia Esofágica/fisiopatologia , Transtornos da Motilidade Esofágica/congênito , Transtornos da Motilidade Esofágica/fisiopatologia , Estenose Esofágica/diagnóstico , Estenose Esofágica/fisiopatologia , Monitoramento do pH Esofágico/métodos , Feminino , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/fisiopatologia , Motilidade Gastrointestinal/fisiologia , Humanos , Concentração de Íons de Hidrogênio , Lactente , Masculino , Período Pós-Prandial/fisiologiaRESUMO
INTRODUCTION: Resting energy expenditure (REE) is expected to be lower in with severe motor and intellectual disabilities (SMID) patients than in healthy subjects because of their relatively low fat-free mass (FFM). Therefore, an REE predictive equation for SMID patients may be required. The aim of this study was to validate existing REE predictive weight-based equations (Harris-Benedict, WHO, Mifflin, Owen, Schofield) and FFM-based REE equations (Mifflin, Owen and Cunningham) and to develop a new SMID patient-specific FFM-based REE equation. METHODS: Twenty-eight (22 males, 6 females) SMID patients over 18â¯years of age were included. The REE was measured using indirect calorimetry. FFM were measured using bioelectrical impedance analysis. A multiple linear regression analysis was used to develop a new FFM-based REE predictive equation. The accurate predictions compared the measured REE and root mean square error. RESULTS: The median measured REE was 950 (25th,75th percentile:712.75, 1102.75) kcal/day. The new FFM-based equation was as follows: REE (kcal/day)â¯=â¯550.62â¯+â¯16.62 FFM (kg). The new FFM-based REE resulted in the highest percentage of accurate predictions within 10% of measured REE (42.9%). The root mean square errors were the smallest for the new FFM-based REE and largest for Harris-Benedict (91.00 and 185.22â¯kcal/day). CONCLUSION: For SMID patients, the REE cannot accurately be predicted using the existing weight-based REE equations. Furthermore, the existing FFM-based REE equations are less accurate with regard to the measured REE than the new FFM-based REE equation. The new FFM-based equation is advised for use in SMID patients.
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Metabolismo Energético/fisiologia , Previsões/métodos , Deficiência Intelectual/fisiopatologia , Adulto , Composição Corporal , Índice de Massa Corporal , Peso Corporal , Calorimetria Indireta/métodos , Impedância Elétrica , Feminino , Humanos , Modelos Lineares , Masculino , Atividade Motora/fisiologia , Valor Preditivo dos Testes , Análise de Regressão , Adulto JovemRESUMO
INTRODUCTION: Lipoblastoma is a rare benign soft tissue tumor that occurs most commonly in infants and children. However, retroperitoneal lipoblastomas are rare, occurring in <5% of cases. We report a case of large retroperitoneal lipoblastoma and the largest collection of known retroperitoneal lipoblastomas in children in the literature. CASE PRESENTATION: A 3-year-old girl presented with left abdominal mass. Magnetic resonance imaging (MRI) revealed a soft tissue mass measuring 12â×â8â×â6âcm in the retroperitoneal region. The mass had a clearly defined margin and a reticular pattern with an interposing fat component. Based on these findings, the mass was suspected to be a soft-tissue tumor, most likely lipoblastoma.Laparotomy with resection of the retroperitoneal mass was performed. The tumor was easily dissected from the retroperitoneal space without injury to surrounding structure.A histopathological examination demonstrated the mature proliferation of adipocytes and spindle-shaped cells separated by fibrovascular septa accompanied by myxoid changes. The cells were separated into lobules by septa, and areas of immature adipocytes showing a signet-ring or multivacuolar appearance were present at the periphery. Histopathological diagnosis was lipoblastoma. Follow-up at 6 months revealed no evidence of recurrence. CONCLUSION: Retroperitoneal lipoblastoma is rare and tends to be large in size when diagnosed at presentation. Complete resection should not be delayed, as impingement on the surrounding structures is imminent.
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Lipoblastoma/patologia , Neoplasias Retroperitoneais/patologia , Pré-Escolar , Feminino , Humanos , Carga TumoralRESUMO
BACKGROUND: The present study aimed to evaluate the characteristics associated with a number of reflux episodes (NoRE) of 70 by comparing the clinical and multichannel intraluminal impedance pH measurements (pH/MII) and pH index (pHI) in neurologically impaired (NI) children. PATIENTS AND METHODS: NI children (1-16 years of age) in whom pH/MII had been measured for GERD study were enrolled in this study. All children were divided into NoRE >70 or ≤70 and pHI >4.0 or ≤4.0, >5.0 or ≤5.0 or >7.0 or ≤7.0. In addition, the NI children with pHI >4.0, >5.0 and >7.0 were subdivided into NoRE >70 and ≤70 groups. The clinical and pH/MII measurements were compared between each of the two groups. The cutoff values of pHI and baseline impedance (BI) (Z6) were calculated to discriminate NoRE >70 and ≤70. RESULTS: A total of 61 NI children were enrolled in this study. There was a significant difference in the acid-related parameters, the NoRE (nonacid) and BI between NoRE >70 and ≤70, acid-related parameters and BI between pHI >4.0 and ≤4.0, >5.0 and ≤5.0 and >7.0 and ≤7.0 groups. Furthermore, a significant difference was still observed in the BI between NoRE >70 and ≤70 groups among patients with pHI >4.0, >5.0 or >7.0. The cutoff values of pHI and BI (Z6) for discriminating NoRE >70 and ≤70 were 9.2 and 1049Ω, respectively. CONCLUSION: The present study indicates that NoRE 70 corresponds to GERD in which patients suffer severe acid exposure with pH of around 9% and esophageal mucosal damage with low BI value in NI children.
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Monitoramento do pH Esofágico/instrumentação , Esôfago/fisiopatologia , Refluxo Gastroesofágico/diagnóstico , Adolescente , Criança , Pré-Escolar , Impedância Elétrica , Monitoramento do pH Esofágico/métodos , Feminino , Refluxo Gastroesofágico/fisiopatologia , Humanos , Concentração de Íons de Hidrogênio , Lactente , Masculino , Curva ROC , Estudos RetrospectivosRESUMO
BACKGROUND: Anterior sagittal anorectoplasty (ASARP) has been a standardised operative treatment for anorectal malformation (ARM). This retrospective study was undertaken to evaluate patients with perineal fistula (PF) and vestibular fistula (VF) treated by ASARP in our institution. PATIENTS AND METHODS: Twenty patients (PF, n = 14; male, n = 8 and female, n = 6 and VF, n = 6) were evaluated. Eighteen patients underwent primary ASARP without protective colostomy. Two patients underwent colostomy because of intestinal atresia and suspected of other type ARM. The age range of operation was from 4 months to 5.0 years. Sixteen patients (PF, n = 13; male, n = 7 and female n = 6 and VF, n = 3) over 3 years of age were evaluated according to the Krickenbeck classification. RESULTS: Operative complications occurred in one patient. Minor wound dehiscence occurred in six patients. Mucosal prolapse occurred in two patients. According to the Krickenbeck classification, amongst male patients with PF, all patients had voluntary bowel movements (VBMs) and two patients had Grade 1 soiling, while four patients had Grade 2 constipation. Amongst female patients with PF, all patients had VBM and no soiling, one patient had Grade 1 and two patients had Grade 2 constipation. In patients with VF, one patient was continent with Grade 1 soiling. One patient had Grade 2 and two patients had Grade 3 constipation. CONCLUSIONS: ASARP without colostomy carried a risk of wound dehiscence. The ASARP technique provided normal or moderate outcomes for VBM and soiling. However, in about half of patients, defecation management with laxative therapy was required to achieve a normal condition.
Assuntos
Canal Anal/cirurgia , Malformações Anorretais/cirurgia , Defecação/fisiologia , Períneo/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Fístula Retal/cirurgia , Reto/cirurgia , Malformações Anorretais/fisiopatologia , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Períneo/anormalidades , Fístula Retal/congênito , Fístula Retal/fisiopatologia , Estudos RetrospectivosRESUMO
BACKGROUND/AIMS: The aim of this study is to investigate the degree of delayed gastric emptying (DGE) and evaluate how the severity of DGE affects gastroesophageal reflux disease (GERD) in neurologically impaired (NI) patients utilizing 24-hour multichannel intraluminal impedance pH measurements (pH/MII) and 13C-acetate breath test (13C-ABT) analyses. METHODS: 13C-ABT and pH/MII were conducted in 26 NI patients who were referred to our institution due to suspected GERD. At first, correlation analyses were performed to investigate the correlation between the 13C-ABT parameters and the clinical or pH/MII parameters. Thereafter, all patients were divided into 2 groups (DGE and severe DGE [SDGE] group) according to each cut off half emptying time (t1/2, 90-170 minutes). Each pH/MII parameter was compared between the 2 groups in each set-up cutoff t1/2. RESULTS: The mean t1/2 of all patients was 215.5 ± 237.2 minutes and the t1/2 of 24 (92.3%) patients were > 100 minutes. Significant moderate positive correlations were observed between both t1/2 and lag phase time and the non-acid reflux related parameters. Furthermore, the patients in the SDGE group demonstrated higher non-acid reflux related parameters than those of the DGE groups when the cutoff was t1/2 ≥ 140 minutes. CONCLUSION: The present study demonstrated that GE with t1/2 ≥ 140 minutes was related to an increase of non-acid exposure reaching up to the proximal esophagus in NI patients, and indicating that NI patients with SDGE might have a high risk of non-acid GERD.
RESUMO
PURPOSE: The aim of the present study was to determine the utility of measuring the salivary pepsin level (SPL) as an objective assessment of gastroesophageal reflux disease (GERD) in severe motor and intellectual disabilities (SMID) patients. SUBJECTS AND METHODS: This prospective study included 26 SMID patients who underwent simultaneous 24-h multichannel intraluminal impedance pH measurement (pH/MII) and SPL evaluation. The enrolled patients were divided into GERD (+) or GERD (-) groups according to the pH/MII findings. The age, gender and pH/MII parameters were compared between the two groups. A correlation analysis was also conducted for the SPL following early-morning fasting and post-enteral feeding and the age, gender, presence of gastrostomy and tracheostomy and pH/MII parameters. The SPL was compared between the two sampling groups. RESULTS: Fifteen patients were classified as GERD (+), and 11 patients were classified as GERD (-). The mean SPL following early-morning fasting and post-enteral feeding among all patients were 104.3 (median: 38, 25th and 75th percentile: 12, 361) ng/ml and 222.2 (median: 152:0, 500) ng/ml, respectively. Regarding positivity, 76.9% and 73.1% of SPL values in early-morning fasting and post-enteral feeding SMID patients, respectively, were positive (â§16ng/ml). The SPL following early-morning fasting demonstrated a weak but significant positive correlation with age. In contrast, we noted no correlation between the pH/MII parameters and the SPL for either the early-morning fasting or post-enteral feeding patients, and no significant difference in the SPL was observed between the GERD (+) and (-) patients. CONCLUSIONS: The present study showed that a high proportion of SMID patients had a relatively high SPL, regardless of the presence of GERD. The SPL in SMID patients might be affected by several distinctive factors in addition to gastroesophageal reflux.