The Evaluation of Gastric Emptying Using the 13 C-Acetate Breath Test in Neurologically Impaired Patients - A Focus on the Stomach Function and Morphology.

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.

Risk Factors in Fetal Ovarian Cysts for Postnatal Adverse Outcomes.

AIM: To investigate the natural history of fetal ovarian cysts and elucidate the risk factors for postnatal adverse outcomes in fetal ovarian cysts. METHODS: The study subjects were 18 cases with ovarian cysts prenatally diagnosed using ultrasonography at our hospital between 2007 and 2020. The subjects were classified by cyst characteristics according to echogenic patterns [simple cyst (S) and complex cyst (C)], changes in echogenic patterns (S-to-S, S-to-C, and C-to-C), and diameters (<40 and ≥ 40 mm). Clinical parameters and outcomes were compared between S and C patterns, S-to-S and S-to-C patterns, and <40 and ≥ 40 mm diameters. RESULTS: Cases with S and C patterns (15 and 3, respectively) had median gestational ages of 35 and 36 weeks, respectively, and maximum cyst diameters of 36 and 57mm, respectively. The number of cases with S-to-S, S-to-C and C-to-C patterns were 11, 4 and 3, respectively. The maximum cyst diameter in cases with S-to-C patterns (58 mm) was larger than that in cases with S-to-S patterns (34 mm) (P<0.05). Placental weight in cases with cysts >40 mm and/or cyst expansion was greater than that in cases with neither or both conditions (P<0.05). Spontaneous resolution (before and after birth) occurred in 8 of 9 and 3 of 9 cases with maximum cyst diameters <40 and ≥ 40 mm, respectively. Ovarian function was lost in 2 cases with S-to-C patterns and in 2 cases with C-to-C patterns. CONCLUSION: Cases with cyst diameters ≥ 40 mm and/or cyst expansion during the late third trimester had greater placental weight and more postnatal adverse outcomes.

Quality of life of caregivers supporting neurologically impaired patients who underwent surgery.

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.

Negative-Pressure Pulmonary Edema After Difficult Endotracheal Intubation in a Patient with Juvenile Rheumatoid Arthritis Undergoing Spigelian Hernia Surgery: A Case Report.

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.

Influence of laparoscopy-aided gastrostomy on gastroesophageal reflux in neurologically impaired patients using multichannel intraluminal impedance pH measurements.

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.

Creatinine-to-cystatin C ratio estimates muscle mass correlating the markers of the patients with severe motor and intellectual disabilities.

AIM: The creatinine-to-cystatin C ratio (CCR) has been acknowledged as a potential marker of muscle mass. The purpose of the present study was to evaluate the relationship between CCR and nutritional status in a bioelectrical impedance analysis (BIA) of patients with severe motor and intellectual disabilities (SMID). METHODS: This study included 39 patients with SMID (17 males, 22 females) over 16 years of age were included retrospectively. CCR was calculated as serum creatinine (mg/dL)/cystatin C (mg/L) × 10. The BIA parameters such as the phase angle (PhA), fat free mass (FFM), appendicular skeletal muscle mass (ASM) and appendicular skeletal muscle mass index (ASMI) values were measured using BIA. Correlation analyses between CCR and the BIA parameters were conducted. RESULTS: The mean CCR is 4.47 ±â€¯1.34. Significant positive relationships between CCR and FFM, PhA, ASM, ASMI were identified (r = 0.3373, p = 0.0357. r = 0.4273, p = 0.0093. r = 0.5008, p = 0.0012. r = 0.4706, p = 0.0025 and r = 0.4751, p = 0.0022, respectively). CONCLUSIONS: The study indicated that CCR in the patients with SMID is a useful parameter that allows for the muscle mass to be estimated easily and accurately. This means that evaluating CCR could be used as a simple and important screening tool for PhA, FFM and muscle mass.

De novo gastric cancer developing after liver transplantation from deceased donor for biliary atresia: a case report.

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.

Simultaneous Evaluation of Laryngopharyngeal Reflux and Swallowing Function Using Hypopharyngeal Multichannel Intraluminal Impedance Measurements in Neurologically Impaired Patients.

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.

The herbal medicines Inchinkoto and Saireito improved hepatic fibrosis via aquaporin 9 in the liver of a rat bile duct ligation model.

OBJECTIVE: To determine if the administration of the Japanese herbal medicines Inchinkoto (ICKT) and Saireito (SRT) ameliorate hepatic fibrosis and derangement of hepatocyte aquaporins (AQPs) following bile duct ligation (BDL) in a rat model of obstructive cholestasis. MATERIALS AND METHODS: Five groups of Wistar rats were used, and the groups included sham surgery (Sham group), BDL with no treatment (NT group), BDL plus ICKT (ICKT group), BDL plus SRT (SRT group), and BDL plus ICKT and SRT (SRT/ICKT group). Each herbal medicine was administered at 1 g/kg/day on the first postoperative day. The serum levels and various clinical markers were measured with real-time polymerase chain reaction. Staining was used to evaluate the degree of fibrosis and the inflammatory responses. RESULTS: Serum aspartate aminotransferase and alanine aminotransferase in the ICKT and SRT/ICKT groups were significantly lower than those in the NT group. NF-κB mRNA expression was significantly decreased in the ICKT group and the SRT/ICKT group compared with the NT group. AQP9 mRNA expression was significantly increased in the ICKT group and the SRT/ICKT group compared with the NT group. The degree of Masson's trichrome staining in the SRT/ICKT group was significantly lower than that in the NT group. The degree of NF-κB staining in the SRT/ICKT group was significantly lower than that in the NT, ICKT, or SRT group. CONCLUSIONS: The postoperative administration of ICKT and SRT induced synergistic beneficial effects, resulting in the reduction of hepatic fibrosis via mechanisms involving the inhibition of NF-κB expression and the improvement of AQP9 downregulation.

Complications in patients with neurological impairment after gastrostomy.

BACKGROUND: Neurological impairment (NI) is responsible for most conditions that require a permanent gastrostomy tube. The present study assessed the occurrence of short- and long-term complications after video-assisted gastrostomy (VAG) in patients with NI. METHODS: The incidence of short- (<6 months) and long-term (over 2 years) complications of VAG were analyzed in a retrospective study. The differences between the incidence of the complications of VAG according to the age at surgery (≤15 years vs. ≥16 years) were also evaluated. The short- and long-term complications observed were granulation tissue formation, infection requiring antibiotic treatment, skin problems, perigastrostomy leakage, vomiting, accidental tube dislodgement, dumping syndrome, ileus, and peritonitis. RESULTS: Eighty-two patients were evaluated for short- and long-term complications. The long-term complication rate was significantly lower than the short-term complication rate (P = 0.0026). Onodera's prognostic nutritional index before VAG in patients with long-term complications was significantly lower than in patients without such complications (P = 0.046). The incidence of long-term granulation tissue formation, infection, and vomiting were significantly lower than those of similar short-term complications. Long-term skin problems were associated with short-term skin problems (odds ratio: 18.95; 95% confidence interval: 4.53-92.98; P < 0.001). The number of patients ≥16 years old with short- and long-term skin problems was significantly higher than in patients ≤15 years old (P = 0.0014 and P = 0.0073, respectively). CONCLUSIONS: The incidence rate of granulation tissue formation and infection after VAG were lower in the long term than in the short term. However, patients ≥16 years old presented with persistent complications.

Laparoscopic identification of combined pediatric femoral hernia and ruptured abdominal cyst of the canal of Nuck: A report of an extremely rare case.

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.

Gastrointestinal stromal tumor in perforated Meckel's diverticulum: a case report and literature review.

INTRODUCTION: Gastrointestinal stromal tumor (GIST) is rare neoplasms of the gastrointestinal tract associated with high rates of malignant transformation. GIST has been found largely in the stomach, small bowel, colon and rectum, and esophagus, but about 5% are found in other locations. We herein report a 56-year-old woman with a GIST in perforated Meckel's diverticulum. After encountering this patient, a review of the literature found reports of 18 similar patients. CASE PRESENTATION: A 56-year-old woman diagnosed with galactosialidosis (ß-galactosidase-neuraminidase deficiency) presented with vomiting. On contrast-enhanced computed tomography, peritonitis due to perforation of the intestine was diagnosed based on the free air and dilated loop of the small bowel. Laparotomy revealed perforation of Meckel's diverticulitis located 50 cm from the ileocecal valve. Partial resection of the ileum, including the diverticulum, and end-to-end anastomosis of the small intestine were performed. Regarding the pathological findings, the edge of the diverticulum wall consisted of a solid mass measuring 1.0 cm in size, and the tumor cells were spindle-shaped with 1 mitosis present per 50 high-power fields. The diagnosis was established as GIST of the Meckel's diverticulum. The postoperative period was uneventful. Follow-up at two years revealed no evidence of recurrence. CONCLUSION: GIST in perforated Meckel's diverticulum is very rare. The potential for the coexistence of GIST or other tumor should be considered in the treatment of perforated Meckel's diverticulum.

Benign mesenteric lipomatous tumor in a child: a case report and literature review.

BACKGROUND: Lipomatous tumors are the most common type of soft-tissue tumors. Benign lipomatous tumors are lipomas and lipoblastoma. We herein report a case of benign mesenteric lipomatous tumor and the largest collection of known benign mesenteric lipomatous tumors in children in the literature. CASE PRESENTATION: A 3-year-old girl presented with repeated dull abdominal pain and left abdominal mass swelling. On a physical examination, the child had a soft, moderately distended left abdomen that was not tender when palpated. Computed tomography and magnetic resonance imaging demonstrated a large fatty mass within the mesentery, measuring approximately 8 × 6 cm. The mass extended from the right upper quadrant to the lower pole of the kidneys. Laparotomy with resection of the mesenteric tumor was performed under general anesthesia. A well-capsuled tumor was a soft, yellow mass and found loosely attached to the mesenterium of the ileum. A histopathological examination demonstrated the lobular proliferation of mature adipocytes. Atypical lipoblasts were not seen. These features are compatible with benign lipomatous tumor, such as lipoma or lipoblastoma with maturation. CONCLUSION: In conclusion, benign mesenteric lipomatous tumors tend to be large in size over 10 cm in longitudinal length. However, resection is well tolerated in the vast majority of cases with benign post-operative courses.

Perioperative reactive oxygen species in infants with biliary atresia: A retrospective observational study.

Biliary atresia (BA) is a devastating cholestatic disorder of infants that presents during the first several months after birth due to an idiopathic obstruction to the bile flow. Without prompt diagnosis, Kasai portoenterostomy, and deliberate follow-ups, the resulting cholestasis leads to progressive hepatic failure. Oxidative stress is an abnormal phenomenon inside cells or tissues caused by a disturbance in the reactive oxygen species (ROS). We aimed to measure perioperative ROS in BA patients.Data are presented as median (25th, 75th percentiles). We evaluated 15 BA patients (age 55 [48, 69] days) and measured ROS; serum superoxide dismutase (SOD), urinary 8-iso prostaglandin F2α (8-iso-PGF2α) and 8-hydroxy-2'-deoxyguanosine (8-OHdG) preoperatively and 30 days later to compare values with serum liver function tests and histologic grades of liver cholestasis. For compared BA patients, 4 normal subjects as control group (age 55 [27, 75] days) measured ROS and serum liver function tests.In BA patients, the preoperative serum SOD was 6.1 IU/mL (4.7, 7.2), urinary 8-iso-PGF2α was 1969 pg/mg Cre (1697, 2374), and urinary 8-OHdG was 37.1 ng/mg Cre (33.1, 53.7). At the postoperative day 30, the serum SOD was 5.2 IU/mL (4.2, 6.7), urinary 8-iso-PGF2α was 1761 pg/mg Cre (1256, 3036), and urinary 8-OHdG was 42.1 ng/mg Cre (29.65, 72.64). In ROS, there were no significant differences between the 2 periods. In control group, urinary 8-iso-PGF2α was significantly lower than that in preoperative BA patient group. However, other ROS were not significant differences between control group and BA patient group. The concentration of urinary 8-iso-PGF2α was positively correlated with total bilirubin and direct bilirubin levels (preoperatively: r = 0.6921, P = .0042 and r = 0.6639, P = .007, postoperatively: r = 0.6036, P = .0172 and r = 0.6464, P = .0092, respectively). The preoperative ROS were not correlated with histologic grades of liver cholestasis. Various factors such as liver inflammation, lipid malabsorption, and tissue disorders due to jaundice might affect the antioxidant activity and elevated urinary 8-iso-PGF2α. However, at least until 30 days later, urinary 8-OHdG as oxidative DNA damage might persist after the operation whether the cholestasis improved or not.

Adherences to oral nutritional supplementation among hospital outpatients: An online cross-sectional survey in Japan.

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.

Cystic biliary atresia with paucity of bile ducts and gene mutation in KDM6A: a case report.

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.

The assessment of the esophageal motility of children with esophageal disorders by the detailed observation of the pH-multichannel intraluminal impedance waveform and baseline impedance: screening test potential.

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.

Resting energy expenditure prediction using bioelectrical impedance analysis in patients with severe motor and intellectual disabilities.

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 18 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) = 550.62 + 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 185.22 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.

A large retroperitoneal lipoblastoma: A case report and literature review.

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 12 × 8 × 6 cm 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.