Factors associated with pyloric hypertrophy severity and post-operative feeding and nutritional recovery in infantile hypertrophic pyloric stenosis.

BACKGROUND: To examine factors that affect the severity of pyloric hypertrophy, post-operative feeding and nutritional recovery in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Medical records of infants diagnosed with IHPS at a single tertiary center between 2009 and 2018 were retrospectively reviewed. Clinical characteristics, biochemistry data and outcome were assessed for their association with the severity of pyloric hypertrophy and post-operative recovery. Nutritional recovery was assessed using weight-for-age status improvement after surgery. RESULTS: Eighty-five patients were recruited in this study. The mean pre-operative weight-for-age percentile was 18.2. Elevated bicarbonate was positively correlated with symptom duration (p = 0.007). Pyloric muscle thickness was significantly correlated with age, weight, and symptom duration (p = 0.004, 0.003, 0.008, respectively). The mean weight-for-age percentile increased to 41.6 by post-operative weeks 6-8. Pyloric muscle thickness was negatively correlated with nutritional recovery by post-operative weeks 6-8 (p = 0.003). In multivariable analysis, pyloric length related to nutritional recovery at week 1-2 postoperatively (OR = 1.42, p = 0.030, 95% CI = 0.03-1.94), and pyloric muscle thickness related to nutritional recovery at week 6-8 postoperatively (OR = 4.08, p = 0.032, 95% CI = 1.13-14.7). CONCLUSION: Our study indicated that favorable nutritional outcome and successful weight gain was observed 6-8 weeks after surgery in children with IHPS. Pyloric muscle thickness positively correlated with age, weight, symptom duration, and favorable nutritional recovery. Serum bicarbonate showed a positive correlation with symptom duration.

Detection of pediatric hepatic steatosis through ultrasound backscattering analysis.

OBJECTIVES: Hepatic steatosis has become a considerable concern in the pediatric population. The objective of this study was to evaluate the feasibility of using ultrasound Nakagami imaging to produce a parametric image for analyzing the echo amplitude distribution to assess pediatric hepatic steatosis. METHODS: A total of 68 pediatric participants were enrolled in healthy control (n = 26) and study groups (n = 42). Raw data from ultrasound imaging were acquired for each participant analysis using AmCAD-US, a software approved by the US Food and Drug Administration for ultrasound Nakagami imaging. The Nakagami parameters were compared with the hepatic steatosis index (HSI) and the steatosis grade (G0: HSI < 30; G1: 30 ≤ HSI < 36; G2: 36 ≤ HSI < 41.6; G3: 41.6 ≤ HSI < 43; G4: HSI ≥ 43) using correlation analysis, one-way analysis of variance (ANOVA), and receiver operating characteristic (ROC) curve analysis. RESULTS: The Nakagami parameter increased from 0.53 ± 0.13 to 0.82 ± 0.05 with increasing severity of hepatic steatosis from G0 to G4 and were significantly different between the different grades of hepatic steatosis (p < .05). The areas under the ROC curves were 0.96, 0.92, 0.85, and 0.82 for diagnosing hepatic steatosis ≥ G1, ≥ G2, ≥ G3, and ≥ G4, respectively. CONCLUSIONS: The Nakagami parameter value quantifies changes in the echo amplitude distribution of ultrasound backscattered signals caused by fatty infiltration, providing a novel, noninvasive, and effective data analysis technique to detect pediatric hepatic steatosis. KEY POINTS: • Ultrasound Nakagami imaging enabled quantification of the echo amplitude distribution for tissue characterization. • The Nakagami parameter increased with the increasing severity of pediatric hepatic steatosis. • The Nakagami parameter demonstrated promising diagnostic performance in evaluating pediatric hepatic steatosis.

Whole-Exome Sequencing Identified Novel CLMP Mutations in a Family With Congenital Short Bowel Syndrome Presenting Differently in Two Probands.

Congenital short bowel syndrome (CSBS) is a rare condition characterized by an inborn shortening of bowel length with loss of intestinal functions, which often combines malrotation. CXADR-like membrane protein (CLMP) and filamin A (FLNA) gene mutations are the two major causes of this inherited defect. We presented two siblings with the older brother suffering from a laparotomy for bowel obstruction due to malrotation on the 17th day after birth. The younger sister encountered a laparotomy for lactobezoar at 6 months old. CSBS was diagnosed by measurement of the bowel length during the operations. Compound heterozygous CLMP mutations with the paternal allele harboring a long deletion across exon 3-5 and the maternal allele bearing a non-sense mutation of exon 3 (c.235C > T, p.Q79∗) were identified in both cases. They are the first reported familial CSBS caused by novel CLMP mutations in Taiwan.