JPGN reports最新文献

Objectives: Inaccurate recording of anthropometrics can compromise nutritional status assessment. Boston Children's Hospital implemented a quality improvement (QI) project which increased anthropometric measurements for hospitalized pediatric patients, and this analysis quantifies its impact on estimated measurement accuracy.

Methods: From January to April 2016 (pre-QI project) and January to April 2019 (post-QI project), all inpatient encounters with age 2-18 years and at least one anthropometric measurement within 26 h of admission were included. Frequency of repeat anthropometrics within 26 h postadmission was quantified. Body mass index (BMI) Z-score was used to classify nutritional status. Chart review was performed for any hospitalization with an extreme anthropometric Z-score >6 or <-6, or difference in Z-score between repeated measurements >1 or <-1. Extremes were deemed errors when discordant with growth trends.

Results: Among inpatient encounters in 2016 with at least one weight, repeat weights were obtained in 97.7% (724/742), declining to 83.0% (802/966) in 2019 (p < 0.001). Frequency of repeat heights did not differ (153/520 [29.4%] in 2016 and 236/857 [27.5%] in 2019, p = 0.48), while frequency of repeat BMI increased following the QI project (84/468 [17.9%] vs. 449/837 [53.6%], p < 0.0001). Repeat BMI was more likely to affect nutritional status classification post-QI (8.9%) compared to pre-QI (2.4%) project (p = 0.04). Errors contributing to extreme first measurements were rare (≤0.23% of weight and BMI, and ≤1.2% of height).

Conclusions: Higher frequency of repeat BMI and lower frequency of repeat weight documentation was observed post-QI project. Repeat measurements were more likely to impact nutritional status classification with this initiative.

Alagille syndrome (ALGS) is a rare, cholestatic, multisystemic disorder characterized by bile duct paucity. Cholestatic pruritus is a common, and often severe, symptom of ALGS and is the leading cause of liver transplantation. The treatment of cholestatic pruritus is challenging and involves medical and surgical options, such as surgical biliary diversion (SBD) for refractory cases. However, SBD is associated with medical/lifestyle challenges. Maralixibat, an ileal bile acid transporter inhibitor, is a recently approved treatment for cholestatic pruritus in patients with ALGS and is used as part of standard of care. We present cases of two patients with ALGS who initiated treatment with maralixibat: one before, with continuation after, reversal of SBD, and one after SBD reversal. In both cases, treatment with maralixibat was well-tolerated and demonstrated marked improvements in cholestatic pruritus. This suggests that maralixibat is a pharmacological alternative for patients who would like to pursue reversal of SBD.

Objective: Collagenous gastritis (CG) is a rare gastrointestinal disorder characterized by the deposition of collagen in the sub-epithelium of the gastric mucosa. CG can cause a variety of symptoms including iron deficiency anemia, abdominal pain, dyspepsia, vomiting, and weight loss. We present four cases of pediatric CG, and the first known reported case of identical twins with collagenous gastritis.

Methods: This is a retrospective chart review of four cases of CG.

Results: Four male patients all presented with anemia, three of which were initially diagnosed with eosinophilic gastritis. They were all eventually diagnosed with CG and most did not respond to medication management.

Conclusions: CG is a rare disorder that is difficult to diagnosis and manage.

Splenosis is an acquired condition in which splenic tissue becomes implanted and grows in abnormal locations within the body, usually after the spleen has been injured or surgically removed. It is often discovered incidentally due to its asymptomatic nature. We report a rare case of asymptomatic pelvic splenosis in a 16-year-old female with a history of splenectomy for sudden splenic infarct at age 3 and inflammatory bowel disease diagnosed at age 15. Magnetic resonance enterography revealed a solid pelvic mass concerning for malignancy, prompting further evaluation with technetium-99m-labeled heat-denatured red blood cell scintigraphy. Imaging confirmed ectopic splenic tissue in both the pelvis and near the cecum. This case underscores the diagnostic challenge splenosis presents, especially in pediatric patients, due to its rarity and radiologic resemblance to neoplastic processes. It also highlights the critical role of nuclear medicine in avoiding unnecessary surgical interventions. Increased clinical awareness is essential for accurate diagnosis and appropriate management of this uncommon entity.

Baraitser-Winter cerebrofrontofacial syndrome (BWCFF) is a rare congenital anomaly syndrome that can present with characteristics in multiple organ systems. These can include pachygyria, intellectual disability, seizures, congenital heart defects, renal malformations and gastrointestinal dysfunction. The diagnosis is established with a pathogenic variant of the ACTB or ACTG1 gene. Few studies report gastrointestinal manifestations in patients with BWCFF. A retrospective chart review was performed on two female patients, ages 4 and 16 years, with the pathogenic variant of the ACTB gene, confirming the diagnosis of BWCFF. Patient A had malrotation at 3 months requiring a Ladd's procedure. Patient B had malrotation and volvulus at 1 year necessitating multiple surgeries for obstruction. Both patients underwent gastrointestinal workups, including endoscopy and imaging, as well as motility evaluations for ongoing abdominal pain and feeding intolerance. Gastrointestinal manometry findings in both patients were consistent with the diagnosis of chronic intestinal pseudo-obstruction. Both patients remain dependent on total parenteral nutrition.

Senna (sennosides) is a natural stimulant laxative containing anthraquinone glycosides, commonly used to treat constipation. We present the case of a healthy 2-year-old female (~12.5 kg) who is not yet toilet-trained and accidentally ingested a single 15 mg chocolate-flavored sennoside chew (1.2 mg/kg). This led to sharply demarcated erythema, pain, and blisters in her diaper area. In the emergency department, the blisters were treated by unroofing, topical mupirocin application, a petrolatum dressing, and a 5-day course of oral cephalexin. The burn injury healed without complications at a follow-up visit. This case highlights the risk of senna-induced burns in diapered toddlers, as prolonged stool-skin contact can cause blistering that mimics abusive injury. Inquiries about sennoside use should be made when perineal burns are observed in pre-continent children to prevent misdiagnosis of child abuse. Preventive measures, including caregiver education, child-resistant packaging, and safe medication storage, are essential.

Objectives: In April 2022, the United Kingdom and the United States reported alarming increases in cases of severe acute hepatitis of unknown etiology in children, indicating a multicountry outbreak. We aimed to determine if Germany was affected by the outbreak.

Methods: Cases were defined as patients 0-16 years with severe acute hepatitis (aspartate transaminase and/or alanine transaminase > 500 IU/l), with adenovirus detection or unknown etiology. Severely impaired liver function resulting in listing for liver transplant, undergoing liver transplant or death was defined as "fulminant" pediatric acute liver failure (pALF). We compared the 2017-2021 case numbers reported by German pediatric liver transplant centers (pLTxCs) with 05/2022-05/2023. Numbers of pediatric inpatients diagnosed with any of 12 selected hepatological ICD-10 codes and number of liver transplants from national hospital discharge data in 2022 were compared with 2015-2021, using a two-sample Poisson test.

Results: From 5/2022-5/2023, eight pLTxCs reported nine hepatitis cases with fulminant pALF, compared to 5-14 cases annually from 2017 to 2021, and a total of 26 hepatitis cases without pALF (no baseline data available). The number of pediatric inpatients diagnosed with any of 12 selected hepatological ICD-10 codes was 373 in 2022, compared to 333-422 annually in 2015-2021. There were 85 liver transplants in 2022, compared to 91-114 annually in 2015-2021.

Conclusions: According to available data, there was no apparent increase in severe acute hepatitis cases of unknown etiology with fulminant pALF in children in Germany, 2022. We recommend implementing syndromic surveillance at pediatric emergency units and timely access to hospital discharge data.

Esophageal food impaction (EFI) requires urgent endoscopy, typically performed by pediatric gastroenterologists (GI) or pediatric surgeons. We investigated differences in the approach between GI and surgeons to EFIs at a single pediatric center. Data collected included demographics, method of removal, whether esophageal biopsies were obtained, and biopsy results. These data were used to compare the approach to EFIs between GI and surgeons. There were 101 cases of EFI, intervened on by GI (n = 78) and surgery (n = 23). GI more frequently performed endoscopic extraction compared with surgery (41% vs. 13%, p = 0.007). GI obtained biopsies more frequently than surgery (92% vs. 13%, p < 0.001). GI and surgeons have different approaches to management and intervention for EFI. GI are more likely to remove the EFI by extraction as well as take biopsies at the time of the endoscopy compared to pediatric surgeons. These practice differences have clinical implications for the long-term management of these patients.

Food protein-induced enterocolitis syndrome (FPIES) is a nonimmunoglobulin E (IgE)-mediated food allergy. Most patients with solid FPIES experience a period of asymptomatic intake of the causative foods before onset. This study aimed to elucidate the pattern of egg yolk (EY) ingestion that triggers FPIES. We retrospectively compared consumption intervals during the asymptomatic period to those just before the first FPIES episode in 24 patients with oral food challenge test-confirmed EY-FPIES, for whom complete data regarding the dates of EY consumption before onset were available. The average interval during the asymptomatic period and that between the last asymptomatic consumption and onset were 2.2 ± 2.3 (median, 1; interquartile range [IQR], 1-3; 95% confidence interval [CI], 1.75-2.68) days and 17.1 ± 12.7 (median, 13; IQR, 8-29 days; 95% CI, 11.70-22.46), respectively. The onset of FPIES is associated with prolonged cessation after asymptomatic consumption.