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Cherubism is a rare autosomal dominant disease characterized by expansile osteolytic jawbone lesions. The effect and safety of off‐label calcitonin treatment during the progressive phase of the disease are not well described. In this retrospective study, we present data on the radiological response and adverse effects of subcutaneously administered calcitonin in a cohort of nine cherubism children (three female, six male). Two of the nine patients underwent two separate treatment courses with a significant off‐treatment interval in between; therefore, a total of 11 treatment courses with a mean duration of 17.9 months (range <1 to 35, SD 10.8) were studied. To measure the response, the cumulative volume of cherubism lesions was calculated from available three‐dimensional imaging. The primary outcome was the change in the volume of lesions during calcitonin treatment and only assessed for the eight treatment courses with a minimal duration of 6 months. A statistically significant reduction in the mean cumulative volume of lesions was seen regardless of treatment duration. Average volume reduction was highest in the first half year of treatment, with a gradual, ongoing reduction thereafter. For the secondary outcome, the change in the cumulative volume of lesions after treatment cessation was assessed for the seven treatment courses with follow‐up imaging available. After six of these seven treatment courses, the cumulative volume increased again but remained undoubtedly smaller than the initial volume at the start of therapy. Adverse effects were assessed for all 11 treatment courses and occurred in 73% of them. Most adverse effects were mild and low grade, with the most severe being one grade 3 symptomatic hypocalcemia requiring hospitalization and early treatment termination. Calcitonin treatment seems effective and tolerable in treating actively progressing cherubism in children. However, further research is required to better understand the pharmacological treatment of cherubism, including also other drugs, dosing, and protocols. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR). [ABSTRACT FROM AUTHOR]

Background: The prevalence of inflicted femur fractures in young children varies (1.5-35.2%), but these data are based on small retrospective studies with high heterogeneity. Age and mobility of the child seem to be indicators of inflicted trauma.Objective: This study describes other factors associated with inflicted and neglectful trauma that can be used to distinguish inflicted and neglectful from accidental femur fractures.Materials and Methods: This retrospective study included children (0-6 years) who presented with an isolated femur fracture at 1 of the 11 level I trauma centers in the Netherlands between January 2010 and January 2016. Outcomes were classified based on the conclusions of the Child Abuse and Neglect teams or the court. Cases in which conclusions were unavailable and there was no clear accidental cause were reviewed by an expert panel.Results: The study included 328 children; 295 (89.9%) cases were classified as accidental trauma. Inflicted trauma was found in 14 (4.3%), while 19 (5.8%) were cases of neglect. Indicators of inflicted trauma were age 0-5 months (29%, positive likelihood ratio [LR +] 8.35), 6-12 months (18%, LR + 5.98) and 18-23 months (14%, LR + 3.74). Indicators of neglect were age 6-11 months (18%, LR + 4.41) and age 18-23 months (8%, LR + 1.65). There was no difference in fracture morphology among groups.Conclusion: It is unlikely that an isolated femur fracture in ambulatory children age > 24 months is caused by inflicted trauma/neglect. Caution is advised in children younger than 24 months because that age is the main factor associated with inflicted trauma/neglect and inflicted femur fractures. [ABSTRACT FROM AUTHOR]

Objective: To evaluate in the Netherlands the national outcomes in providing cause of and insights into sudden and unexplained child deaths among children via the Postmortem Evaluation of Sudden Unexplained Death in Youth (PESUDY) procedure., Study Design: Children aged 0-18 years in the Netherlands who died suddenly were included in the PESUDY procedure if their death was unexplained and their parents gave consent. The PESUDY procedure consists of pediatric and forensic examination, biochemical, and microbiological tests; radiologic imaging; autopsy; and multidisciplinary discussion. Data on history, modifiable factors, previous symptoms, performed diagnostics, and cause of death were collected between October 2016 and December 2021., Results: In total, 212 cases (median age 11 months, 56% boys, 33% comorbidity) were included. Microbiological, toxicological, and metabolic testing was performed in 93%, 34%, and 32% of cases. In 95% a computed tomography scan or magnetic resonance imaging was done and in 62% an autopsy was performed. The cause of death was explained in 58% of cases and a plausible cause was identified in an additional 13%. Most children died from infectious diseases. Noninfectious cardiac causes were the second leading cause of death found. Modifiable factors were identified in 24% of non-sudden infant death syndrome/unclassified sudden infant death cases and mostly involved overlooked alarming symptoms., Conclusions: The PESUDY procedure is valuable and effective for determining the cause of death in children with sudden unexplained deaths and for providing answers to grieving parents and involved health care professionals., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Pyloromyotomy, the treatment for infants with hypertrophic pyloric stenosis, is a procedure with a low risk of complications and quick recovery. We describe a rare and fatal complication., Case Description: A 12-year old boy presents with persistent abdominal pain and vomiting at his general practitioner. After he collapses, cardiopulmonary resuscitation is started and he is brought to the hospital where he died. His medical history mentioned pyloromyotomy, complicated by fascia dehiscence and recurrent abdominal pain since the age of six. No cause was ever found for his abdominal pain. Autopsy was performed and showed feces in the abdominal cavity caused by two perforations and an adhesive small bowel obstruction (ASBO) from the jejunum to the abdominal wall localized at the scar tissue of the pyloromyotomy with internal herniation., Conclusion: Complaints of abdominal pain in children with previous abdominal surgery may be caused by adhesions. If abdominal pain persists and no other cause can be found, diagnostic laparoscopy should be considered.

Objective: We investigated the degree of reporting of child deaths in 2017 and compared this with data from 2012. Furthermore, we assessed the characteristics of these deaths and in how many cases the forensic doctor performed a post-mortem examination., Design: Observational research., Method: We requested data from 25 public health services (GGDs in Dutch) and the private organization Forensisch Artsen Rotterdam-Rijnmond (FARR) concerning child deaths that occurred in 2017. We distinguished between stillbirths, live births with death occurring ≤28 days post-partum and live births with death occurring > 28 days post-partum. Using mortality figures for 2017 obtained from Statistics Netherlands (CBS), the percentage of child death cases where discussions took place between the treating physician and a forensic doctor was determined both at regional and national level., Results: In 2017, a total of 928 out of 1303 child death cases known to CBS was reported to the regional forensic doctor; this means a reporting rate of over 70%. For stillbirths the national reporting rate was 46.0% (200/435), for live births who died ≤ 28 days post-partum 84.0% (300/357) and for live births where death occurred > 28 days post-partum 83.8% (428/511). The reporting rate differed per province, Zuid-Holland having the highest reporting rate (93.1%) and Gelderland the lowest (74.1%). The reporting rate increased compared to 2012 in relation to stillbirths as well as live births. The forensic doctor was most likely to perform a post-mortem examination in cases where the death occurred outside the hospital and where it involved the death of minors older than 28 days., Conclusion: Treating physicians are increasingly fulfilling their legal obligation to report deceased minors to a forensic doctor, but the reporting rate is still not 100%. We therefore advise continued investment in order to improve the reporting rate, especially in regions where figures lag behind the national average.