Hypertrophic Cardiomyopathy (HCM) is an inherited myocardial disease characterised by left ventricular hypertrophy, which carries an increased risk of life-threatening arrhythmias and sudden cardiac death. The age of presentation and the underlying aetiology have a significant impact on the prognosis and quality of life of children with HCM, as childhood-onset HCM is associated with high mortality risk and poor long-term outcomes. Accurate cardiac assessment and identification of the HCM phenotype are therefore crucial to determine the diagnosis, prognostic stratification, and follow-up. Cardiac magnetic resonance (CMR) is a comprehensive evaluation tool capable of providing information on cardiac morphology and function, flow, perfusion, and tissue characterisation. CMR allows to detect subtle abnormalities in the myocardial composition and characterise the heterogeneous phenotypic expression of HCM. In particular, the detection of the degree and extent of myocardial fibrosis, using late-gadolinium enhanced sequences or parametric mapping, is unique for CMR and is of additional value in the clinical assessment and prognostic stratification of paediatric HCM patients. Additionally, childhood HCM can be progressive over time. The rate, timing, and degree of disease progression vary from one patient to the other, so close cardiac monitoring and serial follow-up throughout the life of the diagnosed patients is of paramount importance. In this review, an update of the use of CMR in childhood HCM is provided, focussing on its clinical role in diagnosis, prognosis, and serial follow-up.

A conjoined twin is an uncommon congenital condition that has a very high morbidity and mortality prevalence. Identical twins united in utero are known as conjoined twins. It\'s an uncommon occurrence that poses a special difficulty for paediatric surgeons and obstetricians. Conjoined twins are a complicated by-product of monozygotic twinning, which raises the risk of death in the womb. One of the more prevalent varieties of conjoined twins is the thoraco-omphalopagus type, in which the heart is involved in an anterior, chest-based fusion. This case involves a 26-year-old woman who was diagnosed at 19 weeks with conjoined thoraco-omphalopagus twins using ultrasonography.

UNASSIGNED: Intrachromosomal amplification of chromosome 21 (iAMP21) is a rare subtype of B-cell precursor acute lymphoblastic leukaemia (BCP-ALL). It is unknown how iAMP21 contributes to leukaemia. The currently known commonly amplified region is 5.1 Mb.
UNASSIGNED: We aimed to narrow down the common region of amplification by using high resolution techniques. Array comparative genomic hybridization (aCGH) was used to determine copy number aberrations, Affymetrix U133 Plus2 expression arrays were used to determine gene expression. Genome-wide expression correlations were evaluated using Globaltest.
UNASSIGNED: We narrowed down the common region of amplification by combining copy number data from 12 iAMP21 cases with 52 cases from literature. The combined common region of amplification was 1.57 Mb, located from 36.07 to 37.64 Mb (GRCh38). This region is located telomeric from, but not including, RUNX1, which is the locus commonly used to diagnose iAMP21. This narrow region, which falls inside the Down Syndrome critical region, includes 13 genes of which the expression of eight genes was significantly upregulated compared with 143 non-iAMP21 B-other cases. Among these, transcriptional repressor RIPPLY3 (also known as DSCR6) was the highest overexpressed gene (fold change = 4.2, FDR < 0.001) and most strongly correlated (R = 0.58) with iAMP21-related genome-wide expression changes.
UNASSIGNED: The more precise definition of the common region of amplification could be beneficial in the diagnosis of iAMP21 based on copy number analysis from DNA sequencing or arrays as well as stimulate functional research into the role of the included genes in iAMP21 biology.

UNASSIGNED: The main purpose of this study was to carry out a global risk analysis (GRA) on the subcontracting circuit to determine and evaluate the risks linked to the future subcontracting process and to propose corrective actions for the most critical risks to ensure safety. This study must allow to conclude in an objective way to the feasibility or not of this project.
UNASSIGNED: A GRA was performed, conducted by a multidisciplinary working group that met in 20 meetings, corresponding to about 50 h of work.
UNASSIGNED: We identified 92 scenarios: 13% of scenarios had an initial criticality C1, 40% C2, and 47% C3. The GRA shows that the riskiest scenarios concern the management, material, and equipment with IT system and logistics with transport. The working group identified 25 corrective actions. After implementing those actions, 85% of scenarios had residual criticality C1, 8.5% C2, and 6.5% had residual criticality C3. The working group chose that it was impossible to subcontract part of the activity.
UNASSIGNED: The GRA conducted in this study highlighted the risks related to outsourcing this activity, evaluated and prioritized them, and recommended corrective actions. Therefore, we conclude that subcontracting the totality of sterile preparations would be harmful to patient care quality and reactivity for vital medical emergencies, such as macrophage activation syndrome, preparation of clinical trials, graft rejection therapies, preparation of very short stability chemotherapy, and the pediatric graft conditioning chemotherapy.

Maternal schizophrenia is linked to complications in offspring near the time of birth. Whether there is also a higher future risk of the child having a complex chronic condition (CCC) - a pediatric condition affecting any bodily system expected to last at least 12 months that is severe enough to require specialty care and/or a period of hospitalization - is not known.
In this population-based health administrative data cohort study (Ontario, Canada, 1995-2018), the risk for CCC was compared in 5066 children of women with schizophrenia (the exposed) vs. 2,939,320 unexposed children. Adjusted hazard ratios (aHR) were generated for occurrence of any CCC, by CCC category, and stratified by child sex, and child prematurity.
CCC was more frequent in the exposed (7.7 per 1000 person-years [268 children]) than unexposed (4.2 per 100 person-years [124,452 children]) - an aHR of 1.25 (95% CI 1.10-1.41). aHRs were notably higher in 5 of 9 CCC categories: neuromuscular (1.73, 1.28-2.33), cardiovascular (1.94, 1.64-2.29), respiratory (1.83, 1.32-2.54), hematology/immunodeficiency (2.24, 1.24-4.05) and other congenital or genetic defect (1.59, 1.16-2.17). The aHR for CCC was more pronounced among boys (1.32, 1.13-1.55) than girls (1.16, 0.96-1.40), and of similar magnitude in term (1.22, 1.05-1.42) and preterm infants (1.18, 0.95-1.46).
The risk for a CCC appears to be higher in children born to women with schizophrenia. This finding introduces opportunities for targeted preconception counselling, optimization of maternal risk factors, and intervention to support a vulnerable parent population who will experience unique challenges caring for a child with CCCs.

Our objective was to establish a corridor of normality for the external shape 3D parameters and then to assess these variables in adolescent idiopathic scoliosis (AIS).
Adolescent with mild and severe AIS were included prospectively, as well as a control group of asymptomatic subjects. A quasi-automatic 3D reconstruction of the spine and manual 3D reconstruction of the external envelope was performed from biplanar radiography. The center of mass position, the axial intersegmental moment resulting at the apex and junctional vertebrae, and the coronal trunk balance were automatically computed. A normality corridor of asymptomatic subjects was calculated as the range [5th-95th percentiles] for external shape parameters at each vertebral level.
Forty-one asymptomatic subjects (19 females; 22 males; 21 yo, SD=4) and sixty AIS (56 females; 4 males; 13 years old, SD=1.9; 30 mild and 30 severe; 34 thoracic curves and 26 thoraco-lumbar or lumbar curves) were included. All parameters based on the external shape showed differences between AIS and controls, as well as between mild and severe scoliosis. For instance, the intersegmental moment applied to the upper junctional vertebra was above the 95th percentile of controls in 70% of AIS patient. The percentage of severe patients showing parameters higher than the normality corridor was significantly higher than mild patients (p<0.0001).
The analysis of center of mass, vertebral intersegmental moment and coronal trunk balance parameters appear to be relevant in characterizing the 3D deformity of adolescent idiopathic scoliosis. The upper junctional intersegmental moment seems to be able to distinguish the different stages of curvature severity.

Objectives Growth references of today traditionally describe growth in relation to chronological age. Despite the broad variation in age of pubertal maturation, references related to biological age are lacking. To fill this knowledge gap, we aimed to develop a new type of pubertal height reference for improved growth evaluation during puberty, considering individual variation in pubertal timing. Methods Longitudinal length/height measures were obtained from birth to adult height in 1,572 healthy Swedish children (763 girls) born at term ∼1990 to nonsmoking mothers and Nordic parents, a subgroup of GrowUp1990Gothenburg cohort. A total height reference was constructed from Quadratic-Exponential-Puberty-Stop (QEPS)-function-estimated heights from individual height curves that had been aligned for time/age at onset of pubertal growth (5% of P-function growth). References that separated growth into specific pubertal heightSDS (P-function growth) and basic heightSDS (QES-function growth) were also generated. Results References (cm and SDS) are presented for total height, and height subdivided into that specific to puberty and to basic growth arising independently of puberty. The usefulness of the new pubertal growth reference was explored by identifying differences in the underlying growth functions that translate into differences in pubertal height gain for children of varying body mass, height, and with different pubertal timings. Conclusions A new type of height reference allowing alignment of individual growth curves, based on the timing of the pubertal growth spurt was developed using QEPS-model functions. This represents a paradigm shift in pubertal growth research and growth monitoring during the adolescent period.

Cardiac anesthesia and critical care provide an important continuum of care for patients with congenital heart disease. Clinicians in both areas work in complex environments in which the interactions between humans and technology is critical. Understanding our contributions to outcomes (modifiable risk) and our ability to perceive and predict an evolving clinical state (low failure-to-predict rate) are important performance metrics. Improved methods for capturing continuous physiologic signals will allow for new and interactive approaches to data visualization, and for sophisticated and iterative data modeling that will help define a patient\'s phenotype and response to treatment (precision physiology).

The unique aspect of this case study is the unusual history, presentation, ultrasonography, MRI and blood culture results, which lead to the diagnosis and treatment of adductor pyomyositis with a rare organism in a temperate country. The patient presented with a one-day history of malaise, fever, left groin pain and inability to weight bear on the left leg. There was no history of any trauma, predisposing infections or recent travel. Plain radiograph and ultrasound of the hip was normal with no effusion. Two consecutive blood cultures suggested Streptococcus mitis bacteraemia and MRI scan confirmed pyomyositis of the left hip adductors that was too small to drain. S. mitis is a normal commensal organism however it can lead to opportunistic infections particularly endocarditis. Echocardiogram revealed no cardiac complications, in particular no endocarditic vegetation. Patient was treated with intravenous benzylpenicillin for a week followed by oral phenoxymethylpenicillin for a week. Adductor pyomyositis must be considered as a differential diagnosis in a child with unusual presentation of hip pain. When an ultrasound is normal, MRI scan is warranted to confirm diagnosis. Septic screen should include blood cultures. The commonest causative organisms are the Staphylococcus family. However if S. mitis is isolated, cardiac sources of infection resulting in septic emboli must be investigated. Repeated MRI scans are required particularly if the patient does not respond to medical management.
METHODS: IV.

OBJECTIVE: The management of paediatric airway stenosis is complex, and requires a dedicated team, consisting of thoracic surgeons, phoniatricians, logopaedics, paediatricians and anaesthetists. The majority of paediatric laryngotracheal stenosis is a sequela of prematurity and prolonged post-partal intubation/tracheostomy. Surgical correction is often difficult due to a frequent combination of glottic and subglottic defects.
METHODS: In 2012, the Laryngotracheal Program Vienna was launched. Since then, 18 paediatric patients were surgically treated for (laryngo-)tracheal problems.
RESULTS: The median age of our patients was 26 months (range 2-180 months). Laryngotracheal stenosis extending up to the level of the vocal cords was evident in 9 patients. Three children were diagnosed with an isolated subglottic, and four with a short-segment tracheal stenosis or malacia. Two patients had a long-segment congenital malformation together with vascular ring anomalies. Five children were pretreated by rigid endoscopy before surgical correction, 12 of our 18 patients had a tracheostomy, 3 children were intubated at the time of operation. Different techniques of corrections were applied: laryngotracheal reconstruction (n = 4), extended partial cricotracheal resection (n = 4), cricotracheal resection with or without anterior split or dorsal mucosal flap (n = 4), slide tracheoplasty (n = 2), tracheal resection (n = 4). In 8 patients, a rib cartilage interposition was necessary in order to obtain a sufficient lumen enlargement and in 7 of these patients, an LT-Mold was placed to stabilize the reconstruction. We lost 2 patients, who were referred to our institution after failure of multiple preceding interventions, 2 and 3 months after the operation. Twelve patients are currently in an excellent condition, one is in an acceptable condition without a need for an intervention. Two patients required an endoscopic reintervention 18 and 33 months after the operation, 1 child is currently still cannulated.
CONCLUSIONS: Paediatric airway surgery is complex, and requires a dedicated interdisciplinary team. An armamentarium of different resection and reconstruction techniques is necessary in order to achieve good long-term results.