Notes

3D-printed customised prostheses for navicular bone problem restore and also recouvrement pursuing resection involving metacarpal large mobile tumours.
Frontometaphyseal dysplasia 2 (FMD2) is a rare disease caused by MAP3K7 gene mutation. We report a 7-year-old sporadic patient with FMD2 due to a de novo splicing variant in MAP3K7. He has the common characteristics of FMD2 but also has some characteristics that have never been reported, which increases the clinical phenotype of FMD2. Moreover, no systematic description of the imaging characteristics of FMD2 in computed tomography (CT) is available. In the present work, we found some different features of FMD2, reviewed previous literature, and summarized the general imaging manifestations of FMD2. This case emphasizes the important clinical value of CT and VR in the diagnosis of FMD2. We can clearly find the characteristics of FMD2 by CT examination, indicating its great significance for the prompt diagnosis and treatment of FMD2 patients.Numerous entities in the pediatric population can present in the form of cysts or as lesions with similar characteristics. Of the pathologies that can cause these images in children, infectious diseases are the most frequent. We present the case of a native of Bolivia with recent immigration to Argentina who presented a pulmonary co-infection with tuberculosis and hydatidosis. Both infections can present with similar signs and symptoms and although this association is rarely reported in the literature, certain immunological mechanisms could intervene in the causal association of co-infection between helminth parasites and mycobacteria. Both pathologies are very prevalent infections in our region and should be taken into account among the differential diagnoses in patients with cystic or cavitary pulmonary diseases.The diagnosis of Alport syndrome is a challenge in the pediatric age, due to the absence of expected clinical phenotypes of the disease, its classic characterization of a rare disease and the very restricted practice of renal biopsies with routine analysis of the sample by electron microscopy during infancy. The clinical and genetic characteristics of 6 pediatric patients (4 women) diagnosed with Alport syndrome in two hospital centers between 2018 and 2021 are reported. All patients presented a clearly different clinical debut and none presented auditory or ophthalmological complications. Epicatechin Half had no family history of chronic kidney disease. No kidney biopsy performed confirmed the diagnosis. All patients were genetically confirmed and were the index case in the family study. This series illustrates the presence of unexpected clinical phenotypes in Alport syndrome and reflects the need for the incorporation of the genetic study for its diagnosis.Group B β-hemolytic Streptococcus or Streptococcus agalactiae is a major cause of morbidity and mortality in neonates, especially in premature infants. Current prevention strategies have been effective in reducing the frequency of early onset neonatal sepsis caused by vertical transmission. The incidence of late onset sepsis due to this microorganism has not changed and the route of infection is less clear. In breastfed infants, transmission through breast milk is possible. We report three cases of late group B β-hemolytic streptococcal infection in breastfed preterm infants whose mothers had mastitis. In all cases, both the breast milk culture and the blood cultures of the neonates developed the same microorganism.Pediatric palliative care aims at improving the care and quality of life of children who are terminally ill or have a fatal prognosis. In the hospital setting, end-of-life decisions and treatments are common in intensive care units. This is why the integration of palliative care in these units is a logical evolution of the clinical approach to severely ill children. This study provides an update on the indications and characteristics of the palliative care approach in the context of pediatric intensive care. Here we describe palliative care approach initiation, decision-making, communicative aspects, drug treatment, symptom management, and palliative sedation.Lung ultrasound (LU) has gained ground in the diagnosis of most respiratory conditions present since birth. It is highly sensitive to variations in air content and pulmonary fluids and functions as a true densitometer of the lung parenchyma with a sensitivity superior to that of radiological studies. A LU is a non-invasive, fast and easy tool that can be used at the patient's bedside and, unlike conventional radiology, does not pose risks of radiation. In addition, a LU provides real-time dynamic information in a variety of neonatal settings and, like heart and brain examinations, can be performed by the neonatologist. The objective of this article is to describe the main artifacts and images that can be found in the neonatal LU, as well as the different aeration patterns, and to highlight their usefulness in the study of the most frequent respiratory disorders of neonates.
The use of transcranial Doppler (TCD) ultrasound in neurocritical patients is reported to be increasingly common in pediatric intensive care units. The objective of this survey was to know about the use and practice of TCD ultrasound in neurocritical care and the training process of staff members performing it.

Survey administered to providers from 23 pediatric intensive care units of Argentina.

The percentage of response was 86%. TCD ultrasound was used for suspected brain death (n = 20), head injury (n = 16), and stroke (n = 16). Pediatric intensivists perform the test (n = 13/20). Surveyed participants use TCD ultrasound to decide on treatment and management, start brain death assessment, request brain computed tomography, and manage cerebral perfusion pressure with vasopressors.

All surveyed participants use TCD ultrasound findings to guide management or treatments. Half of surveyed participants are little satisfied with their training.
All surveyed participants use TCD ultrasound findings to guide management or treatments. Half of surveyed participants are little satisfied with their training.
Isolated lateralized overgrowth (ILO), formerly referred to as hemihyperplasia/hemihypertrophy, is the overgrowth of one-half of the body to its contralateral in the absence of a recognizable pattern of malformations or genetic syndromes. Our objective was to analyze the growth clinical and radiological characteristics of patients with ILO under follow-up in a tertiary care hospital in Argentina between 1993 and 2020.

Retrospective, observational, single cohort study of patients with ILO.

A total of 76 cases were included; 41 were males. Median years of follow-up 5.85 (interquartile range [IQR] 2.60-10.96), maximum 15.76 years. Forty-eight of 76 patients had overgrowth compromising more than 1 body segment (complex ILO). The mean birth weight Z-score of term girls with complex ILO was +0.51 (standard deviation [SD] 0.91) (p 0.022). Most children grew between the 50th and 97th centile of the Argentinian population height reference. The median leg length discrepancy was 1.5 cm (IQR 1.01-2.2) in patients receiving medical treatment and 3.70 cm (IQR 2.95-3.98 cm) in those who required epiphysiodesis. Progression of discrepancy ≤ 2 cm was observed in 75% of cases. Renal asymmetry ≥ 1 cm was observed in 8 cases; Wilms tumor was noted in 2 cases mean age at diagnosis 0.75 years.

Prenatal growth of children with ILO is normal, except in girls with complex ILO, in whom it tends to be increased. The average height of boys and girls tends to be located in high centiles with normal growth over time. Embryonal tumor screening is recommended in this group of children.
Prenatal growth of children with ILO is normal, except in girls with complex ILO, in whom it tends to be increased. The average height of boys and girls tends to be located in high centiles with normal growth over time. Embryonal tumor screening is recommended in this group of children.
The continuity of care from pediatrics to adult medicine is key to optimal health care.

To describe the experience of the transition process of adolescent patients with chronic diseases from pediatric to adult care in a general hospital.

Cross-sectional study of patients aged 16-24 years with a history of liver transplantation, kidney transplantation, endocrine, metabolic, rheumatic diseases, and myelomeningocele seen at a tertiary care teaching general hospital between 2015 and 2019 during the transition process. The process of health care and transition success were assessed. The Transition Readiness Assessment Questionnaire (TRAQ) was used.

A total of 372 patients were included. The myelomeningocele clinic, the kidney transplant and the liver transplant teams were the most common specialties. Thirty-seven percent of participants were involved in the transition process. The mean duration of follow-up by pediatrics until transition initiation was 9 years. The mean age at the beginning of transition was 19 years, and the mean age at the end, 21 years. The joint clinic transition strategy was the most frequent, used in 96% of cases. The median value of the ordinal TRAQ was 4; of these, 32% had already seen adult care physicians. A successful transition was achieved by 32.7%.

The continuity of care during transition is a process that took almost 2 years; more than one third of the patients had a successful transition.
The continuity of care during transition is a process that took almost 2 years; more than one third of the patients had a successful transition.
Childhood neurodevelopmental disorders account for 10% of the causes of childhood disability. The search for medical care leads to therapeutic itineraries routes taken by individuals to seek health care where diagnostic and treatment opportunities arise. Our objective was to explore these itineraries in order to understand the opportunities and barriers to the implementation of therapies and child rearing patterns promoting neurodevelopment.

Qualitative study using in-depth interviews with children's parents (between June 2018 and November 2019). The analysis was based on the social model of disability and Vygotsky's approach to child development.

A total of 16 interviews were conducted. Considering the time of diagnosis and the age when the therapeutic itinerary started, 2 groups were identified those who started from birth to 2 years old (early initiation) and those who started from 3 years old (late childhood initiation). In the first group, the search for treatment starts at an early stage, while in the other group, decisions on the initiation and/or type of treatments are prolonged over time. Late initiation was accompanied by difficulties in school, periods of uncertainty, distress and/or family conflicts due to the complexities of parenting.

Therapeutic itineraries started early in some cases and at a later stage in others. The initiation of treatments made it possible to use tools to bridge the gap of discrepancies between the biological and cultural lines of development.
Therapeutic itineraries started early in some cases and at a later stage in others. The initiation of treatments made it possible to use tools to bridge the gap of discrepancies between the biological and cultural lines of development.
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