Prevention of motor ascending branch of the rectus femoris injuries in Bernese periacetabular osteotomy: a cadaveric study.

The Bernese periacetabular osteotomy (PAO) is a surgical procedure used to treat hip dysplasia in young adults, but it carries the risk of neurological complications, including injury to the motor ascending branch of the rectus femoris (MABRF). This study aimed to describe anatomical considerations to prevent MABRF injuries during PAO. A cadaveric study was conducted on seven specimens. The original and modified PAO approaches were used, with and without disinsertion of the rectus femoris muscle origin. The femoral nerve was dissected in all specimens from the endopelvic position to the MABRF origin (T-point). The average distance from the anterosuperior iliac spine to the T-point was 10.2 ± 0.4 cm. To protect the MABRF, a safety zone was identified for the osteotome placement during the ischial cut. The osteotome was slid over the joint capsule, deflecting the iliocapsularis muscle medially and distally. This manoeuvre shields the MABRF with the iliocapsularis muscle, reducing the risk of neurological injury. Both the original and modified PAO approaches were considered safe techniques with low risk to the rectus femoris innervation. These findings offer valuable insights for surgeons performing PAO, emphasizing the significance of understanding anatomical relationships and implementing protective measures to enhance patient outcomes and minimize complications. In conclusion, implementing these anatomical considerations can help prevent MABRF injuries during PAO, contributing to safer and more successful surgical interventions for hip dysplasia in young adults.

Bernese Periacetabular Osteotomy: A Comparative Study of Four Types of Incisions.

Bernese periacetabular osteotomy has diverse complications associated with incisions, such as dehiscence, hypertrophy, depression, and hyperpigmentation on scars, which affect patient satisfaction. The objective was to evaluate aesthetics and satisfaction outcomes in four different incisions. We evaluated 176 incisions in 148 patients. The incisions performed were the original modified (16, group I), straight and shortened, (64, group II), "Z" shaped (16, group III), and oblique inguinal (48, group IV). The scars were evaluated for width and length, development of a hypertrophic scar, depression or hyperpigmentation, and dehiscence and resuture. A scale of satisfaction was applied (points ranging from 1 to 10). The Bartlett test and Kruskal-Wallis test were used. The mean width and length of the scars were 20.3 and 6.8 cm for group I, 6.5 and 8.1 for group II, 12.1 and 7.1 cm for group III, 13 and 1.4 cm for group IV, respectively. Hypertrophic scars were found in 18% in group I, 12.5% in group II, and 31.2% in group III. Depressed scars were found in 10.8% in group I and 7.1% in group II. Hyperpigmentation was found in 16% in groups I and II, 37% in group III, and 2% in group IV. Dehiscence was found in 8.1% in group I and 8.9% in group II. Satisfaction for group IV was nine points. The difference in length and width and satisfaction were statistically significant (P < 0.05). The oblique inguinal incision (group IV) showed a smaller percentage of complications, with an adequate aesthetic result, and a high grade of patient's satisfaction.

Bernese periacetabular osteotomy: functional outcomes in patients with untreated intra-articular lesions.

Developmental dysplasia of the hip (DDH) has been recognized to be a condition leading to osteoarthritis. Periacetabular osteotomy (PAO) has showed good results on hip preservation treatment for these cases. Nevertheless, intra-articular damage may be responsible for persistent post-operative symptoms, so treat the articular damage before or during the PAO has emerged as an alternative to address it. The objective is to identify the prevalence of intra-articular damage, functional outcomes of patients undergoing PAO with untreated intra-articular lesions and the survivorship free total hip arthroplasty (THA) at long-term follow-up. A retrospective review of 103 hips in 92 patients, mean age 26 years old (19-31), 96% females. Mean follow-up 7 years (range: 3-16). Intra-articular damage was evaluated with high-resolution magnetic resonance imaging (MRI) previous to perform the PAO, the chondral damage was evaluated using International Cartilage Repair Society classification. Harris Hip Score (HHS) was obtained in all patients. One hundred per cent of the cases had labral tears on MRI, hypertrophic labrum in 80.8% and paralabral cysts in 20.8%. Acetabular chondral damage was Grade 2 in 88.5% of the hips. HHS was good and excellent in 94%. Survivorship free of THA at 15 years was 87%. Chondrolabral damage is a common finding in patients with DDH. Despite that, excellent results are obtained with PAO without labral repair. We think the focus should be in the biomechanical and anatomical correction of the hip in patients with DDH.

Later-onset congenital central hypoventilation syndrome due to a heterozygous 24-polyalanine repeat expansion mutation in the PHOX2B gene.

AIM: to describe a family with later onset congenital central hypoventilation syndrome (LO-CCHS) and heterozygosity for a 24-polyalanine repeat expansion mutation in the PHOX2B gene, rendered phenotypically apparent with exposure to anesthetics. CASE SUMMARY: An otherwise healthy 2.75-year-old boy presented with alveolar hypoventilation after adenoidectomy and tonsillectomy for obstructive sleep apnea, requiring invasive ventilatory support during sleep. He had a heterozygous 24-polyalanine repeat expansion in the PHOX2B gene (20/24 genotype), a genotype that has not been previously described in association with CCHS or LO-CCHS symptoms. Clinical findings in members of the family with the same 20/24 genotype ranged from asymptomatic to prolonged sedation after benzodiazepines. CONCLUSION: CCHS should be suspected in individuals presenting with unexplained hypoventilation and/or seizures after anesthetics or sedatives. This is the first report of LO-CCHS in a kindred with the PHOX2B 20/24 genotype. The incomplete penetrance observed in this family suggests a gene-environment interaction.

Análisis de causas atribuidas a "BRUE" entre los años 2017 a 2020 / Analysis of etiologies attributed to "BRUE" between the years 2017 to 2020

El término BRUE describe un evento en un lactante menor, repentino, breve, ya resuelto y sólo aplica cuando no existe una explicación para este episodio. Es escasa la literatura nacional e internacional sobre el estudio etiológico en BRUE. Objetivos: Caracterizar lactantes con episodio de BRUE y hacer un análisis etiológico. Métodos: Estudio retrospectivo, descriptivo lactantes hospitalizados por BRUE. Resultados: Se encontraron 50 lactantes con BRUE, la mayoría de ellos presentó un solo evento y ninguno requirió reanimación cardiopulmonar. Las características principales de los eventos fueron apnea, cianosis y tono disminuido. Las etiologías encontradas, más habituales, fueron reflujo gastro-esofágico, infección respiratoria, mala técnica alimentaria y crisis epilépticas. La evaluación clínica fue el principal elemento diagnóstico. Discusión: Nuestro análisis etiológico concuerda con la literatura nacional e internacional. La anamnesis y examen físico son la principal herramienta diagnóstica. Es fundamental contar con guías, adaptadas a la realidad nacional y local, que dirijan el estudio de lactantes con BRUE.

BRUE is an event occurring in an infant when the observer reports a sudden, brief, and now-resolved episode. BRUE is a diagnosis of exclusion and is used only when there is no explanation for the event after conducting an appropriate history and physical examination. There is little literature on the etiological study in BRUE. Objectives: To characterize infants with a BRUE episode and to carry out an etiological analysis. Methods: A retrospective study including infants who have experienced a BRUE between the years 2017 to 2020. Results: 50 infants with BRUE, most of them presented a single event and none required cardiopulmonary resuscitation. The main characteristics of the events were apnea, cyanosis and decreased tone. The most common etiologies found were gastroesophageal reflux, respiratory infection, poor feeding technique, and seizures. History and physical examination are the fundamental diagnostic tools. Discussion: Our etiological analysis agrees with the national and international literature. The clinical evaluation was the main diagnostic tool. It is essential to create local guidelines for the evaluation investigation and management of infants with BRUE.

Recomendación de experto: apneas respiratorias y parámetros de sueño en el recién nacido y lactantes menores de 3 meses / Expert consensus: management of respiratory apneas and sleep parameters in newborn and infants under 3 months

El diagnóstico de apneas en lactantes menores de tres meses constituye un gran desafío y es un área en pleno desarrollo. Es por esto, que diferentes especialistas en sueño, pertenecientes a dos Sociedades Científicas de Chile: la Comisión de Sueño, de la Sociedad Chilena de Neumología Pediátrica (SOCHINEP) y el Grupo de Trabajo Trastornos del Sueño en Pediatría de la Sociedad de Psiquiatría y Neurología de la Infancia y Adolescencia (SOPNIA), se han puesto de acuerdo en proponer un consenso básico sobre los parámetros de sueño y del manejo de las apneas en los menores de 3 meses de vida. El objetivo, es que podamos contribuir al manejo de estos pacientes, con un lenguaje y manejo similar, y valores de referencia apropiados para ese grupo etario, respaldado con las últimas investigaciones al respecto.

Sleep apnea diagnosis in infants younger than 3 months has been a major challenge for modern medicine. Using current literature, experts from the Chilean Society of Pediatric Pulmonology Sleep Commission, and the Chilean Society of Psychiatry and Neurology for Children and Adolescents, have produced a national state-of-the-art consensus. The main goal of this statement is to unify our language in this matter, based on the latest evidence.

Vitamina D y asma / Vitamin D and asthma

The main source of Vitamin D is the bioconversion of 7-dehydrocholesterol to cholecalciferol, however, the level of sun exposition has been drastically reduced during the last decades, so it is not unusual to find a high deficit of Vitamin D in the population. There is evidence to suggest that the deficit of Vitamin D is associated with a worse evolution of asthma and a greater risk of serious exacerbations. Possibly prenatal Vitamin D supplementation to pregnant mothers reduces the risk of wheezing and asthma in the offspring, and supplementation to asthmatic children could improve the evolution of the disease.

La principal fuente de Vitamina D es la bioconversión del 7-dehidrocolesterol a colecalciferol, sin embargo, el nivel de exposición solar se ha reducido drásticamente durante las últimas décadas, por lo que no es extraño encontrar un déficit elevado de Vitamina D en la población. Existe evidencia que sugiere que el déficit de Vitamina D se asocia a peor evolución del asma y mayor riesgo de exacerbaciones graves. Posiblemente la suplementación prenatal de Vitamina D a madres embarazadas reduzca el riesgo de sibilancias y asma en los hijos que nacerán, y la suplementación a niños asmáticos podría mejorar la evolución de la enfermedad.

Obesidad y sueño / Obesity and sleep

The progress of the obesity epidemic in children and adolescents and its close relationship with sleep and their comorbidities are a reason for growing concern. In this articule we review the epidemiology, prenantal, and postnatal risk factors of obesity, and associated comorbidities, such as: metabolic syndrome; chronic pro-inflammatory state and sleep-disordered breathing (SDB), with the involved pathophysiological mechanisms. We also analyze the links between obesity and SDB, the consequences of this association, in terms of enhancing the damage to physical and psychological health and the main therapeutic available measures

El avance de la epidemia de la obesidad en niños y adolescentes, su estrecha relación con el sueño y sus comorbilidades son motivo de una preocupación creciente. En este artículo revisaremos la epidemiología y factores de riesgo prenatales y postnatales de la obesidad. y las comorbilidades asociadas, tales como: síndrome metabólico, estado pro-inflamatorio crónico y trastornos respiratorios del sueño (TRS), con los mecanismos fisiopatológicos involucrados. También analizaremod las interconexiones entre la obesidad y los TRS, las consecuencias de esta asociación, en cuanto a potenciarel daño a la salud física y psicológica y las principales medidad terapéuticas disponibles

Guía clínica para el diagnóstico y cuidado de niños/adolescentes con bronquiolitis obliterante post-infecciosa, 2009 / Clinical guidelines for the diagnosis and care of children and adolescents with post-infectious bronchiolitis obliterans, 2009

La bronquiolitis obliterante (BO) es un síndrome clínico poco frecuente en niños, caracterizado por la obstrucción crónica al flujo de aire asociado a cambios inflamatorios y distintos grados de fibrosis en la vía aérea pequeña. Si bien existen muchas etiologías, la causa mas frecuente se asocia a infeccionesrespiratorias virales, principalmente adenovirus. No existe un consenso para establecer su diagnóstico; sin embargo, se considera un espectro de síntomas persistentes asociados a un patrón en mosaico, bronquiectasias y atelectasias persistentes. El rol de la biopsia pulmonar ha sido cuestionado por subajo rendimiento, invasividad y complicaciones. No existe un tratamiento específico por lo que elmanejo es soporte. Probablemente la mejor estrategia constituya el empleo de antibióticos en forma agresiva, soporte kinésico y nutricional constante y una precoz rehabilitación pulmonar. Estas guías clínicas representan un esfuerzo multidisciplinario, basado en evidencias actuales para brindarherramientas prácticas para el diagnóstico y cuidado de niños y adolescentes con BO post infecciosa.

Metapneumovirus humano: un nuevo agente en IRA alta y baja / Human metapneumovirus and acute respiratory disease

El metapneumovirus humano luego de su descubrimiento a principios de este siglo por van den Hoogen, ha mostrado tener un rol importante en muchas enfermedades del tracto respiratorio bajo en niños pequeños y adultos. Diversos estudios recientes han permitido entender su patogenia,epidemiología y manejo, explorando nuevas técnicas diagnósticas; sin embargo, aún existen muchaspreguntas sin respuestas. Brindamos una actualización en torno a este interesante y nuevo agente infeccioso.

Pseudohipoparatiroidismo y cardiomiopatía hipocalcémica / Pseudohypoparathyroidism an hypocalcemic myocardiopathy

Se presenta un caso de pseudohipoparatiroidismo complicado con una cardiomiopatía hipocalcémica y un síndrome convulsivo. En el momento del diagnóstico, los hechos más destacados fueron: rasgos fenotípicos característicos, cardiomegalia radiológica, alteraciones electrocardiográficas compatibles con hipocalcemia, osteoporosis generalizada y calcificaciones de tejidos blandos en la radiografía de esqueleto, calcio plasmático 4,53 mg% y fosfato plasmático 7,7mg%. En respuesta al tratamiento con vitamina D3, gluconato de calcio, hidróxido de aluminio, acetildigitoxina, hidroclorotiazida, y con un régimen pobre en fosfatos, se obtuvo una mejoría progresiva del calcio y fósforo plamáticos, junto con la normalización de la silueta cardíaca y del trazado electrocardiográfico