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1.
Psychol Med ; : 1-10, 2020 May 18.
Artículo en Inglés | MEDLINE | ID: mdl-32419675

RESUMEN

BACKGROUND: Institutional deprivation in early childhood is associated with neuropsychological deficits in adolescence. Using 20-year follow-up data from a unique natural experiment - the large-scale adoption of children exposed to extreme deprivation in Romanian institutions in the 1980s -we examined, for the first time, whether such deficits are still present in adulthood and whether they are associated with deprivation-related symptoms of attention-deficit/hyperactivity disorder (ADHD) and autism spectrum disorder (ASD). METHODS: Adult neuropsychological functioning was assessed across five domains (inhibitory control, emotion recognition, decision-making, prospective memory and IQ) in 70 previously institutionalized adoptees (mean age = 25.3, 50% female) and 22 non-deprived UK adoptees (comparison group, mean age = 24.6, 41% female). ADHD and ASD symptoms were assessed using parent-completed questionnaires. RESULTS: Early institutionalization was associated with impaired performance on all tasks in adulthood. Prospective memory deficits persisted after controlling for IQ. ADHD and ASD symptoms were positively correlated. After controlling for ASD symptoms, ADHD symptoms remained associated with deficits in IQ, prospective memory, proactive inhibition, decision-making quality and emotion recognition. ASD symptoms were not independently associated with neuropsychological deficits when accounting for their overlap with ADHD symptoms. Multiple regression analysis revealed that the link between childhood deprivation and adult ADHD symptoms was statistically explained by deprivation-related differences in adult IQ and prospective memory. CONCLUSIONS: These results represent some of the most compelling evidence to date of the enduring power of early, time-limited childhood adversity to impair long-term neuropsychological functioning across the lifespan - effects that are linked specifically to deprivation-related adult ADHD symptoms.

2.
Int J Pediatr Otorhinolaryngol ; 133: 109971, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32179205

RESUMEN

OBJECTIVE: Characterize patients with complete tracheal rings and tracheoesophageal fistula (TEF) and summarize management options. METHODS: A systematic review of patients under 18 years of age with complete tracheal rings and TEF was conducted. Authors were contacted for additional patient information and new cases were added. Patients with iatrogenic TEF and tracheal stenosis due to other causes were excluded. RESULTS: Sixteen patients with a median (IQR) follow-up of 10 months (3-12 months) were identified. All had a distal TEF with complete tracheal rings distal to the TEF. There were 10 (63%) type C esophageal atresia + TEF (EA/TEF), and 1 (6%) type D (5 missing data). Median (IQR) airway diameter was 2 mm (1.5-2.2 mm). Complete tracheal rings were diagnosed prior to TEF repair in 5 (31.3%) patients, after ≥1 failed extubation in 3 (12.5%) patients, and intra-operatively during respiratory distress in 1 patient. Ten patients (62.5%) were intubated with an endotracheal tube and one with a 6 Fr flexible aortic canula (5 missing data). Four patients with an endotracheal tube for TEF repair developed ventilatory problems. Complete tracheal rings were repaired in 9 (56%) patients (8 slide tracheoplasty, 1 pericardial patch) and followed conservatively in 3 (19%). One patient required tracheotomy. Four patients died. CONCLUSIONS: Complete tracheal rings with concurrent TEF is a rare entity that pose challenges for ventilatory management during operative repair. Bronchoscopy prior to TEF repair is critical to allow for proper preoperative planning.

3.
Artículo en Inglés | MEDLINE | ID: mdl-32026473

RESUMEN

BACKGROUND: Using data from the English & Romanian Adoptees (ERA) study, we recently reported that early time-limited exposure to severe institutional deprivation is associated with early-onset and persistent neurodevelopmental problems and later-onset emotional problems. Here, we examine possible reasons for the late emergence of emotional problems in this cohort. Our main focus is on testing a developmental cascade mediated via the functional impact of early-appearing neurodevelopmental problems on late adolescent functioning. We also explore a second putative pathway via sensitization to stress. METHODS: The ERA study includes 165 Romanian individuals who spent their early lives in grossly depriving institutions and were subsequently adopted into UK families, along with 52 UK adoptees with no history of deprivation. Age six years symptoms of neurodevelopmental problems and age 15 anxiety/depression symptoms were assessed via parental reports. Young adult symptoms of depression and anxiety were assessed by both parent and self-reports; young adults also completed measures of stress reactivity, exposure to adverse life events, and functioning in work and interpersonal relationships. RESULTS: The path between early institutional deprivation and adult emotional problems was mediated via the impact of early neurodevelopmental problems on unemployment and poor friendship functioning during the transition to adulthood. The findings with regard to early deprivation, later life stress reactivity, and emotional problems were inconclusive. CONCLUSIONS: Our analysis suggests that the risk for adult depression and anxiety following extreme institutional deprivation is explained through the effects of early neurodevelopmental problems on later social and vocational functioning. Future research should more fully examine the role of stress susceptibility in this model.

5.
Proc Natl Acad Sci U S A ; 117(1): 641-649, 2020 01 07.
Artículo en Inglés | MEDLINE | ID: mdl-31907309

RESUMEN

Early childhood deprivation is associated with higher rates of neurodevelopmental and mental disorders in adulthood. The impact of childhood deprivation on the adult brain and the extent to which structural changes underpin these effects are currently unknown. To investigate these questions, we utilized MRI data collected from young adults who were exposed to severe deprivation in early childhood in the Romanian orphanages of the Ceaușescu era and then, subsequently adopted by UK families; 67 Romanian adoptees (with between 3 and 41 mo of deprivation) were compared with 21 nondeprived UK adoptees. Romanian adoptees had substantially smaller total brain volumes (TBVs) than nondeprived adoptees (8.6% reduction), and TBV was strongly negatively associated with deprivation duration. This effect persisted after covarying for potential environmental and genetic confounds. In whole-brain analyses, deprived adoptees showed lower right inferior frontal surface area and volume but greater right inferior temporal lobe thickness, surface area, and volume than the nondeprived adoptees. Right medial prefrontal volume and surface area were positively associated with deprivation duration. No deprivation-related effects were observed in limbic regions. Global reductions in TBV statistically mediated the observed relationship between institutionalization and both lower intelligence quotient (IQ) and higher levels of attention deficit/hyperactivity disorder symptoms. The deprivation-related increase in right inferior temporal volume seemed to be compensatory, as it was associated with lower levels of attention deficit/hyperactivity disorder symptoms. We provide compelling evidence that time-limited severe deprivation in the first years of life is related to alterations in adult brain structure, despite extended enrichment in adoptive homes in the intervening years.


Asunto(s)
Trastorno por Déficit de Atención con Hiperactividad/patología , Encéfalo/patología , Desarrollo Infantil/fisiología , Niño Institucionalizado/psicología , Carencia Psicosocial , Adopción , Adulto , Trastorno por Déficit de Atención con Hiperactividad/diagnóstico , Trastorno por Déficit de Atención con Hiperactividad/etiología , Encéfalo/diagnóstico por imagen , Encéfalo/crecimiento & desarrollo , Estudios Transversales , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Inteligencia , Pruebas de Inteligencia , Estudios Longitudinales , Imagen por Resonancia Magnética , Masculino , Tamaño de los Órganos , Orfanatos , Estudios Prospectivos , Rumanía , Factores de Tiempo , Reino Unido , Adulto Joven
6.
Int J Pediatr Otorhinolaryngol ; 130: 109855, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31896499

RESUMEN

OBJECTIVE: To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management. CONCLUSION: Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

7.
Int J Pediatr Otorhinolaryngol ; 128: 109697, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31698245

RESUMEN

OBJECTIVES: To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. RESULTS: Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. CONCLUSIONS: Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

8.
Laryngoscope ; 2019 Dec 10.
Artículo en Inglés | MEDLINE | ID: mdl-31821571

RESUMEN

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 2019.

9.
Laryngoscope ; 2019 Oct 11.
Artículo en Inglés | MEDLINE | ID: mdl-31603535

RESUMEN

A 6-month-old female presented for 2 months of noisy breathing. Flexible laryngoscopy showed limited bilateral vocal fold abduction. Computed tomography revealed a non-enhancing 3.6 × 2.3 × 3.5 cystic prevertebral mass spanning C2-T. Using an endoscopic approach, the overlying mucosa was incised, and the cyst was freed and fully excised from the surrounding mucosa with blunt microlaryngeal instruments without complication. Three months postoperatively she had no respiratory issues and was eating well. Flexible laryngoscopy revealed bilateral vocal fold mobility. We propose that endoscopic removal of a cervical esophageal duplication cyst in selected cases is an alternative to open excision. Laryngoscope, 2019.

11.
Otolaryngol Head Neck Surg ; 161(4): 652-657, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31184262

RESUMEN

OBJECTIVES: To compare the clinical outcomes of patients with prophylactic petiole suspension (PPS) at the time of laryngofissure and patients without PPS. STUDY DESIGN: Retrospective case series. SETTING: Tertiary pediatric hospital from May 2003 to August 2017. SUBJECTS AND METHODS: Patients included those undergoing airway reconstruction with complete laryngofissure. Patients in the study group had PPS at the time of laryngofissure, while control group patients did not. RESULTS: Eighty-one patients underwent complete laryngofissure (22 study patients, 59 controls) at a median age of 4.8 years (interquartile range, 2.1-9.7). Patients in the control group were younger at the time of the surgery (3.7 vs 6.5 years, P = .04). Other demographic and clinical data, including subglottic stenosis (SGS) grades, were similar. For patients with SGS, 53.8% and 46.2% in the study group had a grade 1-2 and 3-4 SGS, respectively, as compared with 46.2% and 53.8% in the control group (P = .65). Manifestations of petiole prolapse included failure of decannulation (8.6%), exercise intolerance (16.0%), and obstructive sleep apnea (11.1%). After 4 years of follow-up, 4.5% of the study group versus 27.1% of the control group had prolapse of the petiole (P = .04). Petiole prolapsed affected >50% of patients with no PPS after 10 years of follow-up. Patients with petiole prolapse at the first surveillance microlaryngoscopy and bronchoscopy after stent removal had an odds ratio of 10.2 (95% CI, 1.1-94.8; P = .04) of becoming symptomatic. CONCLUSION: Patients with PPS had significantly fewer symptoms after complete laryngofissure as compared with patients without PPS. PPS should be considered when a complete laryngofissure is being performed during airway reconstruction.


Asunto(s)
Epiglotis/cirugía , Enfermedades de la Laringe/cirugía , Laringoplastia/métodos , Broncoscopía , Estudios de Casos y Controles , Niño , Preescolar , Remoción de Dispositivos , Femenino , Humanos , Lactante , Laringoscopía , Masculino , Complicaciones Posoperatorias , Procedimientos Quirúrgicos Reconstructivos/métodos , Estudios Retrospectivos , Resultado del Tratamiento
12.
Am J Respir Crit Care Med ; 200(10): 1267-1281, 2019 Nov 15.
Artículo en Inglés | MEDLINE | ID: mdl-31215789

RESUMEN

Rationale: Complete tracheal ring deformity (CTRD) is a rare congenital abnormality of unknown etiology characterized by circumferentially continuous or nearly continuous cartilaginous tracheal rings, variable degrees of tracheal stenosis and/or shortening, and/or pulmonary arterial sling anomaly.Objectives: To test the hypothesis that CTRD is caused by inherited or de novo mutations in genes required for normal tracheal development.Methods: CTRD and normal tracheal tissues were examined microscopically to define the tracheal abnormalities present in CTRD. Whole-exome sequencing was performed in children with CTRD and their biological parents ("trio analysis") to identify gene variants in patients with CTRD. Mutations were confirmed by Sanger sequencing, and their potential impact on structure and/or function of encoded proteins was examined using human gene mutation databases. Relevance was further examined by comparison with the effects of targeted deletion of murine homologs important to tracheal development in mice.Measurements and Main Results: The trachealis muscle was absent in all of five patients with CTRD. Exome analysis identified six de novo, three recessive, and multiple compound-heterozygous or rare hemizygous variants in children with CTRD. De novo variants were identified in SHH (Sonic Hedgehog), and inherited variants were identified in HSPG2 (perlecan), ROR2 (receptor tyrosine kinase-like orphan receptor 2), and WLS (Wntless), genes involved in morphogenetic pathways known to mediate tracheoesophageal development in mice.Conclusions: The results of the present study demonstrate that absence of the trachealis muscle is associated with CTRD. Variants predicted to cause disease were identified in genes encoding Hedgehog and Wnt signaling pathway molecules, which are critical to cartilage formation and normal upper airway development in mice.

13.
Laryngoscope ; 2019 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-31090942

RESUMEN

OBJECTIVE: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. METHODS: Patients < 18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered. RESULTS: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). CONCLUSION: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. LEVEL OF EVIDENCE: NA Laryngoscope, 2019.

14.
Otolaryngol Head Neck Surg ; 161(1): 164-170, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-30961421

RESUMEN

OBJECTIVE: This study documents the growth and course of repaired complete tracheal rings over time after slide tracheoplasty. STUDY DESIGN: Case series with review. SETTING: Tertiary pediatric academic medical center. SUBJECTS/METHODS: Medical records of pediatric patients with confirmed tracheal rings on bronchoscopy who underwent slide tracheoplasty between January 2001 and December 2015 were reviewed. Patients who had operative notes documenting tracheal sizing over time were included. Exclusion criteria included tracheal stenosis not caused by complete tracheal rings, surgical repair prior to presentation at our institution, or lack of adequate sizing information. The postoperative follow-up was examined and airway growth over time documented. RESULTS: Of 197 slide tracheoplasties performed during the study time period, 139 were for complete tracheal rings, and 40 of those children met inclusion criteria. The median age at time of surgery was 7 months, and the median initial airway size was 3.9 mm (n = 34). The median growth postoperatively was 1.9 mm over a median follow-up period of 57 months (0.42 mm/year), which is similar to growth rates of unrepaired complete tracheal rings (P = .53). Children underwent a median of 10 postoperative endoscopies, with time between endoscopies increasing further out from surgery. The most commonly performed adjunctive procedure was balloon dilation. CONCLUSIONS: This is the first study documenting continued growth of repaired complete tracheal rings after slide tracheoplasty. Postoperative endoscopic surveillance ensures adequate growth. Intervals between airway endoscopies can be increased as the child gets older, as the airway increases in size, and as long as symptoms are minimal.


Asunto(s)
Tráquea/crecimiento & desarrollo , Tráquea/cirugía , Estenosis Traqueal/congénito , Estenosis Traqueal/cirugía , Broncoscopía , Femenino , Humanos , Lactante , Masculino , Traqueostomía
16.
J Pediatr Hematol Oncol ; 41(7): 532-536, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31033789

RESUMEN

Differentiated thyroid cancer (DTC) is the most common cancer in adolescents and young adults. In 2015, the American Thyroid Association published guidelines for management of pediatric DTC. We report our institutional experience and highlight changing practices and new opportunities. A retrospective analysis of all patients diagnosed with DTC from 2001 to 2016 was performed. Among 59 eligible patients, 31 (53%), 15 (25%), and 13 (22%) had low-risk, intermediate-risk, and high-risk disease, respectively. Half (15/31) of low-risk and all intermediate-risk/high-risk patients received radioactive iodine (I-131) ablation. For low-risk patients, average I-131 dose decreased from 80 to 42.05 mCi, and the percentage of patients who received I-131 decreased over time. Eleven of 16 patients with tumor genomic data were found to have somatic targetable (n=6) or germline (n=5) mutations. Persistent/recurrent disease was only present in high-risk (n=8) and intermediate-risk (n=1) patients. Two patients with iodine-refractory disease received trametinib to enhance radioiodine uptake. All patients were alive at follow-up (median, 5 y; range, 1 to 15 y). Coincident with the recent American Thyroid Association guidelines, the use of I-131 in low-risk patients has decreased over time in our practice. Tumor sequencing and cancer genetic evaluation may help redefine opportunities for treatment of high-risk patients and family counseling.

17.
Vet Rec ; 184(10): 320, 2019 Mar 09.
Artículo en Inglés | MEDLINE | ID: mdl-30846642
18.
Int J Pediatr Otorhinolaryngol ; 120: 162-165, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-30822686

RESUMEN

Chronic epiglottitis and supraglottitis are clinical entities that present with respiratory distress and are primarily associated with autoimmune disorders, gastroesophageal reflux disease, or angioedema. First described in adults with sarcoidosis in 2010, CO2 laser epiglottis resurfacing has been effective in reducing epiglottic edema. We present two cases of adolescent males with non-granulomatous chronic supraglottitis who were successfully treated with CO2 laser supraglottic resurfacing.


Asunto(s)
Láseres de Gas/uso terapéutico , Supraglotitis/cirugía , Adolescente , Humanos , Masculino
19.
Toxicol Pathol ; 47(2): 174-189, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30798780

RESUMEN

The prevalence of liver and skin tumors in brown bullhead ( Ameiurus nebulosus) from the Anacostia River (Washington, DC) and nearby areas was determined in 2014, 2015, and 2016. The objectives were to (1) compare tumor prevalence across space and time; (2) analyze the 1992-2016 Chesapeake Bay Tumor Database to identify reference locations and test age, length, weight, and sex as covariates; and (3) explore whether changes in bullhead exposure to contaminants can explain the observed trends. With logistic regression, we reported large statistically significant decreases in liver tumor probabilities in bullheads from the Anacostia CSX Bridge (ANAC) area between 1996 and 2001 (merged: female, 77.8%; male, 48.6%), 2009 to 2011 (female, 42.5%; male, 16.6%), and 2014 to 2016 (female, 18.0%; male, 5.7%). Skin tumors decreased by a factor of six in both females and males. Polycyclic aromatic compounds (PAC) initiate liver neoplasms and polychlorinated biphenyls (PCBs) and DDT compounds are promoters. The causes of skin tumors in bullhead are uncertain. Biomarker and tissue data show decreases in PAC-DNA adducts and PCB and DDT contamination in ANAC bullheads. It is likely that the decreased liver tumor prevalence is associated with decreased exposure to these contaminants.


Asunto(s)
Enfermedades de los Peces/epidemiología , Neoplasias Hepáticas/veterinaria , Neoplasias Cutáneas/veterinaria , Animales , District of Columbia , Femenino , Enfermedades de los Peces/etiología , Ictaluridae , Masculino , Prevalencia , Ríos/química , Contaminantes Químicos del Agua/efectos adversos , Contaminación Química del Agua/efectos adversos
20.
Psychol Med ; 49(7): 1079-1088, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-30606278

RESUMEN

There is an enormous interest in identifying the causes of psychiatric disorders but there are considerable challenges in identifying which risks are genuinely causal. Traditionally risk factors have been inferred from observational designs. However, association with psychiatric outcome does not equate to causation. There are a number of threats that clinicians and researchers face in making causal inferences from traditional observational designs because adversities or exposures are not randomly allocated to individuals. Natural experiments provide an alternative strategy to randomized controlled trials as they take advantage of situations whereby links between exposure and other variables are separated by naturally occurring events or situations. In this review, we describe a growing range of different types of natural experiment and highlight that there is a greater confidence about findings where there is a convergence of findings across different designs. For example, exposure to hostile parenting is consistently found to be associated with conduct problems using different natural experiment designs providing support for this being a causal risk factor. Different genetically informative designs have repeatedly found that exposure to negative life events and being bullied are linked to later depression. However, for exposure to prenatal cigarette smoking, while findings from natural experiment designs are consistent with a causal effect on offspring lower birth weight, they do not support the hypothesis that intra-uterine cigarette smoking has a causal effect on attention-deficit/hyperactivity disorder and conduct problems and emerging findings highlight caution about inferring causal effects on bipolar disorder and schizophrenia.

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