RESUMEN
BACKGROUND: The prevalence of major coronal and sagittal spinal curves (scoliosis and kyphosis) in Turner syndrome (TS) is not well established due to limited reporting. The relationship between growth hormone (GH) therapy and its effect on TS spinal curve incidence is also not well established. METHODS: A retrospective chart review of 306 TS patients from 2007 to 2021 evaluated major coronal and sagittal spinal curves, progression of the curve, and treatment with GH. Statistical significance (defined as P <0.05) between curvature rates and curve progression was compared between GH-treated patients and non-GH-treated patients using a χ 2 or Fisher exact test when appropriate. RESULTS: Thirty-seven of 306 (12%) TS patients had a radiographically relevant spinal deformity. Twenty-seven of 37 (73%) had mild; 4 of 37 (11%) had moderate, and 6 of 37 (16%) had severe curves. Of those with severe, 4 underwent spinal fusion, 1 was treated with bracing, and 1 was braced before a cardiovascular-related death. Regarding GH use among TS patients, 190 of 306 (62%) used GH versus 116 of 306 (38%) who did not. Of those with a spinal curve, 24 of 37 (65%) used GH compared with 13 of 37 (35%) who did not. On univariate analysis, GH therapy was not a risk factor for the diagnosis of a major spinal curve, a more severe degree of the curve at the time of diagnosis, or spinal curve progression ( P >0.05 for all). CONCLUSIONS: This is the largest single institution retrospective review of a TS cohort known to the authors assessing spinal curve prevalence and relation to GH treatment and demonstrates a TS spinal curve rate of 12% (37/306). Four of six (11%) TS patients with a severe curve underwent corrective spine fusion. There was no relationship between the use of GH and the presence of a spinal curve or curve progression. Further study is warranted to determine risk factors for curve progression. LEVEL OF EVIDENCE: Level III. CLINICAL RELEVANCE: This retrospective case series serves to review and address the prevalence of spinal deformity in TS patients and whether GH impacts worsening deformity.
Asunto(s)
Cifosis , Escoliosis , Fusión Vertebral , Síndrome de Turner , Humanos , Niño , Escoliosis/epidemiología , Escoliosis/etiología , Escoliosis/terapia , Estudios Retrospectivos , Prevalencia , Atención Terciaria de Salud , Síndrome de Turner/complicaciones , Síndrome de Turner/epidemiología , Cifosis/epidemiología , Cifosis/etiología , Fusión Vertebral/efectos adversos , Resultado del TratamientoRESUMEN
BACKGROUND: The introduction of insulin pumps with bolus calculators (BCs) has improved glycemic outcomes and quality of life for those with type 1 diabetes. Despite the increased reliance on BCs, the formulas used to derive recommended boluses are not standardized. Our objective was to examine whether recommendations from different pump BCs vary significantly for identical clinical scenarios. METHODS: Three commercially available insulin pump BCs were programmed with identical settings and then presented with combinations of blood glucose (BG) and carbohydrates (CHOs) to generate a 4-unit bolus. At one- and two-hour time points, while there was insulin-on-board (IOB) present, we simulated various BG and CHO scenarios in order to compare BC-recommended doses. RESULTS: Differences in suggested doses were noted between BCs, as well as within the same brand. The greatest variation was apparent when BG was below target. Doses suggested by one BC varied depending on whether the IOB resulted from a previous dose given for BG or CHO, while the other two BCs adjusted for total IOB regardless of the source. CONCLUSIONS: In this simulation study, there were large differences in recommended doses between BCs due to the unique way each manufacturer incorporates IOB into their formulas as well as the pharmacokinetics used to derive the IOB amount. Providers should be aware that identical pump settings will result in a different dose recommendation for each pump brand and advise patients accordingly.
Asunto(s)
Diabetes Mellitus Tipo 1 , Calidad de Vida , Glucemia , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Humanos , Hipoglucemiantes/uso terapéutico , Insulina/uso terapéutico , Sistemas de Infusión de InsulinaRESUMEN
Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas/cirugía , Complicaciones Posoperatorias/epidemiología , Adolescente , Cateterismo Cardíaco , Niño , Preescolar , Femenino , Florida/epidemiología , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Humanos , Incidencia , Lactante , Masculino , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Factores de Tiempo , Resultado del TratamientoRESUMEN
The management of complete heart block in premature low birth-weight infants, particularly those with hydrops fetalis, is challenging. We report emergent implantation of permanent epicardial pacemakers in the first 48 hours of life in two premature infants (one with hydrops fetalis) with birth weights of 1,400 grams and 1,000 grams.
Asunto(s)
Estimulación Cardíaca Artificial , Bloqueo Cardíaco/terapia , Adulto , Cesárea , Enfermedad Crítica , Femenino , Bloqueo Cardíaco/complicaciones , Humanos , Hidropesía Fetal , Recién Nacido , Recien Nacido Prematuro , Recién Nacido de muy Bajo Peso , EmbarazoRESUMEN
OBJECTIVE: The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease. PATIENTS AND METHODS: This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program's web-based clinical electronic health record for patient and family access. The Electronic Health Record continuously measured frequency and time of logins, logins during and between hospitalisations, and page views by type (imaging versus textual data). RESULTS: Of the first 270 patients offered access to the system, 252 became users (93% adoption rate). System uptime was 99.9%, and no security breaches were reported. Users accessed the system more often while the patients were in hospital (67% of total logins) than after discharge (33% of total logins). The maximum number of logins by a family was 440, and the minimum was 1. The average number of logins per family was 25. Imaging data were viewed significantly more frequently than textual data (p 0.001). A total of 12 patients died during the study period and 11 members of their families continued to access their Electronic Health Records after the date of death. CONCLUSIONS: A web-based Patient Accessible Electronic Health Record was designed for patients with congenital cardiac disease. The adoption rate was high, and utilisation patterns suggest that the Electronic Health Record could become a useful tool for health information exchange.
Asunto(s)
Registros Electrónicos de Salud/organización & administración , Familia , Registros de Salud Personal , Internet , Sistemas de Registros Médicos Computarizados/organización & administración , Multimedia , Acceso de los Pacientes a los Registros , Adolescente , Actitud hacia los Computadores , Niño , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Masculino , Estudios Prospectivos , Reproducibilidad de los ResultadosRESUMEN
Initial palliation for pulmonary atresia with intact ventricular septum continues to evolve in the face of significant early and late morbidity. In patients with suitable anatomy, decompression of the right ventricle may be the first step in treatment. A hybrid approach to right ventricular decompression, combining surgery and interventional catheterization techniques is described. Direct access to the right ventricle through a subxiphoid incision with transventricular sheath placement is used to provide optimum catheter position for radiofrequency perforation of membranous pulmonary atresia followed by balloon dilation. The technique may address key limitations of the traditional surgical and interventional approaches.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Ablación por Catéter/métodos , Ventrículos Cardíacos/cirugía , Atresia Pulmonar/cirugía , Cateterismo Cardíaco/métodos , Cateterismo , Descompresión Quirúrgica , Tabiques Cardíacos , Humanos , Válvula PulmonarRESUMEN
A 10-year-old boy with a history of renal failure and hemodialysis by indwelling superior vena cava (SVC) catheters was diagnosed with SVC obstruction and clinically severe SVC syndrome. During attempted recanalization of the SVC in the cardiac catheterization laboratory, he suffered a perforation of his SVC with pericardial tamponade. After treatment of the perforation and relief of tamponade, he underwent a hybrid procedure to recanalize his SVC. A needle and then guidewire were passed directly from the right atrium through the SVC obstruction and were used to successfully dilate and stent the obstruction.
Asunto(s)
Oclusión con Balón , Cateterismo/métodos , Catéteres de Permanencia/efectos adversos , Diálisis Renal/efectos adversos , Stents , Síndrome de la Vena Cava Superior/etiología , Síndrome de la Vena Cava Superior/terapia , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/métodos , Taponamiento Cardíaco/etiología , Taponamiento Cardíaco/terapia , Niño , Terapia Combinada , Estudios de Seguimiento , Humanos , Fallo Renal Crónico/diagnóstico , Fallo Renal Crónico/terapia , Masculino , Radiografía , Diálisis Renal/métodos , Medición de Riesgo , Síndrome de la Vena Cava Superior/diagnóstico por imagen , Resultado del Tratamiento , Grado de Desobstrucción Vascular/fisiología , Vena Cava Superior/diagnóstico por imagen , Vena Cava Superior/patologíaRESUMEN
BACKGROUND: We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum. METHODS: A retrospective review was conducted of all neonates admitted with pulmonary atresia with intact ventricular septum from 1996 to March 2007. RESULTS: We identified 24 patients with a mean age at first intervention of 4.5 days with mean follow-up of 6.05 years (range, 1.9 to 12.7 years). Initial palliation was determined by right ventricular size, morphology, and presence or absence of right ventricular-dependent coronary circulation. Initial catheter-based pulmonary valve perforation and valvuloplasty was performed in 41.7% (10 of 24 patients; group A), and 58.3% (14 of 24 patients) had an initial systemic-to-pulmonary artery shunt (group B). Tricuspid valve size was significantly smaller in group B (median z-score, -0.52 group A versus -2.40 group B; p < 0.001). Placement of a shunt after valvuloplasty in group A was required in 70.0% (7 of 10 patients). There was no mortality in group A, and 70.0% (7 of 10 patients) are in a two-ventricle pathway and 30.0% (3 of 10 patients) are in a 1.5-ventricle pathway. Group B had mortality of 14.3% (2 of 14 patients), both within 5 days of surgery. All group B patients remain in a single-ventricle pathway. Overall survival is 91.7% (22 of 24 patients). CONCLUSIONS: An individualized approach to this complex lesion has good results. If the right ventricle can be safely decompressed and appears usable, the need for a shunt after valvuloplasty does not preclude two-ventricle (or 1.5-ventricle) repair. Anatomy mandating a shunt as initial palliation has substantial early mortality.