Standardizing Diabetes Care in Colorado Schools: Nearly Two Decades of Success.

Since 2005, a group of stakeholders in Colorado has collaborated to improve diabetes management in schools and childcare centers by standardizing care and embedding local expertise to support school personnel in this task. This effort has included the development of toolkits for parents, school nurses, and other health care providers; the establishment of a diabetes resource nurses program; and annually updated guidelines titled Standards of Care for Diabetes Management in the School Setting & Licensed Child Care Facilities. This collaboration has fundamentally changed how school nurses and staff in Colorado care for students with diabetes.

Management of diabetic ketoacidosis in children: Does early insulin glargine help improve outcomes?

BACKGROUND: Rebound hyperglycemia following the resolution of diabetic ketoacidosis (DKA) is common in pediatric patients with type 1 diabetes, increasing the risk of recurrent DKA and complicating the transition to subcutaneous insulin. Multiple studies suggest that early administration of long-acting insulin analogs during DKA management safely improves this transition. OBJECTIVE: This study aimed to determine whether early insulin glargine administration in children with DKA prevents rebound hyperglycemia and recurrent ketosis without increasing the rate of hypoglycemia or hypokalemia. METHODS: Patients aged <21 years presenting with DKA to Children's Mercy Kansas City between October 2012 and October 2016 were reviewed. They were categorized as Early (>4 h of overlap with intravenous [IV] insulin) and Late (<2 h of overlap) cohorts. RESULTS: We reviewed 546 DKA admissions (365 Early and 181 Late). Rebound hyperglycemia (>180 mg/dL) was lower in the Early group (66% vs. 85%, p ≤ 0.0001). Hypoglycemia (<70 mg/dL) during IV insulin administration was higher in the Early group than in the Late group (27% vs. 19%, p = 0.042). Hypoglycemia within 12 h of IV insulin discontinuation was lower in the Early group (16% vs. 26%, p = 0.012). Recurrent ketosis, hypokalemia, and cerebral edema were not different between the groups. CONCLUSIONS: Early glargine administration in pediatric DKA management is safe, decreases the rate of rebound hyperglycemia, and improves the transition to subcutaneous insulin. Hypoglycemia is less frequent following IV insulin discontinuation with early glargine, but the IV insulin rate may need to be reduced to minimize hypoglycemia during IV insulin infusion.

Worldwide Survey on Approach to Thrombolysis in Acute Ischemic Stroke With Large Vessel Occlusion.

Background and Objectives: With recent trials suggesting that endovascular thrombectomy (EVT) alone may be noninferior to combined intravenous thrombolysis (IVT) with alteplase and EVT and that tenecteplase is non-inferior to alteplase in treating acute ischemic stroke, we sought to understand current practices around the world for treating acute ischemic stroke with large vessel occlusion (LVO) depending on the center of practice (IVT-capable vs IVT and EVT-capable stroke center). Methods: The electronic survey launched by the Practice Current section of Neurology: Clinical Practice included 6 clinical and 8 demographic questions. A single-case scenario was presented of a 65-year-old man presenting with right hemiplegia with aphasia with a duration of 1 hour. Imaging showed left M1-MCA occlusion with no early ischemic changes. The respondents were asked about their treatment approach in 2 settings: the patient presented to (1) the IVT-only capable center and (2) the IVT and EVT-capable center. They were also asked about the thrombolytic agent of choice in current and ideal circumstances for these settings. Results: A total of 203 physicians (42.9% vascular neurologists) from 44 countries completed the survey. Most participants (55.2%) spent ≥50% of their time delivering stroke care. The survey results showed that in current practice, more than 90% of respondents would offer IVT + EVT to patients with LVO stroke presenting to either an EVT-capable (91.1%) or IVT-only-capable center (93.6%). Although nearly 80% currently use alteplase for thrombolysis, around 60% would ideally like to switch to tenecteplase independent of the practice setting. These results were similar between stroke and non-stroke neurologists. Discussion: Most physicians prefer IVT before EVT in patients with acute ischemic stroke attributable to large vessel occlusion independent of the practice setting.

Association between continuous glucose monitoring metrics and clinical outcomes in adults with type 1 diabetes in a real-world setting.

AIMS: Continuous glucose monitoring (CGM) metrics can assist diabetes management. Consensus statements recommend > 70 % time in range (TIR) and ≤ 36 % glucose coefficient of variation (CV). However, how these targets perform in clinical practice is unknown. This retrospective, longitudinal cohort study analyzed relationships between TIR, CV, glycated hemoglobin (HbA1c), and hypoglycemia in a real-world setting. METHODS: Data of 542 adults with type 1 diabetes who used CGM (January 2014-July 2020) were analyzed. Associations between TIR and HbA1c at the same and subsequent visits, incidence rate ratios (IRRs) for hypoglycemia at different CVs, and number of hypoglycemic events at cross-sections of HbA1c and CV were estimated by regression. RESULTS: TIR was inversely related to HbA1c; for every 10 % increase in TIR, HbA1c was significantly reduced by 0.34 % (4 mmol/mol) and 0.20 % (2 mmol/mol) at the same and subsequent visits, respectively. Level 2 hypoglycemia was significantly reduced at CV < 30 %, 30-33 %, 33.1-36 %, and 36.1-40 %: adjusted IRRs vs CV ≥ 40.1 % of 0.14, 0.28, 0.32, and 0.50, respectively. Hypoglycemic events were reduced at lower CV across HbA1c levels and at higher HbA1c across CV levels. CONCLUSION: This study quantifies HbA1c improvements with increased TIR and hypoglycemia reductions with improved CV in clinical practice.

Lower Prevalence of Diabetic Ketoacidosis at Diagnosis in Research Participants Monitored for Hyperglycemia.

CONTEXT: In Colorado children, the prevalence of diabetic ketoacidosis (DKA) at diagnosis of type 1 diabetes (T1D) has been increasing over time. OBJECTIVE: Evaluate the prevalence of and factors involved in DKA at T1D diagnosis among participants followed in monitoring research studies before diagnosis compared to patients from the community. SETTING AND PARTICIPANTS: Patients < 18 years diagnosed with T1D between 2005 and 2021 at the Barbara Davis Center for Diabetes. OUTCOME: Prevalence of and factors associated with DKA at diagnosis among participants in preclinical monitoring studies compared to those diagnosed in the community. RESULTS: Of 5049 subjects, 164 were active study participants, 42 inactive study participants, and 4843 were community patients. Active study participants, compared to community patients, had lower HbA1c (7.3% vs 11.9%]; P < 0.001) and less frequently experienced DKA (4.9% vs 48.5%; P < 0.001), including severe DKA (1.2% vs 16.2%; P < 0.001). Inactive study participants had intermediate levels for both prevalence and severity of DKA. DKA prevalence increased in community patients, from 44.0% to 55%, with less evidence for a temporal trend in study participants. DKA prevalence was highest in children <2 years (13% in active study participants vs 83% in community patients). In community patients, younger age (P = 0.0038), public insurance (P < 0.0001), rural residence (P < 0.0076), higher HbA1c (P < 0.0001), and ethnicity minority status (P < 0.0001) were associated with DKA at diagnosis. CONCLUSIONS: While DKA prevalence increases in community patients over time, it stayed <5% in active research participants, who have a 10 times lower prevalence of DKA at diagnosis, including in minorities.

Body mass index moderates the association between diabetes distress and objective self-management behaviours in adolescents with type 1 diabetes and elevated A1Cs.

OBJECTIVE: To examine the cross-sectional associations between diabetes distress, BMI (zBMI; BMI z-score), objectively measured mean daily blood glucose readings and insulin boluses administered, and A1C in adolescents with type 1 diabetes (T1D) using insulin pumps. METHODS: T1D self-management behaviour data were downloaded from adolescents' (N = 79) devices and mean daily frequency of blood glucose readings and insulin boluses were calculated. Diabetes distress was measured (Problem Areas in Diabetes-Teen questionnaire [PAID-T]), A1C collected, and zBMI calculated from height and weight. Three multiple linear regressions were performed with blood glucose readings, insulin boluses, and A1C as the three dependent variables and covariates (age, T1D duration), zBMI, diabetes distress, and the diabetes distress x zBMI interaction as independent variables. RESULTS: Participants (55.7% female) were 14.9 ± 1.9 years old with T1D for 6.6 ± 3.4 years. zBMI moderated the relationship between diabetes distress and mean daily insulin boluses administered (b = -0.02, p = 0.02); those with higher zBMI and higher diabetes distress administered fewer daily insulin boluses. zBMI was not a moderator of the association between diabetes distress and blood glucose readings (b = -0.01, p = 0.29) or A1C (b = 0.002, p = 0.81). CONCLUSIONS: Using objective behavioural data is useful for identifying how adolescent diabetes distress and zBMI affect daily bolusing behaviour amongst adolescent insulin pump users. Although distinct interventions exist to improve T1D self-management or diabetes distress, none addresses them together while considering zBMI. Decreasing diabetes distress could be especially important for youth with high zBMI.

Comparison of bupivacaine liposome injectable solution and fentanyl for postoperative analgesia in dogs undergoing limb amputation.

OBJECTIVE: The objectives of the study were to compare the clinical efficacy and adverse effects of two analgesic protocols consisting of bupivacaine liposome injectable solution (BLIS) and 0.5% bupivacaine and fentanyl for postsurgical analgesia in dogs undergoing limb amputation. STUDY DESIGN: Randomized, double-blind, prospective, controlled, intent-to-treat, clinical noninferiority trial. ANIMALS: Forty client-owned dogs. METHODS: Dogs undergoing amputation were randomly assigned to either the BLIS or control group. Postoperative pain, sedation, nausea, and amount eaten were assessed using appropriate scales at 6, 12, 18, and 24 h by trained individuals blinded to the treatment protocol. Rescue analgesia was provided for Glasgow composite measure pain scale (short form) (CMPS-SF) scores of 5 or above. Clients were requested to pain score their dogs at home using a visual analogue scale (VAS) for 48 h following discharge. RESULTS: Forty dogs completed this study (20 control dogs and 20 BLIS dogs). The BLIS and control groups were equivalent for sedation, nausea, amount eaten, and pain, at all time periods except at 6 h (p < .01), when the BLIS group pain score was lower. CONCLUSION: The BLIS provided equivalent analgesia with fewer adverse effects than fentanyl constant rate infusion (CRI) following limb amputation. Rescue analgesia was provided to five dogs in the BLIS group and four in the control group, and there was no statistical difference. Nausea scores did not differ statistically. CLINICAL SIGNIFICANCE: As BLIS provides equivalent analgesia, this may allow for decreased reliance on opioids in the immediate postoperative period.

Emerging Subspecialties: Pediatric Movement Disorders Neurology.

Pediatric movement disorders (PMD) neurologists care for infants, children, and adolescents with conditions that disrupt typical movement; serving as important subspecialist child neurologists in both academic and private practice settings. In contrast to adult movement disorders neurologists whose "bread and butter" is hypokinetic Parkinson disease, PMD subspecialty practice is often dominated by hyperkinetic movement disorders including tics, dystonia, chorea, tremor, and myoclonus. PMD neurology practice intersects with a variety of subspecialties, including neonatology, developmental pediatrics, rehabilitation medicine, epilepsy, child & adolescent psychiatry, psychology, orthopedics, genetics & metabolism, and neurosurgery. Over the past several decades, significant advancements in the PMD field have included operationalizing definitions for distinct movement disorders, recognizing the spectrum of clinical phenotypes, expanding research on genetic and neuroimmunologic causes of movement disorders, and advancing available treatments. Subspecialty training in PMD provides trainees with advanced clinical, diagnostic, procedural, and management skills that reflect the complexities of contemporary practice. The child neurologist who is fascinated by the intricacies of child motor development, appreciates the power of observation skills coupled with a thoughtful physical examination, and is excited by the challenge of the unknown may be well-suited to a career as a PMD specialist.

Error traps in the perioperative management of children with type 1 diabetes.

Patients with type 1 diabetes mellitus (T1D) require insulin administration at all times to maintain euglycemia and metabolic stability. Insulin administration in the perioperative period is complicated by fasting requirements and perioperative stressors that can change the patient's insulin needs. In addition, many anesthesia providers are not familiar with insulin dosing strategies and technology, such as insulin pumps and continuous glucose monitors (CGMs), that are commonly used by patients with T1D. Errors in perioperative insulin administration can lead to hypoglycemia, hyperglycemia, and diabetic ketoacidosis. This article reviews common errors of associated with the perioperative management of patients with T1D, including failure to assess and coordinate patient care preoperatively; failure to understand diabetes management and technology; failure to monitor blood glucose and recognize dysglycemia; and failure to appropriately administer basal insulin.

Testing an Automated Approach to Identify Variation in Outcomes among Children with Type 1 Diabetes across Multiple Sites.

Introduction: Efficient methods to obtain and benchmark national data are needed to improve comparative quality assessment for children with type 1 diabetes (T1D). PCORnet is a network of clinical data research networks whose infrastructure includes standardization to a Common Data Model (CDM) incorporating electronic health record (EHR)-derived data across multiple clinical institutions. The study aimed to determine the feasibility of the automated use of EHR data to assess comparative quality for T1D. Methods: In two PCORnet networks, PEDSnet and OneFlorida, the study assessed measures of glycemic control, diabetic ketoacidosis admissions, and clinic visits in 2016-2018 among youth 0-20 years of age. The study team developed measure EHR-based specifications, identified institution-specific rates using data stored in the CDM, and assessed agreement with manual chart review. Results: Among 9,740 youth with T1D across 12 institutions, one quarter (26%) had two or more measures of A1c greater than 9% annually (min 5%, max 47%). The median A1c was 8.5% (min site 7.9, max site 10.2). Overall, 4% were hospitalized for diabetic ketoacidosis (min 2%, max 8%). The predictive value of the PCORnet CDM was >75% for all measures and >90% for three measures. Conclusions: Using EHR-derived data to assess comparative quality for T1D is a valid, efficient, and reliable data collection tool for measuring T1D care and outcomes. Wide variations across institutions were observed, and even the best-performing institutions often failed to achieve the American Diabetes Association HbA1C goals (<7.5%).

Prótesis total de fémur en pacientes no oncológicos / Total femur prosthesis in non-oncological patients

Resumen: Introducción: Las prótesis totales de fémur (PTF) son un procedimiento muy excepcional y más aún en pacientes no oncológicos. El objetivo es analizar una serie de casos de prótesis total de fémur, su seguimiento y resultados a largo plazo. Material y métodos: Estudio retrospectivo en el que se incluyeron siete pacientes, intervenidos desde 2011 hasta 2014. Tras aplicar los criterios de exclusión (seguimiento y pacientes oncológicos), se analizaron cuatro pacientes. Se utilizó la clasificación de Paprosky para medir el defecto óseo y se valoraron diferentes parámetros: número de cirugías previas, duración de la cirugía, complicaciones, cirugías de revisión, escala de valoración funcional y seguimiento clínico radiológico. Resultados: Edad media de 78.5 años al implante de la prótesis total de fémur (PTF). El defecto óseo en el fémur (1-IIIA, 2-IIIB y 1-IV) fue más importante que en el acetábulo (2-I, 1-IIA y 1-IIB). El tiempo medio de la cirugía fue de 110 minutos. El motivo de la cirugía de revisión más frecuente fue el recambio del componente acetabular en tres pacientes debido a inestabilidad. La complicación más frecuente fue la infección. Flexión media de cadera de 85 grados y extensión de 0. La diferencia entre la valoración funcional Harris Hip Score pre- y post- es de 54.9 puntos, en la escala de Enneking fue de 14.25 puntos de media y la escala de dolor (VAS) medio es inferior a 2. El seguimiento promedio es de aproximadamente siete años y medio. Conclusiones: La PTF es una cirugía sin protocolo estandarizado al día de hoy. La infección en nuestra serie está siempre presente y el componente acetabular constreñido evitaría inestabilidades. Por lo que el implante de PTF debe ser a pacientes muy bien seleccionados.

Abstract: Introduction: Total femoral replacement (TFR), it is an uncommon surgery in non-oncological patients. Our main objective is to review our total femur replacement surgeries and analyze functional and clinical outcomes. Material and methods: We retrospectively review our series of seven non-oncological patients treated at our center with a TFR from 2011 to 2014. After excluding patients (Oncological patients or non-follow up) we revised four patients. Paprosky bone loos classification and different values were studied: number and time of surgeries, complications, revision surgeries, functional scales, and follow-up. Results: Mean age of 78.5 years. According to Paprosky classification of femur bone loss: 1 IIIA, 2 were IIIB, 1 IV. On the other side acetabular loss: 2 I, 1 IIA, 1 IIB. Surgical time for TFR was 110 minutes on average. Three patients were need revision surgery due to instability. And every single patient suffered from chronic infection. Mean range of motion was 85 flexion, 0o extension. Comparing preoperative and postoperative Harris Hip Score for hip function was 54.9 points and Enneking score was an average of 14.25 points better in our patients with TFR. Visual analogue pain score was on average less than two points. Mean follow-up is almost seven and a half years. Conclusions: TFP has not a standardized surgical protocol yet. Infection is always present in our series and acetabular constrained components in TFR avoid instability complications. TFP should be implanted only in selected patients.

Cirugía de mínima invasión en veterinaria: evolución, impacto y perspectivas para el futuro. Revisión / Minimally invasive veterinary surgery: evolution, impact and future perspective. Review

RESUMEN La cirugía mínima invasiva ha revolucionado la manera de tratar ciertas patologías en medicina. No obstante, factores como la falta de entrenamiento y los altos costos de equipamiento han limitado su implementación en medicina veterinaria. Un primer paso hacia el uso masivo de estos procedimientos es establecer el potencial que actualmente tienen. Asimismo, es importante conocer el concepto, la filosofía y las múltiples ventajas de estas técnicas, lo que permitirá a los médicos veterinarios ver la cirugía mínima invasiva desde otra perspectiva. De otra parte, se deben hacer a un lado algunos paradigmas teóricos que indican que la cirugía mínima invasiva es solo la laparoscopia y analizar las muchas posibilidades de desarrollo de técnicas en distintas patologías. El futuro de la cirugía veterinaria implica cerrar la brecha tecnológica y aprovechar el camino ya recorrido por la cirugía humana, con el propósito de ofrecer estos beneficios a pacientes veterinarios. Por último, esta revisión concluye con un análisis de algunas de las técnicas de vanguardia en medicina humana que bien pueden ser desarrolladas y empleadas en medicina veterinaria.

ABSTRACT Minimally invasive surgery has revolutionized how to treat certain diseases in Medicine. Factors such as lack of training and high costs of equipment have limited its implementation in veterinary practice. A first step towards the widespread use of these procedures is to establish its potential. It is also important to learn the concept, philosophy and advantages of these techniques, in order to allow veterinarians to see minimally invasive surgery from another perspective. On the other hand, some theoretical paradigms that indicate that minimally invasive surgery is only laparoscopy should be set aside, and then to analyze the many possibilities of developing techniques in different pathologies. The future of veterinary surgery involves closing the technologic gap, and learning from the current advances in human surgery, in order to offer these benefits to veterinary patients. Finally, this review concludes with an analysis of some of the cutting-edge techniques in human medicine that can be adopted for use in veterinary medicine.

Complicaciones de la artroplastía de hombro: reporte de un caso / Shoulder arthroplasty complications: case report

Resumen: Antecedentes: La infección e inestabilidad se encuentran entre las complicaciones de más difícil manejo en la cirugía protésica de hombro. La inestabilidad acumula hasta la mitad de los casos, mientras que la infección por hongos representa menos de 1% del total de infecciones y pueden causar daño severo a nivel óseo y de partes blandas. Métodos: En este caso clínico se presenta una infección fúngica por Candida parapsilosis en una hemiartroplastía de hombro indicada por una fractura de húmero proximal. Se dio tratamiento quirúrgico en dos tiempos y administración de fluconazol durante ocho semanas previo al segundo tiempo quirúrgico. En la revisión se colocó una prótesis reversa como tratamiento definitivo; sin embargo, el paciente sufrió varios episodios de luxación en el postoperatorio. Fue necesaria una segunda cirugía de revisión protésica para sustituir por componentes de mayor estabilidad. Durante la misma, se extrajeron muestras que fueron analizadas, obteniéndose resultados negativos para infección. Resultados: Actualmente tras tres años de seguimiento, el paciente se encuentra bien, sin limitaciones en su labor diaria y tiene un Constant Score de 50.5. Discusión: Reportamos nuestra experiencia en esta situación excepcional. A nuestro conocer, éste es uno de los primeros casos en los que la infección fúngica y la luxación protésica coinciden en un mismo paciente. Esta situación es un reto para el cirujano, el cual tiene que tratar las dos complicaciones prácticamente a la vez. No se dispone de una evidencia científica para establecer un criterio unificado para el tratamiento de las complicaciones tras cirugía de revisión de artroplastía de hombro, especialmente en la prótesis reversa o megaprótesis.

Abstract: Background: Infection and instability are the complications of prosthetic shoulder surgery of more difficult management. Instability builds up to half of the cases, while the fungal infection accounts for less than 1% of all and can cause severe damage to bone and soft tissue. Methods: In this case clinical fungal infection by Candida parapsilosis is presented in a shoulder hemiarthroplasty indicated by a fracture of the proximal humerus. He received surgical treatment in two-stages and administration of fluconazole for eight weeks prior to the second surgical time. In revision surgery was placed a reverse prosthesis as definitive treatment; however, the patient suffered several episodes of dislocation in the postoperative period; a second revision prosthetic surgery was necessary to use more stable components. During this surgery, we extracted samples that were analyzed, obtaining negative results for infection. Results: Now after three years of follow-up, the patient is well, no limitations in their daily work and has a Constant Score of 50.5. Discussion: We report our experience in this exceptional situation. We know, this is one of the first cases where the fungal infection and dislocation prosthetics in the same patient meet. This situation is a challenge for the surgeon, which has to treat complications two practically at the same time; we do not have scientific evidence to establish a criterion unified for the treatment of complications after surgery for revision of arthroplasty of the shoulder, especially in reverse or mega-denture prosthesis.

Estudio anatómioo de la amplitud de los discos ntervertebrales y de la zona fooraminal a nivel toracolumbar en perros adultos / Antomical study of intervertebral disc width and dog´s foraminal zone

La columna vertebral del perro ha sido utilizada como modelo experimental en medicina humana, y es uno de los animales domésticos más estudiados en medicina veterinaria. Sin embargo, existen limitados datos sobre la amplitud del disco intervertebral (DIV) y el foramen intervertebral (FIV). El propósito de este trabajo es medir cuantitativamente la amplitud de los DIV y FIV, e igualmente, la distancia entre la emergencia de los nervios espinales lumbares y el borde anterior del cuerpo vertebral caudal adyacente o altura del triángulo de seguridad de Kambin (ATK). Se realizó un estudio descriptivo en cinco cadáveres de perros. Se midieron: los diametros discales ventrales, la distancia entre la emergencia de los nervios espinales y el borde anterior del cuerpo vertebral caudal adyacente (CVCA), y los diámetros de los FIV de los niveles toracolumbares T12 - L6. La amplitud del DIV en el borde ventral fue en promedio de 7,34 mm (SD± 2,209), y la distancia entre los nervios espinales y el borde posterior del DIV, de 3,56 mm (SD 1,366). Los diámetros promedio del FIV fueron en su aspecto craneocaudal de 5,24 mm (SD 1,838) y dorsoventral de 4,73 mm (SD 1,098). Se evidenció para esta muestra una correlación gráfica entre la ATK y la ADIV, y una longitud máxima de todas las estructuras medidas en el segmento L4 - L5. Además, se plantea la posibilidad del desarrollo de instrumentales intradiscales y transforaminales con un diámetro de 3 mm, hechos a medida para caninos a partir de los 10 kg de peso.

Dog´s spine has been used as an experimental model in human medicine and is also one of the most studied animals in veterinary medicine. However, there are limited descriptions of intervertebral disc IV and intervertebral foramen IF width. The purpose of this study is to measure the width of the IV (IVW) and IF and its relations with the exit of the spinal nerves. A descriptive study was performed in 5-dogs´ cadavers. Were measured IV width, distance between emerging spinal nerve and the adjacent vertebral body (KTH), and the IF diameters. The IV ventral width average was 7.34 mm (SD 2.209) and distance from the spinal nerve and the posterior edge of IV was 3.56 mm (SD 1.366). The average diameters were, in craniocaudal aspect IF was 5.24 mm (SD 1.838) and dorsoventral was 4.73 mm (SD 1.098). The anatomical relationships of ID and IF are fundamental in the development of approaches and instruments for minimally invasive surgery. For this sample, it showed a graphical relationship between KTH and IDW and a maximum length of all measured structures in L4 - L5. This study raises the possibility of developing instrumental for canine dorsolateral percutaneous approach, made ​​for dogs from 10 kg.

Mielolipomas adrenales bilaterales como hallazgo incidental / Bilateral adrenal myelolipomas as an incidental finding

Los mielolipomas son tumores benignos raros, no funcionantes, compuestos por tejido adiposo maduro y precursores hematopoyéticos que involucran principalmente a las glándulas adrenales aunque pueden presentarse en localizaciones extraadrenales. Las características clínicas son muy variables, con descripciones de mielolipomas gigantes, bilaterales, asociados a síndromes de hipersecreción hormonal o a sangrado retroperitoneal. Con el incremento de la utilización de técnicas de imagen abdominales en la práctica clínica habitual, se ha observado un aumento de detección de incidentalomas adrenales, entre los cuales pueden encontrarse en raras ocasiones mielolipomas. Se describe el caso de una presentación inusual de mielolipomas adrenales bilaterales asintomáticos como hallazgo incidental en radiografías convencionales de abdomen, junto con un revisión de la literatura referida a estos tumores.

Myelolipomas are rare, non-functioning, benign tumors composed of mature fat tissue and myeloid hematopoietic precursors that involve mainly the adrenal glands and rarely other extra-adrenal tissues. The clinical features are heterogeneous and there are reports of giant and bilateral myelolipomas. Few tumors have been associated with endocrine dysfunction or retroperitoneal bleeding. With the development and wide-spread use of modern imaging techniques, this adrenal entity is sometimes found incidentally during radiology procedures for other reasons. We describe an unusual clinical presentation of asymptomatic bilateral myelolipomas incidentally discovered in abdominal X-Rays.

Comparison of relative renal function measured with either 99mTc-DTPA or 99mTc-EC dynamic scintigraphies with that measured with 99mTc-DMSA static scintigraphy

OBJECTIVE: The aim of this study was to compare the renal function measured with either 99mTc-DTPA or 99mTc-EC dynamic scintigraphies with that measured using 99mTc-DMSA static scintigraphy. METHODS: the values of relative renal function measured in 111 renal dynamic scintigraphies performed either with 99mTc-DTPA (55 studies) or with 99mTc-EC (56 studies) were compared with the relative function measured using 99mTc-DMSA static scintigraphy performed within a 1-month period. The comparisons were performed using Wilcoxon signed rank test. The number of 99mTc-DTPA and 99mTc-EC studies that presented relative renal function different by more than 5 percent from that measured with 99mTc-DMSA, using chi square test were also compared. RESULTS: the relative renal function measured with 99mTc-EC is not statistically different from that measured with 99mTc-DMSA (p = 0.97). The relative renal function measured with 99mTc-DTPA was statistically different from that measured using 99mTc-DMSA, but with a borderline statistical significance (p = 0.05). The number of studies with relative renal function different by more than 5 percent from that measured with 99mTc-DMSA is higher for the 99mTc-DTPA scintigraphy (p = 0.04) than for 99mTc-EC. CONCLUSION: the relative renal function measured with 99mTc-EC dynamic scintigraphy is comparable with that measured with 99mTc-DMSA static scintigraphy, while the relative renal function measured with 99mTc-DTPA dynamic scintigraphy presents a significant statistical difference from that measured with 99mTc-DMSA static scintigraphy.

Distintas formas de presentación clínica de un raquitismo hipofosfatémico autosómico dominante por mutación del factor de crecimiento fibroblástico 23 en una familia / Different forms of clinical presentation of an autosomal dominant hypophosphatemic rickets caused by a FGF23 mutation in one family

In this report we describe different forms of clinical presentation of an autosomal dominant hypophosphatemic rickets (ADHR) in 4 members of the same family as well as the treatment used in these patients and their response to it. Patient No 1: a 60 year old female who consulted for bone pain: Bone densitometry showed osteoporosis. Laboratory assays showed hypophosphatemia with low renal phosphate threshold, high total alkaline phosphatase, normal intact PTH and normal serum calcium. With neutral phosphate and calcitriol, the biochemical parameters normalized and bone densitometry improved significantly in less than a year. Patient No 2 her grand daughter consulted at 1 year and 8 months of age for growth retardation (height at percentile 3) and genu varum. Laboratory assays showed low serum phosphate and high total alkaline phosphatase; thickening and irregular epiphyseal borders of the wrists were observed radiologically. She began treatment with calcitriol and phosphorus with normalization of laboratory parameters and increase in growth (height increasing to percentile 50 after 20 months of therapy). Patient No 3: mother of patient No 2, she had no clinical manifestations and normal densitometry but presented low serum phosphate (1.9 mg/dl) that normalized with neutral phosphate therapy. Patient No 4: he was the youngest son of Patient No 1, who had had hypophosphatemic rickets, by age 5; his serum phosphate normalized without treatment At age 29, he presented normal serum phosphate and bone densitometry. Genomic DNA analysis performed in patient No 3, showed missense mutation with substitution of arginine at position 179 for glutamine. The family was catalogued as having autosomal dominant hypophosphatemic rickets/osteomalacia.

Describimos distintas formas de presentación clínica de un raquitismo hipofosfatémico autosómicodominante en 4 miembros de una misma familia y su respuesta al tratamiento. Paciente N° 1: de sexofemenino de 60 años que consultó por dolores costales y pélvicos, con osteoporosis densitométrica, hipofosfatemia con bajo umbral renal de fósforo, PTH intacta normal y calcemia normal. Tratada con fósforo neutro y calcitriol logró la normalización bioquímica y una notable mejoría de la densitometría en menos de un año. Paciente N° 2: su nieta, consultó al año y ocho meses de edad por presentar talla en percentil 3 y genu varum. En el laboratorio mostró hipofosfatemia y fosfatasa alcalina total muy elevada y en la Rx de mano, ensanchamiento y deflecamiento epifisario compatible con raquitismo. Tratada con fósforo neutro y calcitriol, normalizó los parámetros bioquímicos y logró un ascenso en el percentil de talla de 3 a 50 a los 20 meses de tratamiento. Paciente N° 3: la madre de la paciente N° 2, quien sin ninguna manifestación clínica y con densitometría ósea normal presentó hipofosfatemia que se normalizócon tratamiento con fosfato neutro. Paciente N° 4: el tío de la paciente N° 2, tuvo raquitismo hipofosfatémico de niño,y luego de los 5 años normalizó el fósforo sin tratamiento. Estudiado a los 29 años presentó fósforo normal y densitometría ósea normal. El análisis del ADN genómico de la paciente N° 3 mostró una mutación con sentido erróneo en el gen del factor de crecimiento fifroblástico 23 (sustitución de arginina por una glutamina en posición 179). Por lo tanto se llegó al diagnóstico de raquitismo/osteomalacia hipofosfatémico autosómico dominante.