Pancreatitis aguda en el escenario de hipercalcemia por hiperparatiroidismo primario / Acute pancreatitis in the setting of hypercalcemia due to primary hyperparathyroidism

Introducción: describir el caso de un paciente con pancreatitis aguda secundaria a hipercalcemia por hiperparatiroidismo prImario. Esta es una causa poco frecuente de pancreatitis, asociada a morbimortalidad significativa en caso de no ser diagnosticada oportunamente Caso clínico: un hombre de 44 años, con antecedente de pancreatitis de presunto origen biliar que había requerido previamente colecistectomía, consultó por dolor abdominal y náuseas. Los estudios complementarios fueron compatibles con un nuevo episodio de pancreatitis aguda. Presentaba hipercalcemia y hormona paratiroidea (PTH) elevada, configurando hiperparatiroidismo primario. La gammagrafía informó hallazgos compatibles con adenoma paratiroideo. Se inició tratamiento con reanimación hídrica y analgesia con adecuada disminución de calcio sérico y resolución de dolor abdominal. Después de la paratiroidectomía se logró normalizar los niveles de calcio y PTH. Discusión: la pancreatitis aguda es una condición potencialmente fatal, por lo que la sospecha de causas poco frecuentes como la hipercalcemia debe tenerse en cuenta. El tratamiento de la hipercalcemia por adenoma paratiroideo se basa en reanimación hídrica adecuada y manejo quirúrgico del adenoma, con el fin de evitar recurrencia de pancreatitis y mortalidad. (AU)

Introduction: we describe the case of a patient with acute pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism. This is a rare cause of pancreatitis associated with significant morbidity and mortality if not diagnosed in time. Clinical case: a 44-year-old man with a history of pancreatitis of presumed biliary origin, which had previously required cholecystectomy, consulted for abdominal pain and nausea. The laboratory findings were compatible with a new episode of acute pancreatitis. He presented hypercalcemia and an elevated parathyroid hormone (PTH), configuring primary hyperparathyroidism. Scintigraphy was performed, yielding findings compatible with parathyroid adenoma. Treatment with fluid resuscitation and analgesia was started, resulting in an adequate decrease in serum calcium and resolution of abdominal pain. After parathyroidectomy, calcium and PTH levels were normalized. Discussion: acute pancreatitis is a potentially fatal condition; therefore the suspicion of rare causes, such as hypercalcemia, should be considered. The treatment of hypercalcemia due to parathyroid adenoma is based on adequate fluid resuscitation and surgical management of the adenoma, to avoid recurrence of pancreatitis and death. (AU)

Esclerosis bilateral de huesos largos: enfermedad de Erdheim-Chester. Reporte de un caso / Bilateral long bone sclerosis: Erdheim-Chester disease. Case report

La enfermedad de Erdheim-Chester (EEC) es una patología poco frecuente, caracterizada por presentar infiltración xantogranulomatosa sistémica, con afección de diversos sistemas incluido el óseo. La EEC se encuentra descripta dentro de las enfermedades osteocon-densantes (EO), las cuales se reconocen por presentar aumento de la masa ósea y compromiso tanto de huesos largos como planos. La presentación clínica de la EEC es variada: puede presentar desde un curso indolente hasta manifestaciones multisistémicas. Las características radiológicas son de gran importancia para establecer su diagnóstico. Presentamos una paciente con EEC, con esclerosis bilateral de huesos largos, que exhibe algunas características diferenciales con relación a otros casos reportados: a) afectación exclusivamente ósea a 10 años de evolución, b) compromiso bilateral y simétrico de distinta magnitud, c) esclerosis cortical endóstica y perióstica, d) signos radiológicos sugestivos de periostitis, d) ausencia de compromiso metafisario, e) ausencia de actividad metabólica de las lesiones en las imágenes de 18F-FDG PET/CT.Conclusión: la presencia de lesiones osteocondensantes bilaterales exclusivamente en huesos largos deben hacer sospechar EEC. La ausencia de compromiso metafisario y de actividad metabólica en 18F-FDG PET/CT ha sido raramente descripta. (AU)

Erdheim - Chester disease (ECD) is a rare disease, characterized by systemic xanthogranulomatous infiltration, with involvement of various organs including bone. ECD is described within the sclerosing bone disorders, which are recognized for presenting increased bone mass and involvement of both long and flat bones. The clinical presentation of ECD is diverse, ranging from an asymptomatic course to multisystemic manifestations. Radiological features are of great importance to establish the diagnosis. We describe here a patient with ECD, with bilateral sclerosis of long bones that presents some differential characteristics in relation to other reported cases: a) exclusively bone involvement at 10 years of evolution, b) bilateral and symmetric involvement of different magnitude, c) endosteal and periosteal cortical sclerosis d) radiological signs suggestive of periostitis, d) absence of metaphyseal involvement, e) absence of metabolic activity of the lesions in 18F-FDG PET/CT.Conclusion: the presence of bilateral osteosclerosis exclusively in long bones should lead to suspect ECD. The absence of metaphyseal involvement and metabolic activity in 18F-FDG PET/CT have been rarely described. (AU)

Early versus delayed post-therapy whole body scintigraphy for well-differentiated thyroid carcinoma: A meta-analysis

Introduction@#No clear consensus exists as to the optimal timing for conducting whole body scintigraphy (WBS) after radioactive iodine (RAI) therapy for differentiated thyroid carcinoma. @*Objective@#This study aimed to compare the utility of early versus delayed post-therapy WBS in identifying residual lesions and metastases.@*Methods@#A systematic review of existing literature was done, yielding 6 observational studies relevant to the subject. Meta-analyses were done comparing lesion detecting rates of early (3-4 days post-RAI) and delayed (7-11 days post-RAI) post-therapy WBS for thyroid remnants and metastases in the lymph nodes, lungs, and bone using a random-effects model with odds ratios (OR) and 95% confidence intervals (CIs). A subgroup analysis was also done relating to the type of collimator used in imaging.

Kummel's disease

Kummel's disease is a crush fracture of a vertebral body caused by a minor trauma, with the most accepted etiology being avascular necrosis. It is more frequent in individuals with risk factors such as osteoporosis or prolonged treatment with corticosteroids. Initially, it usually has normal radiological tests and an asymptomatic period, followed by a progressive onset of pain along with probable kyphosis and a sign of void or fluid abscess on radiological tests, which may create nerve/spinal involvement. The case is presented of a 76-year-old man, who was admitted to this center for the differential diagnosis of a single vertebral lesion. After imaging tests and biopsy, the definitive diagnosis of Kummel's disease was reached. The confirmatory diagnosis was reached by vertebral biopsy, but given its invasive nature, imaging techniques can play a significant role. As regards metabolic imaging tests, bone scintigraphy has shown to be one of the most sensitive tools to detect ischemia in earlier stages or to determine if it affects other locations. The whole body scan with diphosphonates shows an increase in activity in relation to bone remodeling activity in this condition. The 3-phase study makes it possible to differentiate whether it is an acute/subacute or chronic process, and can influence the therapeutic decision. Knowledge of this disease is important to make a differential diagnosis with tumour or infectious pathology, with emphasis on performing imaging tests in the event of persistent pain with a normal initial plaque.

La enfermedad de Kummel es una fractura-aplastamiento de un cuerpo vertebral precedida por un traumatismo menor, cuya etiología más aceptada es la necrosis avascular. Es más frecuente en individuos con factores de riesgo como osteoporosis o tratamiento prolongado con corticoides. Inicialmente, suele presentar pruebas radiológicas normales y un periodo asintomático, con aparición progresiva del dolor junto a probable cifosis y signo del vacío o absceso líquido en las pruebas radiológicas, pudiendo llegar a crear compromiso nervioso/medular. Presentamos el caso de un varón de 76 arios que ingresa en nuestro centro para el diagnóstico diferencial de una lesión única vertebral; tras la realización de las pruebas de imagen y biopsia se llega al diagnóstico definitivo de enfermedad de Kummel. El diagnóstico de confirmación de esta enfermedad se alcanza mediante la biopsia vertebral, pero dada su naturaleza invasiva, las técnicas de imagen toman un papel relevante. En relación con las pruebas de imagen metabólicas, la gammagrafía ósea ha demostrado ser una de las herramientas más sensibles para detectar isquemia en fases más tempranas o para conocer si afecta a otras localizaciones. El rastreo corporal de cuerpo completo con difosfonatos muestra un aumento de actividad en relación con la actividad ósea remodelativa en este cuadro. El estudio de tres fases permite diferenciar si se trata de un proceso agudo/subagudo o crónico, lo que influye en la decisión terapéutica. Es importante el conocimiento de esta enfermedad para realizar diagnóstico diferencial con patología tumoral o infecciosa e insistir en la realización de pruebas de imagen ante la persistencia del dolor con una placa inicial normal.

Aporte de la centellografía con trazadores tecneciados fosfatados al diagnóstico de amiloidosis cardíaca / Contribution of scintigraphy with technetium phosphated tracers to the diagnosis of cardiac amyloidosis / Contribuição da cintilografia com traçadores fosfatados com tecnécio para o diagnóstico de amiloidose cardíaca

Las amiloidosis son enfermedades causadas por el depósito patológico extracelular de un material proteico fibrilar e insoluble denominado amiloide, que puede estar vinculado a cadenas livianas (AL) o transtirretina (TTR). La amiloidosis cardíaca provoca una cardiomiopatía restrictiva de carácter progresivo caracterizada por falla cardíaca con función sistólica relativamente preservada, que se asocia a elevada mortalidad. Aunque el diagnóstico definitivo tradicionalmente se basa en la biopsia endomiocárdica, los avances en imagenología han mejorado su abordaje y la reciente introducción de terapias especificas permiten augurar cambios significativos en el pronóstico. El tratamiento difiere según el tipo de amiloide involucrado y su resultado depende de la instauración precoz de este, por lo cual resulta esencial un diagnóstico preciso y temprano. El centellograma cardíaco con fosfatos marcados (99mTc-PYP u otros), ampliamente disponible y de relativo bajo costo, se considera en la actualidad como una "biopsia molecular no invasiva" para el diagnóstico de la amiloidosis tipo ATTR, que debe ser usado en conjunto con la investigación de proteínas monoclonales en pacientes con sospecha clínica de la enfermedad.

Amyloidoses are diseases caused by the extracellular deposition of a fibrillar and insoluble protein material called amyloid, which can be linked either to light chains (AL) or transthyretin (TTR). Cardiac amyloidosis causes a progressive restrictive cardiomyopathy characterized by heart failure with relatively preserved systolic function, which is associated with high mortality. Although a definitive diagnosis is traditionally based on endomyocardial biopsy, advances in cardiac imaging have improved its approach, and the recent introduction of specific therapies predicts significant changes in prognosis. Since treatment differs according to the type of amyloid involved and the results depend on a prompt implementation, an accurate and early diagnosis is essential. Cardiac scintigraphy with labeled phosphates (99mTc-PYP or others), widely available and relatively inexpensive, is currently considered a "noninvasive molecular biopsy" for the diagnosis of ATTR type amyloidosis, which should be used in conjunction with investigation of monoclonal proteins in patients with clinical suspicion of the disease.

As amiloidoses são doenças causadas pela deposição patológica extracelular de um material proteico fibrilar e insolúvel, denominado amiloide, que pode estar ligado a cadeias leves (AL) ou transtirretina (TTR). A amiloidose cardíaca causa cardiomiopatia restritiva progressiva caracterizada por insuficiência cardíaca com função sistólica relativamente preservada, que está associada a alta mortalidade. Embora o diagnóstico definitivo seja tradicionalmente baseado na biópsia endomiocárdica, os avanços nos exames de imagem aprimoraram sua abordagem e a recente introdução de terapias específicas pode predizer mudanças significativas no prognóstico. O tratamento varia de acordo com o tipo de amiloide envolvida e seu resultado depende do início precoce, por isso um diagnóstico preciso e precoce é essencial. A cintilografia cardíaca com fosfatos marcados (99mTc-PYP ou outros), amplamente disponível e relativamente econômico, é atualmente considerada uma "biópsia molecular não invasiva" para o diagnóstico de amiloidose do tipo ATTR, que deve ser usada em conjunto com a investigação de proteínas monoclonais em pacientes com suspeita clínica da doença.

Nuevos avances en cirugía hepática: evaluación y planificación preoperatoria (Parte 1) / New advances in liver surgery: evaluation and preoperative planning

Resumen Los avances en la cirugía hepática de los últimos años han permitido resecciones hepáticas más extensa y complejas para el tratamiento de diferentes patologías del hígado sin un aumento excesivo de la morbimortalidad perioperatoria. El desarrollo de diferentes técnicas, tecnologías y herramientas para la evaluación preoperatoria han mejorado la planificación quirúrgica con el uso por ejemplo de las tecnologías audiovisuales e impresión de modelos en 3 dimensiones (3D) de alta fidelidad. Otros avances, han permitido realizar una mejor evaluación funcional del parénquima hepático y una caracterización más precisa de las lesiones con el uso por ejemplo de verde de indocianina, cintigrafía hepática y resonancia magnética con contraste hepatoespecífico. Este artículo describe algunos de los nuevos avances en la evaluación y planificación preoperatoria en cirugía hepática.

Advances in liver surgery in recent years have made it possible to achieve more extensive and complex liver resections for the treatment of different liver diseases without an excessive increase in perioperative morbidity and mortality. The development of different techniques, technologies and tools for preoperative evaluation have improved surgical planning with the use, for example, of audiovisual technologies and printing of high-fidelity 3-dimensional (3D) models. Other advances have allowed a better functional evaluation of the liver parenchyma and a more precise characterization of the lesions with the use, for example of indocyanine green or liver scintigraphy and magnetic resonance with hepatospecific contrast. This article describes some of the new advances in preoperative evaluation and planning in liver surgery.

Clinical efficacy of combined therapy in children with stage 4 neuroblastoma / 中国当代儿科杂志

OBJECTIVES@#To study the early clinical efficacy of combined therapy of stage 4 neuroblastoma.@*METHODS@#A retrospective analysis was performed on the medical data and follow-up data of 14 children with stage 4 neuroblastoma who were diagnosed in Hong Kong University-Shenzhen Hospital from January 2016 to June 2021.@*RESULTS@#The median age of onset was 3 years and 7.5 months in these 14 children. Among these children, 9 had positive results of bone marrow biopsy, 4 had N-Myc gene amplification, 13 had an increase in neuron-specific enolase, and 7 had an increase in vanilmandelic acid in urine. Based on the results of pathological examination, differentiated type was observed in 6 children, undifferentiated type in one child, mixed type, in one child and poorly differentiated type in 6 children. Of all the children, 10 received chemotherapy with the N7 regimen (including 2 children receiving arsenic trioxide in addition) and 4 received chemotherapy with the Rapid COJEC regimen. Thirteen children underwent surgery, 14 received hematopoietic stem cell transplantation, and 10 received radiotherapy. A total of 8 children received Ch14.18/CHO immunotherapy, among whom 1 child discontinued due to anaphylactic shock during immunotherapy, and the other 7 children completed Ch14.18/CHO treatment without serious adverse events, among whom 1 child was treated with Lu177 Dotatate 3 times after recurrence and is still undergoing chemotherapy at present. The median follow-up time was 45 months for all the 14 children. Four children experienced recurrence within 2 years, and the 2-year overall survival rate was 100%; 4 children experienced recurrence within 3 years, and 7 achieved disease-free survival within 3 years.@*CONCLUSIONS@#Multidisciplinary combined therapy is recommended for children with stage 4 neuroblastoma and can help them achieve better survival and prognosis.

Single-center, retrospective study on changes in pain-relieving therapy after bone metastasis detection by bone scintigraphy in prostate cancer patients

Abstract Whole-body bone scintigraphy (WB-BS) is used for detecting and monitoring metastatic spread of prostate cancer (PCa) and to investigate bone pain episodes. To investigate the impact of a positive WB-BS on pain-relieving medicine prescription in PCa patients, a single-center, retrospective cohort study with PCa patients classified as positive for bone metastases (BM) by WB-BS was conducted. Demographic, clinical, and ambulatory pain-relieving medicine prescription data were evaluated. Pain-relieving medicines were categorized according to the WHO 'Analgesic Ladder'. Regimens adopted before and after WB-BS were compared. Differences were considered significant at p<0.05. A total of 180 PCa patients were diagnosed with BM, 64.4% of whom were ≥65 years of age. Thirty-three patients were prescribed analgesics only after WB-BS, mostly including NSAIDs and weak opioids. Pain-relieving prescription changed after WB-BS in patients with prescriptions before and after WB-BS, with a reduction in NSAIDs and adjuvants and an increase in weak and strong opioids. In addition, 40% of patients with WHO analgesic step 1 drugs and 21.7% of patients with WHO step 2 drugs before WB-BS changed to other WHO steps after WB-BS. Pain-relieving prescriptions changed after a positive WB-BS, providing evidence that it could contribute to clinical management of painful metastatic PCa patients.

Síndrome de Sjögren en un adolescente: caso Clínico / Sjögren syndrome. report of one case

Sjögren Syndrome (SS), a slowly Progressive disease that has unified, validated diagnostic criteria, with excellent evidence and performance in adults, but not in adolescents. We report a 17 year old teenager with a family history of SS in his sister and mother. He presented with fever and fatigue. He had an elevated C reactive protein, leukopenia, positive antinuclear and anti-Rho antibodies and rheumatoid factor. A scintigraphy showed a severe salivary gland dysfunction. The syndrome in this patient had a pediatric clinical behavior despite his proximity to adulthood.

Aproximación diagnóstica a la amiloidosis cardiaca por transtiretina en un área no endémica. ¿Qué variables no invasivas orientan a su diagnóstico? / Diagnostic approach to transthyretin amyloidosis in a non-endemic area. Which non-invasive variables could guide the diagnosis?

Al editor: Clásicamente se ha considerado la amiloidosis cardiaca como una afección rara, con un amplio espectro de síntomas que requiere un alto índice de sospecha. Sin embargo, los estudios han demostrado que la amiloidosis cardiaca por transtiretina (TTR) es más común de lo que previamente se creía1,2. Las características clínicas que se han asociado a la amiloidosis cardiaca por TTR son el sexo masculino, la edad avanzada, la hipertrofia concéntrica y la función ventricular izquierda preservada1. Se realizó un análisis descriptivo retrospectivo de las gammagrafías solicitadas en nuestro centro para descartar amiloidosis cardiaca por TTR desde septiembre de 2016 hasta noviembre de 2019. En dicho periodo se realizaron 39 gammagrafías, con una tendencia al alza en los últimos meses. Los objetivos fueron evaluar las gammagrafías solicitadas y conocer el porcentaje de gammagrafías diagnósticas de amiloidosis por TTR, establecer qué características son más frecuentes en los pacientes con amiloidosis por TTR en nuestra población de referencia y analizar las características diferenciales de las distintas posibilidades diagnósticas. Del total de las pruebas, 22 (56.4% de la muestra) mostraron una captación de grado 2-3 de Perugini, diagnóstica de amiloidosis por TTR. De acuerdo con las recomendaciones de diagnóstico no invasivo de amiloidosis cardiaca por TTR3, se descartó la presencia de pico monoclonal. Únicamente se realizó estudio genético a 10 pacientes, en dos de los cuales se detectó una mutación patogénica (Val50Met y variante patogénica c.290C>A en heterocigosis); los ocho restantes no mostraron mutaciones en el estudio molecular del gen TTR.

Concordance between estimated glomerular filtration rate using equations and that measured using an imaging method / Concordancia entre la tasa de filtración glomerular estimada con ecuaciones y aquella medida mediante imágenes

ABSTRACT Background: Guidelines recommend estimating glomerular filtration rate (GFR) using creatinine-based equations (CBE). AIM: To evaluate the agreement between GFR measured using radionuclide imaging and estimated using creatinine-based equations. MATERIAL AND METHODS: In 421 patients aged 54 ± 17 years (47% women) GFR was estimated using the MDRD-4, CKD-EPI and the body surface adjusted Cockroft Gault equation. GFR was also measured using a radionuclide imaging method with 99mTc-DTPA. The concordance between estimated and measured GFR was calculated using Lin's concordance coefficient and Bland and Altman plots. RESULTS: Average GFR values obtained with CKD-EPI, MDRD-4, body surface adjusted Cockroft Gault equation and 99mTc-DTPA imaging were 75.9 ± 26.6, 76.3 ± 28.8, 77.1 ± 31.6 and 77.9 ± 28.4 ml/min/1.73 m2, respectively. There was no significant difference in means and 29% of participants had a GFR < 60 ml/min/1.73 m2 by CKD-EPI. The correlation was good between equations, but acceptable when compared with the 99mTc-DTPA imaging. The weighted kappa between CBEs was good, but low when comparing CBEs with measured GFR. The Lin's concordance coefficient between estimated and measured GFR was low. Conclusions: GFR measured by 99mTc-DTPA radionuclide imaging has a low correlation and poor concordance with estimations using CBE.

ANTECEDENTES: Las guías clínicas recomiendan estimar la tasa de filtración glomerular (TFG) usando ecuaciones basadas en la creatinina sérica. Objetivo: Estudiar la concordancia entre la TFG medida usando un método de imágenes usando radioisótopos y aquella estimada con ecuaciones. MATERIAL Y MÉTODOS: En 421 pacientes de 54 ± 17 años (47% mujeres), la TFG se estimó utilizando las ecuaciones MDRD-4, CKD-EPI y Cockroft Gault ajustada para superficie corporal. La TFG se midió también con una técnica de imágenes usando 99mTc-DTPA. La concordancia entre la estimación y medición se calculó usando el coeficiente de concordancia de Lin y gráficos de Bland y Altman. RESULTADOS: Los promedios de TFG obtenidos con CKD-EPI, MDRD-4, ecuación de Cockroft Gault e imágenes con 99mTc-DTPA fueron 75,9 ± 26,6, 76,3 ± 28,8, 77,1 ± 31,6 y 77,9 ± 28,4 ml/min/1,73 m2, respectivamente. No hubo diferencias significativas en los promedios y el 29% de los participantes tuvo una TFG < 60 ml/min/1,73 m2. La correlación entre las ecuaciones fue buena, pero sólo aceptable cuando se comparó con la medición por imágenes. El kappa ajustado entre las ecuaciones fue adecuado, pero malo cuando se comparó las ecuaciones con la medición por imágenes. El coeficiente de Lin mostró una baja concordancia entre la estimación y medición de TFG. Conclusiones: La concordancia entre la estimación de TFG usando ecuaciones y su medición directa mediante imágenes es baja.

The Role of I-131 MIBG cardiac scintigraphy in diagnosing dementia with lewy bodies : A case report

@#The objective of this case report is to highlight the role of Iodine-131 metaiodobenzylguanidine (MIBG) cardiac scintigraphy in discriminating Dementia with Lewy Bodies (DLB) from other neurodegenerative diseases such as Alzheimer’s Disease. This patient is a known case of Parkinson’s disease and has been treated as such since 2011. However, the patient also concurrently deals with visual hallucinations and because of this, the patient’s attending neurologist wanted to rule in the diagnosis of DLB rather than AD. Hence, an I-131 MIBG cardiac scan was requested in order to support the diagnosis of DLB. The use of I-131 MIBG cardiac scintigraphy as a diagnostic tool for diagnosing Lewy Body Dementia is not prevalent and to our knowledge, this was the first time in the country that this procedure was done (December 9, 2019).

Application of molecular probes in nuclear imaging of neuroendocrine tumors / 浙江大学学报·医学版

Neuroendocrine tumors are a type of heterogeneous tumors originating from neuroendocrine cells derived from the neural crest，which can secrete a variety of amines and peptide hormones.Based on different molecular biomarkers，histologic types and differentiation degrees，individualized nuclear imaging can provide information for the early diagnosis，clinical staging，treatment guidance，and detection of the recurrence and metastasis of neuroendocrine tumor. In this paper，we review the development and application of nuclear medicine molecular imaging probes such as glucose analogs，somatostatin analogues，amine precursors，hormone analogs and enzyme inhibitors in the diagnosis and treatment of neuroendocrine tumors.

Clinical features of neurogenic bladder with vesicoureteral reflux in children / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features of vesicoureteral reflux (VUR) in children with neurogenic bladder (NB), and to provide a reference for its early diagnosis and treatment.@*METHODS@#Clinical data were collected from 26 children with NB and urinary tract infection who were admitted to the Department of Pediatric Nephrology from January 2014 to December 2019. According to the presence or absence of VUR, the children were divided into a VUR group with 11 children and a non-VUR group with 15 children. Clinical features were compared between the two groups.@*RESULTS@#Compared with the non-VUR group, the VUR group had a significantly higher proportion of children with non-@*CONCLUSIONS@#When NB children have the clinical manifestations of non-