Taquipnea persistente como manifestación inicial de hiperplasia de células neuroendocrinas: reporte de un caso clínico / Persistent tachypnea as initial manifestation of hyperplasia of neuroendocrine cells: case report

Se realiza revisión de la literatura y presentación de un caso clínico de Hiperplasia de Células Neuroendocrinas en paciente lactante masculino que inicia su padecimiento a los 3 meses de vida con dificultad respiratoria caracterizada por retracciones subcostales y taquipnea persistente, posterior-mente a los 8 meses de edad se agrega hipoxemia respirando aire ambiente que requiere uso de oxígeno suplementario continuo. Tiene antecedente de tres hospitalizaciones, con diagnóstico de Bronquiolitis y Neumonía atípica, realizándose panel viral respiratorio con reporte negativo. El paciente persiste con sintomatología respiratoria a pesar de tratamientos médicos, por lo que se deriva a neumología pediátrica, unidad de enfermedad pulmonar intersticial del lactante, iniciando protocolo de estudio, se realiza tomografía tórax de alta resolución, que evidencia imágenes en vidrio despulido en lóbulo medio y región lingular, además de atrapamiento aéreo. Se concluye el diagnóstico de Hiperplasia de Células neuroendocrinas con base a la clínica y hallazgos tomográficos. La Hiperplasia de Células Neuroendocrinas es una patología pulmonar intersticial poco frecuente, cuyo diagnóstico es clínico y radiológico, en la minoría de los casos se requiere biopsia pulmonar para confirmación. Puede ser fácilmente confundida con otras enfermedades respiratorias comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en los primeros años de vida.

A review of the literature and presentation of a clinical case of Neuroendocrine Cell Hyperplasia in a male infant patient who begins his condition at 3 months of age with respiratory distress characterized by subcostal retractions and persistent tachypnea is presented. After 8 months of age hypoxemia is added requiring continuous oxygen therapy. He has a history of three hospitalizations, with a diagnosis of bronchiolitis and atypical pneumonia, respiratory viral panel has a negative report. The patient persists with respiratory symptoms despite medical treatments, so it is referred to pediatric pulmonology, initiating study protocol for interstitial lung disease of the infant. A high resolution chest tomography is performed, which evidences images in polished glass in the middle lobe and lingular region, in addition to air entrapment. The diagnosis of neuroendocrine cell hyperplasia is concluded based on clinical and tomographic findings. Neuroendocrine Cell Hyperplasia is a rare interstitial pulmonary pathology, whose diagnosis is clinical and radiological. Lung biopsy is required only in the minority of cases for confirming diagnosis. It can be easily confused with other common respiratory diseases, so it is important to suspect it to make an early diagnosis. Most cases evolve with decline in symptoms, improving spontaneously in the first years of life.

Modificación de la actividad metabólica por medio de un dispositivo intraoral en pacientes con hiperplasia condilar confirmada por SPECT / Metabolic activity modification by means of an intraoral device in patients with condylar hyperplasia confirmed by SPECT

Objetivo: Evaluar los efectos de la aplicación de un dispositivo intraoral de uso permanente en el comportamien- to de los cóndilos con hiperplasia condilar (HC) confirmada por tomografía computarizada de emisión por fotón único (SPECT), estableciendo una comparación con un grupo de pacientes con HC que no utilizó el dispositivo. Materiales y métodos: 30 pacientes con una edad promedio de 21,7 años (+/-5,56) con HC confirmada con SPECT fueron asignados al azar a dos grupos: a los del grupo I (n=18) se les colocó un dispositivo intraoral de uso perma- nente para modificar la posición de la mandíbula, mientras que a los del grupo II (n=12) no se les colocó ningún dispo- sitivo. Se realizaron evaluaciones de dolor, del desvío de la línea media, de la apertura máxima y del disconfort al inicio del estudio y a los 2, 4, 6, 10, 12 y 14 meses. A los 19 meses promedio, la actividad osteoblástica (AO) fue reevaluada me- diante SPECT. Resultados: En el grupo I, la AO en los cortes coro- nales y transversales cesó o disminuyó (p<0,001) respecto a la condición inicial, mientras que en el grupo II la AO au- mentó (p<0,001). Los datos fueron analizados utilizando el test de Wilcoxon de rangos signados. Al ajustar un modelo de ANCOVA robusto utilizando el valor inicial como covariable también se observa que el efecto del grupo fue estadística- mente significativo en ambos cortes (p<0,001). Conclusiones: La aplicación de un dispositivo intrao- ral de uso permanente mejora la evolución de la hiperplasia condilar, lo que lo puede convertir en un tratamiento de uti- lidad para el tiempo que se aguarda para realizar una condi- lectomía alta de cuello de cóndilo, o incluso para evitar este procedimiento (AU)

Objective: To evaluate the effects of the application of an intraoral device for permanent use on the behavior of con- dyles with condylar hyperplasia (CH) confirmed by single photon emission computed tomography (SPECT), establish- ing a comparison with a group of patients with CH that did not use the device. Materials and methods: Thirty patients with an aver- age age of 21.7 years (+/-5.56) with CH confirmed by SPECT were randomly divided into two groups: the ones in group I (n=18) received an intraoral device for permanent use to align the mandible, while those in group II (n=12) did not get any device. Pain, midline shift, maximum opening, and discomfort were evaluated at the beginning of the study and at 2, 4, 6, 10, 12, and 14 months. At an average of 19 months, osteoblastic activity (AO) was reassessed by SPECT. Results: In group I, the AO in the coronal and trans- verse sections ceased or decreased (p<0.001) in comparison to the initial condition, while in group II the AO increased (p<0.001). The data was analyzed by the Wilcoxon signed rank test. Adjusting a robust ANCOVA model using the ini-tial value as a covariate made it possible to observe that the effect of the group was statistically significant in both cuts (p<0.001). Conclusions: The application of an intraoral device for permanent use improves the evolution of condylar hyperpla- sia, which can make it a useful treatment until a high condylectomy of the neck of the condyle is performed, or even to avoid this procedure (AU)

Hiperplasia de hipófise secundária a hipotireoidismo primário - relato de caso / Pituitary hyperplasia secondary to primary hypothyroidism - case report

A hiperplasia hipofisária é definida como um aumento não neoplásico no número de um dos tipos de células presentes na hipófise. Ela pode ocorrer por um processo fisiológico ou patológico. O hipotireoidismo primário prolongado é uma das causas patológicas desta condição, e ocorre devido a perda do feedback negativo. O objetivo desse relato foi demonstrar a presença de hiperplasia hipofisária em um paciente masculino com características corporais sugestivas de acromegalia. A investigação laboratorial confirmou a presença de hipotireoidismo primário e descartou a acromegalia. Foi instituído tratamento com levotiroxina, levando a regressão da hiperplasia hipofisária. Esse caso ilustra a importância de uma investigação apropriada em pacientes com hiperplasia hipofisária, bem como discute a fisiopatologia e o tratamento dessa doença.

Pituitary hyperplasia is defined as a non-neoplastic increase in the number of one of the cell types present in the pituitary gland. It can occur by a physiological or pathological process. Prolonged primary hypothyroidism is one of the pathological causes of this condition and occurs due to the lack of negative feedback. The objective of this report was to demonstrate the presence of pituitary hyperplasia in a male patient with body characteristics suggestive of acromegaly. Laboratory investigation confirmed the presence of primary hypothyroidism and ruled out acromegaly. Treatment with levothyroxine was instituted, leading to regression of pituitary hyperplasia. This case illustrates the importance of an appropriate investigation in patients with pituitary hyperplasia, as well as discussing the pathophysiology and treatment of this disease.

Análisis de falsos negativos en la cintigrafía SPECT de paratiroides con sestamibi en pacientes con hiperparatiroidismo primario sometidos a cirugía entre 2008-2015 en hospital universitario / False negative spect parathyroid scintigraphy with sestamibi in patients with primary hyperparathyroidism

Background: 99mTc-sestamibi parathyroid SPECT scintigraphy is a useful tool in the pre-operative study of hyperparathyroidism. False negatives (FN) have been reported in 5.7-14% of the examinations. Aim: To characterize 99mTc-sestamibi FN in cases referred for primary hyperparathyroidism (PHP) to a university hospital. Material and Methods: Descriptive retrospective analysis. We included patients with PHP, studied with SPECT scintigraphy, operated at our center between 2008 and 2015. Clinical and surgical data were recorded; biopsies of the FN were blindly reviewed by one pathologist. Results: One hundred twenty one scintigraphies fulfilled the inclusion criteria. Seven (5.8%) were negative and 114 positive. There was no difference in age, sex and PTH levels between FN and true positive scintigraphies. At surgery, one FN case had two hyperplasic glands and two cases had ectopic glands. Pathology reported adenoma in three cases, hyperplasia in three and carcinoma in one. The largest diameter of the lesion was lower in FN (1.3 and 2.1 cm respectively, p = 0.02) and the proportion of adenomas was higher in true positive cases (29% and 75% respectively; p < 0.01). The interval between scintigraphy and parathyroidectomy was greater in FN with a median of 92 days (range 20 days-3.2 years, p < 0.01). The percentage of oxyphilic cells observed was similar in both groups. Conclusions: FN parathyroid SPECT scintigraphies in PHP are uncommon. They corresponded to lesions under the equipment's resolution limit and resulted in longer time lags between scintigraphy and surgery.

Crossed hemifacial hyperplasia: a diagnostic dilemma.

Crossed hemifacial hyperplasia is a rare condition producing facial asymmetry and overgrowth of the extremities of the opposite side. Very few cases are reported in literature. A case of an 11-year-old female is presented here to supplement existing clinical knowledge with many of the reported clinical and orofacial findings. Emphasis is placed on diagnosis of the condition by thoughtful elimination and the necessity of a multi-disciplinary approach to its management.