Renal medullary carcinoma: a nearly fatal malignancy specifically affecting patients with a so-called benign condition.

Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis. There is very limited experience treating disseminated disease and the prognosis is dismal. We report the case of a young 9-year-old boy with SCT, who presented with 4 months' progression of abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory distress. Cervical lymphadenopathy was evident and abdomen was diffusely tender. A whole-body CT scan showed a left kidney lesion with associated cervical, mediastinal, and retroperitoneal lymphadenopathy. Biopsy of a cervical lymph node revealed metastatic RMC. Patient was started on combination chemotherapy with paclitaxel, carboplatin, and gemcitabine followed by left adrenalectomy. In spite of having advanced disease, our patient achieved an excellent response with a progression-free survival of 17 months. Although SCT is thought to be a "benign" condition, RMC is one devastating complication associated with it. Considering its rarity, the near uniform associated fatality should prompt the question of whether clinical practice should change regarding proper counseling of these patients and raise awareness in the medical community.

The symptom experiences of Puerto Rican children undergoing cancer treatments and alleviation practices as reported by their mothers.

Although symptoms during cancer treatments are prevalent and are important clinical outcomes of childhood cancer, the symptom experiences of Puerto Rican children along with the symptom alleviation/care practices that parents provide during cancer treatments have received limited attention. To examine the occurrence/severity of symptoms on the Therapy-Related Symptom Checklist-Children (TRSC-C), reported by mothers of Puerto Rican children undergoing cancer treatments and identifying mothers' symptom alleviation/management strategies. Descriptive study conducted between January and May 2012. Mothers of 65 Puerto Rican children/adolescents undergoing cancer treatments responded to the Spanish versions of the TRSC-C, Symptom Alleviation: Self-Care Methods, and a Demographic and Health form. The children/adolescents' mean age was 9.2 (1-17) years; 62% were boys; 56 had chemotherapy; 9 had chemoradiotherapy. Children diagnoses were 35.4% leukemia, 24.6% solid tumors, 24.6% nervous system tumors, and 15.4% other. On the TRSC-C, the symptoms experienced by 70% or more of the children were: irritability (77%), nausea (75%), and hair loss (72%). On the Symptom Alleviation: Self-Care Methods, the most commonly reported symptom alleviation category was "taking prescribed medicines." Puerto Rican mothers reported the use of alleviation practices to treat their children experiencing symptoms during pediatric cancer treatments. Patients and caregivers need to be educated about treatment-induced side effects, and the life-threatening consequences of underreporting and undermanagement. Symptoms should always be addressed at the time of initiation of primary or adjuvant cancer therapy because pretreatment symptoms may persist or get worse across the trajectory of treatment. A continuous assessment and management of symptoms during the childhood cancer trajectory can optimize clinical care and improve quality of life of patients and families.

Synchronous multicentric osteosarcoma in Puerto Rico: a pediatric case report.

Synchronous multicentric osteosarcoma is a very rare condition that might be confused with metastatic osteosarcoma. We report the first pediatric case of synchronous multicentric osteosarcoma reported in the Puerto Rican. The child presented with multiple osteoblastic bone lesions followed by right upper lid ptosis. Orbital CT-Scan showed a lytic lesion involving the sphenoid bone. Management consisted of chemotherapy for forty-two weeks after which she remained without new secondary bone lesions.

Pilomyxoid astrocytoma in unusual location in a child with neurofibromatosis type 1: case report and review of the literature.

Pilomyxoid astrocytoma (PMA) is a recently defined brain tumor believed to be a variant of pilocytic astrocytoma (PA), but with a more aggressive course. Most PMAs occur in the optic-chiasmatic/hypothalamic (OCH) region but they have also been described in the posterior fossa, temporal lobe, and in the spinal cord. We report a girl with history of neurofibromatosis type 1 (NF-1) who presented with a PMA located in the left lateral ventricle. Despite the fact that most of PMAs occur in the hypothalamic region, high awareness should be given to lesions in unusual locations, thus expanding the current epidemiologically known locations for this tumor.

Perioperative outcomes in octogenarians undergoing cardiac surgery in Puerto Rico

With the advances in surgical techniques and the advent of an aging population, the application of cardiac surgeries has broadened to include those patients over the age of 80. In order to characterize the in-hospital morbidity and mortality of elderly patients undergoing cardiac surgery at our cardiovascular center, a retrospective review of all medical records of consecutive octogenarian patients was made. Between January 1998 and April 1999, 76 patients (mean age (SD), 82.5 (2.6)) underwent cardiac surgery, of which, 61.8 percent were males. Surgical procedures consisted of isolated coronary artery bypass grafting (CABG)in 64 patients, isolated aortic valve replacement (AVR) in 4 patients, combined CABG and left carotid endarterectomy in 4 patients, combined CABG and AVR in 2 patients, combined CABG and aortic aneurysm repair in 1 patient and combined AVR and aortic dissection repair in 1 patient. Surgery was elective in 51.3 percent of patients and seventy-one percent (54) experienced at least one postoperative complication. The most common complication was atrial arrhythmia (28) followed by low cardiac output (23) and pneumonia (16). Hospital mortality occurred in 11 (14.7 percent) patients, an estimate in agreement with previous published studies on octogenarians. Since the number of patients studied was small, these results require confirmation by other cardiovascular centers in the island. Further research to determine independent predictors of postoperative morbidity and mortality, to assess survival and quality of life after cardiac surgery and to evaluate cost-effectiveness considering new trends in managed health care is warranted.

Presencia del fenómeno de prozone en pruebas de VDRL en recien nacidos en el hospital Dr. Luis E Aybar / Presence of the Prozoneïs phenomenon in VDRL tests in newborns at Dr. Luis E. Aybar Hospital

De enero a abril de 1993 se realizó una revisión prospectiva y analítica en el área de perinatología del Servicio de Pediatría del Hospital Dr. Luis E. Aybar en Santo Domingo R.D. con el objetivo de comparar suero del cordón umbilical, suero tomado directamente del recién nacido y suero materno, para el diagnóstico de sífilis congénita y descubrir disparidad en los resultados serológicos falso-negativo y falso-positivo que se presentan al a ser comparados los especímenes que fueron enviados para prueba de VDRL. Se estudiaron 461 pares o parejas de madre-recién nacido, de los cuales 22 pares presentaron VDRL reactivo en cada miembro de la pareja para una frecuencia general de sífilis en mujeres embarazadas de 4.8//, de las cuales 13 (59//) de las parejas presentaron disparidad entre los resultados. De las parejas 9 (40.9//), presentaron las 3 pruebas positivas, 11 (//0//) recién nacidos (RN) presentaron disparidad en resultados entre suero del cordón (SC) y de sangre periferica (SP) tomada directamente del R N. 5 (22.7//) tenían VDRL del SC positivo y de la SP negativo (falso positivo); 6 (27.3//) tenían VDRL del SC negativo y de la SP positivo (falso negativo). Mientras sus madres presentaron VDRL positivo, 5 (22.7//) R N tenían muestran del SC negativas y mientras 5 madres tenían el VDRL negativo, sus RN tenían el VDRL positivo bien sea en SC o SP. Es posible que algunos de estos falsos negativos sean debidos al fenómeno de prozone

Riesgo y prevalencia de isoinmunización en madres Rh negativas / Risks and prevalence of isoimmunization in rh negative mothers

Se estudiaron 2518 madres que se desembarazaron en el Servicio de Obstetricia del Hospital Dr. Luis E. Aybar en el período comprendido entre el 1 de Junio al 31 de Diciembre 1992, de las cuales 119 resultaron ser Rh neg, para una prevalencia de 4.7//. De estas 119 madres, 3 resultaron con el Test de Coombs indirecto positivo, para una prevalencia de isomunización de 2.5//. De estas 119 madres, 49 que tuvieron hijos Rh pos, pudieron ser seguidas por 6 meses post parto y 4 de ellas al término de este período resultaron con el test de Coombs indirecto positivo, para una prevalencia de 8.1//de isoinmunización y un riesgo de p=0.08

Retraso de crecimiento intrauterino, en el hospital Dr. Luis E. Aybar, junio-diciembre 1992 / Fetal growth retardation : in the Hospital Dr. Luis E. Aybar, June-December 1992

Se realizó un estudio prospectio en el periodo junio-diciembre de 1992 en el Servicio de Pediatria del Hospital Dr. Luis E. Aybar sobre retraso de crecimiento intrauterino en los recién nacidos en el Servicio de Obstetricia de este hospital. En el periodo señalado hubo 2,463 nacimientos de los cuales 227 fueron de bajo peso (BP) (peso menor de 2,500 G), para un 9.2 por ciento de los nacimientos y una tasa de 92 por mil. De estos 227 recien nacidos BP, 101 fueron pequeños para su edad gestacional (PEG) para un 44 por ciento de los BP y una tasa de 41 por mil nacimientos; los restantes 126 fueron adecuados para su edad gestacional (AEG), para un 56 por ciento de los BP y una tasa de 51 por cada mil nacimientos. De los 101 PEG, 70 por ciento fueron simétricos, 85 por ciento nacieron a termino y 72 por ciento fueron varones. En este estudio utilizamos la tabla de Lubchenco para la clasificación de los recien nacidos