[Hepatic glycogenosis: a case report]. / Glucogenosis hepá tica: a propósito de un caso.

The glycogen storage diseases contain a range of diseases that are characterized by the abnormal storage or utilization of glycogen, the organs most affected being muscle and / or liver. Hepatomegaly may be a clinical sign that could guide to the diagnosis. We describe a 15-year-old patient with hepatomegaly, hypertransaminasemia and growth retardation. He was diagnosed with a glycogen storage disease by liver biopsy.

Pediatric Kidney Transplantation in Perú: A Single-Center Initial Experience.

BACKGROUND: Pediatric kidney transplantation (PKTx) is the preferred therapy for children with end-stage renal disease (ESRD) worldwide. Regrettably, in Perú, access to PKTx is extremely difficult due to recipient/donor socio-economic status, health care structure and especially, scarcity of organs. Our center (the only pediatric institute in the country) has recently started a PKTx program with good midterm results. The aim of this study was to present our outcomes. METHODS: Retrospective analysis of prospectively collected data between December 2017 and August 2019. Fourteen PKTx (< 18 years old) were achieved. As per our protocol: pre-implantation/protocol biopsies, antibody assessment (T/B cell flow cytometric plus HLA testing applying polymerase chain reaction-based technology), triple immunosuppression (tacrolimus, mycophenolate mofetil, steroids) and induction therapy was performed in every case. RESULTS: The recipient's mean age at the time of PKTx was 14.14 ± 2.62, 8/14 (57.14%) were male, 50% developed ESRD due to undetermined etiology, 11/14 (78.57%) received a deceased donor allograft, and 9/14 (64.28%) required induction with thymoglobulin. Postoperative complications included: delayed graft function (1/14, 7.14%), 1 (7.14%) developed gross hematuria associated with allograft disfunction post-protocol allograft biopsy that was managed conservatively and 1 recipient (7.14%) developed grade II oligoastrocytoma, at 10 months post PKTx. CONCLUSIONS: PKTx is the best therapeutic option for children with ESRD. Our group demonstrated that even in countries with limited resources like Perú, good midterm results can be achieved. Emphasis should be given to improve access to transplantation especially in the setting of pediatric recipients.

Glucogenosis hepática: a propósito de un caso / Hepatic glycogenosis: a case report

RESUMEN Las glucogenosis abarcan un rango de enfermedades que se caracterizan por el almacenamiento o utilización anormal del glucógeno, siendo los órganos más afectados el músculo y/o el hígado. La hepatomegalia puede ser un signo clínico que guie al diagnóstico. Describimos a un paciente de 15 años de edad con hepatomegalia, hipertransaminasemia y retraso del crecimiento, a quien se le diagnosticó glucogenosis por biopsia hepática.

ABSTRACT The glycogen storage diseases contain a range of diseases that are characterized by the abnormal storage or utilization of glycogen, the organs most affected being muscle and / or liver. Hepatomegaly may be a clinical sign that could guide to the diagnosis. We describe a 15-year-old patient with hepatomegaly, hypertransaminasemia and growth retardation. He was diagnosed with a glycogen storage disease by liver biopsy.

Criptococosis cutánea localizada en paciente trasplantada renal: una entidad infrecuente / Localized cutaneous cryptococcosis in a patient who underwent a kidney transplantation: an unusual condition

La criptococosis cutánea es una entidad infrecuente en pacientes receptores de trasplante de órganos sólidos (TOS). La forma cutánea localizada es descrita en pacientes inmunocomprometidos y probablemente sea secundaria a una reactivación de un estado latente en ganglios linfáticos o pulmón, además requiere exclusión de compromiso sistémico. Presentamos el caso de una mujer de 40 años receptora de trasplante renal hace 1 año, en inmunosupresión con tacrolimus y prednisona, que desarrolló en 1 semana lesión en cara interna de muslo izquierdo sugestiva de celulitis bacteriana. Se inició tratamiento antibiótico de amplio espectro, pero la lesión progresó a una úlcera necrótica con necesidad de limpieza quirúrgica. La biopsia cutánea evidenció dermatitis nodular crónica granulomatosa no caseificante con múltiples levaduras de criptococo. Se aisló Cryptococcus neoformans y se inició tratamiento antifúngico con respuesta favorable. Es necesario establecer estrategias de screening para prevenir la infección por criptococo en receptores de TOS

Cutaneous cryptococcosis is an uncommon condition in patients undergoing solid-organ transplantation (SOT). The localized cutaneous form has been described in immunosuppressed patients, and it may likely be secondary to a reactivation of a dormant infection in the lymph nodes or in the lungs; also, systemic involvement should be ruled out. We present the case of a 40-year old woman who underwent a renal transplantation one year ago. She received tacrolimus and prednisone for rejection control, and after one week she developed a lesion in the internal aspect of her left thigh, which suggested a bacterial skin and soft-tissues infection. Wide spectrum antimicrobials were started, but the lesion progressed to a necrotic ulcer which had to undergo surgical cleansing. The skin biopsy revealed chronic non-caseating nodular granulomatous dermatitis, with the presence of multiple Cryptococcus yeasts. Cryptococcus neoformans was isolated in the cultured material and antifungal therapy was started. A satisfactory response was achieved. It is necessary to establish screening strategies aiming to prevent cryptococcal infections in patients undergoing SOT

Colangiocarcinoma en tercio medio de la vía biliar principal tratado con cirugía radical en el Hospital Nacional Guillermo Almenara, Lima, Perú / Cholangiocarcinoma in the middle third of the bile duct treated with radical surgery in Guillermo Almenara Hospital, Lima, Peru

Reportamos el caso de una paciente en quien se hizo el diagnóstico inicial de tumoración en vía biliar principal en su tercio medio. Paciente se presentó con baja ponderal de 10 kilogramos en 2 meses y dolor moderado en epigastrio, no ictericia. El paciente fue sometido a cirugía radical de la vía biliar con biopsia por congelación múltiple de bordes quirúrgicos, coledocoscopía intraoperatoria, colangiografía intraoperatoria y reconstrucción con anastomosis bilio-digestiva en Y de Roux transmesocólica, tuvo una recuperación sin complicaciones y alta precoz. El resultado anátomo-patológico evidenció adenocarcinoma tubular moderadamente diferenciado sobre la base de un adenoma. Carcinoma in situ sobre la base del adenoma. Ganglios retroperitoneales, retropancreáticos, pericoledocianos, curvatura menor y arteria hepática negativos. Bordes quirúrgicos y ampliación de bordes proximal y distal libres de neoplasia. Cirugía R0. pT1N0Mx. Estadio 1. Después de los resultados quirúrgicos óptimos es manejado por cirugía de hígado y vías biliares y oncología médica para seguimiento y controles periódicos. Presentamos aquí la secuencia de hechos y una revisión de la literatura...

We report the case of a patient who had the initial diagnosis of tumor in the bile duct in the middle third. Patient presented with lost weight of 10 kilograms in two months and moderate epigastric pain, no jaundice. The patient underwent radical surgery of the bile duct with multiple freeze biopsy surgical margins, intraoperative choledochoscopy, intraoperative cholangiography and reconstruction bilioenteric anastomosis Y Roux transmesocolic, he had a great recovery and early discharge. The pathological results showed moderately differentiated tubular adenocarcinoma on the basis of an adenoma. Negatives retroperitoneal, retropancreatic, pericholedochal, lesser curvature and negative hepatic artery nodes, and extension of surgical margins free of neoplasia proximal and distal edges. R0 surgery. pT1N0Mx. Stage 1. After the optimal surgical outcomes, is managed by liver and biliary tract surgery service and medical oncology service for regular monitoring and controls. We present here the sequence of events and a review of the literature...

[Cholangiocarcinoma in the middle third of the bile duct treated with radical surgery in Guillermo Almenara Hospital, Lima, Peru]. / Colangiocarcinoma en tercio medio de la vía biliar principal tratado con cirugía radical en el Hospital Nacional Guillermo Almenara, Lima, Perú.

We report the case of a patient who had the initial diagnosis of tumor in the bile duct in the middle third. Patient presented with lost weight of 10 kilograms in two months and moderate epigastric pain, no jaundice. The patient underwent radical surgery of the bile duct with multiple freeze biopsy surgical margins, intraoperative choledochoscopy, intraoperative cholangiography and reconstruction bilioenteric anastomosis Y Roux transmesocolic, he had a great recovery and early discharge. The pathological results showed moderately differentiated tubular adenocarcinoma on the basis of an adenoma. Negatives retroperitoneal, retropancreatic, pericholedochal, lesser curvature and negative hepatic artery nodes, and extension of surgical margins free of neoplasia proximal and distal edges. R0 surgery. pT1N0Mx. Stage 1. After the optimal surgical outcomes, is managed by liver and biliary tract surgery service and medical oncology service for regular monitoring and controls. We present here the sequence of events and a review of the literature.

High proportion of T-cell systemic non-Hodgkin lymphoma in HIV-infected patients in Lima, Peru.

OBJECTIVE: Few reports have described the clinical and pathologic characteristics of HIV-related systemic non-Hodgkin lymphoma (sNHL) in developing countries. We aimed to determine these characteristics from a national HIV reference center in Peru and to evaluate factors associated with survival. METHODS: A retrospective/prospective study of patients with HIV-related sNHL from the Guillermo Almenara General Hospital in Lima, Peru between 1993 and 2004. Clinical characteristics at diagnosis included age, gender, risk behavior, previous AIDS diagnosis, opportunistic diseases, previous highly active antiretroviral therapy, Karnofsky score, origin, clinical stage and B-cell symptoms of sNHL, and CD4 cell count. Cases of sNHL were classified according to the criteria of the World Health Organization. RESULTS: Thirty-three cases were identified (26 male, age range: 38 +/- 10 years). Ten patients (30%) had a prior history of AIDS, 14 (42%) had a Karnofsky score of