Compromiso renal atípico en amiloidosis de cadena ligera

Introducción la amiloidosis de cadena ligera (AL) es una entidad desencadenada por la proliferación de un clon de células plasmáticas que genera la acumulación de cadenas ligeras, las cuales se depositan en forma de fibrillas amiloides generando una disfunción orgánica. El compromiso renal generalmente se manifiesta como síndrome nefrótico, con un deterioro lento y progresivo de la función renal que puede llevar a un requerimiento de terapia dialítica. Objetivo demostrar el compromiso renal agresivo y subagudo de la amiloidosis sistémica. Presentación del caso paciente masculino de 35 años que consulta por malestar general, visión borrosa, mareos y oliguria con elevación de azoados, y que progresó rápidamente hasta el requerimiento de hemodiálisis en aproximadamente tres meses. Asociado se documenta polineuropatía periférica, infiltración cardiaca y ligera elevación de transaminasas. Ante negatividad de estudios de extensión, se logra documentar la presencia histopatológica de depósitos amiloides con inmunofluorescencia positiva para AL. Actualmente, se encuentra recibiendo esquema de quimioterapia con adecuada estabilidad clínica y tolerancia. Discusión y conclusión la amiloidosis AL es una entidad infrecuente, con compromiso multiorgánico importante y altas tasas de morbilidad y mortalidad. Se recalca en este caso el compromiso subagudo con requerimiento temprano de terapia dialítica y además se enfatiza la importancia de una sospecha y un diagnóstico oportuno en pacientes con compromiso renal y otras manifestaciones sistémicas.

Introduction Light chain amyloidosis is an entity triggered by the proliferation of a clone of plasma cells that generates the accumulation of light chains, which are deposited in the form of amyloid fibrils generating organic dysfunction. Renal compromise generally manifests as nephrotic syndrome, with a slow and progressive decline of renal function that can lead to dialysis therapy. Purpose The objective of this case report is to demonstrate the aggressive and subacute renal involvement of systemic amyloidosis. Case presentation We present a case of a 35-year-old male patient who consulted for general malaise, blurred vision, dizziness and oliguria with elevated nitrogen levels that progressed fastly to the requirement of hemodialysis in approximately 3 months. It was also reveal peripheral polyneuropathy, cardiac infiltration, and slight elevation of transaminases. Given the negativity of extension studies, amyloid deposits were documented histopathologically with positive immunofluorescence for LA. He is currently receiving chemotherapy regimen with adequate clinical stability and tolerance. Conclusion and discussion To conclude, AL amyloidosis is a rare entity, with significant multi-organ involvement and high rates of morbidity and mortality. In this case, the subacute involvement with an early requirement for dialysis therapy is emphasized, and the importance of suspicion and timely diagnosis in patients with kidney involvement and other systemic manifestations is emphasized.

Sleeplessness in Patients with end-Stage Renal Disease Undergoing Dialysis Therapy in a Tertiary Care Center

Introduction: Maintenance dialysis patients experience a highburden of physical and emotional symptoms that directly affecttheir quality of life and health care utilization. Patient with endstage renal disease (ESRD), 80% of them have reported withcomplaints of subjective sleep abnormalities. Frequent reportshave documented in these patients such as sleep disturbancesmanifestation as insomnia, sleep apnea syndrome, restlessleg syndrome (RLS) periodic limb movement disorders, andexcessive day time sleepiness. Present study focused to findthe quality of sleep and sleep abnormalities in patients withESRD.Material and methods: Patient data were collected usingPittsburgh Sleep Quality Index(PSQI); The demographic andclinic questionnaire contained questions about age, gender,place of residence, dialysis frequency, HD (Haemodialysis)duration. Scoring of seven components answers is based ona 0 to 3 scale which reflects the various states of sleepingdisorders.Results: Study showed that during the earlier stage ofhemodialysis patients may have a high PSQI test score. In theperiod of 3- 12-month patients are facing poor sleep quality;indicating that in the earlier period of treatment patientsmay have taken more stress and conscious to adjust withhemodialysis as a routine treatment procedure for the rest ofthe life. This may instigate to have a high score in PSQI testscore.Conclusion: The current study indicates that RLS, poorquality sleep, and EDS are common in ESRD patients underhemodialysis. Additional studies involving the change ofhemodialysis shift may provide a better understanding ofthe correlation between time duration dialysis and sleepingdisturbances.

Complementary and Alternative Medicine in Dialysis Therapy / 全日本鍼灸学会雑誌

Dialysis therapy is the most common therapeutic modality in the treatment of end-stage renal failure. Over 200, 000 patients were maintained under dialysis therapy. An important goal in clinical nephrology is to control complications associated with long-term dialysis. Recently, complementary and alternative medicine (CAM) usehave increased dramatically in the world. This article demonstrates the efficacy of CAM including acupuncture for dialysis patients suffering from refractory complications.

Pre-End-Stage Renal Disease(Pre-ESRD) Care in the U.S.A / 日本農村医学会雑誌

A significant delay in identifying pre-ESRD patients is not infrequent in the U.S.A. because an early examination for proteinuria is not common, and renal disease is infrequently documented even when proteinuria and/or an elevated serum creatinine level have existed. Although there are accepted strategies for slowing the progression of renal disease such as the use of an angiotensin converting enzyme (ACE) inhibitor in diabetic patients or suboptimal blood pressure control in hypertensive patients, these strategies are seldom employed. It is also particularly alarming that the patients are often low in the overall health status, hypoalbumic, or anemic at dialysis initiation, and begin dialysis without permanent vascular access or without any education regarding dialysis therapy in many instances.