Composite Zonal Scaffolds of Collagen I/II for Meniscus Regeneration.

The meniscus is divided into three zones according to its vascularity: an external vascularized red-red zone mainly comprising collagen I, a red-white interphase zone mainly comprising collagens I and II, and an internal white-white zone rich in collagen II. Known scaffolds used to treat meniscal injuries do not reflect the chemical composition of the vascular areas of the meniscus. Therefore, in this study, four composite zonal scaffolds (named A, B, C, and D) were developed and characterized; the developed scaffolds exhibited the main chemical components of the external (collagen I), interphase (collagens I/II), and internal (collagen II) zones of the meniscus. Noncomposite scaffolds were also produced (named E), which had the same shape as the composite scaffolds but were entirely made of collagen I. The composite zonal scaffolds were prepared using different concentrations of collagen I and the same concentration of collagen II and were either cross-linked with genipin or not cross-linked. Porous, biodegradable, and hydrophilic scaffolds with an expected chemical composition were obtained. Their pore size was smaller than the size reported for the meniscus substitutes; however, all scaffolds allowed the adhesion and proliferation of human adipose-derived stem cells (hADSCs) and were not cytotoxic. Data from enzymatic degradation and hADSC proliferation assays were considered for choosing the cross-linked composite scaffolds along with the collagen I scaffold and to test if composite zonal scaffolds seeded with hADSC and cultured with differentiation medium produced fibrocartilage-like tissue different from that formed in noncomposite scaffolds. After 21 days of culture, hADSCs seeded on composite scaffolds afforded an extracellular matrix with aggrecan, whereas hADSCs seeded on noncomposite collagen I scaffolds formed a matrix-like fibrocartilage without aggrecan.

Role of motivation and well-being indicators in interventions to improve well-being at work among primary care physicians: a systematic review.

OBJECTIVE: The well-being of primary care physicians (PCPs) has become an object of concern for governments due to staff shortages and high staff turnover. The objective of this study was to carry out a systematic review of individualised interventions aimed at improving the well-being of PCPs, which allowed us to determine (1) the type of interventions being carried out; (2) the well-being indicators being used and the instruments used to assess them; (3) the theories proposed to support the interventions and the mechanisms of action (MoA) put forward to explain the results obtained and (4) the role that individual motivation plays in the interventions to improve well-being among PCPs. DESIGN: Systematic review. ELIGIBILITY CRITERIA: Clinical trials on interventions aimed at improving the well-being of PCPs. INFORMATION SOURCES: a search of studies published between 2000 and 2022 was carried out in MEDLINE/PubMed, SCOPUS and Web of Science (WOS). RESULTS: From the search, 250 articles were retrieved. The two authors each reviewed the articles independently, duplicate articles and those that did not meet the inclusion criteria were discarded. A total of 14 studies that met the criteria were included: 6 randomised clinical trials, 4 controlled clinical trials and 4 unique cohorts, with a before-and-after assessment of the intervention, involving a total of 655 individuals participating in the interventions. A meta-analysis was not possible due to the heterogeneity of the studies. CONCLUSIONS: The information evaluated is insufficient to accurately assess which outcomes are the best indicators of PCPs well-being or what role plays in the individual motivation in the results of the interventions. More studies need to be carried out on the subject to determine the MoA of the different interventions on the results and the motivation of the participating PCPs.

Unusual primary central nervous system T-cell/histiocyte-rich large B-cell lymphoma: a case report.

We report the case of a 54-year-old immunocompetent woman who presented with a primary T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL) of the central nervous system without systemic involvement, diagnosed by means of a brain biopsy. She was treated with corticosteroids and we subsequently started chemotherapy with rituximab, methotrexate, ifosfamide and intrathecal cytarabine. The patient's symptoms gradually improved over the first weeks and we followed-up with autologous haematopoietic cell transplantation. The patient has been in complete remission for a year. Primary TCHRLBCL of the central nervous system in an immunocompetent patient is an extremely rare condition that requires a multidisciplinary approach. This case highlights the importance of undergoing a sequential work-up and establishing a treatment despite the absence of evidence-based guidelines.

Neutral lipid storage disease with myopathy and dropped head syndrome. Report of a new variant susceptible of treatment with late diagnosis.

Neutral lipid storage disease with myopathy (NLSDM) is characterized by the accumulation of cytoplasmic triglyceride droplets in various tissues; this very rare condition is caused by mutations in the PNPLA2 gene, susceptible to specific pharmacological management that decreases clinical progression. We describe the clinical and biochemical characteristics of a Colombian patient with a previously unreported homozygous mutation in the PNPLA2 gene with a difficult to manage disease, who was diagnosed late by advances in molecular techniques.

Primary hiperoxaluria diagnosed after kidney transplantation: report of 2 cases and literature review / Hiperoxalúria primária diagnosticada após transplante renal: relato de dois casos e revisão da literatura

Abstract Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.

Resumo A hiperoxalúria primária (HP) é um distúrbio genético muito raro, caracterizado por deficiência total ou parcial das enzimas relacionadas ao metabolismo do glioxilato, superprodução de oxalato de cálcio que se deposita em vários órgãos (principalmente os rins) resultando em litíase recorrente, nefrocalcinose e doença renal terminal (DRT). Nos pacientes com DRT que recebem transplante renal, a doença apresenta recidiva em 100% dos casos, com perda do enxerto nos primeiros cinco anos após o transplante num elevado percentual de pacientes. Três distúrbios moleculares foram descritos na HP: mutação dos genes da alanina-glioxilato aminotransferase (AGXT), glioxilato redutase/hidroxipiruvato redutase (GRHPR) e 4-OH-2-oxoglutarato aldolase (HOGA1). Apresentamos dois casos de pacientes com histórico de litíase renal diagnosticados com hiperoxalúria primária no período pós-transplante, manifestada na forma de perda precoce do enxerto com evidências de cristais de oxalato de cálcio na biópsia renal, hiperoxalúria, hiperoxalemia e testes genéticos compatíveis. Os pacientes foram tratados com abordagem nutricional, líquidos orais em abundância, piridoxina, hidroclorotiazida e citrato de potássio. Contudo, os pacientes apresentaram deterioração lenta e gradual da função do enxerto e evoluíram para doença renal terminal.

Primary hiperoxaluria diagnosed after kidney transplantation: report of 2 cases and literature review.

Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.

[Three cases of de novo multiple myeloma after kidney transplantation].

Light chain-associated kidney compromise is frequent in patients with monoclonal gammopathies; it affects the glomeruli or the tubules, and its most common cause is multiple myeloma. It may develop after a kidney transplant due to recurrence of a preexisting multiple myeloma or it can be a de novo disease manifesting as graft dysfunction and proteinuria. A kidney biopsy is always necessary to confirm the diagnosis.We describe three cases of kidney graft dysfunction due to multiple myeloma in patients without presence of the disease before the transplant.

Falla cardiaca y flutter auricular como manifestación de distrofia miotónica tipo 1 / Heart failure and atrial flutter as a manifestation of myotonic dystrophy type 1

La distrofia miotónica es una enfermedad hereditaria del sistema neuromuscular con afección cardiaca, se presenta fundamentalmente con alteraciones del ritmo y conducción auriculoventricular y rara vez insuficiencia cardiaca. Se expone el caso de un paciente de 37 años de edad que ingresa por falla cardiaca aguda y flutter auricular asociado a debilidad muscular progresiva de larga data en quien se realiza diagnóstico de distrofia miotónica tipo 1 luego de estudios complementarios. Se presenta una corta revisión de la literatura acerca de esta enfermedad y sus manifestaciones cardiacas.

Myotonic dystrophy is a hereditary disease of the neuromuscular system with cardiac impairment, mainly showing rhythm disturbances and atrioventricular conduction defects, and rarely heart failure. We report the case of a 37 year-old patient who was admitted for acute heart failure and atrial flutter associated with long standing progressive muscle weakness. A diagnosis of myotonic dystrophy type 1 was made after complementary studies. A short review of the literature about this pathology and the cardiac manifestations is presented.

Gammapatía monoclonal de significado incierto A propósito de un caso de nefropatía por depósito de cadenas ligeras lambda / Monoclonal gammopathy of undetermined significance Apropos of a case of lambda light chain deposition nephropathy

La nefropatía asociada a las gammapatías monoclonales es debida principalmente al depósito de cadenas ligeras. Las enfermedades renales paraproteinémicas son lesiones asociadas con depósito de inmunoglobulinas intactas o fragmentos de inmunoglobulinas (cadenas pesadas y cadenas ligeras). La enfermedad por depósito de cadenas ligeras es una condición rara, caracterizada por el depósito de cadenas ligeras monoclonales en muchos órganos y a nivel renal predominantemente en glomérulos y membranas basales tubulares. La enfermedad está frecuentemente asociada con desórdenes linfoproliferativos, y la mayoría de casos son causados por depósito de cadenas ligeras kappa. Aunque se presenta sobre todo en cuadros malignos, en ocasiones no se detecta patología hematológica y se denomina idiopática o "primaria". Suele manifestarse como una insuficiencia renal severa con proteinuria nefrótica, no tiene tratamiento claramente establecido y el pronóstico es malo. Se describen las características clínicas e histológicas del segundo caso informado en Colombia de nefropatía por depósito de cadenas ligeras diagnosticado en el contexto de una enfermedad renal paraproteinémica sin datos de malignidad. (Acta Med Colomb 2014; 39: 196-201).

Nephropathy associated with monoclonal gammopathies is mainly due to light chain deposition. The paraproteinemic kidney diseases are lesions associated with deposition of intact immunoglobulins or fragments of immunoglobulins (heavy and light chains). The disease due to deposition of light chains is a rare condition characterized by deposition of monoclonal light chains in many organs and as for the kidney, predominantly in glomeruli and tubular basement membranes. The disease is frequently associated with lymphoproliferative disorders and the majority of cases are caused by deposition of kappa light chains. Although presented primarily in clinical pictures of malignancy, sometimes no hematological pathology is detected and is called idiopathic or "primary". It usually manifests as severe renal failure with nephrotic proteinuria, has not a clearly established treatment and the prognosis is poor. The clinical and histological features of the second case reported in Colombia of a light chain deposition nephropathy diagnosed in the context of a kidney paraproteinemic disease without malignancy data, is presented. (Acta Med Colomb 2014; 39: 196-201).

Kidney pathological changes in metabolic syndrome: a cross-sectional study.

BACKGROUND: The worldwide prevalence of metabolic syndrome is increasing and has been associated with chronic kidney disease. Kidney pathological findings in patients with metabolic syndrome have not been well described, as was explored in this study. STUDY DESIGN: Cross-sectional study. SETTING & PARTICIPANTS: We retrospectively screened clinical information for 146 patients who underwent elective nephrectomy for renal cell carcinoma between January 2005 and March 2007 at Brigham and Women's Hospital, Boston, MA. Twelve patients with metabolic syndrome were identified. Twelve age- and sex-matched patients who did not have any of the criteria for metabolic syndrome were used as controls. PREDICTOR: Presence of metabolic syndrome defined by using Adult Treatment Panel III criteria. OUTCOMES: Histological characteristics in each group, decrease in kidney function at 1-year follow-up. MEASUREMENTS: Two pathologists blinded to the clinical diagnosis independently evaluated nephrectomy specimens using Banff criteria to objectively assess histological characteristics. RESULTS: Baseline characteristics were similar between the 2 groups. On histopathologic examination, patients with metabolic syndrome compared with controls had a greater prevalence of tubular atrophy (P = 0.006), interstitial fibrosis (P = 0.001), and arterial sclerosis (P = 0.001), suggesting microvascular disease. Patients with metabolic syndrome had greater global (P = 0.04) and segmental glomerulosclerosis (P = 0.05). Glomerular volume and cross-sectional surface area were not different. The combined end point of tubular atrophy greater than 5%, interstitial fibrosis greater than 5%, and presence of arterial sclerosis was more prevalent in patients with metabolic syndrome (P = 0.003; odds ratio, 33; confidence interval, 2.9 to 374.3) than controls. After 1 year, estimated glomerular filtration rate was significantly lower in patients with metabolic syndrome compared with controls (P = 0.03). LIMITATIONS: Small sample size, retrospective design. CONCLUSIONS: We report a high prevalence of microvascular disease in patients with metabolic syndrome. There was a steeper decrease in kidney function over time in patients with metabolic syndrome, suggesting limited renal reserve. Aggressive screening and management may be warranted in patients with metabolic syndrome to protect kidney function.

Electrocardiografía en las arritmias cardíacas supraventriculares auriculares / Electrocardiography in cardiac supraventricular atrial arrhythmias

En esta segunda publicación sobre el uso de electrocardiograma de superficie como medio diagnóstico, se discutirán los principales hallazgos sobre las arritmias, específicamente las ubicadas en el plano supraventricular auricular

Electrocardiografía en las arritmias supraventriculares, nodales y ventriculares / Electrocardiography in supraventricular, nodal, and ventricular arrhythmias

En esta tercera publicación sobre el uso del electrocardiograma de superficie como medio diagnóstico, se discutirán los principales hallazgos sobre las arritmias, específicamente las ubicadas en el plano supraventricular nodal y en el plano ventricular