Seventy years of pheochromocytomas and paragangliomas in Argentina. The FRENAR database.

Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors characterized by the excessive production of catecholamines. This study aims to describe the clinical characteristics of PPGL cases in Argentina over recent decades. A multicenter retrospective cross-sectional analysis was carried out using a database comprising both pediatric and adult patients with confirmed PPGL diagnoses based on pathological reports. A cohort of 486 patients with PPGL was recruited. Women represent 58.4% of the patients, with a mean age of 38.3 years old at the time of diagnosis and 15.2% of the patients were under the age of 18. Hypertension, as well as classic signs and symptoms, were present in 80.9% of the patients. The adrenal incidentaloma, as a mode of presentation, increased in the last two decades rising from 3.9% (1953-2000) to 21.8% (2001-2022), p<0.001. Most tumors were located within the adrenal glands, accounting 83.0% of the cases, with bilateral occurrences noted in 20.0%. The median tumor size was 4.8cm. Local recurrence and metastases were observed in 10.9% and 12.2%. Out of 412 patients, 87.0% exhibited urinary excretion elevation of catecholamines and/or their metabolites. Furthermore, 148 patients, representing 30.4% of the study population, displayed a distinct genetic profile indicative of hereditary syndromes. The distribution of hereditary syndromes revealed that MEN2, VHL, and PGL4 constituted the most prevalent syndromes. This population-based study, spanning seven decades, offers valuable insights into the demographic and clinical characteristics of PPGL patients in Argentina.

Advanced-stage mycosis fungoides: role of the signal transducer and activator of transcription 3, nuclear factor-κB and nuclear factor of activated T cells pathways.

BACKGROUND: The malignant mechanisms that control the development of cutaneous T-cell lymphoma (CTCL) are beginning to be identified. Recent evidence suggests that disturbances in specific intracellular signalling pathways, such as RAS-mitogen-activated protein kinase, T-cell receptor (TCR)-phospholipase C gamma 1 (PLCG1)-nuclear factor of activated T cells (NFAT) and Janus kinase (JAK)-signal transducer and activator of transcription (STAT), may play an essential role in the pathogenesis of CTCL. OBJECTIVES: To investigate the mechanisms controlling disease development and progression in mycosis fungoides (MF), the most common form of CTCL. METHODS: We collected 100 samples that were submitted for diagnosis of, or a second opinion regarding, MF between 2001 and 2018, 80% of which were in the early clinical stages of the disease. Formalin-fixed paraffin-embedded tissues were used for histological review and to measure the expression by immunohistochemistry of surrogate markers of activation of the TCR-PLCG1-NFAT, JAK-STAT and NF-κB pathways. Folliculotropism and large-cell transformation were also examined. RESULTS: NFAT and nuclear factor kappa B (NF-κB) markers showed a comparable activation status in early and advanced stages, while STAT3 activation was more frequent in advanced stages and was associated with large-cell transformation. Consistently with this observation, STAT3 activation occurred in parallel with MF progression in two initially MF-negative cases. A significant association of NFAT with NF-κB markers was also found, reflecting a common mechanism of activation in the two pathways. Genomic studies identified nine mutations in seven genes known to play a potential role in tumorigenesis in T-cell leukaemia/lymphoma, including PLCG1, JAK3 and STAT3, which underlies the activation of these key cell-survival pathways. A higher mutational allele frequency was detected in advanced stages. CONCLUSIONS: Our results show that STAT3 is activated in advanced cases and is associated with large-cell transformation, while the activation of NFAT and NF-κB is maintained throughout the disease. These findings could have important diagnostic and therapeutic implications. What's already known about this topic? Mycosis fungoides is characterized by a clonal expansion of T cells in the skin. The mechanisms controlling disease development and progression are not fully understood. What does this study add? An association of the nuclear factor of activated T cells and nuclear factor kappa B pathways was found, which could reflect a common mechanism of activation. These pathways were activated in early and advanced stages at the same level. Signal transducer and activator of transcription 3 activation was associated with large-cell transformation and was more frequent in advanced stages. A genomic analysis of cutaneous T-cell lymphoma-associated genes was performed. Nine mutations were detected. What is the translational message? These results could have important implications for the treatment of MF in the near future.

[Diagnostic approach of pheochromocytomas and paragangliomas]. / Enfoque diagnóstico de feocromocitomas y paragangliomas.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumours associated with high morbidity and mortality. Recognizing the clinical presentation is the first step for diagnosis. Biochemical studies may determine an excess of catecholamines and their metabolites. However, the available tests offer varying diagnosis precision. Computed tomography and magnetic resonance are highly sensitive for locating these tumours. Functional tests are reserved for when metastatic and multifocal disease are suspected. One third of the patients have a germline mutation and many genes are involved in the development of these tumours.

Lesión nodular acral tras traumatismo / Acral Nodular Lesion Following Trauma

No disponible

Síndrome de Buschke-Ollendorff / Buschke-Ollendorff syndrome

No disponible

Alitretinoína oral: tratamiento específicio del eczema crónico de manos grave refractario a corticoides / Oral alitretinoin: specific therapy for severe chronic eczema of the hands refractory to corticosteroids

El eczema de manos (ECM) es la dermatosis más frecuente de la manos, y se trata de un importante (..) (AU)

Eczema of the hands (EOH) is the most prevalent hand dermatosis and represents and important healthcare, social (..) (AU)

[Evacuation plan of an intensive care unit: a new quality indicator?]. / Plan de evacuación de la unidad de cuidados intensivos: ¿un nuevo indicador de calidad?

The intensive care units must be prepared for a possible disaster, whether internal or external, in case it becomes necessary to evacuate the in-patients. They must have an Emergency and Self-protection Plan that includes the patient evacuation criteria and this must be known by all the personnel who work in the service. For that reason, the patients must be triaged, based on their attention priorities, according to their survival possibilities. Having an evacuation, known by all the personnel and updated by means of the performance of periodic drills, should be included as a quality indicator that must be met, since this would achieve better attention to the patient in case of a disaster situation requiring the evacuation of the ICU.

Plan de evacuación de la unidad de cuidados intensivos: ¿un nuevo indicador de calidad? / Evacuation plan of an intensive care unit: A new quality indicator?

Las unidades de cuidados intensivos deben estar preparadas para un eventual desastre, bien sea de índole interno o externo, donde sea necesario evacuar a los pacientes ingresados. Deben disponer de un Plan de Emergencia y Autoprotección donde estén recogidos los criterios de evacuación de los pacientes, que debe conocer todo el personal que trabaja en el servicio. Para esto, habrá que hacer un triaje a los pacientes basándose en las prioridades de atención y según las posibilidades de supervivencia. El disponer de un plan de evacuación, conocido por todo el personal y actualizado mediante la realización de simulacros periódicos, debería incluirse como un indicador de calidad que se debería cumplir, ya que con esto se conseguiría una mejor atención al paciente en caso de presentarse una situación de desastre donde hubiere que evacuar la unidad de cuidados intensivos (AU)

The intensive care units must be prepared for a possible disaster, whether internal or external, in case it becomes necessary to evacuate the in-patients. They must have an Emergency and Self-protection Plan that includes the patient evacuation criteria and this must be known by all the personnel who work in the service. For that reason, the patients must be triaged, based on their attention priorities, according to their survival possibilities. Having an evacuation, known by all the personnel and updated by means of the performance of periodic drills, should be included as a quality indicator that must be met, since this would achieve better attention to the patient in case of a disaster situation requiring the evacuation of the ICU (AU)

[Long-term control of psoriasis is necessary]. / Es necesario el control de la psoriasis a largo plazo.

Psoriasis is an immune mediated, inflammatory skin condition affecting approximately 1.43% of Spanish population. In clinical practice, physicians use PASI index to assess the severity. Psoriasis causes physical and mental disability comparable to other chronic diseases and affects seriously the patients quality of life. For treatment we have different options. Conventional systemic treatment such as methotrexate, cyclosporine and acitretin may be associated with relevant side effects, and organ toxicity that avoid long term therapy. Several psoriasis patients have other comorbid disorders like obesity, diabetes, dyslipemia, hypertension and an increased rate of cardiovascular disease and metabolic syndrome and these patients need safer treatments. The accumulating clinical experience with new therapies consistent on biological agents like efalizumab indicates that are effective therapies, with safety profile and no evidence of cumulative toxicity that allows a long term use. A better control of psoriasis improves patients quality of life.

Failure of a short-term prednisone regimen to prevent nevirapine-associated rash: a double-blind placebo-controlled trial: the GESIDA 09/99 study.

OBJECTIVES: Rash is the most frequent adverse event associated with nevirapine. The use of prednisone has been controversial in this setting. A double-blind placebo-controlled study was performed to evaluate its efficacy in nevirapine-induced rash prevention. DESIGN: Multicentered, randomized, double-blind, placebo-controlled clinical trial with prednisone (30 mg/day x 2 weeks). INCLUSION CRITERIA: HIV-1 infection; CD4 count >200 cells/mm3; plasma viral load (PVL) <5 log10 copies/ml; nevirapine (200 mg/day x 2 weeks, followed by 200 mg twice daily) plus stavudine and didanosine. Clinical follow-up was performed at 15, 30, and 60 days and thereafter every 2 months. RESULTS: In all, 75 evaluable patients were enrolled (39 prednisone/36 placebo). Median baseline CD4 + cell count was 390 cells/mm3 and PVL, 20,200 copies/ml. Overall, nine cases of rash (12.5%) were detected, seven (18%) in the prednisone group and two (5.5%) in the placebo group (odds ratio [OR], 3.85; 95% confidence interval [CI], 0.65-29.3; p =.11). Incidence of moderate-to-severe rashes leading to nevirapine withdrawal was 13.5% (5 of 37) in the prednisone group and 3% (1 of 35) in the placebo group ( p =.2). Median time to rash in both groups was 16 days. Adverse events that motivated withdrawal of therapy appeared in 6 patients from the prednisone group (15.4%) and 3 from the placebo group (8.3%) ( p =.3). CONCLUSION: Short-term prednisone administration does not prevent nevirapine rash, but might even increase its incidence.

[Acute nephrotic syndrome associated with secondary syphilis]. / Síndrome nefrótico agudo asociado a sífilis secundaria.

We present a case of a 38 year old woman with nephrotic syndrome and skin rash. She had been previously healthy. At the beginning she showed nonpruritic macular papular lesion in trunk and upper and lower extremities with twenty days of evolution. The week previous to hospitalization, she presented eyelid and pretibial edema. The laboratory informed normal kidney function with proteinuria of 10 g/day, plasma proteins of 4.20 g/dl., cholesterol 334 mg/l, VDRL (+), 1/32 FTA abs (+) HIV non reactive, normal collagenogram. She was diagnosed as secondary syphilitic nephrotic syndrome. She was prescribed penicillin, rest and salt restriction. She presented good evolution before ending treatment and the syndrome was completely solved at the third week. The incidence of kidney condition associated with early staging of syphilis is lower than 0.3%. We call attention to this association, for such an uncommon case may not be detected.

Síndrome nefrótico agudo asociado a sífilis secundaria. / [Acute nephrotic syndrome associated with secondary syphilis]

We present a case of a 38 year old woman with nephrotic syndrome and skin rash. She had been previously healthy. At the beginning she showed nonpruritic macular papular lesion in trunk and upper and lower extremities with twenty days of evolution. The week previous to hospitalization, she presented eyelid and pretibial edema. The laboratory informed normal kidney function with proteinuria of 10 g/day, plasma proteins of 4.20 g/dl., cholesterol 334 mg/l, VDRL (+), 1/32 FTA abs (+) HIV non reactive, normal collagenogram. She was diagnosed as secondary syphilitic nephrotic syndrome. She was prescribed penicillin, rest and salt restriction. She presented good evolution before ending treatment and the syndrome was completely solved at the third week. The incidence of kidney condition associated with early staging of syphilis is lower than 0.3

. We call attention to this association, for such an uncommon case may not be detected.

Use of albumin in two Spanish university hospitals.

OBJECTIVES: The aim of this study was to characterize the use of seralbumin, evaluating how appropriate its prescription is and what possible economic repercussions may result from inappropriate use. METHODS: We performed a prospective study that included all patients receiving albumin in two University Hospitals from October 1995 to March 1996. The reasons for albumin use were considered appropriate if they coincided with the recommendations of a panel of experts. RESULTS: During the study period, 197 patients received albumin and a total of 3208 50-ml vials (20%) were used. The internal medicine and gastroenterology services prescribed this drug the most often. The most frequent prescription motives were paracentesis in cirrhotic patients (25.9%), hypoalbuminemia (24.9%) and chronic handling of cirrhotic patients (18.6%). Only 16 prescriptions (8.1%) (corresponding to 315 vials, 9.8%) were considered appropriate. One cause of inappropriate prescribing was that colloid solutions had not previously been used in 56 (30.9%) of the 186 inappropriate prescriptions. During the study period, 74,306 ECUs were spent on inappropriate indications. CONCLUSIONS: The use of albumin in our centers is incorrect and has important economic repercussions. Some educational and informative measures must be established to change this situation.

[Kaposi sarcoma and Hodgkin disease]. / Sarcoma de Kaposi y enfermedad de Hodgkin.

Kaposi's sarcoma is frequently associated to other primary neoplasias, especially those arising from mononuclear-phagocytic system. A case of Kaposi's sarcoma associated to Hodgkin's disease is presented.