Treatment of Chronic Heart Failure in Advanced Chronic Kidney Disease: The HAKA Multicenter Retrospective Real-World Study.

INTRODUCTION: Chronic heart failure (HF) has high rates of mortality and hospitalization in patients with advanced chronic kidney disease (aCKD). However, randomized clinical trials have systematically excluded aCKD population. We have investigated current HF therapy in patients receiving clinical care in specialized aCKD units. METHODS: The Heart And Kidney Audit (HAKA) was a cross-sectional and retrospective real-world study including outpatients with aCKD and HF from 29 Spanish centers. The objective was to evaluate how the treatment of HF in patients with aCKD complied with the recommendations of the European Society of Cardiology Guidelines for the diagnosis and treatment of HF, especially regarding the foundational drugs: renin-angiotensin system inhibitors (RASi), angiotensin receptor blocker/neprilysin inhibitors (ARNI), beta-blockers (BBs), mineralocorticoid receptor antagonists (MRAs), and sodium-glucose cotransporter-2 inhibitors (SGLT2i). RESULTS: Among 5,012 aCKD patients, 532 (13%) had a diagnosis of HF. Of them, 20% had reduced ejection fraction (HFrEF), 13% mildly reduced EF (HFmrEF), and 67% preserved EF (HFpEF). Only 9.3% of patients with HFrEF were receiving quadruple therapy with RASi/ARNI, BB, MRA, and SGLT2i, but the majority were not on the maximum recommended doses. None of the patients with HFrEF and CKD G5 received quadruple therapy. Among HFmrEF patients, approximately half and two-thirds were receiving RASi and/or BB, respectively, while less than 15% received ARNI, MRA, or SGLT2i. Less than 10% of patients with HFpEF were receiving SGLT2i. CONCLUSIONS: Under real-world conditions, HF in aCKD patients is sub-optimally treated. Increased awareness of current guidelines and pragmatic trials specifically enrolling these patients represent unmet medical needs.

La enfermedad de Lhermitte-Duclos (ELD): Reporte del primer caso registrado en Paraguay con correlación radiológica-patológica

La enfermedad de Lhermitte-Duclos, o gangliocitoma displásico cerebeloso, es una rara entidad que se manifiesta como un proceso expansivo de crecimiento lento en la fosa posterior, específicamente en el cerebelo, con una patogenia no muy bien comprendida, que puede estar relacionada con anomalías congénitas y mutaciones del gen PTEN (fosfatasa y homólogo de tensina) sobre todo en la forma de presentación adulta. Los casos documentados de esta enfermedad son escasos, con alrededor de 230 registros en la literatura médica y una prevalencia menor a 1 por cada millón de personas. Presentamos el primer caso en Paraguay de Enfermedad de Lhermitte-Duclos en un paciente masculino de 44 años, sin antecedentes médicos o familiares relevantes, que fue intervenido quirúrgicamente por proceso expansivo de fosa posterior, con hallazgos característicos en la resonancia magnética, que permitió sospechar preoperatoriamente de la enfermedad, con diagnóstico diferencial de meduloblastoma, debido a restricciones observadas en la secuencia de Difusión. La resección fue completa con adecuada evolución y sin recaídas del proceso.

Lhermitte-Duclos disease, or cerebellar dysplastic gangliocytoma, is a rare entity that manifests as a slow-growing expansive process in the posterior fossa, specifically in the cerebellum, with a pathogenesis that is not well understood, which may be related to congenital anomalies and PTEN gene mutations (phosphatase and tensin homologue), especially in the form of adult presentation. Documented cases of this disease are scarce, with around 230 records in the medical literature and a prevalence of less than 1 per million people. We present the first case in Paraguay of dysplastic gangliocytoma or Lhermitte-Duclos disease in a 44-year-old male patient, with no relevant medical or family history, who underwent surgery due to an expansive process in the posterior fossa, with characteristic findings on magnetic resonance, which allowed preoperative suspicion of the disease, with differential diagnosis of medulloblastoma, due to restrictions observed in the Diffusion sequence. The resection was complete with adequate evolution and without relapses of the process. Keywords: Lhermitte- Duclos; Gangliocytoma; dysplastic; cerebellar; neuroradiology.

Interrupción del tallo hipofisario como causa poco frecuente de amenorrea primaria / Pituitary stalk disruption as a rare cause of primary amenorrhea

El síndrome de interrupción del tallo hipofisario es una anomalía congénita de probable origen genético causante de hipopituitarismo, que radiológicamente se caracteriza por hipoplasia de la adenohipófisis, neurohipófisis ectópica e interrupción del tallo hipofisario. Suele presentarse con baja estatura y deficiencia de hormonas adenohipofisarias. El retraso en el diagnóstico se relaciona con alta morbimortalidad. Se reporta el caso de una mujer de 24 años que consulta por amenorrea primaria. Presentaba talla baja y ausencia de caracteres sexuales secundarios, con genitales infantiles. Los exámenes paraclínicos mostraron compromiso de dos ejes hipofisarios. Una resonancia magnética nuclear mostró hallazgos consistentes con síndrome de interrupción de tallo hipofisario.

Pituitary stalk disruption syndrome is a congenital abnormality of probable genetic origin that causes hypopituitarism, which is radiologically characterized by adenohypophysis hypoplasia, ectopic neurohypophysis, and pituitary stalk disruption. It usuallypresents with short stature and adenohypophyseal hormone deficiency. The delay in diagnosis is related to high morbidity and mortality. The case of a 24-year-old woman who consulted for primary amenorrhea is reported. She had short stature and no secondary sexual characteristics, with infantile genitalia. Paraclinical examinations showed involvement of two pituitary axes. An MRI scan showed findings consistent with pituitary stalk disruption syndrome.

Causa poco frecuente de insuficiencia suprarrenal. A propósito de un caso / A rare cause of adrenal insufficiency. A case report

Se presenta un caso de sarcoidosis que presentó afectación suprarrenal. Hombre de 44 años fue diagnosticado de sarcoidosis pulmonar y ganglionar según hallazgos clínicos, laboratoriales y anatomopatológicos. Tres años después acude por debilidad generalizada, náuseas, hiponatremia persistente, hipotensión arterial, pérdida de peso e hiperpigmentación cutánea. La evaluación endocrinológica confirma insuficiencia suprarrenal primaria, lo que sugiere la infiltración de la sarcoidosis en las glándulas suprarrenales.

We present a case of sarcoidosis that presented adrenal involvement. A 44-year- old man was diagnosed with pulmonary and lymph node sarcoidosis based on clinical, laboratory, and pathological findings. Three years later, he presented with generalized weakness, nausea, persistent hyponatraemia, arterial hypotension, weight loss, and skin hyperpigmentation. Endocrinologic evaluation confirms primary adrenal insufficiency, suggesting infiltration of sarcoidosis into the adrenal glands.

Quimioterapia y reactivación oculta del VHB en pacientes de hemodiálisis / Chemotherapy and occult reactivation of HBV in hemodialysis patients

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Nuevos aspectos en la vigilancia epidemiológica con relación a las enfermedades virales en hemodiálisis / New aspects in epidemiological surveillance in relation to viral diseases in hemodialysis

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¿Cuándo puede ser útil buscar VHB oculto en pacientes en hemodiálisis? / When can it be useful to look for occult HBV in hemodialysis patients?

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Use of calix[4]arenes in the redox chemistry of lanthanides: the reduction of dinitrogen by a calix[4]arene-samarium complex.

The synthesis and structural characterization of a samarium-dinitrogen complex supported by a calix[4]arene ligand in which the N-N bond distance has been stretched to 1.611(16) Angstrom are described. The central mu(3)-eta(2):eta(2):eta(2)-hydrazido tetraanion formed is bonded to three Sm(III) centers with an overall butterfly-type arrangement.

Ascitis urinosa en trasplantado renal / Urinary ascites after kidney transplantation

Objetivo: Presentamos un caso clínico de ascitis urinosa secundaria a urinoma producido por una fístula piélica y de uréter proximal en un varón de 21 años, trasplantado renal. Métodos: El diagnóstico se estableció mediante análisis bioquímico de la colección y líquido ascítico y la manipulación conservadora de la vía urinaria mediante nefrostomía percutánea. Resultado: El caso fue resuelto mediante la reparación quirúrgica de la fístula. Conclusión: La ascitis urinosa representa una rara complicación en el paciente trasplantado renal, cuyo origen puede ser idiopático, o como en la mayoría de las veces, secundario a una fístula en la vía urinaria. En este caso su resolución puede conseguirse mediante manipulación conservadora de la vía urinaria, o si fracasa, mediante cirugía reparadora. pueden ser indicativos de un pronóstico desfavorable. Nosotros consideramos que este tipo de tumor adrenal debe de ser considerado histológicamente y biológicamente como un sarcoma de alto grado de origen endotelial (AU)

[Urine ascites in renal transplantation]. / Ascitis urinosa en trasplantado renal.

OBJECTIVE: To report a case of urinary ascites secondary to an urinoma produced by a pielic and proximal ureter fistula after kidney transplantation in a 21 year old male patient. METHODS: Diagnosis was established by biochemical analysis of the perirenal fluid collection and ascitic fluid, and conservative management of the urinary tract by percutaneous nephrostomy was undertaken. RESULTS: Surgical repair of the fistulae solved the case. CONCLUSIONS: Urinary ascites is an unusual complication after kidney transplantation. Urinary tract fistula is the etiology in most of the cases although it can be idiopathic. In the case of urinary tract fistulae, conservative management can solve the case and surgical repair is the option when conservative treatment fails.

Reactivity of a Vanadium(III) Center over an Oxo Surface Modeled by Calix[4]arene.

Metalation of the monomethoxycalix[4]arene [p-Bu(t)-calix[4]-(OMe)(OH)(3)], 1, using [VMes(3).THF] led to the coordinatively unsaturated V(III)-d(2) fragment [p-Bu(t)-calix[4]-(OMe)(O)(3)V] collapsing to the dimer [{&mgr;-p-Bu(t)-calix[4]-(OMe)(O)(3)}V](2), 2, where each calix[4]arene shares a bridging oxygen donor atom. The dimeric complexity remains intact in the reaction with Bu(t)NC and PhCN, which bond to the metal inside the cavity of the calix[4]arene leading to [{&mgr;-p-Bu(t)-calix[4]-(OMe)(O)(3)}(V-L)](2) [L = Bu(t)NC, 3; L = PhCN, 4]. In contrast, the reaction of 2 with pyridine and 4,4'-dipyridyl cleaves the dimeric form into a monomeric complex [{&mgr;-p-Bu(t)-calix[4]-(OMe)(O)(3)}V(Py)], 5, or to a different dimer containing a bridging 4,4'-dipyridyl, [{p-Bu(t)-calix[4]-(OMe)(O)(3)}(2)V(2)(&mgr;-4,4'-dipyridyl)], 6. Complex 2 undergoes one electron oxidation by I(2) to the corresponding vanadium(IV) dimer [{&mgr;-p-Bu(t)-calix[4]-(O)(4)}(2)V(2)], 7, and by p-benzoquinone to [{p-Bu(t)-calix[4]-(OMe)(O)(3)}V](2)(&mgr;-C(6)H(4)O(2)), 8. A two-electron oxidation of the V(III)-d(2) to vanadium(V) derivatives has been achieved using styrene epoxide and diphenyldiazomethane. In the former case the oxovanadium(V) derivative was obtained [p-Bu(t)-calix[4]-(OMe)(O)(3)V=O], 9, and in the latter case a metallahydrazone complex [p-Bu(t)-calix[4]-(OMe)(O)(3)V&tbd1;N-N=CPh(2)], 10. The dimeric d(2) and d(1) derivatives display significant antiferromagnetic couplings between the two metal centers, namely as follows: 2, J = -74.6 cm(-)(1); 3, J = -17 cm(-)(1); 4, J = -33.4 cm(-)(1); 7, J = -131.7 cm(-)(1). The extended Hückel calculations gave an appropriate picture of the two-electron reduction of the diphenyldiazomethane substrate. The proposed structures have been supported by X-ray analyses on 3, 7, 9, and 10.