Children with MEN1 gene mutations may present first (and at a young age) with Cushing disease.

OBJECTIVE: Cushing disease (CD) is a rare entity caused by ACTH-secreting pituitary tumours, leading to prolonged hypercortisolism. Most cases are sporadic but can rarely occur in the context of familial predisposition, due to germline mutations in genes such as MEN1, leading to multiple endocrine neoplasia type 1, MEN1. We have reported previously that CD can be the first and only presenting manifestation of MEN1. In this report, we describe a cohort of paediatric patients who presented with CD as the first manifestation of MEN1. MATERIALS AND METHODS: A retrospective analysis of paediatric patients admitted to the National Institutes of Health (NIH) Clinical Center for evaluation of hypercortisolism, between 1997 and 2017. MEN1 was diagnosed on a clinical, familial and/or genetic basis. RESULTS: Of a total of 238 children with CD, six patients were subsequently diagnosed with MEN1, three males and three females with a mean age at diagnosis of CD at 13.4 ± 2.9 years. Five of the six patients had familial MEN1 and one patient was a sporadic case. Additional manifestations of MEN1 included primary hyperparathyroidism in three patients and hyperprolactinemia in two patients. DISCUSSION: This report describes a paediatric patient population with MEN1 in whom CD was the initial manifestation, confirming a previous observation that paediatric patients with MEN1 may present first with an ACTH-producing adenoma. Therefore, germline MEN1 mutations should be sought in paediatric CD and tested for when there is a suggestive family history and/or other manifestations.

Prevalence and risk factors of long-term proton pump inhibitors-associated hypomagnesemia: a cross-sectional study in hospitalized patients.

BACKGROUND: Proton pump inhibitors (PPI)-related hypomagnesemia is a potentially life-threatening adverse event first described in 2006. PPIs are widely used in the general population. Information regarding prevalence and risk factors is scarce. We conducted a cross-sectional study in inpatients to evaluate prevalence and associated factors with hypomagnesemia in chronic PPIs users. This is a cross-sectional study of hospitalized adult patients with chronic use of PPIs from January 01, 2012, to December 31, 2018. Chronic use was defined as taking PPIs at least 6 months before hospital admittance. Data were collected from informatized medical records from a University Hospital (Hospital Italiano de Buenos Aires). Hypomagnesemia was defined as a value equal to or less than 1.7 mg/dl. The first hospitalization measurement was retrieved. Thirty-six percent of patients (95% CI 30-43) with chronic PPI use presented hypomagnesemia at admission. Patients with hypomagnesemia presented a higher prevalence of chronic kidney disease (18.6% vs 8%, p < 0.05), more use of oral magnesium supplementation (20.9% vs 8%, p < 0.05), use of corticosteroids (32.6% vs 19.3%, p = 0.06) and calcineurin inhibitors (17.4% vs 6.7%, p < 0.05). Regarding laboratory findings, they presented lower hematocrit (28.7% vs 32.8%, p < 0.05), phosphatemia (3 mg/dl vs 3.4 mg/dl, p < 0.05), natremia (135 mg/dl vs 136 mg/dl, p < 0.05) and albumin levels (2.8 g/dl vs 3.2 g/dl p < 0.05) when compared to those who presented normomagnesemia. Hypocalcemia was more frequent among patients with hypomagnesemia (57% vs 38.7%, p < 0.05). In the multivariate analysis, hyponatremia, decreasing levels of hematocrit (odds ratio, OR 0.93-CI 95% 0.88-0.98) and malignant bone compromise (OR 2.83-CI 95% 1.04-7.7) were associated with hypomagnesemia. Adult patients with long-term use of PPIs have a high prevalence of hypomagnesemia. Increasing age, female sex, concomitant use of drugs that impair tubular function and chronic kidney disease may enhance this phenomenon. Anemia, hyponatremia and malignant bone compromise were associated factors with PPIs-related hypomagnesemia.

[Invasive liver abscess syndrome caused by Klebsiella pneumoniae, case series]. / Síndrome de absceso hepático invasor por Klebsiella pneumoniae, serie de casos.

BACKGROUND: Primary liver abscesses caused by Klebsiella pneumoniae and their related systemic complications produce the invasive liver abscess syndrome due to Klebsiella pneumoniae. AIM: To describe the clinical, epidemiological and evolution characteristics in our center. METHODS: A retrospective cohort of hospitalized adults in Hospital Italiano de Buenos Aires between January 1st, 2001 and May 1st, 2020. We included patients with diagnosis of abscess in any organ with rescue in culture or positive blood culture for Klebsiella pneumoniae. Epidemiological, clinical characteristics and prognosis of those with hepatic primary localization were analyzed. RESULTS: 10 patients were included. Two (20%) patients were Oriental. Median age was 69 years (interquartile range 64-79), nine (90%) were men. The most frequent comorbidity was type 2 diabetes (40%). Four (40%) patients had spread to other organs. The median hospitalization was 21.5 days (IIC 15-43), 60% (n: 6) were hospitalized in the intensive care unit and 30% (n: 3) died. CONCLUSIONS: The invasive liver abscess syndrome due to Klebsiella pneumoniae is a rare life-threatening disease. Our study reports the characteristics of patients with this syndrome in our population.

Emergency department visits and hospital readmissions in an Argentine health system.

BACKGROUND AND GOAL OF STUDY: The scope of health in the Sustainable Development Goals is much broader than the Millennium Development Goals, spanning functions such as health-system access and quality of care. Hospital readmission rate and ED-visits within 30 days from discharge are considered low-cost quality indicators. This work assesses an indicator of quality of care in a tertiary referral hospital in Argentina, using data available from clinical records. PURPOSE: To estimate the rate of ED-visits and the hospital readmission rate (HRR) after a first hospitalization (First-H), and to identify associated factors. METHODS: This retrospective cohort included patients who had a First-H in Hospital Italiano de Buenos Aires between 2014-2015. Follow-up occurred from discharge until ED-visit, readmission, death, disaffiliation from health insurance, or 13 months. We present HRR at 30 days and ED-visits rate at 72 h, using the Cox proportional-hazards regression model to explore associated factors, and reporting adjusted hazard ratios (HR) with their respective 95 %CI. RESULTS: The study comprised 10,598 hospitalizations (median age was 68 years). Of these, 5966 had at least one consultation to the ED during follow up, resulting in a 24 h rate of consultations to ED of 1.51 % (95 %CI 1.29-1.72); at 48 h 3.18 % (95 %CI 2.86-3.54); at 72 h 4.71 % (95 %CI 4.32-5.13). In multivariable models, factors associated for 72 h ED-visits were: age (aHR 1.06), male (aHR 1.14), Charlson Comorbidity Index (aHR 1.16), unscheduled hospitalization (aHR 1.39), prior consultation with the ED (aHR 1.08) and long hospital stay (aHR 1.39). Meanwhile, 2345 patients had at least one hospital readmission (98 % unscheduled), resulting a 24 h rate of 0.5 % (95 %CI 0.42-0.71), at 48 h 0.98 % (95 %CI 0.80-1.18), at 72 h 1.4 % (95 %CI 1.2-1.6); at 30 days 7.7 % (95 %CI 7.2-8.2); at 90 days 13 % (95 %CI 12.4-13.8); and one-year 22.5 % (95 %CI 21.7-23.4). Associated factors for HRR at 30 days were: age (HR 1.16), male (HR 1.09), Charlson comorbidities score (HR 1.27), social service requirement during First-H (HR 1.37), unscheduled First-H (HR 1.16), previous ED-visits (HR 1.03) and length of stay (HR 1.08). CONCLUSION: Priorities efforts to improve must include greater attention to patients' readiness prior discharge, to explore causes of preventable readmissions, and better support for patient self-management.

[Associated factors with the presence of secondary neoplastic cells in the cerebrospinal fluid of patients with suspected carcinomatous meningitis]. / Factores asociados a presencia de células neoplásicas secundarias en el liquido cefalorraquideo de pacientescon sospecha de meningitis carcinomatosa.

BACKGROUND: Improvement in oncologic therapy has increased survival in oncologic patients. There has been a concomitant increase in the incidence of secondary meningeal involvement. Early diagnosis is mandatory. OBJECTIVE: To identify factors associated with the presence of secondary neoplastic cells in cerebrospinal fluid of patients with suspected carcinomatous meningitis (CM) Methods: Cross-sectional study involving adult patients with solid cancer diagnosis and suspected CM between 2004 and 2014 at Hospital Italiano de Buenos Aires. All included patients had at least one lumbar puncture with cerebrospinal fluid (CSF) analysis. CM cases were defined by the presence of neoplastic cells in CFS. We evaluated the association of each factor (cancer characteristics, clinical engagement of central nervous system, CSF analysis) with CM using a logistic regression model. RESULTS: We included 77 patients: mean age was 62 years (SD 13.1), 58.4% (45) were female. The most common oncologic disease was lung cancer 29.9% (23), followed by breast 23.4% (18) cancer. CM was detected in 23.4% (18) patients. In univariate analysis, glychorrachia, the CSF leukocyte count, the meningeal involvement on MRI, headache and delirium were significantly associated with MC. In the multivariate model that included the variables significantly associated in the crude analysis, the only variable that remained significantly associated with MC was the glychorrachia (OR 0.93 95%CI 0.9 - 0.97, p <0.001). DISCUSSION: These results suggest that as the glychorrachia increases, the probability of having MC decreases. These findings are consistent with previous studies.

Síndrome de absceso hepático invasor por Klebsiella pneumoniae, serie de casos / Invasive liver abscess syndrome caused by Klebsiella pneumoniae, case series

Resumen Introducción: Los abscesos hepáticos primarios producidos por Klebsiella pneumoniae y las implicancias sistémicas relacionadas constituyen el síndrome de absceso hepático invasor por Klebsiella pneumoniae. Objetivo: Describir las características clínicas, epidemiológicas y la evolución de esta entidad en nuestro centro. Pacientes y Métodos: Cohorte retrospectiva de pacientes adultos internados en el Hospital Italiano de Buenos aires entre el 1 de enero de 2001 y el 1 de mayo de 2020. Se incluyeron pacientes con diagnóstico de absceso en cualquier órgano con aislamiento en cultivo de absceso o hemocultivo positivo para Klebsiella pneumoniae para ser analizadas las características epidemiológicas, clínicas y la evolución de aquellos con localización primaria hepática. Resultados: 10 pacientes fueron incluidos. Dos (20%) pacientes fueron orientales. La mediana de edad fue 69 años (IIC 64-79), nueve (90%) fueron hombres. La co-morbilidad más frecuente fue diabetes mellitus tipo 2 (40%). Cuatro (40%) pacientes tuvieron diseminación a otros órganos. La mediana de internación fue 21,5 días (IIC 15-43), 60% (n: 6) requirió internación en unidad de cuidados intensivos y 30% (n: 3) de los pacientes falleció. Conclusión: El síndrome de absceso hepático invasor por Klebsiella pneumoniae es una enfermedad infrecuente, potencialmente mortal. Este estudio intenta reportar las características de los pacientes con esta patología en nuestra población.

Abstract Background: Primary liver abscesses caused by Klebsiella pneumoniae and their related systemic complications produce the invasive liver abscess syndrome due to Klebsiella pneumoniae. Aim: To describe the clinical, epidemiological and evolution characteristics in our center. Methods: A retrospective cohort of hospitalized adults in Hospital Italiano de Buenos Aires between January 1st, 2001 and May 1st, 2020. We included patients with diagnosis of abscess in any organ with rescue in culture or positive blood culture for Klebsiella pneumoniae. Epidemiological, clinical characteristics and prognosis of those with hepatic primary localization were analyzed. Results: 10 patients were included. Two (20%) patients were Oriental. Median age was 69 years (interquartile range 64-79), nine (90%) were men. The most frequent comorbidity was type 2 diabetes (40%). Four (40%) patients had spread to other organs. The median hospitalization was 21.5 days (IIC 15-43), 60% (n: 6) were hospitalized in the intensive care unit and 30% (n: 3) died. Conclusions: The invasive liver abscess syndrome due to Klebsiella pneumoniae is a rare life-threatening disease. Our study reports the characteristics of patients with this syndrome in our population.