Results of the home mechanical ventilation national program among adults in Chile between 2008 and 2017.

BACKGROUND: Home mechanical ventilation (HMV) is a viable and effective strategy for patients with chronic respiratory failure (CRF). The Chilean Ministry of Health started a program for adults in 2008. METHODS: This study examined the following data from a prospective cohort of patients with CRF admitted to the national HMV program: characteristics, mode of admission, quality of life, time in the program and survival. RESULTS: A total of 1105 patients were included. The median age was 59 years (44-58). Women accounted for 58.1% of the sample. The average body mass index (BMI) was 34.9 (26-46) kg/m2. A total of 76.2% of patients started HMV in the stable chronic mode, while 23.8% initiated HMV in the acute mode. A total of 99 patients were transferred from the children's program. There were 1047 patients on non-invasive ventilation and 58 patients on invasive ventilation. The median baseline PaCO2 level was 58.2 (52-65) mmHg. The device usage time was 7.3 h/d (5.8-8.8), and the time in HMV was 21.6 (12.2-49.5) months. The diagnoses were COPD (35%), obesity hypoventilation syndrome (OHS; 23.9%), neuromuscular disease (NMD; 16.3%), non-cystic fibrosis bronchiectasis or tuberculosis (non-CF BC or TBC; 8.3%), scoliosis (5.9%) and amyotrophic lateral sclerosis (ALS; 5.24%). The baseline score on the Severe Respiratory Insufficiency questionnaire (SRI) was 47 (± 17.9) points and significantly improved over time. The lowest 1- and 3-year survival rates were observed in the ALS group, and the lowest 9-year survival rate was observed in the non-CF BC or TB and COPD groups. The best survival rates at 9 years were OHS, scoliosis and NMD. In 2017, there were 701 patients in the children's program and 722 in the adult´s program, with a prevalence of 10.4 per 100,000 inhabitants. CONCLUSION: The most common diagnoses were COPD and OHS. The best survival was observed in patients with OHS, scoliosis and NMD. The SRI score improved significantly in the follow-up of patients with HMV. The prevalence of HMV was 10.4 per 100,000 inhabitants. Trial registration This study was approved by and registered at the ethics committee of North Metropolitan Health Service of Santiago, Chile (N° 018/2021).

Catastrophic antiphospholipid antibody syndrome presenting as acute vascular occlusion in a young female patient / Síndrome de anticuerpos antifosfolípidos catastrófico que se presenta con oclusión vascular aguda en una paciente joven

Acquired thrombotic and thromboembolic disorders may be presented initially with symptoms and signs of acute ischaemia or organ dysfunction that will lead many of these patients to seek care in the emergency department. We report a case of a 19-year-old female patient who developed catastrophic antiphospholipid syndrome (CAPS syndrome or Asherson syndrome) 6 weeks post stillbirth with an initial presentation of acute vascular occlusion. The patient was immediately operated and anticoagulated with significant improvement (AU)

Los trastornos trombóticos y tromboembólicos adquiridos pueden manifestarse inicialmente con signos y síntomas de isquemia aguda o disfunción orgánica que derivará a muchos de estos pacientes al servicio de urgencias. Se presenta el caso de una paciente de 19 años de edad que desarrolló un síndrome antifosfolípido catastrófico (o síndrome de Asherson) 6 semanas después del parto de un feto muerto con una presentación inicial de oclusión vascular aguda. La paciente fue intervenida inmediatamente y se inició un tratamiento con anticoagulantes que supuso una mejora significativa (AU)

Catastrophic antiphospholipid antibody syndrome presenting as acute vascular occlusion in a young female patient.

Acquired thrombotic and thromboembolic disorders may be presented initially with symptoms and signs of acute ischaemia or organ dysfunction that will lead many of these patients to seek care in the emergency department. We report a case of a 19-year-old female patient who developed catastrophic antiphospholipid syndrome (CAPS syndrome or Asherson syndrome) 6 weeks post stillbirth with an initial presentation of acute vascular occlusion. The patient was immediately operated and anticoagulated with significant improvement.

A report of disseminated adenocarcinoma presenting as thrombotic thrombocytopenic purpura.

Thrombotic microangiopathies (TMAs) represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP), typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS), in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP) Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.

Knee arthrodesis with the Vari-Wall nail for treatment of infected total knee arthroplasty.

We reviewed 20 patients who had undergone one-stage (7 cases) or two-stage (13 cases) knee arthrodesis using the Vari-Wall intramedullary nail, as a salvage operation following infection of a total knee arthroplasty. The procedure was followed by systemic antibiotic administration and early rehabilitation. Intraoperative microbiological cultures were taken. The average period of follow-up was 20 months. Solid union was achieved in 80%; mean time to fusion was 9 months. There was no recurrence of infection. The average limb length discrepancy was 2.45 cm. A walking aid was needed by 95% of the patients. The complication rate was 30% including 4 pseudarthroses, one intraoperative fracture and one peroneal nerve palsy. The Vari-Wall intramedullary nail is a good option when an arthrodesis is indicated for salvage of an infected total knee arthroplasty. It can be performed in one or two stages depending on several factors such as microbiologic culture results. It achieved good pain relief and acceptable functional results in this study.