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Sickle Cell Disease
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
PulmonaryAcute chest syndrome is a serious complication of sickle cell disease presents with chest pain, dyspnea, tachypnea, fever, cough, leukocytosis, and pulmonary infiltrates. It is usually a result of infection, vaso-occlusion, or bone marrow embolizationChronic restrict lung disease as a consequence of chronic vaso-occlusive damagePulmonary hypertension
Haematological disorders
Published in Judy Bothamley, Maureen Boyle, Medical Conditions Affecting Pregnancy and Childbirth, 2020
Acute chest syndrome is a serious and often fatal complication due to sickling with vasoocclusion in the lungs. Acute chest syndrome can complicate 7–20% of pregnancies (Serjeant, et al., 2004). Symptoms include fever, coughing, chest pain and shortness of breath with audible crackles. Infection may be involved (Oteng-Ntim, et al., 2005) but recent findings also suggest embolus of bone marrow (Duffy, 2004). Treatment is difficult and may include mechanical ventilation, exchange blood transfusion, heparin and antibiotics (Oteng-Ntim, et al., 2005).
Hemoglobinopathies and Thalassemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Children and adults with sickle cell diseases are prone to pulmonary complications referred to as the acute chest syndrome (ACS). An epidemiologic study has shown that new infiltrates occur in 12.8 per 100 patient-years. In young children the ACS was more apt to be due to infection and is seldom associated with pain. In older children and adults concomitant rib infarction, demonstrable by radionuclide bone scintigraphy in 50% of cases, is the primary event. Presumably the inflammation surrounding infarcts in ribs creates pleuritis, splinting, atelectasis, hypoxemia, and increased sickling. The clinical picture may rapidly proceed to adult respiratory distress syndrome or multiorgan failure. (See below for therapy.) The number of episodes of ACS determines life expectancy. Severe episodes of bone infarction may result in embolism of necrotic marrow into the lungs, brain, kidney, and other organs. It is difficult to recognize and is usually fatal.
Cerebral fat embolism syndrome in a patient with homozygous sickle cell disease in the setting of multisystem inflammatory syndrome in children
Published in Baylor University Medical Center Proceedings, 2023
Kirstin Sepulveda, Tesneem Issa, Gueorgui Dubrocq
A 19-year-old man with a history of homozygous sickle cell disease was emergently evaluated for symptoms of a vaso-occlusive pain crisis after presenting with fever, tachycardia, tachypnea, hypertension, altered mental status, and a 1-day history of pain in his chest, shoulders, and bilateral knees. Due to concern for acute chest syndrome, the patient was immediately started on an aggressive pain management regimen and empiric antibiotics with minimal improvement. Despite escalation of treatment, the patient continued to decompensate and was subsequently transferred to the pediatric intensive care unit for management of acute encephalopathy, cardiogenic shock, sepsis, acute hypoxic respiratory failure, coagulopathy, thrombocytopenia, acute kidney injury, elevated troponins, transaminitis, and metabolic acidosis.
Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Outcomes. The Experience of a Single Thalassemia and Sickle Cell Unit in a University Hospital
Published in Hemoglobin, 2021
Sophia Delicou, Konstantina Aggeli, Konstantinos Magganas, Dimitrios Patsourakos, Aikaterini Xydaki, John Koskinas
Acute chest syndrome is a complication of sickle cell disease characterized by pleuritic chest pain, fever, rales and chest X-ray pulmonary infiltrations. In 1979, Charache et al. [1] first proposed the use of the word “acute chest syndrome” for this complication, recognizing the difficulties in evaluating its pathogenesis. Acute chest syndrome occurs in as many as half of sickle cell disease patients [2], is among the primary causes of hospitalization and a potential cause of death. In the sickling conditions, the pathophysiology of acute lung injury is complex. This process can be caused by microbial or viral infection, in situ vaso-occlusion, fat embolisms from ischemic/necrotic bone marrow or thromboembolism, and it is often difficult to determine a definite cause. Important factors in ACS pathogenesis include functional asplenia, decreased serum opsonic activity and relatively poor antibody response to the bacteria’s capsule polysaccharide component [3].
Asthmonia: A clinical definition of a commonly used colloquial term
Published in Journal of Asthma, 2018
Caleb D. Wasser, Anna Grushevsky, Stephanie T. Johnson, Sharon R. Smith
Medical diagnoses are constantly evolving as evidenced by Sir William Osler's 1892 definition of asthma as a neurotic affection compared to the more recent definition of asthma as chronic inflammation with constriction (6). With time, further clinical research and pharmaceutical developments, advancements are made to better elicit medical entities and their therapies. With this progress, we need to consider if the colloquial use of terms such as asthmonia, bear weight in influencing medical care. The overlap of asthma with chronic obstructive pulmonary disease lead to a descriptive paper regarding therapeutic options for this newly defined syndrome (7). Another example of a syndrome defined by the intersection of two disease entities is acute chest syndrome; sickle cell pain crisis with concurrent new infiltrate on chest radiograph (8). There is an accepted definition of acute chest syndrome that includes a new infiltrate, chest pain, fever, hypoxia and respiratory symptoms. Although the etiology of acute chest syndrome is complex, infections have been documented in about 1/3 of cases. Management for acute chest syndrome is now recognized as requiring therapy for sickle cell pain crises, respiratory support as necessary and broad spectrum antibiotics (9). We believe that management of asthmonia will also require a multifactorial approach in proper management as well.