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Pathophysiology of the acute chest syndrome in sickle cell disease.
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New insights into the pathophysiology of acute chest syndrome (ACS) have highlighted potential therapeutic strategies including the use of nitric oxide. A multidisciplinary approach to the management of patients admitted with a sickle cell crisis is needed to prevent progression to ACS and need for mechanical ventilation.
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... 8,12 However, it is important to note that our study did not address whether patients were on the maximum tolerated dose of hydroxyurea or not and at which age hydroxyurea was offered. 13,14 Several previous studies have reported concurring findings of increased risk of mortality among patients with SCD admitted to the ICU. In addition, five-year mortality is linked with a history of long ICU stay. ...
Purpose
Sickle cell disease (SCD) comprises a complex group of hematologic disorders that are collectively the most common monogenic disorder and are associated with increased risk of intensive care unit admission (ICU). The purpose of this study is to investigate factors that predict admission of adult patients with SCD to the ICU.
Patients and Methods
This was a cross-sectional study that enrolled adult patients with SCD from Saudi Arabia.
Results
A total of 107 patients with SCD, with a median age 31.9±12.1 years, were evaluated retrospectively. Regarding predictors of ICU admission, patients who indicated a history of blood transfusions were at 8.047-fold higher risk of ICU admission (OR=8.047; 95% CI=2.392–27.07; p=0.001). Patients who started hydroxyurea were at least 3.071 times more likely to be admitted than those who did not (OR=3.071; 95% CI=1.164–8.104; p=0.023). We also observed three or more hospitalizations per year to be associated with increased risk of ICU admission (OR=3.393; 95% CI=1.285–8.960; p=0.014), with those making 3 to 5 visits annually having at least 10.4 times higher risk (OR=10.38; 95% CI=10.098–98.19; p=0.041) and those with 6 to 10 ER admissions having 18 times higher risk (OR=18.00; 95% CI=2.149–150.8; p=0.008). Finally, patients with high WBC were predicted to have at least 3.34 times higher risk of ICU admission (OR=3.337; 95% CI=1.131–9.846; p=0.029).
Conclusion
SCD is a multi-systemic disease associated with increased morbidity and mortality. Recognition of high-risk features in patients helps to eliminate subjectivity in ICU referral decision. Frequent hospitalization and emergency visits, multiple blood transfusions, and elevated white blood cell count were significantly associated with a higher rate of ICU admission despite hydroxyurea usage.
... One of them is pulmonary infection caused most frequently by Chlamydia, Mycoplasma and viral infections. 2 On the other hand, severe vaso-occlusive crisis of multiple bones can cause fat embolism with pulmonary vascular obstruction and pulmonary infarction. When it affects the thorax may cause regional alveolar hypoventilation. ...
... 30,35 A frequent cause of hospitalisation and a leading cause of death is the acute chest syndrome (ACS), which is a clinical syndrome characterised by a new pulmonary infiltrate on chest X-ray in a patient with either dyspnoea, pleuritic pain, cough or fever and often a fall in the haemoglobin level ( figure 2). 31,37,[42][43][44] Importantly, it can initially present with a normal chest X-ray or solely with perfusion defects on a lung radioisotope scan. The ACS occurs in 15 to 40% of patients, and often recurs. ...
... With deterioration of the patient's condition or progression of pulmonary infiltrates the diagnosis is more likely an ACS and blood (exchange) transfusions should be immediately instituted. 42,44,93 There are no data regarding the role of anticoagulation in the management of an ACS, but with documentation of thromboembolism anticoagulation is warranted. ...
Sickle cell disease (SCD) is a heterogeneous disorder, with clinical manifestations including chronic haemolysis, an increased susceptibility to infections and vaso-occlusive complications often requiring medical care. Patients with SCD can develop specific and sometimes life-threatening complications, as well as extensive organ damage reducing both their quality of life and their life expectancy. Proven effective treatment options for sickle cell patients are limited to hydroxyurea, blood transfusions and bone marrow transplantation. With the increasing prevalence of SCD in the Netherlands, a fundamental understanding of its pathophysiology and clinical syndromes is of importance for local medical practitioners.
Les complications pulmonaires aiguës au cours de la drépanocytose sont fréquentes et sont regroupées sous le terme de syndrome thoracique aigu. Ce syndrome clinique est défini par l’association d’une anomalie radiologique thoracique récente à une manifestation clinique : douleur thoracique, ou fièvre, ou dyspnée. Nous rapportons l’observation d’un patient âgé de 26 ans chez qui la survenue d’une embolie graisseuse avec syndrome thoracique aigu nous a permis de découvrir une drépanocytose, jusque-là méconnue. Le lavage broncho-alvéolaire a contribué à l’enquête étiologique par la mise en évidence des macrophages lipophagiques. Ce travail insiste sur la fréquence de la survenue des embolies graisseuses au cours d’un syndrome thoracique aigu et sur leur gravité potentielle.
There are three haptoglobin phenotypes in humans designated: Hp1–1, Hp2–1, and Hp2–2. The Hp1–1 phenotype has been shown to be protective against certain diseases, and this has been suggested to be the result of better anti-inflammatory and antioxidative properties compared to haptoglobin polymers of the other phenotypes when clearing cell-free haemoglobin. We propose the use of haptoglobin for the treatment of sickle cell disease, where an oxidative state exists caused by a high level of cell-free haemoglobin. A significant number of sickle cell disease patients are severely affected and experience regular acute painful episodes resulting in hospitalisation.Therapeutic treatments for sickle cell disease are limited and therefore haptoglobin could represent a vital alternative therapy. A method has been developed as part of the commercial fractionation of plasma for preparing haptoglobin enriched for dimers. This is significant as it uses a mixture of plasma of all haptoglobin phenotypes, and allows annual production of hundreds of kilograms quantities of haptoglobin that may be required to allow treatment of thousands of sickle cell disease patients worldwide.
Acquired immune deficiency syndrome (AIDS) caused by human immune deficiency virus (HIV), once dominated by infected males has become feminized especially in sub-Saharan Africa where the majority of adults living with the condition are females. Positive life styles, belonging to social support groups and stigma-free HIV services by providers may have good impact on the quality of life of HIV-positive mothers. This study was aimed at assessing the determinants of subjective health status of HIV-positive mothers accessing prevention-of-mother-to-child-transmission (PMTCT) of HIV services in Nnamdi Azikiwe University Teaching Hospital (NAUTH) Nnewi.
This is a descriptive study in which 288 consenting HIV positive mothers were selected using the systematic sampling technique. Data on demographics, life style, social support, contraceptive use and subjective feeling about current health status were collected from the subjects using a pre-tested, structured, interviewer-administered questionnaire.
The mean age of the respondents was 30.46 +/- 4.86 years. Majority (89.2%) of them were married while 55.2% were traders. A significantly higher proportion of the mothers on highly active anti retroviral therapy (HAART) (70.8%) than non users (29.2%) described their current health status as 'excellent' (p<0.001). Also a significantly higher proportion of condom users (99%) than pill users (1%) described their health status as 'excellent' (p<0.02). The same significantly higher proportions of 'excellent' response were given by subjects who engage in social support activities (p<0.001), who practice good feeding (p<0.01) andpersonal hygiene (p<0.01).
Access to family planning services and HAART, participation in support group activities and positive lifestyle practices tend to improve subjective health status and should be comprehensively encouraged among the HIV positive mothers.
Surgery in patients with sickle cell disease is associated with high morbidity. To reduce this high morbidity, different preoperative transfusion regimens were introduced. However, blood transfusion is associated with problems. This prospective study aims to establish the safety of conducting laparoscopic cholecystectomy without transfusion in sickle cell disease patients.
Forty patients (16 males and 24 females; mean age 26.6 years) undergoing laparoscopic cholecystectomy for cholelithiasis were divided into 2 matched groups: Group I "no transfusion" (n=24 patients; 60%) and Group II "transfusion" (n=16; 40%). In Group II, 9 patients (22.5%) received a simple transfusion and 7 (17.5%) a partial exchange transfusion.
Group II patients had significantly higher levels of Hb-S prior to transfusion. They developed a significantly higher complication rate (25% vs. 0%) and subsequently longer hospital stay (3.9±2 vs. 2.1±1.4). Moreover, there was no significant difference in the complications between the simple transfusion and partial exchange transfusion subgroups.
Surgery in SCD patients is safe without a preoperative blood transfusion. Moreover, preoperative blood transfusion is associated with significantly higher postoperative complications and longer hospital stay. Hence, a "no transfusion" policy is recommended.
Cardiovascular risk control has become one of the hallmarks in the treatment of diabetes and coronary heart disease, yet assessment of individual risk factors is suboptimal. We have designed a new Hypertension Screening Facility (HSF) for the evaluation of cardiovascular risk in hypertensive patients, based on 1) systematic, protocol-driven (WHO/ISH-based) analysis by nurse practitioners, 2) computer-assisted reporting of results and advice, 3) risk assessment using a Decision Support System (DSS), 4) maintenance of the autonomy of the GP. In a pilot study we wanted to investigate this HSF.
Survey 1 addressed a. how general practitioners deal with hypertension, b. whether they intend to and do use existing clinical guidelines, c. what their opinions are towards changes in the current process of care. In survey 2, we evaluated the attitude of GPs using the HSF. Responses were 43% (51 out of 120) to the first survey and 100% (20 out of 20) to the second.
The majority of physicians included lifestyle in their assessment of risk factors and management of hypertension. Consideration of age and a positive family history was extremely high. In contrast, vision disturbances, ECG and microalbuminuria were not often considered. In the absence of additional risk factors, drug treatment was initiated in patients with a mean systolic blood pressure of 162+/-6 over 99+/-4 mmHg. In the presence of risk factors (obesity, smoking and a positive family history of cardiovascular disease) treatment is started at an average blood pressure of 154+/-8 over 96+/-4 mmHg. Opinions towards a change in management of hypertensive patients were generally positive. The opinions about the new HSF and the cardiovascular risk were reported to the general practitioner and considered useful or very useful by 79%.
The present study thus confirms that cardiovascular risk evaluation by GPs is suboptimal, but there is a positive attitude towards an improvement in their assessment by HSF. The novelty of the HSF is that it respects the autonomy of the GP and brings the expertise to the GP.
