Rib infarcts and acute chest syndrome in sickle cell diseases

doi:10.1016/0140-6736(91)92525-7

The Lancet

Volume 337, Issue 8745, 6 April 1991, Pages 831-833

https://doi.org/10.1016/0140-6736(91)92525-7 Get rights and content

Abstract

In the absence of evidence for pneumonia or pulmonary embolus, primary pulmonary infarction has been assumed to be the cause of the syndrome of chest pain, fever, and pulmonary infiltrate on chest X-ray that commonly complicates sickle cell anaemia. To find out whether the syndrome might be due to rib infarction, ^99mTc-diphosphonate bone scans were done. In the eleven episodes thus investigated (10 patients) the scans showed segmental areas of increased radionuclide uptake in ribs, indicative of bone infarction. A possible sequence of events is that the rib infarcts are primary and cause bone pain, followed by soft tissue reaction, pleuritis, and splinting. The resultant hypoventilation leads to atelectasis and subsequent development of the radiographic changes of the acute chest syndrome. Prevention of hypoventilation and treatment of bone pain are important therapeutic goals.

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La drépanocytose, maladie génétique due à une mutation du gène de la β-globine, est une pathologie systémique fréquente en France, caractérisée par de multiples complications vaso-occlusives. Le syndrome thoracique aigu, à l’origine d’une morbi-mortalité significative, doit être connu de l’interniste. Il est défini par la survenue brutale d’un ou plusieurs signes cliniques respiratoires associés à une nouvelle image radiologique. Sa physiopathologie, complexe, associe des phénomènes vaso-occlusifs (thrombus intravasculaire pulmonaire, embolie graisseuse), infectieux, et une hypoventilation alvéolaire. Le génotype S/S ou S/β⁰-thalassémie, un antécédent d’hospitalisation pour crise vaso-occlusive osseuse ou syndrome thoracique aigu, un taux d’hémoglobine F bas (< 5 %), une hémoglobine de base élevée (> 10 g/dL), ou une leucocytose (> 10 G/L) sont des facteurs de risque reconnus. Le syndrome thoracique aigu chez l’adulte se présente habituellement par une douleur thoracique fébrile, une dyspnée et des crépitants ou un souffle tubaire uni ou bi-basal. Des opacités alvéolaires des lobes inférieurs associées ou non à un épanchement pleural sont caractéristiques à l’imagerie. La prise en charge doit être précoce. Elle associe un traitement antalgique multimodal, une oxygénothérapie, parfois une antibiothérapie, et l’utilisation large des transfusions dès qu’apparaissent des signes de gravité. La prévention du syndrome thoracique aigu passe par la surveillance rapprochée des patients en hospitalisation et l’utilisation de la spirométrie incitative.
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Sickle cell disease is a common but often poorly understood by chest physicians. The acute chest syndrome represents its main respiratory complication.
Sickle cell disease is an autosomal recessive disorder inducing, in certain circumstances, sickling of red cells. Natives from western or central Africa and from the Caribbean islands are mainly affected. Acute chest syndrome is defined by the association of chest pain or fever and recent radiographic infiltrates, in patients suffering from sickle cell disease. Determination of etiology, infection, fat embolism or hypoventilation, is difficult, as a self-perpetuating vicious circle is ongoing. Support, largely undervalued, is based on etiological treatment and measures to avoid worsening linked to complications, especially microcirculatory disease.
Acute chest syndrome is a severe respiratory complication of sickle cell disease. Therapeutic measures are simple but undervalued.
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La drépanocytose est une maladie génétique caractérisée par la présence d'une hémoglobine anormale circulante. Internistes, urgentistes et réanimateurs sont impliqués dans la prise en charge des complications de la drépanocytose, en particulier des crises vaso-occlusives. En dehors de ces crises vaso-occlusives, la drépanocytose se complique de défaillances d'organe chroniques d'origine ischémique, et d'infections potentiellement sévères. Chez l'adulte, les crises vaso-occlusives sont la principale raison d'admission à l'hôpital, voire de décès. En dehors des douleurs articulaires, le syndrome thoracique aigu, le priapisme, l'accident vasculaire cérébral ischémique ou hémorragique, les douleurs abdominales et le syndrome de défaillance multiviscérale sont les principaux modes de révélation de ces crises. La prise en charge thérapeutique est multidisciplinaire. Elle comprend le traitement symptomatique des crises vaso-occlusives, la transfusion, voire l'exsanguinotransfusion selon la sévérité de la crise. Une meilleure compréhension de la maladie et de la physiopathologie des complications fait qu'aujourd'hui la moitié des patients drépanocytaires vivent au-delà de 50 ans.
Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the most prevalent genetic disease at birth in the Ile-de-France area. Internists and intensivists are involved in the management of acute complications, particularly acute vaso-occlusive crisis. Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to the ischaemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to the hospital and the main cause of death. It mainly manifests by osteoarticular pain but other clinical complications can be observed such as acute chest syndrome, priapism, ischaemic or haemorrhagic stroke, abdominal pain and acute multivisceral failure. The treatment of acute vaso-occlusive crisis is symptomatic. Simple transfusion or partial exchange transfusion is required in the more severe form of vaso-occlusive crisis. The management of adult patients with sickle cell disease must be based on a multidisciplinary approach. At the present time, more than 50% of patients survive beyond the fifth decade. This better and longer life in developed countries has resulted from basic investigations and symptomatic treatments.
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Citation Excerpt :
Bone tenderness over the site of infarction is commonly seen. A bone scan 48 to 72 hours after admission can confirm the diagnosis [37,38]. The cause of acute lung injury in SCD is multifactorial.
The acute chest syndrome
2005, Hematology/Oncology Clinics of North America

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CLINICAL PRACTICERib infarcts and acute chest syndrome in sickle cell diseases

Abstract

J Pediatr

Blood

'Acute chest syndrome' in adults with sickle cell anemia

Arch Int Med

Clinical features of the genetic variants of sickle cell disease

Bull Johns Hopkins Hosp

Acute pulmonary disease in sickle cell anemia

Am Rev Resp Dis

Acute lung syndrome during painful sickle cell crisis—relation to site of pain and narcotic requirement

Blood

Acute chest syndrome in children with sickle cell disease

Am J Ped Hematol/Oncol

Rib infarcts may cause acute chest syndrome in sickle cell anemia

Blood

Sickle cell anemia

Arch Pathol

Pulmonary changes in sickle cell disease

Am Rev Resp Dis

CLINICAL PRACTICE
Rib infarcts and acute chest syndrome in sickle cell diseases