Postoperative acute chest syndrome in sickle cell disease

62 patients with homozygous sickle cell (SS) disease who were subjected to cholecystectomy for symptomatic gall stones were analysed to determine the frequency and outcome of postoperative acute chest syndrome (ACS). Patients were selectively transfused preoperatively where haemoglobin levels were found to be >1 g/dl below steady state values. All patients had open cholecystectomy. 11 patients developed postoperative ACS. Age range was 8 to 54 years among cholecystectomy patients and 10 to 38 years among ACS cases. Two of the 11 received preoperative simple transfusion and haemoglobin levels ranged from 5.6 to 8.6 g/dl among the 9 not transfused. ACS precipitated falls in O2 partial pressures below 70 mm Hg on room air in 10 of the 11 cases. All but one of the patients received therapeutic transfusion (3 exchange and 7 simple) during their phase of clinical deterioration culminating in multi-system failure. ACS remains the leading cause of post operative morbidity and mortality in sickle cell disease. Hydration attention to infection, oxygen therapy via mask or positive pressure ventilation, simple or exchange transfusion and chest physiotherapy remain standard treatment. The potential for multi-system failure makes patient outcome unpredictable.(AU)

Myocardial infarction in Antigua: 1990 to 1995

Between January 1990 and May 1995, 117 patients were admitted to the Intentsive Care at Holberton Hospital, Antigua, for chest pain due to suspected acute myocardial infarction. 39 (45 percent) of 86 patients whose records were available for retrospective review had confirmed (27 patients) or probable (12 patients) acute myocardial infarction. Risk factors identified among the patients included hypertension, diabetes, tobacco smoking, hypercholesterolaemia and obesity. On admission, 82 percent were Killip class I and 18 percent were Killip class II. Medications in the Intensive Care Unit included nitrates, aspirin, calcium and channel blockers, beta-adrenergic blockers, heparin and angiotensin converting enzyme inhibitors (21 percent). No thrombolytic agents were available. THe average hospital stay was 10 days and the in-hospital mortality rate was 13 percent. These data indicate that early mortality from acute myocardial infarction can be reduced in developing countries by early admission to an Intensive Care Unit and use of drugs known to be effective in its treatment.(AU)

Pulse oximetry in a cohort study of sickle cell disease

Oxygen saturation was determined by pulse oximetry in a representative sample of Jamaican patients with steady state sickle cell disease in a cohort study from birth. There were 220 with homozygous sickle cell (SS) disease and 142 with sickle cell-haemoglobin C (SC) disease aged 9-18 years, and 122 with a normal haemoglobin (AA) genotype aged 15-18 years. Pulse oximetry (SpO2) values were lower in SS disease (mean [95 percent confidence interval]), 92.5 [92.0-93.3]. Inhalation of 100 percent oxygen in SS patients with 02 saturations below 90 percent consistency increased saturation to 99-100 percent. In SS disease, Sp02 correlated positively with haemoglobin and fetal haemoglobin and negatively with reticulocyte counts but not MCHC, MCV or bilirubin level. Mean Sp02 in SS subjects with a normal alpha globin gene complement (mean[SD], 91.7 [3.9]percent) was lower than in heterozygotes (93.4 [4.0] percent) or homozygotes (96.1 [3.0] percent) for alpha+thalassaemia, the effects of alpha-thalassaemia not being explained by differences in haemoglobin or MCHC. In SS disease, Sp02 levels were not associated with age (within this age range), sex, number of sick clinic visits or number of hospital admissions. Higher Sp02 levels were associated with greater heights and weights, more frequent painful crises and less frequent acute chest syndrome, but these associations were not significant after adjustment for haemoglobin level. Desaturation is common in steady-state SS disease and knowledge of the individual's steady-state value may be important in the interpreting low value during acute complications.(AU)

Pulse oximetry in cohort study of sickle cell disease

Oxygen saturation was determined by pulse oximetry in a representative sample of Jamaican patients with steady-state sickle cell disease in a cohort study from birth. There were 220 with homozygous sickle cell (SS) disease and 142 with sickle cell- haemoglobin C (SC) disease aged 9-18 years, and 122 with a normal haemoglobin (AA) genotype aged 15-18 years. Pulse oximetry (SpO2) values were lower in SS disease (mean [95 percent confidence interval], 92.5 [92.0-93.0]) than in SC disease (96.7 [96.9-96.9]) or AA controls (97.1 [96.8-97.3]). Inhalation of 100 percent oxygen in SS patients with O2 saturations below 90 percent consistently increased saturation to 99-100 percent. In SS disease, SpO2 correlated positively with haemoglobin and fetal haemoglobin and negatively with reticulocyte counts but not with MCHC, MCV or bilrubin level. Mean SpO2 in SS subjects with a normal alpha globin gene complement (mean [SD], 91.7 [3.9] percent) was lower than in heterozygotes (93.4 [4.0] percent) or homozygotes (96.1 [3.0] percent) for O+ thalassaemia, the effects of O-thalassaemia not being explained by differences in haemoglobin or MCHC. In SS disease, SpO2 levels were not associated with age (within this age range), sex, number of sick clinic visits or number of hospital admissions. Higher SpO2 levels were associated with greater height and weight, more frequent painful crises and less frequent acute chest syndrome, but these associations were not significant after adjustment for haemoglobin level. Desaturation is common in steady-state SS disease and knowledge of the individual's steady-state value may be important in the interpreting low values during acute complications.(AU)

Analysis of 81 cases of acute chest syndrome in adult patients with sickle-cell disease in Curacao - abstract

All patients more than 14 years old with sickle-cell disease (SCD) discharged from hospital with a diagnosis of acute chest syndrome (ACS) from January, 1980 to December, 1981 were enrolled in this retrospective study. Data were collected from patients' records to investigate frequency, risk factors, effect of intravenous heparin and outcome in ACS. There were 109 adult SCD patients of whom 53 (49 per cent) were admitted for 81 episodes of ACS. Hb SS and Hb SC patients were affected equally often. Chest pain was the most frequent presenting symptom (64 per cent) and 91 per cent of patients had pulmonary abnormalities on physical examination, but only 45 per cent had radiological evidence of ACS at diagnosis. Forty-three per cent of sputum cultures grew pathogenic micro-organisms. The overall case-fatality rate was 6 per cent. All 5 non-survivors were female Hb SS patients. Intention-to-treat analysis revealed no benefit of intravenous heparin on outcome (mortality 5 per cent versus 11 per cent in non-heparin-treated patients, p = 0.3), hospital stay (p = 0.7) or duration of hypoxaemia (p = 0.4) (AU)