Acute splenic sequestration together with aplastic crisis caused by human parvovirus B19 in patients with sickle cell disease

Three children with homozygous sickle cell disease, 22 months and 8 and 10 years of age, had clinical and hematologic manifestations of aplastic and splenic sequestration crisis simultaneously. They had an acute drop in hemoglobin level (16, 20, and 45 gm/L), reticulocytopenia (0.1%, 0.6%, and 0.3%), and sudden splenic enlargement. Evidence of recent parvovirus infection was demonstrated.     

Reversal of liver failure in sickle cell vaso-occlusive crisis

Issues concerning patient advocacy and nursing advocacy are explored in relation to the nurse's role and the UKCC code of conduct. The author's personal experiences of being an advocate are described. Potential conflict between the nurse's role and doctor's role in relation to advocacy is identified. The best person to take on the role of patient advocate is discussed.     

Psychosocial complications and management of sickle cell disease

Five adolescent girls with Turner syndrome (mean age 13.9 years, mean bone age 12.0 years) were treated with both recombinant human growth hormone (rhGH) and oxandrolone for 2 years with an average increment in height of 13.4 cm. The mean bone age advanced by only 1.2 years, providing an increase in the mean estimated mature height of 9.2 cm. We conclude that rhGH and oxandrolone benefit older teenagers with Turner syndrome because of an increased growth rate with slow progression of skeletal maturation.     

Septic arthritis and Reiter's syndrome in sickle cell disorders: case reports and implications for management

Two patients with sickle cell disorders developed gram-negative septic arthritis and responded poorly to conventional managment. Unfavorable factors included (1) confusion with the nonseptic arthropathy of a sickle crisis; (2) impaired articular perfusion, perhaps related to local sickling; (3) conversion of an infecting organism to an L-form in the presence of cell-wall inhibitors; and (4) interference with cultures and antibacterial titrations of synovial fluid caused by intro-articular administration of antibiotics. Prolonged treatment with very high doses of antibiotics was required to eradicate infection in both patients. Because sickling and local hypoxia apparently interfere with defenses against infection, anemia should be corrected by transfusion. One patient had had Reiter's syndrome; the dysenteric form of this disease may be a cause of arthritis in some sicklemic patients.     

Acute splenic sequestration in hemoglobin sickle O-Arab disease

A white girl with sickle O-Arab disease (Hb S/O-Arab) had three separate episodes of acute splenic sequestration at 8, 12, and 15 months of age with hemoglobin levels falling to 3.9, 2.8, and 4.2 g/dl, respectively. Functional hyposplenism was suggested by the radionucleotide spleen scan. Following splenectomy her hemoglobin stabilized in the range of 0.7--8.5 g/dl. Life-threatening episodes of splenic sequestration, pneumococcal infections and osteomyelitis may occur in both Hb S/O-Arab and homozygous sickle cell diseases.     

Neurological complications after autologous stem cell transplantation

Clinically detectable well-differentiated metastatic thyroid cancer to the kidney is rare, with only 12 cases reported in the medical literature. The authors report a case of metastatic thyroid carcinoma to the kidney in a patient with widespread dissemination. She underwent total thyroidectomy, radical left nephrectomy, radioactive ablation with I-131, radiotherapy, and thyroid suppression therapy. Well-differentiated thyroid metastatic cancer can be amenable to treatment with successful long-term results.     

The role of CT and MR in imaging the complications of sickle cell disease

Sickle cell disease is the most common inherited haemoglobinopathy described. Complications of sickle cell disease (SCD) are due to chronic haemolysis of fragile red cells or secondary to vascular occlusion by sickled red cells with subsequent tissue infarction. Traditionally plain film radiography has been the mainstay in the assessment of patients with SCD, but increasingly magnetic resonance (MR) imaging and computed tomography (CT) are being used. In this review the imaging features of a range of complications of SCD are demonstrated with particular emphasis on CT and MR.     

Laparoscopic splenectomy after arterial embolisation

Laparoscopic splenectomy remains a challenging procedure, as haemorrhage causes the most complications. In order to reduce this risk, preoperative selective embolisation of the splicing artery has been performed in a series of six successful laparoscopic splenectomies in one male and five female patients with a mean age of 34.6 years (range 17-53 yrs). Indications for surgery were immune thrombocytopenic purpura (ITP)(n = 3), non-Hodgkin lymphoma with secondary haemolytic anaemia (n = 1), autoimmune haemolytic anaemia (n = 1) and congenital spherocytosis (n = 1). The mean splenic length was 12.3 cm (range 9-16 cm) and no accessory spleens were identified. Mean operative time was 96.7 min (range 90-150 min). There were no deaths nor haemorrhagic or septic complications. Recovery after surgery was excellent with a mean hospital stay of 5.2 days (range 2-10 days). We conclude that selective embolisation of the splenic artery, just prior to laparoscopic splenectomy adds to the safety, and operating time may be shortened.     

Septic complications after biliary tract stone surgery: a review and report of the European prospective study

We report a prospective, controlled study of the incidence of septic complications following biliary tract stone surgery. This study included a total of 280 patients operated on in eight hospitals in various European countries. In this study the computer program "Surgery" was used. Of 280 patients, 77 (27.5%) were male and 203 (72.5%) were female. The age ranged from 20 to 92 years (mean 54.8 years); 78.9% of the cases corresponded to clean-contaminated surgery; 85% of the patients received antibiotic prophylaxis with cefazolin. Twenty-one patients developed postoperative septic complications (7.5%) of which 12 (4.3%) were wound infections; five patients (1.8%) had intra-abdominal infections. The wound infection rate was 3.2% in clean-contaminated surgery, 7.7% in contaminated and 20% in dirty (p < 0.02). In laparoscopic cholecystectomy the global rate of septic complications was 3.6% vs. 12.6% in open cholecystectomy (p < 0.01); 2.4% and 6.3% wound infection respectively. The mean age of patients who developed postoperative septic complications was 61.5 years and 54.2 years old who did not develop any complications (p < 0.03). The duration of the postoperative period was 5 days in patients without infection and 13 days in patients with infection (p < 0.0001). Two patients died, one of them (0.4%) caused by sepsis. In addition to the European prospective study, a review of the problems of sepsis in biliary surgery was carried out.     

Septic shock from Mycobacterium tuberculosis after therapy for Pneumocystis carinii

Septic shock resulting from Mycobacterium tuberculosis (TB) occurs only rarely, even among patients infected with human immunodeficiency virus. We report a case of fulminant TB sepsis in the setting of human immunodeficiency virus infection. This case illustrates the hazards of corticosteroid use as a part of empirical treatment of Pneumocystis carinii pneumonia, as well as the unique appearance of TB on chest x-ray films.     

Septic complications of total parenteral nutrition. A five year experience

Elevated levels of serum albumin have been noted in patients on chronic methadone maintenance and in heroin addicts. This observation was investigated in rabbits maintained on daily methadone 4 mg per kg of body weight after a period of 3 months on increasing dosage to assure drug tolerance. Albumin distribution and metabolism were measured with tested lots of 125I rabbit albumin. Studies were made before and again after the attainment of the methadone maintenance state. Albumin distribution was altered markedly with a shift of intravascular albumin to extravascular sites. Associated with this change, the serum albumin level rose by an average of 0.5 g per 100 ml. Albumin degradation increased by 32% from 248 to 327 mg per kg per day. The total exchangeable albumin pool increased 35%, or 3.6 g. Since the exchangeable albumin pool increased in the face of an increment in albumin degradation, albumin synthesis must have increased even further to account for this change. Although the specific factors responsible for these alterations in albumin metabolism and distribution are not known at present, to date, this hyperalbuminemic hypercatabolic state is not produceable in any other clinical or experimental situation.     

Hematuria and sickle cell disorders

To examine the relationship between gross hematuria and sickle cell disorders, all patients admitted to Grady Memorial Hospital with the diagnosis of a sickle cell disorder during a 14-month period were reviewed. Of 115 such patients, 65% had sickle cell disease and 35% had sickle cell trait. None of the former but seven of the latter group had gross hematuria. Mean age of the seven was 30 years. Comprehensive examinations and laboratory studies showed that all were free of concomitant disease. Physical findings, diagnostic modalities, and treatment were reviewed. Findings suggest that conservative therapy (bed rest, hydration, and diuresis) is usually effective.