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PubMed2001

[Operating Procedure of Collection, Processing and Preservation of 3000 Units Umbilical Cord Blood in Shangdong Cord Blood Bank]

The experience with the umbilical cord blood (UCB) stem cells for unrelated transplantation from our 3 000 UCB storage was described. UCB, collected from closed blood bags, were mixed with hydroxyethyl starch for nucleated cell (NC) enrichment. After finishing CD34 analysis, culture of hematopoietic progenitors (CFU-GM and CFU-GEMM) assays, microbial culture, HLA Class I (A, B) serology and class II (DR) low resolution SSP typing, cord blood units are stored in the liquid nitrogen for clinical applicatoin. Cord blood contained an average of nuclear cell (NC) (1.2 +/- 0.6) x 10(9), CD34(+) cells (3.0 +/- 3.7) x 10(6), CFU-GM (1.1 +/- 0.7) x 10(6) and CFU-GEMM (1.1 +/- 1.2) x 10(6) for storage and the recovery rates were 91%, 88%, 85% and 82%, respectively. The recovery rates for red blood cell and Hb were (39 +/- 9)% and (40 +/- 8)%, respectively. The storage volume was (35.1 +/- 7.1) ml in a 50 ml storage bags. The mean time from collection to processing of 15 hours (range 4 - 24 hours) had no influence on cell viability. The cell viability before processing is more than 95% and 92% after UCB thawing. The recovery rates of NC, CD34(+) cells and CFU-GM post-thawing were 96%, 90% and 91%, respectively. There were no HIV antibody (HIVAb) positive in all of UCB units. For an incidence of processed samples, infection with syphilis, HBsAg, HBcAb, HCVAb, CMV, bacterial contamination and abnormal hemoglobin were 0.1%, 0.8%, 3.2%, 0.2%, 87.1%, 1.2% and 0.1%, respectively. More than 3 HLA loci matched can be found for random patients in our cord blood bank and 6 HLA loci matched have 5%. For transplantation with nucleated cell counts of > 2.7 x 10(7) cells/kg, our cord blood bank will be able to provide all of the umbilical cord blood stem cell samples for children and 50% of units can be used for some of adult recipients transplantation in the country. It is concluded that: (1) The large cord blood banking for 20 000 UCB storage is feasible in China. (2) Our system of whole procedure and methods is functionable for supplying qualified cord blood units in transplantation. (3) The volume for collection is critical to the yield of CD34(+) cells or hematopoietic progenitor cells, however cord blood NC is also important and proportional with CD34(+) cells. Only the units containing more than 8 x 10(8) cells and more than 60 ml of cord blood can be in the procession for storage.

Journal Article↗
PubMedDate not supplied

Combined androgen blockade-induced anemia in prostate cancer patients without bone involvement.

BACKGROUND: To determine the onset and extent of combined androgen blockade (CAB)-induced anemia in prostate cancer patients without bone involvement. PATIENTS AND METHODS: Forty-two patients with biopsy-proven prostatic adenocarcinoma [26 with stage C (T3N0M0) and 16 with stage D1 (T3N1M0)] were included in this study. All patients received CAB [leuprolide acetate (LHRH-A) 3.75 mg, intramuscularly, every 28 days plus 250 mg flutamide, tid, per Os] and were evaluated for anemia by physical examination and laboratory tests at baseline and 4 subsequent intervals (1, 2, 3 and 6 months post-CAB). Hb, PSA and Testosterone measurements were recorded. Patients with stage D2-3 disease, abnormal hemoglobin level or renal and liver function tests that were higher than the upper limits were excluded from the study. The duration of the study was six months. RESULTS: The mean hemoglobin (Hb) levels were significantly declined in all patients from baseline of 14.2 g/dl to 14.0 g/dl, 13.5 g/dl, 13.2 g/dl and 12.7 g/dl at 1, 2, 3 and 6 months post-CAB, respectively. Severe and clinically evident anemia of Hb < 11 g/dl with clinical symptoms was detected in 6 patients (14.3%). This CAB-induced anemia was normochromic and normocytic. At six months post-CAB, patients with severe anemia had a Hb mean value of 10.2 +/- 0.1 g/dl (X +/- SE), whereas the other patients had mild anemia with Hb mean value of 13.2 +/- 0.17 (X +/- SE). The development of severe anemia at 6 months post-CAB was predictable by the reduction of Hb baseline value of more than 2.5 g/dl after 3 months of CAB (p = 0.01). The development of severe CAB-induced anemia in prostate cancer patients did not correlate with T baseline values (T < 3 ng/ml versus T > or = 3 ng/ml), with age (< 76 yrs versus > or = 76 yrs), and clinical stage (stage C versus stage D1). Severe and clinically evident anemia was easily corrected by subcutaneous injections (3 times/week for 1 month) of recombinant erythropoietin (rHuEPO-beta). CONCLUSION: Our data suggest that rHuEPO-beta correctable CAB-induced anemia occurs in 14.3% of prostate cancer patients after 6 months of therapy.

Adenocarcinoma↗
PubMed1992

Malaria in a rural area of eastern Thailand: baseline epidemiological studies at Bo Thong.

Malaria is still a serious health problem in Thailand. Present attempts at controlling the disease by drug treatment and other means remain unsatisfactory. Thus, development of vaccination against malaria is a major research goal of malaria immunology. The objective of this study was to acquire epidemiological base line data for subsequent vaccine trials. A cross-sectional descriptive survey was conducted among 253 local inhabitants during the beginning of the transmission season in July 1989 at Bo Thong District, Chonburi Province, Eastern Thailand where malaria transmission was likely to be moderately high. Following the cross-sectional survey weekly morbidity surveillance was started to detect new cases of malaria by using active and passive case detection at the district hospital, local health centers and at neighboring malaria clinics. Fifty-four percent of the population were male and forty-six percent female: nearly a half (48.3%) were under the age of 15 and 17% under the age of 5 years. Eighty percent of the adults were married. Seventy percent of the subjects interviewed gave a history of malarial illness in the past. Malaria, malnutrition, anemia abnormal hemoglobin diseases and parasitic infestations were the main health problems in the study area. The annual parasite incidence of malaria was 169.4/1,000 population and 77% of parasitemic individuals were asymptomatic, indicating the existence of a semi-immune condition among these subjects. Antibody level to crude parasite antigen increased with age. It is hoped that the information obtained from these field studies may be useful in malaria vaccine trials in the near future.

Adolescent↗
PubMed1992

[Aerobic capacity training in heterozygote sickle cell athletes].

Two sport groups, all training physical educational and sports teachers at the H.N.I.P.S.S. of Dakar have been tested by measure of maximum consumption of oxygen (VO2max) before and after a three month aerobic training. The first group contained 10 abnormal hemoglobin (AS) subjects. The second group 11 normal hemoglobin (AA) subjects. The results do not show any difference in the reaction of adaptation between the two groups.

Adult↗
PubMed1992

[Hereditary glucose-6-phosphate dehydrogenase deficiency in newborn infants].

Early diagnosis of the deficiency of glucose 6-phosphate dehydrogenase was made in examining 428 samples of funic blood from 230 boys and 198 girls. The normal level of the enzyme activity was established in red blood cells of the healthy newborn with regard to the national and sexual differences. The hereditary character of the deficiency of glucose 6-phosphate dehydrogenase was supported in 37 neonates by analyzing the pedigrees. The enzyme deficiency was associated with different forms of hemoglobinopathies: alpha- and beta-thalassemia, structurally abnormal hemoglobin S and methemoglobinemia. The considerable prevalence of the deficiency of glucose 6-phosphate dehydrogenase was revealed in Azerbaijan for the first time. The phenotypic frequency amounted to 8.64% whereas the gene one to 0.0623.

Age Factors↗
PubMed2004

Hemoglobinopathy and pattern of musculoskeletal infection in children.

BACKGROUND: The bacterial pathogens associated with osteomyelitis in patients with hemoglobinopathy (HbSS) are controversial. There is insufficient data on the common musculoskeletal (MSS) infections in patients with hemoglobinopathies and the associated bacterial pathogens in Nigeria. OBJECTIVES: To identify the common MSS infections in children and the associated bacterial pathogens, especially in those with abnormal hemoglobin genotypes. METHODS: A retrospective case review was done of pediatric and adolescent patients admitted with bone and soft tissue infections over a seven-year period in a Nigerian teaching hospital. RESULTS: One-hundred-nineteen patients were studied, with mean age 7.9+/-5.6 years and male-female ratio of 1.3:1. Out of 78 patients with known genotype, 62.8% had HbAA, 20.5% HbSS, 9% HbAS, and 7.7% HbAC. The most common MSS infections were osteomyelitis (64.7%), pyomyositis (17.6%), and septic arthritis (10.1%). Others were cellulitis (5.9%), tuberculous arthritis (0.8%), and necrotizing fasciitis (0.8%). Staphylococcus aureus (S. aureus) was isolated from 62% of chronic osteomyelitis (COM) and 58% of pyomyositis, while gram-negative bacilli accounted for 34% and 42%, respectively. S. aureus was isolated from 63% of patients with HbAA, 40% with HbSS, 58% with HbAS, and 58% with HbAC, while gram-negative bacilli accounted for 33%, 60%, 33%, and 33%, respectively. Osteomyelitis and pyomyositis appear to be similarly common in patients with and without HbSS (RR 1.29; 95% CI 0.47-3.50). Osteomyelitis and soft tissue infections in general also appear to be equally common in them (RR 1.16; 95% CI 0.39-4.11). However, osteomyelitis appears to be more common among patients with HbSS than those with HbAA (RR 2.29; 95% CI 0.58-8.99) and those with other hemoglobinopathies (RR 2.23; 95% CI 0.66-7.49) CONCLUSIONS: Osteomyelitis, pyomyositis and septic arthritis are common MSS infections in hospitalized pediatric and adolescent patients, while tuberculous arthritis is uncommon. The most common bacterial pathogen in these infections is S. aureus-even in patients with HbSS and other hemoglobinopathies. Gram-negative bacilli are important causes of MSS infections in hospitalized children with HbSS. HbSS may be an independent risk factor for osteomyelitis in these patients.

Adolescent↗
PubMed2005

Sickle cell anemia patient with sarcoidosis-associated inguinal lymph node and lung infiltration.

Sarcoidosis is a chronic, systemic inflammatory disease of unknown etiology, characterized by noncaseating granulomatous infiltration of any organ. Sickle cell anemia (SCA) is the homozygoid form of sickle cell disease (SCD), which includes a group of genetic disorders characterized by production of an abnormal hemoglobin S (HbS). There are a few case reports with coexistence of sarcoidosis and SCA. We reported a 47-year-old female with SCA and sarcoidosis.

Anemia, Sickle Cell↗
PubMed2006

Co-existince of sickle cell disease and hemidiaphragm paralysis.

Sickle cell anemia is a disease caused by production of abnormal hemoglobin. Infection, acute splenic sequestration crisis, aplastic crises, acute chest syndrome, stroke, cholelithiasis, renal disease and pain are the major complications. Unilateral or bilateral diaphragm paralysis maybe seen following phrenic nerve injury and with a variety of motor-neuron diseases, myelopathies, neuropathies, and myopathies. Prominent right hemi-diaphragma elevation was observed on chest radiograph of a 14 years' old female patient with sickle cell disease. Her medical history yielded neither trauma nor intra-thoracic surgery. She didn't have either motor deficit or sensation disorder on any region of her body. Thorax CT yielded no lesion except the significantly elevated right diaphragm. Her cranial CT showed no lesion, too. Diagnosis of right hemidiaphragm paralysis was confirmed by positive Hitzenberg Sniff test on fluoroscopy. Although several pathophysiologic mechanisms are known to be involved and lead to central neurologic complications in sickle cell disease, involvement of peripheric nerves have not been reported. Here we present a 14 years' old female patient with sickle cell anemia and unilateral diaphragm paralysis, co-existence of which have not been reported so far.

Adolescent↗
PubMed1991

Usefulness of the pulse oximeter as a respiratory monitor during intravenous sedation.

During intravenous sedation, the pulse oximeter was applied as a respiratory monitor to 17 patients (Physical status classification of the American Society of Anesthesiologists, Class I-II) who were to have oral surgical operations, and the changes in arterial saturation of oxygen (SaO2) was observed consecutively. SaO2 dropped after administration of sedative agents in each case; there was some individual variation. Respiratory depression during sedation was evaluated from arterial blood gas analysis. The reduction in SaO2 caused by surgical manipulations was also noted. Furthermore, arterial partial pressures of oxygen (PaO2) and saturation calculated from PaO2 (SAT) measured from blood gas analysis were compared with SaO2 measured by the pulse oximeter. Both PaO2 and SAT correlated well with SaO2. The differences between SAT and SaO2 exceeded the range of error (+/- 2%) in many cases after the administration of sedative agents. These results suggest that the pulse oximeter is useful as a respiratory monitor during oral surgery. However, the pulse oximeter gives incorrect SaO2 readings in the presence of abnormal hemoglobin due to medication with nitrites or smoking habit.

Anesthesia, Dental↗
PubMed1991

Beta-thalassemia major and sickle cell disease.

Beta-thalassemia major and sickle cell disease are genetic disorders of red blood cells, caused by abnormal hemoglobin. These hemoglobinopathies affect males and females equally. Both are chronic disorders requiring lifelong treatment for affected individuals and education and support for them and their families. Special nursing considerations for the care of women facing the unique challenges of these disorders will be discussed.

Anemia, Sickle Cell↗
PubMed1989

[Chronic myelomonocytic leukemia (CMMoL) with systemic lymph node swelling and paroxysmal nocturnal hemoglobinuria (PNH)-like complication].

A 37-year-old male was diagnosed as having chronic myelomonocytic leukemia (CMMoL) with chief complaint of systemic lymph node swelling. On admission, his peripheral blood revealed mild anemia and mild thrombocytopenia with giant platelets, and monocytosis (1480/microliters). NAP score was low. Serum lysozyme increased. The bone marrow showed normal cellularity consisting of 4% myeloblasts and 14.4% promyelocytes, and a few myeloid cells were positive for double staining by alpha-naphthyl butyrate and naphthol ASD chloroacetate esterase. Biopsied specimens of the cervical lymph node showed infiltration of monocytoid cells, which were positive for lysozyme staining, into interfollicular tissue. As for chromosome variation, 21 large satellite was observed in all dividing cells from his bone marrow and peripheral blood. Furthermore, hemolytic anemia with hemoglobinuria developed during his course. Sugar water test was positive, but Ham test negative. Coombs test and Donath-Landsteiner reaction were negative. Abnormal hemoglobin, spherocyte and fragmentation were not found. Hemolysis disappeared about two months later. However, blastic crisis appeared and he died. We showed a case of CMMoL with 21 large satellite and paroxysmal nocturnal hemoglobinuria (PNH)-like complication. Satellite have usually been reported as asymptomatic, and thus this chromosome variant and CMMoL may have been coincidentally observed.

Adult↗
PubMed1989

Erythromelalgia and myeloproliferative disorders.

Erythromelalgia (erythermalgia) is characterized by attacks of severe burning pain, erythema, and warmth of the extremities, primarily the feet and, to a lesser extent, the hands. The distress is provoked by environmental heat, exercise, and dependency; it is relieved by exposure to cold and elevation of the extremity. Primary and secondary forms of erythromelalgia exist. Secondary erythromelalgia has been linked to a wide variety of diseases, the most common of which are certain myeloproliferative disorders: polycythemia vera and essential thrombocythemia. We describe, for the first time, a patient in whom chronic myelogenous leukemia was associated with the development of erythromelalgia, review the 60 cases in the world literature of erythromelalgia in patients with myeloproliferative syndromes, and compare the primary and secondary forms of the disease. Importantly, symptoms of erythromelalgia preceded the onset of a myeloproliferative disease by a median of 2 1/2 years. Therefore, all patients with erythromelalgia should be monitored with periodic blood cell counts. An abnormal hemoglobin level, white blood cell or platelet count, or immature cells in the differential count are not seen in idiopathic erythromelalgia and should alert the physician to the possibility of a more serious underlying disease process. Treatment of the myeloproliferative syndrome will sometimes alleviate the symptoms of erythromelalgia. Alternatively, a single daily dose of aspirin results in dramatic improvement in most patients with either primary or secondary erythromelalgia.

Erythromelalgia↗
PubMedDate not supplied

[Evaluation of the incidence of glucose-6-phosphate dehydrogenase deficiency in children with sickle cell anemia in Brazzaville (Congo)].

Following a survey on prevalence of sickle-cell anemia in Brazzaville's Region, the authors surveyed, in the same population, the incidence of Glucose-6-Phosphate dehydrogenase deficiency (G6PD) and the sharing of enzyme deficiency among sickle cell anemia patients (AA). This survey was carried out among children from 0 to 3 years of age, thanks to a very close collaboration between the Laboratory of bio-chemistry of the Faculty of Sciences, Maternity Hospitals of Blanche-Gomez, Makelekele, and Maternity Ward of the Brazzaville General Hospital. 214 children were examined in these Health Units. Blood samples were analysed by the thin layer isoelectric focusing technics. 128 children were safe from hemoglobin abnormalities, 44 had heterozygous and 42 homozygous sickle cell anemia, i.e. 19.6 p.c. of the total population surveyed. Measurement of the G6PD activity in these children revealed that 25.8 p.c. of the AA had an incomplete deficiency in G6PD, 31.8 p.c. of the AS and 45.2 p.c. of the SS were G6PD deficient (table I). These results, although fragmentary, demonstrate that G6PD deficiency does exist in the Congolese population, and that this enzyme deficiency might be more frequent in the carriers of sickle cell trait, evaluated by the authors at 22.4 p.c. of the population.

Anemia, Sickle Cell↗
PubMed1988

The University of Florida sickle cell screening program for neonates: design and results.

During the first 18 months of a pilot program for sickle cell screening at the University of Florida College of Medicine, Gainesville, 2,058 black neonates were screened. An incidence of homozygous sickle disease of 0.5 percent was greater than that expected or predicted by carrier frequency (8.3 percent). Fifty percent of all infants with abnormal cord blood electrophoreses were retested. All infants with actual homozygous disease or other clinically significant variants had confirmation of their diagnosis and were channeled for appropriate care. A change of phenotypic diagnosis based on a follow-up sample was made in eight cases. Errors were either interpretational or through contamination of cord blood samples by maternal blood at the time of delivery. Although location of infants for retesting after discharge was made more difficult by the largely rural composition of the target population, certain measures were taken to improve patient retrieval: use of public health personnel; enlistment of the aid of private physicians in the community; and inclusion of information regarding the screening program in the hospital discharge packets of black mothers. It is concluded that screening programs serving rural populations can adequately identify infants with abnormal hemoglobin patterns while educating and caring for families of these infants in a cost-efficient and effective manner.

Anemia, Sickle Cell↗
PubMed1986

Hémoglobinopathies in France.

A first approximation of the incidences of abnormal hemoglobin traits in France is given. The data include all the large ethnic minorities living in France, and also take into account the second and third generations. We estimate 1.3 X 10(5) and 1.8 X 10(5) individuals with HbS trait and beta-thalassemia trait respectively.

Anemia, Sickle Cell↗