Search

Aim: Athletes are not immune to human immunodeficiency virus (HIV) and Glucose-6-Phosphate Dehydrogenase (G-6-PD) deficiency, and these conditions do not cause any harm or damage to their body as long as the necessary precautions in term of medications and others are adhered to. This research’s main objective was to determine the prevalence rate of G-6-PD deficiency and HIV among the University athletes in Rivers State, Nigeria. Study Design: Cross-sectional study. Place and Duration of Study: Sports Institute, University of Port Harcourt (UNIPORT), Nigeria, between June 2012 and July 2015. Methods: A total of 258 athletes were screened (134 females and 124 males) for HIV and G-6-PD deficiency. The athletes’ ages, gender and dates of birth were obtained and recorded. The presence of the HIV-1 and HIV-2 antibody was detected using the Uni-Gold™ Recombigen® HIV-1/2 for the detection of HIV. While Beutler Semi-quantitative G-6-PD Test Kit (BSA-3000) was utilized for the quantitative detection of G-6-PD deficiency in whole blood. Results: Of the 258 athletes tested, 0.7% was G-6-PD deficient while 1.2% was positive for HIV. The G-6-PD deficient positive cases were found in the age range of 21-25 years while the higher prevalence of HIV was observed in the age range 31-35 years (4.8%), followed by 26-30 years (1.9%) and the least was observed in the age group 21-25 years (0.7%). Age and gender had no significant relationship with the positivity of the athletes to HIV and G-6-PD (p-value >0.05). Conclusion: This study confirms the presence of HIV and G-6-PD deficiency among university athletes in Rivers State, Nigeria. This calls for routine testing of both the athletes and the general public for G-6-PD deficiency to prevent hemolysis causes by G-6-PD deficiency.

Background Therapeutic whole blood exchange (TWBE) has been used as an alternative when methylene blue (MB) fails in severe methaemoglobinemia. However, there are limited data on the efficacy and safety of TWBE. Objectives Our aim was to report our institutional experience with TWBE. We also perform a systematic review of published literature. Methods We retrospectively reviewed our respiratory intensive care unit database to identify cases of methaemoglobinemia managed with TWBE. A systematic review of the PubMed database was performed to identify similar cases (≥12 years). We report the indications, utility, and safety of therapeutic exchange in methaemoglobinemia. The procedural details were also noted. Results We identified five subjects who received TWBE for methaemoglobinemia (median methaemoglobin level 39%; range 19.6-42.4%). TWBE was successful in all five cases and no adverse events were encountered. Our review identified 27 additional subjects. The median methaemoglobin level was 37.5% (range 3.7-81%). The most common indication (n = 24, 75%) for therapeutic exchange was a lack of response to MB. A majority of the subjects (n = 26/32, 81.2%) survived. No procedure-related complications were reported. Conclusion TWBE is a safe and effective salvage modality for adults with methaemoglobinemia, when MB is either contraindicated or ineffective. Future studies should standardise therapeutic exchange in the management of methaemoglobinemia.

Este trabalho teve por objetivo estudar a deficiência de G-6-PD em uma comunidade do interior do Estado de São Paulo (Bragança Paulista). Durante 36 meses foram selecionados 4.621 doadores de sangue do sexo masculino, detectando-se 80 deficientes em G-6-PD. A análise molecular foi realizada em 70 deficientes não consangüíneos mediante a amplificação de DNA por PCR seguida de digestão por enzimas de restrição e análise de polimorfismo de conformação em hélice simples (SSCP). Em 98,6% dos casos, foi identificada a mutação G-6-PD A- (202 G->A), por digestão do exon 4 com Nla III. Verificou-se a presença de mutação mais rara no exon 9, por SSCP. Não foi constatado caso da variante Mediterrânea. Tais resultados mostraram que a variante A- (202 G->A), quase que exclusiva, foi introduzida na comunidade não apenas por descendentes de africanos, como também pelos imigrantes italianos, espanhóis e portugueses. A contribuição italiana em termos da variante Mediterrânea de G-6-PD foi menor do que a sua participação em termos de talassemia beta, provavelmente devido à origem no Norte da Itália.

Hematological effects of antiretroviral (ARV) drugs in HIV-infected patients with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are unclear. The aim of this study was to assess effects of highly active antiretroviral therapy (HAART) on hematological and plasma bilirubin changes in these patients. A hundred and nine HIV-infected Thai patients were tested for G-6-PD deficiency and its variant by using fluorescent spot test and polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) analysis, accordingly. Changes of hematological parameters and plasma bilirubin at baseline and 6 months of HAART were analyzed in G-6-PD deficiency patients. G-6- PD deficiency was found in 10 (9.17%) patients and G-6-PD Canton1376G>T was the most frequent. Of these, 3 patients had coinheritance of G-6-PD deficiency and thalassemia. Increased mean levels of lymphocyte counts, CD4+ T-cells, mean corpuscular hemoglobin (MCH) and hemoglobin from baseline to 6 months of HAART were observed. Whereas, mean levels of total bilirubin and direct bilirubin at baseline were not significantly different from those at 6 months of HAART. Therefore, HAART did not cause hemolytic anemia and hyperbilirubinemia in HIV-infected patients with G-6- PD deficiency. On the other hand, the effective use of HAART is associated with improvements in hemoglobin and MCH levels of these patients.

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is widely associated with oxidant induced haemolytic episodes, especially in people on malaria treatment. A cross sectional study on the prevalence of G6PD deficiency in was carried out on 200 blood samples from various private surgeries and three major Hospitals in Harare during October and November, 2001. The blood samples were screened for the prevalence of G6PD deficiency using the NAPD oxidoreductase method. One hundred and eight (54%) were males and 92 (46%) were females. A p-value of 0.1096 indicated no statistically significant difference between the two sexes. Twenty-three out of the 200 (12 %) were G-6-PD deficient while the remainder 177 (88%) were found to be normal. Stratifying the 23 optical density values of the deficient samples in this study showed that four (17%) had values equal to and more than (>) 0.400 indicating severe deficiency and 19 (83%) had values between 0.330 and less than 0.400 indicating mild deficiency. Optical density stratification may give a possible grading system in the deficiency rates. A well planned, large and more controlled research study is required to assess G6PD deficiency in Zimbabwe more effectively. Key words : Glucose-6-phosphate, oxidant, haemolysis, malaria, optical density

In a study of 92 cases of neonatal tetanus (NNT), it was found that 23 (25%) had associated neonatal jaundice (NNJ) but in none of them was NNJ severe enough to cause kernicterus. Glucose‐6‐Phosphate dehydrogenase (G‐6‐PD) deficiency was less common in babies with NNT than expected in general population (p 0.02). These babies with NNT were as exposed to icterogenic agents as jaundiced babies previously reported from the same institution. The lower incidence of G‐6‐PD deficiency in babies with NNT was probably due to the fact that G‐6‐PD deficient babies on exposure to icterogenic agents develop severe NNJ early and may die of kernicterus. Antibiotic therapy in jaundiced babies may prevent NNT in some of the G‐6‐PD deficient babies who are more prone to severe NNJ. More of the G‐6‐PD normal babies therefore remained at home till they develop NNT. It is speculated that steps taken to prevent NNJ in the G‐6‐PD deficient babies by avoiding application of potentially infected icterogenic agents (i.e. menthol c...

Introduction and Background: Sickle cell disease (SCD) is an extremely challenging disease of global concern. The highest prevalence of SCD in Saudi Arabia is in the Eastern province. Compared to all other areas of Saudi Arabia, Al-Qatif area has the highest gene frequencies for HbS and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency genes. Objective: The objective of this study was to assess the knowledge and misconceptions about SCD of adults (≥18 years) with sickle cell anemia in Al-Qatif area, Eastern Province, Saudi Arabia, and study factors that may affect them. Materials and Methods: A cross-sectional study using a self-administered questionnaire was distributed to 320 patients aged ≥18 years with sickle cell anemia, who attend the medical outpatient clinics in Qatif central hospital and primary care centers in the Qatif area, Eastern Province, Saudi Arabia. Result: A total of 300 participants completed their questionnaire. In general, 56.3% had poor knowledge of the disease. About 58.3% had good knowledge of the genetic transmission. The knowledge of 46.7% about the precipitating factors was poor. Moreover, 59.3% had poor knowledge of the diet of people with SCD and 81.3% had poor knowledge of the diet of people with G-6-PD deficiency. Conclusion: Our study revealed significant widespread misconceptions of patients with sickle cell anemia especially relating to their diet.

HbA1 was determined in 15 G-6-PD-deficient subjects during a hemolytic crisis with hemoglobinuria due to ingestion of fresh fava beans. The same G-6-PD-deficient subjects were studied again

40 full-term newborn infants with erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) deficiency were used for a study concerning the effectiveness of agar per os in preventing severe hyperbilirubinemia. 20 randomly selected neonates were given agar(l g/kg/day) orally in 4 daily doses from their 1st to their 5th day of life. 20 infants were not treated and served as controls. Three exchange transfusions were performed in the experimental as well as in the control group. According to these results, agar does not seem to be effective in preventing severe hyperbilirubinemia, which frequently occurs in newborn infants with erythrocyte G-6-PD deficiency.

The frequency of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in 50 Hb S homozygotes (SS) and 98 Hb S heterozygotes (AS) was determined and compared with the frequency obtained in individuals

Prophylactic treatment with barbituric acid has been made for preventing severe hyperbilirubinemia in G-6-PD deficient newborn infants. In 17 out of 46 untreated and in 7 out of 171 treated cases exchange transfusions were needed since the biliriibin serum level exceeded 18 mg/100 ml. These data show that this prevention can be useful in areas, such as Sardinia, where the incidence of G-6-PD deficiency is very high. It has been further demonstrated that this particular form of neonatal hyperbilirubinemia is not due to increased red cell destruction.

Salicylamide glucuronide formation has been studied in 23 newborn babies with erythrocyte G-6-PD deficiency and in 15 normal newborns on the first day of life. Glucuronide formation was significantly lower (p < 0.001) in the former in comparison with the controls. In the newborns with G-6-PD deficiency who subsequently became hyperbilirubinemic an even lower mean glucuronide formation was observed (p < 0.01) in respect to the non-jaundiced G-6-PD-deficient newborns.

Erythrocytes of both G‐6‐PD deficient humans and Dorset sheep, an animal model with an erythrocyte G‐6‐PD deficiency, both responded in a dose‐dependent manner to the oxidant stress of MLHP as meas...

Salicylamide-glucuronide formation has been determined in 27 children who underwent a favism crisis, 25 parents, and in 25 normal children who served as controls. A highly significant mean lower glucuronide formation was observed in the favism group in respect to the controls. The difference between fabic children and their parents was significant, and between parents and controls there was no significant difference.

Summary Fifty mature male newborns with erythrocyte G-6-PD deficiency were used for a study con cerning the effectiveness of orotic acid in preventing severe hyperbilirubinemia. Twentyfive randomly selected neonates were given orotic acid (100 mg/kg/day) orally in two daily doses from their 1st to their 5th day of life. Twenty-five newborns were not treated According to these results orotic acid does not seem to be effective in preventing severe hyperbilirubinemia, which frequently occurs in newborn babies' with erythrocyte G-6-PD deficiency. and served as controls. Six exchange transfusions were performed in the test group and six in the controls.

ERYTHROCYTE g l u c o s e 6 p h o s p h a t e d e h y d r o g e n a s e def ic iency is a well k n o w n cause of n e o n a t a l j aund ice , w h i c h usual ly first appears on the s e c o n d day of life or la ter a n d suggests tha t .the cond i t ions or agen t s respons ible for hemolys i s have b e e n e n c o u n t e r e d pos tpa r tum. Occasional ly , j aund i ce is p r e s e n t shor t ly after b i r t h and the re is an an t ena t a l his tory of m a t e r n a l inges t ion of potent ia l ly hemoly t i c agents . W e repor t here a case o f mat e rna l inges t ion of fava b e a n s a n d ascorbic acid associa ted with hydrops fetalis a n d death .

Phototherapy was employed in treatment of newborn infants with erythrocyte G-6-PD deficiency whose serum bilirubin concentrations exceeded 10 mg/100 ml on the second or third day of life. Exchange transfusions were required for two of the 12 treated babies and for six of the 12 control infants. It is noteworthy that exposure to light did not affect the erythrocyte GSH content nor did it increase hemolysis.

A major cause of neonatal morbidity and mortality in Lagos, Nigeria, is severe neonatal jaundice seen in G-6-PD deficient babies. The observation that the jaundice is more severe in outpatient than in inpatient babies suggests that its cause is exogenous. "Mentholated" powder which is commonly used in many clinics and at home to dress umbilical cords was suspected to be the offending agent. A controlled study of the effects of one of these powders was carried out on 60 consecutive G-6-PD deficient babies. In 30 of them the umbilical cords were dressed daily with the powder while the remaining half who were untreated served as controls. The treated babies developed statistically more significant jaundice than the controls. Inability of neonates to conjugate menthol in this power is probably responsible for the jaundice developed by these G-6-PD deficient babies. It is concluded that the use of menthol and/or camphor-containing commerical products on neonates be discontinued, especially in communities where the incidence of G-6-PD deficiency is high as the use of such products may be contributiing to the severity of neonatal jaundice.

SummaryFive neonates with moderate hyperbilirubinemia and G-6-PD deficiency underwent phototherapy for periods of 48 to 120 hr. No biochemical evidence of ribo-flavin deficiency resulted. This contrasts with evidence of riboflavin deficiency observed in 15 of 16 infants with moderate hyperbilirubinemia but no G-6-PD deficiency who were exposed to light for periods of 48 hr or longer. The elevated FAD levels of the erythrocyte of the G-6-PD-deficient infants may have protected them from the light-induced vitamin deficiency.

A 10 year old boy from an ethnically German family developed severe hemolysis after ingestion of fava beans. Extremely low activity of G-6-PD (1–4% of normal) was found in the patient's red cells. The enzyme deficiency was also observed in the red cells of two other members of this family.

SICKLE-cell trait, which occurs in 8 to 14 per cent of all American Negroes, has generally been regarded as a benign condition. Occasional reports of splenic, pulmonary, pituitary or cerebral infar...

IT has now generally been accepted that haemoglobin H, which is a tetramer of normal β-chains, is found only in α-thalassaemia, where the deficiency of α-chains has led to a surplus of β-chains. The problem, however, why in only a small portion of cases of α-thalassaemia can Hb H be detected, has not been solved until now1. Fessas2 reports that at birth all patients with α-thalassaemia have 5 per cent or more Hb Bart's, which is a γ-chain tetramer, but that these patients afterwards develop Hb H only rarely. In family studies of patients with Hb H disease often one parent shows a thalassaemic blood picture, but no Hb H.

A case report is presented of a 31-year-old single gravida 7 who had easily borne 6 children and requested abortion at 8 weeks. She complained of leg pains; and after symptomatic treatment was given for the leg pains she was referred to a psychiatrist and social workers with a diagnosis of myositis intrauterine pregnancy grand multiparity leimyomas of the uteri obesity and emotional disorder. After 4 weeks of administrative delays gravid hysterectomy was performed under endotracheal anesthesia. Postoperatively everything was normal until the third day at which time the abdomen was moderately distended and the patient complained of nausea and severe malaise. The temperature was just over 37.8 degrees centigrade and she was incontinent of urine. An intravenous infusion was given. Afebrile again on the fourth day the patient was much improved. Afebrile and with a soft abdomen on the sixth day after complaining of gas pains at 5 p.m. the patient screamed vomited complained of severe abdominal pain and said she was blind. She became dyspneic screamed again and then became comatose. Despite immediate treatment the patient was pronounced dead at about 6 p.m. Autopsy disclosed massive distention of the large and small bowel massive pulmonary aspiration of intestinal contents with partial digestion of the lung parenchyma and massive bacterial colonization of the lungs brain and abdominal viscera. There was no evidence of phlebitis or embolism. It was postulated that bowel wall compromised by ileus may have accounted for the extensive proliferation of intravascular bacteria. This case demonstrates the need for a heightened sense of suspicion in postoperative obstetric patients. Repeated abdominal films would have been revealing; and enemas yielding poor results might have been highly suggestive.

Excerpt Though the origins of erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are rooted in human antiquity, the presence of drug-induced hemolysis in individuals subsequently fel...

To the Editor:— I noted with considerable interest your editorial comment, "Hemolytic Anemia Complicating Viral Hepatitis and G-6-PD Deficiency" ( 208 :2468, 1969), and particularly your reference to two reports of acute renal shut-down in such patients. I wonder whether your warning of the dangers of renal complications in patients with this enzyme defect should not be extended to those with virus disorders other than hepatitis. I should like to report briefly a patient who, because of massive intravascular hemolysis, was considered to be in great danger of intravascular coagulation, with possible kidney damage, and who received heparin without waiting for evidences of coagulation. Report of a Case:— A 17-month-old boy of Greek-American extraction woke one morning appearing pale and listless, and with his wet diapers stained deep red. The patient had had a small-pox vaccination four days before, and had had an upper-respiratory tract infection for the prior two days.