Some Clinical Presentations And The Use Of Conventional And Non-Conventional Haemato-Biochemical Parameters For The Characterisation Of Young Sickle Cell Disease Out-Patients by Kwabena Nsiah


Mr. K. NsiaBecause of the differences in the clinical expression of sickle cell disease (SCD), it is imperative that for each locality, the characteristics of the patients be studied, as a means of judging the health status of the group under study. In this study, several conventional and non-conventional haemato-biochemical parameters were measured and used to characterise some identified clinical states of out-patients attending the Sickle Cell Clinic of the Komfo Anokye Teaching Hospital. The study involved 330 SCD subjects, 164 males and 166 females, and showed that about 20% of the subjects reported to the clinic in steady state, while 80% reported with adverse clinical conditions. The top five adverse conditions were pain (40%), malaria (22%), fever (6%), respiratory tract infections (3.6%) and urinary tract infections (2%). The prevalence rates of acute chest syndrome, priapism, splenomegaly and leg ulcers in the two main genotypes, SS and SC, were respectively, 2.4%, 3.2%, 2.4% and 1.4%. The mean red cell count of 2.33 ×1012/L and haemoglobin level of 6.7 g/dl in these four conditions, compared to the mean RBC count of 3.1 ×1012/L and Hb of 8.2 g/dl of the overall population, showed the haemolytic tendency has been exacerbated in the four conditions. In the patients with Plasmodium falciparum parasites, only the mean reticulocyte count was significantly lower in the parasitaemics, compared to the non-parasitaemics. Furthermore, through a novel mathematical modeling, it has been shown that the frequency of distribution of reticulocytes was exponential in the parasitaemics but polynomial in the non-parasitaemics. The pattern supports the higher likelihood of severe anaemia in the parasitaemic. The study has also shown that compared to non-sickle cell disease controls, persons with SCD have significantly elevated aspartate aminotransferase (AST) activities. The overall mean AST and ALT activities were 48.24 and 26.48 U respectively, compared to levels of 23.0 U for non-sickle cell control, the difference being statistically significant, (p< 0.05) for AST  but not ALT. Thus, SCD patients, due to the haemolytic tendency, have AST to ALT ratio greater than 1. Therefore, it has been proposed from this study that in SCD, this ratio should be used as a haemolytic index, provided hepatic and cardiac integrity has not been compromised. Through simple and multiple regression analyses, it has been shown that the ratio has inverse association with haemoglobin, but direct association with reticulocytes and lactate dehydrogenase, known markers of haemolysis. The study has also shown a 3.6% incidence of seropositivity for HBsAg, but despite the presence of the hepatitis B infection, the hepatic and kidney functions had not been compromised. The use of spot urine microscopic and macroscopic analyses, have shown 36.1% of the subjects showed various markers of kidney dysfunction, and an urine colour number scale has also been proposed to offer a quantitative measure, for comparison with some clinical indices and outcomes. In conclusion, vaso-occlusive crisis has been shown to be the most prevalent clinical condition, similar to many other studies, but for the prevalence of 40% . Typical of a tropical environment, malaria fever is common, with a recorded prevalence of 22%. A novel mathematical modeling has been used to describe reticulocyte distribution in subjects with the parasitaes and those without the parasitaes. Another novel parameter, the AST:ALT ratio, has been proposed as a haemolytic marker for the characterisation of the various clinical presentations. Furthermore, the use of spot urine microscopic and macroscopic analyses, have shown 36.1% of the sampled population to show various markers suggestive of incipient or progressing kidney damage, which the attending clinicians need to monitor and manage appropriately, in order to avoid end stage renal disease.


Contact: Mr. Kwabena Nsiah


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