The plasma cortisol levels of 108 children aged 1–16 years with various Hb genotypes (AA, AS and SS) were measured. The mean plasma cortisol levels of both the AS and SS groups (during steady state) fell within the normal range but the mean of the SS group is significantly lower than the AA group. In fact, 9 of the sickle cell children had plasma cortisol levels below the normal range while no child in the HbAS and HbAA groups had plasma cortisol below the normal range. These results indicate that in sickle cell disease, there is low cortisol production while the secretion is increased during painful crisis.
The serum cobalamin, serum folate, erythrocyte folate concentrations and total leucocyte and neutrophil counts have been studied in sickle cell children (with HbSS) during the steady state. There was a significant reduction in serum cobalamin concentration with significant increases in both the total leucocyte count and neutrophil differential. A relationship was also observed between the serum cobalamin and erythrocyte folate. The lowest erythrocyte folate levels were observed in patients with the lowest serum cobalamin concentration. These abnormalities are discussed in relation to the possible role of cobalamin in the synthesis of erythrocyte folate polyglutamate and possible immunological role of cobalamin among these children. These results lend support to the view that daily cobalamin supplementation along with folate (which is being given) may prove beneficial to the sickle cell patients as it may lower the frequency and severity of infections.
The nephrotic syndrome in Nigerian children is known to be largely associated with the endemicity of quartan malaria. Routine thyroid function studies were carried out on 24 children with clinical and biochemical evidence of the nephrotic syndrome. The children, aged four to 14 years, were all in the active phase of their disease, presenting with facial and pedal oedema and ascites. There was severe hypoalbuninaemia [mean (S.E.); 19.2 (1.1) g/l], hypercholesterolaemia; 10.5 (1.0) mmol/l and severe albuminuria ranging from 1 to 10 g/l. There was no clinical evidence of thyroid disease. The results of thyroid function tests in these children were compared with those of 181 apparently healthy children of the same age range. The mean total serum thyroxine levels (S.E.) were 118.3 (2.6) and 50.0 (6.4) nmol/l in controls and patients, respectively; T3 resin uptake values were 29.8 (0.2)% and 33.1 (1.2)%; the free thyroxine index (FTI) was 34.7 (0.8) and 16.7 (1.9) while thyrotropin (TSH) levels were 4.8 (0.2) and 10.6 (1.0) mU/l (IRP. MRC 68/38), respectively. The findings of low levels of thyroxine and FTI in association with high levels of TSH suggest that a state of primary hypothyroidism exists in these nephrotic children.
An attempt was made to quantify sedation with lorazepam in terms of changes in plasma Cortisol and blood sugar and to compare the results with clinical observation. The results indicate that lorazepam caused satisfactory preoperative sedation when compared with the findings in the patients given placebo. In conformity with its adequate sedative effect, lorazepam caused decrease in both the plasma Cortisol and blood sugar in the samples obtained in the anaesthetic room.
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