Pitted red cell counts in Nigerian children with sickle cell anemia: correlation with age and splenic size.

Using direct interference phase-contrast microscopy (Normansky Optics), pit counts were performed on 32 HbSS patients, aged 3 to 17 years. The influence of age and splenic size on counts were also investigated. Nine HbSS and 15 HbAA age and sex-matched, healthy individuals served as controls. The mean +/- SD counts in the three groups were 11.1 +/- 9.1%, 1.7 +/- 1.4% and 1.8 +/- 1.7%, respectively. The older SS patients tended to have higher values, but the linear correlation with age was not impressive (r = 0.28). Seventeen (53.1%) patients had counts greater than 10%, while 8 (25%) had less than 3.5%. Five patients with gross splenomegaly had a mean count of 4.3 +/- 1.9%, significantly lower than the figure of 12.3 +/- 7.9% for the patients without splenomegaly (P less than .001), demonstrating retained reticulo-endothelial function in such patients.

Pitted red cell counts in Nigerian children with sickle cell anaemia: correlation with age and splenic size.

Pit counts, using direct interference phase-contrast microscopy (Normansky Optics) were carried out on 32 HbSS patients, aged 3-17 years. The influence of age and splenic size on the counts was also investigated. Nine (9) HbAS and HbAA age and sex-matched, healthy individuals served as controls. The mean +/- SD counts in the 3 groups were 11.1 +/- 9.1%, 1.7 +/- 1.4% and 1.8 +/- 1.7% respectively. The older SS patients tended to have higher values, but the linear correlation with age was not impressive (r = 0.28). Seventeen (53.1%) of the patients had counts in excess of 10%, while 8 (25%) had less than 3.5%. Five patients who had gross splenomegaly had a mean count of 4.3 +/- 1.9%, which was significantly lower than the figure of 12.3 +/- 7.9% for the patients without splenomeglay (p less than 0.001), thus demonstrating retained reticuloendothelial function in such patients.

Chronic hepatomegaly in steady state haemoglobin S children: some clinical and abdominal duplex ultrasonographic observations.

Chronic hepatomegaly (CH) is an index of severe disease in children with sickle cell anaemia (HbS). We studied 14HbS children with CH and two groups of controls made up of age and sex-matched HbA and HbS children without CH. The objective was to determine and compare hepatic perfusions in the children and also determine which clinical and ultrasonographic features are associated with CH. Hepatic perfusion was assessed using abdominal duplex ultrasonography which made possible the measurement of portal vein diameter (PVD) and the velocity of portal blood flow. The mean weights, blood pressure profiles and ultrasonographic splenic span and common bile duct diameters (BDD) did not differ significantly among the three groups. Similarly, the man PVDs of the three groups were not significantly different despite the large livers of the CH group. The CH group however showed linear growth faltering, a significantly lower mean haematocrit, faster pulse rate but paradoxically reduced hepatic portal blood flow velocity than HbA controls. Because of the disproportion of PVD relative to liver size in HbS patients with CH and the reduced portal blood flow velocity, hepatic perfusion is probably poorer in these patients. HbS children with CH therefore need careful management of hepatic and anaemic crises to prevent severe hypoxic liver damage.