Table 1. Comparative Diagnostic Data on... | American Society of Hematology

Table 1.

Comparative Diagnostic Data on Present Kindred With FP and Previously Mapped DHS Family

Condition	Hematology		Intracellular Electrolytes		Tracer Flux Rates
	Hb	Retic	[Na]_i	[K]_i	Pump	Cotransp	Leak
	(g/dL)	(%)	(mmol/L cells⁻¹)		(mmol/L cells⁻¹ h⁻¹)
FP (Edinburgh)	14 to 18	2 to 4	12	85	2.6	1.2	0.12
DHS (Irish family)	11 to 12	6 to 12	12 to 14	75 to 86	4.6 to 5.9	0.9 to 1.2	0.2
Normal	14 to 18	<2	5 to 11	88 to 105	0.8 to 2.0	0 to 1.2	0.075 to 0.090

Condition	Hematology		Intracellular Electrolytes		Tracer Flux Rates
	Hb	Retic	[Na]_i	[K]_i	Pump	Cotransp	Leak
	(g/dL)	(%)	(mmol/L cells⁻¹)		(mmol/L cells⁻¹ h⁻¹)
FP (Edinburgh)	14 to 18	2 to 4	12	85	2.6	1.2	0.12
DHS (Irish family)	11 to 12	6 to 12	12 to 14	75 to 86	4.6 to 5.9	0.9 to 1.2	0.2
Normal	14 to 18	<2	5 to 11	88 to 105	0.8 to 2.0	0 to 1.2	0.075 to 0.090

Intracellular Na and K were measured by flame photometry on fresh, washed RBCs. K influx was measured at 37°C using 86Rb as a tracer in MOPS with inhibitors at 0.1 mmol/L if required (see text). “Pump” denotes the ouabain-sensitive NaK pump fraction: “Cotransp,” the bumetanide-sensitive NaK2Cl cotransport; and “Leak,” the ouabain + bumetanide-resistant, residual passive leak fraction. Hematological values denote typical range on all 11 affected family members; isotopic flux results denote typical value on proposita.15

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