Table 1.

Summary of cases. Plasma results of sodium, potassium, bicarbonate and pH are from the day of presentation, unless indicated. Initial urinary electrolytes, renin and aldosterone concentrations are from the initial admission

	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6	Case 7
Age at presentation (days)	25	28	8	23	10	5	10
Symptoms	Vomiting and weight loss	Weight loss and poor feeding	Weight loss	Vomiting and weight loss	Inpatient	Jaundice and weight loss	Weight loss and drowsiness
Plasma Na (mmol/l) (RR 135–145)	119	126	118	118	109	142 (112 on D10)	126
Plasma K (mmol/l) (RR 3.5–5)	NA	6.8	8.2	7.3	6.8	6.1 (11.3 on D10)	10
Plasma HCO3− (mmol/l) (RR 17–25)	19.4	17.9	19.6	NA	18.6	19.3 (D10)	10.4
pH	7.39	7.27	NA	NA	7.27	7.38	7.21
Creatinine (μmol/l) (RR 37–89)	67	61	29	48	96	74 (87 on D10)	49 (D11)
Urine K (mmol/l)	37	20	NA	19 (24)	10	22	7 (D15)
Urine Na (mmol/l)	50	16	15	20 (D24)	35	111	142 (D15)
Serum aldosterone (pmol/l) (RR 400–3000)	35 700	39 900	83 390	48 000	49 500	57 000	16 580
PRA (nmol/l per h) (RR 1.9–29)	41.3	250	>250	16	>250	>250	131.5
Duration of follow-up (months)	62	38	25	14	36	39	42
Weight centile at last clinic appointment	91st–98th	9th–25th	25th	50th	91st–98th	9th	75th
Ongoing management	Initial Na supplementation 2.5 mmol/kg per day	Initial Na supplementation 1.5 mmol/kg per day	Initial Na supplementation 2 mmol/kg per day	Discharged from follow-up. Not on medication	Initial Na supplementation 4 mmol/kg per day	Initial Na supplementation 3.8 mmol/kg per day*	Na supplements 8.5 mmol/kg per day*
	Now on 0.7 mmol/kg per day	Now on Na 0.7 mmol/kg per day			Now on Na 0.8 mmol/kg per day	NaHCO3 1.6 mmol/kg per day*	NaHCO3 2 mmol/kg per day*
						Indomethacin 1.5 mg/kg b.d.	Flucloxacillin 125 mg b.d.
						Gaviscon	Penicillin V 125 mg b.d.
						Ranitidine	Omeprazole 0.6 mg/kg b.d.
						Low potassium feeds	Domperidone 0.35 mg/kg b.d.
						Intermittent requirement for i.v. 0.9% NaCl	Montelukast 4 mg o.d.
							Beclometasone 200 μg b.d.
							Sodium cromoglycate 5 mg q.d.s.
Low potassium feeds
PRA (nmol/l per h) after long-term management	6.1	24.9	NA	7.4	1.1	0.3	Not done
Aldosterone (pmol/l) after long-term management	1230	5450	23.3	3400	385	<55	Not done
Renal and bladder USS	Normal	Normal	Normal	Normal	Normal	Normal	Normal

RR, reference range (age appropriate); PRA, plasma renin activity; NA, not available; o.d., once daily; b.d., twice daily; q.d.s., four times daily; USS, ultrasound scan.

^*Doses subject to variation.