TABLE 2.

Literature Review of Patient Cases Describing Individuals Who Were Diagnosed as Having Pancreatitis Before CF and Their Respective Manifestations

Reference	Cases: Age/Sex/Ethnicity	Age at Pancreatitis Diagnosis, y	Pancreatic Function at Pancreatitis Diagnosis		Age at CF Diagnosis, y	Time From Pancreatitis to CF Diagnosis, y	Highest SCT Result	Other CF Manifestations	CFTR Mutation, If Known
Reference	Cases: Age/Sex/Ethnicity	Age at Pancreatitis Diagnosis, y	Exocrine	Endocrine	Age at CF Diagnosis, y	Time From Pancreatitis to CF Diagnosis, y	Highest SCT Result	Other CF Manifestations	CFTR Mutation, If Known
Conklin et al¹²	4/F/White	4	N/A	N/A	6	2	WNL	Abnormal nasal potential difference	delF508; L997F
Koyano et al¹³	14/F/Asian	6	N/A	N/A	14	8	23.2 mEq/L	Low pancreatic bicarbonate	D924N, 5T
Tomaiuolo et al¹⁴	9/M/White	9	PS	N/A	11	2	78 mmol/L	None	delF508, G91G
Montagnani et al¹⁵	17/M/Wliite	13	PS	N/A	17	4	84 mEq/L	None	HomozygousIVS8 5T-12TG
Volkan et al¹⁶	14/M/White	14	N/A	N/A	14	0	110 mmol/L	Recurrent cough	D110H; 2789 + 5G > A
Conway et al¹⁷	23/M/White	17	PS	WNL	23	6	25 mmol/L	Infertility, bilateral absence of vas deferens	Homozygous, del1123Glu; intron 8 homozygous 7T
Dray et al¹⁸	17/F/N/A	1: 17	N/A	N/A	1: 17	0	1:11 mmol/L	1: Asthma	1: R31C; IVS8–5T-12TG
	22/F/N/A	2: 22			2: 22		2: 81 mmol/L	2: nasal polyps, bronchitis, salivary litliiasis	2: delF508;IVS8–5 T-12TG
Sinha et al¹⁹	29/M/Asian	18	PI	WNL	29	11	N/A	Underweight	c.3125A > G
Masaryk and Aclikar²⁰	20/M/White	1: 20	N/A	N/A	1: 20	1: 0	1: 143 mEq/L	1: Sibling with CF	N/A
	22/M/White	2: 22			2: not reported	2: not reported	2: 85.4 mEq/L	2: Azoospermia
Fierbinteanu-Braticevici et al²¹	21/F/White	21	PS	WNL	21	0	78 mmol/L	Bronchiectasis	2183AA > G
Gross et al²²	21/M/Asian	21	PI	N/A	26	5	92 mEq/L	Azoospermia: bronchitis	N/A
Vanderbruggen et al²³	38/M/White	23	PI, postpancreatectomy	Required insulin postpancreatectomy	38	15	86 mEq/L	Asthma, azoospermia	delF508; R117H
Kopp et al²⁴	30/F/White	25	PI	N/A	30	5	106 mmol/L	Recurrent sinusitis, GERD, cholelithiasis	CFTR: G551D; duplication of exon 19 and 7T/7T SPINK1: IVS3 + 184T > A
Brunson et al²⁵	45/F/White	45	N/A	WNL	45	0	N/A	Recurrent pneumonia; nasal polyps	Compound heterozygote: delF508, D1152H
Villalona et al²⁶	39/M/White	N/A	N/A	N/A	1: 32	N/A	1:47 mEq/L	1: Congenital bilateral absence of vas deferens	1, 2, 3 (siblings): novel mutation, R248G, in trans with N1303K
	32/F/White				2: 25		2: 40 mEq/L	2: Infertility
	29/F/White				3: 22		3: 42 mEq/L	3: Pneumonia

F indicates female; GERD, gastroesophageal reflux disease; M, male; N/A, not available; PI, pancreatic insufficient; PS, pancreatic sufficient; WNL, within normal limits.