{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:49:37Z","timestamp":1790833777400,"version":"4.1.0"},"reference-count":30,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[1991,3,1]],"date-time":"1991-03-01T00:00:00Z","timestamp":667785600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Digest Dis Sci"],"published-print":{"date-parts":[[1991,3]]},"DOI":"10.1007\/bf01318210","type":"journal-article","created":{"date-parts":[[2005,3,26]],"date-time":"2005-03-26T00:57:40Z","timestamp":1111798660000},"page":"365-369","source":"Crossref","is-referenced-by-count":14,"title":["Johanson-Blizzard syndrome"],"prefix":"10.1007","volume":"36","author":[{"given":"Dan R.","family":"Trellis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ray E.","family":"Clouse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","reference":[{"key":"CR1","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1016\/S0022-3476(71)80194-4","volume":"79","author":"AJ Johanson","year":"1971","unstructured":"Johanson AJ, Blizzard RM: A syndrome of congenital aplasia of the alae nasi, deafness, hypothyroidism, dwarfism, absent permanent teeth and malabsorption. J Pediatr 79:982?987, 1971","journal-title":"J Pediatr"},{"key":"CR2","first-page":"203","volume":"114","author":"MD Morris","year":"1967","unstructured":"Morris MD, Fisher DA: Trypsinogen deficiency disease. Am J Dis Child 114:203?208, 1967","journal-title":"Am J Dis Child"},{"key":"CR3","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/S0022-3476(69)80392-6","volume":"75","author":"PL Townes","year":"1969","unstructured":"Townes PL: Proteolytic and lypolytic deficiency of the exocrine pancreas. J Pediatr 75:221?228, 1969","journal-title":"J Pediatr"},{"key":"CR4","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1001\/archpedi.1981.02130270040014","volume":"135","author":"PL Townes","year":"1981","unstructured":"Townes PL, White MR: Identity of two syndromes. Proteolytic, lipolytic, and amylolytic deficiency of the exocrine pancreas and congenital anomalies. Am J Dis Child 135:248?250, 1981","journal-title":"Am J Dis Child"},{"key":"CR5","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1002\/ajmg.1320030203","volume":"3","author":"DL Daentl","year":"1979","unstructured":"Daentl DL, Frias JL, Gilbert EF, Opitz JM: The Johanson-Blizzard syndrome. Case report and autopsy findings. Am J Med Genet 3:129?135, 1979","journal-title":"Am J Med Genet"},{"key":"CR6","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1002\/ajmg.1320260120","volume":"26","author":"JB Moeschler","year":"1987","unstructured":"Moeschler JB, Polak MJ, Jenkins JJ, Amato RSS: The Johanson-Blizzard syndrome: A second report of full autopsy findings. Am J Med Genet 26:133?138, 1987","journal-title":"Am J Med Genet"},{"key":"CR7","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1001\/archotol.1979.00790160056015","volume":"105","author":"MD Sismansis","year":"1979","unstructured":"Sismansis MD, Polisar IA, Ruffy ML, Lambert JC: Rare congenital syndrome associated with profound hearing loss. Arch Otolaryngol 105:222?224, 1979","journal-title":"Arch Otolaryngol"},{"key":"CR8","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1002\/ajmg.1320220107","volume":"22","author":"JB Moeschler","year":"1985","unstructured":"Moeschler JB, Lubinsky MS: Brief clinical report: Johanson-Blizzard syndrome with normal intelligence. Am J Med Genet 22:69?73, 1985","journal-title":"Am J Med Genet"},{"key":"CR9","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1016\/S0022-3476(76)80806-2","volume":"89","author":"A Schussheim","year":"1976","unstructured":"Schussheim A, Choi SJ, Silverberg M: Exocrine pancreatic insufficiency with congenital anomalies. J Pediatr 89:782?784, 1976","journal-title":"J Pediatr"},{"issue":"6B","key":"CR10","first-page":"275","volume":"14","author":"DW Day","year":"1978","unstructured":"Day DW, Israel JN: Johanson-Blizzard syndrome. Birth Defects 14(6B):275?287, 1978","journal-title":"Birth Defects"},{"issue":"2","key":"CR11","first-page":"95","volume":"8","author":"PL Townes","year":"1972","unstructured":"Townes PL: Trypsinogen deficiency and other proteolytic deficiency diseases. Birth Defects 8(2):95?101, 1972","journal-title":"Birth Defects"},{"key":"CR12","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1136\/jmg.19.4.302","volume":"19","author":"M Baraitser","year":"1982","unstructured":"Baraitser, M, Hodgson SV: The Johanson-Blizzard syndrome. J Med Genet 19:302?303, 1982","journal-title":"J Med Genet"},{"key":"CR13","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1111\/j.1399-0004.1978.tb02141.x","volume":"14","author":"MK Mardini","year":"1978","unstructured":"Mardini MK, Ghandown I, Sakati NA, Nyhan WL: Johanson-Blizzard syndrome in a large inbred kindred with three involved members. Clin Genet 14:247?250, 1978","journal-title":"Clin Genet"},{"key":"CR14","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1111\/j.1399-0004.1986.tb00592.x","volume":"30","author":"K Zerres","year":"1986","unstructured":"Zerres K, Holtgrave EA: The Johanson-Blizzard syndrome: report of a new case with special reference to the dentition and review of the literature. Clin Genet 30:177?183, 1986","journal-title":"Clin Genet"},{"key":"CR15","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1016\/S0022-3476(81)80026-1","volume":"99","author":"I Helin","year":"1981","unstructured":"Helin I, Jodal U: A syndrome of congenital hypoplasia of the alae nasi, situs inversus, and severe hypoproteinemia in two siblings. J Pediatr 99:932?934, 1981","journal-title":"J Pediatr"},{"key":"CR16","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/S0022-3476(63)80304-2","volume":"63","author":"RJ Gorlin","year":"1963","unstructured":"Gorlin RJ, Meskin LH, St Geme JW: Oculodentodigital dysplasia. J Pediatr 63:69?75, 1963","journal-title":"J Pediatr"},{"key":"CR17","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1093\/oxfordjournals.jhered.a105415","volume":"36","author":"FH Adams","year":"1945","unstructured":"Adams FH, Oliver CP: Hereditary deformities in man due to arrested development. J Hered 36:3?7, 1945","journal-title":"J Hered"},{"key":"CR18","first-page":"21","volume":"37","author":"JL Bresson","year":"1980","unstructured":"Bresson JL, Schmitz J, Saudubray JM, Lesec G, Hummel JA, Rey J: Le syndrome de Johanson-Blizzard. Une autre cause de lipomatose pancreatique. Arch Fr Pediatr 37:21?24, 1980","journal-title":"Arch Fr Pediatr"},{"key":"CR19","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1136\/jmg.26.1.45","volume":"26","author":"JA Hurst","year":"1989","unstructured":"Hurst JA, Baraitser M: Johanson-Blizzard syndrome. J Med Genet 26:45?48, 1989","journal-title":"J Med Genet"},{"key":"CR20","unstructured":"National Institute of Arthritis, Metabolism, and Digestive Diseases: Cystic Fibrosis: A Disease in Search of Ideas. Clinical Sciences, Vol 2. Bethesda, Maryland, National Institutes of Health, DHEW publication No. NIHD 79-1643, 1979, pp 33?34"},{"key":"CR21","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1097\/00005792-198611000-00004","volume":"65","author":"HM Rodman","year":"1986","unstructured":"Rodman HM, Doershuk CF, Roland JM: The interaction of 2 diseases: diabetes mellitus and cystic fibrosis. Medicine 65:389?397, 1986","journal-title":"Medicine"},{"key":"CR22","first-page":"818","volume-title":"Nelson Textbook of Pediatrics","author":"G Forstner","year":"1987","unstructured":"Forstner G: The exocrine pancreas.In Nelson Textbook of Pediatrics, 13th ed. RE Behrman, VC Vaughan, WE Nelson (eds). Philadelphia, WB Saunders, 1987, pp 818?819","edition":"13th ed"},{"key":"CR23","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1016\/S0022-3476(80)80270-8","volume":"97","author":"CP Howard","year":"1980","unstructured":"Howard CP, Go VLW, Infante AJ, Perrault J, Gerich JE, Haymond MW: Long-term survival in a case of functional pancreatic agenesis. J Pediatr 97:786?789, 1980","journal-title":"J Pediatr"},{"key":"CR24","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1016\/0016-5085(83)90033-1","volume":"85","author":"FK Ghishan","year":"1983","unstructured":"Ghishan FK, Lee PC, Lebenthal E, Johnson P, Bradley CA, Greene HL: Isolated congenital enterokinase deficiency. Gastroenterology 85:727?731, 1983","journal-title":"Gastroenterology"},{"key":"CR25","first-page":"2649","volume-title":"The Metabolic Basis of Inherited Disease","author":"TF Boat","year":"1989","unstructured":"Boat TF, Welsh MJ, Beaudet AL: Cystic fibrosis.In The Metabolic Basis of Inherited Disease, 6th ed. CR Scriver, AL Beaudet, WS Sly, D Valle (eds). New York, McGraw-Hill, 1989, pp 2649?2880","edition":"6th ed"},{"key":"CR26","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1136\/adc.55.5.331","volume":"55","author":"PF Aggett","year":"1980","unstructured":"Aggett PF, Cavanagh NPC, Matthew DJ, Pincott JR, Sutcliffe J, Harries JT: Shwachman's syndrome. Arch Dis Child 55:331?347, 1980","journal-title":"Arch Dis Child"},{"key":"CR27","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/S0022-3476(79)80286-3","volume":"95","author":"HA Pearson","year":"1979","unstructured":"Pearson HA, Lobel JS, Kocoshis SA, Naiman JF, Windmiller J, Lammi AT, Hoffman R, Marsh JC. A new syndrome of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction. J Pediatr 95:976?984, 1979","journal-title":"J Pediatr"},{"key":"CR28","doi-asserted-by":"crossref","unstructured":"Frase LL, Strickland AD, Kachel GW, Krejs GJ: Enhananced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterology 88:478?484","DOI":"10.1016\/0016-5085(85)90510-4"},{"key":"CR29","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1093\/clinchem\/33.7.1211","volume":"33","author":"JA Flick","year":"1987","unstructured":"Flick JA, Schnaar RL, Perman JA: Thin-layer chromatographic determination of urinary excretion of lactulose, simplified and applied to cystic fibrosis patients. Clin Chem 33:1211?1212, 1987","journal-title":"Clin Chem"},{"key":"CR30","first-page":"A350","volume":"94","author":"JA Perman","year":"1988","unstructured":"Perman JA, Flick JA, Kinnear JM, Ellis LE, Durie PR: Measurement of intestinal lactulose permeation detects mild pancreatic dysfunction. Gastroenterology 94:A350, 1988","journal-title":"Gastroenterology"}],"container-title":["Digestive Diseases and Sciences"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/BF01318210.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/BF01318210\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/BF01318210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,4,6]],"date-time":"2020-04-06T14:22:23Z","timestamp":1586182943000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/BF01318210"}},"subtitle":["Progression of pancreatic involvement in adulthood"],"short-title":[],"issued":{"date-parts":[[1991,3]]},"references-count":30,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1991,3]]}},"alternative-id":["BF01318210"],"URL":"https:\/\/doi.org\/10.1007\/bf01318210","relation":{},"ISSN":["0163-2116","1573-2568"],"issn-type":[{"value":"0163-2116","type":"print"},{"value":"1573-2568","type":"electronic"}],"subject":[],"published":{"date-parts":[[1991,3]]}}}