Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome

Sheng-Chieh Lin, Shyh Dar Shyur, Yi-Chun Ma, Li-Hsin Huang, Hung-Chang Lee, Wen-I. Lee

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare genetic primary immunodeficiency disease caused by mutations of the CD40 ligand (CD40L) gene with normal or elevated levels of IgM and markedly decreased serum IgG, IgA, and IgE. Liver disease may occur as a clinical manifestation in XHIGM. This complication appears to increase with age. We report an 18-year-old male patient who had recurrent episodes of acalculous cholecystitis (AC) and sclerosing cholangitis (SC). The diagnosis of XHIGM was confirmed by the finding of CD40L expression <1% of normal and a tyrosine 169 asparaginase (t526a) mutation in exon 5 (the tumor necrosis factor domain) of the CD40L gene. The patient had direct hyperbilirubinemia (direct bilirubin 5.5 mg/dL, total bilirubin 8.7 mg/dL), cholestasis (alkaline phosphatase 1133 U/L, γ-glutamyl transferase 1019 U/L) and elevated transaminases (aspartate aminotransferase 70 U/L, alanine aminotransferase 101 U/L). Findings on abdominal ultrasound and abdominal computed tomography were compatible with AC. After the fourth episode of cholecystitis, cholecystectomy and liver biopsy were performed. Operative cholangiography revealed poor opacification of the hepatic duct and proximal common bile duct; the upstream intrahepatic bile ducts were not visualized. The biopsy specimen showed marked fibrosis of the portal areas. Enterococcus species was cultured from the bile. Children or adolescents with recurrent AC and SC should be evaluated for an underlying immunodeficiency syndrome such as XHIGM.
Original languageEnglish
Pages (from-to)421-426
Number of pages6
JournalJournal of the Formosan Medical Association
Volume104
Issue number6
Publication statusPublished - 2005
Externally publishedYes

Fingerprint

Acalculous Cholecystitis
Sclerosing Cholangitis
CD40 Ligand
Immunoglobulin M
Bilirubin
Intrahepatic Bile Ducts
Biopsy
Common Hepatic Duct
Asparaginase
Mutation
Cholecystitis
Hyperbilirubinemia
Cholangiography
Cholestasis
Enterococcus
Common Bile Duct
Cholecystectomy
Transferases
Aspartate Aminotransferases
Transaminases

Keywords

  • Acalculous cholecystitis
  • CD40 ligand
  • Cholangitis, sclerosing
  • Genetic diseases, X-linked
  • Immunologic deficiency syndromes
  • alanine aminotransferase
  • alkaline phosphatase
  • ampicillin
  • asparaginase
  • aspartate aminotransferase
  • bilirubin
  • cefotaxime
  • gamma glutamyltransferase
  • immunoglobulin M
  • tumor necrosis factor
  • tyrosine
  • acalculous cholecystitis
  • adult
  • anamnesis
  • article
  • case report
  • cholecystectomy
  • cholestasis
  • clinical feature
  • computer assisted tomography
  • disease association
  • Enterococcus
  • exon
  • gene mutation
  • hospital admission
  • hospital discharge
  • human
  • human tissue
  • hyperbilirubinemia
  • hyperimmunoglobulinemia M
  • laboratory test
  • liver biopsy
  • liver fibrosis
  • male
  • peroperative cholangiography
  • physical examination
  • protein domain
  • protein expression
  • recurrent disease
  • sclerosing cholangitis
  • ultrasound
  • X chromosome linked disorder
  • adolescent
  • blood
  • cholecystitis
  • genetic linkage
  • genetics
  • hypergammaglobulinemia
  • immune deficiency
  • liver
  • pathology
  • X chromosome
  • Adolescent
  • CD40 Ligand
  • Cholangitis, Sclerosing
  • Cholecystitis
  • Chromosomes, Human, X
  • Humans
  • Hypergammaglobulinemia
  • Immunoglobulin M
  • Immunologic Deficiency Syndromes
  • Linkage (Genetics)
  • Liver
  • Male
  • Recurrence

Cite this

Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome. / Lin, Sheng-Chieh; Shyur, Shyh Dar; Ma, Yi-Chun; Huang, Li-Hsin; Lee, Hung-Chang; Lee, Wen-I.

In: Journal of the Formosan Medical Association, Vol. 104, No. 6, 2005, p. 421-426.

Research output: Contribution to journalArticle

Lin, Sheng-Chieh ; Shyur, Shyh Dar ; Ma, Yi-Chun ; Huang, Li-Hsin ; Lee, Hung-Chang ; Lee, Wen-I. / Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome. In: Journal of the Formosan Medical Association. 2005 ; Vol. 104, No. 6. pp. 421-426.
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title = "Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome",
abstract = "X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare genetic primary immunodeficiency disease caused by mutations of the CD40 ligand (CD40L) gene with normal or elevated levels of IgM and markedly decreased serum IgG, IgA, and IgE. Liver disease may occur as a clinical manifestation in XHIGM. This complication appears to increase with age. We report an 18-year-old male patient who had recurrent episodes of acalculous cholecystitis (AC) and sclerosing cholangitis (SC). The diagnosis of XHIGM was confirmed by the finding of CD40L expression <1{\%} of normal and a tyrosine 169 asparaginase (t526a) mutation in exon 5 (the tumor necrosis factor domain) of the CD40L gene. The patient had direct hyperbilirubinemia (direct bilirubin 5.5 mg/dL, total bilirubin 8.7 mg/dL), cholestasis (alkaline phosphatase 1133 U/L, γ-glutamyl transferase 1019 U/L) and elevated transaminases (aspartate aminotransferase 70 U/L, alanine aminotransferase 101 U/L). Findings on abdominal ultrasound and abdominal computed tomography were compatible with AC. After the fourth episode of cholecystitis, cholecystectomy and liver biopsy were performed. Operative cholangiography revealed poor opacification of the hepatic duct and proximal common bile duct; the upstream intrahepatic bile ducts were not visualized. The biopsy specimen showed marked fibrosis of the portal areas. Enterococcus species was cultured from the bile. Children or adolescents with recurrent AC and SC should be evaluated for an underlying immunodeficiency syndrome such as XHIGM.",
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author = "Sheng-Chieh Lin and Shyur, {Shyh Dar} and Yi-Chun Ma and Li-Hsin Huang and Hung-Chang Lee and Wen-I. Lee",
note = "被引用次數:7 Export Date: 7 April 2016 CODEN: JFASE 通訊地址: Shyur, S.-D.; Division of Allergy and Immunology, Department of Pediatrics, Mackay Memorial Hospital, No.92, Sec. 2, Chung-Shan North Road, Taipei 104, Taiwan 化學物質/CAS: alanine aminotransferase, 9000-86-6, 9014-30-6; alkaline phosphatase, 9001-78-9; ampicillin, 69-52-3, 69-53-4, 7177-48-2, 74083-13-9, 94586-58-0; asparaginase, 9015-68-3; aspartate aminotransferase, 9000-97-9; bilirubin, 18422-02-1, 635-65-4; CD40 ligand, 226713-27-5; cefotaxime, 63527-52-6, 64485-93-4; gamma glutamyltransferase, 85876-02-4; immunoglobulin M, 9007-85-6; tyrosine, 16870-43-2, 55520-40-6, 60-18-4; CD40 Ligand, 147205-72-9; Immunoglobulin M 參考文獻: Kroczek, R.A., Graf, D., Brugnoni, D., Defective expression of CD40 ligand on T cells causes X-linked immunodeficiency with hyper-IgM (1994) Immunol Rev, 138, p. 39; Banatvala, N., Davies, J., Kanariou, M., Hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome): A case series review (1994) Arch Dis Child, 71, pp. 150-152; Levy, J., Espanol-Boren, T., Thomas, C., Clinical spectrum of X-linked hyper-IgM syndrome (1997) J Pediatr, 131, pp. 47-54; Winkelstein, J.A., Marino, M.C., Ochs, H., The X-linked hyper-IgM syndrome: Clinical and immunologic features of 79 patients (2003) Medicine, 82, pp. 373-384; Notarangelo, L.D., Hayward, A.R., X-linked immunodeficiency with hyper-IgM (XHIM) (2000) Clin Exp Immunol, 120, pp. 399-405; Hollenbaugh, D., Grosmaire, L.S., Kullas, C.D., The human T cell antigen gp39, a member of the TNF gene family, is a ligand for the CD40 receptor: Expression of a soluble form of gp39 with B cell co-stimulatory activity (1992) EMBO J, 11, pp. 4313-4321; Rosen, F.S., Kevy, S.V., Merier, E., Recurrent bacterial infections and dysgammaglobulinemia: Deficiency of 7S gamma-globulins in the presence of elevated 19S gamma-globulins (1961) Pediatrics, 28, pp. 182-195; Leiva, J.I., Etter, E.L., Gathe, J., Surgical therapy for 101 patients with acquired immunodeficiency syndrome and symptomatic cholecystitis (1997) Am J Surg, 174, pp. 414-416; Keaveny, A., Karasik, M.S., Hepatobiliary and pancreatic infections on AIDS (1998) AIDS Patient Care STDS, 12, pp. 347-357; Hayward, A.R., Levy, J., Facchetti, F., Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM (1997) J Immunol, 158, pp. 977-983; Mieli-Vergani, G., Vergani, D., Sclerosing cholangitis in the paediatric patient (2001) Best Pract Res Clin Gastroenterol, 15, pp. 681-690; Vitellas, K.M., Keogan, M.T., Freed, K.S., Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography (2000) Radiographics, 20, pp. 959-975; Abdo, A., Klassen, J., Urbanski, S., Reversible sclerosing cholangitis secondary to cryptosporidiosis in a renal transplant patient (2003) J Hepatol, 38, pp. 688-691; Hadzic, N., Pagliuca, A., Rela, M., Correction of the hyper-IgM syndrome after liver and bone marrow transplantation (2000) N Engl J Med, 342, pp. 320-324; Ter Borg, P.C., Van Buuren, H.R., Depla, A.C., Bacterial cholangitis causing secondary sclerosing cholangitis: A case report (2002) BMC Gastroenterol, 2, p. 14; Tanaka, Y., Koshiyama, H., Nakao, K., Rapid progress of acute suppurative cholangitis to secondary sclerosing cholangitis sequentially followed-up by endoscopic retrograde cholangiography (2001) Endoscopy, 33, pp. 633-635; Debray, D., Pariente, D., Urvoas, E., Sclerosing cholangitis in children (1994) J Pediatr, 124, pp. 49-56; Sebagh, M., Farges, O., Kalil, A., Sclerosing cholangitis following human orthotopic liver transplantation (1995) Am J Surg Pathol, 19, pp. 81-90; Kalliafas, S., Ziegler, D.W., Flancbaum, L., Acute acalculous cholecystitis: Incidence, risk factors, and outcome (1998) Am Surg, 64, pp. 471-475; Teixeira, J.P.A., Malheiro, L., Pontinha, N., Infectious factors in acute acalculous cholecystitis (2002) Hepato-Gastroenterology, 49, pp. 1484-1486; Ryu, J.K., Ryu, K.H., Kim, K.H., Clinical features of acute acalculous cholecystitis (2003) J Clin Gastroenterol, 36, pp. 166-169; Ikeda, S., Kimura, W., Futakawa, N., Acute acalculous cholecystitis with a decrease in CD4/CD8 ratio (1997) J Gastroenterol, 32, pp. 268-272; Notarangelo, L.D., Hayward, A.R., X-linked immunodeficiency with hyper-IgM (XHIM) (2000) Clin Exp Immunol, 120, pp. 399-405; Okolicsanyi, L., Groppo, M., Floreani, A., Treatment of primary sclerosing cholangitis with low-dose ursodeoxycholic acid: Results of a retrospective Italian multicentre survey (2003) Dig Liver Dis, 35, pp. 325-331; Beuers, U., Spengler, U., Kruis, W., Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: A placebo-controlled trial (1992) Hepatology, 16, pp. 707-714; Gilger, M.A., Gann, M.E., Opekun, A.R., Efficacy of ursodeoxycholic acid in the treatment of primary sclerosing cholangitis in children (2000) J Pediatr Gastroenterol Nutr, 31, pp. 136-141; Pardi, D.S., Loftus, E.V., Kremers, W.K., Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis (2003) Gastroenterology, 124, pp. 889-893",
year = "2005",
language = "English",
volume = "104",
pages = "421--426",
journal = "Journal of the Formosan Medical Association",
issn = "0929-6646",
publisher = "Elsevier Science Publishers B.V.",
number = "6",

}

TY - JOUR

T1 - Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome

AU - Lin, Sheng-Chieh

AU - Shyur, Shyh Dar

AU - Ma, Yi-Chun

AU - Huang, Li-Hsin

AU - Lee, Hung-Chang

AU - Lee, Wen-I.

N1 - 被引用次數:7 Export Date: 7 April 2016 CODEN: JFASE 通訊地址: Shyur, S.-D.; Division of Allergy and Immunology, Department of Pediatrics, Mackay Memorial Hospital, No.92, Sec. 2, Chung-Shan North Road, Taipei 104, Taiwan 化學物質/CAS: alanine aminotransferase, 9000-86-6, 9014-30-6; alkaline phosphatase, 9001-78-9; ampicillin, 69-52-3, 69-53-4, 7177-48-2, 74083-13-9, 94586-58-0; asparaginase, 9015-68-3; aspartate aminotransferase, 9000-97-9; bilirubin, 18422-02-1, 635-65-4; CD40 ligand, 226713-27-5; cefotaxime, 63527-52-6, 64485-93-4; gamma glutamyltransferase, 85876-02-4; immunoglobulin M, 9007-85-6; tyrosine, 16870-43-2, 55520-40-6, 60-18-4; CD40 Ligand, 147205-72-9; Immunoglobulin M 參考文獻: Kroczek, R.A., Graf, D., Brugnoni, D., Defective expression of CD40 ligand on T cells causes X-linked immunodeficiency with hyper-IgM (1994) Immunol Rev, 138, p. 39; Banatvala, N., Davies, J., Kanariou, M., Hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome): A case series review (1994) Arch Dis Child, 71, pp. 150-152; Levy, J., Espanol-Boren, T., Thomas, C., Clinical spectrum of X-linked hyper-IgM syndrome (1997) J Pediatr, 131, pp. 47-54; Winkelstein, J.A., Marino, M.C., Ochs, H., The X-linked hyper-IgM syndrome: Clinical and immunologic features of 79 patients (2003) Medicine, 82, pp. 373-384; Notarangelo, L.D., Hayward, A.R., X-linked immunodeficiency with hyper-IgM (XHIM) (2000) Clin Exp Immunol, 120, pp. 399-405; Hollenbaugh, D., Grosmaire, L.S., Kullas, C.D., The human T cell antigen gp39, a member of the TNF gene family, is a ligand for the CD40 receptor: Expression of a soluble form of gp39 with B cell co-stimulatory activity (1992) EMBO J, 11, pp. 4313-4321; Rosen, F.S., Kevy, S.V., Merier, E., Recurrent bacterial infections and dysgammaglobulinemia: Deficiency of 7S gamma-globulins in the presence of elevated 19S gamma-globulins (1961) Pediatrics, 28, pp. 182-195; Leiva, J.I., Etter, E.L., Gathe, J., Surgical therapy for 101 patients with acquired immunodeficiency syndrome and symptomatic cholecystitis (1997) Am J Surg, 174, pp. 414-416; Keaveny, A., Karasik, M.S., Hepatobiliary and pancreatic infections on AIDS (1998) AIDS Patient Care STDS, 12, pp. 347-357; Hayward, A.R., Levy, J., Facchetti, F., Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM (1997) J Immunol, 158, pp. 977-983; Mieli-Vergani, G., Vergani, D., Sclerosing cholangitis in the paediatric patient (2001) Best Pract Res Clin Gastroenterol, 15, pp. 681-690; Vitellas, K.M., Keogan, M.T., Freed, K.S., Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography (2000) Radiographics, 20, pp. 959-975; Abdo, A., Klassen, J., Urbanski, S., Reversible sclerosing cholangitis secondary to cryptosporidiosis in a renal transplant patient (2003) J Hepatol, 38, pp. 688-691; Hadzic, N., Pagliuca, A., Rela, M., Correction of the hyper-IgM syndrome after liver and bone marrow transplantation (2000) N Engl J Med, 342, pp. 320-324; Ter Borg, P.C., Van Buuren, H.R., Depla, A.C., Bacterial cholangitis causing secondary sclerosing cholangitis: A case report (2002) BMC Gastroenterol, 2, p. 14; Tanaka, Y., Koshiyama, H., Nakao, K., Rapid progress of acute suppurative cholangitis to secondary sclerosing cholangitis sequentially followed-up by endoscopic retrograde cholangiography (2001) Endoscopy, 33, pp. 633-635; Debray, D., Pariente, D., Urvoas, E., Sclerosing cholangitis in children (1994) J Pediatr, 124, pp. 49-56; Sebagh, M., Farges, O., Kalil, A., Sclerosing cholangitis following human orthotopic liver transplantation (1995) Am J Surg Pathol, 19, pp. 81-90; Kalliafas, S., Ziegler, D.W., Flancbaum, L., Acute acalculous cholecystitis: Incidence, risk factors, and outcome (1998) Am Surg, 64, pp. 471-475; Teixeira, J.P.A., Malheiro, L., Pontinha, N., Infectious factors in acute acalculous cholecystitis (2002) Hepato-Gastroenterology, 49, pp. 1484-1486; Ryu, J.K., Ryu, K.H., Kim, K.H., Clinical features of acute acalculous cholecystitis (2003) J Clin Gastroenterol, 36, pp. 166-169; Ikeda, S., Kimura, W., Futakawa, N., Acute acalculous cholecystitis with a decrease in CD4/CD8 ratio (1997) J Gastroenterol, 32, pp. 268-272; Notarangelo, L.D., Hayward, A.R., X-linked immunodeficiency with hyper-IgM (XHIM) (2000) Clin Exp Immunol, 120, pp. 399-405; Okolicsanyi, L., Groppo, M., Floreani, A., Treatment of primary sclerosing cholangitis with low-dose ursodeoxycholic acid: Results of a retrospective Italian multicentre survey (2003) Dig Liver Dis, 35, pp. 325-331; Beuers, U., Spengler, U., Kruis, W., Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: A placebo-controlled trial (1992) Hepatology, 16, pp. 707-714; Gilger, M.A., Gann, M.E., Opekun, A.R., Efficacy of ursodeoxycholic acid in the treatment of primary sclerosing cholangitis in children (2000) J Pediatr Gastroenterol Nutr, 31, pp. 136-141; Pardi, D.S., Loftus, E.V., Kremers, W.K., Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis (2003) Gastroenterology, 124, pp. 889-893

PY - 2005

Y1 - 2005

N2 - X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare genetic primary immunodeficiency disease caused by mutations of the CD40 ligand (CD40L) gene with normal or elevated levels of IgM and markedly decreased serum IgG, IgA, and IgE. Liver disease may occur as a clinical manifestation in XHIGM. This complication appears to increase with age. We report an 18-year-old male patient who had recurrent episodes of acalculous cholecystitis (AC) and sclerosing cholangitis (SC). The diagnosis of XHIGM was confirmed by the finding of CD40L expression <1% of normal and a tyrosine 169 asparaginase (t526a) mutation in exon 5 (the tumor necrosis factor domain) of the CD40L gene. The patient had direct hyperbilirubinemia (direct bilirubin 5.5 mg/dL, total bilirubin 8.7 mg/dL), cholestasis (alkaline phosphatase 1133 U/L, γ-glutamyl transferase 1019 U/L) and elevated transaminases (aspartate aminotransferase 70 U/L, alanine aminotransferase 101 U/L). Findings on abdominal ultrasound and abdominal computed tomography were compatible with AC. After the fourth episode of cholecystitis, cholecystectomy and liver biopsy were performed. Operative cholangiography revealed poor opacification of the hepatic duct and proximal common bile duct; the upstream intrahepatic bile ducts were not visualized. The biopsy specimen showed marked fibrosis of the portal areas. Enterococcus species was cultured from the bile. Children or adolescents with recurrent AC and SC should be evaluated for an underlying immunodeficiency syndrome such as XHIGM.

AB - X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare genetic primary immunodeficiency disease caused by mutations of the CD40 ligand (CD40L) gene with normal or elevated levels of IgM and markedly decreased serum IgG, IgA, and IgE. Liver disease may occur as a clinical manifestation in XHIGM. This complication appears to increase with age. We report an 18-year-old male patient who had recurrent episodes of acalculous cholecystitis (AC) and sclerosing cholangitis (SC). The diagnosis of XHIGM was confirmed by the finding of CD40L expression <1% of normal and a tyrosine 169 asparaginase (t526a) mutation in exon 5 (the tumor necrosis factor domain) of the CD40L gene. The patient had direct hyperbilirubinemia (direct bilirubin 5.5 mg/dL, total bilirubin 8.7 mg/dL), cholestasis (alkaline phosphatase 1133 U/L, γ-glutamyl transferase 1019 U/L) and elevated transaminases (aspartate aminotransferase 70 U/L, alanine aminotransferase 101 U/L). Findings on abdominal ultrasound and abdominal computed tomography were compatible with AC. After the fourth episode of cholecystitis, cholecystectomy and liver biopsy were performed. Operative cholangiography revealed poor opacification of the hepatic duct and proximal common bile duct; the upstream intrahepatic bile ducts were not visualized. The biopsy specimen showed marked fibrosis of the portal areas. Enterococcus species was cultured from the bile. Children or adolescents with recurrent AC and SC should be evaluated for an underlying immunodeficiency syndrome such as XHIGM.

KW - Acalculous cholecystitis

KW - CD40 ligand

KW - Cholangitis, sclerosing

KW - Genetic diseases, X-linked

KW - Immunologic deficiency syndromes

KW - alanine aminotransferase

KW - alkaline phosphatase

KW - ampicillin

KW - asparaginase

KW - aspartate aminotransferase

KW - bilirubin

KW - cefotaxime

KW - gamma glutamyltransferase

KW - immunoglobulin M

KW - tumor necrosis factor

KW - tyrosine

KW - acalculous cholecystitis

KW - adult

KW - anamnesis

KW - article

KW - case report

KW - cholecystectomy

KW - cholestasis

KW - clinical feature

KW - computer assisted tomography

KW - disease association

KW - Enterococcus

KW - exon

KW - gene mutation

KW - hospital admission

KW - hospital discharge

KW - human

KW - human tissue

KW - hyperbilirubinemia

KW - hyperimmunoglobulinemia M

KW - laboratory test

KW - liver biopsy

KW - liver fibrosis

KW - male

KW - peroperative cholangiography

KW - physical examination

KW - protein domain

KW - protein expression

KW - recurrent disease

KW - sclerosing cholangitis

KW - ultrasound

KW - X chromosome linked disorder

KW - adolescent

KW - blood

KW - cholecystitis

KW - genetic linkage

KW - genetics

KW - hypergammaglobulinemia

KW - immune deficiency

KW - liver

KW - pathology

KW - X chromosome

KW - Adolescent

KW - CD40 Ligand

KW - Cholangitis, Sclerosing

KW - Cholecystitis

KW - Chromosomes, Human, X

KW - Humans

KW - Hypergammaglobulinemia

KW - Immunoglobulin M

KW - Immunologic Deficiency Syndromes

KW - Linkage (Genetics)

KW - Liver

KW - Male

KW - Recurrence

M3 - Article

VL - 104

SP - 421

EP - 426

JO - Journal of the Formosan Medical Association

JF - Journal of the Formosan Medical Association

SN - 0929-6646

IS - 6

ER -