Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study
Abstract
:1. Background
2. Methods
3. Results
4. Discussion
5. Conclusions
- Monitoring liver markers in IBD patients is important since most PSC cases are asymptomatic and elevated liver markers might be the first sign of the disease.
- Patients diagnosed with PSC before IBD diagnosis are more likely to have a more aggressive course of the disease.
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
Abbreviations
PSC | primary sclerosing cholangitis |
IBD | inflammatory bowel disease |
UC | ulcerative colitis |
CD | Crohn’s diseae |
EC | Eosinophilic Colitis |
AIH | Autoimmune hepatitis |
AST | aspartate aminotransferase |
ALT | alkaline aminotransferase |
AP | alkaline phosphatase |
BLB | total bilirubin |
GTP | glutamyltranspeptidase |
APRI | AST-to-platelet-ratio Index |
ANCA | antineutrophil cytoplasmic antibodies |
ANA | antinuclear antibodies |
ASCA | anti Saccharomyces cerevisiae anibodies |
anti-SMA | anti smooth muscle antibodies |
US | ultrasonography examination |
MRCP | magnetic resonance cholangiopancreatography |
ERCP | endoscopic retrograde cholangiopancreatography |
CCA | cholangiocarcinoma |
5-ASA | 5-aminosalicylic acid |
AZA | azathioprine |
UDCA | ursodeoxycholic acid |
References
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Ulcerative | Colitis N = 22 | Eosinophilic Colitis N = 2 | Crohn’s | Disease N = 4 |
---|---|---|---|---|
Paris Classification | Number of Patients | Number of Patients | Paris Classification | Number of Patients |
E1 E2 E3 E4 Other S0 S1 | 0/22 (0%) 3/22 (13.6%) 2/22 (9.1%) 16/22 (72.7%) 1/22 (4.6%) 17/22 (77.3%) 5/22 (22.7%) | 1/2 (50%) 1/2 (50%) | A1L1B2 A1L2B3 A1L3B2 | 1/4 (25%) 1/4 (25%) 2/4 (50%) |
Total | 22 (100%) | 2 (100%) | Total | 4 (100%) |
At Diagnosis | Follow-Up- 3 Years | p | |
---|---|---|---|
ALT (U/L) | 38 | 23 | 0.12 |
Mean activity (Min-Max) | (9–412) | (9–179) | |
AST (U/L) | 35.5 | 30 | 0.18 |
Mean activity (Min-Max) | (16–285) | (17–254) | |
GGT (U/L) | 73 | 37 | 0.01 |
Mean activity (Min-Max) | (48–778 | (12–235) | |
BLB (µmol/L) | 8.95 | 10.8 | 0.08 |
Mean concentration (Min–Max) | (4.08–140.7) | (4.8–45.6) | |
APRI index | 0.33 | 0.273 | 0.28 |
(Min-Max) | (0.112–2.679) | (0.079–2.759) |
COMPLICATIONS | PSC Diagnosed before IBD | IBD Diagnosed before PSC/toGETHER | Total | |
---|---|---|---|---|
N = 6 | N = 22 | N = 30 | ||
Portal hypertension | 2/6 (33.3%) | 0/22 (0.0%) | 2/30 (6.6%) | p < 0.05 |
Liver Cirrhosis | 2/6 (33.3%) | 0/22 (0.0%) | 2/30 (6.6%) | |
Critical stricture of bile ducts | 3/6 (50.0%) | 2/22 (9.1%) | 5/30 (16.6%) |
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Wiecek, S.; Wojtyniak, A.; Pindur, B.; Machnikowska-Sokołowska, M.; Gruszczyńska, K.; Grzybowska-Chlebowczyk, U. Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study. Medicina 2021, 57, 663. https://doi.org/10.3390/medicina57070663
Wiecek S, Wojtyniak A, Pindur B, Machnikowska-Sokołowska M, Gruszczyńska K, Grzybowska-Chlebowczyk U. Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study. Medicina. 2021; 57(7):663. https://doi.org/10.3390/medicina57070663
Chicago/Turabian StyleWiecek, Sabina, Alicja Wojtyniak, Barbara Pindur, Magdalena Machnikowska-Sokołowska, Katarzyna Gruszczyńska, and Urszula Grzybowska-Chlebowczyk. 2021. "Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study" Medicina 57, no. 7: 663. https://doi.org/10.3390/medicina57070663