20 research outputs found
Study of the frequency, natural history and therapeutics of complications of liver cirrhosis
No full textThis thesis aimed: (1) to study the characteristics and prognostic patterns in a Greek cirrhotic patient population, (2) to prospectively screen cirrhotics with arterial blood gases and albumin perfusion scan, identify those fulfilling the classic hepatopulmonary syndrome (HPS) criteria, correlate with clinical parameters and evaluate the survival of patients with HPS compared to those without HPS, (3) to compare the prognostic accuracy of ALBI, MELD, MELDNa, Child-Pugh and the corrected for Creatinine Child-Pugh score in a genetically homogeneous Cretan cirrhotic population. Commonest aetiology was hepatitis C virus (HCV, 41%) followed by alcohol (31%). The median survival time in compensated cirrhotics was 115 months (95%CI: 95-133), whereas in decompensated patients was 55 months (95%CI: 36-75). HCV patients survived longer while HBV patients had over twice the risk of death of HCV patients. The median time to decompensation was 65 months (95%CI: 51-79), with alcoholics having the highest risk (RR = 2.1 vs HCV patients). Hepatitis B virus patients had the highest risk of hepatocellular carcinoma and alcoholics the lowest. Leading causes of death: liver failure, hepatorenal syndrome, sepsis and HCC progression. There was no difference in HPS between decompensated (24.6%) and compensated cirrhosis (27.3%). In the multivariate analysis only the quantitative index was significant for the diagnosis of HPS (p= 0.001, OR; 95% CI: 7.05; 2.27-21.87). Kaplan- Mayer survival curves indicated a similar overall prognosis for patients diagnosed with HPS (p-value= 0.105). ALBI had an optimum balance between sensitivity and specificity (AUC =0.704, 95% CI= 0.630-0.778) compared to the others scores. In the multivariate analysis, the only factors independently associated with death were the ALBI score (HR= 2.51; 95% CI: 1.69-3.73, p<0.001), the MELDNa score (HR=1.04; 95% CI: 1.00-1.09, p=0.045) and age (HR= 1.05; 95% CI: 1.03-1.07, p<0.001). When only decompensated cirrhosis was evaluated, the multivariate analysis showed that the ALBI score (HR= 3.03; 95% CI: 1.92-4.78, p<0.001) and age (HR= 1.05; 95% CI: 1.03-1.07, p<0.001) were independently associated with death. The results of this study indicate that cirrhosis aetiology and decompensation at presentation are predictors of survival. Alcoholics have the highest decompensation risk, HBV cirrhotics the highest risk of HCC and HCV cirrhotics the highest decompensation-free time. HPS is a frequent complication of cirrhosis. Mild to moderate HPS has no significant effect on survival of cirrhotic patients. The quantitative Tc-MAA test is a reliable tool for diagnosis. ALBI score might be a better prognostic indicator of mortality in cirrhosis and due to its simplicity may substitute for the Child-Pugh scores, MELD and MELD Na score.Αυτή η διατριβή είχε ως στόχο: (1) τη μελέτη των χαρακτηριστικών και των προγνωστικών παραγόντων σε έναν ελληνικό πληθυσμό κιρρωτικών ασθενών, (2) την προοπτική μελέτη των κιρρωτικών ασθενών με αέρια αρτηριακού αίματος και σπινθηρογραφήματος αιμάτωσης πνευμόνων με μακρομόρια αλβουμίνης σεσημασμένα με ραδιενεργό τεχνήτιο (99mTc-MAA), ώστε να διαγνωστούν όσοι πληρούν τα κριτήρια του ηπατοπνευμονικού συνδρόμου (HPS) και να γίνουν συσχετίσεις με κλινικές παραμέτρους και μελέτη της επιβίωσης των ασθενών με HPS σε σύγκριση με εκείνους που δεν είχαν, (3) τη σύγκριση των προγνωστικών μοντέλων επιβίωσης ALBI, MELD, MELDNa, Child-Pugh και τα τροποποιημένα με βάση την κρεατινίνη ορού Child-Pugh score.Η πιο συχνή αιτιολογία της κίρρωσης ήταν ο ιός της ηπατίτιδας C (HCV, 41%) ακολουθούμενος από το αλκοόλ (31%). Ο διάμεσος χρόνος επιβίωσης στους ασθενείς με αντιρροπούμενη κίρρωση ήταν 115 μήνες (95% CI: 95-133), ενώ στους ασθενείς με μη αντιρροπούμενη κίρρωση ήταν 55 μήνες (95% CI: 36-75). Οι κιρρωτικοί ασθενείς με ηπατίτιδα C επέζησαν περισσότερο, ενώ οι ασθενείς με ηπατίτιδα Β είχαν πάνω από το διπλάσιο κίνδυνο θανάτου σε σχέση με αυτούς με ηπατίτιδα C. Ο διάμεσος χρόνος για τη ρήξη της αντιρρόπησης ήταν 65 μήνες (95% CI: 51-79), με τους αλκοολικούς να έχουν τον υψηλότερο κίνδυνο (RR = 2,1 έναντι ασθενών με ηπατίτιδα C). Οι ασθενείς με κίρρωση λόγω της ηπατίτιδας Β είχαν τον υψηλότερο κίνδυνο ανάπτυξης ηπατοκυτταρικού καρκίνου και οι αλκοολικοί το χαμηλότερο. Κυριότερες αιτίες θανάτου ήταν η ηπατική ανεπάρκεια, το ηπατονεφρικό σύνδρομο, η σηψαιμία και η εξέλιξη του ηπατοκυτταρικού καρκίνου.Όσον αφορά το ηπατοπνευμονικό σύνδρομο δεν υπήρχε διαφορά μεταξύ των ασθενών με αντιρροπούμενη (24,6%) και μη αντιρροπούμενη κίρρωση (27,3%). Στην πολυπαραγοντική ανάλυση μόνο ο ποσοτικός δείκτης του σπινθηρογραφήματος ήταν σημαντικός για τη διάγνωση του HPS (p = 0,001, OR, 95% CI: 7,05, 2,27-21,87). Οι καμπύλες επιβίωσης Kaplan-Mayer έδειξαν παρόμοια συνολική πρόγνωση για ασθενείς που είχαν διαγνωστεί με HPS (τιμή p = 0,105).Το ALBI είχε τη βέλτιστη ισορροπία μεταξύ ευαισθησίας και ειδικότητας (AUC = 0,704, 95% CI = 0,630-0,778) σε σύγκριση με τα υπόλοιπα αποτελέσματα. Στην πολυπαραγοντική ανάλυση, οι μόνοι παράγοντες που σχετίζονται ανεξάρτητα με το θάνατο ήταν η τιμή του ALBI (HR = 2,51, 95% CI: 1,69-3,73, p <0,001), η τιμή του MELDNa (HR = 1,04, 95% CI: 1,00-1,09, p = 0,045) και η ηλικία (HR = 1,05, 95% CI: 1,03-1,07, p <0,001). Όταν αξιολογήθηκε μόνο η αντιρροπούμενη κίρρωση, η πολυπαραγοντική ανάλυση έδειξε ότι η τιμή του ALBI (HR = 3,03, 95% CI: 1,92-4,78, p <0,001) και η ηλικία (HR = 1,05, 95% CI: 1,03-1,07, p <0,001 ) συνδέονταν ανεξάρτητα με το θάνατο.Τα αποτελέσματα αυτής της μελέτης δείχνουν ότι η αιτιολογία της κίρρωσης και η ρήξη της αντιρρόπησης στη διάγνωση είναι προγνωστικοί παράγοντες επιβίωσης. Οι αλκοολικοί έχουν τον υψηλότερο κίνδυνο ρήξης της αντιρρόπησης, οι ασθενείς με κίρρωση λόγω ηπατιτίδας Β τον υψηλότερο κίνδυνο ανάπτυξης ΗΚΚ και οι ασθενείς με κίρρωση λόγω ηπατιτίδας C το μεγαλύτερο χρονικό διάστημα έως τη ρήξη της αντιρρόπησης. Το HPS είναι μια συχνή επιπλοκή της κίρρωσης. Το ήπιο έως μέτριο HPS δεν έχει σημαντική επίδραση στην επιβίωση των κιρρωτικών ασθενών. Ο ποσοτικός δείκτης του σπινθηρογραφήματος αιμάτωσης πνευμόνων με μακρομόρια αλβουμίνης σεσημασμένα με ραδιενεργό τεχνήτιο (99mTc-MAA)είναι ένα αξιόπιστο εργαλείο για τη διάγνωση. Η βαθμολογία ALBI μπορεί να είναι ένας καλύτερος προγνωστικός δείκτης της θνησιμότητας στην κίρρωση και λόγω της απλότητάς του μπορεί να αντικαταστήσει τις βαθμολογίες Child-Pugh, MELD και MELD Na
Paired comparison between water and nutrient drink tests in healthy volunteers Teste de bebidas: comparação entre água e solução de nutrientes em voluntários sadios
No full textCONTEXT: Drink tests constitute an inexpensive and non-invasive tool, which has been proposed to discriminate individuals with altered fluid intake, as dyspeptics. However, their use in everyday clinical practice is still limited as standardization still lacks. OBJECTIVE: To perform a direct, paired comparison between the water and the nutrient drink test in normal volunteers. METHODS: Thirty eight normal volunteers (19 males, 19 females, mean age 24.4 ± 0.4 years) underwent drink test with water and nutrient (Nutridrink) within 7-10 days. Both tests included a loading (consumption of 100 mL/min for water and 15 mL/min for Nutridrink for the longest possible period of time) and a recuperation phase (observation after cessation of fluid intake), being separated by the maximal saturation point. During phases, satiety, fullness, discomfort, bloating, belching, nausea, pain and burning sensation (epigastric and thoracic) were recorded using a 0-100 visual analogue scale score (VAS). For the purpose of configuration, four variables were considered: time (t), VAS score (V), VAS slope (S) for a given time period, and probability of participation (Q) at a given timepoint. RESULTS: The loading phase lasted for 11.6 ± 1.7 min in water (total VAS: 879 ± 123, total VAS slope 72.6 ± 10.9 min-1) and 93.3 ± 18.4 min in Nutridrink test (total VAS: 1462 ± 411, total VAS slope 15.9 ± 3.2 min-1); PCONTEXTO: Os testes de bebidas se constituem em meios baratos e não-invasivos propostos para distinguir diferenças de volume ingeridos por indivíduos, como os dispépticos, por exemplo. Entretanto, seu uso na prática clínica ainda é limitado pela falta de parâmetros lineares. OBJETIVOS: Realizar comparação entre ingestão de água e solução de nutrientes em voluntários, utilizando-se o teste de bebidas e escala analógica visual. MÉTODOS: Trinta e oito voluntários (19 homens, 19 mulheres, com média de idade: 24,4 ± 0,4 anos) submeteram-se a teste de bebidas com água e Nutridrink, em intervalo de 7 a 10 dias. Ambos os testes incluíram a fase de ingestão (consumo de 100 mL/min para água e 15 mL/min para o Nutridrink, pelo maior tempo possível), e pela fase de recuperação (observação após o término da ingestão), separados pelo máximo ponto de saturação. Durante as fases observou-se a saciedade, a plenitude, o desconforto, a eructação, os borborigmos, a náusea, a queimação epigástrica ou torácica e a dor, que foram anotadas utilizando-se um escore de escala analógica visual (EAV) variando entre 0-100. Para este propósito quatro variáveis foram consideradas: tempo (T), escore EAV (V), e curva EAV (S), para o período de tempo e a probabilidade de participação a um tempo determinado (Q). RESULTADOS: O tempo de ingestão durou 11,6 ± 1,7 min para a água (total EAV: 879 ±123, total S: 72,6 ± 10,9 min-1) e 93,3 ± 18,4 min para o Nutridrink (total EAV: 1462 ± 411, total S: 15,9 ± 3,2 min-1); P<0.001. O volume médio ingerido foi de 1155 ± 164 mL para água e 1399 ± 276 mL para o nutriente; P = 0.076. A parada de ingestão do líquido foi atribuída à sensação de plenitude em 76,3% para a água e a saciedade em 69,2% para o Nutridrink. Náusea foi relatada em 15,4% somente para o teste de nutriente. Nenhum voluntário reportou dor substancial persistente ou sensação de queimação. A fase de recuperação durou 63,6 ± 7,8 min para a água (total EAV: 278 ± 75, total S 3,97 ± 0,95 min-1) e para o nutriente 123,2 ± 17,5 min (total EAV: 841 ± 126, total S: 6,81 ± 1,63 min-1); P<0.001. Considerando-se o escore total EAV para ambas as fases dos dois testes, a plenitude e a saciedade representaram 4/5 do total (43% e 36%, respectivamente). Eructação (8%), borborigmos (6%), náusea (4%) e desconforto (3%) seguiram-se, enquanto dor e queimação representaram menos de 1% do total. Entretanto, correlações compreendendo o escore total e de sintomas específicos intra e entre testes, revelaram diferenças estatísticas subjacentes significativas na fisiologia da ingestão líquida. Modelo de regressão considerando índice de massa corporal, sexo e idade como variáveis dependentes e escores total e de sintomas específicos, e curvas para ambas as fases do dois testes como variáveis independentes, não revelaram qualquer correlação primaria. A função que estabelece a ligação da probabilidade de participação Q e o escore de sintoma especifico EAV como tempo t é feito pelas fórmulas Q(t)=1/[1+(t/c)^k] e V(t)=Vo*e^(-t/c), respectivamente. Vo é a média do escore sintoma específico; c e k são fase- e teste- constantes relacionadas, e e = 2,718 é base natural logarítimica. CONCLUSÕES: A padronização comparativa de ambos os testes de bebidas pode produzir uma ferramenta clínica útil, objetivando o diagnóstico e o tratamento de eventuais distúrbios funcionais
Diagnosis and outcome of oesophageal Crohn's disease
No full textBACKGROUND AND AIMS
Crohn's disease (CD) can involve any part of the gastrointestinal tract. We aimed to characterize clinical, endoscopic, histologic features and treatment outcomes of CD patients with oesophageal involvement.
METHODS
We collected cases through a retrospective multicentre European Crohn's and Colitis Organisation CONFER [COllaborative Network For Exceptionally Rare case reports] project. Clinical data were recorded in a standardized case report form.
RESULTS
A total of 40 patients were reported [22 males, mean (±SD, range) age at oesophageal CD diagnosis: 25 (±13.3, 10-71) years and mean time of follow-up: 67 (±68.1, 3-240) months]. Oesophageal involvement was established at CD diagnosis in 26 patients (65%) and during follow-up in 14. CD was exclusively located in the oesophagus in 2 patients. Thirteen patients (32.2%) were asymptomatic at oesophageal disease diagnosis. Oesophageal strictures were present in 5 patients and fistulizing oesophageal disease in one. Eight patients exhibited granulomas on biopsies. Proton-pump inhibitors (PPIs) were administered in 37 patients (92.5%). Three patients underwent endoscopic dilation for symptomatic strictures and none oesophageal-related surgery. Diagnosis in pre-established CD resulted in treatment modifications in 9/14 patients. Clinical remission of oesophageal disease was seen in 33/40 patients (82.5%) after a mean time of 7 (±5.6, 1-18) months. Follow-up endoscopy was performed in 29/40 patients and 26/29 (89.7%) achieved mucosal healing.
CONCLUSION
In this case series the endoscopic and histologic characteristics of isolated oesophageal CD were similar to those reported in other sites of involvement. Treatment was primarily conservative, with PPIs administered in the majority of patients and modifications in pre-existing IBD-related therapy occurring in two thirds of them. Clinical and endoscopic remission was achieved in more than 80% of the patients
Screening for Hepatopulmonary Syndrome in Cirrhotic Patients Using Technetium 99m-macroaggregated Albumin Perfusion Lung Scan (Tc-MAA)
No full textLarge adrenal cyst masquerading as a pancreatic cystic tumor: a rare diagnosis based on endoscopic ultrasound
No full textNodular Lymphoid Hyperplasia with Aggressive Endoscopic Appearance in the Colon of an Adult Woman
No full textReply to the letter to the Editor: ‘The role of coexisting cardiovascular disease on disease severity in patients with inflammatory bowel disease’
No full text
Modern industrialisation may increase primary open-angle glaucoma prevalence through easier transmission of Helicobacter pylori infection
No full textCorrigendum: A novel CISD2 mutation associated with a classical wolfram syndrome phenotype alters Ca21 homeostasis and ER-mitochondria interactions. [Human Molecular Genetics (2017)], doi: 10.1093/hmg/ddx060
No full text\ua9 The Author 2017. Published by Oxford University Press. All rights reserved. The Authors would like to apologize for missing information in their acknowledgement. The full acknowledgement should read as follows: We thank Didier Dormant (MD, PhD) for performing the cerebral MRI of the patient and Georges Challe (MD) for the ophthalmological examination. We thank C\ue9cile Delettre from the Institute of Neurosciences (Montpellier, France) for providing fibroblasts from the WFS1 patient. We are also grateful to Alain Lacampagne and Jeremy Fauconnier (Montpellier, France) for their help with the calcium experiments. We thank Ga\ueblle Aug\ue9, Bernadette Chafino and Charlotte Cochaud for technical help. This has now been corrected in the original paper and online
