Considerations for Low Phospholipid-Associated Cholelithiasis (LPAC) Syndrome: Report of Three Cases.

Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare underdiagnosed genetic feature presenting less than 1% of symptomatic cholelithiasis, with variable clinical forms ranging from simple to complications. Diagnosis criteria are recurrent biliary symptomatology occurring in young patients (<40 years old) and/or recurrence after cholecystectomy and/or having a history of biliary gallstones in first-degree relatives and characteristic ultrasound findings. Early detection of this entity, due to the awareness of gastroenterologists, radiologists, and visceral surgeons, will allow an improvement in the quality of life of patients and the prevention of complications. We report three cases of the LPAC syndrome and discuss its different aspects.

What would be the impact of COVID-19 on liver function of a patient with chronic hepatitis B? About a case and literature review.

Liver damage during COVID-19 disease has been described in numerous studies. Its mechanism is poorly understood. It is mainly reserved for severe forms and is manifested by abnormalities of the hepatic assessment and more particularly cytolysis. Particular attention must be paid to patients with chronic liver disease, both in terms of follow-up and treatment. We wanted to know the evolution of COVID-19 and its treatment, on the liver function of a 27-year-old patient followed for chronic non-cirrhotic hepatitis B at the Hassan II University Hospital in Fez. Our patient had stopped the antiviral B treatment and presented COVID-19 infection with minimal to moderate impairment. The initial evaluation showed cytolysis at 4 times upper limit of normal (ULN). Management consisted in the immediate resumption of Tenofovir in combination with hydroxychloroquine (HCQ) and azythromycin with good clinical and biological evolution.

[IgG4-related disease: about 3 cases]. / La maladie à IgG4: à propos de 3 cas.

IgG4-Related disease (IgG4-RD), formerly known as IgG4-related autoimmune polyexocrinopathy, is a new condition including Plasminogen Activator Inhibitor-1 (PAI-1). It can affect different organs (central nervous system, salivary glands, thyroid, lungs, pancreas, bile ducts, liver, digestive tract, kidneys, prostate, etc.) with symptoms depending on the organ that is affected. It is more common in men older than 50 years of age. Its incidence and prevalence are poorly known because it is an uncommon disease. It is most common in Asia, accounting for only 20-30% of PAI in the Western world. Diagnosis is based on histological examination which shows dense lymphoplasmocytic infiltration in the organ affected associated with IgG4-positive plasma cells (immunohistochemistry), organ fibrosis and obliterating venulitis, all this in the context of increased serum IgG4 levels in more than 80% of cases. Patients are sensitive to corticosteroid therapy, with a high risk of relapse after discontinuation of corticosteroid therapy. This leads to the use of immunomodulators, mainly: thiopurines (azathioprine or 6-mercaptopurine), methotrexate and more recently rituximab, which can also be used as induction therapy. Given recent advances, accurate histological and clinical criteria are currently known to limit inappropriate management such as surgery. However, knowledge gaps remain concerning: pathophysiology, identification of specific biomarkers other than IgG4, natural history of the disease and long-term cancer risk assessment, performances of diagnostic tools such as endoscopic ultrasound-guided pancreatic biopsy. As well, consensual international management should be defined in the early stages of the disease and when patients develop recurrences. The purpose of this study was to report 3 cases of IgG4-Related disease on the basis of clinical and radiological criteria as well as therapeutic response.

[Primitive Budd-Chiari syndrome: a diagnostic and therapeutic challenge]. / Budd-Chiari primitif: défi diagnostique et thérapeutique.

Primary Budd-Chiari syndrome is an hepatopathy characterized by obstruction of the hepatic venous flow in the vascular space between the hepatic venules and the junction between the inferior vena cava and the right atrium, thus excluding the causes of upstream (sinusoidal obstruction syndrome) and downstream (cardiac causes). This endoluminal venous obstruction is mainly due to thrombosis or its fibrotic consequences. This is a rare disease, mainly affecting young adults. Clinical manifestations are extremely variable; it can be asymptomatic, acute, subacute or chronic. The diagnosis is mainly based on Doppler ultrasound and/or magnetic resonance imaging. Several causes have been identified, in particular, myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, inherited thrombotic disorders. Symptomatic and etiologic treatments are the commonly used approaches, treatment recommendations are organized in algorithm. Recent therapeutic advances can significantly improve the prognosis.

Non invasive assessment of liver fibrosis in chronic hemodialysis patients with viral hepatitis C.

The liver biopsy has long been the "gold standard" for assessing liver fibrosis in patients with hepatitis C. It's an invasive procedure which is associated with an elevated bleeding, especially in chronic hemodialysis patients. Main goal is to assess liver fibrosis in chronic hemodialysis with HCV by Fibroscan and by biological scores (APRI, Forns and Fib-4), and to measure the correlation between these tests. Cross-sectional study including all chronic hemodialysis patients with hepatitis C virus, in two public hemodialysis centers of Fez. All patients were evaluated for liver fibrosis using noninvasive methods (FibroScan and laboratory tests). Subsequently, the correlation between different tests has been measured. 95 chronic hemodialysis were studied, twenty nine patients (30.5%) with chronic hepatitis C. The average age was 52.38 ± 16.8 years. Nine liver fibrosis cases have been concluded by forns score. Fibroscan has objectified significant fibrosis in 6 cases. On the other side APRI has objectified sgnifivant fibrosis only in 3 cases. The Fib-4 showed severe fibrosis in five cases. The results have been most consistent between APRI and Fib-4, followed by Fibroscan and Forns, then APRI and FibroScan.

Serum transferrin saturation increase is associated with decrease of antibacterial activity of serum in patients with HFE-related genetic hemochromatosis.

OBJECTIVES: Patients with type 1 (HFE-related) genetic hemochromatosis are usually excluded from blood donation on the basis that this disease may facilitate bacterial infections. The aim of the present study was to evaluate the serum antibacterial effect against Salmonella enterica Typhimurium LT2 in relation to iron status. METHODS: Serum samples were collected in 26 iron-overloaded (homozygous C282Y mutation) and 35 iron-depleted hemochromatosis patients and 33 healthy control subjects. The antibacterial activity of sera and iron parameters were tested for each patient. RESULTS: Serum from normal controls had an antibacterial effect against Salmonella Typhimurium LT2. The antibacterial effect decreased from the 1:2 to the 1:8 dilution and was always significantly lower in the iron-overloaded group. In both control and iron-depleted patients, a positive correlation was found between the decrease of antibacterial effect and the increase of both serum iron and transferrin saturation. CONCLUSIONS: These results (a) support the view that chronic iron overload decreases serum antibacterial effect against Salmonella enterica Typhimurium LT2, (b) favor the interest of including, besides serum ferritinemia, serum transferrin saturation levels as a further criterion for iron-depletive treatment efficacy, and (c) provide an argument for not discouraging the use of blood from iron-depleted hemochromatosis patients for transfusion.

Peritoneal tuberculosis in the Fes University Hospital (Morocco). Report of 123 cases.

AIMS: Peritoneal tuberculosis is an important public health issue in Morocco. Our aim was to describe the clinical, biological, and therapeutic features of peritoneal tuberculosis treated in a University Hospital in Morocco. PATIENTS AND METHODS: We retrospectively included 123 patients with peritoneal tuberculosis diagnosed at the gastroenterology unit of the Fes University Hospital between January 2001 and August 2003. RESULTS: The mean age was 28 years with a clear female predominance (sex ratio 2.61). Ascites associated with fever were the most frequent signs found in 80.5% of patients. The ascitic fluid was exsudative in 90% of cases and lymphocytic in 88%. The diagnosis was based on laparoscopy or laparotomy with peritoneal biopsy demonstrating caseating granulomatous lesions in 92.4% of patients. Patients were given antituberculous therapy for 6 months, and the outcome was favourable in 90%. CONCLUSION: Peritoneal tuberculosis is very frequent in Morocco, where the diagnosis is based exclusively on peritoneal biopsies obtained during laparoscopy. With an adapted treatment, the course of the disease is favourable in most cases.