Jaime Poniachik T

Fulminant Hepatic Failure.

Fulminant hepatic failure (FHF) is an acute and eventually fatal illness, caused by a severe hepatocyte damage with massive necrosis. Its hallmarks are hepatic encephalopathy and a prolonged prothrombin time (<40%). FHF is currently defined as hyperacute (encephalopathy appearing within 7 days of the onset of jaundice), acute (encephalopathy appearing between 8 and 28 days) or subacute (encephalopathy appearing between 5 and 12 weeks). FHF can be caused by viruses, drugs, toxins, and miscellaneous conditions such as Wilson’s disease, Budd-Chiari syndrome, ischemia and others. However, a single most common etiology is still not defined. Factors that are valuable in assessing the likelihood of spontaneous recovery are age, etiology, degree of encephalopathy, prothrombin time and serum bilirubin. The management is based in the early treatment of infections, hemodynamic abnormalities, cerebral edema, and other associated conditions. Liver transplant has emerged as the most important advance in the therapy of FHF, with a survival rate that ranges between 60 and 80%. The use of hepatic support systems, extracorporeal liver support and auxiliary liver transplantation are innovative therapies (Rev Med Chile 2002; 130: 691-698).

Heparina e insulina en el tratamiento de la pancreatitis aguda por hipertrigliceridemia: experiencia en 5 casos

Background: Hypertriglyceridemia over 1,000 mg/dl can provoke acute pancreatitis and its persistence can worsen the clinical outcome. On the contrary, a rapid decrease in triglyceride level is beneficial. Plasmapheresis has been performed in some patients to remove chylomicrons from the circulation, while heparin and/or insulin have been administered in some other cases to rapidly reduce blood triglycerides. Heparin and insulin stimulate lipoprotein-lipase activity and accelerate chylomicron degradation. Aim: To report five patients with acute pancreatitis treated with heparin and insulin. Patients and methods: Five patients (4 females and 1 male) seen in the last two years, who suffered acute pancreatitis induced by hypertriglyceridemia are reported. Initial blood triglyceride levels were above 1,000 mg/dl (range 1,590-8,690 mg/dl). Besides the usual treatment of acute pancreatitis, heparin and/or insulin were administered intravenously in continuous infusion. Heparin dose was guided by usual parameters of blood coagulation, and insulin dose, by serial determinations of blood glucose. Pancreatic necrosis was demonstrated in 4 patients. Results: Serum triglyceride levels decreased to <500 mg/dl within 3 days in all cases. No complication of treatment was observed and all patients survived. Early and late complications of pancreatitis occurred in one patient. Conclusion: Administration of heparin and/or insulin is an efficient alternative to reduce triglyceride levels in patients with acute pancreatitis and hypertriglyceridemia (Rev Med Chile 2001; 129: 1373-8)

Ecotomografía Doppler arterial intrarrenal en pacientes cirróticos con ascitis, con y sin síndrome hepatorrenal

Background: The pathophysiological hallmark of the hepatorenal syndrome (HRS) is renal vasoconstriction. Doppler ultrasonography can be used to assess the vascular resistance in small renal intraparenchymal vessels through analysis of the Doppler waveform by a parameter termed Resistive Index (RI). We postulated that the RI could be important for the diagnosis and prognosis of HRS. Aims: to assess the RI in cirrhotic patients with ascites, with and without HRS. Patients and methods: We studied 48 cirrhotics with ascites, of whom 12 were with and 36 without HRS and other 23 were normal subjects. We measured the intrarenal arterial RI (Resistive index = Peak systolic velocity - Minimum diastolic velocity/Peak systolic velocity) with color Doppler ultrasonography after visualization of interlobular or arcuate arteries. It was considered abnormal when higher than 0.70. Results: The RI values, mean and SD) were: normal subjects: 0.58 ± 0.05, cirrhotics with ascites: 0.65 ± 0.05 and cirrhotics with ascites and HRS: 0.78 ± 0.11. Patients with HRS had significantly higher values than those without HRS (p < 0.001). The Relative Risk of developing the HRS in patients with a RI ≥ 0.70 were 3.32 (CI 95% = 1.79 - 6.2) Conclusions: The RI was useful in patients with cirrhosis and ascites for the prognosis of HRS and could suggest diagnosis of HRS with values of 0.78 or higher, if other clinical conditions that produce renal vasoconstriction are excluded (Rev Med Chile 2002; 130: 173-80)

Obesidad: factor de riesgo para esteatohepatitis y fibrosis hepática

Background: Nonalcoholic fatty liver (NAFL) has been recognized as a cause of chronic liver disease. Its main risk factor is obesity. Aim: To describe the clinical and liver pathological findings in a group of patients who underwent surgery as obesity treatment. Patients and Methods: Sixty eight patients with severe or morbid obesity were subjected to surgery as obesity treatment. Each patient was evaluated with a complete clinical and laboratory medical assessment. A wedge of liver was excised during surgery. Liver biopsies were analyzed without knowledge of clinical and laboratory findings. The presence of steatosis, inflammation (portal or lobular), fibrosis and cirrhosis were recorded in the pathological analysis. Age and body mass index (BMI) were correlated with pathological data. Significance was set at a p value of less than 0.05. Results: Ninety one percent of patients had steatosis, 45% inflammation and 47% fibrosis. One patient had cirrhosis (1,4%). There was a statistically significant association between BMI and moderate or severe steatosis (p <0.03). There was also an association between BMI and portal (p=0.017) and lobular inflammation (p=0.034). A BMI over 40 kg/m2 (morbid obesity) was significantly associated with the presence of fibrosis (p=0.032). Moreover, the presence of moderate or severe steatosis was a risk factor for the development of hepatic fibrosis (p=0.026). Conclusions: Obesity is a major and independent risk factor for steatohepatitis and fibrosis. The degree of steatosis in the liver biopsy, is a risk factor for the development of fibrosis (Rev Med Chile 2002; 130: 731-6)

Alteraciones de la tolerancia a la glucosa y frecuencia de síndrome metabólico en pacientes con enfermedad por hígado graso no alcohólico

Dr. Jaime Poniachik. Centro de Gastroenterologia, Hospital ClinicoUniversidad de Chile. Santos Dumont 999, Santiago-Chile. Fax: (56-2) 978 8353.E-mail: jponiach@redclinicauchile.clDra. Veronica Araya. Seccion Endocrinologia. Hospital Clinico U. de Chile. SantosDumont 999. Santiago de Chile. Fax: (56-2) 777 6891. E-mail: varaya@redclinicauchile.cl

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia

Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjogren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments

Trombosis venosa mesentérica: manifestaciones clínicas, terapia y evolución

Background: Mesenteric vein thrombosis (MVT) is uncommon and accounts for 5-10% of all mesenteric ischemic events. In 80% of cases, an etiologic factor is found. The clinical presentation varies and the diagnosis is made based on imaging studies. The treatment involves anticoagulation alone or in combination with surgery. Aim: To describe the clinical characteristics of patients with MVT. Patients and methods: Retrospective and prospective review of all cases with MVT, treated between 1995-2001. The clinical presentation, imaging studies, treatment and outcome were evaluated. Results: 29 cases of MVT were reviewed (14 females, age 56 ± 15 years). Twenty two patients (76%) had recognizable risk factors. The main symptoms were abdomianl pain (86%) and vomiting (55%). The diagnosis of acute mesenteric ischemia was suspected on admission only in 6 patients (21%). Thirteen patients underwent transabdominal color Doppler ultrasonography and the diagnosis was confirmed for 11 of these (85%). Twenty out of 24 patients (85%) studied with computed tomography, had positive signs of MVT. Twenty one patients (72%) recieved anticoagulation, 10 of whom also underwent surgery. Four patients (14%) received surgical treatment alone. Four patients were not treated. Seven patients (24%) died. Conclusions: MVT is difficult to identify. It is necessary to have a high degree of suspicion in patients who have risk factors. The diagnosis is made with imaging studies. The treatment consists of early anticoagulation and surgical intervention when indicated (Rev Med Chile 2005; 133: 17-22). (Key-words: Anticoagulant therapy; Mesenteric vascular occlusion; Venous thrombosis)

Daño hepático por fármacos: características clínicas e histológicas en 33 casos

Background: Drug induced liver disease (DILD) is common and of difficult diagnosis. Aim: To report the clinical, laboratory and pathological findings in 33 patients with DILD. Patients and methods: We revised 1,164 liver biopsies and 57 were selected as suspicious of DILD. In these, the scale proposed by Maria et al was applied to assess the possibility of hepatotoxicity reactions and 33 were selected. Results: The 33 cases had a mean age of 48±18 years and 14 were male. Forty eight medications were involved, with an average of 1.4 drugs per patient. The main drugs were antimicrobials, antineoplastics-immunosuppresives and non-steroidal antiinflammatory drugs. The clinical presentations in order of frequency were cholestasis, hepatitis, asymptomatic, fulminant hepatitis and cirrhosis. The laboratory alterations observed in cases with hepatitis were 20 fold transaminase and bilirubin elevation. In cholestasis, moderate elevations of alkaline phosphatases and gamma glytamyl transferase were observed. Pathology showed hepatocellular damage, cholestasis and mixed damage, but also submassive necrosis and cirrhosis in one case. Conclusions: The present study confirms that DILD is frequently unpredictable and that it can cause a wide variety of clinical and pathological presentations, that can even evolve to chronicity (Rev Med Chile 2003; 131: 1128-34). (Key Words: Drug hypersensitivity; Drug toxicity)

Transmisión familiar del virus de la hepatitis C en un grupo de población chilena

Background: Intravenous drug use, sexual promiscuity and a longer cohabitation period are considered risk factors for familial transmission of hepatitis C virus. The relative importance of this type of transmission is a subject of controversy. Aim: To study familial clustering of hepatitis C virus infection and its risk factors. Material and methods: HCV positive patients (91 with chronic hepatitis, 88 with cirrhosis, nine with hepatic carcinoma and 29 hemophiliacs) were the index cases. HCV antibodies were measured by ELISA and the type of relationship with the index case was investigated in 317 family members. Results: Positive anti HVC antibodies were detected in 12 of 243 family members of patients with chronic liver disease and in none of the family members of patients with hemophilia. Of these, five were couples of an index case with a long cohabitation period. Ten members had an index case with a severe liver disease (three with Child C cirrhosis and seven with liver carcinoma). Conclusions: Family transmission of HCV infection is uncommon in Chile. The association of severe liver disease and family transmission could be due to a higher viral load as responsible for transmission during the early periods of a long lasting disease (Rev Med Chile 2003; 131: 1123-27). (Key Words: Antibodies, viral; Family health; Hepatitis C antibodies)

Estudio de enfermedad celíaca en pacientes con psoriasis

Background: A possible relationship has been reported between psoriasis and celiac disease, with common pathogenic mechanisms that may need further investigation. Aim: To investigate the presence of clinical and serological markers for celiac disease in a group of Chilean psoriatic patients. Material and methods: We included 80 psoriatic patients (42 males) aged 16 to 79 years, whose serum was tested for antitransglutaminase antibodies (ATGA) and antiendomysial antibodies (AEMA). Patients with weakly positive AEMA tests were also tested for antigliadin antibodies (AGA). Results: In six patients (7.5%), AEMA and AGA were positive and one patient was positive for ATGA. An upper gastrointestinal endoscopy and duodenal biopsy was offered to these six patients and five accepted the procedure. Only one had a pathological diagnosis of celiac disease. Conclusions: Only one of 80 patients with psoriasis had celiac disease (1.2%). Other four patients with positive serologic markers had a normal duodenal biopsy. This group of patients may have latent celiac disease and they should be followed up (Rev Med Chile 2007; 135: 1296-303). (Key words: Celiac disease; Gliadin; Psoriasis)