In hereditary hemochromatosis, red cell apheresis removes excess iron twice as fast as manual whole blood phlebotomy.

The current treatment of hereditary hemochromatosis (HH) consists of performing periodic manual whole blood phlebotomies. Erythroapheresis (EPH) is considered to be an alternative procedure if the classic treatment is contra-indicated. A prospective study of 13 consecutive cases of HH were included in a periodic EPH program with the aim of assessing the efficacy, feasibility, and tolerability of EPH in the treatment of HH by induction and maintenance. Iron depletion (ferritin <20 microg/l) was achieved in all patients after a mean of 6.7 +/- 2.9 months of treatment and a mean of 13.5 +/- 7.2 EPH sessions. The procedure was well tolerated and there were no complications. After a follow-up period of 10.5 +/- 6.6 months, only four patients have required further maintenance sessions beyond 6 months after completing the induction therapy. The efficacy, speed, tolerability, and more favorable schedule of an EPH program facilitate treatment of HH.

Prevalence of the C282Y mutation for haemochromatosis on the Island of Majorca.

The C282Y mutation of the HFE gene has been reported to be present in most of the patients with hereditary haemochromatosis (HH) of Northern European ancestry. HH affects approximately 1/300 individuals, but it is not evenly distributed in the different European countries. In the present study, polymerase chain reaction (PCR) and restriction-enzyme digestion were used to analyse the frequency of the most important mutation in haemochromatosis (C282Y) in subjects from Majorca (Balearic Islands, Spain) and patients with haemochromatosis. The results were compared with other studies from Spain and Europe. A total of 420 Majorcan chromosomes were analysed and the C282Y mutation was observed at a frequency of 2.62%+/-0.8 (11 heterozygotes: eight men and three women). In the group of hereditary haemochromatosis probands, 13 out of 14 were homozygous for the C282Y mutation. In the distribution of the C282Y mutation, a north-west to south-east cline was detected, supporting the Celtic origin of this mutation.

[Celiac disease of the adult following orthotopic liver transplantation. Description of a case]. / Enfermedad celíaca del adulto tras trasplante ortotópico de hígado. Descripción de un caso.

A case of celiac disease of the adult is herein described in a patient with a history of orthotopic liver transplantation because of cirrhosis due to hepatitis C virus. The patient presented with a decrease in the levels of cyclosporinemia, diarrhea and an increase in transaminases. This is an infrequent form of presentation leading to differential diagnosis with a complication related to the liver disease.

[Prevalence of monosymptomatic celiac disease in patients with iron deficiency anemia]. / Prevalencia de enfermedad celíaca monosintomática en pacientes con anemia ferropénica.

A prospective study of the prevalence of monosymptomatic celiac disease presented as ferropenic anemia in patients admitted for study such complication of was carried out. All the patients were evaluated by gastroscopy and biopsy of the distal duodenal segment, regardless of endoscopic appearance. Patients presenting an endoscopic lesion clearly suggestive as the origin of the chronic bleeding were excluded from the study. The prevalence of celiac disease, the only manifestation of which was ferropenic anemia, was 3.3% in this series. What is important to note in this study is the importance of duodenal biopsy in the study of ferropenic anemia, with the aim of avoiding diagnostic delay of a possible monosymptomatic celiac disease as the cause of the anemia.

Histological evolution of chronic hepatitis C. Factors related to progression.

We have evaluated the histological progression of liver disease in 29 untreated patients with chronic hepatitis C. All patients were positive to antibodies to hepatitis C virus by ELISA2 and RIBA2. Two liver biopsies were carried out for each patient, with an interval ranging between 12 and 126 months (mean 50.2 +/- 30.7). In all cases the usual histological classification was applied and the histological activity index scoring system according to Knodell et al. was determined. Fifteen cases worsened (51.7%), 12 cases showed no histological changes (41.4%) and two patients improved (6.9%). Cirrhosis was found in five patients (18.5%) in the second liver biopsy. Epidemiological, clinical, biochemical and histological progression and the group with impairment in liver histology. Factors related to histological worsening were: more advanced age (p = 0.002), high levels of aspartate aminotransferase (p = 0.04), high global histological activity index (p = 0.03) and piecemeal necrosis and bridging necrosis scores (p = 0.02) at first biopsy. The histological activity index can be applied to assess the natural history of chronic viral hepatitis, and is a good tool to evaluate the prognosis. Thus chronic hepatitis C virus infection is a histologically progressive disease in at least half the cases.

[A prospective study of the functioning of 2 types of enteral nutrition catheters]. / Estudio prospectivo del funcionamiento de dos tipos de sondas de nutrición enteral.

The problems of disintubation and obstruction of nutritional catheters are common during treatment with enteral nutrition. This prospective study has been designed to evaluate the differences in the frequency of obstruction between two different types of enteral nutrition catheter. Catheter A (Silk, Ibys) with one single lateral outlet, wide, long and with a curved edge, and Catheter B (Nutrisoft M, Kabi-Pfrimmer), with several smaller lateral holes, 46 nutritional periods were studied, divided into two groups: first group, n = 32 (catheter A) and second group n = 14 (catheter B, Nutrisoft M, Kabi-Pfrimmer). There were no significant differences between both groups for the type of diet administered, osmolarity, speed, volume administered, number and duration of interruptions, external fixation, washing of the catheter and duration of the nutritional period. The catheter slipped out in 21 periods (46%). Obstruction of the catheter was the cause of disintubation of the catheter in eight cases, five of the cases with catheter A (15%) and three cases with catheter B (21%); there were no significant differences between the values for both. These results indicate that the frequency of obstruction is similar with both catheters; however, the Silk catheter may have a better tolerance and be easier to intubate, due to its smaller external diameter.