Bone mineral density and bone acquisition in children and young adults with cystic fibrosis: a follow-up study

OBJECTIVE: To investigate bone mineral density and bone homeostasis in cystic fibrosis (CF) and to assess changes in a 2-year period. METHODS: Thirty-eight patients with clinically stable CF (11 children, 16 adolescents, 11 young adults) were enrolled. No patient was treated with corticosteroids before or during the study. Weight and height Z scores and bone mineral density (BMD) Z-score at the femoral neck and the lumbar spine were recorded at the beginning of the study and after 2 years. Osteocalcin and cross-link excretion, both measurements of bone turnover were also measured. Correlations between BMD, bone turnover parameters, disease severity, pubertal stage, and nutritional state were calculated. The maternal BMD was also determined and related to that of the child. RESULTS: Height and weight Z scores were normal in children and below normal in adolescents. Puberty was delayed in most patients. Bone age was lower than chronological age in adolescents. Lumbar spine and femoral neck BMD Z scores were below normal in each age group. Disease severity determined by Schwachman score correlated with lumbar BMD (r = 0.45, P < 0.02). BMD Z scores did not change during 2 year follow-up. Maternal and patient lumbar and femoral BMD correlated significantly (r = 0.51, P < 0.01, and r = 0.54, P < 0.01, respectively). CONCLUSIONS: Bone deficit is present in patients with CF who have never received steroid treatment. Delay of puberty, chronic inflammation, or genetic susceptibility might be responsible for this phenomenon which was found in patients who had never received steroids and who were in relatively good clinical state.     

Clinical significance of HNPCC, surgical aspects of early recognition

A hereditary background may be demonstrated in approximately 15-20% of colorectal carcinomas. Familial adenomatous polyposis syndrome (FAP) constitutes about 1% of this patient population whereas hereditary non-polyposis colorectal carcinoma (HNPCC) makes up a further 3-6% of colorectal malignancies. The clinical features of HNPCC are dominant right colon localization, early age of onset, high prevalence of synchronous and metachronous tumors. Germline mutations of the so-called mismatch repair genes can be demonstrated in the genetic background of HNPCC. Screening and careful follow-up of these families are essential since the lifetime occurrence of colorectal carcinomas and HNPCC associated tumors has an 80-85% prevalence. The recognition of the affected families may be accomplished by taking a thorough family history, spanning several generations based on the Amsterdam and Bethesda Criteria, immunohistological investigations of the removed specimens and finally the exact identification of the pathologic MMR gene mutations. Radical surgical intervention is advised in cases of proven mutation carriers who are suffering from CRC. The index persons and their family members must be under regular control for their lifetime, with one-to-two year intervals to prevent fatal disease. The initiation of a national HNPCC register would further decrease the mortality and morbidity of the disease.     

Use of buccal mucosa patch graft for recurrent large urethrocutaneous fistula after hypospadias repair

INTRODUCTION: To assess the effectiveness of buccal mucosa patch graft in the treatment of recurrent large urethrocutaneous fistula after hypospadias repair. MATERIALS AND METHODS: A free graft of buccal mucosa was used for closure in 7 boys (mean age 4.8 years) with large (>4 mm) urethocutaneous fistula. Four fistulas were in the midshaft, 2 of them penoscrotal and 1 coronal type. All patients had undergone at least two previous unsuccessful fistula repairs, and 3 of them had undergone three attempts for closure. Fistula repairs were similar in all cases. RESULTS: The repair was successful in 6 out of 7 cases, and in these cases the urinary stream was good after the removal of the catheter. The unsuccessful case was the coronal one. CONCLUSION: Based on our experience it seems that in cases with recurrent large fistula after hypospadias reconstruction, the use of buccal mucosa patch graft for closure is a good treatment choice.     

Short communication: seasonal onset of menopause?

BACKGROUND: Seasonal variations of reproductive functions in wild mammals are well known. Similar but blunted seasonal trends have also been described for humans. METHODS: We performed a questionnaire-based study of 149 patients that was designed to search for environmental influence on symptom presentation among patients attending an open menopause service. RESULTS: The evaluated data show a conspicuous seasonality in cessation of menstrual bleeding, with a higher peak after the vernal (spring) equinox and a lower one after the autumn equinox. CONCLUSIONS: Of the several environmental factors considered in this study, the sequence of seasons seems to affect most obviously the process leading to the loss of menstrual cycling. The triggering factor(s) eliciting the onset of the menopausal process and the mediators involved, however, need further analysis.     

Isolation of an integron-borne blaVIM-4 type metallo-beta-lactamase gene from a carbapenem-resistant Pseudomonas aeruginosa clinical isolate in Hungary

The first integron-borne metallo-beta-lactamase gene was isolated in Hungary. The bla(VIM-4) gene is located on a class 1 integron that also carries a novel bla(OXA)-like gene. The integron is harbored by a serotype O12 Pseudomonas aeruginosa strain and shows high structural similarity to integrons isolated in Greece and Poland.     

New perspectives in the evolution of prognosis and treatment of chronic lymphocytic leukaemia

Chronic lymphocytic leukaemia is a disease with a variable clinical course and prognosis. New prognostic factors like immunoglobulin gene mutational status, cytogenetic abnormalities, CD38 and ZAP70 expression of malignant cells have been described recently. Conventional and biological prognostic factors allow to identify patients with unfavorable prognosis at early stage. Purin analogues, fludarabine and fludarabine based combinations can achieve complete hematological remission in approximately one third of patients with chronic lymphocytic leukaemia. Chemoimmunotherapy (most experience is obtained by combination of fludarabine + cyclophosphamide + rituximab) can increase not only complete remission rate, but also induce molecular remission in some cases. Stem cell transplantation as well as early and effective chemotherapy are curative choices of treatment in patients with chronic lymphocytic leukaemia.     

Application of robots in the upper limb physiotherapy of patients with hemiparesis

OBJECTIVE: Authors describe the robotic physiotherapy system developed in the scope of Reharob project belonging to the 5th Framework Programme of the European Union. The system is able to execute robot mediated physiotherapy of the shoulder and the elbow of patients with spastic hemiparesis. Due to the fact, that spasticity is velocity dependent, it is important to execute the exercises with a relatively slow and constant velocity. In such cases a robot can support the work of the physiotherapist first of all when delivering exercises with high repetition number. The objective of the first clinical trial was to gain experiences with the system, to prove its functionality and security. METHOD: 4 healthy subjects and 8 patients with spastic hemiparesis were given 30 minutes long robot mediated physiotherapy on 20 consecutive days. RESULTS: During 240 therapeutic events the robots executed the exercises according to the programme established by the physiotherapist, without any significant mechanical problem or dangerous situation. CONCLUSIONS: The clinical trial confirmed the functionality of the Reharob Therapeutic System. The development of the system will be continued according to the experiences gained during the trial.     

Eradication of Helicobacter pylori infection in Europe: a meta-analysis based on congress abstracts, 1997-2002

BACKGROUND: Meta-analyses evaluated several aspects of Helicobacter pylori eradication based on the randomised controlled trials. AIM: to perform a meta-analysis of the papers presented at the European Helicobacter Pylori Study Group and United European Gastroenterology Week meetings from 1997 to 2002. METHODS: Abstracts dealing with the eradication of Helicobacter pylori have been reviewed and the randomised, controlled studies from European countries were included. The studies were classified into groups based on eradication schedules, antibiotics used and country of provenience. The pooled eradication rates were calculated and the differences were assessed by multiple variance analysis. RESULTS: One-hundred and two studies were accepted comprising 25,644 cases and 398 treatment arms. The eradication rate of proton pump inhibitor-based first line triple therapies was 80.4% (confidence interval: 78.9-81.8); no difference was observed between the five proton pump inhibitors (p > 0.05). Ranitidine bismuth citrate based regimens were efficient in 79.9% (75.7-84.0) (p = 0.95 vs PPI). H2 blockers-based therapies achieved 68.6% (59.0-78.1) (p = 0.0007 vs proton pump inhibitor and p = 0.005 vs ranitidine bismuth citrate-based regimens). Proton pump inhibitor-based double combinations were efficient in 47.1 (31.9-62.4) (p = 0.001 vs triple regimens). Clarithromycin+amoxicillin/nitroimidazole combinations achieved rates of 79.6% and 84.1%, respectively, while amoxicillin-nitroimidazole regimens were less efficient (72.5%, 66.6-78.5) (p = 0.006). The pooled eradication rate of second-line triple regimens was 75.5% (69.9-86.4)(p = 0.08 vs primary treatment). Quadruple therapies were successful in 81.1% (76.6-85.6) of cases as first-line and 73.8% (61.2-86.4) as second-line regimens (p = 0.77 and p = 0.02 vs triple regimens). The pooled eradication rates varied from 58% to 92% in the European countries. CONCLUSIONS: The pooled eradication rate of the primary proton pump inhibitor/ranitidine bismuth citrate-based triple regimens are comparable with the results of meta-analyses. H2 blocker-based triple and proton pump inhibitor-based double regimens are of lower efficacy. Quadruple regimens were not better than triple therapies. The eradication rates per country varied, approaching 80% in most places.The results confirm in part post-hoc the validity of the Maastricht consensus recommendations.