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Biomedical subjects

J Y Mary

Publications and source records attributed to J Y Mary.

At least 19 recordsLinked to original sources
PubMed2002

Osteoporosis in inflammatory bowel disease: effect of calcium and vitamin D with or without fluoride.

BACKGROUND: Previous data have indicated low bone formation as a mechanism of osteoporosis in inflammatory bowel disease. Fluoride can stimulate bone formation. AIM: To assess the effect of fluoride supplementation on lumbar spine bone mineral density in osteoporotic patients with inflammatory bowel disease treated in parallel with calcium and vitamin D. METHODS: In this prospective, randomized, double-blind, parallel and placebo-controlled study, 94 patients with inflammatory bowel disease (lumbar spine T score below - 2 standard deviations, normal serum 25OH vitamin D), with a median age of 35 years, were included. Bone mineral density was measured by dual-energy X-ray absorptiometry. Patients were randomized to receive daily either sodium monofluorophosphate (150 mg, n=45) or placebo (n=49) for 1 year, and all received calcium (1 g) and vitamin D (800 IU). The relative change in bone mineral density from 0 to 12 months was tested in each group (fluoride or placebo) and compared between the groups. RESULTS: Lumbar spine bone mineral density increased significantly in both groups after 1 year: 4.8 +/- 5.6% (n=29) and 3.2 +/- 3.8% (n=31) in the calcium-vitamin D-fluoride and calcium-vitamin D-placebo groups, respectively (P < 0.001 for each group). There was no difference between the groups (P=0.403). Similar results were observed according to corticosteroid intake or disease activity. CONCLUSIONS: Calcium and vitamin D seem to increase lumbar spine density in osteoporotic patients with inflammatory bowel disease; fluoride does not provide further benefit.

Absorptiometry, Photon↗
PubMed2002

A French cluster of Creutzfeldt-Jakob disease: a molecular analysis.

We report the molecular and phenotypic analysis of a French cluster of three cases of Creutzfeldt-Jakob disease (CJD), two of them occurring in 1998 in the same village and the other in 1995 in a neighboring village. Analyses of the occurrence of these events in a close area with less than 3000 inhabitants over the 1992-1999 notification period confirmed that they are rare. This could be explained either by a common source of contamination or by the coincidental occurrence of either sporadic or genetic CJD. We applied genetic analysis and brain PrPres typing to explore these CJD cases. The three patients did not carry any mutation in their prion protein gene coding sequence. All were homozygous for methionine at the polymorphic codon 129. Brain tissue was available from two cases that died in 1998. The two patients showed different PrPres profiles on Western blot and distinct clinico-pathological features. These findings do not support the conclusion that in these three cases, CJD was acquired from a unique source of contamination and suggest that concurrent occurrence of sporadic CJD accounted for this CJD cluster.

Aged↗
PubMed2001

Planning a reproducibility study: how many subjects and how many replicates per subject for an expected width of the 95 per cent confidence interval of the intraclass correlation coefficient.

Reproducibility of a quantitative outcome is usually assessed by means of the intraclass correlation coefficient (ICC). When we are interested in assessing reproducibility from only one sample, we suggest that the study be planned with regards to the expected width of the 95 per cent confidence interval of the ICC. An approximation of this latter width is derived, which allows appraisal of the influence of n the number of subjects and p the number of replicates. Through simulation studies, the approximation is shown to be of good accuracy and can therefore be used reliably. Optimal designs are also discussed such as the optimal distribution between the number of subjects and the number of replicates per subject for a fixed total number of measures.

Biometry↗
PubMed2001

Chromosome 13 abnormalities identified by FISH analysis and serum beta2-microglobulin produce a powerful myeloma staging system for patients receiving high-dose therapy.

A careful prognostic evaluation of patients referred for high-dose therapy (HDT) is warranted to identify those who maximally benefit from HDT as well as those who clearly fail current HDT and are candidates for more innovative treatments. In a series of 110 patients with myeloma who received HDT as first-line therapy, times to event (disease progression and death) were studied through proportional hazard models, in relation to different prognostic factors, including a chromosome 13 fluorescence in situ hybridization (FISH) analysis using a D13S319 probe. Delta13 was detected in 42 patients (38%). Follow-up time among surviving patients and survival time were 48 +/- 3 and 51 +/- 7 months, respectively (median +/- SE). In the univariate analysis, Delta13 was the most powerful adverse prognostic factor for all times to event, especially for the survival time (P <.0001) and was followed by beta2-microglobulin (beta2m) levels 2.5 mg/L or higher (P =.0001). The comparison of survival prognostic models including beta2m 2.5 mg/L or greater and another factor favored the Delta13/beta2m combination. In 22 patients (20%) with no unfavorable factor, the median survival time was not reached at 111 months. In contrast, among 55 patients (50%) with one unfavorable factor and 33 patients (30%) with 2 unfavorable factors, median survival times were 47.3 +/- 4.6 months and 25.3 +/- 3.2 months, respectively (P <.0001). We conclude that delta13, adequately detected by FISH analysis, is a very strong factor related to poor survival, especially when associated with a beta2m level of 2.5 mg/L or higher. Routine FISH Delta13 assessment is strongly recommended for patients considered for HDT.

Actuarial Analysis↗
PubMed2001

Ability of the American College of Rheumatology 1987 criteria to predict rheumatoid arthritis in patients with early arthritis and classification of these patients two years later.

OBJECTIVE: To determine how well the American College of Rheumatology (ACR; formerly, the American Rheumatism Association) 1987 classification criteria for rheumatoid arthritis (RA), when used at study inclusion in a cohort of 270 patients with early (<1 year) arthritis, predicted a diagnosis of RA 2 years later and how well they classified these patients at the end of the 2 years. METHODS: Patients were evaluated during 1995-1997 at 7 hospitals in the Brittany region of France. Patients were evaluated at 6-month intervals until November 1999. The diagnosis made by a panel of 5 rheumatologists (P5R) after the last visit was used as the "gold standard." The ACR 1987 criteria for RA were applied prospectively, without taking into account the initial diagnosis. RESULTS: At the last visit (mean +/- SD followup 29.1 +/- 11.8 months; median 30 months), the P5R diagnosed RA in 98 patients. At the last visit, classification by the ACR criteria was satisfactory, and the combination of an office-based rheumatologist's (OBR's) diagnosis of RA and fulfillment of the ACR criteria was sensitive (87%; 85 of 98 RA patients had both) and highly specific (99%; 170 of 172 non-RA patients did not have both). Application of the criteria at the first visit was of limited value for predicting a diagnosis of RA 2 years later. CONCLUSION: After a 2-year followup, the ACR 1987 classification criteria used in combination with an OBR's diagnosis were effective in distinguishing patients with and without RA. The criteria were not useful for predicting RA in patients with arthritis onset within the previous year. Some patients who met the criteria at baseline and after 2 years did not have RA, suggesting that incorporating exclusion criteria may improve the performance of the ACR criteria when used without taking into account the diagnosis by a rheumatologist, particularly in early arthritis.

Adolescent↗
PubMed2001

Health and functional status of adult recipients 1 year after allogeneic haematopoietic stem cell transplantation.

Increasing numbers of patients are surviving after allogeneic haematopoietic stem cell transplantation (SCT). Among these patients, a number of late complications have been described but few data on the risk factors of these long-term effects of SCT are available. We report the analysis on 105 adult patients, surviving free of haematological disease at a median time of 15 months after SCT. At the time of screening, 52% had returned to work, general health status was normal in 67% and 47% were sexually active. Female patient gender odds ratio (OR) 2.9 (P = 0.01) and age > 25 years (OR = 3.2, P = 0.02) were associated with non-return to work. Decreased general status was associated with chronic graft-versus-host disease (GvHD) (OR = 3.2, P = 0.009) and irradiation (OR = 3.6, P = 0.004). Sexual inactivity was associated with younger age (OR = 7.0, P = 0.0002) and chronic GvHD (OR = 3.3, P = 0.006). Risk factors for altered pulmonary function tests included previous smoking habits, irradiation and chronic GvHD. Obstructive lung disease was associated with a previous history of asthma. Sicca syndrome and conjunctivitis were increased in patients with previous acute GvHD and cataracts were less frequent in patients with aplastic anaemia. Persistent impaired hair re-growth was less frequent in patients who received irradiation (OR = 0.18, P = 0.002) but increased in patients with previous acute GvHD (OR = 5.3, P = 0.007). Microalbuminuria was more frequent in irradiated patients (OR = 9.4, P = 0.05). Raised cholesterol was associated with age (OR = 20.8, P < 0.001), previous acute GvHD (OR = 4.7, P = 0.03), steroid use (OR = 6.3, P = 0.001) and familial hypercholesterolaemia (OR = 4.4, P = 0.04). Decreased bone density was associated with chronic GvHD (OR = 3.9, P = 0.001). Thus, using routine tests in adult patients we were able to detect significant numbers of-non-symptomatic complications enabling early treatment.

Acute Disease↗
PubMed2001

Prolonged immune deficiency following allogeneic stem cell transplantation: risk factors and complications in adult patients.

To evaluate the long-term immune reconstitution after allogeneic haematopoietic stem cell transplantation (SCT), we prospectively screened standard immune parameters in a series of 105 patients, at a median time of 15 months after SCT. Analysing lymphoid phenotypes, in vitro immune functions and immunoglobulin levels, we found that, more than 1 year post SCT, cellular and humoral immunity was still altered in a significant number of patients. CD4+ T cells were < 200/microl in one third of patients, and the CD4/CD8 ratio was still reversed in 78% of patients. Almost all patients showed positive T-cell responses against mitogens, but antigen-specific proliferation assays identified 20% to 80% of non-responders. B-cell counts were reconstituted in 61% of the patients, but levels of total immunoglobulins were still low in 59%. In multivariate analyses, human leucocyte antigen (HLA) disparity between donor and recipient and chronic graft-versus-host disease were the leading causes affecting immune reconstitution. Interestingly, cytomegalovirus (CMV) infections were strongly associated with normal CD8+ T-cell counts. Studying the impact of impaired immune reconstitution on the rate of infections occurring in the 6 years following screening, we identified three parameters (low B-cell count, inverted CD4/CD8 ratio, and negative response to tetanus toxin) as significant risk factors for developing such late infections.

Adolescent↗
PubMed2001

Survival and therapeutic modalities in patients with bone metastases of differentiated thyroid carcinomas.

Data for patients with bone metastases (BMs) of differentiated thyroid carcinoma (DTC) were retrospectively studied to identify factors associated with survival. We especially studied the impact of therapies. Among the 1977 patients followed for DTC in our department from 1958 to 1999, 109 (77 females and 32 males; age range, 20--87 yr) presented BMS: All patients except 1 underwent total thyroidectomy, followed by radioiodine therapy (> or =3.7 gigabecquerels) in 95 cases. Survival rates at 5 and 10 yr were 41% and 15%, respectively. Univariate analyses indicated that a young age at BM discovery (P < 0.005) and the discovery of BM as a revealing symptom of DTC (P < 0.05) were features significantly associated with improved survival as well as radioiodine therapy (P < 10(-4)) and BM complete surgery (P < 0.02). Using multivariate analysis, the detection of BMs as a revealing symptom of thyroid carcinoma (P < 0.0005), the absence of metastasis appearance in other organs than bones during the follow-up (P < 0.03), the cumulative dose of radioiodine therapy (P < 0.0001), and complete BM surgery in young patients (P < 0.04) appeared as independent prognostic features associated with an improved survival.

Adult↗
PubMed2000

Effect of centre on outcome of bone-marrow transplantation for acute myeloid leukaemia. Acute Leukaemia Working Party of the European Group for Blood and Marrow Transplantation.

BACKGROUND: There is increasing pressure for the recognition and replication of good clinical practice. We undertook a study to assess the variability in outcome of allogeneic bone-marrow transplantation among major European centres. METHODS: We studied 13 centres, including 522 patients (aged 16-55 years), which had undertaken more than 30 bone-marrow transplantations between Jan 1, 1987, and Dec 31, 1995, for acute myeloid leukaemia in first complete remission. We undertook a (global) multivariate analysis of all factors known previously to influence outcome and a stratified analysis that initially defined, by multivariate analysis, significant variables in this study and then used a proportional-hazard model including centres. FINDINGS: The overall results at 3 years were 57% (95% CI 53-61) for leukaemia-free survival (LFS), 23% (19-27) for relapse incidence (RI), and 26% (22-30) for treatment-related mortality (TRM) with a range for centres of 36-75%, 10-37%, and 8-54%, respectively. Both methods of analysis showed the centre effect to be highly significant for LFS and TRM, but not for RI. Variables associated with a significantly poor outcome were age over 43 years (p=0.01), time from diagnosis to first complete remission longer than 65 days (p=0.02), and centre (p=0.013) for LFS, and age over 43 years (p=0.023), time from first complete remission to transplantation of longer than 93 days (p=0.03), and centre (p=0.001) for TRM. Moreover, different centres had different prognostic criteria for good-risk or bad-risk patients indicating that risk factors do not have the same impact in each individual centre. INTERPRETATION: The outcome of bone-marrow transplantation for acute myeloid leukaemia in first complete remission is influenced by the centre in which the procedure is done, even with adjustment for known prognostic risk factors. Significant prognostic factors vary among centres, which means that the relative risk is not the same in each individual centre. However, centres may treat populations with different risks of as yet unidentified prognostic factors. Experience may partly account for the difference in outcome among centres.

Adolescent↗
PubMed1999

A controlled trial comparing ciprofloxacin with mesalazine for the treatment of active Crohn's disease. Groupe d'Etudes Thérapeutiques des Affections Inflammatoires Digestives (GETAID).

OBJECTIVE: The aim of this randomized controlled study was to investigate the efficacy of ciprofloxacin compared with mesalazine in treating active Crohn's disease. METHODS: Patients with a mild to moderate flare-up of Crohn's disease (mean Crohn's Disease Activity Index [CDAI]; 217; range, 160-305) were randomized to receive ciprofloxacin 1 g/day or Pentasa 4 g/day for 6 wk. Complete remission was defined at wk 6 as a CDAI < or = 150 associated with a decrease (delta) in CDAI > 75. Partial remission was defined as a CDAI < or = 150 with 50 < delta CDAI < 75 or a CDAI > 150 with delta CDAI > 50 at wk 6. Group sequential procedure with triangular continuation regions was used to monitor the trial through the difference in complete remission rates, every 20 patients included. RESULTS: Inclusion of patients was stopped at the second step, i.e., after 40 inclusions, with the conclusion of no difference in complete remission rates between ciprofloxacin- and Pentasa-treated groups. Among the 18 patients taking ciprofloxacin, two decided to stop treatment during the trial and three were considered as treatment failures because of deterioration at wk 3. Among the 22 patients taking mesalazine, one patient was lost to follow-up and eight patients were considered as treatment failures. Complete remission was observed in 10 patients (56%) treated with ciprofloxacin and 12 patients (55%) treated with mesalazine and partial remission was observed in three and one patient, respectively. CONCLUSIONS: This study suggests that ciprofloxacin 1 g/day is as effective as mesalazine 4 g/day in treating mild to moderate flare-up of Crohn's disease.

Adult↗
PubMed1998

Role of adhesion molecules in the homing and mobilization of murine hematopoietic stem and progenitor cells.

Bone marrow (BM) transplantation still must overcome multiple difficulties and should benefit from better understanding of stem-cell homing and mobilization. Here, we analyzed the involvement of several adhesion molecules in the two processes by treating mice with monoclonal antibodies against these molecules. Treatment of lethally irradiated mice grafted with isogeneic BM cells showed that at least two migration pathways are important for stem-cell homing to the BM, whereas only one of them is involved in lodging of colony-forming unit-spleen (CFU-S) in the spleen. We confirm that the VLA-4/VCAM-1 adhesion pathway is important for stem-cell homing to the BM only and show that CD44 is involved in CFU-S lodging in both BM and spleen. These results show that entry of CFU-S into the spleen is regulated. The observation that when one migration pathway is altered, CFU-S do not enter the BM via the other pathway may indicate that the two mechanisms involved in CFU-S homing into the BM are linked. The adhesion molecules VLA-4 and CD44 are also implied in the mobilization of stem cells into the blood stream of mice injected once with anti-VLA-4 or anti-CD44. Anti-VLA-4 administration led to a significant increase in circulating stem cells as early as 8 hours after treatment. Stem cells mobilized by anti-VLA-4 comprise cells with high self-renewal potential and thus may be used for long-term reconstitution of the hematopoietic tissue.

Animals↗
PubMed1998

Global and partial agreement among several observers.

The advantage of modelling agreement on a categorical scale among observers rather than using summarizing indices is now well established. However, analysis of agreement among more than two observers is essentially based on pairs of observers. We present a global and partial agreement modelling approach derived from the quasi-independence and quasi-symmetry log-linear models. This approach addresses high order interactions in the contingency table rather than two-way interaction in the pairwise agreement approach. Pairwise, global and partial agreement models were applied to the detection by six pathologists of three lesions in biopsy specimens arising from patients suspected to be affected by Crohn's disease. The global and partial agreement approach surpasses the pairwise agreement approach, especially if there is heterogeneity among ratings.

Biopsy↗
PubMed1997

A model to standardise mortality of severely malnourished children using nutritional status on admission to therapeutic feeding centres.

OBJECTIVE: To determine a simple model to calculate the number of deaths which could be expected in a therapeutic feeding centre from the height, weight and oedema of children on admission. DESIGN: Admission weight, height, presence of oedema of the children and outcome were prospectively recorded. SETTING: Data were recorded in 18 feeding centres set-up during emergency operations in Africa. Ten of the feeding centres were selected, a priori, as reference centres and eight centres as test centres. SUBJECTS: Data for 3858 children were recorded. 837 children absconded from the centres and were excluded from the analysis. Analysis was performed on data from 2753 children who left the centre after recovery and 268 children who died during treatment. INTERVENTIONS: The relation between the risk of death and, anthropometric measurements and presence of oedema has been determined in a previous paper. The maximum likelihood estimate of the constant of the model was determined from global analysis of the data of the reference centres. The model was applied to the data of the reference and test centres. RESULTS: The model to predict the individual probability of death was: P(death) = 1/(1 + exp[-(20.63 - 9.99 1n(weight/height1.74) + 1.36 oedema)]) The predicted number of deaths was close to the recorded number of deaths for each reference centre. For three of the eight test centres there was a significant excess of observed deaths over predicted. CONCLUSION: This model can be easily used by the supervisor of a centre to assess the expected number of deaths during treatment of malnutrition from simple measurements on children that are routinely taken on admission and thus help to determine the nature of variation in observed mortality rates.

Africa↗
PubMed1997

Anal lesions: any significant prognosis in Crohn's disease?

AIM: Work-up of anoperineal lesions usually includes indices of clinical activity as well as diagnostic criteria of Crohn's disease but their prognostic implication remains unclear. This prospective study was conducted in order to evaluate the overall incidence of anoperineal lesions and their relation to the natural history of underlying intestinal Crohn's disease with special reference to the steroid-dependent state of the patients. PATIENTS AND METHODS: One hundred and one patients (46 males, 55 females, aged 34 +/- 14 years; range: 15-79) were consecutively referred to our institution (May 1991 to May 1994) for intestinal symptoms related to Crohn's disease (mean duration 66 +/- 66 months). They all underwent a proctological examination regardless of perineal symptoms. The Cardiff classification was used to describe anoperineal lesions. Patients with anal lesions (64) differed from those without (37): male predisposition (53% vs. 32%, P<0.05), more frequent rectal involvement (75% vs. 24%, P<0.001) and more acute lesions observed at proctoscopic examination (42% vs. 16%, P<0.05). Age of onset, surgical past history of Crohn's disease, colonic or ileal involvement, or Harvey-Bradshaw score were not different between groups. RESULTS: Patients with anal ulceration (43) as compared to patients having anal involvement without ulceration experienced pain more frequently (constant pain: 56 vs. 14%; defecatory pain: 35 vs. 19%) and a more severe evolution of intestinal (40 vs. 22%, P<0.05) and anal (42 vs. 12%, P<0.05) involvement. In those with an aggressive ulceration (U2, 28 patients), daily stool frequency (5.1 +/- 3 vs. 3.6 +/- 2.5, P<0.05) and clinical score (9 +/- 5 vs. 7 +/- 3) were more pronounced. Steroid therapy dependency occurred more frequently in the group with anal ulceration (35 vs. 16% and 40 vs. 17%, respectively, P<0.05). Similar associations were observed for cases of anal involvement (34 vs. 5%, P<0.01) and azathioprine was more frequently required (39 vs. 5%, P<0.01) than in those free of anal lesions. During follow-up, eight other patients required azathioprine (steroid dependence in six) and seven of them had anal lesions at referral. At the endpoint of the study, one out of two patients with anal lesions required azathioprine most often due to steroid dependency of the intestinal involvement (30/64 vs. 4/37, P<0.005). CONCLUSION: Anal ulcerations are a reliable severity index of Crohn's disease in both short- and long-term prognosis but their link to the steroid status of the intestinal disease remains unclear.

Adolescent↗
PubMed1997

A high serum concentration of interleukin-6 is predictive of relapse in quiescent Crohn's disease.

BACKGROUND/AIMS: Relapses of Crohn's disease are difficult to predict. We assessed the value of serum level of interleukin-6, tumour necrosis factor alpha (TNF-alpha) and soluble TNF receptors as predictors of relapse in quiescent Crohn's disease. PATIENTS/METHODS: Thirty-six patients with inactive Crohn's disease, treated or not, were included. Various clinical and biological parameters, including interleukin-6, TNF-alpha and soluble TNF receptors serum levels were measured at inclusion in the study and the patients were followed clinically for 1 year. The relapse was defined as a Crohn's Disease Activity Index (CDAI) greater than 150 with an increase greater than 100 compared to the inclusion value. We analysed the ability of these parameters to predict relapse in parallel to clinical characteristics and other laboratory parameters. RESULTS: Among the 32 variables tested, interleukin-6 serum level had the greatest ability to predict the time-to-relapse, with 17-fold chance of relapse over a 1-year period for patients with an interleukin-6 serum level greater than 20 pg/ml than for patients with a lower level (P < 0.001). A high serum level of the soluble TNF receptors p55 and p75 also had significant predictive value, in contrast to TNF-alpha serum levels. An interleukin-6 serum level greater than 20 pg/ml and either an acid alpha-1-glycoprotein level greater than 1.1 g/l or a soluble interleukin-2 receptor serum level greater than 95 pM/l were risk factors selected by a stepwise multivariate analysis. In both models a good prognosis group was defined by the absence of the two risk factors, a bad prognostic group by the presence of the two risk factors and an intermediate in between. With both models, the good prognosis group included 17 patients who experienced no relapse over the 1-year follow-up, whereas all patients (seven with the first model and six with the second) in the bad prognosis group had a relapse during the follow-up. Looking specifically at two homogeneous subgroups including either naturally/5-aminosalicylic acid (5-ASA) quiescent or corticoid quiescent patients, a very good predictive value for interleukin-6 serum concentration was also found. CONCLUSION: Interleukin-6 serum level alone or in association with other biological parameters such as acid alpha-1-glycoprotein or the soluble interleukin-2 receptor serum level may be useful for predicting the course of the disease in patients with quiescent Crohn's disease.

Adolescent↗
PubMed1996

Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology.

BACKGROUND: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired disorder of haematopoietic stem cells. Although knowledge about the pathophysiology of the disease is increasing, no multivariate analysis of factors influencing survival has been undertaken, mainly because the disease is rare. We undertook such an investigation. METHODS: Data were collected on 220 patients with PNH diagnosed over a 46-year period (1950-1995) from participating French centres. Diagnosis of the disease required, at least, an unequivocally positive Ham's test. FINDINGS: The Kaplan-Meier survival estimate was 65% (SE 4) at 10 years and 48% (6) at 15 years after diagnosis. 8-year cumulative incidence rates of the main complications (pancytopenia, thrombosis, and myelodysplastic syndrome) were 15% (3), 28% (4), and 5% (2), respectively. Demographic data, presenting features, initial treatment, complications, and causes of death were similar to those previously reported. In multivariate analysis, seven factors were significantly associated with survival in patients with PNH. Poor survival was associated with the occurrence of thrombosis as a complication (relative risk 10.2 [95% CI 6-17], p < 0.0001), evolution to pancytopenia (5.5 [2.8-11], p < 0.0001), myelodysplastic syndrome or acute leukaemia (19.1 [7.3-50], p < 0.001), age over 55 years at diagnosis (4 [2.4-6.9], p < 0.0001), need for additional treatment (2.1 [1.3-3.6], p < 0.003), and thrombocytopenia at diagnosis (2.2 [1.3-3.8, p < 0.003). Better survival was shown for patients in whom aplastic anaemia antedated PNH (0.32 [0.14-0.72], p < 0.02). Factors associated in multivariate analysis with a high risk of thrombosis during the disease course were thrombosis at diagnosis (5.1 [2.5-10.6], p = 0.0002), age over 54 years (2.6 [1.5-4.6, p = 0.0014), and infection at diagnosis (2.6 [1.3-5.2], p = 0.0099). The risk factors for progression to pancytopenia were absence at diagnosis of anaemia (4.03 [1.3-12.2], p = 0.03) and neutropenia (2.45 [1.1-5.7], p = 0.03). The risk factors for development of myelodysplastic syndrome or acute leukaemia were abdominal pain crisis at presentation (10.5 [2.5-44.0], p = 0.004) and year of diagnosis after 1983 (8.45 [1.8-40.7], p = 0.004). INTERPRETATION: This large number of cases permitted a detailed analysis of prognostic factors for the first time, in this rare disease. Estimates of PNH prognostic factors may serve as baseline data in the assessment of current and future treatments for this disease.

Adolescent↗
PubMed1996

Comparison of weight- and height-based indices for assessing the risk of death in severely malnourished children.

To compare the effectiveness of treating malnourished children in different centers, the authors believe there is a need to have a simple method of adjusting mortality rates so that differences in the nutritional status of the children are taken into account. The authors compared different anthropometric indices based on weight and height to predict the risk of death among severely malnourished children. Anthropometric data from 1,047 children who survived were compared with those of 147 children who died during treatment in therapeutic feeding centers set up in African countries in 1993. The optimal ratio of weight to height determined by logistic regression was weight (kg)/height (m)1.74 (95% confidence interval of beta estimate 1.65-1.84). The receiver operating curves (sensitivity vs. specificity) showed that the body mass index (weight (kg)/height (m)2), optimal ratio of weight to height, and weight/height index expressed as the percentage of the median of the National Center for Health Statistics' standard were equivalent and superior to the weight/height index expressed as the z score of the National Center for Health Statistics' standard to predict death. As the optimal ratio of weight to height is easier to calculate than the weight/height index expressed as the percentage of the median or z score and does not depend upon either standards or tables, the optimal ratio of weight to height could be conveniently used to adjust mortality rates for nutritional status in therapeutic feeding centers.

Body Height↗