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Biomedical subjects

N Maurin

Publications and source records attributed to N Maurin.

At least 19 recordsLinked to original sources
PubMed2001

[Evaluation of 47,213 infants in neonatal screening for cystic fibrosis, using pancreatitis-associated protein and immunoreactive trypsinogen assays].

OBJECTIVES: The increasing evidence of the benefits of neonatal screening for cystic fibrosis (CF) indicates that this procedure could soon be implemented throughout France. The screening strategy currently used involves the detection of infants with elevated levels of immunoreactive trypsinogen (IRT) (approximately 1% of the population), followed by the detection of CFTR gene mutations. However, genetic analysis has certain drawbacks, the most important of which being the management of heterozygotes, and in France the requirement by law of previous informed consent. In cases of CF, pancreatic alterations are already present in utero. A previous study has demonstrated the value of pancreatitis-associated protein (PAP) as a screening test for CF, and has indicated that a feasible two-stage strategy could involve the following: 1) selection of infants with elevated PAP levels; 2) in this group of infants, subsequent detection of those with elevated IRT levels for direct CF diagnosis by the sweat test thereby avoiding the use of genetic analysis. The study aim was to evaluate this strategy in a large number of neonates. METHODS AND RESULTS: The aforementioned strategy was evaluated in a prospective study involving 47,213 infants in the Provence region of France. In infants with a PAP > 7.5 ng/mL (1.28%), 176 had an elevated IRT level > 700 ng/mL (0.37%). In this limited population sample (0.37% of the total), the sweat test diagnosed five cases of CF. A sixth case involving the monozygous twin of an infant with diagnosed CF remained undetected, probably because of a registration error. Genetic analysis confirmed the diagnosis, and also detected another case in an infant with two CFTR mutations but with a normal phenotype at 20 months of age. As the observed incidence was similar to that which had previously been reported, and as no further case was subsequently detected two years after the end of the study, this indicated that the sensitivity of this screening strategy was satisfactory. Its specificity makes the direct diagnosis of CF cases by the sweat test feasible, without further selection by genetic analysis. CONCLUSION: The PAP/IRT technique for CF detection seems to be suitable for mass screening, without the drawbacks of genetic testing.

Acute-Phase Proteins↗
PubMed1999

[Rational hemostatic screening].

BACKGROUND: In order to exclude hemorrhagic diathesis, e.g. before diagnostic measures carrying the risk of bleeding or in preoperative situations, a graded screening is advisable. PROCEDURE: During the first stage, besides the anamnesis, clinical examination and classification of relevant concomitant diseases (e.g. liver cirrhosis or renal insufficiency), basic laboratory examinations such as prothrombin time, activated partial thromboplastin time (aPTT) and platelet count must be carried out. Should all these measures produce no noteworthy results, no further examinations are necessary. However, in the case of test results within normal limits accompanied by an unsatisfactory anamnesis and/or conspicuous clinical findings, the second stage should include examination of bleeding time according to Mielke to exclude a relevant platelet dysfunction. Should this be inconspicuous a third stage should follow in which successive implementation is made of fibrinogen according to Clauss, the Rumpel-Leede test (to exclude heightened capillary fragility), factor XIII and alpha 2-antiplasmin. The methodical snares of the parameters mentioned will be explained in full.

Blood Coagulation Tests↗
PubMed1999

Severe gastrointestinal hemorrhage due to Mycobacterium avium complex in a patient receiving immunosuppressive therapy.

Intense immunosuppressive therapy is used frequently for treatment of systemic vasculitides, collagenoses, rapidly progressive glomerulonephritis, and after organ transplantation. Numerous serious treatment-related side effects include localized or disseminated opportunistic infections, and require careful monitoring of immunosuppressed patients. Gastrointestinal infections with Mycobacterium avium complex (MAC) or other nontuberculous mycobacteria have been previously identified in HIV seropositive patients only. We now report the first case of an HIV seronegative patient who received immunosuppressive therapy for rapidly progressive glomerulonephritis. The patient presented with severe lower gastrointestinal bleeding and was diagnosed to have ulcerative colitis due to infection with MAC. The patient recovered promptly after administration of antimycobacterial therapy. MAC infection should be included in the differential diagnosis of gastrointestinal bleeding in all immunodeficient patients. The significance of repeated colonoscopy to obtain multiple biopsy specimens with histological examination for foam cells and specific staining for acid-fast organisms is emphasized.

Colitis, Ulcerative↗
PubMed1999

Blood concentrations of pancreatitis associated protein in neonates: relevance to neonatal screening for cystic fibrosis.

AIM: To determine whether pancreatitis associated protein (PAP) is a marker for cystic fibrosis which could be used in neonatal screening for the disease. METHODS: PAP was assayed on screening cards from 202,807 neonates. Babies with PAP > or = 15 ng/ml, or > or = 11.5 ng/ml and immunoreactive trypsinogen (IRT) > or = 700 ng/ml were recalled for clinical examination, sweat testing, and cystic fibrosis transmembrane regulator (CFTR) gene analysis. RESULTS: Median PAP value was 2.8 ng/ml. Forty four cases of cystic fibrosis were recorded. Recalled neonates (n = 398) included only 11 carriers. A receiver operating characteristic curve analysis showed that PAP above 8.0 ng/ml would select 0.76% of babies, including all those with cystic fibrosis, except for one with meconium ileus and two with mild CFTR mutations. Screening 27,146 babies with both PAP and IRT showed that only 0.12% had PAP > 8.0 ng/ml and IRT > 700 ng/ml, including all cases of cystic fibrosis. CONCLUSION: PAP is increased in most neonates with cystic fibrosis and could be used for CF screening. Its combination with IRT looks promising.

Acute-Phase Proteins↗
PubMed1998

Platelet function deficiency in Duchenne muscular dystrophy.

Platelet function and plasmatic coagulation of 51 patients with Duchenne muscular dystrophy (DMD) were studied in order to find an explanation for the known substantial blood loss in spinal surgery of these very patients. A normal plasmatic coagulation and a slight but not significant increase of bleeding time was found. However, a significant deficiency of platelet adhesion and ristocetin induced aggregation (P < 0.001) as well as a marked reduction of expression of glycoprotein i.v. (synonyms: GPIV, GPIIIb, CD36) to 50% were detected. We conclude from our study, that this finding of deficiency of platelet function in DMD patients is of no importance in everyday life and minor operations, e.g. lower limb surgery. In major spinal surgery, however, the platelet function deficiency occurs. A decompensation of platelet adhesion as well as aggregation capacity can be assumed due to the unavoidable intraoperative dilution effect by the inevitable volume replacement.

Adolescent↗
PubMed1998

Hemostasis activation markers in acute renal failure.

Fibrinopeptide A and thrombin-antithrombin III complex were used respectively as markers for in vivo thrombin formation and beta-thromboglobulin as a marker for platelet activation. In cases of acute renal failure (ARF) a heightened plasma concentration in the hemostasis activation markers may occur, because of a renal elimination disturbance, without a previous activation of the hemostasis. In order to check the validity of fibrinopeptide A, thrombin-antithrombin III complex and beta-thromboglobulin as markers for the hemostasis activation in cases of ARF we examined 32 patients prior to renal replacement therapy. A significant rise in fibrinopeptide A (x +/- SD: 34 +/- 22 ng/mL, ref < 3.0), thrombin-antithrombin III complex (19 +/- 15 ng/mL, ref 1.0-4.0) and beta-thromboglobulin (149 +/- 58 U/mL, ref 10-40) was found. None of the parameters examined showed a correlation to the serum creatinine. A correlation was observed respectively between fibrinopeptide A (r = 0.34, p < .05), beta-thromboglobulin (r = 0.39, p < .05) and the beta-thromboglobulin/creatinine coefficient (0.50 +/- 0.30, r = 0.72, p < .001) on the one side and the thrombin-antithrombin III complex on the other. A greater rise in the concentration of all parameters in patients with disseminated intravascular coagulation (DIC) was established, in contrast to patients without DIC (fibrinopeptide A: 44 +/- 31 vs. 32 +/- 20 ng/mL, beta-thromboglobulin: 169 +/- 57 vs. 144 +/- 60 U/mL, thrombin-antithrombin III complex 40 +/- 21 vs. 14 +/- 7 ng/mL, p < .05). Fibrinopeptide A and beta-thromboglobulin/creatinine coefficient in combination with the thrombin-antithrombin III complex can be employed as markers for the activation of hemostasis in cases of ARF there is no direct relationship between restricted kidney function in ARF and the plasma concentration of these markers, which behave similarly in spite of their varying elimination patterns.

Acute Kidney Injury↗
PubMed1997

An in vivo study of the biodegradation of the hydrophilic Mitrathane.

The present study analyzes the kinetics of the in vivo degradation of hydrophilic Mitrathane in the peritoneal cavity of mice over a period ranging from 1 to 180 days. The mechanical milling of the polyurethane films produced regularly flattened fragments that in vivo spontaneously oriented into piles. The morphological observations and analysis with the aid of an image analysis system demonstrated that after seven days of swelling the polymer fragments undergo a continuous degradation that leads to an irregular thinning and phagocytosis of the smaller fragments by macrophages with very little chronic inflammation response from surrounding tissues.

Animals↗
PubMed1996

[Pregnancy and the child of a mother with phenylketonuria].

Pregnant women with hyperphenylalaninemia are at high risk of spontaneous abortion and of giving birth to infants with congenital malformations, microcephaly and mental defect. Among mothers whose phenylalaninemia is greater than 1200 mumol/L (20 mg/100 mL), 95% have at least one child with mental retardation. A low phenylalanine diet with a good control of phenylalaninemia, started before conception, reduces this risk, better results being obtained when plasma phenylalanine levels are maintained below 360 mumol/L (6 mg/100 mL) as compared with levels maintained between 360 to 600 mumol/L (6-10 mg/100 mL). Thus, systematic contraception and planned pregnancies must be recommended in all hyperphenylalanemic young women. This implies early information of phenylketonuric teenage girls and their parents. In addition, efforts must be made to join and inform all women having had hyperphenylalaninemia at birth, whether they received a dietary treatment or not. It is also important that general practitioners, pediatricians and obstetricians be aware of the high recurrence risk in hyperphenylalanemic women who gave birth to a microcephalic or malformed infant.

Female↗
PubMed1996

Hemostatic alterations during continuous venovenous hemofiltration in acute renal failure.

In order to determine changes in hemostasis occurring during continuous venovenous hemofiltration (CVVH), we made a prospective study of 14 patients with acute renal failure. Fibrinopeptide A, thrombin-antithrombin III complex, beta-thromboglobulin and platelet retention were determined serially. Fibrinopeptide A (x +/- SD: 33 +/- 20 ng/ml, ref. < 3.0) and thrombin-antithrombin III complex (11 +/- 5 ng/ml, ref. 1.0-4.0) were enhanced prior to commencement of treatment but showed no further increase during therapy. Platelet retention (Hellem II, ref. 60-99%) fell from 39 +/- 32% before treatment to 16 +/- 15% after treatment, while the beta-thromboglobulin/creatinine ratio (ref. 0.23-0.41) rose from 0.39 +/- 0.20 to 0.64 +/- 0.44. Via platelet activation, CVVH leads to a reinforcement of the existing platelet dysfunction (thrombocytopathy), without influencing plasmatic coagulation. In order to analyze the influence of pre-existing hemostatic alterations on filter running time during CVVH, 60 patients were examined retrospectively in a second study. Filter running time, global coagulation tests, fibrinogen, antithrombin III, platelet count and hematocrit were registered daily. There was no significant correlation between filter running time and fibrinogen concentration, thrombin time, platelet count or hematocrit. Apart from filter occlusion, no thrombotic complications were observed. The frequency of filter occlusion increased with falling activated clotting time (ACT) (p < 0.05). Rising platelet count led to an increase in heparin dose (p < 0.05), primarily due to the anti-heparin effect of platelet factor 4.

Acute Kidney Injury↗
PubMed1995

[Primary hyperparathyroidism in the 3d pregnancy trimester].

HISTORY AND FINDINGS: A 24-year-old pregnant woman had to be hospitalized in the 33rd week of pregnancy because of premature contractions and clinical signs of pyelonephritis. She had a history of nephrolithiasis. Laboratory tests showed a total calcium of 3.6 mmol/l, hypophosphataemia of 0.59 mmol/l and an increased parathormone level of 420 ng/l. Ultrasonography demonstrated a large parathyroid adenoma, confirming the diagnosis of primary hyperparathyroidism. COURSE AND TREATMENT: Despite several recommendations of conservative treatment in the literature it was decided to perform a parathyroidectomy, which was done without complication during the 35th week of pregnancy. A healthy, mature boy was born at the beginning ot the 41st week: at no time did he show any signs of hypoparathyroidism. CONCLUSIONS: Surgical treatment of hyperparathyroidism is a reasonable and possible choice even in the 3rd trimester, because it allows regeneration of the fetal parathyroid. However, this decision must be individualized, in relation to the period of pregnancy and any progression of the disease.

Adenoma↗
PubMed1995

Delayed in vitro immune response to long-term intraperitoneal polymer implant in mice.

This paper presents an original experimental procedure designed to evaluate, by a two step in vivo/in vitro combination, the long-term biocompatibility of synthetic materials developed for clinical applications. The efficacy of the test is illustrated by the experimental results obtained with various materials currently used in medicine (Dacron, RCH) and also with three recently synthesized compounds. In the first step of the procedure, the sample was grafted for at least 5 months in the peritoneal cavity of mice, allowing the possible development of delayed sensitization. In the second step, this sensitization was detected by an in vitro test, the Lymphoblastic Transformation Test (LTT), adapted for this new purpose. On the basis of this experiment, the 5 polymers tested were classified into 2 groups: one group did not significantly differ from the control, and the other had a significantly higher incidence of sensitization. As these results were in agreement with the known biocompatibility of these polymers, they demonstrate the validity of the test to classify new polymers.

Animals↗