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M Monfort-Gouraud

Publications and source records attributed to M Monfort-Gouraud.

13 recordsLinked to original sources
PubMed2006

[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection].

Recurrent and serious otitis media, and 2 Streptococcus pneumoniae bacteraemia episodes evoked an immune system deficiency in a 6-year-old girl. Upon investigation of the complement system, CH50 activity was moderately reduced and C4 antigen level was normal contrasting with low C3 antigen level. Factor 1 was undetectable. Factor I deficiency is rare, and its diagnosis has important practical consequences. Thanks to preventive antibiotic therapy with penicillin V and vaccinations against Neisseria meningitidis and S. pneumoniae, life expectancy and quality of this child can be significantly improved.

Anti-Bacterial Agents↗
PubMed1999

[Hypertransaminasemia in an adolescent].

CASE REPORT: An adolescent admitted to hospital because of an obvious convulsion seizure presented with a high level of serum macroaspartate aminotransferase. This macroaspartate aminotransferase was discovered by chance when blood tests were made. DISCUSSION: Macroaspartate aminotransferase is a persistent, benign phenomenon, probably not congenital, discovered either in healthy patients, or in adults suffering from malignancies or autoimmune diseases. Macroenzymes have been identified as a cause of benign increase in a number of serum enzymes, like macroamylase serum levels. The macroenzyme is often an immunoglobulin G-complexed enzyme. CONCLUSION: It is important for clinicians to be aware of their existence in order to avoid unnecessary procedures. It is important that the patient is informed of the macroaspartate aminotransferase and that the same is stated in his health record.

Adolescent↗
PubMed1996

[Inflammatory pseudotumor of the orbit and suspected sarcoidosis].

BACKGROUND: Orbital inflammatory lesions may be due to infection, neoplasia, systemic disease such as sarcoidosis or be idiopathic. CASE REPORT: A 13 year-old boy had uveitis and symptoms of unilateral periglobular orbital inflammation, confirmed by computed tomography. Infection and neoplasia were excluded. The serum angiotensin-converting enzyme level (ACE) was normal. Steroids were effective but three recurrences occurred with bilateralisation of the lesion. Serum ACE level increased making sarcoidosis probable. Moreover renal lesion were later found. Outcome was good with a prolonged steroid administration for ten months. CONCLUSIONS: Sarcoidosis may be revealed by an orbital inflammatory lesion. Steroids are necessary to prevent ocular functional complications.

Adolescent↗
PubMed1995

[Transient correction of partial congenital factor V deficiency in nephrotic syndrome].

Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.

Child, Preschool↗
PubMed1993

[Candida meningitis, in a premature infant, treated with liposomal amphotericin B and flucytosine].

BACKGROUND: Candida meningitis in infancy is becoming more common. Its treatment is difficult and may benefit from liposomal amphotericin B. CASE REPORT: A preterm infant developed necrotizing enterocolitis on day 4. Antibiotic therapy included cefotaxime, gentamicin, vancomycin and metronidazole; a central catheter was inserted for nutrition. An acute meningitis developed on day 17 and CT scan showed several brain abscesses. Candida albicans was recovered from the feces, urine and gastric fluid on day 19 and the infant was treated with fluconazole. This drug was replaced by amphotericin B and fluorocytosin when CSF studies a few days later showed persistent meningitis and the presence of Candida albicans. There was no sign of endocarditis. 3 days later, amphotericin B was replaced by liposomal amphotericin B at a dose of 3 mg/kg/day, while the initial catheter was removed. The CSF values and CT scan images gradually improved on this treatment. Liposomal amphotericin B and fluorocytosin treatment was interrupted on day 94, and replaced by oral fluconazole for 5 weeks. These drugs were very well tolerated and further studies at 6 months of age showed that the infant was normal, with no sign of immune deficiency. CONCLUSION: This infant showed several indications of a bad prognosis. But treatment of Candida meningitis liposomal amphotericin B seemed to greatly improve the management of this severe infection.

Amphotericin B↗
PubMed1993

[C2 deficiency discovered in pneumococcal meningitis].

BACKGROUND: Congenital deficiencies of complement system proteins are rare. Patients with C2 deficiency have a high incidence of vascularitis syndromes. Most patients with this deficiency have no problems with increased susceptibility to infection, most commonly due to pneumococci, presumably because of the protective function of the alternative pathway. CASE REPORT: A 22 month-old girl was admitted because of acute meningitis and otitis. She had had 2 episodes of otitis media at the age of 1 year. Analysis of the CSF showed that this meningitis was due to pneumococcal infection. Recovery was complete after 15 days of antibiotic therapy. Total hemolytic complement activity (CH50) was low during the infection; one month later, the CH50 value was about zero as was C2, while C3 and C4 were normal. The patient was given polyvalent pneumococcal and anti-Haemophilus vaccines plus prophylactic penicillin G. Laboratory tests for systemic lupus erythematosus were negative. CONCLUSION: A defect of complement function should be suspected in any patient with severe of recurring pyogenic infections. Complement disorders can be detected one month later by means of the relatively simple hemolytic complement assay.

Complement C2↗
PubMed1993

[A rare cause of hypercalcemia: familial hypocalciuric hypercalcemia].

BACKGROUND: Familial hypocalciuric hypercalcemia is a rare disease with autosomal dominant transmission. Its basic defect is unknown and it requires no treatment. CASE REPORT: A 4 month-old girl was admitted for unexplained crying. She was found to have hypercalcemia (2.8 mmol/l) and later values of blood calcium were 3, 3.1 and 3 mmol/l. The serum free ionic calcium level was also elevated. The serum concentrations of protein, phosphorus, magnesium and the alkaline phosphatase activity were all normal. Serum concentrations of 25-(OH)-D3, 1.25-(OH)-2-D3 and PTH were also normal. The urinary calcium/creatinine ratio was normal and the urinary calcium excretion was 1.08 mg/kg/d. Screening of family members showed hypercalcemia in the father (2.8 mmol/l) and a brother aged 7 years (2.9 mmol/l). Short-term treatment with disodium etidronate lowered the serum calcium level to normal, but hypercalcemia reappeared once the treatment was discontinued. CONCLUSIONS: This asymptomatic familial hypercalcemia has the characteristics of familial hypocalciuric hypercalcemia. There was no associated endocrine disorder. Screening of family members is worthwhile.

Calcium↗
PubMed1993

[Severe megaloblastic anemia in child breast fed by a vegetarian mother].

The case of a 15-month-old, strictly breast-fed infant whose mother had been following a vegetarian diet for ten years is reported. The infant had severe megaloblastic anemia with an arrest in growth, hypotonia, and failure of psychomotor development. The very low levels of vitamin B12 in the infant's serum and mother's milk confirmed the diagnosis. Management of such cases consists in administration of vitamin B12 supplements, with a blood transfusion if needed. Other concomitant deficiencies should be looked for. The outcome is rapidly favorable. The patient reported here is now four years of age and has normal statural growth and psychomotor development.

Anemia, Megaloblastic↗
PubMed1991

[Superficial pseudophlebitis in a HIV seropositive child].

A case of "hyperalgesic pseudothrombophlebitis" in an hemophilic child with acquired immune deficiency syndrome (AIDS) is reported. Known causes such as associated blood clotting defect, collagen vascular disease, infection and homocystinuria were ruled out. Five similar cases were reported in 1985, in homosexual American adult males with AIDS, in whom venography did not show evidence of venous occlusion. Recovery occurred spontaneously and progressively, while the patients were given an anticoagulant therapy. Despite our hemophilic patient did not receive any anticoagulant treatment, a similar favorable course was observed. This AIDS related syndrome has not been reported in children yet, and its pathogenesis remains unknown.

Child↗
PubMed1990

[Delayed measles encephalitis in a leukemic child].

A case of measles encephalitis of the delayed type in a 5 year-old girl is reported. The encephalitis occurred 6 months after a measles which supervened just after the 18th monthly reinduction treatment for acute lymphoblastic leukemia. The child died one month later. This measles inclusion body encephalitis (MIBE) was confirmed by the presence of intracellular inclusions in the brain cells visualized by electron microscopy. The evidence of viral related intracellular nucleocapsides was confirmed by in situ hybridization. These nucleocapsides were identical to those seen in subacute sclerosing panencephalitis.

Child, Preschool↗
PubMed1988

[Infantile polycystic disease. Renal pseudotumoral growth in the postnatal period].

In the most common clinical expression of infantile polycystic disease, the kidneys are greatly enlarged in the neonatal period. When the child gets older the kidneys become smaller. In this case report, both kidneys kept growing after birth causing abdominal distention and respiratory distress. The differential diagnosis was a bilateral massive nephroblastomatosis, but the renal pathology showed characteristic features of infantile polycystic disease.

Genes, Recessive↗