Search PubMed⌕ Search

Biomedical subjects

C Galambrun

Publications and source records attributed to C Galambrun.

26 records · Page 2Linked to original sources

Myelofibrosis: an unusual presentation of vitamin D-deficient rickets.

UNLABELLED: We present the case of a breast-fed 5-month-old infant who presented with pancytopenia, secondary to intense myelofibrosis during the winter months because of undiagnosed rickets. The patient responded to oral vitamin D with rapid resolution of symptoms. Secondary hyperparathyroidism was the probable cause of the myelofibrosis. CONCLUSION: Although nutritional rickets remains a problem in developing countries, children in northern climates in industrialized countries may also be at risk. Rickets must be considered when assessing myelofibrosis in a very young child.

Humans↗

[Interest of gene amplification by PCR for the diagnosis of Mycoplasma pneumoniae infections in the child].

Between december 1996 and february 1998, rhinopharyngeal and tracheal aspirates from 165 children exhibiting symptoms compatible with M. pneumoniae infection were tested by a PCR method using in the same tube primers specific for M. pneumoniae P1 adhesin gene and for a human control gene. The positive cases were controlled using culture and/or serology. PCR was positive in 22 out of 165 samples (13.3%); an evaluation of the clinical and biological data was possible in 20 of these infected children. From 17 PCR positive respiratory samples tested by culture, 13 (76.5%) grew M. pneumoniae. From 14 serum specimens tested by ELISA, 12 exhibited specific IgM (3 of them with low titers); cold agglutinins were detected in all 7 tested sera. Only one case was not confirmed by any of the 3 former markers. The mean age of patients was 8.1 years. The main clinical symptoms included fever > 38 degrees C, cough, clinical and radiological pneumonia in 90, 95, 50 and 85% of cases, respectively. Neurological symptoms were the main clinical manifestation in 3 patients; another child exhibited a pneumonia associated to an hemophagocytic syndrome and a bone marrow failure which needed a graft. These results emphasize the value of PCR for the rapid diagnosis of M. pneumoniae infection in children.

Adhesins, Bacterial↗

Pemphigus foliaceus.

Pemphigus foliaceus is a skin disease in which antibodies against the cell surface of keratinocytes destroy the adhesion between epidermal cells, thereby producing blisters. It is a rare disease in childhood, and treatment guidelines for juvenile pemphigus foliaceus are lacking. An 8 year old boy with pemphigus foliaceus is described. He did not respond to topical steroids, and the condition flared up when high dose oral steroids were tapered. The lesions resolved completely in four weeks on dapsone, which was maintained for nine months with no major adverse effects, except for a moderate increase of the methaemoglobin concentration at the outset of treatment. There has been no evidence of disease reactivation in more than nine months of follow up since dapsone withdrawal.

Child↗

[Lymphohistiocyte activation syndromes associated with Epstein-Barr virus].

Lymphohistiocyte activation syndromes associated with the Epstein-Barr virus and characterized by activation and proliferation of T-cells and macrophages reflecting an inappropriate response of the host to the viral infection. The clinical manifestations are often alarming symptoms suggestive of severe sepsis. Most patients have a known underlying disease (hemopathy, lupus, Still's disease, HIV infection, bone marrow or organ transplantation). In the few cases that occur in the apparent absence of any risk factors, investigations should be done to look for a predisposing inherited disease, such as familial lymphohistiocytosis of Purtilo syndrome in boys. The treatment rests on immunosuppressive agents followed by bone marrow transplantation, which can provide a definitive cure in genetic forms.

Female↗

[Anticardiolipin antibodies, cerebral ischemia and adrenal hemorrhage in a newborn infant].

BACKGROUND: Anticardiolipin antibodies, usually associated with vascular thrombosis in systemic lupus erythematosus, have been recently found associated with strokes in childhood, and acute adrenal hemorrhage in the adult. CASE REPORT: A 3 day-old fullterm newborn suffering from cerebrovascular ischemia and bilateral massive hemorrhage was found to have anticardiolipin antibodies detected during the neonatal period and 7 months later. There was no evidence of pathology in the mother or perinatal asphyxia. However, anticardiolipin antibodies were present in the mother 5 and 15 weeks after delivery. CONCLUSION: Association of anticardiolipin antibodies and vascular thrombosis has never been reported in the neonate. Persistence of such antibodies favor the hypothesis that they are not transmitted by the mother; they could represent an early manifestation of primary antiphospholipid syndrome.

Adrenal Gland Diseases↗

Aplastic anemia after Mycoplasma pneumoniae infection: a report of two cases.

Two very unusual cases of aplastic anemia complicating mycoplasma infection are described. Each patient had preexisting hematologic abnormalities at the time of the infection: reactive hemophagocytic syndrome in one, and autoimmune hemolytic anemia associated with cold autoantibodies in the other. This adds another entity to the protean manifestations of Mycoplasma pneumoniae infection.

Anemia, Aplastic↗

Epstein-Barr virus--positive undifferentiated thymic carcinoma in a 12-year-old white girl.

Thymic epithelial malignant diseases are extremely rare in children. The authors report a 12-year-old white girl admitted for a polymetastatic tumor of the anterior mediastinum. Tumor proliferation was typical of an undifferentiated thymic carcinoma. A close link between Epstein-Barr virus (EBV) and the tumor was established by a high titer of anti-VCA IgA and the presence of EBV RNA and DNA in the tumor. In addition, monoclonal viral episomes were present in tumor cells, indicating that EBV infection was an early event in the oncogenic process. The patient died despite resection, irradiation, and chemotherapy.

Carcinoma↗