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1.
Arch Pediatr ; 18(7): 745-9, 2011 Jul.
Article in French | MEDLINE | ID: mdl-21596536

ABSTRACT

BACKGROUND: Thymoma is an uncommon tumor of childhood. It is an anterior mediastinal tumor with few symptoms. The pleura is the most frequent metastatic site. Surgery is the treatment of choice and the most important prognostic factors are the stage at initial presentation and whether complete resection can be performed. CASE REPORT: A 6-year-old girl with no pathological antecedent presented with a history of prolonged fever. Frontal chest radiography showed a large mediastinal soft tissue mass with sharp lateral and inferior margins. Computed tomography showed a large anterosuperior and medium mediastinal mass measuring 83 mm × 70 mm, associated with variable size mediastinal adenopathy, suggesting the diagnosis of lymphoma. Histological examination of bone marrow biopsy found no haematological malignant diseases such as lymphoma. The histological examination of computed tomography (CT)-guided transthoracic biopsy demonstrated proliferation of polyclonal lymphocyte T cells, confirming the diagnosis of thymoma. The patient benefited from induction chemotherapy (cisplatin and VP16) followed by surgery with complete resection. The patient is doing well 24 months after resection and has no signs of recurrence; CT follow-up showed a stable size of residual thymus. CONCLUSION: Thymoma is a benign tumor but the significant risk of recurrence warrants long follow-up.


Subject(s)
Thymoma/diagnosis , Thymus Neoplasms/diagnosis , Biopsy , Chemotherapy, Adjuvant , Child , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Thymectomy , Thymoma/drug therapy , Thymoma/pathology , Thymoma/surgery , Thymus Gland/pathology , Thymus Neoplasms/drug therapy , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery , Tomography, X-Ray Computed
2.
Arch Pediatr ; 17(8): 1156-8, 2010 Aug.
Article in French | MEDLINE | ID: mdl-20542671

ABSTRACT

Cavernous malformations are vascular lesions of the central nervous system. They are very rare in childhood. We report the case of sporadic cavernous cerebral angioma in a 1-year-old girl without pathologic antecedents revealed by a partial seizure. With this case and a review of the literature, we show the clinical and therapeutic characteristics of cerebral cavernomatosis in childhood.


Subject(s)
Brain Neoplasms/diagnosis , Hemangioma, Cavernous/diagnosis , Valproic Acid/therapeutic use , Brain Neoplasms/drug therapy , Brain Neoplasms/pathology , Female , Hemangioma, Cavernous/drug therapy , Hemangioma, Cavernous/pathology , Humans , Infant , Magnetic Resonance Imaging , Seizures/etiology , Treatment Outcome
3.
Arch Pediatr ; 17(7): 1062-4, 2010 Jul.
Article in French | MEDLINE | ID: mdl-20570498

ABSTRACT

Glanzmann thrombasthenia (TG) is a congenital platelet function disorder characterized by frequent and occasionally severe bleeding events. Treatment is based on platelet transfusion at the time of bleeding. We report a case of GT revealed in the neonatal period, a severe hemorrhagic syndrome refractory to transfusions, treated at the age of 6 years. Activated recombinant factor VII (Novoseven) injections were necessary. The advantages of recombinant activated factor VII in GT patients with platelet antibodies and/or platelet transfusions refractoriness are discussed.


Subject(s)
Factor VIIa/therapeutic use , Hemostatics/therapeutic use , Thrombasthenia/drug therapy , Child , Humans , Male , Recombinant Proteins/therapeutic use
4.
Arch Pediatr ; 14(11): 1337-40, 2007 Nov.
Article in French | MEDLINE | ID: mdl-17942291

ABSTRACT

UNLABELLED: Devic neuromyelitis optica (NMO) or Devic's syndrome is an uncommon clinical syndrome associating unilateral or bilateral optic neuritis and transverse myelitis. Usually reported in adults, childhood cases constitute a distinctive clinical entity. CASE REPORT: We report a case of NMO occurring in a 9-year-old girl, admitted for paraplegia, sphincter troubles as acute installation bladder retention and of a sudden decline of the visual acuity. Magnetic resonance imaging (MRI) revealed abnormalities of spinal cord signal with hypo intensity in T1-weighted images and hyper intensity in T2-weighted images along the spinal cord. However, the cerebral region was normal. Visual evoked potentials were consistent with retrobulbar optic neuropathy. Our patient received corticosteroids (methyl prednisolone) during 5 days followed by oral prednisone. At week three, an immunosuppressant (azathioprine) was added. Clinical outcome was favourable with disappearance of sphincter troubles, a correction of the visual acuity and a progressive disappearance of motor troubles. CONCLUSION: Pediatric Devic's NMO is rare. It is a different clinical entity with an excellent visual and neurological prognosis. Review of the literature shows that recurrence is rare in children and seems to be without long-term sequelae with corticosteroids and immunosuppressant therapy.


Subject(s)
Neuromyelitis Optica/complications , Azathioprine/therapeutic use , Child , Evoked Potentials, Visual/physiology , Female , Glucocorticoids/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Magnetic Resonance Imaging , Neuromyelitis Optica/drug therapy , Paraplegia/complications , Prednisolone/analogs & derivatives , Prednisolone/therapeutic use , Spinal Cord/abnormalities , Spinal Cord/pathology , Urinary Retention/complications , Visual Acuity/physiology
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