Archives francaises de pediatrie最新文献

Background: Mycotic aneurysms are classic complications of infective endocarditis. Their diagnosis can be difficult when they are located in deep vessels.

Case report: A 8 year-old boy was admitted for psoitis, tenderness of the left iliac fossa and a palpable mass. He was treated with antibiotics for 2 months for infective endocarditis, but vomiting and fever began after 3 weeks of therapy. Blood cultures showed Staphylococcus epidermidis. Ultrasonography of the mass showed findings compatible with an abscess but sampling of its contents showed blood. Immediate Doppler echography showed that the mass was an aneurysm of the left iliac artery. This diagnosis was confirmed by CT scan and aortography. The aneurysm was excised and a satisfactory repair was made using a Gore-tex graft.

Conclusion: Although this mass had no clinical vascular characteristics, its origin should have been recognized because of the history of infective endocarditis and a better analysis of the ultrasonographic findings.

Background: Total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac disease. When this includes pulmonary artery hypertension, the infant is severely ill. Survival beyond infancy is rare without surgical correction.

Case report: A girl in whom hydramnios and a short femur were discovered by ultrasonography at the 25th week of gestation was admitted to the intensive care unit a few hours after birth because of respiratory distress. Blood PaO2 was 37 mmHg, PaCO2, 36 mmHg, and pH 7.25. She had tachycardia (190/min) and circulatory insufficiency. Echocardiography showed right ventricular overload, a right-to-left shunt through a patent ductus arteriosus and foramen ovale and tricuspid insufficiency. This refractory hypoxemia was not corrected by conventional respiratory support, high-frequency oscillation plus dobutamine and dopamine followed by noradrenaline infusion. Because of further deterioration, the baby was given extracorporeal lung support; this rapidly improved the respiratory and hemodynamic conditions. Persistent pulmonary artery hypertension led to a second investigation that showed TAPVR She underwent emergency surgery.

Conclusion: Extracorporeal lung support can stabilize a precarious state in a case of severe congenital cardiac disease, so allowing surgical treatment under satisfactory conditions.

Background: Netherton's syndrome is characterized by ichthyosis, trichorrhexis invaginata and other air shaft anomalies, and atopic diathesis. The ichthyosis is present at birth and can be complicated by hypernatremic dehydration.

Case report: Adrien was the first child of non-consanguineous parents. His birth weight was 3,110 g. Ichthyosis was present at birth and the diagnosis of Netherton's syndrome was subsequently confirmed by skin biopsy. He was admitted to intensive care at the age of 4 days for a 20% loss of weight and dehydration. His blood chemistry was: Na+ = 192 mmol/l; K+ = 6.9 mmol/l; Cl- = 136 mmol/l; urea = 21 mmol/l; glucose = 12.1 mmol/l; creatinine = 209 mumol/l; hematocrit = 51%. Urinary analysis gave: Na+ = 113 mmol/l; K+ = 97 mmol/l; urea = 105 mmol/l. Progressive rehydration resulted in normal diuresis 24 hours later and a slow normalization of natremia to 138 mmol/l on day 10. Despite preventive i.v. phenobarbital, he developed convulsions on day 5 when his natremia was 177 mmol/l i.e. after a drop of 0.6 mmol/hour. The convulsions disappeared when he was given phenytoin and placed on respiratory support, but status epilepticus appeared on day 18 with normal natremia. This status epilepticus was not influenced by several antiepileptic drugs and was only cured when the patient was given tetracosactide followed by hydrocortisone plus sodium valproate. Adrien is now 3 years old and is neurologically and mentally normal.

Conclusion: Hypernatremic dehydration can be a complication of neonatal ichthyosis; it may be severe with very high blood sodium concentration. Corticosteroids can be useful for treating persistent convulsions.

Background: Proteus syndrome is characterized by a range of various manifestations. The main ones are partial gigantism of hands and feet, nevi, hemihypertrophy, macrocephaly. Urinary tract abnormalities are exceptional.

Case report: A 6 year-old boy was examined because he had presented numerous abnormalities from birth. His weight was 26 kg (+3 SD) and his height was 135 cm (+4 SD). The main abnormalities were ptosis, pterygium colli, nevi of the cervical area, plagiocephaly, frontal bossing, scoliosis, hemihypertrophy involving the skin, mucosa and bones, macrodactyly, varicose veins and lipomatosis. He underwent surgery at the age of 3 years for urinary lithiasis associated with an ureterovesical reflux on the left side, i.e. the side of hemihypertrophy.

Conclusion: This case suggests that Proteus syndrome may be an example of ectomesodermal dysembryoplasy but it requires confirmation by a report of at least one other case of obstruction of the ureteropelvic junction on the same side as hemihypertrophy.

Background: Hyperpigmentation can be due to different cause or have no apparent cause.

Case report: A 4 year-old boy was examined because pigmentation had appeared 5 months earlier. The cutaneous lesions were blue-grey, macular (diameter: 2-3 cm), with a predilection for the trunk, the lower part of the neck and the proximal part of the legs. There was no pruritus and the lesions did not urticate when rubbed. Histologically, there was epidermal acanthosis, moderate hyperkeratosis and dermal infiltration with numerous melanophages. The clinical lesions remained stable 6 months later.

Conclusion: The cutaneous manifestations and their course are similar to those of idiopathic macular pigmentation usually reported in adolescents. Ultramicroscopic findings have led some authors to classify it as lichen planus.

Background: Pulmonary fibroxanthogranuloma is a benign tumor that is rarely seen in children. Its association with renal amyloidosis is not known.

Case report: A 5 year-old boy developed arthritis in his wrists and his hands became clubbed. X-rays showed a round mass in the left inferior pulmonary lobe. Sonography revealed that this mass contained fluid. Nephrotic syndrome appeared one month later. The pulmonary mass was then excised; histology indicated a fibroxanthogranuloma with no malignancy. Renal biopsy showed infiltration with amyloid substance. The nephrotic syndrome was improved one month after surgery, but contact with the child was then lost.

Conclusion: The association of this tumor with apparently secondary amyloidosis may indicate that fibroxanthogranuloma is a pseudotumor of inflammatory origin.

Background: Behçet's disease is rare in children. The possibility of incomplete forms and the fact that the criteria for its diagnosis are not still agreed upon may explain why it could occur more frequently.

Population and methods: A questionnaire was sent to all 362 French pediatric units. It requested data on possible sufferers from Behçet's disease, including EEG, CSF examination, brain CT scan, coloscopy, HLA groups, age at the first symptom and age at diagnosis. Among the 24 questionnaires concerning a possible case of Behçet's disease, 15 patients satisfied the international criteria for a diagnosis of Behçet's disease.

Results: These 15 patients (10 boys and 5 girls) were Caucasians (n = 9) or Africans (n = 6). The first symptoms were seen at 2 months to 14 years (mean: 10 yrs). The diagnosis was made between 7 and 18 years of age (mean: 11 yrs). HLA antigen B5 was identified in 7 patients and HLA-B12 in 1 patient. The first symptoms were oral ulcers (10 patients) plus skin lesions, genital ulcers and headache. All 15 patients suffered from oral ulcers, 8 from genital ulcers, 12 from skin lesions, 11 from arthritis, 9 from neurologic abnormalities, 6 from abdominal pain and 7 from eye inflammation; 10 patients had a complete form with ocular, neurologic and/or vascular abnormalities while 5 patients had an incomplete form with cutaneo-mucosal and/or GI and/or articular involvement. Two patients had family histories of Behçet's disease.

Conclusions: Behçet's disease is not so exceptional in France. A better knowledge of its symptoms should result in its more frequent diagnosis in childhood.

Background: Kawasaki disease is an acute inflammatory condition characterized by various combinations of features but renal involvement is rare. This report is of a case of Kawasaki disease complicated by acute kidney failure.

Case report: A 10 year-old girl was admitted because of acute renal failure with fever. She developed a high fever, and her general condition was poor; she had developed a macular erythematous rash 10 days earlier for which she was given cefadroxil. At admission, she remained febrile and had strawberry tongue, pharyngitis, dry erythematous lips, bilateral conjunctivitis, cervical lymphadenopathy and desquamation of the skin on her hands. She was anemic (hemoglobin = 9.6 g%), leukocytotic (33,100/mm3), but with no burr, fragmented red blood cells or thrombocytopenia. Her plasma C-reactive protein level was 236 mg/l; her blood urea was 9.5 mmol/l, her creatininemia 288 mumol/l and proteinuria was 0.5 g/l without hematuria. Urine cultures did not grow. Her blood transaminase and gammaglutamyltransferase activities were elevated. Ultrasonography of kidneys and coronary arteries was normal. Kidney biopsy performed one day after admission showed no vascular or glomerular changes, but renal tubular necrosis, indicating urinary excretion of pigments. Tests for myoglobinemia, myoglobinuria and blood muscle enzyme activities were all positive. The renal failure disappeared within 10 days but the fever and inflammatory manifestations persisted for 1 1/2-2 months despite two treatments of intravenous gammaglobulins and continuous salicylate administration. The patient developed arthralgias at the end of the first month of disease, but recovered without renal or vascular complications.

Conclusions: Several cases of renal involvement have been reported during the course of Kawasaki disease. They have been rarely documented by histological examination so that the vascular origin of changes has not been demonstrated. Myoglobinuria, as seen in muscular crush injury, and in our case possibly due to malignant hyperthermia, may be responsible for the transient acute renal failure.