Life-threatening subglottic hemangioma in an infant successfully treated with propranolol.

Subglottic hemangioma is a rare benign tumor in children. It causes an obstruction of the upper airway and can be life-threatening. Several therapeutic options have been used in the literature, including surgery. Currently, the treatment is essentially based on propranolol. We report the case of a 2-month-old female infant hospitalized for severe obstructive dyspnea secondary to a subglottic hemangioma. Diagnosis was confirmed by laryngoscopy and magnetic resonance imaging. The child underwent a tracheotomy and was treated with propranolol. The progression was favorable with regression of the subglottic hemangioma and improvement of laryngotracheal airway obstruction.

Staphylococcal scalded skin syndrome: An uncommon symptomatology revealing an immune deficiency.

Primary immune deficiencies associated with hyper-IgE syndrome are rare diseases with clinical features dominated by recurring cutaneous and visceral bacterial infections, particularly infections due to Staphylococcus species. Most of these infections are associated with milder inflammation compared to normal. We report a primary immune deficiency associated with a hyper-IgE syndrome revealed by a staphylococcal scalded skin syndrome in a 5-year-old girl. The patient presented with a severe staphylococcal infection with extensive skin lesions and disseminated intravascular coagulation. She received intravenous fluids to compensate for fluid losses and anti-staphylococcal antibiotics. Coagulopathy was also corrected. However, the progression was rapidly fatal.

[A rare cause of recurrent meningitis in children: cochleovestibular dysplasia]. / Une cause rare de méningite récurrente chez l'enfant: la dysplasie cochléovestibulaire.

Recurrent bacterial meningitis is an uncommon disease of childhood. It occurs most often in children who have an underlying predisposing disorder that can result from anatomic fistula or immunodeficiency. Cochleovestibular dysplasia is a rare malformation of the inner ear that is often associated with translabyrinthine cerebrospinal fistula and then can cause recurrent bacterial meningitis. We report an unusual case of recurrent meningitis revealing cochleovestibular dysplasia in a 9-year-old child. The malformation was confirmed by imaging and the child had surgery. The outcome was favourable with no recurrence of meningitis during the 3 years after the operation.

[Effects of acute decrease in preload on echocardiographic indices of systolic and diastolic function of the left ventricle in children with end-stage renal disease (ESRD). Comparative study before and after hemodialysis]. / Effets de la réduction de la précharge sur les paramètres échographiques de la fonction systolique et diastolique du ventricule gauche de l'enfant en insuffisance rénale terminale: étude comparative avant et après hémodialyse.

PURPOSE: Contrasting data exist about the hemodialysis induced changes of ventricular diastolic and systolic functions in adults. Few data in children with end-stage renal disease (ESRD) are reported. The aim of the present study was to evaluate the effect of a single hemodialysis (HD) session on left ventricular (LV) systolic and diastolic function using conventional pulsed-Doppler echocardiography and pulsed tissue Doppler imaging (TDI) in hemodialysis children. METHODS: Thirty-five children with chronic renal failure (15 males, aged 12.8+/-3.8 years) on maintenance hemodialysis underwent conventional 2D and Doppler Echo together with measurement of longitudinal mitral annular motion velocities. Echocardiographic parameters were obtained 30 minutes before and 30 minutes after HD. Paired data were compared. RESULTS: Hemodialysis led to reduction in LV end-diastolic volume (p=0.001), end-systolic volume (p=0.05), left atrium area (p<0.0001), peak early (E wave) transmitral flow velocity (p=0.005), peak S velocity of pulmonary vein flow (p=0.002), aortic time velocity integral (p<0.0001) and aortic ejection time (p<0.0001). No significant change in Tei Index was observed after HD. Regarding TDI measures, velocities were not affected by preload reduction. Only the early diastolic velocities on the septal side of the mitral annulus decreased significantly (p=0.001) and the systolic velocities on the lateral side of the mitral annulus increased significantly (p=0.042) after hemodialysis. CONCLUSIONS: Most of Doppler-derived indices of diastolic function are preload-dependent. TDI velocities and Tei Index were not or minimally affected by preload reduction in hemodialysis children.

[Exceptional digestive location of crystal deposits in primary hyperoxaluria]. / Localisation digestive exceptionnelle des dépôts cristallins dans l'hyperoxalurie primitive.

We report 3 cases of primary oxalosis with nephrocalcinosis and severe renal failure. Extrarenal involvement was noted in bones in 3 cases, the heart in 2 cases, the central nervous system in 2 cases, the skin in 1 case and the eye in 1 case. The 3 patients presented with acute digestive disorders. Ultrasonography and CT scans showed digestive wall calcifications in addition to the classic appearance of primary oxalosis such as nephrocalcinosis or bone involvement. Primary hyperoxaluria is characterized by a calcium deposit in different tissues, mainly in kidneys. Digestive wall involvement has never been reported in the literature. Primary oxaluria should be considered in the presence of such a deposit in the gut wall.

[Contribution of leukoconcentration in the diagnosis of Kala-azar in Tunisia]. / Apport de la leucoconcentration dans le diagnostic du Kala-azar en Tunisie.

OBJECTIVE: The authors had for aim to determine the role of leukoconcentration in the diagnosis of visceral leishmaniosis in immunocompetent children. MATERIALS AND METHODS: A study was made on leukoconcentration in blood samples of 84 immunocompetent children presenting with visceral leishmaniosis, hospitalised in the paediatric units of Sousse and Kairouan (Tunisian center) between April 1996 and March 2005. RESULTS: The study group included 34 girls and 50 boys (sex-ratio = 1.47) aged six months to ten years. In this group, 47 patients (56%) presented with positive leukoconcentration. The number of leishmania detected ranged from 1 to 64 per slide; parasitism of PMN leucocytes was noted in nearly half of the cases. CONCLUSION: Parasitemia is frequent in the Mediterranean Kala-azar; therefore leukoconcentration on peripheral blood can be proposed as a first intention examination for the diagnosis of visceral leishmaniosis in immunocompetent children.

[Cerebral hydatid disease: imaging features]. / Hydatidose cérébrale: aspects en imagerie.

Cerebral hytatid cysts (HC) are extremely rare, forming 2% of all intra cranial space occupying lesions even in counties where the disease is endemic. HC diagnosis is usually based on a pathognomonic computed tomography (CT) pattern. In order to assess the value of MR we reviewed the CT (n=25) and magnetic resonance (MR, n=4 including diffusion and proton magnetic resonance spectroscopy in 1) imaging of 25 patients with pathologically confirmed cerebral hydatid disease. 19 HC were seen in children under 16 years. All were supra tentorial with 22 in the middle cerebral artery territory. HC was solitary in 18 cases, unilocular in 23 and multi-vesicular in 2 with heavily calcified pericyst in 1. 2 cysts were intra ventricular and 1 intra aqueducal. The most typical features were well defined, smooth thin walled spherical or oval cystic lesions of CSF density and/or signal with considerable mass effect (20/25). Surrounding oedema with complete or incomplete rim enhancement was seen in 3 cases which were labelled as complicated and/or infected cysts. Although CT is diagnostic of hydatid disease in almost all cases (22/25), MRI including diffusion and spectroscopy precisely demonstrate location, number, cyst capsule, type of signal and enhancement and allows diagnosis of atypical or complicated HC and appears more helpful in surgical planning.

[Hepatitis A infection and Henoch-Schonlein purpura: a rare association]. / Hépatite A et purpura rhumatoïde: une association rare.

OBSERVATION: A 10-year-old boy presented cholestatic hepatitis A virus infection confirmed by IGM anti-HAV antibody. Three days after admission, he presented a palpable purpuric rash on the declivous regions, arthralgia and abdominal pain. He met all criteria set by the American College of Rheumatology (ACR) for Henoch Schonlein purpura. The evolution was gradually favorable with no renal involvement (recoil of 3 years and half). CONCLUSION: Henoch Schonlein purpura is an exceptional extra-hepatic manifestation of hepatitis A infection.

[Systemic infantile mastocytosis: about a case with respiratory and digestive involvement]. / Mastocytose systémique du nourrisson: à propos d'un cas avec atteinte respiratoire et digestive.

UNLABELLED: Systemic mastocytosis is rare in children and is characterized by an abnormal proliferation and infiltration of mast cells in different tissues. CASE REPORT: We report a case of systemic mastocytosis presenting cutaneous symptoms during the neonatal period. Later evolution was characterized by systemic manifestations consisting of recurrent respiratory infections with wheezing and a digestive involvement that included abdominal pain, hepatosplenomegaly and a nodular, hemorrhagic infiltrate in a low esophagus. The diagnosis was confirmed by histology and biology, notably increased histamine concentrations in blood and urines. Improvement of the respiratory and digestive symptoms was obtained with treatment by histamine H1 and H2 receptors antagonists. CONCLUSION: Respiratory manifestations and nodular infiltration of the digestive tract are rare in systemic mastocytosis. The prognosis is conditioned by complications such as malignancy and the persistence of the disease till the adult age.

[Guillain-Barre syndrome in children. Report of 39 cases]. / Syndrome de Guillain-Barré chez l'enfant. Etude de 39 observations.

The authors report a retrospective study of 39 cases of Guillain-Barré syndrome in children. Including 23 boys and 16 girls. The mean age was 4 years (14 months to 10 years). A history of infection preceded the polyradiculoneuritis in 75% of cases. The mean duration of extensive phase and of the maximum paralysis was 10 days. A severe respiratory involvement was observed in 9 children and cranial nerve paralysis in 8 children. An early raised cerebrospinal fluid protein concentration was found in 95%. Electrodiagnostic studies, performed in 37 patients, showed nerve conduction velocity slowing in all cases. 28 children (73%) recovered clinically, 8 patients (20%) had residual deficits and 3 patients (7%) died.

[Medullary arteriovenous malformations in children]. / Les malformations artérioveineuses médullaires chez l'enfant.

UNLABELLED: Spinal arteriovenous malformations are rare lesions in children but may produce serious functional disorders and residual handicaps. CASE REPORT: We report the case of a 10-year-old patient who had weakness in his left leg with intermittent urinary incontinence. Neurological examination showed a spastic paraparesis of both legs, more obvious in the left leg. MRI led to the diagnosis of a spinal vascular malformation, which was confirmed by angiography. The patient underwent a session of embolization, which resulted in a significant clinical improvement. CONCLUSION: The importance of MRI in such a case is emphasized as is therapeutic management with embolization.

[Idiopathic pulmonary hemosiderosis, celiac disease and cardiomyopathy]. / Hémosidérose pulmonaire idiopathique, maladie coeliaque et cardiomyopathie.

UNLABELLED: BACKGROUND--Idiopathic pulmonary hemosiderosis (IPH), a rare and possibly immune disease, is sometimes associated with coeliac disease and myocardiopathy. CASE REPORTS: CASE NO 1--A 2 year-old boy with IPH was investigated because he suffered from frequent, soft stools. Small bowel biopsy showed partial villous atrophy. Circulating gliadin antibodies were present. The patient was placed on a gluten-free diet. CASE NO 2--An 8 year-old girl was admitted because she suffered from severe anemia (Hb: 4 g/100 ml). She was found to have IPH and myocardiopathy. She had no manifestation, but a systematic search for coeliac disease was positive (total villous atrophy; presence of circulating gliadin and alveolar basement membrane antibodies). The patient was placed on a gluten-free diet, prednisone and diuretics, but she died during a relapse 2 months later. CONCLUSION--It is worthwhile checking for coeliac disease in all patients with IPH. The presence of myocardiopathy is a negative prognosis.

[The "blue rubber bleb nevus" (Bean's syndrome): uncommon cause of gastrointestinal bleeding]. / Le syndrome de "blue rubber bleb naevus" (syndrome de Bean): cause inhabituelle de saignement digestif.

A ten year old boy had six cutaneous angiomas and recurrent rectal bleeding responsible for iron deficiency anemia. Histological examination established the diagnosis of blue rubber bleb naevus syndrome. Endoscopy, arteriography, computed tomography, and angioscintigraphy disclosed additional angiomas in the stomach and rectum and along the blood vessels in the lower limbs. The value of angioscintigraphy for identifying angiomas escaping detection by other means was illustrated in this case. Embolization of the inferior mesenteric artery was successful in preventing further rectal bleeding. Treatment of this disorder remains controversial.

[C-reactive protein in bacterial meningitis in adults]. / La protéine c-réactive dans les méningites bactériennes de l'adulte.

In order to assess the benefits of serial assays of C-reactive protein in the course of bacterial meningitis in adults, daily blood samples were taken for CRP measurement during 10 days in 21 consecutive patients (mean age: 24 +/- 8 years) hospitalized for bacterial meningitis principally due to Neisseria meningitidis (n = 15). The highest CRP level (178 +/- 38 mg/l) was present on admission, followed by a regular decrease occurring in uncomplicated meningitis until normal level was achieved on day 9. The CRP kinetics was not influenced by the type of causative micro-organism. This study shows that CRP kinetics in adults is similar to that reported in children. The benefit of CRP assays in optimizing the duration of antibiotic treatment of meningitis needs to be more carefully assessed.

Hellp syndrome: incidence and maternal-fetal outcome--a prospective study.

OBJECTIVE: To determine the incidence of Hellp syndrome (HS) and the maternal fetal outcome associated with its occurrence. DESIGN: A prospective study during a 6-month period. SETTING: The department of obstetrics and gynecology and the Intensive Care Unit of a 700 bed teaching hospital. PATIENTS: Sixty-two consecutive preeclamptic and eclamptic women. MEASUREMENTS AND RESULTS: All patients were systematically investigated for the biological markers of HS. The effects of the occurrence of HS on maternal and fetal prognosis were evaluated by comparing for prognosis indicators usually assessed in gravidic hypertension, pre-eclamptic and eclamptic woman who exhibited HS (HS+ subgroup) with HS free patients (HS- subgroup). HS was found in 12 out of the 62 pre-eclamptic and eclamptic women (19.3%). Its occurrence was associated with higher maternal mortality (16.7% vs 0%; p = 0.03), a greater incidence of eclamptic crisis (50% vs 20%; p = 0.03), severe hypertension (33% vs 8%; p = 0.03) and episodes of acute renal failure (66% vs 30%; p = 0.02). Mean proteinuria was also higher in HS+ patients (4.6 +/- 3.3 vs 2.2 +/- 2.5 g/day; p = 0.001). However, fetal outcome was not significantly altered. CONCLUSION: Pre-eclampsia and eclampsia may be more severe in the presence of HS with a worsening of maternal prognosis while fetal outcome seems not altered.