[High doses of steroids in the management of caustic esophageal burns in children]. / Corticothérapie à forte dose dans le traitement des oesophagites caustiques sévères chez l'enfant.

UNLABELLED: Esophageal burns are frequent in some countries. Esophageal stricture is a severe complication after caustic ingestion. Its frequency is estimated to 5% and reaches 47% in severe esophagitis. AIM OF THE STUDY: To study the influence of management of severe esophageal burns with high doses of steroids in the occurrence of esophageal stricture through the experience of an endoscopic unit. PATIENTS AND METHODS: Twenty-six children with a mean age of 3.5 years (15 months-8 years) with a second b- (N =22) or a third-degree (N =4) esophageal burns due to accidental ingestion of a caustic substance were included between 1993 and 1999. Corrosive substances ingested were sodium hydroxide (N =17), bleach (N =4), alkali (N =3), others (N =2). Upper GI endoscopy was performed in 17 children within the 24 hours of caustic ingestion and within the first 48 hours in all cases. All patients received methylprednisolone (1 g/1.73 m2/day) plus cimetidine and ampicillin plus early oral feeding resumption. RESULTS: Three children (2 grade II et one grade I) were not controlled on day 30 of the protocol and have then been excluded. The remaining 23 children were divided in two groups depending on the upper GI endoscopic results: group I of 12 patients completely or partially healed and group II of 11 patients who developed an esophageal stricture. No difference was observed between the two groups with regard to the delay between caustic ingestion and the start of treatment and the number of shots of methylprednisolone. Second b- degree esophagitis complicated with an esophageal stricture underwent a median of five esophageal dilations (1-12). After a median follow-up of three years, four of them have a normal esophagus. All children with a third-degree esophagitis developed an esophageal stricture. One had a surgical replacement of the esophagus with a segment of colon with good outcome. The three others underwent a median of seven esophageal dilations (5-10). One of them has a normal esophagus after a follow-up of five years while the two others were lost to follow-up. CONCLUSION: High dose of corticosteroids seems to improve second b-degree esophagitis prognosis and may prevent from esophageal stricture.

Methylmalonic acidaemia with bilateral globus pallidus involvement: a neuropathological study.

A 16-month-old boy was hospitalized because of a 1-day history of severe ketoacidosis with lethargy, hypotonia, vomiting, and important dyspnoea. Organic acid assay by gas chromatography-mass spectrometry confirmed the diagnosis of methylmalonic acidaemia (MMA). On the sixteenth day, he developed an acute extrapyramidal disorder. The CT scan of the brain disclosed bilaterally symmetric lucency of basal ganglia. He died at 17 months of age. Post-mortem neuropathological examination, showed severe necrosis with spongiosis, cystic cavitation and numerous lipid-laden macrophages of the globi pallidi, and mild spongiosis of subthalamic nuclei, mammillary bodies, portion of internal capsule adjacent to globus pallidus, superior cerebellar peduncles and tegmentum of brainstem. Pallidal infarction, a focal ischaemic lesion, demonstrates that ischaemia/energy depletion may be important in the etiology of the neuropathology of MMA.

[Periostal effusion in hypernatremic dehydration with disseminated intravascular coagulation]. / Décollement périosté au cours d'une déshydratation hypernatrémique avec coagulation intravasculaire disséminée.

BACKGROUND: Several cases of hypernatremic dehydration complicated by consumption coagulopathy and peripheral gangrene have been reported. CASE REPORT: A 4.5 month-old boy was admitted suffering from acute gastroenteritis with cyanotic edema of the distal part of the left leg and foot. His serum sodium was 160 mEq/l, potassium 5.8 mEq/l and urea 27.6 mMol/l. His prothrombin level was 44%, fibrinogen 0.65 g/l and the number of platelets was 20,000/mm3. Blood cultures were negative. Ultrasonography showed a subperiostal effusion in the posterolateral part of the left tibia with edema of the adjacent soft tissues. Culture of the fluid was negative. The patient was rehydrated. Several phlyctenes appeared on the left foot; cultures of the exudate remained sterile. His condition gradually normalized within two weeks and ultrasonography performed at the 15th day was normal. CONCLUSION: The subperiostal effusion seen in our patient could have been due to microthrombosis of the metaphyseal vessels, following severe dehydration.

[Scurvy in children]. / Scorbut chez l'enfant.

The authors report two cases of scurvy in two encephalopathic 3 year-old girls. The first case was misleading and was initially operated with the diagnosis of osteomyelitis. The second one presented with bleeding syndrome and subperiosteal hemorrhage. The diagnosis of scurvy was based upon deficient diet, clinical features, and periosteal cleavage at ultrasonographic evaluation. Treatment with ascorbic acid, 400 mg/d, led to a complete resolution of the clinical features. The authors emphasize the misleading features of scurvy at bone ultrasonographic evaluation and recommend a supplementation with ascorbic acid of children with severe mental retardation.

[Neonatal ascites caused by obstructive urologic disease. Apropos of 2 cases]. / Ascite néonatale par uropathie obstructive. A propos de daux cas.

We report two cases of neonatal ascites. The first case is a 24 day old male referred for abdominal distention and edema. Peritoneal tap removed a transudative fluid. Ultrasonographic evaluation revealed obstructive posterior urethral valves. Bladder drainage led to resolution of the urinary ascites and renal function normalization. Long term follow-up after endoscopic resection of valves was good. The second case is a male infant who presented at birth with abdominal distention. Radiology revealed an urinoma and a left side hydronephrosis secondary to ureteropyelic junction syndrome which underwent a successful surgical treatment. Urinary ascites is a rare entity which calls for immediate diagnosis and management to preserve renal function.

[Antenatal bilateral sylvian infarction and congenital syphilis]. / Ramollissement sylvien bilatéral anténatal et syphilis congénitale.

BACKGROUND: The classical symptoms of congenital neurosyphilis include meningovascular lesions that are responsible for CSF abnormalities. Lesions of larger vessels are very unusual. CASE REPORT: A boy was born from a neglected pregnancy, weighing 2.7 kg. He was abandoned by his parents and was admitted to hospital at 40 days of age (weight: 2,220 g; height: 47 cm; head circumference: 22 cm) with axial hypotonia, peripheral spasticity and shock syndrome. The TORCH screen was negative but the Treponema Pallidium Hemagglutination Assay (TPHA) was positive. The infant died 4 days later. Brain pathological studies revealed meningoencephalitis and bilateral sylvian infarction. CONCLUSION: Local arteritis due to syphilis usually concerns small arteries, such as the meningeal and cortical vessels. The occlusion of the two middle cerebral arteries seen in this patient, which were responsible for atrophy of the parietal lobes, is uncommon in congenital syphilis.

[Association of Hbo Arab/beta-thalassemia discovered fortuitously in 2 brothers]. / Association d'une HbO Arab/bêta-thalassémie découverte fortuitement chez deux frères.

A four-year-old boy admitted for fever and a skin rash was diagnosed as having a rickettsial infection. Regenerative microcytic anemia and enlargement of the spleen were also found. Hemoglobin electrophoresis and a family study disclosed a combination of two heterozygous hemoglobinopathies, i.e., HbO Arab and beta-thalassemia. A male sibling had the same anomalies as the index patient and was free of symptoms.

[Galactosialidosis with Kayser-Fleischer's ring]. / La galactosialidose avec anneau de Kayser-Fleischer.

The case of a 4 year-old boy presenting with dysmorphic facies, hepatomegaly, splenomegaly, growth and psychomotor retardation is reported. Radiological pattern suggested a storage disease. Bone marrow differential cell count showed numerous storage cells with vacuolated lymphocytes. Enzymatic studies showed decreased beta-galactosidase and neuraminidase levels, leading to the diagnosis of galactosialidosis. This is the first Tunisian case reported, which differs from the other cases published by the presence of a Kayser-Fleischer ring.

[An analytic study of cases of childhood diabetes in a pediatric department in Tunis]. / Etude analytique des cas de diabètes infantiles dans un service de pédiatrie de Tunis.

Insulin-dependent diabetes mellitus is common in Tunisia. Eighty-six pediatric cases managed at the diabetes clinic of a department of pediatrics in Tunis from 1979 through 1989 were studied. Relevant clinical and biological findings were abstracted from case-records. Admissions of patients with diabetes mellitus accounted for approximately 0.44% of admissions to the pediatric ward during the study period. Mean age of patients was 7 years. Sex ratio was 0.89. Polyuria with polydipsia and ketoacidosis were the two most common presenting manifestations. Mean blood glucose level at diagnosis was 22.44 mmol/l. Rate of consanguinity was 48%. HLA typing studies demonstrated a high prevalence of DR3 and DR4 alleles and especially of simultaneous expression of both these alleles. Several factors are incriminated in the development of childhood insulin-dependent diabetes mellitus.

[Kocher-Debre-Semelaigne syndrome. Apropos of a case]. / Le syndrome de Kocher-Debré-Semelaigne. A propos d'une observation.

We report a new case of Kocher-Debré-Semelaigne syndrome in a child and describe this condition on the basis of a review of the literature. Affected individuals exhibit both hypothyroidism and pseudohypertrophic myopathy and consequently have an athletic appearance that contrasts with physical and psychological sluggishness.

[A purely cutaneous form of periarteritis nodosa in children. A new case with discussion of the literature]. / Forme cutanée pure de périartérite noueuse de l'enfant. Une nouvelle observation avec discussion de la littérature.

We report a new case of strictly cutaneous periarteritis nodosa in a girl aged 5 1/2 years at onset of the disease. No visceral involvement has developed during the seven years follow-up. Our patient is the fourth published pediatric case of strictly cutaneous periarteritis nodosa.

[Typhoid fever in children in Tunisian rural areas. Epidemiologic and clinical study]. / La fièvre typhoïde chez l'enfant en zone rurale tunisienne. Etude épidémiologique et clinique.

Based on 140 cases of typhoid fever observed in the pediatrics service of the Menzel Bourguiba hospital (Tunisia), the authors demonstrate that such an endemic disease still exists in rural areas, with a peak during autumn-winter season. They underline the importance of the contamination due to water. The disease strikes all groups of age. Infants represent 15% of the patients. On its clinical and biological aspect, the disease is significantly different in child or infant. Blood cultures are positive in 2/3 of the cases, while fecal cultures are positive only in 1/3 of the cases. Despite some complications occurring in 1/6 of the cases, evolution is generally favourable. However, infant septicemia is severe, causing death in 1/3 of the cases.