Estimating the prevalence of autism spectrum disorder in Lebanon.

This cross-sectional survey examines the prevalence rate of Autism spectrum disorder (ASD) in 818 children (16-48 months) across all Lebanese regions. Screening was done using the revised form of the Modified-Checklist for Autism in Toddlers. Based on the total score of items failed, children were classified into 3 categories of ASD risk (low, moderate and high). Phone calls follow-up interviews and clinical assessments for diagnosis ascertainment were conducted. Given the caregivers' reluctance to participate, the prevalence rate was estimated between 49 and 513 per 10,000 with a male predominance. Our prevalence estimation, even under restrictive assumptions, is higher than elsewhere in the Arab region. Anti- stigma interventions adapted to the socio-cultural context are needed prior to future research in the field.

[Chaotic atrial tachycardia-induced cardiomyopathy: report of an isolated case]. / La tachycardie atriale chaotique compliquée de cardiomyopathie: à propos d'un cas insolite.

Chaotic atrial tachycardia is a rare arrhythmia that has no known etiology and that usually inflicts upon newborn infants. The diagnosis is established using the surface electrocardiogram (ECG) which shows a spectacular polymorphism and irregularity of the atrial electrical activity. Clinical tolerability is variable depending on the ventricular rhythm. Cases that are not well tolerated and cases who do not recover spontaneously require medical treatment which relies mainly on amiodarone and other class IC anti-arrhythmic drugs. There is usually complete recovery during the first few months of life. The authors present the case of a female patient who was diagnosed with chaotic atrial tachycardia with induced cardiomyopathy following birth. She was successfully treated with amiodarone but had several relapses of the arrhythmia upon discontinuation of the drug. Although this observation is classic in its presentation, we consider that it is useful to remember this rare and frequently forgotten syndrome and to report the unique and particular aspects of our case and its evolution.

Infantile spasms in Down syndrome: good response to a short course of vigabatrin.

PURPOSE: To evaluate the efficacy of vigabatrin (VGB) in the treatment of infantile spasms (ISs) associated with Down syndrome (DS) and to assess the feasibility of early discontinuation to reduce the possible retinal toxicity. METHODS: Five children with ISs with DS were treated with vigabatrin as first-line monotherapy in an open prospective study. The short-term response was evaluated, and VGB was continued in responders. The treatment was stopped after 6 months in children who were still spasm free. RESULTS: Four children of five became spasm free with VGB, three of them responding within 1 week. This response was maintained during the 6 months of VGB treatment. After VGB discontinuation, and with a follow-up ranging from 2 to 4 years, none of the responders experienced spasm recurrence or other types of seizures. CONCLUSIONS: This study confirms the efficacy of VGB in ISs associated with DS. Moreover, it shows that the duration of VGB treatment can be reduced to 6 months without relapse of ISs. This short treatment might reduce the risk of developing visual field constriction.

[Familial lymphohistiocytosis. Evolution of management apropos of 3 cases]. / La lymphohistiocytose familiale. Evolution de la prise en charge à propos de trois cas.

Familial lymphohistiocytosis is a rare rapidly lethal genetic disease. It is characterized by an uncontrolled activation of T lymphocytes and macrophages, with multiple organ infiltration, beginning with fever and unexplained coagulopathy. Recently, one of the genes implicated in 50% of families at risk was identified (locus FHL1, chromosome 10, region q21-22). Based on data suggesting an essential role of T lymphocytes in the genesis of familial lymphohistiocytosis, the treatment has recently evolved from a chemotherapy including Etoposide (VP16) and corticosteroids, sometimes efficient but toxic, to an almost always efficient and slightly toxic immunosuppressive treatment. These two treatments achieved a remission somewhat lasting with no definite cure. In fact, all patients relapsed in the central nervous system and died. Bone marrow transplantation (BMT) is the only curative treatment. However only 20% of patients benefit from an HLA identical BMT. Recent improvements in HLA non-identical BMT offer an acceptable alternative to the other 80% of patients. In this review, we present three cases illustrating the evolution and optimization in the management of infants with familial lymphohistiocytosis.

[Kidney transplantation in the child. Experiences of l'Hôtel-Dieu de France]. / La transplantation rénale chez l'enfant. Expérience de l'Hôtel-Dieu de France.

A retrospective study was conducted on all kidney transplantations performed between January 1993 and June 1996 in our multidisciplinary pediatric department. Thirteen children with a mean age of 6 years (3.5-12) were transplanted during the study period after an average waiting time of 2 years on dialysis for cadaveric transplants and 3.5 months for living related ones. No urologic complication was noted; however, intra-abdominal approach was decided to be performed only in children less than 9 kg. This decision was taken after the occurrence of one intestinal intussusception and two ileal obstructions. During these 3.5 years, five rejection episodes were treated: three were steroid responsive, two were resistant but responded respectively to plasmapheresis and to OKT3. The actuarial survival of the grafts and recipients were 100% for an average mean time of follow-up of 18 months. The latest serum creatinine and creatinine clearance averages were respectively 66 mumol/l and 105 ml/min/1.73 m2. In seven transplanted children for more than 18 months growth was satisfactory. Eleven children returned to school. Kidney transplantation is the optimal treatment for children and infants with chronic renal failure; however, this technique needs a highly experienced team and a permanent close follow-up. Some children with end stage renal disease can directly have preemptive transplantation which is becoming our recent choice to avoid the hemodialysis strain.

[Recurrent multifocal chronic osteomyelitis: scintigraphy or MRI. Apropos of 2 cases]. / Ostéomyélite chronique multifocale récurrente: scintigraphie ou IRM. A propos de deux cas.

We report two cases of chronic recurrent multifocal osteomyelitis. In both cases, MRI demonstrated the presence of asymptomatic lesions that the technetium bone scan failed to show. The asymptomatic lesions were located in the acetabelum and the distal femur. Despite the actual literature, MRI was more specific than bone scan in these two cases.

Holocarboxylase synthetase deficiency: report of a case with onset in late infancy.

A case of holocarboxylase synthetase (HCS) deficiency of late-infantile onset is presented and compared with the common manifestations in previously reported patients. Our patient had her first episode at 20 months followed by recurrent episodes of metabolic acidosis with ketolactic acidosis responding dramatically to a short trial of biotin and thiamin. The main clinical findings were metabolic acidosis with alteration in consciousness and respiration, which are in accordance with findings in earlier reported patients with both neonatal-onset and infantile-onset forms of HCS deficiency. The diagnosis of HCS deficiency was made only at the age of 5.5 years during a metabolic work-up when organic acid analysis was performed. This revealed elevated urinary excretion of the characteristics metabolites, 3-hydroxypropionate, 3-hydroxyisovalerate and methylcitrate, suggesting multiple carboxylase deficiency (MCD). MCD was demonstrated in fibroblasts of our patient, but only when the cells were grown in a medium with a very low biotin concentration of 10(-10) mol/L. Kinetics studies of reactivation of deficient propionyl-CoA carboxylase activity with biotin in intact fibroblasts revealed a midly decreased reactivation rate and only a 3-5 times higher biotin requirement as compared with controls. These findings are in accordance with a mild form of HCS deficiency. This child responded to 10 mg/day of biotin with normal lymphocyte carboxylase activities and adequate school performance at 10 years of age.

Renal biopsy in children in a developing country in 61 consecutive cases.

Percutaneous renal biopsy in children is a safe procedure. It is used for definitive diagnosis, prognosis and evaluation of the response to therapy. Repeated percutaneous biopsies are currently performed especially in allograft kidneys and in native kidneys as well. No major complications were noted in our series. The use of the new automated technique with small disposable needles in a well sedated child and experienced operator minimizes the complication rate. The important clinical benefit of the percutaneous renal biopsy overcomes the minimal risk of the procedure. Mesangiocapillary glomerulonephritis and focal segmental glomerulosclerosis are the predominant histological findings in our series. More data from other centers are necessary to evaluate the real incidence of the different entities of renal diseases in our country.

[Foreign bodies of the respiratory tract in children. A retrospective study of 100 cases]. / Corps étrangers des voies respiratoires chez l'enfant. Etude rétrospective de 100 cas.

Foreign body inhalation is a universal problem, most common cause of death from domestic accident in children aged five years and less. Over 15 years, one hundred children aged less than six years were evaluated in Hotel Dieu de France; findings are comparable to previous data, with one avoidable death; boys are chiefly concerned (64%); mean age is 22.5 months; circumstances are often hazy (65%); vegetables are prominently responsible (90%) especially peanuts and pistachios (48%); foreign bodies are seldom radiopaque (1%); autumnal predominance is noted. Inhalation is not reported in 25% of cases; immediate risk is subglottic impaction; the child survives if the foreign body is expelled one way or another. The most frequent site is the bronchial system (87%) with a slight right predominance (49%); symptoms include: dyspnea, persistent cough, and, in case of bronchial obstruction: wheezing and asymmetry of breath sounds; 15% of children are free of symptoms. Delay before hospital care is long (21.5 days), mostly because diagnosis is misread particularly in case of bronchial foreign body; pulmonary distension is a frequent finding (45%). In case of asphyxia, first aid resuscitation is performed immediately: in fact it is rarely useful, sometimes harmful. Extraction is mandatory with the stiff bronchoscope; otherwise, bronchopulmonary infection and destruction is the usual outcome ... (25%). Management is revisited, and prevention is recalled.

[Accidents in children. Retrospective epidemiological study of 1671 cases collected at the Hotel-Dieu of Beirut]. / Accidents chez l'enfant. Etude epidémiologique rétrospective de 1671 observations recueillies a l'Hôtel-Dieu de Beyrouth.

Injuries are the first cause of death in children after the neonatal age; their prevention is still critical in Lebanon. At Hotel-Dieu de France Hospital, 1671 children aged less than 18 years have had their files reviewed in the emergency department. Minor traumas represent less than half the cases. Though most of the injuries were light, in 5% of cases primary care was necessary, and 11% were admitted (10% in the Pediatric Intensive Care Unit); 0.5% of the children died upon arrival. Moreover, 15% of our Pediatric Intensive Care Unit population are admitted for accidents. Boys are more frequently affected (69%); toddlers, small children and adolescents are risky populations. After minor traumas, the most frequent accidents are: falls (21.3%), traffic road accidents (8.9%), poisoning (5.7%) and burns (5%). Firearms injuries seem rare (2.8%) but are, in this study, the first cause of mortality. Foreign body inhalation are very rare (< 1%) but are a significant source of morbidity. This analysis is compared to published data, and allows accurate and general recommendations for injuries prevention in children.

[Hydrocarbon poisoning in children]. / Intoxications aux hydrocarbures chez l'enfant.

Carbohydrate poisoning is among serious children intoxications, made possible by inadequate stocking; boys, one to five years old, are particularly at risk in a series of 43 children examined and admitted in Hôtel-Dieu de France Hospital over a 10-year period. Outcome is constantly good, after a short hospital care; clinical exam and chest X-ray are of particular value on admission. Some cases may need home care, whereas others should undertake hospital day care; treatment is discussed and long-term follow-up is being stressed on.

[Infantile anorexia nervosa]. / Anorexie nerveuse du nourrisson.

Infantile anorexia nervosa is different from anorexia nervosa of the adolescent; it occurs early in life, more often since the use of milk powder. The main pathway is the dyadic unit "mother and child"; the author reports two female cases of unequivalent severities, with 6 months back-up. Various presentations are discussed and focused on the struggle and duality between the mother and the child as a major element in the genesis of infantile anorexia nervosa; no organic illness was found, preventive attitude is proposed.

[Neonatal hydrometrocolpos. A cause of immediate neonatal respiratory distress]. / Hydrométrocolpos néonatal. Une cause de détresse respiratoire immédiatement néonatale.

The authors report a case of acute fetal distress and immediate respiratory distress in a neonate presenting with large hydrometrocolpos. This diagnosis, infrequently concerned in such a situation, should be suggested in cases with abdominal tumors.