ABSTRACT
Congenital diaphragmatic hernia [CHD] is the result of a defect of the musculo-aponevrotic septui which separate the thoracic cavity from the abdominal cavity allowing migration of the abdominal viscera in thorE, Its global frequency is estimated at 1/300010 1/5000 living births. In the majority of cases, it appears in the fir 24 hours of life revealed by an acute respiratory distress. In 5 to 25% of cases, the CHD was lately reveale beyond 4 weeks of life to several months or sometimes several years
Patients and methods: We report retrospective study of 8 cases of CHD with delayed revelation enrolled in the department of pediatrics on a peric of 22 years between 1984 and 2005
Results: Six patients of our cases had postero lateral hernia by th Bochdalek foramen of divided on 5 in the left side and one case on the right side. Only 2 patients had retro-cost[xyphoidien hernia of Larrey. Epidemiologic data reported 3 girls and 5 boys with the mean age of one year an half [range from 47 days to 3 years and half]. On the clinical plan the mode of beginning was acute on 6 case or chronic on' one case. CHD was fortuity discovered in one case during exploration of axillary lymph node! Respiratory symptoms were predominant in 7 cases associated to gastrointestinal symptoms in 4 cases. patients benefited from chest radiography that revealed digestive clarities on the lung bases. TOGD has bee also achieved in all cases and permitted to confirm the diagnosis and to specify the herniated organs. Thorac[abdominal scan in 2 cases and IRM in only one case revealed a right HDC with ascension of the liver. Seve patients have been operated. One child died quickly some hours after his admission by refractory hypoxernii Post operative outcome are favourable in six cases. One child dead one day after surgery by refractor respiratory distress
Conclusion: The diagnosis of the delayed CHD must be evoked in presence of respirator or digestive symptoms associated to unusual radiological abnormalities of the lung bases. Early surgic; treatment is efficient in the majority of the cases
ABSTRACT
The West syndrome is a scarce epileptic syndrome in infant characterized by a symptomatic triad associating epileptic spasms, psychomotor regression and hypsarythmy. The goal of this work is to analyze the electroclinic and etiologic aspects of the West syndrome and to propose a therapeutic control
Patients and methods: We report a retrospective study of 25 cases of West syndrome collected in the department of pediatric between 1991 and 2005. Several epidemiologic and evolutionary parameters were studied
Results: The middle age of our patients is 5 months and half with the extreme going from 45 days to 12 months. The spasms are in bending in 96% of cases; only one child had spasms in extension. A delay of psychomotor development was noted in all cases, it was previous to spasms in 48% of cases. The initial electroencephalogram objectified a typical hypsarythmy in 20 patients [80 %] and an atypical hypsarythmy in 5 cases [20%]. On the etiologic plan, it acts of 24 symptomatic forms. The cryptogenic form was noted in only one case. The treatment of first intension was variable and the evolution under treatment was marked by a disappearance of spasms in 22 cases with persistence of a significant psychomotor delay in 9 cases, 3 cases evolved toward a syndrome of Lennox Gastaut
Conclusion: The gravity of this pathology incited us to know better its different electroclinic and therapeutic aspects in order to improve the forecast in these children
ABSTRACT
Introduction: Sweet's syndrome was described for the first time in 1964. It is usually described in adults and remains rare in children. We report a case in a 18 month-old infant
Observation: A 18 month-old boy presented with multiple round erythematous papules and plaques involving the extremities, face and ears. Laboratory examinations revealed neutrophilic polynuclear hyperleukocytosis, anemia and inflammatory syndrome. Physical examination and skin biopsy led to diagnosis of Sweet's syndrome. The boy was treated by oral corticosteroids. Because he developed rectorragia and hematemesis we change to dermocorticoides class II and colchicine that led to spectacular improvement
Conclusion: Sweet's syndrome is exceptional in infants. The frequent association with a malignant blood disease should prompt appropriate investigations and prolonged survey. Systemic corticosteroids therapy is the reference
ABSTRACT
In children, the lymphedema often results from a primitive abnormality of lymphatic vessels. Through four observations depicted in the pediatric department of Sfax, we try to show the clinical and etiological polymorphism of this disease and to underline the difficulties of the coverage especially at the child. 1: a girl hospitalized at birth for Bonnevie-Ullrich's syndrome associating an innate lymphedema and Turner's syndrome. A boy hospitalized at the age of 13 months for congenital unilateral lymphedema with penio-scrotal atteinte, associated to urinary malformation [not obstructive pyelo-ureteral connective syndrome]. A 7 years old boy hospitalized for familial primitive premature bilateral lymphedema type Meige. A 13 years old boy, hospitalized for recidivated erysipele. The diagnosis of sporadic primitive premature unilateral lymphedema associated to venous malformation was retained
ABSTRACT
The bacterian meningitis is an affection which is more frequent and grave in our country. We reported a retrospective study of 33 cases of purulent meningitis in infants aged between 2 and 14 years. This study had been collected in pediatric department of UHC Hedi Chaker of Sfax during a period of 9 years [1996-2004]. The average age was 6 years with a sex ratio of 1,35. The clinical syndrome was dominated by fever and neurologic signs. The neck stiffness and the Brudzinski sign had been respectively noted in84,8 and 60,6% of cases. The lumbar puncture realized in all infants took of suspicious liquid in 28 cases, clearly in 2 cases and haemorrhagic in 3 cases. The pleiocytosis had been higher in the all cases, il was of neutrophile polynuclear predominance in 84% of cases. A germ had been identified in 14 cases [pneumocoque: 6 cases, haemophilus: predominance in 84% of cases. A germ had been identified in 14 cases [penumocoque: 6 cases, haemophilus: 5 cases, meningocoque: 2 cases and Klebsiella pneumoniae: 1 case. The first intention antibiotherapy was mainly cefotaxim [19 cases], ampicillin [9 cases] or cefotaxim and vancomycin [3 cases]. The initial evolution was favourable in 60% of cases. The neurological and extra neurological complications had been noted in 39% of cases. An infant is dead in a grave septicaemia. The later evolution was favourable in 81,8% of cases definitive after effects had noted in 5 infants [deafness = 4 cases and ataxia = 1 cas]
ABSTRACT
Diffuse pulmonary lymphangiomatosis is a rare innate pathology characterized by a multifocal proliferation then a dilation of the lung lymphalic vessels. It can be shown at any age, but especially at the infant essentially by non specific respiratory signs. Diagnosis is histopathological. The purpose of our study is to clarify the interest of the interferon in the treatment of this pathology. One-year-old infant Nasr, hospitalized for investigation of a dyspnea evolving since the age of 4 months. The radiography of the chest under treatment antibiotic without clinical or radiological improvement. An etiological inquiry was begun allowing eliminating most frequent pathologies responsible of interstitial chronicle pneumopathies. A surgical lung biopsy was practiced, ended in a diffuse pulmonary lymphangiomatosis. A treatment with interferon was prescribed. Evolution was favorable with a recession of three years and a half. Interferon would be an effective treatment in the diffuse pulmonary lymphangiomatosis but other studies are necessary to appreciate better its effect in this pathology
ABSTRACT
Peripheral primitive neuroectodermal tumors are malignant small round cell tumors which occur rarely in children we retrospectively reviwed the clinical data and radiological studies of four such cases. the youngest child had a pelvic tumor and each of two children had a chest wall tumor. the fourth patient had a bone swelling tumors were all diagnosed by histological and immunhistochimical examinations most patients had combined treatment with chemotherapy surgery and radiation therapy. Best results were obtained in two cases and another died. in this report the clinical aspects of PNET in children are described reviewing the presentation of the disease in four patients and detailing the treatment strategies