[Liver involvement in Gaucher's disease: a tunisian cohort study]

Gaucher disease is a sphingolipidosis related to glucocerebroside storage in reticuloendothelial cells leading to multisystemic disease. Liver involvement is frequent but clinical expression is rare. The aim of this study is to evaluate liver involvement among a cohort of 45 patients with type 1 Gaucher disease. Hepatomegaly often mild to moderate was seen in 86 per cent of cases. A correlation was noted between hepatic involvement, spleen enlargement and severity index score. Portal hypertension was documented in 20 per cent of cases and seemed to be primitive. Four children had cirrhosis and two a hepatopulmonary syndrome. Splenectomised patient didn't show worsening of liver involvement. Liver complications were more frequent in pediatric patients comparatively to adult patients in this cohort

[Cardiovascular disorders in Kawasaki disease: results of a Tunisian collaborative study]

Kawasaki disease [KD] is the first cause of cardiopathy acquired by children in developed countries. The aim of this study is to find out, in a precise way, the epidemiological, clinical and evolutionary aspects of the cardiovascular disorders in the Kawasaki disease and to determine their potential risk factors. It is a multicenter retrospective study conducted over the laps of 10 years [1007-2006] and which was a subject of interest for five hospital-university pediatrics services, during which the total of 29 observations of KD including 16 boys and 13 girls whose ages ranged between 6 months and 12 years [average age = 4 years] were analysed. The cardiovascular disorder was noticed in 11 cases [37, 93 per cent]. It consists in a myocarditis in 4 cases, a pericarditis in 1 cas and an endocarditis in 1 other case. The coronary artery problem [6 cases] from 54, 54 per cent of the cardiovascular disorders and 20, 68 per cent of the total number of patients. The diagnosis of the coronary disorders was made between 7 and 90 days [average 24 days]. Asai score was not correlated at the risk of appearance of coronary aneurysms. 5 patients out of 6 had received veinoglobulins and 3 of them before the 10[th] day of the disease evolution. The evolution was favourable in 4 cases who developed anevrysms under 8 mm. The 2 patients with multiple and huge anevrysms were complicated with a thrombosis and myocardial ischemia in 1 case and with stenosis of the left interventricular in the other. The cardiovascular disorder in the KD is dominated by the anevrysmal coronary disorder. This conditions the short term prognosis and causes the later complications

[ epidemiological data of children burns in Tunis. about 134 cases]

Burns injuries represented the most children common domestic accident which can be responsible of a dramatic consequences. To analyse the epidemiological data and preventive aspects of this victims, a retrospective study is reported between 1 January and 31 December 2006 concerning 134 children who were examined during this period in the emergency of the surgery department [B] of the children Hospital of Tunis. In 43 cases, a hospitalisation was necessary. The mean age of the patients was 36 months. The accident occurs in the house in 99 per cent of cases, especially in the kitchen in 93 per cent. The most common agents were scalding liquid in 89.5 per cent followed by flames in 8 percent. The mean total burns surface areas was 4 per cent for the non-hospitalised children and 14 per cent for those whose been hospitalised, 16 per cent of this children developed an infection of the burns injuries during their hospitalisation. In 3 cases an amputation was necessary due to a third degrees burn with carbonisation of the members. One patient died 48hrs after his admission by a respiratory distress. The children burn injuries can be considerably reduced by preventive measures and active public health programs to promote immediate cooling of burns with cool water which is at least as important as subsequent medical and surgical treatment in determining the outcome of burns in children

[Epidemiologic profile of skin diseases in Tunisian children]

Skin diseases are fairly common in children and can be related to a heavy morbidity. However, very little data about the epidemiology of pediatric skin diseases are available in the literature. retrospective study about 2007 children seen at the dermatological outpatient's department of children's hospital of Tunis between January 1997 and December 2004. The children's sex, age and diagnosis were noted. the median age was 5 years 2 months +/- 4.5 years [range 3 days, 18 years]. Infants and preschool children represented 67.3per cent of the study population. There was 1023 boys and 984 girls [sex-ratio: 1.03]. There were 2100 skin diseases in the 2007 children. Skin infections constituted the largest group [35.3 per cent] followed by allergic skin diseases [26.3 per cent]. Among skin infections, viral infections were most prevalent [31.2 per cent], followed by fungal infections [28.2 per cent], bacterial infections [21.1 per cent] and parasitic infections [19.4 per cent]. Atopic dermatitis was the most prevalent allergic disease. It was also the most prevalent dermatosis in the infant group. The number of children with atopic dermatitis decreased with age, suggesting that this condition tends to clear with age. epidemiologic data are useful in monitoring changes in diseases trends in children and planning healthcare programs for them

[Chronic hepatitis B: a study of 15 cases]

The aim of this study is to describe clinical, biological, histological aspects and outcome of children with chronic hepatitis B [CHB], and to analysis treatment modalities. a retrospective study of 15 children with CHB was conducted between January 1992 and December 2003. All patients had HBs Ag> 6 months, fourteen had Hbe Ag. Initial histological examination was performed in eight cases. intrafamilial horizontal transmission was noted in five cases. Fifty three percent of patients presented symptoms: asthenia [57 percent], anorexia [1 case], loss of weight [1 case], digestive disturbance [3 cases], abdominal pain [5 cases]. Cytolysis was noted in eight patients [53, 3 per cent], they had positive Ag Hbe. Liver emzymes were increased in eight cases [53.3%], they had positive Hbe Ag, mean rate of AST was 95.6 UI/I [extremes: 56 -251], mean rate of ALT was 118,8 Ul/l [extremes: 50 - 204]. Four patients had chronic active hepatitis and four had chronic persistent hepatitis. Three patients received I'interferon a; seroclearance was noted in two patients without negativation of HBs Ag. Twelve patients didn't receive any antiviral therapy, liver emzymes were decreased in three patients, seroclearance of HBe Ag was noted in five cases and HBs Ag elimination was noted in only one patient. Chronic hepatitis B is often asymptomatic and transmitted horizontaly. Antiviral therapy must be indicated in children with viral replication and active chronic hepatitis. Large vaccination at birth reduces vertical transmission and prevalence particularly in endemic areas

[Multiple carboxylase deficiency]

Multiple carboxylase deficiency is a syndrom in which biotin-dependant carboxylases show diminished activity du to a lack of biotin or autosomal recessively inherited disorders of biotin metabolism. The clinical picture involves the nervous system, skin, respiratory system, digestive system, and immune system. This syndrome is fatal in the absence of prompt diagnosis and treatment with biotin. Authors report the case of 2-month-old child explored for myoclonic seizures that do not respond well to classic anticonvulsivant therapy, hypotonia, skin problems with alopecia, appeared at age of one month. The laboratory examinations showed hyperammonemia and hyperlactacedemia.Multiple carboxylase deficiency was suspected and treatment with biotin [5mg/day] achieved a rapid improvement of the seizures and the skin problems

[Brucellosis. report of a case]

The brucellosis is an infectious disease caused by brucella. We reported a case of brucellosis in a 14 year old boy admitted for a trailing fever and slimming. The diagnosis was confirmed by two blood cultures and the serodiagnosis of Wright. He was treated by rifampicin and doxycyclin. Two weeks later, he developed a brucella arthritis [spondylodiscitis]. The evolution after six months of treatment was good. This arthritis must be searched earlier with the radiologic explorations

[Systemic juvenile scleroderma: about 6 cases]

Introduction: The systemic juvenile scleroderma [SJS] is a rare acquired auto-immune disease. The aim of our study was to analyse the epidemiologic, clinical, therapeutic and prognostic features

Patients and Methods: through a retrospective study, 6 patients were observed with SJS. We adopted the Barnett's classification. Results: The six patients were females, with a mean age of began on 9 +/- 3 years. Five patients were classified type II of Barnett and for 1 patient the classification was difficult. Raynaud's phenomen was observed in 4 cases. The cutaneous sclerosis was noted in the all cases. Five children presented oesophageal damage. Lung involvement was noted in 3 cases. No cardiac dysfunction was found at the clinical exam. Mild renal involvement was noted in 1 case. SJS was associated to systemic lupus in 2 cases and to dermatomyositis in 1 case

Discussion: Systemic juvenile scleroderma is rare. The cardiac involvement is a major concern in children; it appears to be a more common cause of death in SJS. The therapeutic problems were similar to those in adults

[Acute poisoning in the pediatric service at the infant hospital of Tunis. report of 325 cases]

We reported a retrospective study concerning 325 cases of acute poisoning in the pediatric service at the infant hospital of Tunis during a period of one year from 1 January to 31 December. The aim of this report is to evaluated the epidemiologic, clinical and evolutive aspects. They were 194 boys and 131 girls. 60 per cent of the infants are aged under 3 years. The chemical products occupied the first place with 153 infants [49.7 per cent], the inhalation of petroleum represented 62 cases, the water of javel 58 cases. The medicines occupied the second place with 128 infants [39 per cent]. The evolution was favourable in 277 infants [85 per cent]. 12 patients [3.6 per cent] are presented a pneumonia after inhalation of petroleum. In 4 patients [1.2 per cent], we constated a caustic oesophagitis. One case of death was occurred in a patient with medicinal polypoisoning.The prevention stays the better way to fight this phenomenon

[Surgical aspects of intussusception due to lymphoma in children]

Introduction: Bowel's intussuception is a rare kind of child Burkitt lymphoma revelation mode. The management of this situation is full of traps. The retrospective study of 6 observations aims to analyse the clinical, evolutionary and therapeutic side of this revelation mode

Results: Four boys and two girls, aged from three to seven years with an average of 5 years, had Burkitt lymphoma revealed by a Bowel's intussusception. The abdominal ultrasound find the secondary cause in 3 cases .One patient had a full-column barium enema All the cases had a laparotomy. Three cases had a surgical resection. A biopsy of mesenteric lymph nodes was done in 3 cases. A chemotherapy was initiated as soon as we made the diagnosis .There was no after effects in all the cases. No recurrence was noted until two years and a half after the treatment

Conclusion: The diagnosis of lymphoma must be suspected when the bowel's intussuception occurs in more than 5 years old children. A biopsy must be done in the event of suspicion of lymphoma. The surgical procedure must be adapted to each case

[Ambulatory pediatric morbidity. Evaluation of one year activity of consultation in department of Pediatrics, emergency and consultations of children's hospital of Tunis]

Pediatric morbidity has been the subject of many studies, nevertheless those concerning with ambulatory morbidity are rare. The subject of this study is to establish the epidemiologic profile of patients. Identify the encountered pathologies and their frequency, precise morbidity characteristics and raise difficulties in the diagnostic and therapeutic management. In this retrospective study we reviewed the data of 4387 children that have consult for the first time external consultation of the department of Pediatric External Consultations and Emergency [PUC] of the Children's Hospital of Tunis during the year 2003. The 4387 patients benefit from 7323 consultations which represent 44.3 per cent of the total of consultations of the year 2003 [16 507]. 59.4 percent of the studied population was male [sex ratio = 1.2] and two thirds were aged under 6 years old. The main motif of consultations was recurrent bronchopneumonia [15.2 percent], cutaneous disease [6.2 percent], vomiting [6.1 percent] and abdominal pain [5.6 percent]. 3.2 percent of the children were admitted. Ambulatory morbidity was dominated by four pathologies: digestive 16 percent, pulmonary 14.9 percent, neurologic 6.7 percent and hematologic pathothologies [6 percent]. Gastro-oesophageal reflux was the major digestive disease followed by acute diarrhea [10.6 percent]. The principal pulmonary disease were bronchopneumonia [60.5 percent], acute bronchitis [21.6 percent] and asthma [14.4 percent]. 59.9 percent of the neurologic pathology were represented by seizures. 28.4 percent of the seizures were feverous. Anemia is the most frequent hematologic pathology [55.1 percent]. It was caused by iron deficiency in 87 percent of cases. The endocrinologic pathology is essentially represented by the short stature. We suggest to informatize medical data so it would be more exploited for studies, to make more complementary explorations available in the Children's Hospital of Tunis and to decentralize the management of the simples diseases

[Doose syndrome or myoclonic-astatic epilepsy: two new Tunisian cases]

Myoclonic astatic epilepsy belongs to the epilepsies with generalized seizures. It occurs in 1-2 per cent of all childhood epilepsies up to age 9. The disease is characterized by age of onset mostly between 2 and 6 years and by various clinical and EEG criteria [myoclonic or astatic seizures, 4-7 Hz background rhythm, polyspikes and waves in electroencephalogram]. The authors report two cases answering these criteria. It is about 2 old boys respectively of 2 years and a half and of 18 months during first seizures. Attacks were polymorphic associating myoclonic seizures, absences and astatic crises causing traumatism. These attacks are associated to clonic generalized seizures in one case. The electroencephalograph showed a thorough rhythm of 4-7 Hz. Cerebral imaging was normal at the 2 childs. The psychomotor development is normal in spite of the rebel character of crises justifying the use of several therapeutic alternatives during the evolution

[Epidemiological characteristics of the traffic accidents at the child in big Tunis]

Purpose: to kick away epidemiological profile of traffic accidents at the child in the region of Tunis

Patient and methods: It is about a retrospective study of 1191 files concerning children aged between 0 to15 years, victims of public highway accident admitted to the emergency of the institution of the Paediatric Surgery [B] between 1st January and 31 December 2003

Results: The age bracket the most concerned was that of children aged between 4 and 8 years [53.5 per cent]. Twice on three, the victim was a male [63 per cent]. The accidents were more frequent in spring and graver during the weekend. Three peaks of frequency were noted during the day; at twelve o'clock, at 15 p.m and 18 p.m. The victims became especially from the most unfavourable cities of the the capital, 98.6 per cent were pedestrians. The light care was responsible for 77 per cent of accidents. In 84 percent of cases, the driver having caused accident alerted the helps. The transport was not medically in 70 per cent of cases. The cranio-cerebral region was the most concerned in this series [66 per cent], while the thoraco-abdominal region was interested in 28.7 percent and members in 21.2 percent of cases. 11.68 per cent were hospitalized. The rate of death represents 1.1 per cent of patients. Neurological after-effects represented 1/4 of observed after-effects

Conclusion: The analysis of results every year conclude at the decrease of the traffic accident generally, contrasting with an increase of grave accidents responsible for a more important number of death and this at the same time as the increase of the number of vehicles on the road in Tunisia. We conclude to the importance of the prevention and the amelioration of the quality of the coverage to be begun by, the improvement of the conditions of collection and the implementation of structures of reception multi-field

[Crossed renal ectora : a two cases report]

Crossed renal ectopia is a rare congenital anomaly, in which, the two kidneys are in the same side with one of the ureters which crosses the midline to be brought together in the bladder on the opposite side. Generally asymptomatic and of fortuitous discovery, this anomaly can appear by abdominal pains, urinary tract infection or a hematuria. The diagnosis rests on the radiological examinations in particular the ultrasonography, the intravenous urography and the CT scann. The treatment is surgical and is reserved for the complicated forms. We report two observations of children with a crossed renal ectopia The first observation is that of a 5 years old girl, presenting urinary tract infections at repetition whose etiologic assessment comprising a renal ultrasonography, a voiding cystourethrography as well as a three-dimensional tomodensitometry objectified a vesico-ureteral reflux grade HI, on left kidney in crossed ectopia. After sterilization of the urines, this patient profited from a surgical cure of her left vesicoureteral reflux with simple continuations.The second observation concerns a 10 years old boy, carrying a malformation anorectale, operated at birth, and at which the malformatif assessment [renal ultrasonography, vertebral radiography] objectified a left kidney in crossed ectopia with vesicoureteral ipsilateral reflux grade Ill associated with complex vertebral anomalies. This child was operated according to the same technique with simple operational continuations. From these two observations and after review of the literature, we recall the clinical, radiological and therapeutic characteristics of this malformation and discuss the pathogenic assumptions

[Mortality in juvenile idiopathic arthritis]

Mortality in juvenil Arthritis is variable from a serie to another and with form of IJA. The authors have analysed the observations of child dead by IJA. They report three observatios concerning 2 boys and 1 girl aged respectively of 7 years 10 months, 10 years 3 months ant 14 years 4 months: which is compatible of a level mortality of 4, 2 % in the period of study [1986 -2003] IJA is polyarticular in the three patients, with systemic beginning in one boy and it's a spondylarthro pathy in the second. The etiology of death is a macrophagic activation syndrom in 2 cases revealed by neurologic and hemorragic symptoms. The association salazopyrin - acetyl slycylic acid is incriminated in one boy and rofecoxib in the girl. In the other boy death is secondary to renal amylodosis associated to nephritic syndrom, renal deficiency, denutrition and Ionic disorders. This amylodosis was pertisting, in spite of treatment by methotrexate for three months. The authors conclude to elevated level of mortality and insist on depistage and rapid treatment of macrophagic activation syndrome for the prognosis of IJA