[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

[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

[Clinical and etiological features of primary adrenal insufficiencies in children]

The chronic primary adrenal insufficiency or Addison's disease is uncommon in children and belongs generally to a complex syndrome. Study of the clinical and aetiological features of primary adrenal insufficiencies in children. In a retrospective study, we reviewed clinical and diagnostic data of all cases of Addison's disease admitted within a period of 15 years [from January 1991 to December 2006], in a department of paediatrics. Cases due to congenital adrenal hyperplasia were excluded. 6 cases of Addison's disease were diagnosed. Five patients are the product of consanguineous marriage. The age at the diagnosis of adrenal insufficiency yarned from 15 months to 9 years 8 months. The adrenal insufficiency was associated to Allgrove syndrome in three cases, to autoimmune polyendocrinopathy type 1 in one patient and to probable peroxisomal disease in another one. The etiological disease was not determined in one patient. A substitutive hormonal therapy was conducted in all patients. During a mean follow-up of 26 months, two adrenal crises were noted. larger studies about Addison's disease are needed to confirm the preponderance of the Allgrove syndrome

[Focal xanthogranulomatous pyelonephritis success of the conservative treatment about a case report]

Focal xanthogranulomatous pyelonephritis is an atypical form of chronic renal infection, It is frequently misdiagnosed preoperatively because of its resemblance to malignancy. We report on a fifty years old woman who presented with a right kidney mass. The CT scan showed a solid aspect mass with a lithiasis of the upper ureter. The diagnosis of local xanthogranulomatous pyelonephritis was suspected by clinical and radiological findings and confirmed by histopathologic examination of percutaneous biopsy specimens of the lesion. Successful treatement of the patient was achieved with leaving on the lithiasis and antibiotic therapy dosing eight weeks. Three months later the CT scan was completely normal. The conservative treatment of focal xanthogranulomatous pyelonephritis makes possible to avoid a needless nephrectomy usually on young patients. However a close follow-up must be undergone on these patients because of the possible association of focal xanthogranulomatous pyelonephritis and renal cell carcinoma of the kidney

[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

Acute hematogenous osteomyelitis of the ischio pubic ramus. A case report

Pelvic osteomyelitis is rare. We report the case of a 10-year-old boy presented with a five-day history of pyrexia and left painful lameness. Passive range of hip was normal. Initial biological investigations revealed an inflammatory syndrome. Plain radiographs of the pelvis and ultrasound scan of the left hip joint were normal. Bone scintigraphy showed a tracer uptake in the left obturator ring. The blood cultures were positive for methicillin-sensitive staphylococcus aureus. The diagnosis of pelvic osteomyelitis was held. Magnetic Resonance Imaging [MRI] showed a left ischio-pubic ramus osteomyelitis with a subperiostal abscess decompressing into the surrounding tissues. This case history draw attention to the distinctive clinical and bacteriological features of this rare focus of acute hematogenous osteomyelitis and emphasize the MRI interest in the investigation of deep localizations. It may facilitate early recognition of pelvic osteomyelitis in child

[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

[Cutaneous mastocytosis in children. A review of 15 cases]

Introduction: Cutaneous mastocytosis [CM] is a rare disease defined as the infiltration of the skin by mast cells. This disorder is more frequentin children. The evolution of the pediatric form to a systemic disease is rare

Patients and Methods: Our study concerned 15 children seen in the dermatology department of Charles Nicolle Hospital between 1984 and 2002. The aim of this work is to show clinic, therapeutic and evolutive particularities of this disease

Results: Sex-ratio was about 0.87 [7 males and 8 females]. The age at onset of the lesion ranged from one month to 11.5 years. Urticaria pigmentosa was observed in 11 cases, diffuse cutaneous mastocytosis in 2 cases and and xanthelasmoid form in two other children. The treatment of our patients was based essentially on preventive measures and symptomatic therapy with histamine receptor antagonists. Over a mean follow-up period of 11 months we noted regression or resolution of the lesions in 9 cases

Discussion: Urticaria pigmentosa is the most benign. However, systemic signs are more frequent in the diffuse cutaneous mastocytosis form. Preventive measures are the main therapeutic mean; especially avoidance of histamine-releasing agents

[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