[Vasculitis with renal involvement and antineutrophil cytoplasmic antibodies (ANCA) in a child receiving benzylthiouracil]. / Vascularite avec atteinte rénale et anticorps anticytoplasme des polynucléaires neutrophiles (ANCA) après prise de benzylthio-uracile chez l'enfant.

Vasculitis associated to antineutrophil cytoplasmic antibodies (ANCA) is a rare complication of therapy with antithyroid medication. They were mainly described in patients treated with propylthiouracil (PTU), carbimazole, methimazole and rarely by benzylthiouracil (Basden). We report a case of 12-years-old girl treated by benzylthiouracil for Grave's disease who developed after 2 years vasculitis associated with cutaneous involvement (generalized ulcer necrotic purpura) and glomerulonephritis with proteinuria of 24 hours at 26 mg/kg/day, microscopic hematuria and renal failure with creatinemia level at 135 micromol/l. The ANCA type antiMPO (myeloperoxidase) was positive. The histology study of the renal needle biopsy was in favour with focal necrotizing glomerulonephritisand crescents with different evolutive stages. The discontinuation of benzylthiouracil and the treatment by the corticoids involved a disappearance of cutaneous lesions, a negative result of proteinuria, a normalization of the renal function (creatinemia=84 micromol/l) and a disappearance of hematuria and ANCA. These results permitted to announce hypothesis that benzylthiouracil was implicated in development of vasculitis associated to ANCA.

[The cortico-resistent idiopathic nephrotic syndrome of child. Study of 14 observations]. / Le syndrome neprotique idiopathique corticoresistant de l'enfant. Etude de 14 observations.

Cortico-resistent nephrosis represent 10% of idiopathic nephrosis in nephrosis in children and progresses in almost half of the cases to end-stage renal failure. 14 cases of cortico-resistant idiopathic nephrotic syndrome, collected over a period of 13 years (1990 - 2002), are reported. This study involved 10 boys and 4 girls, aged 8 years on average. A history of allergy was present in two cases. Two other children had a family history of cortico-resistant nephrosis (CRN). Consanguinity was found in 6 patients. The diagnosis of cortico-resistant idiopathic nephrotic syndrome (CRINS) was suggested by oedema in all cases. Two patients had a transitory high blood pressure and 5 others had a macroscopic hematuria associated with oedema. Laboratory studies confirmed the diagnosis of nephrotic syndrome, which was severe in 10 cases, and revealed a transitory renal insufficiency in 5 cases, a hyperlipidemia in 3 cases and signs of hyperaldosteronism in 12 cases. Renal biopsy practised in all patients shaved small glomerular lesions in 6 cases, a focal and segmental hyalinosis in 8 cases, one of which was associated with chronic tubulo-interstitial lesions. Treatment was based on alkylant agents in 11 cases (cyclophasplamide in 6 cases and chlorambucil in 5 cases) and ciclosparine in 6 cases. 4 years later on average, the course was marked by complete remission in one case, relapse of the nephrotic syndrome in 3 cases, persistant CRINS without end-stage renal failure in 4 cases and progression to end-stage renal failure in 4 cases.

[Cystic fibrosis of the child]. / La mucoviscidose chez l'enfant: expérience d'un service tunisien de pédiatrie générale.

Cystic fibrosis was regarded a long time as exceptional in the Nord Africaine population and in particular in Tunisia what was at the origin of the ignorance of its various diagnostic and therapeutic aspects in our country. Nevertheless, with the development of the means of the diagnosis, several cases of cystic fibrosis were diagnosed these last years what will pose true problems of assumption of responsibility of these children like illustrates it well our experiment in the pediatric department of Sfax university hospital. In 10 years going of 1991 to 2000 we reported 7 cases of cystic fibrosis in the pediatric department of Sfax university hospital. Our patients are 3 boys and 4 girls. The age of revelation of the disease varied from 3 months to 14 years with 4 years and 10 months an average age. All our patients had a respiratory symptomatology in the foreground. The evolution was marked by the death of 4 patients at an average age of 5 years and half whereas the 3 surviving patients for the moment are more or less balanced on the nutritional level, digestive and respiratory. Cystic fibrosis is not exceptional in our area, its assumption of responsibility therapeutic is difficult, and it requires the collaboration of several experts and a good compliance of the child and of his family.

[Inhaled corticosteroids in idiopathic pulmonary hemosiderosis: 2 cases]. / Intérêt de la corticothérapie inhalée dans le traitement de l'hémosidérose pulmonaire idiopathique. A propos de deux cas.

Idiopathic pulmonary hemosiderosis, a rare clinical entity, is characterized by bleeding into the alveoli and progressive pulmonary fibrosis. It is usually treated with systemic corticosteriod during acute bleeding episodes and with prolonged courses of oral corticosteroids and/or other immunosuppressant drugs for maintenance therapy. Because prolonged treatment with these agents is frequently associated with significant side effects, favorable response to inhaled corticosteroids would be ideal in this condition. The authors reports 2 cases of idiopathic pulmonary hemosiderosis diagnoses in the pediatric department of Sfax: a 20 month old boy and an 8 year old girl successfully treated with inhaled beclomethazone dipropionate using doses of 500_g/day and low dose oral prednisolone (0.5 mg/kg one day/2).

[Congenital lobular emphysema. Eight case reports]. / L'emphyseme lobaire congenital. A propos de 8 cas.

We have conducted a retrospective study about 8 infants having CLE and who were hospitalised for 11 years in the Pediatric department of Sfax university hospital (1989-1999). The average age of these patients having revealing symptoms ranges from birth to 8 months, with an average age of 2 months and 3 weeks. During the neo-natal period (< 1 month), the disease was found among 35.5% of the patients. The discovery circumstances are represented by a permanent dyspnea in 4 cases, repetitive bronchopneumopathies with paroxystic dyspnea in 2 cases, a prolonged bronchopneumopathy in one case and a whooping cough in one case. The pre-operatory diagnosis was suspected on the chest-radiography in all cases and on the chest scanner in 7 cases. All patients have undergone a surgical treatment. The anatomy-pathological exam has confirmed the diagnosis in all cases. The immediate post-operatory results were simple in all the 8 cases and the long-term evolution has shown minor respiratory and orthopedic defects only in one patient aged 8 at present. The CLE is a lung-malformation often responsible for serious respiratory problems. The symptomatic forms should be operated very early because the ulterior "prognosis" depends on the patient's age at the moment of the surgery.

[Persistent forms of cow's milk allergy. Report of 6 cases]. / Les formes persistantes de l'allergie aux protéines du lait de vache. A propos de 6 cas.

BACKGROUND: Cow's milk allergy is defined as reproducible adverse reaction to a food protein antigen which is immune medieted. About 80 to 90 % become clinically tolerated within the first three year of live. The aim of this study is to evaluate the clinical, immunological and evolutive characteristics of the 10 % to 20 % of persistent form of cow's milk allergy. METHODS: This study included six persistent form of cow's milk allergy (2 boys and 4 girls). The provocation challenge test according to Rance recommendation, have been made from 6 months to one year. RESULTS: 4/6 infants with persistent cow's milk allergy have an atopic familial disease. Clinical presentation changed over time at once symptoms were preventely gastrointestinal, at the end there was an increased frequency of atopic disease (asthma: 4/6 infants) CONCLUSION: Persistent form of cow's milk allergy are characterized by considerable importance of familial atopic disease; change in cow's milk allergy manifestations over time and more prolonged delay between cow's milk persistent consumption and manifestations of symptoms.

[Cyclosporine A in the treatment of autoimmune hepatitis in the child: a case report]. / Ciclosporine a dans le traitement de l'hépatite auto-immune de l'enfant: à propos de 1 cas.

UNLABELLED: The current immunosuppressive treatment of patients with autoimmune hepatitis consists of prednisone and azathioprine. High doses of prednisone used to obtain the remission of the disease are associated with serious adverse effects. To avoid harmful consequences of prednisone, we proposed to treat patients with oral cyclosporine to obtain the remission of the inflammatory process. CONCLUSION: This case and review of the cases in the literature show that cyclosporine can induce remission of liver disease in children with autoimmune hepatitis, with few and well tolerated adverse effects.

[Hypothyroidism in children. 82 cases]. / Les hypothyroïdies de l'enfant. A propos de 82 cas.

The hypothyroidism is a frequent endocrinopathos. The authors report a retrospective study of 67 cases gathered at pediatric department of Sfax, during 23 years. 40 boys and 42 girls aged from 1 to 164 months, with an average of 55 months old children. The most clinical symptoms have been the dysmorphic syndrome (81.17%), Huge tongue (59.75%), the T3 is low in 30.48%, the FT4 or the T4 is low in 79% and the TSH is high in 84.14%. All the patients have benefitted a treatment based on thyroidian extract from 1977 to 1984, and since 1985 it was based on L. Thyroxin. The neuropsychiatric prognosis satisfactory in 72% and 28% have a mental delay and 56% a delay in growth. The authors insist on the necessity of doing an early diagnosis and treatment which is the only way and issue leading to a favorable neuropsychiatric evolution.

[Delayed growth due to growth hormone deficiency (study of 16 cases)]. / Retard de croissance par déficit en hormone de croissance (étude de 16 observations).

Growth hormone deficiency is one of the scarce statural backward causes. It is difficult to make a diagnosis. The purpose of growth hormone treatment is to reach a final normal height and to avoid hypoglycemia after-effects. We give a retrospective account of 16 children (11 boys and 5 girls) who have a growth-delay due to a total growth hormone deficiency confirmed by the stimulation tests and who have also been given benefit of a biosynthetic growth hormone from 1990 to 1999. The statural backwardness varies from--2.5 DS to--4 DS, with an average of--3.5 DS. In all the cases it is a matter of a harmonious backwardness discovered at an average age of 6 years. The bone age has revealed an important backwardness of bone maturation: average bone age of 3 years for boys and 4 years for girls. The hormone balance sheet reveals, in all the cases, a total growth hormone deficiency (GH < 5 ng/ml) combined with a corticotrope deficiency (2 cases) and thyreotrope (3 cases). It is a growth hormone idiopathic deficiency among our patients. The growth hormone treatment has been administered at the average age of 8 years. The weekly doses were (0.4 to 0.8 U/kg). The evolution was favorable with an average growth speed that has gone up from 3 cm/year before the treatment to 10 cm in the first year of the treatment and to 5.5 cm during the second year of the treatment. An average statural gain of 0.8 DS and a bone maturation gain of one year over one year treatment. The authors put into relief the importance of diagnosis criteria of growth backwardness through a GH deficiency and suggest a therapeutic diagram, and a follow-up of the GH biosynthetic treatment.