Susceptibility to coeliac disease in Tunisian children and GM immunoglobulin allotypes.

Coeliac disease is a malabsorption disorder of the small intestine resulting from ingestion of gluten. The immunogenetic component is clearly demonstrated by the association of the disease with human leukocyte antigens (HLA). Among other candidate genes are the GM allotypes, which are the markers of the constant parts of heavy chains of the subclasses IgG1, IgG2 and IgG3.

[Breast feeding effect relative to age of onset of celiac disease].

Background: Age at onset and clinical presentation of celiac disease have often been related to the age of gluten introduction into the diet. It has also been shown that breast feeding delays the onset of the disease.

Patients And Methods: This retrospective study attempts to evaluate the respective contributions of these two parameters in the determination of the age at onset of the symptoms in celiac Tunisian children.

[Clinical, therapeutic and developmental aspects of acute bronchiolitis in Tunisia].

Fourteen infants with severe acute bronchiolitis were admitted to the Intensive Care Unit (ICU) of Tunis. This pathology represents 36% of severe bronchopulmonary infections admitted to this ICU. Their age ranged between 2 and 48 weeks (mean: 15 weeks).

High risk genotypes for celiac disease.

It is known that celiac disease is strongly associated with an HLA class II component and that most patients carry the dimer DQA1*0501, DQB1*0201. We show in this study that the risk for a carrier of this heterodimer is independent from the number of possible heterodimers, from whether DQA1*0501 and DQB1*0201 are in cis or trans position and from the number of DQA1*0501 (one or two) but strongly depends on the number of DQB1*0201. In the Tunisian population we studied, the risk of developing celiac disease is estimated to be 6.

[Hepatic hydatidosis and portal hypertension in children. Is it the Budd-Chiari syndrome?].

A case of portal hypertension in a five-year-old with multiple hydatid cysts in the liver is reported. Compression of the portal vein was the likely mechanism. Abdominal ultrasonography disclosed four hydatid cysts of which the largest was located in segments VII and VIII and caused compression of the supra-hepatic veins and inferior vena cava.

[Skull fracture with progressive separation. Apropos of a case].

A case of fracture of the skull with progressive separation of the fracture line in a seven-month-old is reported. The patient presented with a swelling in the right parieto-occipital area and paresis of the upper left limb; there was no clear history of trauma. The roentgenogram of the skull disclosed a large bone defect and the cerebral CT scan showed herniation of intracranial contents through this defect, confirming the diagnosis of fracture of the skull with progressive separation.

[Congenital sensory neuropathy with anhidrosis: type IV. Apropos of 2 new cases].

Two new cases of congenital sensory neuropathy (CSN) type IV in brothers aged 10 and 5 years are reported. Features included diffuse lack of response to pain without loss of response to touch, temperature and proprioceptive stimuli. No other neurologic anomalies were found.

[Genetic study of congenital afibrinogenemia. Review of 12 cases].

Twelve cases of congenital afibrinogenemia in 11 families are reported. A family study was performed in six cases. The parents were genetically related in 8 of the 11 families.

[Hemiballism. Description of a clinical case in childhood].

Biballism is an infrequent hyperkinetic disorder characterized by involuntary, intermittent, violent, uncontrollable contractions of the proximal muscles of the limbs. Biballism is classically ascribed to a lesion in the controlateral subthalamic nucleus or its connections but other causes have been reported. These include infections (bacterial, viral parasitic), cerebrovascular lesions, tumors, toxics, and systemic disease (systemic lupus erythematosus).

[Budd-Chiari syndrome in children. Apropos of 7 cases].

Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients.

[Interstitial nephropathy, tapeto-retinal degeneration and cataract. Apropos of a new case].

A case of chronic interstitial renal disease is reported. Onset was manifested at the age of three by polyuria and polydipsia. The child was hospitalized at the age of eleven for renal failure and tapetoretinal degenerescence with cataract were found.

[Thalassemia intermedia. Report of two cases].

We report two cases of intermediate beta-thalassemia diagnosed at the age of 2 years and 3 1/2 years respectively. Characteristic features of this disease include delayed onset, moderate blood transfusion requirements, and frequent development of hypersplenism. Major iron overload develops even in patients who have received no transfusions.

[Weaver's syndrome. Apropos of a new case].

We report a new case of Weaver syndrome in a male infant. This clinical entity is rare and was first described in 1974. Patients exhibit accelerated growth and skeletal maturation, craniofacial dysmorphism, and widening of the distal femoral metaphyses.

[Severe forms of liver involvement in visceral leishmaniasis. Apropos of 7 cases].

In a series of 180 cases of Kala-azar, hepatic involvement was found in 16 patients. The authors report 7 cases of severe hepatitis with cytolysis, cholestasis and liver failure. These patients presented with high triglyceride, low cholesterol and low alpha-lipoprotein blood levels.

[Ocular toxocariasis. Apropos of a case].

We report a case of ocular toxocariasis characterized by the global involvement of both eyes. Ophthalmologic evaluation demonstrated involvement of the cornea, iris and lens, an inflammatory granuloma, and hyalitis. Immunologic tests were negative in the serum and positive in the aqueous humor.

[Comparative effects of couscous and pasta on glycemia in normal subjects and type I diabetics].

8 healthy subjects have eaten in the morning, after an overnight fast, in two separated occasions and in a randomised order 50 gr of CHO as pasta or couscous. Blood glucose after pasta ingestion was lower at 30 mn (p less than 0.05) at 45 mn (p less than 0.

[Waardenburg syndrome. Report of a familial case].

We report a familial case of Waardenburg syndrome. A four and a half year old boy had displacement of the canthi, a white forelock, perceptive deafness, and a congenital heart defect. The mother and younger brother had the same syndrome.

[Accidental ingestion of caustics in Tunisian children. Report of 125 cases].

The authors reviewed 125 cases of accidental ingestion of caustic substances admitted to a general pediatrics department over the last four years. L'eau de Javel (bleaching agent with sodium hypochloride) was the most frequently encountered caustic substance (89%). Esophagogastric fibroscopy was performed in 100 cases and esophageal lesions were classified according to tree grades of severity.