Delayed growth due to growth hormone deficiency (study of 16 cases)

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.     

Community-acquired poliovirus infection in children with primary immunodeficiencies in Tunisia

The global polio eradication program recommends the use of massive vaccination campaigns with live vaccine through National Immunization Days (NIDs) to displace the wild virus from the community. Immunodeficient patients may be indirectly infected and become chronic excretors and potential reservoirs of polioviruses, a concern for the posteradication era. This prospective study aimed to assess the risk of community-acquired infection of immunodeficient patients following NIDs, the dynamics of viral excretion and the genetic variation of excreted viruses. Sixteen children with various primary immunodeficiencies, who did not receive the vaccine during the campaign, were investigated. Stool samples were collected weekly, shortly after the NIDs, during at least 3 months, and were processed for viral isolation. Isolates were characterized by three intratypic differentiation methods and partial sequencing of the VP1/2A region. Polioviruses were detected in 4 out of 16 patients (serotype 1 in 3 patients and serotype 3 in 1 patient). Sequencing revealed more than 99% homology with homotypic Sabin strains, suggesting recent infection. Duration of viral excretion ranged from 1 to 7 weeks. Nine out of eleven isolates from the three poliovirus serotype 1-infected patients disclosed a non-Sabin-like phenotype by enzyme-linked immunosorbent assay and had recurrent mutations within or close to the neutralizing antigenic sites. In summary, the risk of secondary infection in immunodeficient patients is within the range previously reported for the general population. Although none of the four infected patients developed prolonged viral excretion, particular viral variants were selected and may be of epidemiological significance.     

A huge cardiac hydatid cyst: An unusual cause of chest pain revealing multivisceral hydatidosis in a young woman

Hydatid disease remains endemic in some parts of the world. Cardiac hydatidosis with multivisceral involvement is uncommon but potentially fatal. We report the case of a 36-year-old Tunisian woman admitted with chest pain and T-wave inversion in the inferior leads on her electrocardiogram. Transthoracic echocardiography revealed a large hydatid cyst in the epicardium throughout the left ventricle. Thoraco-abdominal computerized tomography (CT) scan showed several hydatid cysts in the left lung, the liver, and in both breasts.After one week of albendazole treatment, surgical excision of the cardiac cyst on cardiopulmonary bypass was carried out as well as excision of the pulmonary and breast cysts. The postoperative course was uneventful and albendazole treatment was continued for six months. Though hydatid cardiac involvement is very rare, it should be considered in the differential diagnosis of atypical chest pain in young patients, especially those living in regions where hydatid disease is endemic.     

Contrasting patterns of hepatitis C virus infection in two regions from Tunisia

This report is a population-based study describing the pattern of hepatitis C virus (HCV) infection in two distinct regions in Tunisia. The study included a total of 11,507 individuals sampled in 1996 from both genders, all age groups, urban and rural settings belonging to 2,973 families. HCV infection was assessed by commercial enzyme immunoassay (EIA) and immunoblot assays and detection of HCV RNA by PCR. HCV genotypes and subtypes were determined by sequencing in the 5'-untranslated region (UTR) viral genomic region and the INNO-LiPA HCVII genotyping kit. Genetic relatedness between HCV strains was assessed by sequencing of a portion of the NS5B region. HCV prevalence was significantly higher in the North-Western region than in the Southern one: 1.7% versus 0.2% (P < 10(-3), chi(2) = 8,506). There was no difference in positivity according to gender or living in rural or urban settings; the only significant risk factor was advanced age. HCV prevalence among household contacts of HCV positives was not significantly higher than the prevalence in the whole study population. These results indicate a heterogeneity in the geographical distribution of HCV in Tunisia. An increased HCV transmission occurs in the North-Western region with large predominance of genotype 1b (88%) and low contribution of intrafamilial transmission.     

Hemopigmented villonodular synovitis of the knee. Apropos of 2 cases

We report two cases of hemopigmented villonodular synovitis in two girls aged 5 years 9 months and 13 years respectively. This condition is a clinicopathologic entity of unknown pathogenesis and is exceedingly rare in childhood. Patients of both sexes can be affected, with no particular predominance. The clinical picture in our two patients consisted only in chronic hemarthrosis of the knee with occasionally tumor-like features. Arthrographic changes were not specific. Diagnosis was established upon arthrography and results of pathologic studies of biopsy specimens. Therapy of this condition is surgical and recurrences are infrequent.     

Clinical, biological and genetic study of 24 patients with ataxia telangiectasia from southern Tunisia

Ataxia telangiectasia is a multisystem disease with an autosomal recessive inheritance. It is characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, humoral and cellular immunodeficiencies and high incidence of neoplasia and radiosensitivity. A 5 year retrospective survey included 24 patients belonging to 17 families. Cerebellar ataxia was the first clinical symptom and was usually noticed when the child began to walk. Mean age of onset was 2.9+/-1.8 years. Oculocutaneous telangiectasia was present in 17 cases and appeared between 2 and 8 years and then spread in a characteristic symmetrical pattern. When ocular telangiectasia was absent (6 cases), the diagnostic of ataxia telangiectasia was retained on oculomotor apraxia (2 cases), recurrent sinopulmonary infections (3 cases) and/or a sib with typical ataxia telangiectasia (1 case). Recurrent sinopulmonary infections, absence or low serum level of IgA (78 p.100) and lymphopenia revealed immunodeficiency. Among 12 patients, chromosomal instability was observed in 5. Balanced rearrangements involving chromosomes 2, 7, 14, 22, 1, 3 and 11. The responsible gene, ATM, encodes a large protein kinase with a phosphatidylinositol 3-kinase-like domain. Ataxia telangiectasia patients have a 100 fold higher risk of cancer than the general population. We reported, in the same family two patients who developed neoplasia, (lymphoma and leukemia). During follow-up, a progressive worsening was observed in all cases. Three patients have died.     

Rôle du virus Toscana dans les infections neuroméningées en Tunisie

Objectives

To detect the presence of Toscana virus (TOSV) circulation in Tunisia and to study its role in viral meningo-encephalitis.

Patients and methods

A total of 315 (167 sera and 178 cerobrospinal fluid [CSF]) samples was investigated. These samples are colleted from Tunisian patients with neurological diseases during the period between January 2003 and December 2009. All samples were tested negative for enterovirus, Herpes Simplex virus and West Nile virus. Detection for IgM and IgG specific to TOSV was done by ELISA tests.

Results

Specific IgM for TOSV were detected in 10 % of patients with neurological diseases (31 cases). These recent infections were distributed throughout the study period and predominated during summer and automn. Patients were originated, in the majority from the coastal region. IgG were isolated in 22 cases (7 %) corresponding to previous infection.

Conclusion

This is the first report of TOSV circulating in Tunisia and its frequent implication in neurological diseases. These results incited to include TOSV as one of the viral etiologies to target in the diagnosis of viral meningitis and encephalitis in the country.     

Is It possible to recognize pulmonary infarction on multisection CT images?

PURPOSE: To retrospectively determine sensitivity and specificity of four findings for distinguishing pulmonary infarction from other causes of peripheral pulmonary consolidations on multidetector computed tomographic (CT) images, with other CT and clinical findings as reference. MATERIALS AND METHODS: Institutional review board approved the study and waived informed consent. Three independent radiologists blindly analyzed selected multisection CT images of 50 pulmonary infarctions-not showing direct arterial signs of pulmonary embolism-and 100 peripheral consolidations of other origins. Readers analyzed four findings: triangular shape, vessel sign (defined as presence of an enlarged vessel at the apex of consolidation), central lucencies, and air bronchograms. Interobserver agreement; frequency on CT images with and without infarct; and sensitivity, specificity, and positive likelihood ratio (LR) for diagnosis of pulmonary infarction were assessed for each finding. RESULTS: One hundred fifty peripheral consolidations were analyzed in 134 (75 men, 59 women) patients (mean age, 55.9 years+/-17.4 [standard deviation] vs 54.7+/-19.9; P=.71). Interobserver agreement was good for central lucencies and air bronchograms and poor to moderate for the other two findings (kappa<0.61). Compared with CT images without infarct, CT images with infarct had a higher frequency of vessel sign (32% [16 of 50] vs 11% [11 of 100], P=.029) and central lucencies (46% [23 of 50] vs 2% [two of 100], P<.001) and a lower frequency of air bronchograms (8% [four of 50] vs 40% [40 of 100], P=.003). Frequency of triangular shape was similar in both groups (52% [26 of 50] vs 40% [40 of 100], P=.17). Positive LR was 23.0 for central lucencies, 2.9 for vessel sign, 1.3 for triangular shape, and 0.2 for air bronchograms. Presence of central lucencies had 98% specificity and 46% sensitivity for pulmonary infarction. When the vessel sign and negative air bronchogram were combined with central lucencies, specificity increased to 99% but sensitivity decreased to 14%. CONCLUSION: Central lucencies in peripheral consolidations are highly suggestive of pulmonary infarction.