[Clinico-biological and immunohaematological profile of patients with ß-thalassemia in Tunisia: about 26 cases]. / Profil clinico-biologique et immunohématologique des patients atteints de ß-thalassémie en Tunisie : à propos de 26 cas.

AIM OF THE STUDY: To study the clinical and biological profile of ß-thalassemic patients in our region, reflecting the quality of their care. PATIENTS AND METHODS: A retrospective study (2010-2011) on 26 ß-thalassemic patients followed in the pediatrics service at CHU Farhat Hached Sousse, Tunisia. Epidemiological, clinical and biological data were collected from medical records and transfusion files of patients. The transfusion protocol adopted was to maintain a hemoglobin level>10g/dL by regular transfusions every 3-4 weeks. Iron chelation therapy, in order to maintain serum ferritin<1500ng/mL, was introduced when serum ferritin exceeded 800-1000ng/mL. RESULTS: The mean age of patients at diagnosis was 15 months. The clinical impact of anemia had resulted in failure to thrive in 54% of patients and facial dysmorphism in 23%. The average transfusion requirement was estimated at 311.02mL/kg/year with 6 cases of hyperconsumption. The immunohaematological monitoring showed the appearance of anti-RBC alloimmunization in one patient and 4 cases of autoimmunization. Poor adherence of chelation therapy was 62% and causing 5 cases of cardiac complications, 4 cases of liver injury and 14 cases of endocrine complications. CONCLUSION: Improving the therapeutic care of ß-thalassemic children requires better monitoring of transfusion recovery and improved adherence to chelation therapy.

[Tongue base schwannoma in a child]. / Schwannome de la base de la langue chez l'enfant.

INTRODUCTION: A schwannoma is a benign slow growing tumor originating from the peripheral nerve sheath. Its tongue base location is extremely rare, especially in very young patients. CASE REPORT: A 15-year-old female patient was admitted in emergency for moderate hematemesis. The clinical examination revealed swelling of the tongue base, causing the bleeding. This lesion had appeared when she was 3years of age and no treatment was done. A complete surgical excision was performed and the histopathological examination allowed diagnosing a tongue base schwannoma. The postoperative outcome was uneventful, and no recurrence was detected 6months after surgery. DISCUSSION: The particularities of this report are the patient's very young age and the tumor's tongue base location. The prognosis is usually good after complete excision, if there is only one tumor. Type 2 neurofibromatosis should be excluded, given the risk of malignant degeneration.

[Comparative study of C-reactive protein and procalcitonin in the severity diagnosis of pyelonephritis in children]. / Étude comparative de la protéine C-réactive et de la procalcitonine dans le diagnostic de sévérité des pyélonéphrites aiguës de l'enfant.

OBJECTIVE: The aim of this study is to compare two biologic parameters; C-reactive protein (CRP) and procalcitonin (PCT) in the detection of acute renal lesions assessed by DMSA scintigraphy in the urinary tract infection in child. DESIGN: In a prospective study, serum PCT, CRP and leukocyte counts were measured for children admitted, between January and December 2010, with a first episode of febrile urinary tract infection. RESULTS: Seventy-five children were enrolled in the study. Thirty-three patients had renal lesions (group A) and 42 had a normal DMSA scintigraphy (group B). The mean PCT level was significantly higher in group A than in group B (8.81 ng/mL versus 1.7 ng/mL, P=0.01). In this study, using receiver operating characteristic (ROC) curve, we identified that the optimal cut-off value with ideal sensitivity and specificity for PCT in detection of renal lesions was 0.76 ng/mL and for CRP, it was 70 mg/L. The sensitivity, the negative predictive value and the indice of Youden of the cut-off value of PCT were significantly higher than CRP (82% versus 70%; 84% versus 70% and 0.58 versus 0.25). CONCLUSIONS: This study confirmed that the serum PCT level was more sensitive and specific than the CRP in the detection of renal lesions in the first urinary tract infection in child.

Cervical unicentric Castleman's disease in children.

INTRODUCTION: Unicentric Castleman's disease (CD) is a rare benign lymphoproliferative disorder. A cervical location has rarely been reported in the pediatric literature. CASE REPORT: A 13-year-old boy presented with a mass in the right neck region of 3 months' evolution. Clinical examination revealed a smooth mobile 5 cm node deep in the sternocleidomastoid muscle. The diagnosis of hyaline vascular type CD was confirmed by complete surgical excision and histopathologic examination. Postoperative course was simple, without recurrence over 1 year's follow-up. DISCUSSION AND CONCLUSION: Cervically located CD often shows few symptoms, and is rarely suggested by a cervical mass found in children. Definitive diagnosis is histopathological. The hyaline vascular type of unicentric CD has a good prognosis after complete resection of the lesion.

[Hepatopulmonary syndrome: a complication of type 1 Gaucher disease]. / Syndrome hépatopulmonaire: une complication de la maladie de Gaucher de type 1.

Gaucher's disease is a not exceptional lysosomial disease in Tunisia. Type 1 is by far the most common one. Pulmonary involvement is considered to be rare in type 1 Gaucher's disease. Pulmonary hypertension, infiltration of the lungs with Gaucher cells, and severe hypoxemia due to intrapulmonary arterial-venous shunts, have been described in case reports and small case series. We reported the case of hepatopulmonary syndrome in a 14-year-old boy with type 1 Gaucher disease. The diagnosis of Gaucher disease was established, at 2 years age, by enzyme assay of leucocyte ß-glucosidase. The patient presented dyspnoea, digital clubbing and cyanosis of the lips. The arterial blood gas found severe hypoxaemia with PaO(2) at 56.9 mmHg. The diagnosis of hepatopulmonary syndrome, in our patient, was confirmed by demonstration of the intrapulmonary shunting using contrast-enhanced echocardiography and the technetium-99m-labeled macroaggregated albumin. The patient was treated by symptomatic measure, long term oxygen therapy because the insufficiency of the enzyme replacement therapy. Screening for hypoxemia in children with liver disease should be considered.

[Seroprevalence and risk factors of human herpes virus 8 infection in Central-East Tunisia]. / Séroprévalence et facteurs de risque de l'infection par le virus herpès humain 8 dans le Centre-Est tunisien.

OBJECTIVE: Epidemiology of human herpesvirus 8 (HHV8) is still unknown in Tunisia. We aimed to assess the prevalence of HHV8 infection in adults and children from Central-East Tunisia and in patients with high risk of parenteral or sexual infection. METHODS: We enrolled 553 subjects: 116 blood donors, 100 pregnant women, 100 children, 50 subjects with sexually transmitted infections with positive HIV serology and 50 other without HIV infection, 107 multitransfused patients and 30 kidney transplant patients. Antibodies against HHV8 were tested using a sensitive indirect immunofluorescence assay. RESULTS: The seroprevalence of HHV8 was found to be 13.8% in blood donors, 13% in pregnant women and 12% in children. In healthy adult population, no association was found between HHV8 seropositivity and sex, sociodemographic characteristics, parenteral risk factors or serological markers of hepatitis B. Rates of HHV8 infection were significantly higher in patients having high-risk sexual behavior with or without HIV infection (P<10(-4)), in polytransfused patients (P<10(-4)) and in patients with kidney transplantation (P=0.001). CONCLUSION: Our findings suggest that HHV8 infection is widespread in Central-East Tunisia such as in the Mediterranean area. HHV8 infection appears to be acquired early in life, probably through saliva. HHV8 transmission by blood transfusion, subject of controversy in literature, is well established in our study. Early screening of this infection should be considered in populations with high risk of Kaposi's sarcoma in our areas.

Digestive malacoplakia in children: case report.

Malacoplakia is a form of chronic granulomatous inflammatory reaction that rarely affects the pediatric age group. The gastrointestinal system is the second most common site for the occurrence of malacoplakia. We report the case of a 9-year-old girl who was hospitalized for abdominal pain, chronic diarrhea, and rectal hemorrhage. The endoscopic examinations and histopathology confirmed the diagnosis of intestinal malacoplakia. We successfully treated her with oral levofloxacin. This disease does not have any specific clinical or biological signs, and the diagnosis is exclusively based on histology.

[Viral infection risk in polytransfused adults: seroprevalence of seven viruses in central Tunisia]. / Le risque infectieux viral chez le polytransfusé : séroprévalence de sept agents viraux dans le centre tunisien.

The aim of this study is to evaluate the prevalence of seven transfusion-transmitted viruses in polytransfused adults and children comparatively with a group of healthy control subjects. We studied 107 polytransfused patients (59 adults and 48 children) and 160 control subjects (100 blood donors and 60 children). Immunoenzymatic tests were used for detection of HBs antigen (HBs Ag), antibodies against hepatitis C Virus (anti-HCV), and human immunodeficiency virus (anti-HIV), and IgG antibodies against human cytomegalovirus (IgG anti-CMV), human parvovirus B19 (IgG anti-PB19), and hepatitis E virus (IgG anti-HEV). An immunofluorescent assay was performed for the detection of human herpesvirus 8 antibodies (anti-HHV8). Prevalence of HBs Ag, anti-HCV, anti-HIV, IgG anti-CMV, IgG anti-PB19, IgG anti-HEV, and anti-HHV8 in polytransfused group was 8.4, 4.7, 0, 86.9, 60.7, 28.9, and 47.6%, respectively, and 1.8, 0.6, 0, 86.2, 53.1, 10, and 12.5%, respectively, in the control group. The difference in prevalence between the two groups was statistically significant for HBs Ag (P = 0.01), anti-HCV (P = 0.03), IgG anti-HEV (P < 10(-4)), and IgG anti-HHV8 (P < 10(-4)). Categorization according to age showed that hepatitis B and C risk was limited in adult polytransfused group. HHV8 infection was higher in polytransfused subjects born before the use of leucocyte-depleted blood components. Our results corroborate literature data on the risk of HEV and HHV8 infection by blood transfusion. Hepatitis B vaccination and improvement in screening tests have an important role in reduction of hepatitis B and C risk in transfusion, especially in young polytransfused persons. However, a residual risk of transmitting viral infections persists, and efforts are needed to improve transfusion safety.

[Epidemiological and bacteriological characteristics of uropathogen bacteria isolated in a pediatric environment]. / Caractéristiques épidémiologiques et bactériologiques des bactéries uropathogènes isolées dans un milieu pédiatrique.

Urinary tract infection (UTI) in children is a grave pathology, which requires a fast and effective care. Bacteriological and epidemiological data play a determining role in patient's care. We report a retrospective study, which spreads out from January 1st till August 31st, 2009, having concerned hospitalized children for urinary infection in pediatrics service of Farhat Hached teaching hospital in Sousse. Our series contained 51 children with a sex ratio of 0.76, an average age of 32 months. The majority of cases was pyelonephritis (94.1%). A pathology is associated with the urinary infection in 41.2%. Three cases of vesico-ureteral reflux were noted. The diagnosis of urinary infection was confirmed by cytobacteriological exam of urine (CBEU). Enterobacteriacea were isolated in 96.1%. Escherichia coli remains the most often isolated (80.4%), followed by Klebsiella pneumoniae (9.8%) then by Proteus mirabilis (5.9%). E. coli was resistant to amoxicillin in 78% of cases, to the association amoxicillin-clavulanic acid in 64,8%, to cephalosporins of 3(rd) generation (C3G) in 5% and to cotrimoxazole in 51%. No K. pneumoniae or P. mirabilis strain was resistant to C3G. UTI in children is always an indication for CBEU realization at first intention. The results of this exam are very important considering the diversity of the responsible bacteria and the growing frequency of acquired antibiotic resistance.