No Correlation between Anti-drug Antibodies and Therapeutic Response in Tunisian Patients with Chronic Inflammatory Diseases Treated by TNF Blockers.

INTRODUCTION: Tumor necrosis factor alpha (TNF alpha) blockers such as infliximab (IFX) and adalimumab (ADA) had significantly changed the course of inflammatory diseases such as rheumatoid arthritis (RA), spondyloarthritis (SpA) and Crohn's disease (CD). However, about 30% of patients do not respond to these treatments. This lack of response may be due to the formation of antibodies against these drugs (anti-drug antibodies: ADAbs). The aim of this study was to determine the prevalence of ADAbs against IFX and ADA, and the trough serum concentration of IFX and ADA in RA, SpA or CD patients and to assess their impact on the therapeutic response. METHODS: A cross sectional, multi-centric study was conducted, including patients with RA, SpA or CD treated with IFX or ADA as a first biotherapy for at least 6 months. ADAbs and trough levels were measured by an Enzyme Linked Immunosorbent assay (ELISA). RESULTS: 197 patients were included (57 RA, 73 SpA and 67 CD). ADAbs were positive in 40% of cases for IFX and 25% for ADA. They were positive in 40% of SpA, 35% of RA, and 21% of CD. The presence of ADAbs was inversely correlated to the trough levels of IFX and ADA during RA (p = 0.01 and p < 0.0001), SpA (p < 0.01 and p < 0.0001) and CD (p = 0.001 and p = 0.04). For all pathologies, the presence of ADAbs was not correlated with disease activity. Concomitant methotrexate significantly reduced immunogenicity. CONCLUSION: In our study, the presence of ADAb and low trough levels seem to not affect the therapeutic response in patients on TNF alpha antagonists. Other tracks more than immunogenicity should be investigated to explain the loss of response to these biotherapies.

Fetal Cyst Reveling Retroperitoneal Enteric Duplication.

Retroperitoneum is a very uncommon site of enteric duplication (ED). We report a new case of retroperitoneal ED cyst suspected in utero. Prenatal ultrasound showed an abdominal cystic mass. Noncommunicating retroperitoneal ED cyst measuring 70 mm × 30 mm was resected. Histopathologic examination confirmed the diagnosis.

Prevalence and outcomes of thrombocytopenia in a neonatal intensive care unit.

Background Thrombocytopenia is a common clinical problem in neonatal intensive care units, affecting about 20 to 35% of all admitted neonates. Even most episodes are mild or moderate, severe episodes could be life-threatening or responsible for sequelae. Objectives The aims of this study were to describe the prevalence, clinical diagnoses, and to determine risk factors for poor prognosis of thrombocytopenia in a neonatal intensive care unit. Methods We carried out a retrospective study in the neonatal intensive care unit of Charles Nicolle Hospital of Tunis, a tertiary neonatal care center, over a four years period (January 2010 to December 2013). All Neonates with at least one episode of confirmed thrombocytopenia were included. Poor prognosis was defined as death or intraventricular hemorrhage ≥ grade 2 in survivors. Results Of 808 admitted neonates, one hundred (12.4%) had presented at least one episode of confirmed thrombocytopenia, and 12 had presented two episodes of thrombocytopenia. A total of 112 episodes of thrombocytopenia were collected. Thrombocytopenia occurred in the first 3 days of life in 74.1% of cases. Thrombocytopenia was mild in 22.3%, moderate in 36.7% and severe in 41%. Intrauterine growth restriction was the most common cause of early thrombocytopenia. Nosocomial sepsis was the most common cause of late thrombocytopenia. We found that the outcomes of thrombocytopenic neonates depend on, birth weight, gestational age, platelet count, and the underlying cause. Conclusions Thrombocytopenia in neonates can be life-threatening, appropriate diagnosis, preventive and therapeutic approach is necessary to prevent death or neurological impairment.

Prevalence of vitamin D deficiency in mothers and their newborns in a Tunisian population.

OBJECTIVE: To assess vitamin D status in mothers and their newborns and identify predictive factors of vitamin D deficiency. METHODS: A cross-sectional study was undertaken of healthy women and their full-term newborns delivered at the Charles Nicolle Hospital, Tunis, Tunisia, between October and November 2012. Maternal and neonatal serum 25-hydroxy vitamin D (25(OH)D) concentrations were measured. Correlations were tested. RESULTS: Overall, 87 mothers and their newborns were enrolled. No mother or neonate had an adequate vitamin D status. Mean maternal and neonatal serum 25(OH)D concentrations were 6.82±5.14ng/mL (range 3.60-23.77) and 5.92±4.15ng/mL (range 3.60-22.28), respectively. Vitamin D deficiency (serum 25(OH)D<20ng/mL) was found in 84 (97%) mothers and 85 (98%) neonates, of whom 76 (87%) and 78 (90%), respectively, had severe deficiency (serum 25(OH)D<12ng/mL). Maternal serum 25(OH)D showed a strong positive correlation with neonatal serum 25(OH)D (r=0.69, P<0.001). Maternal dietary vitamin D intake was the only factor shown to be associated with serum 25(OH)D concentrations (P<0.05). CONCLUSION: Vitamin D deficiency is prevalent among Tunisian mothers and their neonates.

Errors in preparation and administration of parenteral drugs in neonatology: evaluation and corrective actions.

BACKGROUND: The medication iatrogenic risk is quite unevaluated in neonatology Objective: Assessment of errors that occurred during the preparation and administration of injectable medicines in a neonatal unit in order to implement corrective actions to reduce the occurrence of these errors. METHODS: A prospective, observational study was performed in a neonatal unit over a period of one month. The practice of preparing and administering injectable medications were identified through a standardized data collection form. These practices were compared with summaries of the characteristics of each product (RCP) and the bibliography. RESULTS: One hundred preparations were observed of 13 different drugs. 85 errors during preparations and administration steps were detected. These errors were divided into preparation errors in 59% of cases such as changing the dilution protocol (32%), the use of bad solvent (11%) and administration errors in 41% of cases as errors timing of administration (18%) or omission of administration (9%). CONCLUSION: This study showed a high rate of errors during stages of preparation and administration of injectable drugs. In order to optimize the care of newborns and reduce the risk of medication errors, corrective actions have been implemented through the establishment of a quality assurance system which consisted of the development of injectable drugs preparation procedures, the introduction of a labeling system and staff training.

Perinatal-lethal Gaucher disease presenting as hydrops fetalis.

Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis. Less common signs of the disease are hepatosplenomegaly, ichthyosis and arthrogryposis. We report a case of Gaucher's disease (type 2) diagnosed in a newborn who presented with Hydrops Fetalis.

Perinatal lethal type II osteogenesis imperfecta: a case report.

We report a new case of osteogenesis imperfecta (OI) type II which is a perinatal lethal form. First trimester ultrasound didn't identified abnormalities. Second trimester ultrasound showed incurved limbs, narrow chest, with hypomineralization and multiple fractures of ribs and long bones. Parents refused pregnancy termination; they felt that the diagnosis was late. At birth, the newborn presented immediate respiratory distress. Postnatal examination and bone radiography confirmed the diagnosis of OI type IIA. Death occurred on day 25 of life related to respiratory failure.

Isolated anti-Ro/SSA thrombocytopenia: a rare feature of neonatal lupus.

We report a rare case of isolated thrombocytopenia related to anti-Ro/SSA antibodies. The mother was followed for unlabeled familial thrombocytopenia. The mother had positive anti-Ro/SSA antibodies. She was asymptomatic without skin lesions or other criteria neither of systemic lupus erythematosus nor other connective tissue disease. Pregnancy was uneventful. The postnatal examination was normal. On the first day of life, blood cells count showed thrombocytopenia at 40 x 10(9)/L. Within the second day of life, platelet level dropped to 20 x 10(9)/L. The management of thrombocytopenia included platelet transfusion and human immunoglobulin infusion. On the fifth day of life, there has been a drop in platelet count to 10 x 10(9)/L requiring renewed platelet transfusion and human immunoglobulin infusion. On the 10(th) of life platelets rate was stable around 60 x 10(9)/L. The infant had no evidence of cardiac, dermatologic or hepatobilary involvement initially or throughout follow up.

Serum leptin concentration in Tunisian non obese children.

Leptin, an adipocyte-derived peptide hormone, is thought to play a key role in the regulation of body fat mass. Beyond this function, it appears to be an integral component of various hypothalamo-pituitary-endocrine feedback loops. Because childhood and puberty are periods of major metabolic and endocrine changes, we investigated leptin levels in 348 non overweight, non obese children (147 boys, 201 girls, age: 6-12 years) and then correlated these levels with age, anthropometric data, pubertal stage and insulin. A blood sample was collected from each subject to measure leptin and insulin levels by radioimmunoassay. Pubertal stage was assigned by physical examination, according to Tanner criteria for breast development in females and genital development in males. The results showed an increase in leptin levels in an age related way (r = 0.32, p < 0.0001 in girls; r = 0.21, p = 0.011 in boys) following a pattern that paralleled body weight (r = 0.6 in girls; r = 0.56 in boys; p < 0.0001) and BMI (r = 0.59 in girls; r = 0.6 in boys, p < 0.0001), suggesting that body fatness is a regulator of leptin levels in both girls and boys. A significant gender difference (3.39 +/- 2.79 ng/mL in girls vs 1.99 +/- 2.08 ng/mL in boys, p < 0.0001) with an increase during pubertal development in girls was also showed, while the levels remained constant in boys from Tanner stages T1 to T3. A correlation between leptin and insulinemia was noted in girls (r = 0.38, p < 0.0001) but not in boys, suggesting that insulinemia could be a stimulator of leptin synthesis in girls.

[Neonatal prognosis of second twin]. / Pronostic neonatal du deuxieme jumeau.

BACKGROUND: Twin pregnancy is associated to high neonatal morbidity particularly for the second twin. AIM: To assess twin delivery practice in our department and prognosis of second twin. METHODS: Retrospective study of medical files of parturition women with twin pregnancy between January 2003 and December 2006. Were excluded women delivered before 28 weeks gestation, twin pregnancies with death or malformation of one of twins. Descriptive and comparative analyses were realised. RESULTS: One hundred forty six twin pregnancies were counted. Vaginal delivery was attempted with 90 parturition women with 85.5% of success. Caesarean section delivery rate was 47.2%. Overall there's no difference between twins considering neonatal complication. In case of vaginal delivery, the second twin's 5 minutes Apgar score was statistically under the one of the 1st twin if compared to the caesarean section delivery. This difference was no more significant if regarding the Apgar score under 7 at 5 minutes. Apgar score of the second twin was also under the one of the first twin in non cephalic presentation. CONCLUSION: Vaginal delivery of twin pregnancy was noot associated to high risk for twins. Obstetrical manoeuvres should be well controlled to reduce obstetrical trauma.

[Isolated fetal pyelectasis: pathologic significance and therapeutic implications]. / Les pyélectasies anténatales isolées: signification pathologique et implications thérapeutiques.

We report a retrospective study of nine neonatal observations of antenatal isolated pyelectasis during a period of two years. Pyelectasis associated with other congenital abnormalities and in utero died foetus were excluded. Finding interesting sex, gestational age at diagnosis, echographic aspect, antenatal management and postnatal follow-up were assigned. Foetal kidneys was noted in two cases and an oligoamnios was noted in two other cases. No foetal urinary intervention was assessed. Postnatal exploration revealed a transitional pyelectasis in four cases, an ureteropelvic junction obstruction in four cases and a congenital megauretere in one case. Postnatal renal function was decreased in two cases. Postnatal surgery was assessed in two cases and a spontaneous regression under a sequential treatment occurred in the other three cases. Isolated foetal pyelectasis can have a pathologic significance.This examination permits, in plus, to evaluate the renal prognosis. Antenatal therapeutic implications of interruption of pregnancy or urinary intervention are still not clear and those after birth depend essentially on renal function determined by scintigraphy.