Extremely preterm infants in Tunisia: Where are we now?

BACKGROUND: Extremely preterm infants are newborns born before 28 weeks of gestation. Survival of these immature newborns depends on resuscitation and the quality of care during hospitalization. OBJECTIVE: To determine survival and neurologic outcomes at2 years after extremely preterm birth. METHODS: It is a retrospective multicentric study in 5 neonatal intensive care units (NICU) in 2012-2013.All live births less than 28 weeks gestation were included. RESULTS: A total of 109 births were recorded. Prenatal corticosteroids were given in 47% of cases. Mean weight was 989g and mean age was 26 weeks gestation. Ninety percent of patients had respiratory distress syndrome and 67% of them needed respiratory support. Surfactant was given to 29% of newborns. The mortality rate atdischarge was 76%.The first cause of mortality was nosocomial infections. At thecorrected age of 2 years, 27% of survivors had abnormal neurologic outcome. CONCLUSION: In our study, survival and neurologic outcomes ofextremely preterm infants were poor.In this high-risk population, improving perinatal care remains a challenge to improve long-term outcome in Tunisia.

Neurodevelopmental Impairment in Children After Group B Streptococcal Disease Worldwide: Systematic Review and Meta-analyses.

BACKGROUND: Survivors of infant group B streptococcal (GBS) disease are at risk of neurodevelopmental impairment (NDI), a burden not previously systematically quantified. This is the 10th of 11 articles estimating the burden of GBS disease. Here we aimed to estimate NDI in survivors of infant GBS disease. METHODS: We conducted systematic literature reviews (PubMed/Medline, Embase, Latin American and Caribbean Health Sciences Literature [LILACS], World Health Organization Library Information System [WHOLIS], and Scopus) and sought unpublished data on the risk of NDI after invasive GBS disease in infants <90 days of age. We did meta-analyses to derive pooled estimates of the percentage of infants with NDI following GBS meningitis. RESULTS: We identified 6127 studies, of which 18 met eligibility criteria, all from middle- or high-income contexts. All 18 studies followed up survivors of GBS meningitis; only 5 of these studies also followed up survivors of GBS sepsis and were too few to pool in a meta-analysis. Of meningitis survivors, 32% (95% CI, 25%-38%) had NDI at 18 months of follow-up, including 18% (95% CI, 13%-22%) with moderate to severe NDI. CONCLUSIONS: GBS meningitis is an important risk factor for moderate to severe NDI, affecting around 1 in 5 survivors. However, data are limited, and we were unable to estimate NDI after GBS sepsis. Comparability of studies is difficult due to methodological differences including variability in timing of clinical reviews and assessment tools. Follow-up of clinical cases and standardization of methods are essential to fully quantify the total burden of NDI associated with GBS disease, and inform program priorities.

Novel ATP6V0A4 mutation described in a Tunisian patient with distal renal tubular acidosis.

UNLABELLED: Few data regarding molecular diagnosis of primary distal renal tubular acidosis (DRTA) in Tunisian population are available. CASE REPORT: 25-day-old male patient from consanguineous parents of Tunisian origin diagnosed with DRTA and without hearing impairment observed later in life. ATP6V0A4 gene sequencing demonstrated a novel homozygous G deletion in exon 13 (c.1221delG, p.Met408CysfsX10), leading to a premature stop codon. CONCLUSION: A novel ATP6V0A4 gene mutation confirmed autosomal recessive DRTA with normal hearing in the patient. Molecular analysis may help to rapidly diagnose autosomal recessive DRTA in Tunisian population.

Subcutaneous Fat Necrosis of the Newborn.

A boy weighing 4500 g was born at 41 weeks' gestation by Cesarean section due to fetal distress. The pregnancy was complicated by gestational diabetes. He had an Apgar score of 5 and 6 after 5 and 10 minutes, respectively. At birth, the newborn manifested respiratory distress, which needed assisted ventilation for 48 hours. He developed a convulsive attack, diagnosed as a grade 2 hypoxic-ischemic encephalopathy, which was controlled by phenobarbital. Four days later, dermatologic examination revealed subcutaneous and firm nodules, ranging from 1 to 4 cm in diameter, on the cheeks, neck, arms, legs, and back (Figure 1). Some nodules became fluctuant as abscesses. Fine-needle aspiration cytology performed on a nodule revealed a dirty background with necrotic fat-containing characteristic, radially- oriented, refractile, needle-shaped crystals (Figure 2), which was diagnosed as subcutaneous fat necrosis. The diagnosis of subcutaneous fat necrosis of the newborn was made. Laboratory studies revealed hypocalcemia at 1.65 mmol/L, hypomagnesemia at 0.48 mmol/L, and hypokalemia at 3 mmol/L. The infant received calcium, magnesium, vitamin D, and potassium supplementation. On day 18, the serum calcium increased to 3.3 mmol/L. It was associated with hypertriglyceridemia at 2.6 mmol/L. Bilateral nephrocalcinosis was detected on renal ultrasound. So, hyperhydration, diuretics, and withdrawal of vitamin D were indicated. The patient was given betamethasone 0.125 mg/kg/day for 3 weeks. After a two months course, there had been complete healing of the fat necrosis (Figure 3), normalization of the calcium and triglyceride levels, and a normal growth pattern.

Transported neonates in Tunisia.

AIMS: To describe the transport of sick neonates to a tertiary care hospital and evaluate their condition at arrival and outcome. METHODS: A multicenter, prospective cohort study was performed in 7 NICUs in Tunisia from 1st april to 31 July 2015.Demographic parameters, transport details and clinical features at arrival were recorded. All neonates were followed up till discharge or death. RESULTS: A total of 239 consecutive neonates were enrolled in the study representing 5.7% of all admitted infants. Maternal risk factors were present in 26% of neonates admitted. Sex-ratio was 1.46. Preterm infants represented 24% of transported babies. Seventeen percent of neonates had severe respiratory distress and 10% had hemodynamic troubles. Referred hospital was not informed in 24% of cases. Regarding the transport mode, 113 newborns (47.5%) were transported in ambulance accompanied by a nurse. Documentation during transfert was present in 14% of cases. Five babies expired on arrival despite resuscitation.  Rate mortality was 8.4%. CONCLUSION: Transporting neonates in developing countries is a challenge. Organized transport services in Tunisia are not always available. So, in cases of at-risk pregnancy, it is safer to transport the mother prior to delivery than to transfer the sick baby after birth.

Prenatal diagnosis of caudal regression syndrome and omphalocele in a fetus of a diabetic mother.

The caudal regression syndrome is defined as total or partial agenesis of the sacrum and lumbar spine, frequently associated with other developmental malformations (orthopedic, neurological, genito-urinary, gastrointestinal…). Prenatal diagnosis is possible through fetal ultrasound (US) and magnetic resonance imaging (MRI). A case of fetal caudal regression syndrome with omphalocele from a diabetic mother is presented, demonstrating the sonographic, MRI, CT and X-Ray features diagnostic. We will also discuss neonatal findings, risk factors and prognosis of this condition.