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 109/L. Within the second day of life, platelet level  dropped to 20 x 109/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 109/L requiring renewed platelet transfusion and human immunoglobulin infusion. On the 10th of life platelets rate was stable around 60 x 109/L. The infant had no evidence of cardiac, dermatologic or hepatobilary involvement initially or throughout follow up.Key words: Neonatal lupus erythematosus, thrombocytopenia, anti-Ro/SS

Diastolic ventricular function in persistent pulmonary hypertension of the newborn

BackgroundPersistent pulmonary hypertension of the newborn (PPHN) is usually considered a consequence of impaired pulmonary circulation. However, little is known regarding the role of cardiac dysfunction in PPHN. In this study, we hypothesized that the tolerance for pulmonary hypertension in newborn infants depends on the biventricular function. The aim of this study is to evaluate biventricular cardiac performance by using Tissue Doppler Imaging (TDI) in an healthy newborn infants with asymptomatic pulmonary hypertension and in newborn infants with PPHN.MethodsRight and left cardiac function were investigated using conventional imaging and TDI in 10 newborn infants with PPHN (“PPHN”) and 10 asymptomatic healthy newborn infants (“asymptomatic PH”).ResultsSystolic pulmonary artery pressure (PAP) as assessed by TDI and the mean systolic velocity of the right ventricular (RV) free wall were similar in both groups. The isovolumic relaxation time of the right ventricle at the tricuspid annulus was significantly longer in the “PPHN” than in the “asymptomatic PH” group (53 ± 14 ms vs. 14 ± 4 ms, respectively; p < 0.05). Left ventricular (LV) function was normal in both groups with a systolic velocity (S'LV) at the LV free wall groups (6 ± 0.5 cm/s vs. 8.3 ± 5.7 cm/s, p > 0.05).ConclusionThe present results suggest that high PAP with or without respiratory failure is not associated with altered right systolic ventricular function and does not affect LV function in newborn infants. PPHN is characterized by a marked right diastolic ventricular dysfunction. These data suggest that the hypoxic respiratory failure in PPHN results, at least in part, from diastolic RV dysfunction and right to left shunting across the foramen ovale. We propose that the severity of the respiratory failure is more related to the RV diastolic dysfunction than the pulmonary artery pressure

Antenatal Assessment of the Prognosis of Congenital Diaphragmatic Hernia: Ethical Considerations and Impact for the Management

Congenital diaphragmatic hernia (CDH) is associated with abnormal pulmonary development, which is responsible for pulmonary hypoplasia with structural and functional abnormalities in pulmonary circulation, leading to the failure of the cardiorespiratory adaptation at birth. Despite improvement in treatment options and advances in neonatal care, mortality remains high, at close to 15 to 30%. Several risk factors of mortality and morbidities have been validated in fetuses with CDH. Antenatal assessment of lung volume is a reliable way to predict the severity of CDH. The two most commonly used measurements are the observed/expected lung to head ratio (LHRo/e) and the total pulmonary volume (TPV) on MRI. The estimation of total pulmonary volume (TPVo/e) by means of prenatal MRI remains the gold standard. In addition to LHR and TPV measurements, the position of the liver (up, in the thorax or down, in the abdomen) also plays a role in the prognostic evaluation. This prenatal prognostic evaluation can be used to select fetuses for antenatal surgery, consisting of fetoscopic endoluminal tracheal occlusion (FETO). The antenatal criteria of severe CDH with an ascended liver (LHRo/e or TPVo/e < 25%) are undoubtedly associated with a high risk of death or significant morbidity. However, despite the possibility of estimating the risk in antenatal care, it is difficult to determine what is in the child’s best interest, as there still are many uncertainties: (1) uncertainty about individual short-term prognosis; (2) uncertainty about long-term prognosis; and (3) uncertainty about the subsequent quality of life, especially when it is known that, with a similar degree of disability, a child’s quality of life varies from poor to good depending on multiple factors, including family support. Nevertheless, as the LHR decreases, the foreseeable “burden” becomes increasingly significant, and the expected benefit is increasingly unlikely. The legal and moral principle of the proportionality of medical procedures, as well as the prohibition of “unreasonable obstinacy” in all investigations or treatments undertaken, is necessary in these situations. However, the scientific and rational basis for assessing the long-term individual prognosis is limited to statistical data that do not adequately reflect individual risk. The risk of self-fulfilling prophecies should be kept in mind. The information given to parents must take this uncertainty into account when deciding on the treatment plan after birth

Systemic Inflammation Is Associated with Pulmonary Hypertension in Isolated Giant Omphalocele: A Population-Based Study

Our objective is to determine perinatal factors contributing to the development of pulmonary hypertension (PH) in patients with isolated giant omphaloceles (GO). All cases of omphaloceles that underwent prenatal and postnatal care at the University Hospital of Lille between 1996 and 2021 were reviewed. We included all infants with isolated GO, including at least a part of the liver, who were treated by delayed surgical closure. Prenatal and postnatal data were recorded and correlated with postnatal morbidities. We compared outcomes between a group of infants with GO who developed PH and infants with GO with no PH. We identified 120 infants with omphalocele. Fifty isolated GO cases fulfilled the inclusion criteria of our study. The incidence of PH was 30%. We highlighted a prolonged inflammatory state, defined as a CRP superior to 15 mg/L, platelets higher than 500 G/L, and white blood cells higher than 15 G/l for more than 14 days in patients who developed PH. This event occurred in 73% of patients with PH versus 21% of patients without PH (p < 0.05). Late-onset infection was not different between the two groups. We speculate that prolonged inflammatory syndrome promotes PH in infants with GO treated with delayed surgical closure