Peri-partum respiratory management of pregnant women with neuro-muscular disorders: a prospective observational study (IT-NEUMA-Pregn study).

BACKGROUND: Pregnant women with neuromuscular diseases (NMDs) often display respiratory muscle impairment which increases the risk for pulmonary complications (PCs). The aim of this study was to identify pregnant NMDs patients with pulmonary risk factors and to apply in these women non-invasive ventilation (NIV) combined with mechanical insufflation-exsufflation (MI-E) in the peri-partum period. METHODS: We conducted a multicenter observational study on women with NMDs undergoing cesarean section or spontaneous labor in a network of 7 national hospitals. In these subjects we applied a protocol for screening and preventing PCs, and we evaluated PCs rate, maternal and neonatal outcome. RESULTS: Twenty-four patients out of the 94 enrolled pregnant women were at risk for PCs and were trained or retrained to use NIV and/or MI-E before delivery. After delivery, 17 patients required NIV with or without MI-E. Despite nine out of the 24 women at pulmonary risk developed postpartum PCs, none of them needed reintubation nor tracheostomy. In addition, the average birth weight and Apgar score were normal. Only one patient without pulmonary risk factors developed postpartum PCs. CONCLUSION: This study showed the feasibility of applying a protocol for screening and treating pregnant NMDs women with pulmonary risk. Despite a PCs rate of 37% was observed in these patients, maternal and neonatal outcome were favorable.

Protean presentation and multiple challenges of nephrocalcinosis in pregnancy (six pregnancies in four patients).

BACKGROUND: Nephrocalcinosis is an umbrella term covering increased content of calcium salts in the renal parenchyma, interstitial damage and potential evolution towards renal failure. Pregnancy is often the first occasion for biochemical or imaging tests in young women and may allow early diagnosis. Conversely, even mild kidney disease may represent a challenge in pregnancy. AIM: The aim of this study was to report on four patients in whom nephrocalcinosis was first diagnosed during pregnancy, exemplifying the protean presentation and multiple challenges of nephrocalcinosis in pregnancy. METHODS: This is a case series study including data on all pregnancies prospectively gathered in the Nephrological-Obstetric Unit dedicated to pregnancy and kidney diseases (2000-11). RESULTS: Six pregnancies were observed in four patients (31-35 years; one twin pregnancy, one ongoing, one patient with three pregnancies). Symptoms were oedema in two (later developed in a further patient), renal functional impairment and electrolyte imbalance in two each. Two patients developed hypertension late in pregnancy. Electrolyte imbalance was life-threatening in one patient (severe acidosis, severe hyperkalaemia: 7.5 mEq/L). Delivery was by Caesarean section in three patients, preterm in one. Multiple or long hospitalizations for metabolic reasons were needed in three patients, the fourth was hospitalized for obstetric reasons. In all patients, diagnosis of nephrocalcinosis was made at ultrasounds during basic nephrological evaluation, confirmed at computerized tomography scan in three. The pathogenesis was linked to diuretic abuse in one case and to collagen disease, inborn errors and prematurity, possibly associated with diuretic misuse, in the others. CONCLUSION: Nephrocalcinosis may have protean presentations in pregnancy. The risk of severe electrolyte derangements, oedema and hypertension warrants strict clinical surveillance.