A 17-day-old term neonate (birth weight 5.5 kg, cesarean section) was treated with antibiotics and given partial parenteral nutrition after the reduction of a small-bowel volvulus. He went on to develop severe sepsis with disseminated intravascular coagulation and extensive hemorrhages on the limbs. Blood cultures and cultures of the tip of a central venous catheter yielded Klebsiella oxytoca. Bullae formed on the limbs, with extensive areas of necrosis (Figure). The infant was treated with broad-spectrum antibiotics, ventilation, diuretics, protein C substitution, and a standard burn protocol. The limbs were successfully preserved, with scarring. Purpura fulminans is a rare type of coagulopathy with more than 50% mortality. Its commonest causes are infection (usually with Gram-positive cocci) and congenital protein C deficiency, which becomes evident in the first few hours after birth. In purpura fulminans due to infection, bacterial endotoxins can activate the clotting system and cause a consumption coagulopathy. Klebsiella accounts for 4-7% of cases of neonatal late-onset sepsis and should be considered as a rare cause of purpura fulminans.

Since the beginning of the COVID-19 pandemic, in-utero transmission of SARS-CoV-2 remains a rarity and only very few cases have been proven across the world. Here we depict the clinical, laboratory and radiologic findings of preterm triplets born at 28 6/7 weeks to a mother who contracted COVID-19 just 1 week before delivery. The triplets showed SARS-CoV-2 positivity right after birth, developed significant leukopenia and early-onset pulmonary interstitial emphysema. The most severely affected triplet I required 10 days of high-frequency oscillatory ventilation due to failure of conventional invasive ventilation, and circulatory support for 4 days. Despite a severe clinical course in two triplets (triplet I and II), clinical management without experimental, targeted antiviral drugs was successful. At discharge home, the triplets showed no signs of neurologic or pulmonary sequelae. Placental immunohistology with SARS-CoV-2 N-protein localized strongly to syncytiotrophoblast cells and, to a lesser extent, to fetal Hofbauer cells, proving intrauterine virus transmission. We discuss the role of maternal viremia as a potential risk factor for vertical transmission. To the best of our knowledge, our report presents the earliest unequivocally confirmed prenatal virus transmission in long-term surviving children, i.e., at the beginning of the third trimester.

Our data indicate that primary FM is negatively associated with childhood obesity.

<b><i>Background:</i></b> PHACE syndrome is a rare inborn condition characterized by large facial hemangiomas and variable malformations of the arterial system, heart, central nervous system, and eyes. According to Orphanet estimates, the prevalence is &#x3c;1.0 per million. Data from Europe are limited to small case series, and there are no population-based data available. <b><i>Objectives:</i></b> We conducted the present study to provide population-based estimates of the disease prevalence of PHACE syndrome in children in Germany, Switzerland, and Austria. We compared these first systematic data on PHACE syndrome from Europe to published data from the PHACE Syndrome International Clinical Registry and Genetic Repository (USA). Clinical features in our cohort with PHACE syndrome were assessed in detail, including the need for early supportive measures. <b><i>Methods:</i></b> We used a population-based approach by means of a previously well-established network of child neurologists from Germany, Switzerland, and Austria (“ESNEK”) to identify potential patients. The patients’ guardians and child neurologists were asked to fill in questionnaires developed in collaboration with the International PHACE Registry. <b><i>Results:</i></b> We identified 19 patients with PHACE syndrome. Estimated prevalence rates were 6.5 per million in Switzerland, 0.59 per million in Germany, and 0.65 per million in Austria. A subset of 10 patients from Germany and Switzerland participated in our study, providing detailed clinical assessment (median age: 2.5 years; 9 females, 1 male). Cerebrovascular involvement was frequent (80%). Facial hemangioma extent correlated significantly with the number of organs involved (<i>p</i> = 0.011). In 9 out of 10 patients, facial hemangiomas were treated successfully with oral propranolol. Baseline demographic data as well as the rate of cerebrovascular and cardiovascular anomalies were in line with those from the US International PHACE Registry and other published PHACE cohorts. <b><i>Conclusions:</i></b> Our study provides population-based estimates for PHACE syndrome in 3 German-speaking countries. The data from Switzerland indicate that PHACE syndrome may be more prevalent than demonstrated by previous reports. Underreporting of PHACE syndrome in Germany and Austria likely accounts for the differences in prevalence rates. The clinical observation of a potential association between the size of facial hemangioma and extent of organ involvement warrants further investigation.

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