[High-Flow Nasal Cannulae (HFNC) in Neonates: A Survey of Current Practice in Level 1 Perinatal Centres in the German State of North Rhine-Westphalia]. / High-flow nasal cannulae (HFNC) in der Neonatologie: Umfrage über Einsatz und Erfahrungen in den nordrheinwestfälischen Level 1 Perinatalzentren.

INTRODUCTION: High-flow nasal cannulae (HFNC) is a kind of non-invasive respiratory support. In recent years, its application has gained increasing popularity for treating neonates with respiratory failure. Within this study, neonatologists employed at high level perinatal centres within the region of North Rhine-Westphalia, Germany were interviewed. We evaluated their personal experience as well as the underlying indication for using HFNC. METHOD: We undertook an online survey. RESULTS: 93% of the interviewed participants use HFNC systems in their NICU. The most prominent indications were CPAP-weaning, nasal trauma, and apnoea of prematurity. Both initial flow and maximum and minimum flow rates varied widely. The primary benefit of HFNC vs. conventional CPAP was the improved neonate tolerance, less nasal traumata and ease of application and care. A common disadvantage was the inability to conduct PEEP measurements. DISCUSSION: The application of the HFNC system is increasing for specific neonatal indications, thereby increasing the data for the evaluation of effectivity and safety. Nevertheless, detailed investigations of the appropriate flow rate settings are still lacking.

Successful treatment of neonatal respiratory failure caused by a novel surfactant protein C p.Cys121Gly mutation with hydroxychloroquine.

SFTPC (surfactant protein C) mutations resulting in SP-C deficiency causing ongoing respiratory failure in the neonatal period represent a rare entity. We report a full-term female infant who developed respiratory distress and respiratory failure shortly after birth. From the first day of life the infant was mechanically ventilated. Application of exogenous surfactant or cortisone did not lead to any clinical improvement. Genetic analysis identified a novel SFTPC mutation as the cause of her lung disease. The patient was diagnosed as heterozygous for a p.Cys121Gly/C121G substitution encoded by exon 4, which could not be detected in both parents. Experimental therapy with hydroxychloroquine resulted in a significant clinical improvement within 2 weeks time. Mechanical ventilation was no longer needed, and the patient was discharged without additional oxygen demand. The patient remained well under therapy till the age of 6 months. After that time, the therapy was successfully discontinued.

Antimitochondrial antibody profiles in primary biliary cirrhosis distinguish at early stages between a benign and a progressive course: a prospective study on 200 patients followed for 10 years.

In recent retrospective studies, it was shown that subtypes of antimitochondrial antibodies (AMA) can help to discriminate between a benign [only anti-M9 and/or anti-M2 positive by enzyme-linked immunosorbent assay (ELISA)] and a rather progressive course (anti-M2, -M4 and/or -M8 positive). According to different constellations of these AMA subspecificities in ELISA and complement fixation test (CFT), four AMA profiles (A-D) were defined. In 1984 we started a prospective study based on 200 PBC patients with known AMA profiles in order to correlate the antibody pattern with the clinical outcome. Progression was defined primarily as the necessity of liver transplantation and death due to hepatic failure or variceal bleeding. At entry, 18 (9%) of the 200 patients had AMA profile A (only anti-M9), 57 (29%) profile B (only anti-M2 with or without anti-M9), 74 (37%) profile C (anti-M2 in association with anti-M4/-M8 by ELISA), and 51 (26%) profile D (anti-M2/-M4/-M8 by ELISA and CFT). At the beginning of the study, 177 patients had PBC stage I/II. During the observation period of ten years, ten patients died and in 18 orthotopic liver transplantation (OLT) was performed; all these patients belonged to profile C/D. Furthermore, 44% of the patients with profile C and 31% of the patients with profile D progressed to late stages, as defined by histology and clinical manifestations such as portal hypertension and increase of bilirubin, while only one of the patients with profile B and none of the profile A-patients developed late stage PBC. A significant increase of bilirubin was observed only in C/D-patients. AMA profiles did not change during the follow-up. In conclusion, AMA profiles discriminate between a benign and a progressive course of PBC already at early stages.

[Ohtahara syndrome]. / Das Ohtahara-Syndrom.

Attacks of cerebral spasm occur in 1.5 to 5 per thousand newborn post partum. Basing on the rare Ohtahara's syndrome it is shown that the obstetrician can be exonerated from the suspicion of a trauma occurring during childbirth and causing the spasms, only if a precise and detailed documentation of the entire obstetric procedure is prepared an differentiated neuropaediatric diagnosis is performed.

[High-dose omeprazole versus famotidine, pirenzepine and antacid in therapy of acute upper gastrointestinal hemorrhage in a retrospective comparison]. / Omeprazol hochdosiert versus Famotidin, Pirenzepin und Antazidum in der Therapie der akuten oberen gastrointestinalen Blutung im retrospektiven Vergleich.

We retrospectively investigated the efficacy of high dose omeprazole compared to a combined therapy of famotidine, pirenzepine and antacid for acute upper gastrointestinal hemorrhage (AUGIH) also adjuvant to endoscopic injection therapy if indicated. The clinical course of AUGIH was evaluated, if emergency endoscopy revealed lesions substantially dependent on intragastric acidity with respect to pathogenesis and/or healing (peptic ulcer, erosive gastroduodenitis, reflux-esophagitis, Mallory-Weiss tears) and patients either received a combined therapy of famotidine (20 mg i.v. every 12 hrs), pirenzepine (10 mg i.v. every 12 hrs) and antacid (control group: n = 96) or omeprazole (40 mg i.v. every 6 hrs; omeprazole group: n = 100). Rate of rebleeding was lower in the omeprazole group without reaching significance (12 vs. 21; p = 0.06). No difference was found for rates of operation (6 vs. 6; p = 0.94), death from bleeding (5 vs. 9; p = 0.22), transfusions ([mean +/- SD] 3.3 +/- 5.0 vs. 3.2 +/- 5.7; p = 0.51) and hospitalisation ([mean +/- SD] 26.8 +/- 12.1 vs. 27.8 +/- 16.0 days; p = 0.88). Considering prognostic risk factors (age > or = 65, actively bleeding lesion, initial state of shock) logistic regression showed that high dose omeprazole inhibited rebleeding (p = 0.01) but had no effect as regards surgery or mortality. Within two selected subgroups defined by additional criteria (no endoscopic treatment and anamnestic peptic lesion) omeprazole-treated cases showed lower rates of rebleeding (3/49 vs. 12/54, p " 0.02 and 3/44 vs. 13/48, p = 0.01 resp.) and death from bleeding (0/46 vs. 6/50, p = 0.03 and 0/43 vs. 5/45, p = 0.03 resp.).(ABSTRACT TRUNCATED AT 250 WORDS)

Antimitochondrial antibody profiles determined at early stages of primary biliary cirrhosis differentiate between a benign and a progressive course of the disease. A retrospective analysis of 76 patients over 6-18 years.

Seventy-six patients with clinically and histologically defined primary biliary cirrhosis, who were in stage I/II at the time of first diagnosis and could be followed for 6-18 years (mean 10 years), were analysed with respect to their clinical course. Forty-four of the 76 patients remained in stage I/II during the observation period (group 1), while 32 progressed to late stages (group 2). Sera of these patients were retested for anti-M2, anti-M4, anti-M8 and anti-M9 antibodies by ELISA and the complement fixation test (CFT). Four different AMA-profiles A-D could be differentiated in these patients: profile A, only anti-M9 positive by ELISA; B, anti-M9 and/or anti-M2 positive by ELISA; C, anti-M2, anti-M4 and/or anti-M8 positive by ELISA; and D, anti-M2, anti-M4 and/or anti-M8 positive by ELISA and CFT. When the natural courses of patients and the four different AMA-profiles were compared retrospectively it became evident that 97% of group 2 patients already had the AMA-profile C/D at entry into the study. Of the patients in group 1 70% had the AMA-profile A/B. Increases in bilirubin levels during the course of the disease were observed preferentially in patients of group 2 (profile C/D). We conclude that the determination of AMA-profiles in PBC patients is a sensitive approach for the early prediction of the outcome of the disease.