Acquired haemophilia A in southern Switzerland from 2013 to 2019: a case series.

AIMS OF THE STUDY: Acquired haemophilia A is a rare disease with an annual incidence of 1.48 per million. Based on clinical observations, we suspect a higher incidence in southern Switzerland, and aimed at providing local epidemiological data, and clinical information regarding diagnosis, treatment and outcome in our region. METHODS: All adult patients with acquired haemophilia A treated between 2013 and 2019 in our facility were included in the present retrospective analysis. RESULTS: We treated 11 patients with acquired haemophilia A between 2013 and 2019, resulting in an annual incidence of 4.5 per million (95% confidence interval [CI] 0-9.0). Median delay from first symptoms to diagnosis was 4.5 days, and the median age at diagnosis was 79 years (range 23-87). Possible causative conditions were: pregnancy (n = 1), polyarteritis nodosa (n = 1), myelodysplastic syndrome (n = 1), chronic human immunodeficiency virus (HIV) (n = 1), and HIV postexposure prophylaxis (n = 1). In five patients no underlying or associated condition was identified. Median activated partial thromboplastin time (aPTT)) at baseline was 79 seconds (65-117; ref. value <38 sec), and FVIII:C 2.15% (<1-3.75%). A FVIII:C <1% was present in 4/10 patients. Median FVIII-inhibitor titre was 10.3 BU/ml (2.4-75.0 BU/ml). All patients had bleeding symptoms, 5/10 patients had major bleedings, and 7/10 patients were treated with bypassing agents. All patients received corticosteroids; 7/10 patients received immunosuppressive combination therapy. FVIII levels of ≥50% were achieved after a median of 40 days (8-62). One patient had a severe immunosuppressive therapy-related infection. An 87-years-old woman died for reasons not related to acquired haemophilia A or immunosuppressive therapy. CONCLUSIONS: Acquired haemophilia A is a rare disease, but manageable despite the advanced patient age and comorbidities. Its incidence in Southern Switzerland is higher than previously suspected.

Abdominal Kikuchi-Fujimoto lymphadenopathy: an uncommon presentation of a rare disease.

A 34-year-old man presented to our hospital with a 5-day history of progressive abdominal pain and fever. A CT scan identified extensive mesenteric lymphadenopathy. Initial diagnostic tests were inconclusive. Abdominal lymph node biopsy showed histiocytic necrotising lymphadenitis, compatible with Kikuchi-Fujimoto disease (KFD). This benign and self-limiting disease generally resolves following supportive treatment. In this case, remission occurred within 3 weeks of initial presentation. KFD is a very uncommon cause of lymphadenopathy, and selective mesenteric involvement is rare. Definitive diagnosis often requires lymph node biopsy. It is important to exclude more common and serious differential diagnoses associated with mesenteric lymphadenopathy, while maintaining a minimally invasive diagnostic approach, before progressing to nodal biopsy.

Nutritional Risk Screening and Body Composition in COVID-19 Patients Hospitalized in an Internal Medicine Ward.

BACKGROUND: Malnutrition in patients hospitalized in internal medicine wards is highly prevalent and represents a prognostic factor of worse outcomes. Previous evidence suggested the prognostic role of the nutritional status in patients affected by the coronavirus disease 2019 (COVID-19). We aim to investigate the nutritional risk in patients with COVID-19 hospitalized in an internal medicine ward and their clinical outcomes using the Nutritional Risk Screening 2002 (NRS-2002) and parameters derived from bioelectrical impedance analysis (BIA). METHODS: Retrospective analysis of patients with COVID-19 aimed at exploring: 1) the prevalence of nutritional risk with NRS-2002 and BIA; 2) the relationship between NRS-2002, BIA parameters and selected outcomes: length of hospital stay (LOS); death and need of intensive care unit (ICU); prolonged LOS; and loss of appetite. RESULTS: Data of 90 patients were analyzed. Patients at nutritional risk were 92% with NRS-2002, with BIA-derived parameters: 88% by phase angle; 86% by body cell mass; 84% by fat-free mass and 84% by fat mass (p-value ≤0.001). In ROC analysis, NRS had the maximum sensitivity in predicting the risk of death and need of ICU and a prolonged hospitalization showing moderate-low specificity; phase angle showed a good predictive power in terms of AUC. NRS-2002 was significantly associated with LOS (ß 12.62, SE 5.79). In a multivariate analysis, blood glucose level and the early warning score are independent predictors of death and need of ICU (OR 2.79, p ≤0.001; 1.59, p-0.029, respectively). CONCLUSION: Present findings confirm the clinical utility of NRS-2002 to assess nutritional risk in patients with COVID-19 at hospital admission and in predicting LOS, and that bioimpedance does not seem to add further predictive value. An early detection of nutritional risk has to be systematically included in the management of COVID-19 patients hospitalized in internal medicine wards.

Fitz-Hugh-Curtis Syndrome: A Diagnosis to Consider in a Woman with Right Upper Quadrant Abdominal Pain without Gallstones.

A young woman presented with right upper quadrant abdominal pain exacerbated by movement and breathing. Extensive evaluation revealed no gallstones or any other specific cause. Urine polymerase chain reaction results for Chlamydia trachomatis were positive, so the clinical diagnosis of Fitz-Hugh-Curtis syndrome was confirmed. This type of localized peritonitis is thought to be a complication of an ascending genital infection leading to pelvic inflammatory disease. The diagnosis is established on clinical grounds after excluding alternative, more common conditions. Proper antibiotic treatment usually leads to recovery and prevents long-term complications. LEARNING POINTS: Right upper quadrant pain in a sexually active woman may be due to Fitz-Hugh-Curtis syndrome, a type of localized peritonitis also called perihepatitis.This condition is considered to be a complication of an ascending genital infection leading to pelvic inflammatory disease.Sexually active women with right upper quadrant abdominal pain without gallstones should be tested for Chlamydia trachomatis and Neisseria gonorrhoeae.

Hypokalaemia in hospitalised patients.

QUESTIONS UNDER STUDY: Hypokalaemia in inpatients is common, and is associated with morbidity and mortality. Its management is risky and not always effective. We launched an educational programme with the aim of increasing the rate of potassium normalisation during hospital stay, and of reducing unmonitored cases. METHODS: The project consisted of three phases: (I) retrospective analysis on 26 471 patients hospitalised in 2012 in five acute care hospitals of southern Switzerland (Ente Ospedaliero Cantonale, EOC) with identification of improvement goals on a sample survey (588 cases of hypokalaemia); (II) revision of internal guidelines, and implementation of educational activities in one of the five hospitals (Ospedale Regionale di Locarno, ODL); (III) follow-up analysis on the 26 726 patients hospitalised in 2014 and second sampling to complete the evaluation of the efficacy of the intervention. RESULTS: Phase I, ODL vs EOC: prevalence of hypokalaemia, 21.7 vs 23.2% (p <0.05); treated 53.1 vs 56.5% (not significant); normalisation 62.4 vs 61.1% (ns); absence of monitoring 18.3 vs 21.1% (p <0.05); time to normalisation 3.0 ± 2.7 vs 2.8 ± 2.4 days (ns); secondary hyperkalaemia 1.1 vs 1.4% (ns). Length of stay hypokalaemic vs normokalaemic 11.2 ± 11.7 vs 6.6 ± 7.9 days (p <0.001); falls 3.5 vs 1.7% (p <0.001), deaths 5.1 vs 3.1% (p <0.001). The severity/performance ratio suggested inefficiency. Phase III, ODL 2012 vs ODL 2014: treated 53.1 vs 75.7% (p <0.001); normalisation 62.4 vs 69.7% (p <0.01); absence of monitoring 20.1 vs 8.7 (p <0.01); time to normalisation 3.1 ± 2.7 vs 2.4 ± 2.6 days (ns); secondary hyperkalaemia 1.1 vs 1.8% (ns). CONCLUSIONS: The management of hypokalaemia is characterised by dysfunctions; it can, however, be ameliorated by the implementation of internal guidelines and targeted educational activities. The length of hospital stay is increased in patients with hypokalaemia, shifting the expected length of hospital stay based on the Swiss Diagnosis Related Group classification.