Allogeneic hematopoietic cell transplantation (HCT) has been shown to restore normal hematopoiesis in patients with Fanconi anemia (FA), with excellent results in matched related donor HCT. Outcomes of alternative donor HCT are less favorable, however. In patients without FA, several reports have documented stable engraftment and/or a low risk of graft-versus-host disease (GVHD) using unmanipulated HLA-mismatched related donors and post-HCT cyclophosphamide (PT-CY) for GVHD prophylaxis. Data on the use of this approach in patients with FA are scarce, and thus we launched a study of HLA-mismatched related donor HCT in these patient. Here we report our findings in 19 patients. The conditioning was fludarabine 30 mg/m 2 /day for 5 days, antithymocyte globulin 5 mg/kg/day for 4 days, and total body irradiation (total dose, 200 cGy). GVHD prophylaxis was cyclosporine and mycophenolate and reduced doses of PT-CY, 25 mg/kg, on days +3 and +5. All patients exhibited absolute neutrophil count recovery. Grade III-IV acute GVHD occurred in 3 patients, and chronic GVHD occurred in 1 patient. At a mean follow-up of 38.3 § 5.8 months, the 5-year probability of overall survival for our patients was 89.2% § 7.2%. The regimen was well tolerated; hemorrhagic cystitis occurred in 7 patients, and severe mucositis occurred in 5 patients. There were 2 deaths; the primary cause of death was severe GVHD in 1 patient and leukemia recurrence in the other. We conclude that in patients with FA lacking a matched related donor, the use of mismatched related HCT with low-dose PT-CY is a viable option; it is well tolerated, with a high rate of engraftment and an acceptable incidence of GVHD.

Percutaneous endoscopic gastrostomy (PEG) is a well-established and successful method of nutritional delivery. Complications, although rare, are divided into early or late. Buried bumper syndrome (BBS) is usually a late complication of PEG tube insertion and can cause many issues such as pressure necrosis, peritonitis, and septic shock. Endoscopic evaluation is the definitive diagnosis, and treatment depends on each patient and the degree of depth of disc migration. We present to you a case of buried bumper syndrome in a 66-year-old female that was initially thought to be complicated with peritonitis, and surprisingly occurring only one week after initial PEG tube placement.

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BackgroundHepatic encephalopathy (HE) is defined as brain dysfunction that occurs because of acute liver failure or liver cirrhosis and is associated with significant morbidity and mortality. Lactulose is the standard of care till this date; however, polyethylene glycol (PEG) has gained the attention of multiple investigators.MethodsWe screened five databases namely PubMed, Scopus, Web of Science, Cochrane Library and Embase from inception to 10 February 2021. Dichotomous and continuous data were analysed using the Mantel-Haenszel and inverse variance methods, respectively, which yielded a meta-analysis comparing PEG versus lactulose in the treatment of HE.ResultsFour trials with 229 patients were included. Compared with lactulose, the pooled effect size demonstrated a significantly lower average HE Scoring Algorithm (HESA) Score at 24 hours (Mean difference (MD)=−0.68, 95% CI (−1.05 to –0.31), p<0.001), a higher proportion of patients with reduction of HESA Score by ≥1 grade at 24 hours (risk ratio (RR)=1.40, 95% CI (1.17 to 1.67), p<0.001), a higher proportion of patients with a HESA Score of grade 0 at 24 hours (RR=4.33, 95% CI (2.27 to 8.28), p<0.0010) and a shorter time to resolution of HE group (MD=−1.45, 95% CI (−1.72 to –1.18), p<0.001) in favour of patients treated with PEG.ConclusionPEG leads to a higher drop in the HESA Score and thus leads to a faster resolution of HE compared with lactulose.

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Pasteurella multocida (PM) is a gram-negative bacterium known to cause soft tissue infections, especially after animal bites, with some human infections occurring after animal exposure, usually via inhalation of contaminated secretions. PM pneumonia mainly affects those who are immunocompromised and in individuals with comorbidities. The spectrum of pulmonary disease due to PM is wide, ranging from pneumonia to empyema. The clinical features are indistinguishable from other pathogens, however, hemoptysis seldom occurs as a consequence of PM infection. We present a case of PM pneumonia in an immunocompetent host who had a chief complaint of hemoptysis, making this the sixth documented case to ever-present with hemoptysis.

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