Fatal overwhelming postsplenectomy infection due to Streptococcus pneumoniae serotype 10A with atypical polysaccharide capsule in a patient with chromosome 22q11.2 deletion syndrome: A case report

We report the first case of a teenage patient with chromosome 22q11.2 deletion syndrome who died of overwhelming postsplenectomy infection (OPSI) by Streptococcus pneumoniae despite appropriate prevention by pneumococcal vaccine. He had congenital heart disease and underwent several surgeries. Immunodeficiency had not been noticed clinically. Two years prior to death, splenectomy was performed for a drug-resistant idiopathic thrombocytopenic purpura and he was immunized with 23-valent pneumococcal polysaccharide vaccine (PPV23) 4 months after splenectomy. He died suddenly after a mild flu-like symptom. Autopsy was performed and OPSI was diagnosed. Blood culture was positive for S. pneumoniae. This isolated S. pneumoniae strain was serotypically un-typable by polyvalent serum agglutination test. On the contrary, multilocus sequence typing followed by DNA sequencing indicated the molecular serotype as 10A. Additional testing using monovalent and factor-specific sera confirmed the strain as serotype 10A. Ultrastructural observation of this S. pneumoniae strain showed that the polysaccharide capsule was thin and sparse. We speculate that the abnormal morphology of the capsule may have accounted for the polyvalent serum agglutination failure and may possibly be associated with severity of OPSI observed in this case. Chromosome 22q11.2 deletion syndrome is associated with certain immunodeficiency, especially susceptible to S. pneumoniae infections; however, fatal OPSI has not been reported. In addition to vaccination, prophylactic antibiotics may be necessary for these patients who are at risk of immunodeficiency.     

The effects of antifungal stewardship programs at a tertiary-care teaching hospital in Japan

Patients with invasive fungal diseases (IFDs) generally have a high mortality rate, and resistance to antifungal drugs and the high costs associated with it have led to recent problems, necessitating the appropriate use of antifungals. To this end, we launched Antifungal Stewardship Programs (AFSPs) in our hospital. Patients who were systemically administered antifungals from January 2011 to December 2016 were enrolled this study and divided into pre-intervention and intervention groups. No significant difference was observed in defined daily doses per 1000 patient-days (23.3 ± 8.0 vs 20.4 ± 10.8, p = 0.251) between the groups. The monthly average for the days of therapy per 1000 patient-days was significantly lower in the intervention group (15.1 ± 3.1 vs 12.7 ± 4.3, p = 0.009). The cost of the antifungals reduced over the 3-year period by $260,520 (13.5%). Furthermore, a decreasing trend was observed in both the 30-day mortality (40.9% vs 30.0%, p = 0.414) and in-hospital mortality (63.6% vs 36.7%, p = 0.054) in patients with candidemia. Our results indicate that AFSPs are efficacious and cost-effective approaches.     

Successful treatment of invasive pulmonary aspergillosis caused by Aspergillus felis,a cryptic species within the Aspergillus section Fumigati: A case report

Aspergillus species are a major cause of life-threatening infections in immunocompromised hosts, and the most common pathogen of invasive aspergillosis is Aspergillus fumigatus. Recently, the development of molecular identification has revealed cryptic Aspergillus species, and A. felis is one such species within the Aspergillus section Fumigati reported in 2013.We describe a case of invasive pulmonary aspergillosis caused by A. felis in a 41-year-old Japanese woman diagnosed with myelodysplastic syndrome. She presented with fever 19 days after undergoing autologous peripheral blood stem cell transplantation and was clinically diagnosed with invasive pulmonary aspergillosis. Bronchoscopy and bronchoalveolar lavage were performed for definitive diagnosis. The β-tubulin genes of the mold isolated from the bronchoalveolar lavage fluid, and sequenced directly from the PCR products using a primer pair were found to have 100% homology with A. felis. We successfully treated the patient with echinocandin following careful susceptibility testing.To the best of our knowledge, this is the first published case reporting the clinical course for diagnosis and successful treatment of invasive aspergillosis by A. felis.     

Clinical significance of immunohistochemically detected lymph node micrometastasis in patients with histologically node-negative esophageal carcinoma: a multi-institutional study

Background  In esophageal carcinoma, the clinical significance of immunohistochemically (IHC)-detected lymph node (LN) micrometastasis is still controversial. We designed this multicenter study to determine the clinical significance of IHC-detected LN micrometastasis in esophageal carcinoma. Methods  The subjects were 164 patients with histopathologically confirmed LN-negative esophageal carcinoma from eight hospitals. A similar IHC technique was used in all institutions, and micrometastasis was diagnosed by a researcher at each hospital as well as independently by pathologists with special interest in esophageal carcinoma. Results  IHC-related micrometastasis in LN was considered positive in 51 (31%) patients by the researchers and in 25 (15%) by the pathologists. The latter micrometastases were further classified into a single (n = 13) and clusters (n = 12) of immunopositive-LN. Kaplan–Meier analysis showed that researcher-based diagnosis of micrometastasis had no significant impact on prognosis whereas the cluster-type micrometastasis diagnosed by pathologists had a significant impact on prognosis. Conclusions  We speculate that the inconsistent results of IHC analyses reported in the literature are caused by the use of different definitions of micrometastasis and the subjective nature of diagnosis of micrometastasis, i.e., dependence on the examiner. Our multiinstitutional study also indicates that the morphological aspects of immunostained cells should be considered when assessing micrometastasis in LN by IHC and that only LN with clusters of IHC-positive cells are prognostically significant in esophageal carcinoma.     

Factors affecting the prognosis of patients with hepatocellular carcinoma invading the portal vein--a retrospective analysis using 952 consecutive HCC patients

BACKGROUND/AIMS: Hepatocellular carcinoma with portal venous invasion has a very poor prognosis. The aim of this study is to clarify the factors contributing to the survival of hepatocellular carcinoma patients with portal venous invasion. METHODOLOGY: Out of 952 patients with hepatocellular carcinoma admitted to Tokyo University hospital and its affiliated hospitals from 1987 to 1999, 53 patients developed portal venous invasion until December 2000. The main portal vein was invaded in 33 patients, and the first branch was invaded in the 20 patients. The factors contributing to the prognosis of hepatocellular carcinoma patients with portal venous invasion were determined by univariate and multivariate analyses using 19 clinicopathological parameters. RESULTS: Overall survival rates of the 53 patients at 6 months, and 1 and 2 years were 40%, 18%, and 12%, respectively. Univariate analysis indicated that the serum albumin level, Child classification, number of tumor foci, portal venous invasion-targeted irradiation, and percutaneous tumor ablation of the parenchymal main tumor were significant. Multivariate analysis showed that percutaneous tumor ablation (P = 0.033; risk ratio = 0.28) was the most important factor contributing to a favorable prognosis followed by number of tumor foci (P = 0.048; risk ratio = 0.41). CONCLUSIONS: This study showed the significance of treatment for the parenchymal main tumor in addition to portal venous invasion in patients with hepatocellular carcinoma involving portal venous invasion. Therefore, the efficacy of combined therapy using portal venous invasion-targeted irradiation and percutaneous tumor ablation for the parenchymal main tumor on survival of hepatocellular carcinoma patients with portal venous invasion is suggested.     

Development of overt hemolytic anemia after splenectomy for thrombocytopenia in Evans syndrome with negative Coombs test

A 69-year-old man was diagnosed as having idiopathic thrombocytopenic purpura (ITP) in April 2000, and treated with prednisolone (PSL) without effect. Splenectomy performed in June 2000 had only a transient and marginal influence on his platelet count. Two months later, he developed autoimmune hemolytic anemia (AIHA) without Coombs test positivity, and his diagnosis was changed to Coombs-negative Evans syndrome. Treatment with PSL led to recovery of his hemoglobin level, but not his platelet count. Although the mechanism responsible for development of AIHA after splenectomy in this patient with ITP remains unknown, close observation is required for any association with other autoimmune diseases such as SLE.     

Minimally invasive video-assisted thoracoscopic left ventricular epicardial lead implantation for biventricular pacing in a patient with persistent left superior vena cava

Cardiac resynchronization therapy (CRT) by biventricular pacing reduces symptoms and improves left ventricular function in many patients with heart failure due to left ventricular systolic dysfunction and cardiac dyssynchrony. Implantation of the biventricular pacing lead in association with persistent left superior vena cava is technically challenging. We report a successful case of minimally invasive video-assisted thoracoscopic left ventricular epicardial lead implantation for biventricular pacing in a patient with persistent left superior vena cava.     

Cholangiographic findings of early-stage extrahepatic bile duct carcinoma

BACKGROUND: To clarify the cholangiographic findings of early-stage (T1, tumor confined to the mucosal or fibromuscular layer) extrahepatic bile duct carcinoma. METHODS: Cholangiographic images were retrospectively analyzed without other information in 55 patients with extrahepatic bile duct carcinoma who underwent surgical treatment. Tumor stages were T1 (n = 10). T2 (n = 17), and T3 (n = 28). Cholangiographic findings were classified as "diffuse sclerosis," "stenosis," "papillary polypoid filling defect," or "nodular polypoid filling defect". "Papillary polypoid filling defect" was the term used when the width of the base was smaller than the width of the polypoid filling defect. RESULTS: T1 patients showed papillary polypoid filling defects (n = 8) or nodular polypoid filling defects (n = 2) on cholangiography. When cholangiography showed papillary polypoid filling defects, 8 of the 14 resected patients showed T1 stage tumor histologically. CONCLUSIONS: In this study, 57% (8/14) of resected patients with papillary polypoid filling defects showed T1 stage tumor. No T1 stage tumor showed stenosis or diffuse sclerosis.     

Recurrent Cholangitis by Biliary Stasis Due to Non-Obstructive Afferent Loop Syndrome After Pylorus-Preserving Pancreatoduodenectomy: Report of a Case

We report a 71-year-old man who had undergone pylorus-preserving pancreatoduodenectomy (PPPD) using PPPD-IV reconstruction for cholangiocarcinoma. For 6 years thereafter, he had suffered recurrent cholangitis, and also a right liver abscess (S5/8), which required percutaneous drainage at 9 years after PPPD. At 16 years after PPPD, he had been admitted to the other hospital because of acute purulent cholangitis. Although medical treatment resolved the cholangitis, the patient was referred to our hospital because of dilatation of the intrahepatic biliary duct (B2). Peroral double-balloon enteroscopy revealed that the diameter of the hepaticojejunostomy anastomosis was 12 mm, and cholangiography detected intrahepatic stones. Lithotripsy was performed using a basket catheter. At 1 year after lithotripsy procedure, the patient is doing well. Hepatobiliary scintigraphy at 60 minutes after intravenous injection demonstrated that deposit of the tracer still remained in the upper afferent loop jejunum. Therefore, we considered that the recurrent cholangitis, liver abscess, and intrahepatic lithiasis have been caused by biliary stasis due to nonobstructive afferent loop syndrome. Biliary retention due to nonobstructive afferent loop syndrome may cause recurrent cholangitis or liver abscess after hepaticojejunostomy, and double-balloon enteroscopy and hepatobiliary scintigraphy are useful for the diagnosis of nonobstructive afferent loop syndrome.Key words: Nonobstructive afferent loop syndrome, Biliary stasis, Hepaticojejunostomy, Hepatobiliary scintigraphy, Double-balloon enteroscopyIt has been reported that cholangitis occurs in between 6.7% and 14.3% of postoperative pancreatoduodenectomy (PD).¹ Most cases of cholangitis originate due to biliary stasis, which is broadly caused by either anastomotic or nonanastomotic stenosis. In many cases, anastomotic stenosis is accompanied by intrahepatic biliary duct dilatation and obstructive jaundice, making early diagnosis and treatment possible.^2–3 On the other hand, nonanastomotic stenosis, including those of afferent loop syndrome, is performed as a conservative treatment for unexplained fever and cholangitis. However, in many cases, the cause remains unidentified, thereby causing this condition to repeat itself. Since cholangitis can at times be fatal, it is therefore important to identify the cause.It has been reported that afferent loop syndrome occurs in around 13% of postoperative PD patients.⁴ Afferent loop syndrome is generally caused by mechanical occlusion due to the recurrence or metastasis of cancer,^4–6 adhesion,^7–8 torsion,⁹ internal hernia,¹⁰ enterolithiasis,^11–12 etc., and thereafter, leads to a syndrome associated with acute abdominal symptom or acute cholangitis. On the other hand, nonobstructive afferent loop syndrome may also be caused by biliary stasis due to jejunal motility failure or the length of the blind end or jejunum, and thereafter, leads to acute cholangitis, liver abscess, and the formation of enterolithiasis and intrahepatic stones. Nonobstructive afferent loop syndrome occurs in around 37% of all of the afferent loop syndrome,^12–13 but few cases have actually been reported.We herein report a rare case in which the patient experienced recurrent cholangitis and liver abscess by biliary stasis due to nonobstructive afferent loop syndrome after pylorus-preserving pancreatoduodenectomy (PPPD) for cholangiocarcinoma.