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.     

Efficacy of combination therapy of interferon-α with ursodeoxycholic acid in chronic hepatitis C: A randomized controlled clinical trial

The efficacy of interferon- therapy in the treatment of chronic hepatitis C is still limited. A combination therapy of interferon- with ursodeoxycholic acid (UDCA) was tested for its efficacy in the treatment of chronic hepatitis C by a randomized controlled study. Eighty consecutive Japanese patients with chronic hepatitis C were randomly divided into two groups: one group was treated with interferon- (group A,n=40) and the other with a combination of interferon- and UDCA (group B,n=40). In both groups, human interferon- (6 million units per day) was intramuscularly injected daily for 2 weeks and then three times a week for 22 weeks: this 24-week period was followed by 24 weeks of observation. In group B, UDCA was also administered, daily at a dose of 600mg orally, from the beginning of the interferon therapy and administration was continued for 48 weeks. The rates for ALT normalization and clearance of hepatitis C virus (HCV) viremia at the end of the 24-week interferon therapy were similar for groups A and B (58% vs 60% and 55% vs 48%, respectively). At the end of the 24-week follow-up, the sustained normalization rates for ALT levels for the two groups were not different (35% vs 43%), while the rate of clearance was higher in group B (40%) than in group A (23%), but the difference was not significant (P=0.14). The sustained complete response, i.e., HCV RNA negativity at the end of the follow-up, as well as the maintenance of ALT normalization during the follow-up period, was more frequent in group B (38%) than in group A (18%) although the difference was not significantP=0.08). The rate of HCV reactivation after interferon was discontinued was significantly lower in group B (16%) than in group A (59%) (P<0.01). Although this combination therapy did not lead to a sufficiently sustained complete response, it could serve as adjuvant antiviral therapy when a suitable dosage and administration period are determined.     

Transthyretin (prealbumin) in the pancreas and sera of newly diagnosed type I (insulin-dependent) diabetic patients.

We investigated transthyretin (TTR) in the pancreases and sera of 10 newly diagnosed type I diabetic patients by immunohistochemistry and nephelometry. In the type I diabetic pancreases, glucagon-positive A-cells showed strong immunoreactivity for TTR, the intensity and distribution pattern of which corresponded to those in normal subjects. Morphometric analysis revealed that the amount of strongly TTR-positive A-cells was not significantly different from that in normal subjects. On the contrary, insulin-positive B-cells, which normally show uneven and weak TTR immunoreactivity, decreased in number, and only a few residual B-cells showed faint immunoreactivity. Neither somatostatin cells nor pancreatic polypeptide cells were positive for TTR. The serum TTR concentration showed a significant decrease in type I diabetic patients compared with that in normal subjects (P less than 0.005). These data suggest that the synthesis or storage of TTR in A-cells is not affected, but that in B-cells is impaired in type I diabetes. The decrease in serum TTR might be one of the features of metabolic disorders in type I diabetes.     

Improved survival rate by temperature control at compression sites in rat model of crush syndrome

Background

Crush syndrome (CS) has been reported in disasters, terrorist incidents, and accidents, and the clinical and pathologic picture has gradually been clarified. Few lethal and reproducible animal models of CS with use of a quantitative load are available. A new model is needed to investigate pathologic and therapeutic aspects of this injury.

Materials and methods

Using a device built from commercially available components, both hindlimbs of anesthetized rats were respectively compressed for 6 h using 3.6-kg blocks. The effects of trunk warming alone without compressed hindlimbs (Group A), non-warming at room temperature (Group B), whole-body warming including compressed hindlimbs (Group C), or warming of compressed hindlimbs alone (Group D) during compression were examined. Survival rates were compared and hematological and histologic analyses were performed at specific time points after compression release.

Results

Limb or whole-body warming significantly worsened the survival of rats. We found a much lower survival rate of 0%–10% in animals, in which the hindlimbs were warmed during compression (Groups C and D) at 12 h after compression release, compared with 90%–100% in animals without warming of the hindlimbs (Groups A and B). Groups C and D showed significantly enhanced hyperkalemia at ≥4 h after compression release and all blood samples from dead cases showed hyperkalemia (>10 mEq/L).

Conclusions

We developed a new lethal and reproducible rat CS model with a quantitative load. This study found that warming of compressed limbs worsened the survival rate and significantly enhanced hyperkalemia, apparently leading to cardiac arrest.     

Definitive Chemoradiotherapy and Salvage Esophagectomy for Esophageal Cancer Associated With Multiple Lung Metastases: A Case Report

The prognosis of esophageal cancer with distant metastasis is dismal. We report a 70-year-old man with esophageal cancer and multiple lung and lymph node metastases. Complete response was achieved following definitive chemoradiotherapy. Twenty-four months after the initial chemoradiotherapy, local recurrence was detected but there was no evidence of distant metastasis. Therefore, the patient underwent salvage esophagectomy. The surgery was well tolerated without any postoperative complications. The patient is still alive 48 months after the salvage surgery. Our experience suggests that salvage esophagectomy is an important component of multimodal therapy for the recurrence of esophageal cancer.Key words: Esophageal cancer, Chemoradiotherapy, Salvage surgeryThe prognosis of esophageal cancer has improved in recent years, but remains poor despite curative resection.¹ The prognosis is extremely dismal in patients with distant metastasis. The Radiation Therapy Oncology Group (RTOG) trial 85-01 showed that chemoradiotherapy (CRT) improved outcomes, with a 5-year overall survival rate of 26% compared with 0% following radiotherapy alone. Moreover, residual cancer was less common following CRT (26%) than following radiotherapy alone (37%).² However, local recurrence occurs in 37% of patients after definitive CRT.³ Salvage esophagectomy is one strategy for residual cancer or local recurrence after definitive CRT. Of note, when R0 resection is achieved, long-term survival can be expected.⁴⁻⁶ On the other hand, this is an invasive procedure associated with high morbidity and mortality⁶ and the patient''s prognosis is extremely poor after R1/R2 resection.⁴⁻⁶ Therefore, salvage esophagectomy should only be performed if complete removal of the tumor is expected.Here, we report a rare case with esophageal cancer and multiple lung metastases, in which complete response (CR) was achieved after definitive CRT and salvage esophagectomy was effective for the local recurrence.     

Glasgow Prognostic Score (GPS) Can Be a Useful Indicator to Determine Prognosis of Patients With Colorectal Carcinoma

The Glasgow Prognostic Score (GPS), an inflammation-based score, has been used to predict the biologic behavior of malignant tumors. The aim of the current study was to elucidate a further significance of GPS in colorectal carcinoma. Correlation of GPS and modified GPS (mGPS), which are composed of combined score provided for serum elevation of C-reactive protein and hypoalbuminemia examined before surgical treatment, with clinicopathologic features was investigated in 272 patients with colorectal carcinoma. Survival of GPS 1 patients was significantly worse than that of GPS 0 patients (P= 0.009), and survival of GPS 2 patients was significantly worse than that of GPS 1 patients (P < 0.0001). Similarly, survival of mGPS 1 patients was significantly worse than that of mGPS 0 patients (P = 0.009), and survival of mGPS 2 patients was significantly worse than that of mGPS 1 patients (P = 0.0006). Multivariate analysis demonstrated that GPS (P < 0.0001) as well as tumor stage (P= 0.004) and venous invasion (P = 0.011) were factors independently associated with worse prognosis. Both GPS and mGPS could classify outcome of patients with a clear stratification, and could be applied as prognostic indicators in colorectal carcinoma.Key words: Colorectal carcinoma, Glasgow prognostic score (GPS), Prognostic indicatorAlthough many tumor-environmental elements, including both tumor-related and host-related factors, have been linked with tumor progression, host inflammatory response is one of the more important factors that has a role in the progression and/or development of tumors.¹Serum elevation of C-reactive protein (CRP), an acute phase protein, has been shown to be a prognostic indicator in a variety of neoplasms, including colorectal carcinoma.^2–5 Moreover, hypoalbuminemia brought about by malnutrition and related to cachexic condition has been reported to be correlated with an unfavorable prognosis of some gastrointestinal tumors.^6,7The Glasgow prognostic score (GPS), which is a cumulative inflammation-based cancer-prognostic marker composed of serum elevation of CRP and decrease in albumin concentration, is likely to reflect host systemic inflammatory response and has been reported to be significant as a prognostic indicator in cancer-bearing patients.^8–10Moreover, it has been found that hypoalbuminemia alone is unlikely to be associated with reduced survival likelihood in patients with colorectal carcinoma¹¹; therefore, the GPS has been modified (mGPS), providing a score of 1 only for a case with serum elevation of CRP, and score of 0 for a case only with hypoalbuminemia or where neither was elevated. Although, until now, there have been some reports regarding the significance of GPS as a prognostic indicator in colorectal carcinoma,^9,11–16 the aim of this study was to elucidate further the significance of GPS and mGPS in colorectal carcinoma.     

Basal cell adenocarcinoma of the parotid gland: Comparison with basal cell adenoma for preoperative diagnosis

ObjectiveTo summarize the diagnosis and treatment outcomes of basal cell adenocarcinoma (BCAC) of the parotid gland, a rare low-grade malignancy, at a single institution, and to investigate the treatment approach for this rare malignancy.MethodsWe conducted a retrospective analysis of 9 patients with BCAC during 20 years from September 1999 to December 2019. Forty-five patients with basal cell adenoma (BCA), who were treated during the same time period, were used for comparison. The clinical characteristics of BCAC, diagnostic imaging, the usefulness of fine-needle aspiration cytology (FNAC) and frozen section biopsy (FSB), histological assessment of malignancy, and treatment outcomes were investigated.ResultsThere were no marked differences in sex, age, tumor diameter, or tumor location between BCAC and BCA cases. Among the 9 patients with BCAC, one patient was noted with pain/tenderness, and two patients were observed with adhesion to the surrounding tissues. Only one patient was diagnosed as malignant based on MRI/US. FNAC for BCAC was suspicious for malignancy in 6 of the 9 cases, which included one patient with the correct grade of malignancy, one patient with malignancy only, and 4 patients suspicious for malignancy. FSB was suspicious for malignancy in 8 of the 9 cases. Malignancy grade was determined based on infiltration to the surrounding tissues and expression of Ki-67, p53, and bcl-2. One patient with infiltration to the surrounding tissue was diagnosed as intermediate-grade malignancy, while the remaining 8 patients were diagnosed as low-grade malignancy. The BCAC cases included 7 patients with T2 and 2 patients with T1. Conservative resection was performed for all patients, and all cases are surviving cancer-free.ConclusionThe malignancy of BCAC can be suspected before surgery based on symptoms/signs, diagnostic imaging, and FNAC. FSB enables the diagnosis of not only malignancy but also the grade of malignancy, which may help determine the appropriate surgical resection. Although all 9 patients with BCAC are surviving free from cancer, a long-term follow-up is warranted.     

Gender differences in the incidence and progression of diabetic retinopathy among Japanese patients with type 2 diabetes mellitus: A clinic-based retrospective longitudinal study

A clinic-based retrospective longitudinal study conducted for 5.8 ± 2.5 years, including 383 (M/F 245/138) Japanese patients with type 2 diabetes mellitus showed that females exhibit a significantly higher prevalence of proliferative diabetic retinopathy (DR) at baseline and that female gender is an independent risk factor for the development of DR.     

Scrobiculosides A and B from the deep-sea sponge Pachastrella scrobiculosa

Journal of Natural Medicines - Two new steroidal saponins, scrobiculosides A and B, were isolated from the deep-sea sponge Pachastrella scrobiculosa, collected at a depth of 200&nbsp;m off...