A case of IgG4-related pulmonary disease with rapid improvement

We report a 72-year-old man with respiratory involvement of immunoglobulin G4 (IgG4)-related disease, who developed dry cough and shortness of breath on effort. The chest computed tomography scan image showed massive and diffuse ground-glass opacity, interlobular thickening, and bronchial wall thickening. The infiltration of IgG4-positive plasma cells in the transbronchial lung biopsy and high serum IgG4 concentrations were found. The patient was treated with 0.6?mg/kg oral prednisolone and showed rapid improvement. This is a case of IgG4-related disease in which the only complication was respiratory involvement.     

Tibial tubercle malposition in patellar joint instability: A computed tomography study in full extension and at 30° flexion

We evaluated the tibial tubercle position in knees with patellar instability. CT in full extension and at 30° flexion was used in 18 knees with instability and 18 control knees. Scans were taken at the level of the femoral epicondyles, tibial tubercle and distal tibia. We found that in full extension, the tibial tubercle was in a more lateral position in the unstable than in the control knees. At 30° flexion, the tibial tubercle in the unstable knees rotated internally, but it was never within the normal range. CT scans taken in full extension and at 30° flexion seem to be of value for ascertaining the degree of tibial tubercle malposition during knee flexion in patellar instability.     

Elevated urinary β2 microglobulin in the first identified Japanese family afflicted by X-linked myopathy with excessive autophagy

Here we report what is to our knowledge the first identified Japanese family afflicted by X-linked myopathy with excessive autophagy. The index case is a 52-year-old man with almost 40 years of progressive proximal muscle weakness. High urinary β2 microglobulin, normal serum β2 microglobulin, autophagic vacuoles with sarcolemmal features, and a hemizygous c.164–7T>G mutation in the VMA21 gene were found. His two maternal uncles had similar clinicopathological findings. High urinary β2 microglobulin without obvious renal dysfunction might result from decreased urine acidification in the distal convoluted tubules caused by the VMA21 gene mutation. These findings might prove to be useful as a preliminary marker suggestive of X-linked myopathy with excessive autophagy.     

Antibody‐mediated rejection after ABO‐incompatible pediatric living donor liver transplantation for propionic acidemia: A case report

We herein present the case of a four‐yr‐old boy with PA who developed AMR after ABO‐incompatible LDLT despite undergoing B cell desensitization using rituximab. Although the CD19+ lymphocyte count decreased to 0.1% nine days after the administration of rituximab, he developed a high fever which was accompanied by arthralgia due to a streptococcal infection 13 days after rituximab prophylaxis. After the clearance of the infection, he underwent ABO‐incompatible LDLT 36 days after the administration of rituximab. The CD19+ lymphocyte count just prior to LDLT was 1.2%. He developed AMR five days after LDLT, and the antidonor‐type IgM and IgG antibody titers increased to 1:1024 and 1:1024, respectively. He was treated by plasma exchange, IVIG, steroid pulse therapy, and rituximab re‐administration; however, his liver dysfunction continued. Despite intensive treatment, he died due to complicated abdominal hernia, acute renal failure, and ARDS. This case suggests that a streptococcal infection may induce the activation of innate immune responses; thus, additional desensitization therapy should be considered prior to ABO‐incompatible LDLT if B cell reactivation is suspected.     

Tonsillectomy reduces recurrence of IgA nephropathy in mesangial hypercellularity type categorized by the Oxford classification

Background

In patients with IgA nephropathy (IgAN), recurrence after steroid pulse therapy is associated with reduced renal survival. However, the predictors of recurrence have not yet been clarified.

Methods

All patients who received 6-month steroid pulse therapy from 2004 to 2010 in our four affiliated hospitals and achieved a reduction of proteinuria to <0.4 g/day 1 year after treatment were retrospectively evaluated. The primary outcome was proteinuria ≥1.0 g/day during follow-up or additional antiproteinuric therapy. Two histological classifications were evaluated, the Oxford Classification with a split system and Japanese histological grades (HGs) with a lumped system.

Results

During a median follow-up of 3.4 years, 27 (26.7 %) of the 101 patients showed recurrence. Multivariate analysis showed that HG was the only significant predictor of recurrence, with HG 2+3+4 vs HG 1 having a hazard ratio of 7.38 (95 % confidence interval 1.52–133). Furthermore, in patients with mesangial hypercellularity according to the Oxford Classification, cumulative rate of recurrence-free survival was greater in patients with steroid therapy plus tonsillectomy compared with those who received steroid therapy alone (Log-rank test, P = 0.022). However, this association was not observed in patients without mesangial hypercellularity.

Conclusions

HG is a novel predictor of recurrence after steroid pulse therapy in patients with IgAN. Moreover, the combination of steroid pulse therapy plus tonsillectomy may indicate a lower risk of recurrence in patients with mesangial hypercellularity, as defined by the Oxford Classification.

     

The Role of Patient–Provider Communication in Engagement and Re-engagement in HIV Treatment in Bamako,Mali: A Qualitative Study

Mounting evidence in sub-Saharan Africa suggests poor patient-provider communication (PPC) negatively impacts patient engagement (retention in care and adherence to medication) in antiretroviral therapy (ART) programs. In Bamako, Mali, where 36% of ART patients are lost to follow-up within 12 months of initiating treatment, we aimed to define features of positive PPC according to patient values and explore the mechanisms by which these features may sustain engagement and re-engagement according to patient and provider experiences. We conducted 33 in-depth interviews and 7 focus groups with 69 patients and 17 providers in five ART clinics. Regarding sustaining engagement, participants highlighted “establishing rapport” as a foundational feature of effective PPC, but also described how “responding to emotional needs”, “eliciting patient conflicts and perspective” and “partnering to mitigate conflicts” functioned to address barriers to engagement and increase connectedness to care. Patients who had disengaged felt that “communicating reacceptance” may have prompted them re-engage sooner and that tailored “partnering to mitigate conflicts” would be more effective in sustaining re-engagement than the standard adherence education providers typically offer. Optimizing provider skills related to these key PPC features may help maximize ART patient engagement, ultimately improving health outcomes and decreasing HIV transmission in sub-Saharan Africa.     

Orbital seeding of mesenchymal stromal cells increases osteogenic differentiation and bone-like tissue formation

In bone tissue engineering (TE), an efficient seeding and homogenous distribution of cells is needed to avoid cell loss and damage as well as to facilitate tissue development. Dynamic seeding methods seem to be superior to the static ones because they tend to result in a more homogeneous cell distribution by using kinetic forces. However, most dynamic seeding techniques are elaborate or require special equipment and its influence on the final bone tissue-engineered construct is not clear. In this study, we applied a simple, dynamic seeding method using an orbital shaker to seed human bone marrow–derived mesenchymal stromal cells (hBMSCs) on silk fibroin scaffolds. Significantly higher cell numbers with a more homogenous cell distribution, increased osteogenic differentiation, and mineral deposition were observed using the dynamic approach both for 4 and 6 hours as compared to the static seeding method. The positive influence of dynamic seeding could be attributed to both cell density and distribution but also nutrient supply during seeding and shear stresses (0.0-3.0 mPa) as determined by computational simulations. The influence of relevant mechanical stimuli during seeding should be investigated in the future, especially regarding the importance of mechanical cues for bone TE applications. Our results highlight the importance of adequate choice of seeding method and its impact on developing tissue-engineered constructs. The application of this simple seeding technique is not only recommended for bone TE but can also be used for seeding similar porous scaffolds with hBMSCs in other TE fields.