Familial hypercholesterolemia presenting with multiple nodules of the hands and elbow

Familial hypercholesterolemia (FH) is a common but commonly missed diagnosis. Tendon xanthomas are a physical sign strongly suggestive of FH. Physicians must identify tendon xanthomas, apply validated clinical scoring such as the Dutch Lipid Clinic Network criteria and offer cascade screening. This approach will increase recognition of FH.     

Nasal tip modifications

Rhinoplasty remains a challenging art but is now systematized at least in part by recent understanding of the supporting mechanisms and how they may be manipulated to control the nasal tip. Nasal tip control is the key to a successful, aesthetically pleasing, rhinoplasty result with preservation of nasal function.     

Oral healthcare considerations for the pregnant woman

Pregnancy is a period of both joy and anxiety in a woman's life and is characterized by various physiological changes in her body brought about by the circulating female sex hormones. The oral cavity is also the seat of changes and these physiologic changes of pregnancy need to be addressed while managing the pregnant woman in the dental clinic. The main goal is to minimize the occurrence of any complications that might harm the mother-to-be and/or the unborn child. Preventive, emergency, and routine dental procedures are all deemed suitable during various phases of pregnancy, with some treatment modifications and initial planning. Clinical Relevance: The dentist should be in a position to manage pregnant women in clinical practice effectively.     

Bortezomib may stabilize pediatric renal transplant recipients with antibody-mediated rejection

Background

Current therapeutic strategies to effectively treat antibody-mediated rejection (AMR) are insufficient. Thus, we aimed to determine the benefit of a therapeutic protocol using bortezomib for refractory C4d + AMR in pediatric kidney transplant patients.

Methods

We examined seven patients with treatment-refractory C4d + AMR. Immunosuppression included antithymocyte globulin or anti-CD25 monoclonal antibody for induction therapy with maintenance corticosteroids, calcineurin inhibitor, and anti-metabolite. Estimated glomerular filtration rate (eGFR) calculated by the Schwartz equation, biopsy findings assessed by 2013 Banff criteria, and human leukocyte antigen (HLA) donor-specific antibodies (DSA) performed using the Luminex single antigen bead assay were monitored pre- and post- bortezomib therapy.

Results

Seven patients (86 % male, 86 % with ≥6/8 HLA mismatch, and 14 % with pre-formed DSA) age 5 to 19 (median 15) years developed refractory C4d + AMR between 1 and 145 (median 65) months post-transplantation. All patients tolerated bortezomib. One patient had allograft loss. Of the six patients with surviving grafts (86 %), mean pre-bortezomib eGFR was 42 ml/min/1.73 m² and the mean 1 year post-bortezomib eGFR was 53 ml/min/1.73 m². Five of seven (71 %) had improvement of histological findings of AMR, C4d staining, and/or acute cellular rejection. Reduction in HLA DSAs was more effective for class I than class II.

Conclusions

Bortezomib appears safe and may correlate with stabilization of eGFR in pediatric kidney transplant patients with refractory C4d + AMR.