Prognostic Utility of the IPS 3 Score for Predicting Outcomes in Advanced Hodgkin Lymphoma

Background

The International Prognostic Scoring System (IPSS) consisting of 7 parameters (IPS7) has been the standard prognostic model used in advanced Hodgkin lymphoma (aHL). However, recent studies have questioned its discriminatory power. For retrospective analyses, its utility might be limited by missing parameters. A recent study has shown that the IPSS consisting of only 3 high-risk features (IPS3; stage IV, age 45 years or older, and hemoglobin <105 g/L) is a simple predictor of survival in aHL. However, there are limited data validating the IPS3.

Bioprinted gelatin hydrogel platform promotes smooth muscle cell contractile phenotype maintenance

Three dimensional (3D) bioprinting has been proposed as a method for fabricating tissue engineered small diameter vascular prostheses. This technique not only involves constructing the structural features to obtain a desired pattern but the morphology of the pattern may also be used to influence the behavior of seeded cells. Herein, we 3D bioprinted a gelatin hydrogel microchannel construct to promote and preserve the contractile phenotype of vascular smooth muscle cells (vSMCs), which is crucial for vasoresponsiveness. The microchanneled surface of a gelatin hydrogel facilitated vSMC attachment and an elongated alignment along the microchannel direction. The cells displayed distinct F-actin anisotropy in the direction of the channel. The vSMC contractile phenotype was confirmed by the positive detection of contractile marker gene proteins (α-smooth muscle actin (α-SMA) and smooth muscle-myosin heavy chain (SM-MHC)). Having demonstrated the effectiveness of the hydrogel channels bioprinted on a film, the bioprinting was applied radially to the surface of a 3D tubular construct by integrating a rotating mandrel into the 3D bioprinter. The hydrogel microchannels printed on the 3D tubular vascular construct also orientated the vSMCs and strongly promoted the contractile phenotype. Together, our study demonstrated that microchannels bioprinted using a transglutaminase crosslinked gelatin hydrogel, could successfully promote and preserve vSMC contractile phenotype. Furthermore, the hydrogel bioink could be retained on the surface of a rotating polymer tube to print radial cell guiding channels onto a vascular graft construct.     

Autosomal recessive otofaciocervical syndrome type 2 with novel homozygous small insertion in PAX1 gene

Otofaciocervical syndrome (OTFCS) is described as a single gene disorder of both autosomal dominant and autosomal recessive inheritance. The major clinical features of OTFCS include ear malformations (external/middle/inner ear), facial dysmorphism, shoulder girdle abnormalities, vertebral anomalies, and mild intellectual disability. The autosomal recessive form of OTFCS syndrome (OTFCS2) has been recently reported to be caused due to homozygous mutations in PAX1 gene. Here we report a third family of OTFCS2 phenotype wherein whole exome sequencing identified a novel homozygous small insertion in PAX1 as the underlying genetic cause.     

Magnetic resonance angiographic evaluation of circle of Willis: A morphologic study in a tertiary hospital set up

Background:

Anatomy of circle of Willis (CW) shows wide variation in different individuals, population groups, and has vital clinical significance in causation and presentation of clinical disease. This study evaluates the anatomical variations, incidence of various common anomalies of CW in south Indian tertiary hospital set up, using three-dimensional time-of-flight (3D-TOF) magnetic resonance angiography (MRA).

Materials and Methods:

A total of 300 patients referred for neuroimaging study over a period of 2-year were included in the analysis. In this prospective and retrospective study, 198 men and 102 women; mean age, 55 years) underwent 3D-TOF MR angiograms of the CW using a 1.5-tesla MR scanner. Images were reviewed for anatomical configuration of the CW using maximum intensity projection (MIP) and 3D volume rendered images.

Results:

On analysis, a complete CW was seen in 50 (16.6%) of 300 subjects. An incomplete anterior and posterior CW was found in 66 (22%) The remaining 184 (61.3%) subjects had partially complete CW configuration. The most common type of CW in a single subject was anterior variant type A and posterior type variant E.

Conclusion:

We observed wide variation in CW configuration in our patients. The prevalence of complete configuration of the circle is 16.6%; slightly higher in females and younger subjects. Complete anterior circle was present in 77.3%. Most common anterior variant is type A (normal anterior configuration) with a prevalence of 66%. The most common posterior circle variant is type E (hypoplasia or absence of both PcomA) with 32.6%. Overall, CW variants are slightly more common among the women in comparison to men. Incidence of associated anomalies like aneurysm or arteriovenous malformation (AVM) was comparable to that described in literature.     

Cardiogenic shock from atypical Takotsubo cardiomyopathy attributed to acute disseminated encephalomyelitis lesion involving the medulla

We present here a case of atypical Takotsubo cardiomyopathy arising as a result of a lesion in the medulla oblongata. The patient was diagnosed with acute disseminated encephalomyelitis, and had improvement with intravenous steroids.