Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with 99m Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow.

Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis. 99m technetium pyrophosphate (99mTc PYP scan) has changed the landscape of the non-biopsy diagnosis of ATTR cardiac amyloidosis (ATTR-CA) by providing remarkably high diagnostic accuracy. We examined our experience with PYP scans in patients undergoing workup for ATTR-CA and evaluated the diagnostic workflow in patients with intermediate PYP scan results. Materials and Methods: Retrospective chart review study in which we analyzed data of 84 patients who underwent c-99m pyrophosphate (PYP) SPECT scan for the diagnosis of ATTR-CA from 2017 till 2021 at our institution. We identified three groups: Low uptake (PYPL uptake ratio < 1.2 + visual grade 1/0), n = 30, Intermediate uptake (PYPI uptake ratio 1.2-1.49, visual grade 2/3), n = 25 and High uptake (PYPH uptake ratio ≥ 1.5 + visual grade 2/3), n = 29. We reviewed patients' demographics, medical histories, echo parameters and diagnostic testing including light chain analysis, cardiac magnetic resonance results, and biopsies. Results: Mean patients' age was 73, male-to=female ratio 3:1, 59% of patients were African American. Cardiovascular comorbidities, cardiac biomarkers (BNP and Troponin) and amyloid-related neuropathy were similar in all groups. A statistically significant difference in septal thickness/posterior wall thickness and final diagnosis were found between the groups. The distribution of overall diagnostic testing ratios for the PYPI group included serum protein electrophoresis 92%, urine protein electrophoresis 65%, free light chain 80%, CMR 32%, tissue biopsy done in 20% and BM biopsy in 16%, which are similar to the ratios of other groups. Overall, 25% (n = 5, 4 TTR-CA and 1 AL Amyloid) of patients in the PYPI group had a final diagnosis of CA established with additional testing (p = 0.001 vs. other groups). Conclusions: The 99mPYP scan is an accurate noninvasive test for cardiac ATTR-CA. Importantly, 25% of the PYPI group had a final diagnosis of ATTR-CA reiterating that diagnosis needs to be pursued in PYPI cases based on clinical suspicion. Routine evaluation and exclusion of light chain disease and establishing a consistent workflow for amyloid diagnosis and continued education for technologists and readers of PYP scans is key to a successful amyloidosis workup.

Traumatic dislocation of phakic anterior chamber iris-claw intraocular lens: A case report.

We are presenting a traumatic dislocation of a Phakic intraocular lens (PIOL) secondary to a blunt trauma to the right eye. The patient presented with a sudden blurring of vision with no other associated injury. His slit lamp examination showed disenclavation of the temporal clip and pre-operatively his best spectacle-corrected visual acuity (BSCVA) was 6/36. The patient was brought to the operating room where his lens was repositioned and re-enclaved. He had a complete restoration of vision with a BSCVA of 6/9 and an endothelial cell count of 2786/mm2. We conclude that a traumatic dislocation of an iris-claw PIOL can be successfully corrected without immediate postoperative complications and endothelial cell count loss.

Assessment and Improvement of Quality of Life in Patients Undergoing Treatment for Head and Neck Cancer.

INTRODUCTION: We compared the pre and post-treatment quality of life in head and neck cancer (HNC) patients and identified factors that could improve the quality of life in such patients.   Methods: The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire C30 (EORTC QLQ-C30) questionnaire was administered to 84 patients' pre and post-treatment. Patients who had non-metastatic, measurable, and untreated HNCs were invited, provided that their age was below 80 years. We did not discriminate based on treatment modality, stage of cancer, or co-morbidities. Patients who were mentally incapacitated, with secondary or recurrent HNC, distant metastasis, skin cancer, congenital anomaly of the head and neck, chronic illness, or any previous or current psychiatric illness were excluded from the study. A high mean score on the functional scale and a low score on symptom scale signify a better quality of life. We used the dependent t-test to compare pre and post-treatment scores. RESULTS: We found no statistically significant differences in any variables, except the four symptom scales of diarrhoea, constipation, nausea/vomiting, and financial difficulty. All of these variables had increased mean scores with p values of < 0.001. Also, we found no statistical significance (p = 0.250) when comparing the pre-treatment (59.4 ± 18.3) and post-treatment (61.2 ± 16.2) scores for the global health status. CONCLUSION: We found no improvement in the quality of life in HNC patients despite intervention. In fact, diarrhoea, constipation, nausea/vomiting, and financial difficulty of these patients worsened post-treatment.

Multiple courses of antenatal corticosteroids for preterm birth study: outcomes in children at 5 years of age (MACS-5).

IMPORTANCE: A single course of antenatal corticosteroid therapy is recommended for pregnant women at risk of preterm birth between 24 and 33 weeks' gestational age. However, 50% of women remain pregnant 7 to 14 days later, leading to the question of whether additional courses should be given to women remaining at risk for preterm birth. The Multiple Courses of Antenatal Corticosteroids for Preterm Birth Study (MACS) was an international randomized clinical trial that compared multiple courses of antenatal corticosteroids with a single course in women at risk of preterm birth. OBJECTIVE: To determine the effects of single vs multiple courses of antenatal corticosteroid therapy on death or neurodevelopmental disability (neuromotor, neurosensory, or neurocognitive/neurobehavioral function) at 5 years of age in children whose mothers participated in MACS. Our secondary aims were to determine the effect on height, weight, head circumference, blood pressure, intelligence, and specific cognitive (visual, spatial, and language) skills. DESIGN, SETTING, AND PARTICIPANTS: Cohort follow-up study of children seen between June 2006 and May 2012 at 55 centers. In total, 1724 women (2141 children) were eligible for the study, of whom 1728 children (80.7% of the 2141 eligible children) participated and 1719 children contributed to the primary outcome. INTERVENTION: Single and multiple courses of antenatal corticosteroid therapy. MAIN OUTCOMES AND MEASURES: The primary outcome was death or survival with a neurodevelopmental disability in 1 of the following domains: neuromotor (nonambulatory cerebral palsy), neurosensory (blindness, deafness, or need for visual/hearing aids), or neurocognitive/neurobehavioral function (abnormal attention, memory, or behavior). RESULTS: There was no significant difference between the groups in the risk of death or neurodevelopmental disability: 217 of 871 children (24.9%) in the multiple-courses group vs 210 of 848 children (24.8%) in the single-course group (odds ratio, 1.02 [95% CI, 0.81 to 1.29]; P = .84). CONCLUSIONS AND RELEVANCE: Multiple courses, compared with a single course, of antenatal corticosteroid therapy did not increase or decrease the risk of death or disability at 5 years of age. Because of a lack of strong conclusive evidence of short-term or long-term benefits, it remains our opinion that multiple courses not be recommended in women with ongoing risk of preterm birth. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00187382.