The Clinical Challenges of Diagnosing Acutely Decompensating Amyloidosis.

Advanced amyloidosis and related multi-organ manifestations are devastating clinical scenarios. Because ambiguous presentation of amyloidosis may occur, early diagnosis and prevention of organ damage, such as cardiac injury, is essential and requires high clinical intuition. Our patient was a middle-aged female with a past medical history of heart failure with several decompensation episodes who presented with jaundice, itchiness, and weight loss. Further workup revealed pulmonary hypertension, restrictive heart disease, possible underlying obstructive liver disorder, and hyperkalemia. During admission, the patient established bradycardia and required a pacemaker temporarily, and later she manifested atrial fibrillation. Liver biopsy primarily was suggestive of hepatic congestion. Unfortunately, the patient died during workup due to cardiac arrest. Premortem laboratory results were suggestive of amyloidosis, which was confirmed later by re-examining the liver biopsy with Congo red. Diagnosis of amyloidosis requires early clinical suspicion and workup to prevent its progression to fatal organ involvement such as cardiac complications

Ethnic differences in lens parameters measured by ocular biometry in a cataract surgery population.

To investigate whether differences exist in lens position and other lens parameters among major ethnic groups with cataractous eyes, which may help explain racial differences in angle closure risk.This retrospective, cross-sectional study included 807 adult patients who had cataract surgery between years 2014 and 2016 at the University of California, San Francisco (UCSF). Adult patients of white, Asian, Hispanic and African-American ethnicity were included. Lens position (LP), defined as anterior chamber depth (ACD) + 1/2 lens thickness (LT), was assessed using measurements from optical biometry. Other assessed biometric parameters included axial length (AL), relative lens position (RLP) (defined as LP/AL), and anterior chamber depth (ACD).A total of 807 patients and 1361 eyes were included in this study from a database of patients having cataract surgery. Mean age was 69.2 years (age range from 18 to 101 years old), and 60.3% of patients were women. The mean LP measurements were 5.54±0.32 mm for white, 5.38±0.32 mm for Asian, 5.32±0.30 mm for Hispanic, and 5.40±0.28 mm for African-American participants. After adjusting for age, sex, and AL, significant differences were found when comparing LP in paired comparisons among White cohort with Asians (P<0.001), Hispanics (P<0.001) and African-Americans (P = 0.003). Additionally, when comparing RLP, similar significant results were found when comparing Whites with Asians (P<0.001), Hispanics (P<0.001) and African-Americans (P = 0.002). Lastly, pair-wise comparison of LT between ethnic groups showed significant differences while comparing Asians with Whites (P = 0.001) and Asians with African-Americans (P<0.001).The results of this study suggest that the LP of Hispanic, Asian, and African-American patients are significantly smaller than that of White patients, and among all ethnic groups, Hispanics and after Asians have the smallest LP (P<0.001) and RLP (P<0.001). These findings may have implications for the relative risk of angle closure and the potential IOP response after cataract surgery among different ethnic groups

Spontaneous Recurrent Pneumoperitoneum due to Opioid-Induced Constipation: A Case Report.

Long-term treatment with or addiction to methadone and other opiates can lead to serious complications such as opioid-induced constipation (OIC). Here we report a case where a long-term opioid user presents in the ER in respiratory distress. Radiographic findings concerning pneumoperitoneum and cooperation with specialists lead to a diagnosis of stercoral colitis with possible micro-perforations. Through fecal disimpaction and counseling on chronic opioid use, the patient initially improved, but consecutively had a fatal outcome

Unilateral Purple Urine Bag Syndrome in an Elderly Man with Nephrostomy.

Purple urine bag syndrome, or PUBS, is a manifestation of a complicated urinary tract infection. Organisms such as Escherichia coli (E. coli) and Enterococcus can reside in urinary catheters and exhibit the purple color detected in this phenomenon. Risk factors described for this syndrome include the use of plastic urinary catheterization, the malfunctioning of the catheter, and long-term institutionalization. This disorder could be the earliest presentation of a urinary catheter flaw and requires immediate intervention and revision. In our case, a male resident of nursing home presented with urosepsis and appropriate antibiotics were initiated. Computed tomography (CT) urogram was done and showed left kidney hydronephrosis and bilateral staghorn calculi. To address the source of infection, a nephroureteral drain was placed in both kidneys. A few days after the initiation of treatment and urological intervention, urine on the left side became purple. The urologist re-evaluated the nephroureteral drainage tubes and replaced them. The purple color in the urine resolved later. In our case, PUBS was the earliest sign of urinary drainage malfunctioning and required early intervention and treatment

Blastic Plasmacytoid Dendritic Cell Neoplasm without Cutaneous Manifestation: A Case Report.

BACKGROUND As an uncommon malignancy with the highest prevalence in the elderly population, blastic plasmacytoid dendritic cell neoplasm or BPDCN is a hematologic disorder with unknown pathogenesis and devastating outcomes. This neoplasm usually manifests in the skin but can also involve the bone marrow, and less frequently the central nervous system (CNS). However, it does not exclude other organs and can even be associated with other malignancies. CASE REPORT Here, we discuss an interesting case of BPDCN in an 85-year-old man who mainly presented with dizziness and weakness. Physical examination revealed splenomegaly, laboratory tests showed pancytopenia, and peripheral blood smear depicted metamyelocytes. Further workup including bone marrow biopsy revealed atypical cells and flow cytometry disclosed 84% blasts positive for cluster of differentiation (CD) 4, CD53, and CD156 suggestive of BPDCN. Moreover, cerebrospinal fluid (CSF) studies came back positive for tumor plasmacytoid dendritic cells. The patient underwent chemotherapy with CHOP, mini-CHOP regimens, and venetoclax, as well as treatment for CNS involvement. He achieved remission, but unfortunately had a recurrence of the disease. Later he was admitted due to pneumonia with concomitant recurrent pulmonary effusions complicated by multiorgan dysfunction and subsequently died. CONCLUSIONS The diagnosis of BPDCN can be very challenging, and high clinical suspicion and intuition are required to reach the diagnosis, especially when patients do not present with cutaneous involvement. Concerning treatment options, novel therapies such as tagraxofusp, a CD123-directed cytotoxin, are emerging in the hope of decreasing the rate of mortality for this aggressive malignancy

Comparisons of relative lens position between ethnic cohorts.

<p>Comparisons of relative lens position between ethnic cohorts.</p

Comparisons of lens position (LP) between ethnic cohorts.

<p>Comparisons of lens position (LP) between ethnic cohorts.</p

Comparison of demographic and clinical characteristics of the ethnic cohorts.

<p>Comparison of demographic and clinical characteristics of the ethnic cohorts.</p