Budd-Chiari Syndrome Leading to Cirrhosis in a Young Woman

Introduction: A young woman with a past medical history of hepatic steatosis and polycystic ovarian syndrome on oral contraceptive pills presents with ascites and diffuse hepatocellular disease of unknown etiology. Advanced liver disease is rather unusual in young adults because cirrhosis typically develops gradually after years of persistent liver injury. Cirrhosis is commonly caused by alcohol abuse, viral hepatitis, or nonalcoholic fatty liver disease. Other potential etiologies include autoimmune, iatrogenic, veno-occlusive, and genetic disorders such as Wilson disease or hemochromatosis. This case report suggests that veno-occlusive conditions such as Budd-Chiari syndrome should be considered in young adults presenting with cirrhosis, especially those with hypercoagulable risk factors and lack of other contributors such as alcohol use and viral hepatitis. Cirrhosis is rare in this age group, but hypercoagulable states may manifest in this manner. The Case: The patient initially presented to an outside hospital for right upper quadrant pain, nausea, and vomiting. Imaging of the gallbladder revealed a thickened wall and a decreased gallbladder ejection fraction. For this reason, a laparoscopic cholecystectomy was performed. During the procedure, the liver appeared cirrhotic and ascitic fluid was present. The team drained two liters of fluid and biopsied the liver. A few days later, the patient noted continuous drainage from the port sites and crampy epigastric pain. A week later, her heart rate was 144 and she was readmitted to the hospital at this time. At the hospital, a CT scan showed diffuse hepatocellular disease with mesenteric lymphadenopathy. Two more paracenteses were done, draining four and three and a half liters respectively. The serum-to-ascitic fluid gradient was greater than 1.1 g/dl, indicating portal hypertension. She drinks one alcoholic beverage per week and viral hepatitis serologies were negative. Despite several other tests, no explanation for the advanced liver disease was discovered, and she was transferred to our hospital for escalation of care. On admission, another CT of the liver was done, which found Budd-Chiari syndrome and bilateral lower lobe pulmonary embolisms. Interventional radiology was consulted, and they successfully performed a direct intrahepatic portocaval shunt (DIPS) procedure. She was taken off of oral contraceptive pills and started on apixaban. Discussion and Conclusion: Budd-Chiari syndrome is uncommon, but when it is diagnosed, the patients are usually hypercoagulable. In primary Budd-Chiari syndrome, a thrombus forms in the hepatic veins, preventing blood from leaving the liver. This patient was likely in a hypercoagulable state secondary to her oral contraceptive use. Treatment of Budd-Chiari syndrome includes addressing the underlying cause, starting anticoagulation, and treating portal hypertension if present. In young patients presenting with a cirrhotic picture, Budd-Chiari syndrome is an important diagnosis to consider in those with hypercoagulable risk factors. Although they may have other risk factors for liver disease, it is unlikely that these conditions would progress rapidly to advanced disease by young adulthood.https://scholarlycommons.henryford.com/merf2020caserpt/1135/thumbnail.jp

Use of PRAME Immunostaining to Distinguish Melanoma in Situ From Lentigo Senilis

PRAME (PReferentially expressed Antigen in MElanoma) is an antigen that is expressed by malignant cells in melanoma, as well as other cancers such as breast carcinoma, renal cell carcinoma, and leukemia. PRAME immunohistochemistry has proved effective in identifying malignant melanocytes in melanoma lesions, but it is unclear if it may be used to distinguish melanoma from benign melanocytic conditions, such as lentigo senilis. In particular, melanoma in situ may be confused with lentigo senilis clinically and histologically, thus PRAME immunostaining is potentially useful for differentiating these two lesions. We evaluated 31 samples of lentigo senilis, 26 of melanoma in situ, and 17 of sun-damaged skin with PRAME immunostain. We found that there is significantly greater PRAME expression in melanoma in situ compared to both lentigo senilis and sun-damaged skin. Although these benign skin lesions contain some cells that are immunoreactive for PRAME, these cells are sparse compared to the dense PRAME-positive cells in melanoma in situ. This suggests that PRAME immunostaining could be a clinically useful tool for distinguishing melanoma in situ from lentigo senilis. However, it should be combined with other data, such as clinical impression and histology with H&E stain, given the possibility for false positive results

28522 The impact of the SARS-CoV-2 pandemic on phototherapy utilization

Phototherapy is a mainstay of treatment for several dermatologic conditions. Patients often require multiple treatments per week for several weeks to months to achieve treatment efficacy. The SARS-CoV-2 global pandemic caused many dermatology clinics to close completely or significantly reduce patient volumes, which may have limited patient access to this beneficial treatment. This retrospective study examines the pandemic’s impact on phototherapy treatment rates and reimbursement at one major tertiary care center and five locations of a private dermatology clinic in Southeast Michigan. Phototherapy CPT reimbursement data from March 1-June 30, 2020 was compared with the same timeframe in 2019. Units of phototherapy performed decreased by an average of 84%, and there was an average decrease of 43% in the number of unique patients receiving treatments. Reimbursement for phototherapy decreased by an average of 83%. The drastic decline in phototherapy reimbursement is a reflection of the pandemic’s financial impact and likely correlates to a larger scale of revenue loss in dermatology practices. Adequate phototherapy treatment was also likely delayed for many patients. As the pandemic continues, implementation of home phototherapy treatments may be necessary for patients to receive proper treatment and to minimize the impact of loss of revenue due to limited in-office phototherapy. Precautions will need to be taken to guarantee the safety of patients and the care team for patients to receive optimal in-office phototherapy treatment. The pandemic’s impact on medical dermatology finances could potentially destabilize access to patients who need this safe and effective treatment

Is photoacoustic imaging clinically safe: evaluation of possible thermal damage due to laser-tissue interaction

Photoacoustic imaging is a breakthrough imaging modality that combines the spatial resolution of ultrasound imaging with the contrast of optical imaging. This imaging technique is being pushed towards clinical acceptance for many applications, such as noninvasive diagnosis and management of a multitude of neoplastic lesions. However, a rigorous evaluation of the tissue thermal response to the laser illumination is required prior to the clinical translation. In this study, we assessed the temperature rise profile and microstructural damage of the skin due to the laser-tissue interaction using in-vivo mouse models. We compared the effect of two different laser frequencies (10 Hz and 30 Hz) on the skin and studied if the use of a cooling method could be clinically useful in preventing tissue necrosis. Two biopsies were taken from each mouse 48 hours after laser exposure; one from the skin directly exposed to the laser and one from neighboring healthy tissue. When the lower frequency laser was used, no necrosis was found on histologic analysis. However, when the higher frequency laser was used, necrosis was noted in the epidermis, dermal collagen, and hair follicles at the site of laser exposure. Use of the cooling method with the higher frequency laser led to no tissue necrosis. Overall, it appears that photoacoustic imaging is likely safe when lower frequency lasers are used, and the implementation of the cooling method seems to mitigate necrosis when the use of a higher frequency laser is warranted. This opens up exciting new possibilities for a noninvasive way of diagnosing and evaluating a variety of lesions, including malignant tumors. However, some further studies are needed before photoacoustic imaging can be clinically used in human subjects

Telogen effluvium associated with COVID-19 infection

Telogen effluvium (TE) is characterized by diffuse hair shedding 2-3 months after a stressor, and COVID-19 infection is potentially one such stressor. Those who were infected with the virus were under immense psychosocial and physiologic stress. We retrospectively reviewed electronic medical records of 552 patients who were evaluated by a Henry Ford Health System dermatologist between February 2020 and September 2020 and had a diagnosis of COVID-19 infection. Ten patients were identified with TE attributed to COVID-19 infection and described their presentations as a case series. For the ten patients selected, the mean age was 48.5 years old and 90% were female. Six of the patients were Black, one Middle Eastern, and three White. On average, the hair shedding began 50 days after the first symptom of COVID-19 infection. About 80% of these patients were treated with antibiotics, systemic corticosteroids, and/or hydroxychloroquine for their COVID-19 infection and 70% were hospitalized. The presentations of these patients suggest that COVID-19 infection may be a significant trigger of TE. TE caused by hydroxychloroquine, azithromycin or other medications cannot be ruled out, and the global pandemic itself is a source of psychosocial stress. Further studies will be needed to understand the long-term prevalence and prognosis of TE associated with COVID-19 infection

Novel imaging biomarkers for mapping the impact of mild mitochondrial uncoupling in the outer retina in vivo.

PURPOSE: To test the hypothesis that imaging biomarkers are useful for evaluating in vivo rod photoreceptor cell responses to a mitochondrial protonophore. METHODS: Intraperitoneal injections of either the mitochondrial uncoupler 2,4 dinitrophenol (DNP) or saline were given to mice with either higher [129S6/eVTac (S6)] or lower [C57BL/6J (B6)] mitochondrial reserve capacities and were studied in dark or light. We measured: (i) the external limiting membrane-retinal pigment epithelium region thickness (ELM-RPE; OCT), which decreases substantially with upregulation of a pH-sensitive water removal co-transporter on the apical portion of the RPE, and (ii) the outer retina R1 (= 1/(spin lattice relaxation time (T1), an MRI parameter proportional to oxygen / free radical content. RESULTS: In darkness, baseline rod energy production and consumption are relatively high compared to that in light, and additional metabolic stimulation with DNP provoked thinning of the ELM-RPE region compared to saline injection in S6 mice; ELM-RPE thickness was unresponsive to DNP in B6 mice. Also, dark-adapted S6 mice given DNP showed a decrease in outer retina R1 values compared to saline injection in the inferior retina. In dark-adapted B6 mice, transretinal R1 values were unresponsive to DNP in superior and inferior regions. In light, with its relatively lower basal rod energy production and consumption, DNP caused ELM-RPE thinning in both S6 and B6 mice. CONCLUSIONS: The present results raise the possibility of non-invasively evaluating the mouse rod mitochondrial energy ecosystem using new DNP-assisted OCT and MRI in vivo assays

Sildenafil-evoked photoreceptor oxidative stress in vivo is unrelated to impaired visual performance in mice.

PurposeThe phosphodiesterase inhibitor sildenafil is a promising treatment for neurodegenerative disease, but it can cause oxidative stress in photoreceptors ex vivo and degrade visual performance in humans. Here, we test the hypotheses that in wildtype mice sildenafil causes i) wide-spread photoreceptor oxidative stress in vivo that is linked with ii) impaired vision.MethodsIn dark or light-adapted C57BL/6 mice ± sildenafil treatment, the presence of oxidative stress was evaluated in retina laminae in vivo by QUEnch-assiSTed (QUEST) magnetic resonance imaging, in the subretinal space in vivo by QUEST optical coherence tomography, and in freshly excised retina by a dichlorofluorescein assay. Visual performance indices were also evaluated by QUEST optokinetic tracking.ResultsIn light-adapted mice, 1 hr post-sildenafil administration, oxidative stress was most evident in the superior peripheral outer retina on both in vivo and ex vivo examinations; little evidence was noted for central retina oxidative stress in vivo and ex vivo. In dark-adapted mice 1 hr after sildenafil, no evidence for outer retina oxidative stress was found in vivo. Evidence for sildenafil-induced central retina rod cGMP accumulation was suggested as a panretinally thinner, dark-like subretinal space thickness in light-adapted mice at 1 hr but not 5 hr post-sildenafil. Cone-based visual performance was impaired by 5 hr post-sildenafil and not corrected with anti-oxidants; vision was normal at 1 hr and 24 hr post-sildenafil.ConclusionsThe sildenafil-induced spatiotemporal pattern of oxidative stress in photoreceptors dominated by rods was unrelated to impairment of cone-based visual performance in wildtype mice