Hepatopulmonary syndrome in a 22 year old gentleman with liver cirrhosis

Background: Hepatopulmonary syndrome is a severe complication of end-stage liver disease characterized by triad of liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia. Although the pathogenesis is not completely understood, pulmonary vascular dilatation occurs due to imbalance between vasodilators and vasoconstrictors. Liver injury is thought to increase endothelin production and cause bacterial translocation, causing increased nitric oxide production, causing vasodilation of pulmonary vasculature. History and physical examination are important in leading the physician to the correct diagnosis as the majority of these patients present with non-specific clinical manifestations and imaging. Identification of specific physical exam findings is important in not missing key features of a patient’s physical exam which will give you direction to a diagnosis of hepatopulmonary syndrome. Case Presentation: We are presenting a 22-year-old hispanic male with past medical history of nonalcoholic steatohepatitis-related liver cirrhosis with evidence of portal hypertensive gastropathy with esophageal varices who presented with intermittent dyspnea and desaturation. Physical exam did not have overt signs of volume overload and was positive for platypnea and orthodeoxia. ABG revealed hypoxemia with PaO2 of 66 and orthodeoxia. Diagnosis of hepatopulmonary syndrome was confirmed with contrast Echo ordered which revealed normal ejection fraction and showed R to L shunting by agitated saline contrast. The patient was managed with medical and oxygen therapy. He was discharged home on oxygen therapy as he improved. Patient evaluated by a hepatologist outpatient and placed on the liver transplant list. Discussion: Hepatopulmonary syndrome can often be missed due to its nonspecific presentation. Dyspnea is its most common presenting symptom. However, being aware of other presenting symptoms are key to diagnosis; such as platypnea and orthodeoxia, as present in our case. Obtaining Echo with contrast is important as it will confirm the presence of an intrapulmonary shunt. Differentiating an intracardiac shunt vs intrapulmonary shunt is important. With an intracardiac shunt, contrast appears in the left heart within three heart beats after injection, however, with an intrapulmonary shunt, contrast appears in the left heart after three beats as in our patient. Once diagnosed, oxygen therapy is recommended. Liver transplantation is the only effective therapy

Takotsubo Cardiomyopathy, presentation as a cardiac arrest in a 67 year old female with depression and anxiety history

Background: Takotsubo cardiomyopathy also known as broken heart syndrome or stress induced cardiomyopathy is a sudden transient reversible dramatic left ventricular apical akinesis mimicking acute coronary syndrome, making it a diagnostic challenge. Most common mechanism for Taktosubo is stress induced catecholamine release causing sympathetic activation leading to microvascular dysfunction or direct toxicity. Mayo Clinic Criteria for making diagnosis at the time of presentation requires 1) transient hypokinesis, dyskinesis, or akinesis of the LV midsegments with or without apical involvement, and a stressful trigger is often but not always present. 2) absence of obstructive coronary disease or angiographic evidence of acute plaque rupture 3) new ECG abnormalities (either ST segment elevation and or T wave inversion) 4) absence of pheochromocytoma or myocarditis. Case presentation: A sixty-seven year old Hispanic woman with past medical history of depression and anxiety, presented to emergency department with generalized body weakness, chronic severe lower back pain. In emergency department, patient experienced cardiac arrest with return of spontaneous circulation achieved in 5 mins, was intubated for airway protection. Electrocardiogram showed sinus tachycardia with elevated cardiac troponins. Echocardiogram revealed large area of akinesis involving mid anteroseptal, lateral wall, inferoapical; severe left ventricular dysfunction with ejection fraction 30-35%. She was found to have pulmonary edema and was started on vasopressors, heparin drip, and aggressive diuresis for cardiogenic shock. Imaging negative. Eventually she was able to be weaned off vasopressor support and extubated. Her repeat echo three days later revealed improved left ventricular systolic function with ejection fraction of 45-50%. Patient underwent left heart catheterization which demonstrated no significant obstructive coronary disease. Patient improved clinically and was discharged to a skilled nursing facility for rehabilitation on beta blocker and angiotensin receptor blocker. Discussion: The exact etiology of our patient’s arrest remains unknown. Early suspicion and use of Mayo clinic criteria at time of presentation is important especially in patients with a history of psychiatric disorders as there is a high rate of recurrence and complications reported among them. Our patient has a history of anxiety and depression, which can be considered precipitating factors predisposing the patient to a stress induced cardiomyopathy

A Rare Cause of Lymphadenopathy in a Young Hispanic Female: Unmasking Recurrent Nodal Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can present with painless bilateral symmetrical cervical lymphadenopathy, mimicking lymphomas. RDD is characterized by excessive tissue infiltration by dendritic cells, macrophages, or monocyte-derived cells, with a histopathologic diagnosis based on the presence of CD68+, CD163+, and S100+ histiocytes, which differentiate it from other histiocytic neoplasms. In this case report, we present a young Hispanic female with recurrent subcutaneous growths and lymphadenopathy, initially thought to be lymphoma, who was diagnosed with RDD after a significant diagnostic workup. Treatment initially consisted of surgical excision; however, due to recurrence, the patient was successfully treated with corticosteroids and a steroid-sparing agent, 6-mercaptopurine, with significant improvement in symptoms. RDD should be considered a differential diagnosis for patients with cervical lymphadenopathy, and an interdisciplinary approach is essential to managing this rare disorder effectively. The report highlights the need for an interdisciplinary approach to managing this rare disorder effectively and underscores the importance of multimodal treatment in disease suppression. As a rare disease with slow advancement of defined guidelines for diagnostic and treatment strategies, this case report adds to the existing literature on RDD

Tale of traveling in a stealth mode

Background: Melanoma is an aggressive malignancy that tends to form metastases in the brain. The nature of metastatic melanoma with unknown primary is poorly understood and has only theoretical assumptions. Less than 10% of melanoma cases present with an unknown primary tumor location. Case presentation: A 68-year-old Caucasian male patient, Canadian citizen, presented to the hospital with left-sided weakness and deviation of right side of angle of mouth for three days. Pertinent positive physical signs were motor weakness of left upper and lower extremities. Labs were unremarkable. CT-scan of the brain showed right temporal lobe lobulated masses associated with vasogenic edema, mass effect, and leftward midline shift, confirmed with MRI, which showed a large 5.4 x 4.2 cm complex right temporal lobe mass. Intravenous dexamethasone and intravenous levetiracetam were started. CT-scan of the chest, abdomen, and pelvis was negative for any suspicious lesion. A positron emission tomography scan showed no suspicious hypermetabolic activity. The patient underwent stereotactic right temporal craniotomy with volumetric resection of the tumor and magnetic resonance image-guided, computer-assisted stereotactic volumetric resection of the right temporal intra-axial lesion and duraplasty by the neurosurgical team, without any complications. Intraoperatively the lesion was noted to be violaceous, whitish, granular, and encapsulated. Later, he also underwent revision of craniotomy and gross total tumor resection under neuronavigation. Histopathology described the tissue from resection as a malignant melanoma, with tumor cells staining positive for vimentin, SOX-10, HMB-45 and MART-1. Despite an extensive evaluation by oncology and dermatology, no primary cutaneous lesion could be found. The patient received palliative radiation therapy with a total dose of 3000 cGy delivered over ten fractions. During his therapy, no significant side effects were observed. He was started on intravenous nivolumab. As the patient was a Canadian citizen, he moved back to Canada for further treatment. Conclusion: Our patient represents an example of malignant melanoma with an unknown primary. The current literature suggests that about 2–3% of all malignant melanoma patients present with a metastasis without a detectable primary tumor. Spontaneous regression of the primary lesion remains a plausible explanation of malignant melanoma with unknown primary

It Is Not Pneumocystis jiroveci (PCP), It Is Cyclophosphamide-Induced Pneumonitis

Cyclophosphamide (CYC) is an immunosuppressive medication used to treat life-threatening complications of various rheumatic diseases like vasculitis and systemic lupus erythematosus. A rare side effect of this medication is pneumonitis, which occurs in less than 1% of patients. We describe a case of an 83-year-old woman with a past medical history of microscopic polyangiitis, who presented with progressive dyspnea at rest, exacerbated on exertion, and associated with orthopnea that was attributed to CYC-induced pneumonitis. Three months before this presentation, the patient was diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-positive pauci-immune crescentic and necrotizing glomerulonephritis and started on CYC. On admission, a computed tomography (CT) chest showed worsening bilateral ground-glass opacities in a mosaic distribution and inter and intralobular septal thickening, not present on the CT performed three months prior. The patient underwent an extensive workup, which included an echocardiogram, bronchoscopy with bronchoalveolar lavage, and viral respiratory panel to rule out infectious and cardiac pathologies. She was started on empiric treatment with antibiotics and diuretics, however, despite these interventions, she continued with respiratory distress. A multidisciplinary team convened, and the diagnosis of CYC-induced lung injury was entertained. The CYC was discontinued, and the patient was started on prednisone with significant improvement in symptoms. This case highlights the importance of recognizing CYC as a rare cause of interstitial pneumonitis. When considering CYC-induced lung toxicity, other etiologies, such as opportunistic infections, cardiac etiologies, and diffuse alveolar hemorrhage, should be ruled out

Deeper Seated Than Skin Deep: Report of a Rare Case of Follicular Occlusion Tetrad and a Literature Review

Follicular occlusion tetrad (FOT) is a clinical syndrome consisting of hidradenitis suppurativa (HS), acne conglobata (AC), dissecting cellulitis of the scalp (DCS), and pilonidal cyst (PC). These entities typically occur independently, but occasionally present simultaneously comprising FOT. The four components share similar pathophysiology affecting the apocrine glands, follicular hyperkeratinization being the hallmark of each entity.Understanding shared similarities of each disease is paramount for the treatment approach as the relapsing and chronic nature of this syndrome can be burdening to patients. We present the case of a 22- year-old obese Hispanic man with a history of tobacco use who presented with worsening skin lesions. The patient developed extensive facial cystic acne 5 years before presentation, followed by left axillary hidradenitis suppurativa lesions two years before the presentation and right axillary involvement one year after. Skin manifestations then expanded to include the lower back, gluteal and perineal areas. The patient was diagnosed with FOT and despite conservative medical management, his lesions failed to improve. He ultimately underwent multiple staged excisional debridement surgeries and skin grafts. Our case underlines the presence of a syndromic association of cutaneous lesions that share a common pathogenesis and emphasizes that this entity requires a multidisciplinary approach. New biologic therapies continue to emerge and may potentially prevent the need for surgical intervention and the burden associated with it

Improvement of diabetic retinopathy screening of diabetic patients seen at the Internal Medicine Clinic​​

Diabetic retinopathy (DR) is a leading cause of vision loss globally. The International Diabetes Federation (IDF) estimated the global population with diabetes to be 463 million in 2019 and 700 million in 2045. Retinopathy is a highly specific neurovascular complication of both type 1 and types 2 diabetes, and the prevalence strongly correlates to both the duration of diabetes and the level of glycemic control. Regular follow-up with early detection and treatment of vision-threatening retinopathy enables the prevention of up to 98% of visual loss due to diabetic retinopathy