A Case of Atypical Scleroderma Renal Crisis.

We present the case of a 63-year-old female diagnosed with atypical SSc in the setting of acute SRC. She was undergoing work-up for progressive dyspnoea in the outpatient setting when she was found to have newly diagnosed restrictive lung pathology and worsening renal function, thus prompting acute hospital admission. Given multisystem involvement of the pulmonary and renal systems, the differential diagnosis included autoimmune and connective tissue disorders. Although serologies were non-specific, renal biopsy confirmed scleroderma renal disease, and she was started on treatment with captopril. This case highlights the importance of clinical judgment and timely diagnosis, even when laboratory data might indicate otherwise. LEARNING POINTS: Scleroderma renal crisis (SRC) remains an important cause of morbidity and mortality in systemic sclerosis (SSc), and clinicians should have a high index of suspicion to diagnose it.The absence of specific serologic markers makes SSc diagnosis challenging and necessitates reliance on clinical findings and additional diagnostic tools such as imaging studies and tissue sampling.

Unveiling the Chameleon: A Case Report on Acute Intermittent Porphyria.

Acute intermittent porphyria (AIP) is a rare autosomal dominant metabolic disorder with low penetrance, often presenting with a broad spectrum of clinical manifestations. Acute neurovisceral attacks commonly occur in young women, mimicking signs and symptoms of other medical and psychiatric conditions, thus delaying the diagnosis. We present the case of an 18-year-old female college student with recurrent hospitalizations for intractable abdominal pain, now again with pain and new subjective hematuria. The patient had previously undergone an endoscopy/colonoscopy with negative biopsies and serologies for acute pathology, including celiac disease. Celiac studies were repeated, given the possibility of inadvertent gluten exposure before the onset of the latest symptoms, but were negative. Basic labs and repeat imaging, including contrast-enhanced CT, MRI, and magnetic resonance (MR) enterography of the abdomen, continued to be unremarkable, and the patient's symptoms were felt to be functional in etiology. The patient's urinalysis was normal, and pregnancy was also ruled out. The patient continued to have pain despite receiving opiate analgesics, thus prompting a psychiatry consultation. She was diagnosed with acute adjustment disorder with anxiety and was started on hydroxyzine. Due to persistent symptoms, serum and urine samples were sent, revealing low levels of porphobilinogen deaminase (PBGD) and hydroxymethylbilane synthase (HMBS) gene mutation, confirming the diagnosis of AIP. She was treated with oral glucose and outpatient IV hemin infusions with the resolution of symptoms. AIP presents a nonspecific and highly variable clinical picture, often making it a challenging diagnosis due to such a broad differential. While our patient was thought to have acute adjustment disorder due to an unremarkable initial workup, further testing revealed otherwise. This case demonstrates how clinicians must have a high suspicion of AIP when caring for young females, manifesting with neurovisceral and psychiatric signs and symptoms. Timely diagnosis improves a patient's quality of life and can decrease overutilization of healthcare resources.

A Case of Omeprazole-Associated Acute Interstitial Nephritis.

Acute interstitial nephritis (AIN) is characterized by an inflammatory infiltrate of the interstitium of the kidney, typically causing a decline in kidney function. Drug-induced AIN (also called allergic AIN) is a type of AIN. Common drugs associated with AIN are antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), and proton pump inhibitors (PPIs). A 59-year-old male with a history of recent laparoscopic robotic sleeve gastrectomy presented to the emergency department with five weeks of progressively worsening fatigue, nausea, and lightheadedness. Postoperatively, he was prescribed omeprazole 20 mg daily for gastric ulcer prophylaxis. His other home medications were amlodipine, atorvastatin, ursodiol, and budesonide-formoterol fumarate nebulizer. His physical examination was normal. Laboratory studies revealed elevated creatinine of 4.19 mg/dL from a baseline of 0.9 mg/dL two months ago and the presence of urine eosinophils. The etiology of this elevated creatinine was unclear, prompting CT-guided left renal biopsy. The biopsy showed diffuse interstitial inflammatory infiltration with numerous lymphocytes, a large number of neutrophils, and scattered eosinophils, consistent with the allergic type of AIN. Omeprazole was discontinued and the patient received a seven-day course of prednisone. Despite treatment, permanent renal damage occurred, and the patient's new baseline creatinine was 2.3 mg/dL. AIN caused by PPIs should be considered in the differential diagnosis of acute kidney injury (AKI). AIN can be difficult to diagnose, presenting with nonspecific symptoms, such as oliguria, malaise, nausea, and vomiting. An accurate and timely diagnosis can help prevent and treat worsening renal failure.

Helicobacter pylori Infection and Complications of Cirrhosis.

INTRODUCTION: Helicobacter pylori is a significant contributor to conditions such as peptic ulcer disease, gastric cancer, gastric mucosa-associated lymphoid tissue lymphoma, and colorectal cancer. Recent studies have suggested a potential link between H. pylori and cirrhosis. However, the impact of H. pylori on cirrhosis-related mortality, inpatient outcomes, and decompensating events remains unclear. Considering the widespread availability of H. pylori testing and effective treatment options, there is a potential rationale for eradicating H. pylori in cirrhotic patients to mitigate the morbidity and mortality associated with cirrhosis. This study aims to investigate the association between H. pylori and inpatient outcomes and complications related to cirrhosis. METHODS: The National Inpatient Sample (NIS) database, a part of the Healthcare Cost & Utilization Project, was utilized for this study. Inpatient data from the years 2016 through 2019 were extracted for patients with a primary discharge diagnosis of cirrhosis and a concurrent diagnosis of H. pylori infection. The primary outcomes included inpatient mortality, length of stay, and cost of care. Secondary outcomes involved cirrhosis-related complications during hospitalization, such as gastrointestinal bleeding, hepatic encephalopathy, and hepatorenal syndrome. RESULTS: Over the years 2016 to 2019, 416,410 patients received a primary discharge diagnosis of cirrhosis. Among them, 990 patients (0.2%) had a secondary diagnosis of H. pylori infection. Those with both cirrhosis and H. pylori tended to be younger on average (mean age 54.25 vs. 57.18 years, p=0.01) and more frequently fell within the age range of 18-49 (33.84% vs. 24.71%, P=0.01). H. pylori-infected patients were also more likely to be male (70.71% vs. 63.11%, P<0.028), of Hispanic race (36.4% vs. 18.6%, p< 0.1), and of Black race (20.2% vs. 8.1%, p< 0.1). While H. pylori-exposed patients had lower in-hospital mortality (0.51% vs. 4.44%, p=0.007), their mean length of stay was higher (6.97 days vs. 5.75, p=0.002). The overall cost of care was comparable between the H. pylori-exposed and non-exposed groups (mean USD18,106.18 vs. $16,543.49, P=0.160). H. pylori-exposed patients had a higher overall rate of cirrhosis-related complications (84.85% vs. 67.59%, p< 0.001), gastrointestinal bleeding (48.48% vs. 27.34%, p< 0.001), and hepatorenal syndrome (70.71% vs. 46.99%, p< 0.001), and these differences persisted in multivariable analysis. Initially, rates of hepatic encephalopathy were higher in H. pylori non-exposed patients (21.57% vs. 15.66%, p=0.04), but this discrepancy was corrected after adjusting for potential confounders. CONCLUSION: While patients in this study were diagnosed with both H. pylori and cirrhosis by discharge, it cannot be definitively concluded that H. pylori was the direct cause of cirrhosis complications. Recognizing this uncertainty, further studies are needed better to understand the associations between cirrhosis and H. pylori complications. Distinguishing the causes of cirrhosis and its relationship with H. pylori may offer deeper insights into whether H. pylori is a causative factor or merely correlated in its effects on patients with cirrhosis.

Symptomatic Meckel's Diverticulum Presenting As Recurrent Gastrointestinal Bleeding in an Adult.

A 51-year-old male presented to the hospital with recurrent gastrointestinal bleeding. Prior work up with an esophagogastroduodenoscopy (EGD), colonoscopy, and video capsule endoscopy failed to reveal a bleeding source. Given a history of a terminal ileum diverticulum noted on previous colonoscopy and persistence of hematochezia, a Meckel's scan was performed, which revealed abnormal uptake suspicious for a Meckel's diverticulum containing ectopic gastric mucosa. After surgical resection, pathology confirmed a Meckel's diverticulum with gastric heterotopia. This case highlights the importance of considering Meckel's diverticulum for instances of recurrent gastrointestinal bleeding, especially in patients who are still symptomatic despite an extensive workup. Moreover, it is important to note that a Meckel's diverticulum can be missed on video capsule endoscopy.

Alcoholic Hepatitis Mimicking Iron Overload Disorders With Hyperferritinemia and Severely Elevated Transferrin Saturation: A Case Report.

Iron overload disorders can present as non-specific symptoms and develop gradually but, if untreated, can be very fatal. The common causes include multiple blood transfusions for chronic anemia and increased iron absorption, including hereditary hemochromatosis (HH). HH is one of the common causes of iron overload disorders and usually presents with liver cirrhosis in a setting of significantly elevated ferritin and elevated transferrin saturation. Alcoholic hepatitis is a clinical syndrome of progressive inflammatory liver injury associated with long-term heavy intake of ethanol. However, in patients with alcohol abuse, excessive alcohol consumption can disrupt iron metabolism releasing large amounts of iron into circulation. This can cause severely elevated ferritin due to disruption of iron metabolism, simulating iron overload disorders such as HH, especially if the patient also has liver cirrhosis. Even though a high transferrin saturation of greater than 45% is recommended as a cutoff transferrin value as high sensitivity for detecting iron overload disorders, it has a low specificity and positive predictive value and often identifies people with other causes of acutely elevated ferritin levels such as alcohol liver disease and hepatitis. Recognizing this feature and timely management can spare the patient from unnecessary phlebotomies and prompt treatment for alcoholic hepatitis. We present an interesting case of severe alcoholic hepatitis mimicking HH with severely elevated ferritin levels and transferrin saturation with underlying liver cirrhosis.

Immune Checkpoint Inhibitor-Induced Lymphocytic Esophagitis.

Immune checkpoint inhibitors (ICIs) have emerged as effective treatments for a wide variety of advanced malignancies. However, their use is associated with numerous immune-related toxicities, including within the gastrointestinal tract. We present a rare case of checkpoint inhibitor-induced lymphocytic esophagitis. A 79-year-old male with a past medical history significant for metastatic renal clear cell carcinoma on nivolumab presented to the hospital with dysphagia and symptomatic choledocholithiasis. The patient underwent endoscopic retrograde cholangiopancreatography (ERCP) for the extraction of stones and esophagogastroduodenoscopy (EGD) for dysphagia, which showed esophagitis. Biopsies revealed lymphocytic infiltration of the epithelium, dyskeratotic keratinocytes, and acanthosis, raising suspicion for nivolumab-associated lymphocytic esophagitis. Treatment includes proton pump inhibitors and steroids; however, efficacy is not well described due to the rarity of the condition.