Idiopathic pulmonary hemosiderosis (IPH) is a rare condition of unknown etiology characterized by hemoptysis, diffuse alveolar infiltrates and iron-deficiency anemia. Lane-Hamilton syndrome (LHS), a condition in which IPH is associated with celiac disease is an extremely rare condition. CASE PRESENTATION: 49-year-old male with no known past medical history presented with progressively worsening shortness of breath for 3 days. No associated fever, chest pain, cough or hemoptysis were noted. On presentation, temperature: 99F, RR: 34/min, O2 saturation of 85% on room air, HR: 130/min and BP: 100/60mmHg. Physical examination revealed diffuse crackles on chest auscultation. Blood work was significant for leukocytosis at 23.61, Hb of 9, MCV 76 and iron deficiency anemia. Liver function test showed mild transaminitis. Chest xray and CT chest showed diffuse ground glass opacities. He was started on broad spectrum antibiotics. Patient's respiratory status worsened requiring mechanical ventilation. Bronchoscopy and lavage showed diffuse alveolar hemorrhage, elevated white count and hemosiderin laden macrophages. Cultures and cytology were negative. A preliminary diagnosis of pulmonary hemosiderosis was made. Patient was started on pulse dose steroids. Rheumatologic workup was negative. On further review of chart, patient had frequent outpatient visits with bloating and diarrhea. Due to association of celiac disease and alveolar hemorrhage, further work up was sent. Anti-tissue glutaminase and Anti-Gliadin antibody were positive with gliadin IgA titers of 68.5 and IgG titers of 34.2. The probable diagnosis of LHS was made. Patient responded to steroids with clearing of infiltrates and decreasing oxygen requirement. DISCUSSION: LHS is an extremely rare combination of IPH and celiac disease. Both diseases are immunologically mediated and respond well to immunosuppression. Several hypotheses have been postulated to explain this association including deposition of circulating immune complexes from food allergens on the basement membrane of alveolar capillaries, the cross-reaction between the antireticulin antibodies and the alveolar basement membrane antigens. Lane-Hamilton syndrome was first seen in 1971. The association is mostly seen in children below 15 years but is very rare among adults. CONCLUSIONS: LHS should remain one of the differentials in patients presenting with diffuse alveolar hemorrhage. Concurrent screening for celiac disease should therefore be undertaken as disease can be successfully treated with a gluten-free diet once the diagnosis has been made.
Thoracic aortic aneurysm (TAA) is an uncommon clinical condition, commonly associated with atherosclerosis. It is mostly asymptomatic but aneurysm exceeding 6 cm are at risk of yearly rate of rupture or dissection of at least 6.9% and a death rate of 11.8%. We present to you a case of TAA with rupture presenting with cough, hemoptysis and pneumonitis. CASE PRESENTATION:Patient is a 68 years male who presented to the ED with cough, hemoptysis, chest discomfort for 2-3 days. He had intermittent episodes of fever. COVID test was negative 3 days ago. Past medical history was significant for HTN, Asthma, COPD, Diabetes mellitus, tobacco use disorder. Patient was afebrile with BP of 137/73, HR 72, RR 16, O2 saturation of 89% in room air. Physical examination including chest was unremarkable. Initial workup including CBC was remarkable for WBC of 12500, Hgb of 10.5, INR 0.8, PTT 24, Troponin <0.02. CXR showed findings worrisome for underlying pneumonitis in the left upper lobe. Presumptive diagnosis of community-acquired pneumonia was made, patient was treated with ceftriaxone and azithromycin but symptoms continued to persist despite the treatment. For further evaluation, CT CTA chest was performed which revealed features suggestive of ruptured thoracic aortic aneurysm. Further, angiogram revealed rupture above the level of the celiac artery. Patient then underwent endovascular thoracic stent graft placement. Patient could not be extubated following the procedure due to advanced COPD and was then transferred to ICU for close monitoring. He was subsequently extubated in the next few days and was discharged in stable condition. No complications were noted in follow-up CT angiography.DISCUSSION: Thoracic aortic aneurysm (TAA) is commonly associated with atherosclerosis but can also occur in genetic disease (Marfan syndrome, Ehler Danlos syndrome, Loeys-Dietzs syndrome, familial thoracic aortic aneurysm syndrome, aneurysms osteoarthritis syndrome), patient with bicuspid aortic valve, infection (tuberculosis, syphilis, mycotic infections), giant cell arteritis, cystic medial necrosis, trauma, familial TAA. It is present in 5.6/100,000 people. Hemoptysis is a unique symptom of TAA rupture that has been a reported due to erosion of the trachea by aneurysm or rupture of the aneurysm into lung parenchyma. Rupture risk is high in population with aneurysm size > 5 cm, smokers, COPD, advanced age, hypertension. Thoracic endovascular stent graft surgery, under epidural anesthesia, is the preferred surgical treatment especially in old TAA patients as in our case because of low morbidity, mortality and hospital stay.CONCLUSIONS: Rupture of TAAs is still very rare, but should remain on the differential for a patient with cough, hemoptysis with features of pneumonitis as it is associated with very high morbidity and mortality rates.
INTRODUCTION: Lactic acidosis is the most common cause of metabolic acidosis in hospitalized patients. Alcoholic ketoacidosis is one of the several etiologies where serum lactate level rarely exceeds 3 mmol/L. We present to you an interesting case of severe lactic acidosis (24 mmol/L) in a patient with acute alcohol intoxication. CASE PRESENTATION:Patient is a 62-year male who presented with intractable abdominal pain for 2 days. Past medical history was significant for alcohol use disorder. On presentation, patient had tachycardia, tachypnea, was restless with slurred speech with mild abdominal tenderness. Laboratory findings were remarkable for leukocytosis of 22.7, serum bicarbonate of 4 mmol/L, serum glucose 104 mg/dL, serum lactate 24 mmol/L. ABG showed pH of < 7.00, pCO2 12 mmHg, HC03 4.6 mmol/L. Anion gap was 41.0. Serum ethanol level was elevated at 0.185 g/dl. Patient was started on bicarbonate infusion, IV thiamine with 5% dextrose fluid and CIWA protocol. Serum beta hydroxybutyrate was elevated at 2.9, serum osmolality was elevated at 344 with osmolar gap of 13. LFT was deranged with unremarkable liver ultrasound. Patient was admitted to the ICU. Serum isopropanol, methanol, and ethylene glycol were negative. Infectious workup including blood culture, urine culture, pneumonia workup were negative. Acetaminophen and salicylate levels were normal. Urine drug screen was unremarkable. No history of GI malabsorption (short-bowel syndrome), renal failure or recent phenobarbital administration. Lactic acidosis was therefore attributed to alcoholic ketoacidosis in a setting of alcoholic hepatitis. Patient gradually improved with final pH 7.38, lactate 1.80 and serum HC03 24.6 by day 2.DISCUSSION: Lactate level of > 4 mmol/L with arterial pH < 7.35 suggests lactic acidosis. Lactic acidosis is broadly classified into type A which occurs due to impaired systemic tissue oxygenation and type B due to toxin induced cellular metabolism impairment and regional ischemia, commonly in alcoholism, HIV, antiretroviral medications, beta adrenergic medications, D lactic acidosis (short-bowel syndrome), diabetes mellitus, metformin, malignancy. Alcoholic ketoacidosis remains important etiology as if missed may result in fatal consequences including sudden death. Occasionally, alcoholic ketoacidosis can be misdiagnosed as diabetic ketoacidosis with subsequent mismanagement resulting in increased morbidity and mortality.CONCLUSIONS: Alcoholic ketoacidosis typically presents with metabolic acidosis but rarely with lactate > 3mmol/L in absence of other contributing factors. Oftentimes, the diagnosis is missed or misdiagnosed as diabetic ketoacidosis resulting in mismanagement contributing to increased morbidity and mortality.
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