Acute interstitial nephritis associated with ingesting a Momordica charantia extract: A case report

Rationale:Momordica charantia is often used to treat type 2 diabetes mellitus in Korea. Drug-induced acute interstitial nephritis (AIN) accounts for 60% to 70% of AIN cases. However, only 1 case of AIN associated with ingesting M charantia has been reported in the English literature. We report an extremely rare case of AIN that occurred after a patient ingested a pure M charantia extract over 7 months.Patient concerns:A 60-year-old Korean woman was admitted to our hospital for a renal biopsy. Her renal function had decreased gradually over the last 9 months without symptoms or signs.Diagnosis:Her blood urea nitrogen and serum creatinine levels were 29.7 mg/dL (range: 8.0–20.0 mg/dL) and 1.45 mg/dL (range: 0.51–0.95 mg/dL) on admission. Renal histology indicated AIN; there was immune cell infiltration into the interstitium, tubulitis, and epithelial casts, although the glomeruli were largely intact.Interventions:M charantia was discontinued and prednisolone was prescribed.Outcomes:The value of serum creatinine has almost been restored to the baseline level after 3 months.Conclusions: This is the first case report of AIN associated with the ingestion of a pure M charantia extract. Recognition of the possible adverse effects of these agents by physicians is very important for early diagnosis and appropriate management.     

Analysis of risk factors for pulmonary tuberculosis with persistent severe inflammation: An observational study

Introduction:Patients with pulmonary tuberculosis (TB) sometimes show persistent severe inflammation for more than 1 month, even if TB treatment is effective. Although this inflammation can be improved through continuous antituberculous therapy, the risk factors for persistent inflammation remain unclear. Therefore, we sought to study the characteristics of patients with persistent severe inflammation.Materials and methods:We retrospectively analyzed 147 hospitalized adult patients with C-reactive protein (CRP) levels of 5 mg/dL or more on admission to Fukujuji Hospital from April 2019 to March 2021. The patients were divided into 2 groups: 40 patients (27.2%) had CRP levels of 5 mg/dL or more at 4 weeks after admission (persistent inflammation group), and 107 patients (72.8%) had CRP levels that fell below 5 mg/dL within 4 weeks of admission (improved inflammation group).Results:The median CRP level on admission in the persistent inflammation group was 10.8 mg/dL (interquartile range 9.1–14.5), which was higher than that in the improved inflammation group (median 8.2 mg/dL [6.5–12.1], P = .002). Patients in the persistent inflammation group had a higher prevalence of large cavities, defined as cavities ≥4 cm in diameter, on chest computed tomography (CT) (n = 20 [50.0%] vs n = 12 [11.2%], P < .001).Discussion and conclusions:This study showed that 27.2% of patients who had high or moderate inflammation on admission did not achieve low CRP levels within 4 weeks after admission. Risk factors for persistent severe inflammation in patients with TB were presence of a large cavity (cavity diameter ≥4 cm) on chest CT and a high CRP level on admission. Therefore, in a patient with a large cavity on chest CT and/or CRP ≥9.0 mg/dL on admission, long-term inflammation may occur despite antituberculous therapy if other diseases are ruled out.     

A case report of PR-3-ANCA-positive glomerulonephritis with histological features of GPA associated with infectious endocarditis

Rationale:Several renal diseases are associated with infectious endocarditis. However, there are few reports on patients with granulomatosis with polyangiitis (GPA) associated with infectious endocarditis, and there is no consensus for appropriate treatment.Patients concerns:A 35 -years-old man with congenital ventricular septal defect presented severe anemia, hematuria and proteinuria. The blood and urine examinations showed elevated white blood cells (12,900 cells/μL), C-reactive protein level (13.1 mg/dL) and proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level (11.0 IU/mL), severe anemia (hemoglobin: 6.1 g/dL) and renal dysfunction [estimated glomerular filtration rate (eGFR): 12.7 ml/min.1.78 m² with hematuria and proteinuria].Diagnoses:The patient was diagnosed with crescentic glomerulonephritis with histological features of GPA associated with infectious endocarditis by renal biopsy and transthoracic echocardiography.Interventions:Antibacterial drugs (ampicillin-sulbactam) were administrated. No immunomodulating agents were used because immunosuppressive drugs may worsen infectious endocarditis. Subsequently, renal function and urinary findings improved. However, infectious endocarditis was not improved. Therefore, valve replacements and ventricular septal closure surgery were conducted.Outcomes:Thereafter, his postoperative course was uneventful, renal function improved (eGFR: 64.3 ml/min.1.78 m²), and PR3-ANCA level normalized.Lessons:We reported a case report of PR3-ANCA positive glomerulonephritis with histological features of GPA associated with infectious endocarditis. Physicians might note this renal complication when they manage infectious endocarditis.     

Acute kidney injury due to direct infiltration by lymphoplasmacytic lymphoma secreting IgG paraproteins: A case report

Introduction:Waldenström''s macroglobulinemia is a lymphoplasmacytic lymphoma (LPL) associated with a monoclonal immunoglobulin M protein. Although acute kidney injury (AKI) due to immunoglobulin M paraprotein infiltration into the renal interstitium has been reported, there has been no report of AKI with invasion of the immunoglobulin G paraprotein into the renal interstitium in a patient with LPL.Patient concerns:A 65-year-old male was admitted to our hospital with fatigue and decreased renal function. He complained of a 3-kg weight loss in the last 3 months.Diagnosis:The initial blood urea nitrogen and serum creatinine levels were 55.9 and 1.83 mg/dL, respectively. Serum protein electrophoresis revealed a monoclonal component (3.5 g/dL) in the gamma region and immunofixation electrophoresis showed an immunoglobulin G kappa monoclonal protein. Renal pathology revealed that CD3–CD20+ CD138+ lymphoid cells had infiltrated the renal interstitium. A bone marrow biopsy was compatible with LPL.Interventions:Intravenous methylprednisolone (1 mg/kg) was administered after confirming the renal pathological findings.Outcomes:Serum creatinine decreased to 0.8 mg/dL 14 days after treatmentConclusions:Physicians should recognize LPL secreting various immunoglobulins as a possible cause of AKI when renal failure of unknown etiology and serum immunoglobulin paraprotein is present. A kidney biopsy should be performed for definitive diagnosis and appropriate management.     

Management of corticosteroid-dependent eosinophilic interstitial nephritis: A case report

Introduction:Drug-induced acute interstitial nephritis (DI-AIN) is an important cause of acute kidney injury. In renal biopsy specimens, tubulitis with eosinophilic infiltration is suggestive of DI-AIN. Although corticosteroid therapy and discontinuation of the offending drug can improve renal dysfunction in most cases of DI-AIN, some patients experience AIN recurrence, leading to corticosteroid dependency. Corticosteroid-dependent eosinophilic interstitial nephritis presents a difficult dilemma in diagnosis and information regarding optimum management is limited.Patient concerns:A 25-year-old man, who received treatment with carbamazepine, zonisamide, valproate, and lacosamide for temporal lobe epilepsy, showed an increase in serum creatinine level from 0.98 to 1.29 mg/dL over a period of 6 months. Although he exhibited no symptoms, his serum creatinine level continued to increase to 1.74 mg/dL.Diagnosis:Renal biopsy revealed tubulitis and interstitial inflammatory infiltrates with eosinophils. Immunological and ophthalmological examinations showed no abnormal findings, and thus, his renal dysfunction was presumed to be caused by DI-AIN. Although oral prednisolone (PSL) administration (40 mg/d) and discontinuation of zonisamide immediately improved his renal function, AIN recurred 10 months later. The increase in PSL dose along with discontinuation of valproate and lacosamide improved renal function. However, 10 months later, recurrent AIN with eosinophilic infiltration was confirmed by further biopsy. The patient was therefore diagnosed with corticosteroid-dependent eosinophilic interstitial nephritis.Interventions:To prevent life-threatening epilepsy, carbamazepine could not be discontinued; hence, he was treated with an increased dose of PSL (60 mg/d) and 1500 mg/d of mycophenolate mofetil (MMF).Outcomes:MMF was well tolerated and PSL was successfully tapered to 5 mg/d; renal function stabilized over a 20-month period.Lessons:The presence of underdetermined autoimmune processes and difficulties in discontinuing the putative offending drug discontinuation are contributing factors to corticosteroid dependency in patients with eosinophilic interstitial nephritis. MMF may be beneficial in the management of corticosteroid-dependent eosinophilic interstitial nephritis by reducing the adverse effects related to high-dose and long-term corticosteroid use.     

Successful treatment with tocilizumab for refractory anemia and slowly progressive renal glomerulosclerosis in multicentric Castleman disease: A case report

Rationale:Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder accompanied by systemic symptoms characterized by polyclonal hypergammaglobulinemia and chronic inflammation due to overexpression of interleukin-6. Histological heterogeneity of renal involvement in MCD has been described, although the number of reports is limited. Tocilizumab, a humanized anti-interleukin-6 receptor antibody, has been reported to be effective for MCD.Patent concerns:A 64-year-old man experienced refractory anemia and slowly progressive renal dysfunction with proteinuria, accompanied by persistent inflammation for 11 years.Diagnosis:Two renal biopsies were obtained. The first biopsy performed 7 years before admission revealed non-specific interstitial inflammation, whereas the second biopsy demonstrated global sclerosis in most glomeruli and interstitial fibrosis. The patient had multiple lymphadenopathies. Cervical lymph node biopsy histological findings were compatible with plasma cell type Castleman disease. The patient had no evidence of human hepatitis virus-8 infection.Intervention:The patient was treated with 60 mg/d prednisolone followed by 8 mg/kg intravenous tocilizumab every 2 weeks.Outcome:His anemia significantly improved, as well as a marked reduction in proteinuria and stabilization of renal function. He did not experience renal function during the 2-years follow-up period.Lessons:The heterogeneity of the renal manifestations of MCD sometimes makes early diagnosis difficult. We need to interpret the histological findings of the renal biopsy carefully. For advanced-stage renal diseases, tocilizumab might be an effective treatment strategy for MCD.     

Vitamin D deficiency osteomalacia triggered by long-term social withdrawal and unbalanced diet in a Japanese middle-aged subject: A case report

Introduction:Osteomalacia is caused by an increase in the number of osteoids owing to mineralization failure. There are various causes of osteomalacia, such as hypophosphatemia due to excess production of fibroblast growth factor 23, vitamin D deficiency, insufficient vitamin D action, and renal tubular disorders.Patient concerns:A 53-year-old man with bone pain and gait disturbance was referred to our institution. At the age of 35, he developed atopic dermatitis. He had eyesight deterioration due to atopic cataracts when he was 37 years old. Subsequently, he stayed home all the time, and his eating habits were unbalanced for a long period of time. Although he had atopic dermatitis, he did not take allergen-free diets, and he did not use sunscreen. Furthermore, when he was 43 years old, he failed to flex his legs and suffered gait disturbance.Diagnosis:Hypocalcemia and hypophosphatemia were observed as follow: calcium, 5.5 mg/dL; adjusted calcium, 6.9 mg/dL; inorganic phosphorous, 1.9 mg/dL. In addition, intact parathyroid hormone levels were as high as 277.4 pg/mL, and 1, 25-(OH)₂ vitamin D and 25-(OH) vitamin D levels were markedly reduced: 1, 25-(OH)₂ vitamin D, ≤4 pg/mL; 25-(OH) vitamin D, 11.0 ng/mL. Fibroblast growth factor 23 levels did not increase. Alkaline phosphatase (ALP) and bone-type ALP (BAP) levels were high: ALP, 784 U/L; BAP, 159.2 μg/L (reference range: 3.7–20.9 μg/L). Based on these findings, we diagnosed this patient with vitamin D-deficient osteomalacia triggered by long-term social withdrawal and an unbalanced diet.Interventions and outcomes:After hospitalization, to treat vitamin D-deficient osteomalacia, we started to administer 1 μg/day of alfacalcidol and 3 g/day of calcium lactate. Approximately one month later, 1,25-(OH)₂ vitamin D levels increased to 214 pg/mL. Consequently, calcium and inorganic phosphorus were also increased up to 7.8 mg/dL and 3.9 mg/dL, respectively, and intact parathyroid hormone was decreased to 132.0 pg/mL.Conclusions:We should bear in mind the possibility of osteomalacia triggered by social withdrawal and vitamin D deficiency even in middle-aged subjects.     

Immunoglobulin A nephropathy in a patient with neurofibromatosis type 1: A case report and literature review

Rationale:Neurofibromatosis type 1 (NF-1) is an autosomal-dominant neurocutaneous disorder that affects the skin, bones, and nervous system. The most common manifestation of kidney involvement is renal artery stenosis; glomerulonephritis is extremely rare. In this case report, we present a patient with NF-1 and immunoglobulin A nephropathy (IgAN).Patient concerns:A 51-year-old Korean man previously diagnosed with NF-1 presented with persistent proteinuria and hematuria identified during a routine medical check-up. He had no history of hypertension or diabetes, and denied a history of alcohol use or smoking.Diagnosis:The contrast-enhanced computed tomography scan revealed normal-sized kidneys and no evidence of renal artery stenosis. On the day of the kidney biopsy, laboratory tests showed a serum creatinine level of 1.1 mg/dL, urine protein/creatinine ratio of 1.3 g/g, and urine red blood cell count of >10 to 15/HPF. The kidney biopsy sample revealed IgAN grade III, according to Lee glomerular grading system.Intervention:The patient was advised to take 4 mg of perindopril.Outcome:Three months after the treatment, the urine protein/creatinine ratio decreased to 0.6 g/g, with no change in the serum creatinine level (1.03 mg/dL).Lessons:A genetic link between NF-1 and IgAN or other glomerular diseases is not established. However, activation of the mTOR pathway may explain this association.     

Dabrafenib- and trametinib-associated glomerular toxicity: A case report

Rationale:Combined treatment with dabrafenib, a B-RAF inhibitor, and trametinib, a mitogen-activated protein kinase inhibitor, is an effective option for patients with metastatic melanoma. A few cases of acute kidney injury associated with tubulointerstitial nephritis and 1 case of nephrotic syndrome have been reported in patients on this drug combination; however, progressive renal injury has not been reported. In this case study, we report a patient with metastatic melanoma who developed glomerular capillary endothelial toxicity and progressive glomerular sclerosis during combination therapy.Patient concern:Our patient was an 80-year-old woman with a history of type 2 diabetes and chronic kidney disease.Diagnosis and intervention:She was diagnosed with metastatic melanoma and commenced combination therapy with dabrafenib and trametinib.Outcomes:Her renal function progressively deteriorated; by month 20 after treatment commencement, her serum creatinine level had increased from 1.59 to 3.74 mg/dL. The first kidney biopsy revealed marked glomerular and endothelial cell damage. Her medication was stopped, but no improvement was evident. At 5 months after the first biopsy, her serum creatinine level had increased to 5.46 mg/dL; a second kidney biopsy revealed focal segmental glomerular sclerosis and marked tubulointerstitial fibrosis. She was started on hemodialysis.Lessons:We describe a patient with a metastatic melanoma who developed progressive kidney failure during treatment with dabrafenib and trametinib. The most prominent microscopy findings were glomerular endothelial damage in the initial kidney biopsy and accelerated glomerular sclerosis and tubulointerstitial fibrosis in the follow-up biopsy. We hypothesize that a decreased renal reserve and impairment of kidney repair capacity caused by inhibition of B-RAF, a downstream mediator of vascular endothelial growth factor, may explain the progressive kidney injury.     

Pazopanib-induced severe acute liver injury: A case report

Rationale:Drug-induced liver injury (DILI) is the most common cause of acute liver failure in the United States. Painkillers and fever antipyretics are the most common cause of DILI. Hepatic injury can be provoked by DILI as hepatocellular or cholestatic type.Patient concerns:A 48-year-old woman presented jaundice accompanied by nausea and vomiting. The patient was an inactive hepatitis B carrier with low viral titer and was diagnosed renal cell carcinoma (RCC) with hepatic metastasis requiring pazopanib treatment. Prior to administration of pazopanib, tenofovir administration was started to prevent exacerbation of hepatitis B. The patient was referred to clinic of gastroenterology department due to sudden elevation of bilirubin after 5 weeks of pazopanib treatment.Diagnoses:Abdominal ultrasound and computed tomography showed non-specific finding other than metastatic nodule in the liver and liver cirrhosis. After then, the patient was performed liver biopsy, and the biopsy result was acute cholestatic hepatitis with centrilobular area necrosis and portal inflammation. Therefore, considering the clinical history and biopsy results, the patient was diagnosed as DILI due to pazopanib.Interventions:After the biopsy, empirical steroid therapy was initiated and after 7 weeks of pazopanib discontinuation.Outcomes:The total bilirubin level returned to normal from peak level of 24.61 to 1.52 mg/dL.Lessons:In patients with renal cell carcinoma, pazopanib treatment requires clinical caution as it causes rare complications such as severe jaundice and acute cholestatic hepatitis.     

HTLV-1 associated acute adult T-cell lymphoma/leukemia presenting as acute liver failure in Micronesian: A case report

Rationale:Malignant infiltration accounts for 0.5% of acute liver failure cases, with non-Hodgkin''s lymphoma the predominant cause. Adult T-cell lymphoma/leukemia (ATLL) is a rarer source of acute hepatitis, with only 3 cases reported and all resulting in immediate deterioration with death. ATLL rises from human T-lymphocytic virus-1 (HTLV-1), commonly found in Japan (southern and northern islands), the Caribbean, Central and South America, intertropical Africa, Romania, and northern Iran. In Micronesia, HTLV-1 infection amongst native-born is absent or exceedingly rare.Patient Concerns:A 77-year-old Marshallese man presented to the emergency department with a 1-week history of generalized weakness, fatigue, and nausea. The physical exam revealed a cervical papulonodular exanthem and scleral icterus.Diagnosis:Laboratory studies were remarkable for aspartate-aminotransferase of 230 IU/L (reference range [RR]: 0–40), alanine-aminotransferase of 227 IU/L (RR: 0–41), alkaline phosphatase of 133 IU/L (RR: 35–129), and total bilirubin of 4.7 mg/dL (RR: 0–1.2), supporting acute liver injury. Platelet count was 11.6x10⁴/μL (RR: 15.1–42.4 × 10⁴), hemoglobin was 13.8 g/dL (RR: 13.7–17.5), and white blood cell count was 7570/μL (RR: 3800–10,800) with 81.8% neutrophils (RR: 34.0–72.0) and 10.4% lymphocytes (RR: 12.0–44.0). The peripheral blood smear demonstrated abnormal lymphocytes with occasional flower cell morphology. HTLV-1/2 antibody tested positive. The skin and liver biopsies confirmed atypical T-cell infiltrate. The diagnosis of ATLL was established.Interventions:The patient elected for palliative chemotherapy with cyclophosphamide, vincristine, and prednisone (CVP). He began antiviral treatment with zidovudine 250 mg bis in die (BID) indefinitely. Ursodiol and cholestyramine were added for his hyperbilirubinemia.Outcomes:Four weeks from admission, the patient returned to near baseline functional status and was discharged home.Lessons:This case highlights that ATLL can initially present as isolated acute hepatitis, and how careful examination of peripheral blood-smear may elucidate hepatitis etiology. We also present support for utilizing ursodiol with cholestyramine for treating a hyperbilirubinemia. Moreover, unlike prior reports of ATLL presenting as liver dysfunction, combined antiviral and CVP chemotherapy was effective in this case. Lastly, there are seldom demographic reports of HTLV-1 infection from the Micronesian area, and our case represents the first indexed case of HTLV-1-associated-ATLL presenting as acute liver failure in a Marshallese patient.     

Coexisting nutcracker phenomenon and superior mesenteric artery syndrome in a patient with IgA nephropathy: A case report

Rationale:Nutcracker and superior mesenteric artery (SMA) syndrome share the same pathogenesis, but the simultaneous occurrence of both diseases is quite rare. A combination of the nutcracker syndrome and IgA nephropathy has previously been reported. Herein, we report what we believe is the first case of coexisting nutcracker and SMA syndrome in a patient with IgA nephropathy.Patient concerns:A 15-year-old Chinese boy who was diagnosed with IgA nephropathy at 8 years of age presented with gross hematuria, fatigue, anorexia, nausea, and recurrent abdominal distension for 1 week without any obvious evidence of preceding infection. Laboratory data showed macroscopic hematuria, heavy proteinuria, and relatively normal renal function. Doppler ultrasonography and upper gastrointestinal gastrografin study were performed, respectively. Since his renal function deteriorated after admission, repeated renal biopsy was performed.Diagnoses:IgA nephropathy with nutcracker phenomenon and SMA syndrome.Intervention:Immunosuppressive therapy combined with conservative therapy for superior mesenteric artery syndrome.Outcomes:One month later, his abdomen symptoms such as anorexia and abdominal distension eased a lot with body weight increase of about 3 kg. After 6 months of follow-up, his body weight increased to 57 kg, serum creatinine decreased to 63 μmol/L, and urine microscopy showed 75.5 RBC/high-power field with 0.3 g urine protein per day.Lessons:Although the association between vascular compression and IgA nephropathy (IgAN) has not been elucidated yet, combination of nutcracker syndrome and SMA syndrome should be considered in patients with IgAN. The combination may increase the complexity of the disease, and renal biopsy should not be hesitated for differential diagnosis.     

The severity and clinical characteristics of COVID-19 among patients with type 2 diabetes mellitus in Jazan,Saudi Arabia

Background:The objectives of the current study were to assess the severity and clinical characteristics of coronavirus disease 2019 (COVID-19) among Saudi adults with type 2 diabetes mellitus (T2DM) in Jazan region, Saudi Arabia.Methods:This retrospective cohort study included 412 patients with COVID-19 selected randomly from the Health Electronic Surveillance Network system, which contains the primary data on COVID-19 infections in Jazan.Results:COVID-19 disease duration was significantly longer in patients with T2DM (mean = 10.7 days) compared with those without T2DM (mean = 8.3 days) (P = .01). Six (7%) patients experienced an increase in blood glucose concentrations and had to escalate their total daily insulin dose accordingly. Median fasting and random blood glucose levels increased after infection with COVID-19 (pre-COVID median = 119 and 172 mg/dL, respectively; post-COVID median = 148 and 216 mg/dL, respectively) (P = .02). The total insulin dose pre-COVID (median = 42 units/d) increased after infection with COVID-19 (median = 58 units/d) (P = .01). Most patients with T2DM had clinical COVID-19 symptoms (91%) and the remainder (9%) were asymptomatic. A large proportion (80%) of T2DM patients with mild COVID-19 symptoms self-isolated at home. COVID-19 patients with T2DM (11%) who had an oxygen saturation of ≤ 90% and admitted to the intensive care unit were higher than those without T2DM (5%) (P =  < .001). COVID-19 patients with T2DM (9%) had higher mortality rate than COVID-19 patients without T2DM (1%) (P =  < .001).Conclusion:COVID-19 patients with T2DM were associated with a higher risk of admission to the intensive care unit and mortality than COVID-19 patients without T2DM.     

Psychosis in a primary hyperparathyroidism patient with mild hypercalcemia: A case report

Introduction:Primary hyperparathyroidism (PHPT) is characterized by hypercalcemia and an elevated level of serum parathyroid hormone (PTH). PHPT presents with a complex set of renal, skeletal, and neuropsychological symptoms. Parathyroidectomy (PTX) is a radical treatment that is recommended for all physically symptomatic patients with PHPT. However, psychiatric symptoms are not considered as an indication for surgery. There remains an important issue from the view of perioperative management of whether PTX should be performed with the presence of uncontrolled psychiatric symptoms or deferred until severe psychiatric symptoms have been controlled. We report a case of mild hypercalcemia that caused severe psychosis in PHPT, which improved dramatically following PTX and resulted in successful postoperative management.Patient concern:Our patient was a 68-year-old Japanese woman. She was diagnosed with PHPT, which was triggered by mild hypercalcemia. She was due to receive an operation for osteoporosis and kidney stones. She had severe psychosis, despite medication. Blood examinations revealed mild hypercalcemia (10.4 mg/dL, 8.8–10.1 mg/dL) and elevated serum levels of intact PTH (184.0 pg/mL, 10–65 pg/mL).Diagnosis:She was diagnosed with severe psychosis caused by mild hypercalcemia in PHPT.Interventions:Although she was treated with 37.5 mg quetiapine and 2 mg risperidone daily, she was excessively sedated and rejected oral treatment. Therefore, we decided to perform the operation.Outcomes:Immediately following surgery, serum levels of calcium, and intact PTH were normalized. Her psychotic symptoms ceased completely 5 days after surgery.Conclusion:We emphasize that PHPT presents with various severe psychiatric symptoms, even in mild hypercalcemia. Psychiatric symptoms may be the only salient symptoms in PHPT, and thus clinicians should suspect PHPT in patients with psychiatric symptoms and mild hypercalcemia. Furthermore, PTX is recommended for PHPT—even in the presence of severe uncontrolled psychiatric symptoms, which carries risks for postoperative management—because psychiatric symptoms are expected to improve and good postoperative management is possible.     

Early critical cortical infarction by anti-angiotensin II type 1 receptor antibody: A case report and literature review

Rationale:Anti-angiotensin II type 1 receptor antibodies (AT₁R-Abs) have been demonstrated to increase the risk of antibody-mediated rejection. We report a case of AT₁R-Ab mediated rejection which caused early critical cortical infarction.Patient concerns:A 52-year-old man with end-stage kidney disease underwent preemptive kidney transplantation (KT) from his wife. He had no immunologic risk except ABO incompatibility. Proper desensitization treatment were applied prior to KT. On postoperative day 1, he showed stable clinical course with adequate urine output, but there was no decrease in serum creatinine level and imaging studies showed hypoperfusion in the transplanted kidney.Diagnoses:Allograft biopsy revealed total cortical infarction with severe necrotizing vasculitis, but the medullary area was preserved. Serum AT₁R-Ab concentration was elevated from 10.9 U/mL before KT to 19.1 U/mL on 7 days after KT.Interventions:He was treated with plasmapheresis, intravenous immunoglobulin, rituximab, high-dose methylprednisolone, and bortezomib.Outcomes:The treatment showed a partial response, and he was discharged with 7.3 mg/dL creatinine level. At 4 months, his creatinine plateaued at 5.5 mg/dL and AT₁R-Ab decreased to 3.6 U/mL.Lessons:This case highlights the risk of early active antibody-mediated rejection by preformed AT₁R-Ab, suggesting its ability to exhibit atypical histopathologic findings, such as total cortical infarction.     

Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review

Rationale:Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib.Patient concerns:The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission.Diagnosis:SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS.Interventions:The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated.Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms.Outcomes:After dexamethasone 15 mg q8 h treatment, the patient''s fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone.Lessons:Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients.     

A rare case of diabetic ketoacidosis presenting with severe hypertriglyceridemia requiring plasmapheresis in an adult with type-2 diabetes mellitus: Case report

Introduction:Severe hypertriglyceridemia (HTG) is a rare complication of insulin resistance. Its presentation with diabetic ketoacidosis (DKA) has been reported in a few cases, where most patients have type-1 diabetes mellitus (DM). Our case represents a unique presentation of DKA associated with severe HTG above 10,000 mg/dL in an adult with type-2 DM.Patient concerns and diagnosis:Case Report: A 51-year-old man with no prior illnesses presented to the emergency department with abdominal pain and nausea. He was found to have DKA with a blood glucose level of 337 mg/dL, pH of 7.17, beta-hydroxybutyrate of 7.93 mmol/L, and anion gap of 20 mmol/L. His triglyceride levels were >10,000 mg/dL. His serum was found to be lipemic. Computerized tomography scan of the abdomen demonstrated mild acute pancreatitis. Negative GAD65 antibodies supported the diagnosis of type-2 DM.Interventions and outcomes:Endocrinology was consulted and one cycle of albumin-bound plasmapheresis was administered. This therapy significantly improved his HTG. DKA gradually resolved with insulin therapy as well. He was discharged home with endocrinology follow-up.Conclusion:This unique case highlights an uncommon but critical consequence of uncontrolled DM. It brings forth the possibility of severe HTG presenting as a complication of uncontrolled type-2 DM. Severe HTG commonly presents with acute pancreatitis, which can be debilitating if not managed promptly. Most patients with this presentation are managed with insulin infusion. The use of plasmapheresis for management of severe HTG has not been well studied. Our case supports the use of plasmapheresis as an effective and rapid treatment for severe HTG.     

Lipoprotein glomerulopathy resulting from compound heterogeneous mutations of APOE gene: A case report

Rationale:Lipoprotein glomerulopathy (LPG) is a rare glomerular disease characterized by the deposition of lipoprotein thrombi in glomerular capillaries. The disease is characterized by proteinuria, progressive renal failure, and characteristic lipoprotein thrombosis in glomerular capillaries. Rare mutations in the apolipoprotein E (APOE) gene mainly contribute to disease pathogenesis.Patient concerns:A 28-year-old man presented with severe proteinuria and hyperlipidemia. The patient was treated with a full dose of prednisone for 2 months and then combined with leflunomide 20 mg daily for 20 days; however, his edema continued to worsen.Diagnosis:The patient was diagnosed LPG by laboratory examination and renal biopsy.Interventions:The patient was treated with atorvastatin (20 mg) combined with irbesartan (75 mg) once a day.Outcomes:The patient''s lipidaemia and proteinuria were significantly reduced. Genetic testing showed that the patient carried compound heterozygous mutations in APOE. The APOE gene was inherited from her mother and father. Parents with a heterogeneous mutation had normal kidney function without proteinuria.Lessons:Usually, a single mutation in APOE can lead to the pathogenesis of LPG. This case shows that LPG could result from compound heterogeneous mutations of the APOE gene inherited from his mother and father. Intensive lipid-lowering combined with RASIs is effective in patients with LPG. Early renal biopsy and genetic mutation detection can avoid the unnecessary use of glucocorticoids and immunosuppressants.     

Membranous nephropathy without vacuolated podocytes in Fabry disease treated with agalsidase-β and carbamazepine: A case report

Rationale:Vacuolated podocytes are the most common form of renal damage in Fabry disease, but other types of renal damage have been reported, such as membranous nephropathy (MN) or IgM nephropathy. Enzyme replacement therapy (ERT) is effective at preventing renal damage, but the nephropathies require appropriate treatment to prevent renal damage.Patient concerns:A 22-year-old male with Fabry disease presented with proteinuria during ERT with agalsidase-β and carbamazepine. He had received the treatment for 10 years and maintained normal plasma globotryaosylceramide levels.Diagnosis:Renal biopsy revealed MN without vacuolated podocytes. Immunofluorescent staining of the IgG subclass revealed granular patterns of IgG1, G2, G4, and C3 deposition in the glomerular basement membrane.Interventions:The carbamazepine dose was reduced from 600 mg/day to 200 mg/day (serum concentration 10.0-11.0–4.0–5.0 μg/mL).Outcomes:After reducing the carbamazepine dose, proteinuria was negative, and the patient has had a normal urinalysis for 17 months. Plasma globotryaosylceramide levels have also remained normal.Lessons:This report is a reminder of the co-existence of MN without vacuolated podocytes in Fabry disease during ERT with agalsidase-β and carbamazepine.Physicians should be aware of this form of renal damage in Fabry disease, even during treatment.