De novo glomerulitis associated with graft-versus-host disease after allogeneic hematopoietic stem cell transplantation: A single-center experience

BACKGROUND: Nephrotic syndrome (NS) and proteinuria are uncommon, often unrecognized manifestations of graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT). Only a few isolated case reports and case series involving smaller number of patients who developed NS after HSCT have been published. METHODS: We reviewed the renal histopathological examination findings and clinical records of 15 patients who developed proteinuria after HSCT at Seoul and Yeouido St. Mary's Hospital (Seoul, Korea). We also measured the anti-PLA2 Rantibodies (M-type phospholipase A2 receptor) in the serum samples from the seven patients at the time of renal biopsy. RESULTS: All patients had GVHD. The most common indication for biopsy was proteinuria ( > 1 g/day), with nine patients having nephrotic range proteinuria. The most common histopathological finding was membranous nephropathy (MN; n = 12).Other findings were membranoproliferative glomerulonephritis, C1q nephropathy, and diabetic nephropathy. Eleven patients were treated with immunosuppressive agents, and three patients were treated only with angiotensin II receptor blocker. The overall response rate, including complete remission (urinary protein level < 0.3 g/day) and partial remission (urinary protein level = 0.31-3.4 g/day), was 73%. The mean follow-up period was 26 months, and none of the patients developed end-stage renal disease. All of the seven patients with MN had negative findings for anti-PLA2R antibodies, measured using an enzyme-linked immunosorbent assay kit. CONCLUSION: In this study the findings of 15 renal biopsies were analyzed and to our knowledge this is the largest clinicopathological study of GVHD-related biopsy-proven nephropathy. Approximately 80% of the patients were MN and 73% responded either partially or completely to immunosuppressive treatment. Currently, there is an increase in the incidence of GVHD-mediated renal disease, and therefore, renal biopsy is essential for diagnosing the nephropathy and preventing the progression of renal disease.

Evaluation of the Relationship between Circadian Blood Pressure Variation and Left Atrial Function Using Strain Imaging

BACKGROUND: Non-dippers were reported as showing different left atrial function, compared to dippers, but no study to date investigated the changes in the left atrial function according to the diurnal blood pressure pattern, using tissue Doppler and strain imaging. METHODS: Forty never treated hypertensive patients between 30 and 80 years of age were enrolled in this study. Patients were classified as non-dippers when, during night time, they had a blood pressure decrease of less than 10%. Strain of the left atrium was measured during late systole, and peak strain rates of the left atrium were measured during systole, early and late diastolic periods. RESULTS: The left atrial expansion index, left atrial active emptying volume and left atrial active emptying fraction were all significantly increased in non-dippers. They also had increased values of mean peak left atrial strain (dippers = 21.26 +/- 4.23% vs. non-dippers = 24.91 +/- 5.20%, p = 0.02), strain rate during reservoir (dippers = 1.29 +/- 0.23 s-1 vs. non-dippers =1.52 +/- 0.27 s-1, p = 0.01) and contractile period (dippers = -1.38 +/- 0.24 s-1 vs. non-dippers = -1.68 +/- 0.32 s-1, p < 0.01). CONCLUSION: Strain and strain rate acquired from color Doppler tissue imaging demonstrate exaggerated reservoir and booster pump function in never-treated, non-dipper hypertensive patients. These methods are simple and sensitive for the early detection of subtle changes in the left atrial function.

A case of plexiform neurofibroma involving the mediastinum and abdomen in a patient with neurofibromatosis / 대한내과학회지

Plexiform neurofibroma is a benign proliferation that arises from the peripheral nerves and represents one of the diagnostic features of neurofibromatosis type I (NF-1). They are commonly found in the gastrointestinal tract, and intrathoracic neurofibroma is relatively uncommon. Ultimately, this tumor grows along the length of any single nerve and may involve multiple fascicles or branches of major nerves. Here, we report a case of multiple-site plexiform neurofibromas in a 40-year-old man previously diagnosed with NF-1. Although he had no perceptible symptoms, contrast-enhanced CT revealed massive diffuse neurofibromas that involved the mediastinum, periportal region, retroperitoneal space, and the mesentery. Histopathological studies of the neck, skin, and intra-abdominal soft tissue showed loose spindle cells and collagen bundles. The microscopic appearance was typical of a plexiform-type neurofibroma. It was decided that we would observe the patient without surgical management, because the neurofibromas were asymptomatic.

A case of rhabdomyolysis during hospitalization for acute hepatitis A / 대한간학회지

A 29-year-old man was admitted to hospital with fever, myalgia, and sore throat. Initial laboratory findings were compatible with acute hepatitis; he was positive for the serologic marker for acute hepatitis A. On the 3rd day of admission, in spite of normalization of body temperature and a reduction in serum liver enzyme levels, serum levels of creatinine phosphokinase had increased up to 16,949 U/L. The patient recovered with supportive therapy and was discharged on the 12th day. We report a case of acute hepatitis A complicated by rhabdomyolysis during hospitalization.