Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib.

BACKGROUND: Primary myelofibrosis (PMF) is a type of myeloproliferative neoplasm (MPN) characterized by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, leading to the deposition of fibrous tissue, and by a propensity toward extramedullary hematopoiesis. Renal involvement in PMF is rare, but kidney tissue samples from these patients reveal MPN-related glomerulopathy, a recently discovered condition, in the late stages of the disease. CASE PRESENTATION: We present the first case described in the medical literature of a patient with early renal glomerular involvement in PMF/MPN. A 60-year-old man with stage 4 chronic kidney disease and a recent diagnosis of PMF (within 4 weeks of presentation at our renal division) presented with generalized body swelling, acute kidney injury, and massive nephrotic-range proteinuria. Kidney biopsy was performed to determine the etiology of the patient's renal dysfunction and revealed early renal glomerular involvement that was histologically characteristic of MPN-related glomerulopathy. Early diagnosis and prompt medical management returned the patient's kidney functionality to the levels seen on initial presentation at our hospital. CONCLUSION: Large studies with long follow-up durations are necessary to identify and categorize the risk factors for the development of MPN-related glomerulopathy, to standardize therapeutic regimens, and to determine whether aggressive management of the myelofibrosis slows the progression of kidney disease.

CT urography for the diagnosis of medullary sponge kidney.

BACKGROUND: Medullary sponge kidney (MSK) is characterized by malformation of the terminal collecting ducts and is associated with an increased risk of nephrolithiasis, nephrocalcinosis, urinary tract infections, renal acidification defects, and reduced bone density. It has been historically diagnosed with intravenous pyelography (IVP), which is falling out of favor as an imaging modality. CT urography (CTU) performed with multidetector CT (MDCT) has been shown to create images of the renal collecting system with similar detail as IVP; however, its utility in diagnosing MSK has not been defined. CASE REPORT: We present the first 15 patients with recurrent symptomatic nephrolithiasis who were evaluated in our renal stone clinic with CTU. Four patients were diagnosed with MSK after visualization of the characteristic radiologic findings. DISCUSSION: CTU effectively demonstrates the characteristic radiologic findings of MSK including collecting tubule dilatation, medullary nephrocalcinosis, nephrolithiasis, and medullary cysts. Dose reduction protocols can reduce radiation exposure below that associated with conventional IVP. We propose CTU be considered for the diagnosis of MSK.

Household Financial Burden and Poverty Impacts of Cancer Treatment in Vietnam.

PURPOSE: This paper aims to analyze the household financial burden and poverty impacts of cancer treatment in Vietnam. METHODS: Under the "ASEAN CosTs in ONcology" study design, three major specialized cancer hospitals were employed to assemble the Vietnamese data. Factors of socioeconomic, direct, and indirect costs of healthcare were collected prospectively through both individual interviews and hospital financial records. RESULTS: The rates of catastrophic expenditure based on the cut-off points of 20%, 30%, 40%, and 50% of household's income were 82.6%, 73.7%, 64.7%, and 56.9%, respectively. 37.4% of the households with patient were impoverished by the treatment costs for cancer. The statistically significant correlates of the impoverishment problem were higher among older patients (40-60 years: 1.77, 95% CI 1.14-2.73; above 60 years: 1.75, 95% CI 1.03-2.98); poorer patients (less than 100% national income: 29, 95% CI 18.6-45.24; less than 200% national income: 2.89, 95% CI 1.69-4.93); patients who underwent surgery alone (receiving nonsurgery treatment: 2.46, 95% CI 1.32-4.59; receiving multiple treatments: 2.4, 95% CI 1.38-4.17). CONCLUSIONS: Lots of households were pushed into poverty due to their expenditure on cancer care; more actions are urgently needed to improve financial protection to the vulnerable groups.