Prevalence of Diagnostic Methods and Treatment Modalities in Vipoma Patients: A Rare Cause of Hormone-Mediated Diarrhea.

INTRODUCTION: VIPoma is a neuroendocrine tumor that secrets vasoactive intestinal peptide and produces a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia, hypochlorhydria and metabolic acidosis. The aim of this study to investigate clinical studies about diagnostic and therapeutic modalities of vipoma patients. In this retrospective study, all patients of vipoma were investigated. Clinical features, laboratory data at initial presentation, management and outcomes were collected. SUBJECTS AND METHODS: The paper has written based on searching PubMed and Google Scholar to identify potentially relevant articles or abstracts. Categorical variables as percentage and continuous variables were reported as mean ± standard deviation (SD). RESULTS: All the patients presented with watery diarrhea (30/30, 100%) and dehydration was reported in 33.3% of them. Prevalence of laboratory findings in these patients were assessed for hypokalaemia (25/30, 83.3%), metabolic acidosis (9/30, 33.6%), hypochloremia and achlorhydria (2/30, 6.6%). Elevated VIP levels have been seen in 73.3% patients with mean values of 882.85 ± 1134.87 pg/ml. Prevalence of diagnostic methods included CT scan in 19 patients (19/30, 63.3%), ultrasonography (15/50, 50%), and somatostatin receptor scintigraphy (8/30, 26.6%). Medical treatments included somatostatin and analogues in 18 patients (18/30, 60%). Surgery included less percentage of treatment in these patients. CONCLUSION: CT scan can be used as a reliable modality for diagnosis of vipoma and somatostatin analogues can be used as the most effective treatment in vipoma patients. Surveillance of these patients needs to close monitoring of patients via history, physical examination, laboratory and imaging.

Juvenile nephronophthisis and dysthyroidism: a rare association.

Nephronophthisis, an autosomal recessive kidney disease, represents the most frequent genetic cause of end-stage kidney disease in the first three decades of life. A 27-year-old male was presented with gait imbalance, sever pruritus since 10 days prior time of admission. In past medical history, he had bilateral cataract, torsional nystagmus, and bilateral optic nerve atrophy since 2 years of age. He was also mentioned history of multinodular goiter with dysfunctional thyroid state since 2 years before admission. At admission bilateral blindness, torsional nystagmus, asymmetric thyromegaly with nodularity was found in physical examination. Laboratory tests showed elevated urea and creatinine (200, 10.7 mg/dl), hypomagnesemia (1.1 mEq/l), decreased thyroid stimulating hormone (<0.004 mIU/l). Ophthalmologist consultation confirmed retinitis pigmentosa. Renal sonography showed small-sized kidneys. Brain magnetic resonance imaging did not reveal molar tooth sign. Genetic testing performed and a large homozygous deletion at the NPHP1 gene locus was found. The patient was diagnosed with juvenile nephronophthisis and consideration of dysthyroidism as extrarenal manifestation of nephronophthisis is suggested in this case. Furthermore, loss of function mutation in SLC41A1 gene that leads to magnesium depletion must be noted in patients with suspected to nephronophthisis.

Fibroblast Growth Factor 23 in Postrenal Transplant: An Often Forgotten Hormone.

Fibroblast growth factor 23 is likely to be the most important regulator of phosphate homeostasis, which mediates its functions through fibroblast growth factor receptors and the coreceptor Klotho. In addition to reducing expression of the sodium-phosphate cotransporters NPT2a and NPT2c in the proximal tubules, fibroblast growth factor 23 inhibits renal 1α-hydroxylase and stimulates 24-hydroxylase and appears to reduce parathyroid hormone secretion in short-term studies. Fibroblast growth factor 23 synthesis and secretion by osteocytes and osteoblasts are upregulated through 1,25-dihydroxyvitamin D3 and through an increased dietary phosphate intake. Recent studies have indicated that a low-protein diet and calcium deficiency reduce circulating fibroblast growth factor 23 levels, but magnesium deficiency increases fibroblast growth factor levels. Drugs such as phosphate binders, bisphosphonate, and estrogens have various effects on circulating fibroblast growth factor 23 levels. The high cardiovascular disease event rates and mortality associated with elevated levels of this hormone may be due to various effects on the cardiovascular system, including left ventricular hypertrophy, arterial stiffness, vascular calcifications, endothelial dysfunction, and increased levels of inflammatory markers. In addition, elevated levels of this hormone may contribute to mineral bone metabolism disorders and to patient and allograft survival after renal transplant. Here, we discuss the effects of fibroblast growth factor 23 on adverse renal, bone, and cardiovascular outcomes after kidney transplant.

Serum tumor markers in chronic kidney disease: as clinical tool in diagnosis, treatment and prognosis of cancers.

Cancer is singled out as the biggest cause of death in the world, predicted to reach 13.1 million cancer-related deaths by the year 2030. Although there are no specific tumor markers used in cancer screening, some markers can be used to assist in making a diagnosis and determining a prognosis. They can be used to follow in cases where the diagnosis is cancer through monitoring of the disease recurrence and/or evaluating the response to therapy. These markers are not specific as the number increases in multiple cases of cancer. Some markers are positive in a single type of cancer; others are detectable in more than one type. An ideal tumor marker should be highly sensitive, specific, and reliable with high prognostic value. Other characteristics of an ideal tumor marker are organ specificity and correlation of it with tumor stages. However, none of the tumor markers reported to date has all these characteristics. Influence of different stages of chronic kidney function on serum tumor markers is variable. Furthermore, hemodialysis, peritoneal dialysis, and kidney transplantation affect on tumor markers differently. Sometimes, no study has been found in the literature review. Combined serum tumor markers may also be valuable. This literature review points the role of serum tumor markers in screening, diagnosis, and follow-up of cancer patients in chronic kidney disease patients and renal allograft recipients. In addition, impact of chronic kidney disease and kidney transplantation on different serum tumor markers is briefly explored.

Contemporary management of phosphorus retention in chronic kidney disease: a review.

Hyperphosphatemia is the most common metabolic complications of end-stage kidney disease (ESKD). Large observational studies have identified hyperphosphatemia as an independent risk factor for cardiovascular disease and mortality in dialysis patients and subsequent studies found that subtle increases in serum phosphate levels even within the normal range are also associated with increased risk for death in predialysis and non-kidney disease population. On the basis of these results, current national practice guidelines advocate more aggressive treatment of hyperphosphatemia to lower serum phosphate targets than in the past . Treatment of hyperphosphatemia requires to strict management through dietary restriction, oral phosphate binders, and dialysis. Calcium-based phosphate binders have low cost and widespread use but cause vascular calcification and hypercalcemia. Non-calcium-based phosphate binders are effective but expensive. Bixalomer is a new Ca-free, metal-free, potent phosphate binder, non-hydrochloride, and non-absorptive polymer, which improves metabolic acidosis. FGF-23 appears as a promising target for novel therapeutic approaches to improve clinical outcomes of CKD patients. This review focuses on novel therapeutic approaches dealing with hyperphosphatemia in chronic kidney disease.

Concurrent acute spontaneous tumor lysis syndrome complicated with multiple organ failure in a patient with pre-existing undiagnosed lung cancer.

Acute kidney injury, a common complication associated with malignancy, occurs in various clinical situations for numerous reasons. Acute tumor lysis syndrome (TLS) is possibly the most significant cause of acute kidney injury in cancer patients, because it is fulminant at onset and associated with severe metabolic derangements. Acute spontaneous tumor lysis syndrome is rare and most of the related malignancies belong to hematologic malignancies but it has seldom been investigated as bulky or advanced metastatic non-hematologic malignancies. TLS comprises a clinical laboratory derangement of cellular metabolism which can lead to acute renal impairments, cardiac arrhythmia, seizures and patient demise. Prevention and treatment of tumor lysis syndrome depends on early recognition of at-risk patients, volume repletion and xanthin oxidase inhibitors. In addition, in patients with high risk tumor types, prophylactic use of rasburicase before chemotherapy is required. If dialysis is required, continuous modalities may be favored, particularly in patients with more severe TLS. This case report discusses a 79-year-old man with controlled Alzheimer's disease presented with picture of septic shock and multiorgan dysfunction (acute kidney injury, acute lung injury, acute brain injury) pulmonary suppuration in the right lung field due to aspiration pneumonia with infection-induced systemic inflammatory response (SIRS) was diagnosed. Further work-up revealed lung cancer on chest CT scan. Antibiotic, respirator and hemodialysis treatment improved his condition but died several days later. Acute spontaneous TLS may present in association with infectious SIRS and multiple organ failure, the combination of which results in significant mortality.

Concurrent emphysematous pyelonephritis and thigh necrotizing fasciitis after intramuscular administration of diclofenac.

Necrotizing fasciitis (NF) is a rapidly progressive, life-threatening soft tissue infection. NF may result from any injury to the skin or from hematogenous spread. However, con-current emphysematous pyelonephritis and necrotizing fasciitis of the left thigh has not been reported. We report a case of emphysematous pyelonephritis and necrotizing fasciitis of the left thigh after intramuscular administration of diclofenac that improved with aggressive management including broad-spectrum antibiotics, nephrectomy and surgical intervention.