Selman Unverdi

Idiopathic Hypoparathyroidism Mimicking Diffuse Idiopathic Skeletal Hyperostosis

Idiopathic hypoparathyroidism is an uncommon disease caused by insufficient secretion of parathyroid hormone. Idiopathic hypoparathyroidism may cause various musculoskeletal findings, including diffuse ligamentous and entheseal ossifications. Diffuse idiopathic skeletal hyperostosis is a disorder of the elderly characterized by ossification of the anterior longitudinal ligament of the spine and various extraspinal ligaments. We present a 50-year-old man with idiopathic hypoparathyroidism who had been diagnosed as having DISH at 40 years of age. Resistant neck, left shoulder, and left hip pain did not improve with calcium and calcitriol treatment after diagnosis of idiopathic hypoparathyroidism.

Acute Kidney Injury due to Rhabdomyolysis in H1N1 Influenza Infection

Acute kidney injury (AKI) is rarely reported in the clinical course of H1N1 infection and this condition is strongly related with increasing of mortality risk. However, there are no sufficient data about the development of AKI due to H1N1 infections. The recent reports were documented for elevation of creatinine phosphokinase levels in the course of influenza infection, but rhabdomyolysis was rarely reported. Herein, we present a 28-year-old female patient and a 19-year-old male patient with AKI in the course of H1N1 influenza infection due to rhabdomyolysis.

Behcet's disease and IgA nephropathy.

Although Behçet’s disease (BD) is a kind of systemic disease, renal involvement is rare, especially IgA nephropathy (IgAN). Renal manifestations in BD range from mild urinary abnormalities to glomerulonephritis with persistent renal failure, which includes minimal change disease, proliferative glomerulonephritis, rapidly crescentic glomerulonephritis, renal amyloidosis and IgA nephropathy. Amyloidosis seems to be the most common type of renal lesion in BD, and several cases of nephrotic syndrome secondary to amyloidosis have been documented. Co-occurrence of BD and IgA nephropathy has only been reported in only few cases. We describe two patients with the rare association of BD and IgAN. We suggested that it is important to periodically perform renal function assessment in patients with BD, through urinalysis and measurement of serum creatinine for detecting any abnormality and providing an early adequate treatment.

Is colchicine therapy effective in all patients with secondary amyloidosis

Abstract Objective: Although colchicine is effective on prevention and regression of amyloidosis in many cases, rate of unresponsiveness to colchicine therapy is not too low. However, there is no sufficient data about which factors effect to response of colchicine therapy on regression of amyloidosis. Materials and methods: 24 patients with renal amyloidosis were enrolled into the study. The patients were divided in two groups according to urinary protein excretions: non-nephrotic stage (14/24) and nephrotic stage (10/24). The patients were also categorized according to the etiology of amyloidosis; familial Mediterranean fever (FMF)-associated amyloidosis (15/24) versus rheumatoid disorders (RD)-associated amyloidosis (9/24). The changes of amount of proteinuria and estimated glomerular filtration rates were investigated after colchicine treatment started in these groups. Results: The mean follow-up period was 27.7 ± 19.2 months. After initiating colchicine therapy, the degree of proteinuria was decreased higher than 50% in 11/14 (78%) of non-nephrotic patients and elevated only in three (22%) patients. In nephrotic group, proteinuria was increased in 5/10 (50%) of patients. Glomerular filtration rates were stable in nephrotic and non-nephrotic groups. Presenting with nephrotic syndrome was higher in RD-associated amyloidosis (RD_A) group (5/9) than FMF-associated amyloidosis (FMF_A) group (5/15) without statistical significance (p > 0.05). After colchicine treatment, proteinuria was decreased in 12/15 patients in FMF_A group, however, the significant decreasing of proteinuria was not observed in RD_A group (p = 0.05 vs. p > 0.05). Conclusion: Colchicine therapy was found more effective in low proteinuric stage of amyloidosis. The beneficial effect of colchicine therapy was not observed in patients with RD- associated amyloidosis.

An unusual effect of colchicine treatment in familial Mediterranean fever-associated glomerulonephritis.

Kidney involvement is usually depend on amyloidosis in Familial mediterranean fever (FMF) and in most cases with glomerulonephritis has been reported [1]. Here, we report remission of proteinuria, which is observed with only regular colchicine treatment, is a case of IgA nephropathy in the course of FMF. A 27-year-old woman was admitted to our hospital because of proteinuria in urinary analysis. She has not regularly taken colchicine treatment who had been diagnosed FMF 15 years ago. The patient’s physical examination findings were normal. The patient’s laboratory findings were 5 erythrocytes identified by microscopic examination and proteinuria (619 mg/day), but others were normal. Percutaneous kidney biopsy was performed due to persistent proteinuria. The diagnosis of IgA nephropathy was made based on light and immunofluorescence microscopic assessment. Staining for amyloidosis was negative. After M694 V/M694 V homozygous mutation was confirming the diagnosis of FMF, regular colchicine treatment was begun (0.5 mg, t.i.d). Because of resolving proteinuria under colchicine treatment alone, immunosuppressive and ACEI/ARB therapies were not used. After taking regular treatment, the patient had no fever and abdominal pain attacks. In the following year, the patient’s creatinine 0.7 mg/dl, creatinine clearance 108 ml/min/ 1.73 m, and urinalysis were completely normal. FMF is an autosomal-recessive genetic disease affecting mainly Turks, non-Ashkenazi Jews, Armenians, and Arabs. It is characterized by recurrent attacks of fever and painful episodes of sterile poliserositis and less frequently erysipelas-like rash. MEFV, the gene responsible for the disease, is localized on the short arm of chromosome 16 and encodes a 781-amino acid protein known as pyrin or marenostrin that is expressed in matur neutrophils plays an essential role in the pressure of inflammation. Adversely, mutated pyrin associates with uncontrolled inflammation through interleukin-1b and NF-jB activation, resulting in up-regulation of inflammatory cytokines pathways [2]. Colchicine is effective in preventing the trigger of FMF attacks and to hinder subclinical inflammation preventing the deposition of amyloid fibrils. It binds to b-tubulin hindering its polarization with consequent defective intracellular transfer and mitosis, inhibition of neutrophil chemotaxis, and reduced expression of adhesion molecules [3]. In addition, it has antioxidant and antifibrotic properties, all of them might have contributed to the remission of proteinuria. In the literature up to now, many times glomerulonephritis associated with FMF has been reported, but there is less resolving proteinuria with colchicine therapy. Cagdas et al. reported was remission of a patient with mesengial proliferative glomerulonephritis occurred after 3 years who took regular treatment [4]. Gok et al. and Koukoui et al. have been reported the remission of FMF-related IgA nephropathy while taking colchicine therapy [5, 6]. Similarly, remission of our patient with IgA nephropathy and FMF was successful after taking 1-year colchicine therapy. In conclusion, several kinds of glomerular diseases can be seen in FMF, and FMF-associated glomerulonephritis M. Ceri (&) S. Unverdi R. Yılmaz M. Duranay Department of Nephrology, Ankara Education and Research Hospital, Cebeci, Ankara, Turkey e-mail: tscer@yahoo.com

Massive intracerebral hemorrhage associated with Wegener granulomatosis

Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7–11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

Focal segmental glomerulosclerosis in association with Gitelman syndrome

Gitelman syndrome (GS) is an autosomal recessive disorder characterized by hypokalemia, metabolic alkalosis, hypomagnesemia and hypocalciuria. Glomerulonephritis associated with GS is rarely documented in the literature. We present an adult patient with GS whose renal biopsy revealed focal segmental glomerulosclerosis.

A patient with Crohn's disease who presented with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome.

Thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS) is characterized with fever, purpura, anemia due to microangiopathic hemolysis, thrombocytopenia, kidney damage, and neurologic symptoms. The development of TTP/HUS during the course of inflammatory bowel diseases was rarely reported. However, coexistence of TTP/HUS and Crohns disease in the same patient has not been reported previously. We herein present a case of TTP/HUS who presented with Crohns disease. He responded to cyclosporine treatment.

Is Tolvaptan in the Treatment of SIADH Following Pituitary Surgery a Right Choice

Background: Fluid and electrolyte disturbances, especially hyponatremia are common complications after pituitary surgeries. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the leading cause of hyponatremia in hospitals. Fluid restriction is the first step in treatment of SIADH. In rare cases vasopressin receptor inhibitors might be useful for medical treatment. Case Presentation: In this paper, we present a case of SIADH after a pituitary surgery with an unpredictable effect of tolvaptan. Conclusion: Tolvaptan is the most preferred agent for the treatment of SIADH nowadays. However tolvaptan might cause unpredictable situations in the course of treatment that clinicians must be very careful and in close follow up the patient.

A rare cause of diarrhea in patients with focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) symbolizes a common histologic pattern of glomerular injury associated with numerous disease mechanisms. Ulcerative colitis (UC) represents one of the types of inflammatory bowel disease, which occurs in genetically predisposed individuals. The coexistence of these two diseases is an unexpected condition. Lately, case reports have been published documenting the development of nephropathy after treatment of ulcerative colitis with mesalamine or sulphasalazine. In cases in the literature, this coexistence has been identified as associated with 5-ASA therapy.1–5 In this case, we report the ulcerative colitis occurring in a patient with focal segmental glomerulosclerosis not affiliated with 5-ASA therapy. A 66-year-old man, with a 3-year history of focal segmental glomerulosclerosis, was admitted with bloody and mucoid diarrhea which had been for lasting for 10 days. There was no fever, nausea, vomiting or infection. There was no feature in the patient’s history except diarrhea. Physical examination also was normal. Laboratory investigation demonstrated impaired renal function and proteinuria due to focal segmental glomerulosclerosis. Renal function test which is similar to the old values showed serum creatinine level of 3.15 mg/dl (0.8–1.3), BUN level of 89 mg/dl (17–43) and 24-h urine protein level of 1876 mg/day (<200). In addition, his erythrocyte sedimentation rate (ESR) was 79 mm/h (<20) and C-reactive protein (CRP) was 6.66 mg/dl (<0.4). A large amount of leukocytes and erythrocytes was seen in the stool microscopy. Stool cultures were detected negative twice. Colonoscopy revealed that there were to exudates of millimetric ulcers descending colon, sigmoid colon and rectum. The colon biopsy confirmed the diagnosis of ulcerative colitis. The patient was started on mesalamine. His symptoms showed marked improvements after starting mesalamine treatment. After treatment, laboratory investigation demonstrated; creatinine level of 3.7 mg/dl (0.8–1.3), BUN level of ria