Aspergiloza kręgu szyjnego u chorej we wczesnym okresie po przeszczepieniu nerki

The article presents a case of a patient who shortly after a kidney transplantation (KTx) developed a fungal infection of the intervertebral disc. Eight weeks after KTx the patient reported neck and shoulder pain. After extensive diagnostics, cervical spine MRI showed an active inflammation within the C6/C7 vertebrae. The patient required neurosurgical intervention. In the course of intraoperative examination, mycelium was found in the direct preparation, the diagnosis of Aspergillus niger was subsequently confirmed in the mycological examination. The initial treatment with voriconazole was followed by one year of itraconazole therapy. As a result of the treatment, the symptoms have subsided and the function of the kidney graft remains optimal.W niniejszym artykule przedstawiono opis przypadku pacjentki we wczesnym okresie po KTx, u której doszło do rozwoju grzybicy krążka międzykręgowego. Po 8 tygodniach od zabiegu chora zgłaszała bóle okolicy szyjno-barkowej. Po rozszerzeniu diagnostyki w MR kręgosłupa szyjnego uwidoczniono aktywne zmiany zapalne w obrębie kręgów C6/C7. Chora wymagała interwencji neurochirurgicznej. Już w trakcie badania śródoperacyjnego stwierdzono obecność strzępków grzybni w preparacie bezpośrednim; rozpoznanie grzybicy Aspergillus niger potwierdzono w badaniu mykologicznym. Włączono terapię worykonazolem, a następnie itrakonazolem, którą kontynuowano przez rok w leczeniu ambulatoryjnym. W związku z zastosowanym leczeniem dolegliwości ustąpiły, a czynność nerki przeszczepionej do dnia dzisiejszego pozostaje bardzo dobra

Associations Between Intravenous Iron, Inflammation and FGF23 in Non-Dialysis Patients with Chronic Kidney Disease Stages 3-5

Background/Aims: Both iron deficiency and chronic inflammation are highly prevalent in patients with chronic kidney disease (CKD). The effect of intravenous iron infusion on mineral metabolism in CKD may be modified by inflammation. Intravenous iron theraphy may reduce peripheral degradation, secretion, clearence of iFGF23 and lead to hypophosphatemia. The aim of the study was to evaluate the effect of intravenous iron on mineral metabolism in CKD patients. Methods: 35 non-dialysis patients with CKD stages 3-5. received 100 mg/24h of ferric oxide saccharated solution for 5 days. Serum calcium (Ca), phosphorus (P), parathormone (PTH), intact-FGF23 (iFGF23), C-terminal-FGF23 (cFGF23), bone alkaline phosphatase (BAP) and high-sensitive CRP were assessed on day 1 and 3 at baseline and 2 hours after each dose administration and once on day 6. Plasma iFGF23 and cFGF23, as well as serum BAP were measured with ELISA and other parameters with standard automated laboratory methods. Results: Serum iFGF23 increased after iv iron on day 1 and 6 (from 268.9±446.5 to 326.3±529.9 on day 1; p=0.05 and to 451.4±601 pg/mL on day 6; p=0.03). cFGF23 was reduced only on day 1 (from 654.3±441.3 to 473.6±414 RU/mL; p=0.016). P concentration decreased significantly two hours after the first iron infusion (from 1.69±0.5 to 1.54±0.35 mmol/l; p=0.003). In following days the changes of cFGF23, P and of other calcium-phosphate metabolism were not significant. Serum CRP correlated neither with iFGF-23 nor cFGF-23. Conclusion: Intravenous iron supplementation may only transiently affect the production and degradation of FGF23 resulting in hypophosphatemia at the commencement of iron therapy. Chronic low-grade inflammation does not seem to play a role in that mechanism

Roux-en-y biliary by-pass – a new approach in the treatment of hypertriglyceridemia induced recurrent acute pancreatitis. Clinical case study

Acute pancreatitis is a disease with significant mortality. Hypertriglyceridemia (HTG) is the third most common etiological factor of this disorder after alcohol and gall-stones. The authors presented a case of 42-years old caucasian female who was hospitalized due to recurrence of acute pancreatitis. She had been diagnosed with HTG. She had earlier seven episodes of acute pancreatitis. Endoscopic papillotomy and conservatory treatment didn’t change her complaints and she was consented for surgery. Exclusion of distal part of bile duct was performed. The common bile duct was anastomosed side-to-side to the 70 cm long Roux loop of the jejunum with the ligation of the distal part of the common bile duct. Following the surgery authors observed normalization of amylase, lipase, leukocytosis and CRP levels. During six months after procedure patient didn’t have any new episode of pancreatitis. Exclusion of distal part of bile duct may be a useful tool in surgical treatment of recurrent acute hypertriglyceridemia-induced pancreatitis

Effect of intravenous iron on endogenous erythropoietin and FGF-23 secretion in patients with chronic kidney disease

AbstractIntroduction It has been observed that intravenous iron administration may suppress endogenous production of erythropoietin (EPO). We postulate that this effect may be mediated by increased FGF-23 secretion.Aim of the study To evaluate the short-term effect of intravenous iron sucrose administration on endogenous EPO secretion in patients with chronic kidney disease (CKD).Materials and methods The cohort comprised 35 nondialysis patients with CKD stages 3–5. All received 100 mg of intravenous iron (III)-hydroxide sucrose complex daily for five consecutive days. Plasma EPO, iFGF-23, cFGF-23, PTH, bone alkaline phosphatase (BAP), phosphorus (PO4), calcium (Ca), and high-sensitive C-reactive protein (CRP) were measured before, and two hours after, the first and third iron infusions, and after completing iron therapy.Results EPO concentration at the end of iron treatment was significantly lower than two hours after the first iron infusion (p = 0.0003) and before the third dose (p = 0.0006) (12.6 [10.2, 41.4] mIU/mL. vs. 30.9 [15.9, 54.2] mIU/mL and 33.4 [15.4, 56.7] mIU/mL, respectively). Conversely, plasma iFGF-23 was significantly higher before the third dose (61.1 [18.6, 420.1 4] pg/mL; p = 0.025) and after the course of treatment (92.1 [28.4, 878.1] pg/mL; p = 0.004) compared to pretreatment value (48.4 [16.2, 420] pg/mL). cFGF-23 concentration was significantly lower than baseline after the first iron dose (491.8 [257.7, 1086.3] vs. 339.2 [75.4, 951.2] RU/mL; p = 0.005) and after treatment (398.7 [90.4, 1022.3] RU/mL; p = 0.025). No significant linear correlation was found between changes in plasma EPO and FGF-23.Conclusions Although intravenous iron therapy causes parallel increase of FGF-23 and supression of endogenous EPO, these two effects seem to be independent

First two years of reimbursed enzyme replacement therapy in the treatment of Fabry disease in Poland [version 2; peer review : 2 approved]

Fabry disease (FD) is an ultra-rare genetic lysosomal storage disease caused by pathologic gene variants resulting in insufficient expression of α-galactosidase A. This enzyme deficiency leads to accumulation of globotriaosylceramide and globotriaosylsphingosine in plasma and in different cells throughout the body, causing major cardiovascular, renal, and nervous system complications. Until 2018, reimbursed enzyme replacement therapy (ERT) for FD was available in all European Union countries except Poland.             We present the preliminary results of the first two years of reimbursed ERT in Poland. We obtained data from the seven largest academic centers in Katowice, Cracow, Wrocław, Poznań, Gdańsk, Warsaw, and Łódź. The questionnaire included the following data: number of patients treated, number of patients qualified for ERT, and patient characteristics.             All centers returned completed questionnaires that included data for a total of 71 patients (28 men and 43 women) as of June 2021. Thirty-five patients with the diagnosis of FD confirmed by genetic testing (22 men and 13 women) had already qualified for reimbursed ERT. Mean (SD) age at the commencement of the ERT program was 39.6 (15.5) years (range 18-79 years). Mean time from the first clinical symptoms reported by the patients to the FD diagnosis was 21.1 (8.9) years, and the mean time from the final diagnosis of FD to the beginning of ERT was 4.7 (4.6) years.             FD is still underdiagnosed in Poland. To identify undiagnosed FD patients and to ensure that patients in Poland benefit fully from ERT, implementation of an effective nationwide screening strategy and close cooperation with a network of rare disease centers is advised