Hypertension and nephrology

[Article Reports]

VÁLYI Péter

DECEMBER 10, 2018

Hypertension and nephrology - 2018;22(06)

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Hypertension and nephrology

[Public Statement of the Hungarian Society of Hypertension – Treatment of Hypertension: the 2018 European Recommendation should be Followed Still]

MAGYAR Hypertonia Társaság

Hypertension and nephrology

[Antiplatelet Treatment in Peripheral Vascular Disease – Paradigm Shift in Practice? ]

FARKAS Katalin, SZŐKE Vince Bertalan, JÁRAI Zoltán

Hypertension and nephrology

[Possible Role of Cannabinoids in the Treatment of Obstructive Sleep Apnea ]

KISS Zoltán, ALFÖLDI Sándor

Hypertension and nephrology

[PAX2: lotium et visus sine pace]

VIOLETTA Antal, KERTI Andrea, JÁVORSZKY Eszter, MÁTTYUS István, REUSZ György, SZABÓ Attila, VÁRKONYI Ildikó, MAKA Erika, TORY Kálmán

[The autosomal dominant papillorenal syndrome results from primarily de novo mutations of PAX2. It encodes a transcription factor expressed in the kidney, urinary tract, nervous system, eye and the ear. Its haploinsufficiency causes primarily hypoplastic and hyperreflective kidney, or other forms of CAKUT. The clinical appearance may be dominated by nephrotic-range proteinuria with focal segmental glomerulosclerosis. The renal survival rate is highly variable: most of the recognized cases lead to ESRD during the first four decades of life. PAX2 mutations cause typical optic papillary alterations, most frequently papillary dysplasia. In contrast to the name of the syndrome, one fourth of the affected patients do not develop ocular involvement. Hearing impairment is associated in less than 10% of the patients. The affected members of the five families that we identified with PAX2 loss-of-function mutations, developed end-stage renal disease during the 2-4. decades of life.]

Hypertension and nephrology

[Prognostic role of arterial stiffness in IgA nephropathy]

SÁGI Balázs, KÉSŐI István, VAS Tibor, CSIKY Botond, KOVÁCS Tibor, NAGY Judit

[Background: Arterial stiffness has a prognostic role in chronic cardiovascular diseases. Pulse wave velocity (PWV) determined by the carotid-femoral pulse detection is accepted as a gold standard method. Further diagnostic procedures are in use to assess the arterial stiffness including the finger photoplethysmography. The prognostic role of this method is limited in chronic renal diseases. The goal of our investigation was to determine the prognostic significance of the stiffness index (SIDVP) measured by the photoplethysmographic method in IgA nephropathy. Patients and methods: One hundred and three histologically proved IgA nephropathy patients with chronic kidney disease stage 1-4 were investigated (67 male, 36 female, 45 ± 11 years) and followed for an average 65 (6-107) months. The stiffness index was determined by the volume alteration of the digital artery during the cardiac cycle (Pulse Trace system, Micro Medical, Gilingham, Kent, UK). The primary combined end point was total mortality, major cardiovascular events (stroke, myocardial infarction or cardiovascular procedure, for example revascularisation) plus achieving end stage renal disease. The secondary end points were cardiovascular and renal end points alone. Results: The patients with increased stiffness index (> 10 m/s) had significantly more combined primary end point events (10/60 vs. 19/43, P = 0.015). In case of the secondary end points the renal end points were significantly more frequent in patients with higher stiffness index. Stiffness index has also proved to be an independent predictor on survival from other cardiovascular risk factors (age, hypertension, diabetes, obesity, lipid disturbances and decrease of renal function) using the Cox regression model in IgA nephropathy. Every 1 m/s increase in stiffness index resulted a 17% gain in the occurrence of the combined primary end point. Conclusions: Stiffness index determined by finger photoplethysmography is an eligible parameter to assess the prognosis in IgA nephropathy. Increased stiffness index in IgA nephropathy seems to be a good prognostic tool for identification of higher risk patients.]

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

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[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

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[Ulcerative carpal tunnel syndrome]

KAMONDI Anita, TEIXEIRA Jose Maria, SZIRMAI Imre

[The carpal tunnel syndrome is the most frequent compression-induced neuropathy. A severe but rare clinical manifestation of this disorder associates with ulceration, acral osteo-lysis and mutilation of the terminal phalanges of the second and third fingers. Recognition of this disorder is difficult, because various dermatological and internal diseases might lead to acral ulcerative lesions, and these patients are seldom referred to neurological and/or electrodiagnostic examination. In this article, we present three cases of this rare clinical form of carpal tunnel syndrome and discuss the electrodiagnostic findings. The early diagnosis is important since decompression of the median nerve in due time might prevent mutilation and could significantly improve the patients’ quality of life.]