Hypertension and nephrology

[Article Reports]

VÁLYI Péter

MAY 20, 2017

Hypertension and nephrology - 2017;21(03)

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Hypertension and nephrology

[A Letter to Our Readers]

KÉKES Ede, KISS István

Hypertension and nephrology

[Systemic ANCA-associated vasculitis. Induction immunosuppression therapy, complications and outcome. Part 1]

HARIS Ágnes, POLNER Kálmán

[The present review is compiled of two parts, the first part aims to summarize the induction immunosuppressive therapy, the second part delineates the outcome and complications of ANCA-associated vasculitis. ANCA-associated vasculitis is a systemic disease, accompanied with rapidly progressive glomerulonephritis and severe, often life-threatening extrarenal complications. By early diagnosis and immediate initiation of immunosuppressive therapy, both patient and renal outcome have been substantially improved. The major aims of modern therapeutic protocols are, besides improving survival, to decrease immunosuppressive drug toxicity and avoid infections. Immunosuppression is based on the combination of large dose of corticosteroid and cyclophosphamide, which is advisable to supplement by plasma exchange. The B-cell depleting anti-CD20 monoclonal antibody rituximab, which has already been available in Hungary, has been proved to be similarly effective in newly diagnosed ANCA-vasculitis, and even more effective in a relapsing disease, compared to cyclophosphamide. Amongst rituximab’s further indications in this disease is the preservation of young women’s fertility, and it also has priority in some other special cases. Early diagnosis and prompt immunosuppressive treatment have resulted that ANCAvasculitis became a treatable disease with reasonably good clinical outcome, yet both the disease and the immunosuppressive medications frequently cause complications, which necessitate continuous alertness of the attending nephrologists.]

Hypertension and nephrology

[Isolated systolic hypertension in children and young adults I.]

FARSANG Csaba

[Prevalence of the isolated increase in systolic blood pressure ≥140 mmHg with normal or low diastolic blood pressure ≤80 mmHg, is defined as isolated systolic hypertension. Its prevalence increases with age up to >90% in patients aged >90 years. Isolated systolic hypertension is also found in the young and the clinical significance of it is still debated. For the therapy, those drugs should be used which have a license for use in children: angiotensin converting enzyme inhibitors, angiotensin AT-1 receptor antagonists, calcium channel blockers beta-blockers and diuretics and their combinations. The young adults with isolated systolic hypertension had a much higher risk of dying from coronary heart disease or cardiovascular disease, then the normotensive individuals, and should be treated to normalise their blood pressure. In the elderly and very elderly (>80 yrs), a wealth of data from large clinical trials are available, showing the necessity of treatment mostly with drug combinations - fix-combinations are preferred for increasing the adherence / persistence to therapy. Using diuretics, ACE-inhibitors / ARBs with calcium antagonists, and when needed diuretics and beta-blockers are suggested by recent European guidelines. The target is <140 mmHg, but in octogenarians <150 mmHg. Some studies are pressing for even lower SBP (to around 120 mm Hg), but it seems to be wise to balance advantages / disadvantages, so the optimal SBP may be around 130 mmHg.]

Hypertension and nephrology

[Prevalence of isolated systolic hypertension in our country]

KÉKES Ede, BARNA István, DAIKI Tenno, DANKOVICS Gergely, KISS István

[Prevalence of hypertension over the age of 56 is increasing in size and a significant proportion (60-80%) of isolated systolic hypertension. Within the population screening in the older age groups - in the light of economic development - 25-40% of the prevalence. We have an opportunity to analyse the prevalence and specificity of isolated systolic hypertension from age 36 to age 10 years on the base of 7 years data of the MÁESZ (Comprehensive Health Protection Screening Program of Hungary 2010- 2020) survey. Between 56-65 years 23.27-24.23% (male/female) 66-75 years 34,89-33,15% and over 76 years 44.04-41.5% occurrence was found. Divergence of systolic and diastolic pressure has begun since 36 years. Pulse pressure was used to separate individuals with varying degree of vascular disorders.]

Hypertension and nephrology

[Prognostic significance of stiffness index determined by digital volume pulse method in polycystic kidney disease]

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

[Introduction: It is known from previous studies, that in chronic renal failure cardiovascular mortality and morbidity are more frequent than in the general population. The prognostic significance of arterial stiffness on cardiovascular outcomes trials was first demonstrated in end-stage renal disease patients by epidemiological longitudinal studies. Our aim was to assess the prognostic significance of arterial stiffness in polycystic kidney disease. Methods: 55 patients with polycystic kidney disease (PKD) were examined and followed in our clinic. Pulse wave velocity was determined by digital volume pulse (DVP) method, and a so-called stiffness index (SI DVP) was calculated. MDRD formula was used for estimating the glomerular filtration rate (eGFR, mL/min/1.73 m2) to determine renal function. Patients were observed regularly, in every 3-6 months, and we checked lab tests, which assessed the patient’s renal function and cardiovascular events occurred in patients were collected in our outpatient department. Results: Our study involved 55 patients, 21 were male, the mean age was 45±12 years. The average follow-up was 63±32 months. The average value of the stiffness index was 11.11±2.22 m/s. We divided the patients by 11 m/s as cut off point of SI values into two groups and analysed their outcome. In the increased arterial stiffness group (SI >11 m / s) the probability of the combined endpoint occurrence was signi - fi cantly higher than in the group with flexible arteries (χ-square: 4.571; p=0.033). Between the two groups we did not found significant difference in cardiovascular endpoint, but we found a statistically significant difference between the two groups in renal outcomes (χ-square: 5.591; p=0.018). Conclusion: In polycystic kidney disease the increased arterial stiffness may predict the onset of end-stage renal failure. Digital Pulse volume as determined by Pulse Trace system appears an appropriate method for making prognosis in chronic kidney disease.]

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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. ]

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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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[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.]