Hypertension and nephrology

[Article Reports]

VÁLYI Péter

JUNE 20, 2016

Hypertension and nephrology - 2016;20(03)

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Hypertension and nephrology

[Sclerosis tuberculosa. Newer aspects of therapy and care]

REUSZ György, DEÁK-PÁL Ákos, REMPORT Ádám

[Tuberous sclerosis (TSC) is an autosomal dominant disease characterized by the development of hamartomas in various organs with variable frequency. The major organs involved are the skin (70%), the brain (75%), the heart (50%) and the kidney (80%). Life threatening acute and chronic events are due initially to cardiac involvement, later neurologic complications and after the second and third decade nephrological and pulmonological involvement may predominate. Skin and osteo - skeletal disease may lead to disfiguring appearance causing additional psychological burden to the patient. Uninhibited activation of the mTOR signaling pathway is at the center of the disease process. The discovery of the pathomechanism established the base of the up-to-date medical therapy presenting a real alternative to the classical supportive and surgical treatment. Due to the progressive nature of the disease early recognition, best standard care and treatment according to the disease stage is of crucial importance. Optimal therapy is based on internationally recognized guide-lines and should be controlled through specialized, multidisciplinary centers.]

Hypertension and nephrology

[Overview of changes in the use of the cardiovascular system (ATC code based) products based on National Health Insurance data]

GYURCSÁNYI András, BARNA István

[Use of cardiovascular products supported by the National Health Insurance in December 2015 exceeded more than 5% the data of December 2007. This is a significant change, because simultaneously the amount paid as a support decreased by 41.1%. Change in the categories of support resulted in an increase in the use of generics. In addition, the expansion of fixed combinations also brought a significant change in drug consumption. The role of products effecting on the reninangiotensin system, lowering serum lipid modifying agents, diuretics, β-receptor blockers, centrally acting agents and vasodilators in the treatment of cardiovascular diseases has increased as well.]

Hypertension and nephrology

[The prevalence of type 2 diabetes mellitus in the Hungarian population with hypertension]

KÉKES Ede, PÁL László, SCHANBERG Zsolt, KISS István

[Authors had found diabetes mellitus type 2 in 30% of 38 886 hypertensive patients (stadium I-III). Diabetes was more frequent in case of women under 30 years. Subsequently all age groups (from 40 to 80 years) incidence was more frequently (p<0.01-0.001) in men, above 80 years again a higher ratio was in women. Presence of diabetes was correlated to rate of BMI value and systolic, diastolic pressure as well. In women - above 140 mmHg systolic pressure - the elevation was exponential. We have found a significant correlation between fasting glucose and waist. Reaching the target blood pressure is not a simply task in hypertensive patients with diabetes. The 140/90 mmHg was reached in 34.2%, 90 mmHg diastolic blood pressure in 62.3%, but the required 80 mmHg only in 16.4% of cases. Achieve the target value was quite different in the different region of our country. The major cardiovascular complications (stroke, renal disease, myocardial infarction, peripheral artery disease) have suffered a higher rate in the hypertensives with diabetes compered to hypertensives without diabetes.]

Hypertension and nephrology

[Success Evaluation of Antihypertensive Treatment in Practice]

KÉKES Ede, KISS István

Hypertension and nephrology

[Presentation of Health Expenditures Over the World]

KÉKES Ede

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Clinical Neuroscience

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.

Clinical Neuroscience

[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.

Clinical Neuroscience

[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.]