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Hypertension and nephrology

APRIL 24, 2020

[Polycystic kidney]

DOLGOS Szilveszter, TÁRNOKI Ádám Domonkos, TÁRNOKI Dávid László

[The most common monogenic nephropathy is a congenital, cystic, bulky process in the kidney that leads to a gradual deterioration in renal function. Renal failure is often associated with cystic liver or pancreatic lesions, cerebral artery aneurysm, or mitral prolapse.]

Hypertension and nephrology

APRIL 24, 2020

[Possibilities and limitations. Dietary difficulties of chronic renal failure in childhood]

REUSZ György, SZABÓ Adrienn

[In chronic kidney disease (CKD), the role of the kidney in assuring homeostasis is gradually deteriorating. Besides fluid, electrolyte and hormonal disturbances, detoxification and control of blood pressure is insufficient without external help. In children, in addition to achieving equilibrium it is also essential to ensure optimal physical and cognitive/psychological development. Adequate calorie intake is a major determinant of growth during infancy. Among the therapeutic options it is essential to ensure a proper diet. In addition to reflecting the special needs of renal failure in its composition, it must be palatable for the child. Children with kidney disease should have a normal energy diet. Protein intake should not be reduced from the baseline recommendation, but lower phosphorus and high bioavailability should be preferred. A low sodium and potassium diet is recommended for a significant proportion of patients and is based on dietary advice. Further, diet planning may be problematic if the child has special dietary requirements and is in need of nasogastric tube feeding. Because diet planning is a complex task, it is difficult to achieve optimal protein supply and mineral restriction along with high energy intake. In such cases, enteral nutrition with special formulas/ drinks developed for pediatric nutrition may provide a solution.]

Clinical Neuroscience

NOVEMBER 30, 2019

[Early experience with CyberKnife treatment in case of intra-, suprasellar hypernephroma metastasis]

SIPOS László, BAJCSAY András, KONTRA Gábor, CZIRJÁK Sándor, JÁNVÁRY Levente, FEDORCSÁK Imre, POLGÁR Csaba

[Among tumours found in the suprasellar region metastases are very rare and the most frequent primary tumours are lung and breast cancer. Data of a patient with clear cell renal carcinoma with intra-suprasellar metastasis will be discussed. As in most of the tumours in the sellar region, the first symptom was visual deterioration with visual field defect. A transsphenoidal debulking of the tumour was performed and the residual tumor was treated by CyberKnife hypofractionated stereotactic radiotherapy. Both our patient’s visual acuity and visual field impairment improved after the surgery and CyberKnife treatment. At 6-month after irradiation, MR of the sella showed a complete remission of the tumour. This was the first treatment with CyberKnife in our country in case of a tumour close to the optic chiasm. According to our best knowledge, there are 21 cases in the literature with renal cell carcinoma metastasis in the suprasellar region.]

Hypertension and nephrology

SEPTEMBER 10, 2019

[Kidney diet and the patient compliance issue ]

LADÁNYI Erzsébet

[There have been significant developments in the field of nephrology and dialysis as for the science and technology are concerned in the past decades. However, CKD patients still show high mortality and morbidity. From among the several factors determining the long-term outcome of CKD patients metabolic disorder and malnutrition play an important role. Malnutrition is often not diagnosed or is not paid enough attention to in the complex treatment of CKD patients. It is important to make both the patients and clinical staff more aware of proper nutrition and importance of prevention and treatment of malnutrition, respectively. The early diagnosis and treatment of malnutrition is of utmost importance in CKD patients. The long term renal failure and the accompanying malnutrition have a negative impact on their long term outcome and quality of life. Since the malnutrition causes a lot of complications, it is indispensable for dietitians and nephrologists to work closely together. Patient compliance is a determining factor in the successful implementation of renal diet.]

Lege Artis Medicinae

MAY 20, 2019

[Notes on the management of hypertension in chronic kidney disease ]

AMBRUS Csaba

[The prevalence of hypertension among pa­tients with chronic kidney disease is high, reaching more than 80%. Hypertension is both one of the main causes and also the most common consequence of chronic kidney disease. It is also a main factor responsible for the high cardiovascular morbidity and mortality in this patient population. Blood pressure control can improve patient outcomes, lower cardiovascular risk and slow down the progression of kidney dis­ease, irrespective of the underlying cause. The optimal therapy should therefore focus not only on blood pressure reduction but also on renoprotection. Basic understanding of the renal pathophysiology in hypertension and renal effects of various medications is of paramount importance. In this review, we summarized cornerstones of the antihypertensive therapy in patients with chronic kidney disease. The management of patients receiving kidney replacement therapies, such as hemodialysis, peritoneal dialysis or transplanta­tion requires special knowledge and expe­rience, therefore it is not discussed here. The aim of this review was to allow non-nephrologist physicians to take care of their kidney patients with more confidence and effectiveness.]

Hypertension and nephrology

MAY 10, 2019

[Diabetology in dialysis]

MÁCSAI Emília, HALMAI Richárd, NEMERE Éva, BRASNYÓ Pál, KISS István

[According to epidemiological data, the number of diabetic patients requiring dialysis is increasing. Burnt-out diabetes, new onset diabetes during chronic dialysis treatment and new onset diabetes after transplantation diabetes are new types of diabetes compared to the traditional division forms. It is utmost important to evaluate education ability and acceptance the core values of lifestyle changes. Clear guidelines for oral anti-diabetic and insulin therapy have not yet been developed since this group of patients did not participate in previous major surveys. In order to formulate individualized therapeutic recommendations, it is imperative to perform regular glucose self-monitoring, which is also the cornerstone of solving unexpected situations. Both in hemodialysis and peritoneal dialysis, special considerations should be applied to the diabetic patient group, this review focuses on the current understanding of available relevant knowledge and summarizes presumably extrarenal diabetic complications as well.]

Hypertension and nephrology

DECEMBER 10, 2018

[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.]

Lege Artis Medicinae

SEPTEMBER 20, 2018

[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]

BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna

[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion bet­ween experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]

Lege Artis Medicinae

JUNE 20, 2018

[Diagnosis of interstitial lung diseases]

MÜLLER Veronika

[Recognition and diagnosis of interstitial lung diseases (ILD) is one of the most challenging tasks of respiratory medicine. In the wide range of different ILD-s idiopathic pulmonary fibrosis (IPF) is one of the most common. Improvements in the diagnosis made the recognition of IPF easier. Typical clinical features and usual interstitial pneumonia pattern on high resolution CT are now enough for the confident diagnosis of IPF in the multidisciplinary ILD-team discussions. Bronchoscopic cryobiopsy is a great achievement of histological sampling as this can give appropriate size tissue for histological analysis making surgical intervention rare. As IPF cannot be cured early diagnosis is crucial. Today available treatments can only slow down progression of the disease, so early stages and better clinical condition of the patients are essential for therapeutic success. Follow-up of IPF patients includes complex lung functional measurements in dedicated centers in Hungary. Former studies confirmed forced vital capacity (FVC) decline as an important measure of disease progression and mortality. Available IPF treatments decrease FVC decline and can prevent the mostly deadly acute exacerbations of the disease. Additionally palliative treatments including supplementary oxygen and in selected patients lung transplantation can be offered. This is the first of a series of three articles about IPF. ]

Clinical Neuroscience

MARCH 30, 2021

[Cause of recurrent rhabdomyolysis, carnitine palmitoyltransferase II deficiency and novel pathogenic mutation ]

ÇAKAR Emel Nafiye, GÖR Zeynep, YEŞIL Gözde

[Carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal inherited metabolic disorder in which the β-oxidation of the long chain fatty acids is defective. The clinical presentation may be in various forms; it presents itself in the severe form during neonatal and infantile periods and as the less severe myopathic form in the school age and adolescence. While the severity of the rhabdomyolysis attacks varies, occasionally the clinical course may be complicated with acute renal failure. Acylcarnitine analysis may help in the diagnosis of CPT II, but its normality does not indicate the absence of the disease. If there is strong suspicion, genetic analysis should be performed on the cases. In this article, we present a 15-year-old male patient who had two rhabdomyolysis attacks triggered by infection and starvation. Acylcarnitine analysis of the case was normal, CPT II deficiency was considered when the history was evaluated, and CPT II gene c.137A>G (p.Gln46Arg) homozygous novel pathogenic mutation was detected. CPT II deficiency is one of the most common causes of metabolic rhabdomyolysis in patients with recurrent episodes of rhabdomyolysis.]