Hungarian Radiology

[Acromesomelic dysplasia du Pan]

ALI Al Kaissi, MAHER Ben Ghachem, FARID Ben Chehida, KAZIMIERZ Kozlowski

OCTOBER 10, 2005

Hungarian Radiology - 2005;79(05)

[INTRODUCTION - Cartilage derived morphogenic protein (CDMP1) mutations account for several related disorders, ranging from prenatal lethal to very mild entities such as brachydactyly C. Two similar severe manifestations of CDMP1 mutations are du Pan and Hunter-Thompson syndromes. CASE REPORTS - We report two second degree relatives with du Pan syndrome. Clinical history and full skeletal surveys were analysed and compared with the data from the literature. Frequent spontaneous abortions - probably manifestation of the lethal forms of CDMP1 mutations - were present in both families. Skeletal surveys of the patients showed similar acromesomelic abnormalities consistent with du Pan syndrome. CONCLUSION - The rare publications of du Pan syndrome present usually insufficient radiographic documentation. Better radiographic imaging is necessary to establish clear-cut criteria of differentiation between du Pan and Hunter-Thompson syndrome.]



Further articles in this publication

Hungarian Radiology

[Thoughts on PET-CT]


Hungarian Radiology

[The role of MRI in the clinical examination following breast cancer screening]


[INTRODUCTION - Breast cancer screening was performed in 27 325 female patients at the National Institute of Oncology from 1st of January 2002 to May 30th of 2005. Complementary examinations were necessary in 1876 women. MR-mammography was performed in 65 of these cases. We were curious about in which cases MR mammography helps to make the diagnosis more accurate, how does it influence the therapy. We also studied, whether the number of surgical interventions because of benign breast lesions decreases due to MR mammography. PATIENTS AND METHODS - In 65 patients MR mammography was performed using non-contrast axial and coronal T1W and STIR sequences. After the injection of gadolinium four series of 3D FLASH (fast low angle shot) dynamic gradiens echo sequences were also applied. Subtraction of the non-contrast and contrast enhanced series were evaluated in addition to the intensity curves of the postcontrast series. RESULTS - MR mammography helped to evaluate dense breasts in 21 cases, to identify multifocal lesions in 6 cases and to differentiate the malignant-benign processes. In the course of the 65 post-screening examinations, malignant processes [BI-RADS IV-V (Breast Imaging Reporting and Data System)] were diagnosed in 21 cases, benign processes (BI-RADS II-III) or negative results were found in 44 patients. CONCLUSION - MR mammography increased diagnostic accuracy, decreased the number of benign lesion-related surgical procedures and increased the accuracy in determining surgical radicality and establishing a therapeutic plan.]

Hungarian Radiology

[Board Meeting of the Hungarian College of Radiology]

PALKÓ András, FORRAI Gábor

Hungarian Radiology

[Quality cost in radiology: the cost of repeated examinations]

KIS Zsuzsanna

[INTRODUCTION - The aim of the author is to describe the definition and types of quality cost in the health care services especially in the field of radiology. The proportion of the quality cost is based on the author's data and data from the literature. The ways of reduction of the quality cost is also discussed. MATERIAL AND METHODS - The author made a research based on a prior multicentric study to determine the loss derived from the excessive use of films used during the repeated radiological examination. The cost of wrong services nationwide is calculated on the basis of the loss per 1000 German point. RESULTS - Our short research showed that 300 000 Ft HUF + VAT per year was paid because of the excessive use of the films during the repeated examination points within the given period of time. The loss percentage per 1000 German points can be calculated based on the points generated during a given time. In this way there was more than 300 million HUF spent on defective services nationwide in 2002. CONCLUSION - The cost of defective services and resulting moral and financial losses justify the need for finding and reducing the costs. Quality control and quality improvement can be used to achieve the aims of controlling the processes by the right indicators. By discussing them the processes will improve, the costs will be lower and quality also improves. The patients and insurance companies who buy our services also have the same expectations.]

Hungarian Radiology



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Clinical Neuroscience

The role of the insula in the parieto-frontomedial epileptic network. Clues from successful surgical treatment

BALOGH Attila, AIMEN Anwar, KELEMEN Anna, ERÕSS Loránd, FABÓ Dániel

We present a case of MRI negative SMA seizure with the seizure onset zone in the secondary leg area on the superior bank of the Sylvian fissure, localized with multiscale electro-clinical and neuroradiological examinations. The 34-year-old female patient’s intractable epilepsy started at age 14. She had diffuse pain aura in her left leg followed by tonic posturing with fully preserved consciousness suggesting parieto-fronto-medial seizure propagation. Her daily nocturnal SMA seizures became drug-resistant. Multiple 3T MRI images and neuropsychological evaluations were normal. Interictal PET detected a right parietal and insular FDG hypometabolism. The seizure onset zone and the symptomatogenic zone were localized by invasive electrophysiology. The insular deep electrode showed the propagation of ictal activity with an onset in the secondary sensory leg area through the insula to the fronto-medial surface. Eighteen spontaneous seizures, electrical cortical stimulation and cortical mapping confirmed the designated area of the resection, which was later proved macroscopically abnormal during surgery. The histological and immunohistological workup confirmed focal cortical dysplasia (IIb type). Postoperative postprocessing morphometry of the preoperative MRI study confirmed the lesion in the right inferior parietal lobe. The patient remained seizure free after surgery for more than 4 years, and medication free for the last two years. Our results concluded that the insula has a „relay” or „node” function in the parieto-opercular-fronto-medial epileptic network. The insular functional connectivity predisposed frontal propagation of the epileptic activity in the connectome of her epilepsy. The three-way insular structural connectivity has determining function on the seizure propagation.

Hungarian Radiology

[Osteogenesis imperfecta and spondylo-costal dysplasia in a male child]


[INTRODUCTION - Co-existence of osteogenesis imperfecta with another bone dysplasia is rare. That of osteogenesis imperfecta and spondylo-costal dysplasia has not yet been reported. CASE REPORT - The authors present a 13 year-old boy. He was disproportionate with shortening of the upper segment, and the upper and lower extremities, and he had dysmorphic face. His parents were healthy. Radiographs revealed osteogenesis imperfecta and spondylocostal dysplasia. CONCLUSION - Patients with two bone dysplasias have a much worse prognosis. Superimposition of any skeletal dysplasia on abnormal osteogenesis enhances the disposition to deformities and fractures.]

Hypertension and nephrology

[Thought about renovascular hypertension by a special case report]

GAJDÁN Nikolett, LÉGRÁDY Péter, BAJCSI Dóra, MORVAY Zita, NAGY Endre, LETOHA Annamária, KYPROS Constantinou, FEJES Imola, SONKODI Sándor, ÁBRAHÁM György

[Renovascular hypertension is a well-known form of secunder hypertension. Two thirds of cases are caused by atherosclerotic plaque and one third are caused by fibromuscular dysplasia. The prevalence of it is less than 1%. Digital subtraction angiography is considered the goldstandard diagnostic method. The 58-year old female patient was hospitalized with resistant hypertension. Duplex ultrasonography showed fibromuscular stenosis the in left renal artery. Percutaneous transluminal angioplasty and stenting were performed. Her blood pressure normalized. The patient did not attend the control examinations. Next time in 2001, she was referred to our emergency department with increased blood pressure of 210/140 mmHg. Following control ultrasonography angiography showed total occlusion of the left renal artery and significant stenosis of the right renal artery. Left nephrectomy was necessary due to shrunken kidney and dilatation and stenting of the right renal artery. The blood pressure normalized again. Since 2004 until 2014 despite of the regular visits, we detected in stent restenosis of the right renal artery almost in each year. Even so, renal function was preserved all the time. In autumn of 2014, the patient suffered severe stroke, and few months later at the age of 74 she died. There are many open questions to discus concerning the right treatment of renovascular hypertension yet. Even so by performing 12 intravascular interventions we could ensure her acceptable quality of life for 16 years.]

Lege Artis Medicinae

[Can we fight with multi- and pan-resistant bacteriums?]


Clinical Neuroscience

[Surgically cured resistant epilepsy - caused by hemispherical dysgenesis - Case report]

HEGYI Márta, SIEGLER Zsuzsa, BARSI Péter, RUDAS Gábor, LENGYEL Zsolt, SZAKÁLL Szabolcs, BOGNÁR László, KOZÁK Lajos Rudolf, NEUWIRTH Magdolna, FOGARASI András

[A part of patients with the therapy resistant epilepsy can be cured by surgical interventions. The more concordant the presurgical evaluation data, the better prognosis the patient has postoperatively. In case of discordant examination data, multimodal evaluation or case-specific decision might be successful. We report on a five-year-old boy with bilateral (left-dominated) cortical dysplasia and therapy resistant epilepsy. The ictal EEG did not help to localize the seizure onset zone, semiology had little lateralization value; however, FDG-PET showed left hemispherial hypermetabolism. The child became almost seizure-free and showed improved development after left-sided hemispherotomy.]