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Hungarian Radiology

DECEMBER 10, 2005

[Ultrasound of the acute pediatric scrotum]

COLEY D. Brian

[Proper evaluation of the acute scrotum, like any condition, starts with a history and physical examination by an experienced clinician. Often this is all that is needed to arrive at an accurate diagnosis, which then allows prompt and appropriate treatment. However, the true nature of the underlying disease producing scrotal pain is not always clear, and the consequences of error (testicular loss) are undesirable. Ultrasound is the single most useful imaging tool for imaging the scrotum. While nuclear medicine studies can help assess blood flow, the combination of anatomic detail provided by modern ultrasound equipment and the ability to assess blood flow and perfusion with color Doppler makes ultrasound invaluable. Properly performed and interpreted, ultrasound provides very high sensitivity and specificity for acute scrotal conditions. Understanding of the conditions that produce acute scrotal pain in children will improve one’s diagnostic abilities. The most important diagnosis to consider is testicular torsion, since untreated this will result in testicular death. While testicular torsion can occur at any age, it is most common in the perinatal and peripubertal age groups. Torsion of a testicular appendage is a frequent cause of scrotal pain in prepubertal males. The sonographic findings can mimic epididymitis, but diligent and focused sonographic examination can make the diagnosis. Epididymitis typically affects postpubertal males, but can be seen in younger patients with functional or anatomic urinary tract anomalies. Sonographic evaluation of the post-traumatic painful scrotum can help to differentiate injuries that can be managed conservatively and those that require surgery. Less common causes of scrotal pain include hernias and hydroceles, vasculitis, and idiopathic edema. Understanding the characteristic sonographic features of these conditions allows the examining physician to make more accurate and confident diagnoses. It is hoped that this review article will help to promote this understanding.]

Lege Artis Medicinae

JULY 20, 2006

[WEGENER’S GRANULOMATOSIS PRESENTING AS MASTOIDITIS: A DIAGNOSTIC CHALLENGE]

NAGY Pál, Z.SZABÓ László, DOMJÁN Gyula, GADÓ Klára, BALOGH Károly

[INTRODUCTION - Wegener's granulomatosis has an uncertain pathomechanism, but is probably autoimmune in origin. In typical cases the mucosa of the nose, paranasal sinuses and of the lower respiratory tract, as well as the lungs and the kidneys are affected. Patients present with sinusitis, recurrent pneumonia or renal disease associated with microhaematuria, pyuria or azotaemia. Fever, polyarthralgia or polyarthritis may also occur. The underlying pathologic changes are necrotizing vasculitis, granulomas and parenchymal necrosis. The diagnosis is based on a combination of the clinical picture, microscopic findings and immunofluorescent demonstration of cANCA. CASE REPORT - A 27-year-old woman presented with symptoms of unilateral mastoiditis. In the following 7 months she underwent 7 operations in 4 hospitals for a locally progressive, destructive process of uncertain etiology showing a septic course. The clinical picture was not specific, the cANCA test was not definitive, and the histologic findings were initially interpreted as nonspecific inflammation. Repeated biopsies, multiple reviews of the microscopic specimens, consultations, differential diagnostic considerations, and, finally, the success of the treatment with corticosteroids and cyclophosphamide led to the diagnosis of Wegener’s granulomatosis. Currently the patient has been in remission for 32 months. CONCLUSION - The definitive diagnosis of Wegener’s granulomatosis, particularly of its localized or limited form, may be problematic despite well-defined diagnostic criteria. Setting up the correct diagnosis may take months or years. In case of unusual respiratory or otological symptoms, and in view of ineffective medical or surgical treatment, Wegener’s granulomatosis has to be considered. An atypical clinical picture, inconclusive histologic, radiologic and laboratory findings warrant the need for close collaboration of various specialists. This is particularly important since state-of-the-art therapy of Wegener’s granulomatosis promises a favourable prognosis.]

Lege Artis Medicinae

SEPTEMBER 10, 2001

[Wegener’s granulomatosis: diagnostic questions]

CSISZÉR Eszter, SOLTÉSZ Ibolya, FÜZESI Katalin

[INTRODUCTION - Wegener’s granulomatosis is a disease of unknown origin classified as an immune vasculitis. The main pathologic feature is necrotising granulomatous small vessel vasculitis. Clinically the upper and/or lower airways are affected most often as well as the kidneys in the generalised form. The highly specific antineutrophil cytoplasmic antibodies have diagnostic significance beside the pathology and clinical symptoms. PATIENTS AND METHODS - We have studied the clinical and diagnostic characteristics of 15 cases diagnosed in our centre from the last 25 years. All patients had pulmonary manifestation. We analysed the organ involvement, clinical signs indicating the diagnosis, chest radiography, chances for biopsies of diagnostic significance and the difficulties of differential diagnostics. RESULTS - The diagnosis was made based on histological samples from the upper airways in three cases and from the lower airways in six cases (in four cases the bronchoscopic biopsies were diagnostic, in two patients thoracotomy was necessary). In six cases, clinical signs and typical organ manifestations were the basis of the diagnosis. CONCLUSION - This very rarely occurring disease which is difficult to recognise requires pulmonological examination due to the involvement of lungs. If this diagnosis comes to mind - based on findings of bilateral, multiplex, round pulmonal shadows with cavitation - the pulmonologist should search for other organ manifestations and involve respective specialists. Ideally, diagnosis is established by histopathology or by positive cytoplasmic antineutrophil cytoplasmic antibodies.]

Clinical Neuroscience

MAY 10, 2005

[A case history and diagnostical rewiev of primary cerebral angiitis]

CSÉPÁNY Tünde, KOLLÁR József, SIKULA Judit, MOLNÁR Mária, CSIBA László

[The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.]

Clinical Neuroscience

SEPTEMBER 30, 2007

[SIMULTANEOUS CENTRAL AND PERIPHERAL NERVOUS SYSTEM INVOLVEMENT IN SYSTEMIC LUPUS ERYTHEMATOSUS]

ILNICZKY Sándor, KAMONDI Anita, ARÁNYI Zsuzsanna, VÁRALLYAY György, GAAL Barbara, SZIRMAI Imre, NAGY György

[Systemic lupus erythematosus is a frequent autoimmune disease, affecting several organs, including the brain, spinal cord and nerves. Cerebral vasculitis, transverse myelitis and polyneuropathy are the most common neurological manifestations. We report a case of a 46 years old woman who suffered incomplete transverse myelitis in her age of 44. After 2 years the second relapse presented with arthralgias, painful paraesthesias and weakness of the lower limbs. Neurological signs suggested involvement of the central and the peripheral nervous system. Based upon clinical and laboratory findings systemic lupus erythematosus was diagnosed. Magnetic resonance imaging revealed two hyperintense lesions on T2 weighted scans within the cervical spinal cord. The brain scan was normal. Protein content was slightly elevated in the cerebrospinal fluid, with normal cell count. Electrophysiological examinations diagnosed a subacute sensory-motor axonal polyneuropathy. On methylprednisolone treatment her condition improved. Simultaneous development of central and peripheral lesions of the nervous system in cases with systemic lupus erythematosus may lead to a challenge to establish the diagnosis.]

Lege Artis Medicinae

MARCH 21, 2009

[SEVERE NECROTISING PRIMARY VASCULITIS]

GRIGER Zoltán, SZÁNTÓ Antónia, SZABÓ Nóra Anna, SOLTÉSZ Pál, ZEHER Margit

[INTRODUCTION - Vasculitis is an inflammatory disorder of the vessels, often associates with necrosis. The primary (idiopathic) form caused by distinct immunological mechanisms, the secondary form caused by infection, tumor, drugs or systemic autoimmune disease. CASE REPORT - 57-year-old smoking female patient with a medical history of the left ankle fracture. Four days earlier had sore throat, at admission was febrile and generally weak and had severe pain in her lower extremities. Physical examination revealed numerous livid alterations in both lower extremities, necrotic change of some toes and new onset of diastolic hypertension. Elevated serum transaminase levels, severe thrombopenia, anemia, leukocytosis suggested the possibility of thrombotic thrombocytopenic purpura and vasculitis. Tests ruled out septic embolism, therefore we applied high dose steroid treatment and plasma exchange. Meanwhile the results fulfilled the classification criteria of polyarteritis nodosa (vasculitis, livedo reticularis, polyneuropathia and new onset of diastolic hypertension), accordingly we synchronized the treatment with fractionally administered intravenous cyclophosphamide. The process revealed responsive to therapy and the substantive function of the legs remained after surgical removal of the necrotic areas. CONCLUSIONS - Early rapid diagnosis of vasculitis is fundamental and we emphasize the significance of the efficient treatment.]

Lege Artis Medicinae

JULY 14, 2008

[IMMUNE-MEDIATED NEUROLOGICAL DISORDERS]

CSÉPÁNY Tünde

[Multiple sclerosis, myasthenia gravis and chronic inflammatory neuropathies share the common feature of chronic course with potential development of disability due to the damage caused by immunological processes. Early detection and precise diagnosis is very important, because most patients respond well to proper immunomodulatory treatment. The diagnosis requires extensive knowledge of the disease and is based on the clinical symptoms recognised by the GP, as well as on complex assessment of the results of special neurophysiological, radiological and laboratory examinations. The present paper reviews the major immune-mediated neurological disorders and discusses their targeted immunological treatment.]

Lege Artis Medicinae

DECEMBER 20, 2004

[LETHAL SEPTIC INFECTION IN RHEUMATOID ARTHRITIS]

BÉLY Miklós, APÁTHY Ágnes

[INTRODUCTION - The lethal septic infection was studied in a randomized (non-selected) autopsy population of 234 in-patients with rheumatoid arthritis. The patients died at the National Institute of Rheumatology between 1970 and 1999. PATIENTS AND METHOD - The aims of this study were to determine the prevalence of lethal septic infection with or without purulent arthritis in rheumatoid arthritis; the relationship between purulent arthritis and lethal septic infection; the clinically missed diagnosis of lethal septic infection and purulent arthritis; the influence of the main complications and associated diseases on lethal septic infection with and without purulent arthritis; the pathogens in lethal septic infection; and the clinico-laboratory parameters associated with lethal septic infection in rheumatoid arthritis. RESULTS - Lethal septic infection was found in 31 (13.24%) of 234 rheumatoid arthritis patients. Purulent arthritis complicated lethal septic infection in 15 (6.4%) of 31 patients. There was a significant association between lethal septic infection and purulent arthritis. Sepsis was detected clinically in 17 of 31 lethal cases and purulent arthritis was detected 9 of 15 septic infection complicated with suppurative articular processes. The coexistent complications (systemic vasculitis, AA amyloidosis), and associated diseases (tuberculosis with or without miliary dissemination, malignant tumors, diabetes mellitus) did not influence the prevalence of septic infection. The most frequent pathogenic agents were: Staphylococcus aureus, Escherichia coli, Proteus mirabilis, Pseudomonas aeruginosa, Klebsiella species, Streptococcus species. The patients with septic infection had significantly lower levels of beta-globulin, and higher values of Waaler-Rose or latex fixation test in comparison to patients without septic infection or without complications. CONCLUSION - Lethal septic infection may exist in rheumatoid arthritis without the classical clinical symptoms of sepsis, and clinically latent suppurative processes may be found at autopsy. The missed clinical diagnosis of a fatal complication is explained by the weak immune response and atypical clinical symptoms of elderly patients mainly treated with steroids and immunosuppressive drugs.]

Hungarian Immunology

MARCH 20, 2006

[Paraneoplastic diseases of the locomotor system]

ANDRÁS Csilla, SZÁNTÓ János, SZEKANECZ Zoltán, CSIKI Zoltán, ILLÉS Árpád, SZEKANECZ Éva, DANKÓ Katalin

[Paraneoplasias in rheumatology can be present in different forms of arthropathies, myopathies. In addition, we often see atypical forms of systemic autoimmune diseases. Vasculitis is mainly associated with lymphoproliferative diseases. The direct invasion of bones and joints is not present in paraneoplasia, as this entity is a collection of symptoms generated by the tumour itself by producing biologic mediators, hormones, peptides, antibodies, cytotoxic lymphocytes, autocrine and paracrine mediators. Recognition of paraneoplasia is of outmost importance because it attires attention to the presence of tumour in the organism and thus enables us for early treatment of the malignancy. Monitoring of the severity of paraneoplastic symptoms serves as a marker for determination of efficacy of the targeted oncological therapy. On the other side, because these severe symptoms affect the patient's quality of life and can lead even to death their in time recognition and treatment is extremely important.]

Lege Artis Medicinae

APRIL 22, 2011

[IgG4-related disease]

ZEHER Margit

[IgG-4-related disease is a clinical entity characterised by significant elevation in serum IgG-4 levels, infiltration of IgG-4+ plasma cells into the involved tissues, enhanced fibrosis, and good therapeutic response to corticosteroids. The IgG-4 associated disease mostly affects two organs. The salivary and lacrimal gland enlargement and inflammation is known as Mikulicz’s disease, which had been previously known as a subtype of Sjögren’s syndrome for a long time. The other commonly involved organ is the pancreas, in which a special form of chronic pancreatitis, namely autoimmune pancreatitis develops. IgG-4 associated disease as a separate disease has been suggested by Japanese authors. Previously published data support the common pathogenesis of autoimmune pancreatitis and Mikulicz’s disease. Besides these two manifestations, similar histological lesions and elevated IgG-4 levels have been demonstrated in many other organs. At present, it is not clearly demonstrated whether IgG-4 syndrome is an autoimmune disorder, and we do not know the exact reason of the elevated IgG-4 levels in patients with this syndrome. IgG-4 is a regulatory immunoglobulin, the main function of which is to decelerate immune responses, thus its pathologic role in tissue destruction is difficult to explain. It is not clear either, whether IgG-4 syndrome is indeed a single disease, or only an example of overlapping symptoms of various diseases. On the basis of the characteristic histological lesions in various organs, IgG-4 syndrome is similar to multi-organ diseases, such as sarcoidosis or vasculitis.]