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The present study aims to discuss two successful identification cases of the search warrants on unknown bodies by the Baranya County Police Department. In both cases, identification was only possible by using lot numbers of the traumatological metal implants removed during exhumation, several years after the bodies had been discovered and the post-mortem investigation was completed. We hope that the cases provided will highlight the importance of secondary identifiers, specifically the lot numbers of medical implants, in forensic identification practice. We would also like to draw attention to the fact that the only way to identify the majority of the over a thousand unknown corpses in Hungary (742 of them have been under warrant for more than ten years) is to re-examine the corpses using recent years' results in technical and technological improvements. The presented cases highlight the importance of documenting the identification numbers of implanted surgical devices during autopsies. Orv Hetil. 2023; 164(23): 911-918.

Összefoglaló. Bevezetés: A Kawasaki-szindróma immunvasculitis, amely kezeletlenül kardiológiai szövődményekhez vezethet. A korai intravénás immunglobulin-terápia mérsékli a szövődményeket, de az esetek 10-20%-a rezisztens a kezelésre. Ennek előrejelzésére világszerte számos rizikóbecslő pontrendszert használnak. Célkitűzés: A Kobayashi- és a Kawanet-pontrendszer prediktív értékének vizsgálata betegeink intravénás immunglobulin-rezisztenciája és kardiológiai szövődményei vonatkozásában. Tudomásunk szerint ez az első magyarországi vizsgálat, amely Kawasaki-szindróma esetében pontrendszerek prediktív értékét méri fel. Módszer: Retrospektív pilotvizsgálatunkban kigyűjtöttük a 2005. január és 2020. április között Kawasaki-szindróma miatt ápolt betegeink adatait. Mindegyiküknél Kobayashi-, illetve Kawanet-pontot számoltunk, valamint megvizsgáltuk azok specificitását, szenzitivitását az intravénás immunglobulin-rezisztencia, illetve a kardiológiai szövődmények előrejelzése szempontjából. A Kobayashi-pontrendszerben 4, a Kawanet-pontrendszerben pedig 2 pont vagy annál magasabb érték jelez rizikót. Eredmények: Kawasaki-szindrómát 28 gyereknél véleményeztünk, 13 esetben észleltünk mérsékelt, 4 esetben súlyos szövődményt. 4 betegünk bizonyult intravénás immunglobulinra rezisztensnek. A rezisztencia szempontjából a Kobayashi-pontrendszer alacsony szenzitivitást (25%), illetve magas specificitást (91,6%), míg a Kawanet-pontrendszer mérsékelt szenzitivitást (50%) és specificitást (50%) mutatott. A szövődmények szempontjából hasonló eredményeket kaptunk, Kobayashi-pontrendszer: szenzitivitás: 17%; specificitás: 100%, illetve Kawanet-pontrendszer: szenzitivitás: 47%; specificitás: 45%. Következtetés: A legtöbb, nem ázsiai országban készült tanulmányhoz hasonlóan az intravénás immunglobulin-rezisztencia előrejelzésében a Kobayashi-pontrendszer vizsgálatunkban sem bizonyult hatékonynak. Ezzel szemben, magasabb szenzitivitása miatt, a Kawanet-pontrendszer intravénás immunglobulin-rezisztenciát előre jelző hatékonyságát érdemes lenne nagyobb esetszámban vizsgálni a hazai populációban is. A kardiológiai szövődmények előrejelzésére egyik pontrendszer sem bizonyult alkalmasnak. Orv Hetil. 2021; 162(47): 1885-1890., Introduction: Kawasaki disease is an immunovasculitis, which, without treatment, leads to cardiac complications. Early intravenous immunoglobulin therapy moderates complications, however, 10-20% of patients are resistant to the therapy. Numerous risk score systems are used worldwide to predict this., Objective: To assess the predictive value of the Kobayashi and Kawanet score systems regarding intravenous immunoglobulin resistance and cardiac complications in our department's patient cohort. To our best knowledge, this is the first study in Hungary, which examines the predictive value of score systems in the case of Kawasaki disease., Method: In our study, we identified the patients treated for Kawasaki disease between January 2005 and April 2020. In each case, we calculated both the Kobayashi and the Kawanet score, and we examined their specificity and sensitivity regarding the prediction of intravenous immunoglobulin resistance and cardiac complications. In the Kobayashi score system, values above 4, in the Kawanet score system, values above 2 signal risk., Results: We identified 28 patients with Kawasaki disease. We observed moderate complications in 13, severe complications in 4 cases. 4 of our patients were resistant to intravenous immunoglobulin therapy. Regarding intravenous immunoglobulin resistance in our patient cohort, we detected low sensitivity (25%) and high specificity (91.6%) in the case of Kobayashi score, and moderate sensitivity (50%) and specificity (50%) in the case of Kawanet score. Regarding complications, we found similar results in the case of Kobayashi (sensitivity: 17%; specificity: 100%) and the Kawanet (sensitivity: 47%; specificity: 45%) score system., Conclusion: Similarly to the majority of non-Asian studies, we found the Kobayashi score system ineffective in predicting intravenous immunoglobulin resistance. However, due to its higher sensitivity, the predictive value of the Kawanet score system regarding intravenous immunoglobulin resistance is worth examining in a larger patient population in Hungary. Regarding the prediction of cardiac complications, both score systems were found to be ineffective. Orv Hetil. 2021; 162(47): 1885-1890.

Since 2017, the nomenclature of Fusarium, Acremonium and Sarocladium species have changed, as these morphologically homogeneous, but phylogenetically heterogeneous species and species complexes may be differentiated using MALDI-TOF MS examination, analyzing nucleotic sequences. This resulted in taxonomical changes. We summarize the clinical course, diagnostic and therapeutic options of keratitis caused by Fusarium and Sarocladium. The challenge of Fusarium and Sarocladium keratitis management for an ophthalmologist lies in delayed diagnosis and therapy, fulminant progression and penetration of the Descemet's membrane, restricted availability, poor penetration of antifungal agents and therapy resistance. The diagnosis is based on the clinical history of corneal trauma or contact lens wear, PCR and MALDI-TOF MS, confocal microscopic examination, microbiological culture and light-microscopic analysis of corneal scrapings. As primary conservative treatment, 5% natamycin eye drops have to be used and with results of an antimycogram, topical 1% voriconazole or 0.15-0.25% amphotericin B, in some cases 0.02% polyhexamethylene-biguanide (PHMB) may be applied. Fusarium keratitis may benefit from additional 2 × 200 mg oral voriconazole treatment, daily. In therapy resistant cases, early, large diameter penetrating keratoplasty (PKP) has to be performed, with complete removal of the infected area. With late diagnosis, delayed specific treatment and surgery, mycotic hyphae may penetrate the Descemet's membrane, leading to the loss of vision and enucleation in about every fourth patient. In our paper, we also present the heterogeneous clinical history of five Fusarium and Sarocladium keratitis cases. Orv Hetil. 2019; 160(1): 2-11.

Deep venous thrombosis is a rare disease in children under the age of 18, with an estimated incidence of 1/100,000 per year in Hungary. Its typical localization in children is in the extremities, usually occurring in newborns and in teenagers. Both congenital and acquired risk factors can be in the background. Although it is a rarity, we should think of it, because late diagnosis can cause life-threatening conditions like pulmonary embolism or central nervous system thrombosis. Detailed medical history can help the diagnosis. Etiology, possible congenital and acquired risk factors, as well as diagnostic and therapeutic options are discussed through three cases of teenage children. Diagnostic difficulties of deep venous thrombosis in childhood are the following: the occurrence is rarer than in adulthood therefore it is often forgotten as a possible diagnosis, coagulation parameters are age-dependent, and diagnosis with imaging techniques is more difficult.

The authors present the case of a 12-year old girl with infective endocarditis. Following an initial three-day period of malaise, she was admitted to the hospital with meningeal signs. The clinical symptoms and the results of the laboratory examinations, lumbar puncture and hemoculture supported the diagnosis of Staphylococcus sepsis and meningitis. The initiation of antimicrobial therapy resulted in temporary improvement while a systolic cardiac murmur appeared. Transthoracal echocardiogram raised the diagnosis of mitral valve endocarditis. Due to the subsequent deterioration in her condition, the patient was referred to the Pediatric Heart Center of the Gottsegen György National Institute of Cardiology where transoesophageal echocardiogram confirmed the diagnosis. The vegetations were removed by extracorporeal cardiac surgery including mitral valve plasty. Retrospectively, septic embolisation caused multiplex brain infarcts were identified by cranial MRI scan as the underlying cause of the initial clinical symptoms and liquor results. Following her recovery, patient remained well with some mild residual mitral regurgitation and without any residual neurological symptoms.

Gaucher disease is the most common lysosomal storage disorder caused by deficiency of the lysosomal enzyme glucocerebrosidase. By the end of 2006, the total enrollment in the international Gaucher Disease Registry included 4584 patients, 34 of them were Hungarian. The disease has three main types: non neuropathic (Type 1), acute neuropathic (Type 2), and chronic neuropathic (Type 3). The non-neuropathic type has the highest prevalence and also the greatest variability. The first symptoms occur before 10 years of age in more than 50% of the patients. Early onset of the clinical symptoms and signs predispose patients to severe phenotype and irreversible complications. Safe and efficient enzyme substitution therapy has been available from 1991 and applied since 1992 in Hungary. Optimal dose and early therapy are effective in stopping disease progression, leading to the regression of visceral and haematological abnormalities, preventing irreversible bone deformities, and providing a better quality of life. The authors present here three patients with Gaucher disease diagnosed in early childhood. They highlight the importance of early diagnosis and treatment before the development of severe co-morbidities or irreversible complications. They also analyse the challenges for pediatricians in establishing correct diagnosis of Gaucher disease in time.

Splenic rupture is a rare complication of infectious mononucleosis. Although it occurs only in 0.1%-0.5% of cases, splenic rupture remains the most common fatal complication of the disease. Mononucleosis related spontaneous rupture of the spleen without any other characteristic symptoms of the disease is extremely unusual, and threatens with fatal outcome due to its rare and unexpected occurrence. The authors report the case of a 16-year-old boy who needed splenectomy following a spontaneous rupture of the spleen. Serological tests proved an acute Epstein-Barr virus infection in the background but without any signs or symptoms of infectious mononucleosis. The diagnosis and treatment of this infection related to spleen ruptures are also discussed.

Progressive multifocal leukoencephalopathy is a rare disease caused by the reactivation of an opportunistic agent, JC virus almost in every cases in immunodeficient conditions. The disease is characterized by multifocal demyelinating lesions of the central nervous system and causes death within a few months. The authors report two patients: a 67 year-old male treated because of chronic lymphoid leukemia, and a 19 year-old male having a hereditary immunodeficiency, X-linked hyper IgM syndrome. In both cases continuously progressive right, later both hemispheric signs were detected. Cerebrospinal fluid was not helpful. Brain MRI showed bilateral large, white matter lesion. The progression was not influenced by the treatment, finally both patient died ten and six weeks after the appearance of first complaints. The diagnosis was confirmed by brain biopsy and autopsy in both cases. Our cases demonstrate that progressive multifocal leukoencephalopathy can develop in various immunodeficiencies.

Renovascular disease is defined as renal insufficiency secondary to fibromuscular or atherosclerotic renal artery stenosis. The immediate cause of renal insufficiency is not hypertension but ischemia and hyper-reninaemia that result from critical (greater than 80%) renal artery stenosis. Hyper-reninaemia damages also the contra lateral non-ischemic kidney. The key to successful treatment of renovascular disease is its diagnosis before the onset of renal insufficiency (> 180-200 micromol/l serum creatinine). The goal of interventional therapy is the prevention of symptomatic renal insufficiency and end-stage renal failure; the indication for intervention is the presence of greater than 80-85% renal artery stenosis. Patients diagnosed with non-critical renal artery stenosis (< 80%) do not need immediate intervention, only close follow-up.

A 16-years old boy with poor family circumstances was admitted to hospital due to severe cardiac failure (stagnation in the pulmonary and systemic circulations, ejection fraction: 29%, pericardial fluid, atrial flatter) needing intensive care. Diagnostic tests revealed extreme hyper-function of the thyroid as the underlying cause. After stabilising the cardiac and hormonal states of the patient with medication, subtotal resection of the thyroid was carried out since the poor family circumstances did not make the provision of long-term thyreostatic pharmacotherapy feasible. Cardioversion solved the arrhythmia. The patient was discharged from hospital with stable circulation and normal thyroid functions after 2 months. The authors report the very uncommon complications of an endocrine syndrome with autoimmune origin rarely observed in childhood. They highlight the importance of early diagnosis and the immediate start of adequate therapy for good long-term outcome.

The authors review two cases of suspected congenital cytomegalovirus (CMV) infections in which modern laboratory approaches were applied to establish the diagnosis postnatally. In the first case, intrauterine infection was suggested by ventriculomegaly, detected by means of a head ultrasonographic scan. The postnatal cranial ultrasonography and computed tomographic scans revealed intracerebral calcifications. CMV was detected in the blood and urine of the newborn. The postnatal serological tests proved that the mother had experienced a primary CMV infection during gestation. Abnormal neurological signs developed in the infant by the age of 9 months. In the second case, the mother had had an active CMV infection at 29 weeks of gestation involving a twin pregnancy. The CMV-specific serological tests demonstrated that this was a recurrent infection. The twins were born without signs or clinical symptoms and CMV was not detected in their urine samples. At 5 months of age, one of the twins excreted CMV in his urine, which must have been a consequence of a postnatal infection. The general screening of young women by CMV serology at the beginning of gestation is recommended. This would establish a CMV serostatus and provide an opportunity for the gynecologist to provide advice concerning the avoidance of infection, especially in cases where the patient is seronegative and therefore at risk of primary CMV infection.

Introduction: A significant part of cryptogenic cirrhosis among adult patients shows that it can be the result of "burn-out" non-alcoholic steatohepatitis beginning in childhood., Aim: Describing the case of a 15-year-old boy the aim of the authors is to raise attention to the fact that doctors should think of the possibility of having a fatty liver in presence of certain etiological features., Patient: Although he was asymptomatic, the screening test revealed an elevated serum aspartate aminotransferase level. Abdominal ultrasound examination raised the possibility of a diffuse liver damage. The liver biopsy demonstrated the features of steatosis in the absence of alcohol abuse., Results: On the basis of these results, NASH was diagnosed. The presence of a severe fatty liver at such an early age is quite unusual. In the background, the authors verified familial combined hyperlipidaemia and heterozygous mutation of the cystic fibrosis gene for delta F508 as genetic predisposing factor. The clinical condition was accelerated and worsened by the fact, that the patient has grown fat since his infancy. It did not prove possible to achieve a weight reduction with a fat- and cholesterol-poor diet, increased physical activity and medical treatment. However, there was significant improvement in the laboratory findings., Conclusions: The conclusion drawn from this case, on one hand, is that doctors should think of the possibility of a fatty liver in case of an elevated isolate serum transaminase level in connection with obese or over weight patients. On the other hand the role of other coexisting etiological features must emerge in the background of severe steatohepatitis.

The authors review the cases of two patients with an atypical form of cat scratch disease. The first case exhibited ileocoecal and hepatoportal lymphadenitis, and the second case the emerging symptoms of a non-specific granulomatous process in the middle third of the right forearm, which caused a differential-diagnostic problem. Establishment of a diagnosis was possible only on the basis of the disease process and the pathological, serological and histopathological pictures of the lymphatic glands after other causes of the lymphadenopathy had been excluded. Following two weeks of antibiotic treatment, the patients were free of complaints- and symptoms. Bartonella antibody tests were performed on 5 cats living in the neighbourhood of the patients with titer determinations too in 3 cases.

The huge variability of hepatitis virus C is well-established. The geographical differences in its nucleotide sequence have important clinical significance by causing variant pathogenicities and affecting sensitivity to therapy. The authors pioneered the determination of the viral type and subtype in patients suffering from chronic viral hepatitis in Hungary. In this present work they report the applied methods and the results. In their virus serological laboratory was introduced the test "HCV Serotyping 1-6 Assay", which is based on the analysis of antibodies, in 1996. By this method they examined the samples of 127 patients and they found type 1 in 75.5%, type 4 in 25%, mixed types (type 1 + 2, 3, 4, 5, 6) in 9% and non-classifiable, non-reacting antibodies in 13% of the cases. Since 1999 they have used the combination of direct reverse-PCR of the viral antigen and reverse hybridization to type-specific specimens. These results, from 211 patients show that 6% belong to 1a, 85.5% to 1b, 3% to 1a + 1b, 1% to 1b + 2, 0.5% to 3 and 4% to mixed subtypes. Genotype 1b was associated with higher viremia. On the basis of the above they can conclude that 90% of the Hungarian population are infected by the most resistant 1b subtype of hepatitis virus C. It could explain the fact that only 20% of their patients with interferon monotherapy have become permanently virus-free. In view of these results they recommend combined, higher dose, long-lasting treatment in therapeutic protocols.

In a prospective open study we investigated Chlamydia pneumoniae infections in 36 consecutively admitted patients: 26 males, mean age 53.4 yr, range 36-70 yr, 10 females mean age 57.7 yr, range 47-70 yr, suffering myocardial infarction (24 acute, 2 previous) or angina pectoris (10). Antibody serum levels were measured by the immunefluorescent method and they were as follows: negative 5, low 12, medium/high 11, chronic infection 5, recent infection 3. The 3 cases considered as recent infections are described in detail.

The sexual, household and nosocomial transmission of Hepatitis C virus infection were studied in patients with HCV-RNA positive chronic active hepatitis, confirmed by PCR. Liver function tests and hepatitis C virus serology were performed in family members living in the same household of patients, sexual partners as well as members of the hospital environment in the Department of Hepatology. Fifty-three members of 27 families and 32 staff-members were examined. Quantitative HCR-RNA-PCR and virus serotype of anti-HCV (III.gen.) positive individuals were also identified. Two of the family members turned out to be anti-HCV positive. The husband in one of the married couples was treated for hepatitis of unidentified etiology in an Infectology Department four years ago. His wife was found to have chronic hepatitis C since two years. They both had C virus of serotype 1. The mother and daughter in another family suffered from von Willebrand factor deficiency syndrome. It was documented that they were infected by blood transfusion at different occasions. Their C virus serotypes were different. All the staff members of Hepatology Department were anti-HCV negative. These results confirmed the possibility of the non-parenteral transmission of hepatitis C virus, however, its incidence is very low.

Hyper-IgM syndrome associating with severe neutropenie presenting in irregularis cycles was diagnosed in a 3-year-old male patient. His elder brother died of sepsis which appeared as a consequence of dysgammaglobulinaemia and neutropenia at the age of 9. We could not achieve a parmanent good result with the monthly immunoglobulin substitution and supportive treatment. The candida infection of the gingiva and of the oral mucous membrane expanded to the esophagus resulting in its complete occlusion and temporarily a gastrostomy was needed to feed him. We started with the recombinant human granulocyta colony stimulating factor (rh-G-CSF) treatment at the age of 6. We tried several ways of applying and finally the 10 micrograms/kg/dose given subcutan, for 5-10 days from the nadir of the neutropenic cycle seemed the most effective. The rh-G-CSF treatment resulted in an increase of ANC and the complete resolution of gingivostomatitis. The incidents of infections, the requirement of antibiotics and the duration of hospitalisation were markedly reduced. The consequent improvement in his physical condition made it possible to finally resolve the esophageal stricture surgically. We have not observed any neutropenic cycle since the end of the 14 month rh-G-CSF treatment. Though the medicine was discontinued there has been no recidiva for more than 22 months.

The authors reviewed the records of paediatric patients treated at the cardiology department of the Svábhegyi Gyermekgyógyintézet during the period from 1 January 1979 to 31 December 1993. All the patients elected for the survey had rheumatic fever and represented about 20 percent of the national patient pool. Among the 62 children referred for suspected rheumatic fever 26 had carditis, and 3 suffered from pancarditis. Two children had chorea minor with carditis and there were 2 additional cases with chorea minor only. Valvular heart disease has developed in 21 patients. There were 16 patients with mitral regurgitation, these conditions have occurred in combination with mitral stenosis in 3 cases, whereas the insuffitientia of the aortic valve developed in two patients. The authors analysed the clinical signs of the patients and established that the chronic syndromes came in front. The authors have observed a favourable trend in the epidemiology of the disease, in particular, while 34 patients were referred during the first 5 years, there were only 18 cases in the second 5-years period. During the interval from 1989 to 1993, only 10 children with rheumatic fever have been referred to or treated at the authors' institute.

63 year old patient is presented with myelo-monocytic leukemia, in whom subcutaneous nodules developed at the lower limb, spreading along the lymph vessels, and partly ulcerating. By mycologic investigation Scedosporium apiospermum was disclosed. Attention is drawn to human infections brought about by opportunistic Scedosporium species.

It is emphasised in connection with the differential diagnosis of chest complaints that after excluded the serious and urgent conditions, a searching for painful muscle bundles and trigger points at the characteristic locations in the muscles can explain the origin of the complaints. By finding of myofibrosis, other expensive or invasive diagnostic tests can be omitted and the patient gets rid off his or her claims.

A computer-based data processing was made at 6762 motor handicapped children. The data were obtained by questionnaires about the health status, educational, social level and rehabilitation of these children. The authors could state the use of therapeutical equipment, the further need for rehabilitation--specialists and the waiting list for the admission to institutions. The number of registered children was 2.4% of the specific population. The aim of the study was to promote the activity of local self governments, local--and national social security--and health authorities competent in the rehabilitation for the social integration of the Hungarian 0-18 years old motor handicapped persons.

Dicynone has been in use in all premature births prophylactically since 1987 by the authors. The administration of the drug begins before or during delivery. The diagnoses of cerebral haemorrhage was established on autopsy and the cases were compared with the previous years when Dicynone was not administered. During prophylactic use of Dicynone the cerebral haemorrhages significantly reduced among premature babies. It is well known, that the etiology of the cerebral haemorrhages are multifactorial. Their favourable experiences confirm the literary communications, whereas use of Dicynone can be one of the efficacious preventive drug against palsy of the premature babies.

The authors found, that the incidence of hypochromic, microcytic anaemia was 4 percent, and more than half of the children were suffered from iron deficiency. The causes are the inadequate daily iron uptake and/or increase iron requirement. It is very important to increase the iron content of the food and/or the prophylactic or therapeutic iron treatment.

To investigate the occurrence of motor disability a pilot study was carried out in the total children's population of Györ-Sopron county and in 57% of the children of Baranya county by computer-based questionnaires. The data of the 492 motor handicapped children were analysed by 55 computer programs. These programs are constructed for the analysis of the somatic and mental health, schooling, rehabilitation possibilities, social background, care of these children and their further needs for specialists and institutions. This procedure is suitable for national and longitudinal data collection and for the complex registration of the status of these children.