Prognostic value of FDG-PET in Hodgkin lymphoma for posttreatment evaluation. Long term follow-up results.

Determining the viability of residual tumor masses is a great challenge after primary treatment of Hodgkin lymphoma. FDG-PET may play a crucial role in this procedure. In this study, files of 128 Hodgkin lymphoma patients were reviewed, who were treated in three Hungarian hematology centers between January 1995 and February 2005. CT scan showed residual tumor mass by all of them. Their median follow-up was 75.5 months from PET examination. The number of true-positive, true-negative, false-positive, false-negative subjects were 29, 83, 10, 6, respectively. Sensitivity of post-treatment FDG-PET was 83 %, specificity 93 %, positive predictive value 74 %, negative predictive value 93 %, and accuracy 88 %. The difference between the event free survival of PET positive and negative cases is highly significant (p=0.0000), according to the Mantel-Cox test. Our results in the largest cohort of patients, in accordance with literature, clearly indicates that patients with negative FDG-PET results are unlikely to progress or relapse during the longest follow-up.

Size at birth in a rapidly developing economy: intrauterine growth pattern of UAE infants.

BACKGROUND: Despite rapid economic growth and the recognition of intrauterine growth pattern as an important indicator of neonatal morbidity and mortality, the size at birth relative to gestation for UAE (United Arab Emirates) live births has not been investigated. AIM: The present study evaluated the intrauterine growth pattern of UAE infants and compared the data with the currently used reference standard. SUBJECTS AND METHODS: A total of 2497 singleton hospital live births to UAE mothers without pregnancy complications were studied. Anthropometric measurements and gestational age assessment of each infant were carried out according to standard procedures. The LMS computer program was used to construct perentile curves. RESULTS: The mean birth weight, length and head circumference of 1113 male term infants were 3298 g, 50.6 cm and 34.5 cm, respectively, and the same parameters for 1118 female term infants were 3201 g, 49.9 cm and 34.0 cm, respectively. These growth parameters were higher in males than females. Mean birth weight data were similar to those reported previously from a study from an economically developed community. The 10th percentile values were higher than in the currently used reference chart. CONCLUSION: Data on size at birth for UAE infants indicate that continuing use of the current reference chart may underestimate the prevalence of fetal growth failure in the population. Data from larger numbers of very preterm infants are needed to generate percentiles charts for very preterm infants.

Neonatal outcome in the United Arab Emirates: the effect of changes in resources and practices.

Selective improvements in neonatal care resources and practices were instituted between 1992/1994 (period 1) and 1995/1998 (period 2) following a neonatal audit in the United Arab Emirates. We evaluated the effect of these changes on neonatal mortality rate (NNMR), birth-weight-specific mortality rates and causes of mortality. Overall there was a 17% decline in the NNMR from periods 1 to 2. Mortality rates in infants with birth weight < 1000 g and > 2500 g decreased by 36% and 35% respectively from periods 1 to 2. Modest declines in deaths from asphyxia, sepsis and complications of preterm births occurred from periods 1 to 2 but the differences were not statistically significant.

Second malignancies after treatment for Hodgkin's disease.

The occurrence of treatment-related second malignancy following Hodgkin's disease (HD) has now been recognized as a major problem. The purpose of this study was to review our experience with second malignancies in patients treated for Hodgkin's disease, comparing the results with the international literature data. Six hundred and sixty five patients with HD were treated in our department, between 1978 and 1996. Second neoplasm developed in 32 cases (4.8%). Seven secondary hematological malignancies were observed: four acute nonlymphocytic leukemias, two non-Hodgkin's lymphomas and one chronic myeloid leukemia. Among patients with second hematological malignancies, the mean age at diagnosis of HD was 44 years and the mean interval until the development of second malignancy was 6.1 years. Five patients received chemo- and radiotherapy and in two cases chemotherapy was used. Three of the seven patients are alive. Twenty-five patients have had solid tumors, affecting lung (5), breast (3), colon (3), stomach (2), urinary bladder (2), head-and-neck (1), thyroid gland (1), esophagus (1), liver (1), pancreas (1), furthermore, three sarcomas and two malignant melanomas were observed. Their mean age at the diagnosis of HD was 46 years and the mean period of latency was 8.3 years. Chemotherapy was applied to nine patients, 16 patients received both chemo- and radiotherapy. Eleven patients had solid tumors in the region irradiated earlier. Ten out of the 25 patients are alive, three patients' present state is unknown. Since alkylating agents increase the risk of leukemia and irradiation contributes mainly to other malignancies, future treatment protocols should attempt to reduce the most serious consequence of therapy without compromising the survival. It is necessary to investigate the impact of additional risk factors. Careful, lifelong observation is indicated for patients with HD, with special attention given to new clinical signs and symptoms.

Novel genotype of mevalonic aciduria with fatalities in premature siblings.

Mevalonic aciduria is described in two very low birthweight siblings with unspecific clinical signs and recurrent septicaemia. Both died within the first 2 months of life. DNA analysis showed a novel mutation in the gene encoding mevalonate kinase.

Microlissencephaly.

An inbred Arab family with three neonates affected by microlissencephaly syndrome is reported. Brain magnetic resonance imaging in the index case revealed very thin brain mantle with agyria-pachygyria, agenesis of the corpus callosum, and hypoplasia of the brainstem and cerebellum. All three neonates had microcephaly, arthrogryposis multiplex congenita, and micropenis. The presence of three affected newborn infants in a consanguineous family suggests an autosomal-recessive mode of inheritance of this syndrome. The spectrum of microlissencephaly syndrome is reviewed.

[Osseous abnormalities and CT findings in Stueve-Wiedemann-Syndrome (SWS)]. / Ossäre Manifestationen und CT-Befunde bei der seltenen Skelettdysplasie Stueve-Wiedemann (SWS).

PURPOSE: Analysis of typical conventional radiological and CT findings in our group of patients with the rare skeletal dysplasia Stueve-Wiedemann-Syndrome (SWS) and comparison with published data. MATERIALS AND METHODS: In 16 newborns with clinically dysmorphic features, dwarfism, and bowed limbs, radiographs of the chest and skeleton were obtained for classification of the underlying skeletal dysplasia. For the first time, computed tomography was performed for further investigation of midface hypoplasia. The early diagnosis of SWS could be made by correlation of the radiological and clinical findings. For evaluation of progression, follow-up radiological examinations of the skeleton were performed in four children surviving infancy. RESULTS: Clinically, the newborns with SWS showed dwarfism, midface hypoplasia, bowed extremities with contractures and had severe problems with respiration, feeding, and swallowing as well as episodes of hyperthermia. Skeletal radiographs revealed bowing of the long tubular bones, most pronounced at the lower extremities. Additional findings were internal triangular cortical diaphyseal thickening at the concave side of the bowing, wide metaphyses with abnormal trabecular pattern and radiolucencies. Four patients survived infancy. Clinically, they suffered from recurrent aspiration pneumonia and recurrent episodes of hyperthermia as well as form cutaneous and mucosal infections. The follow-up radiographs showed progressive bowing of the long tubular bones as well as progressive metaphyseal decalcification. CONCLUSIONS: Skeletal abnormalities in SWS are so characteristic that an early post partum diagnosis can be made. However, a close cooperation between radiologists, clinicians, and geneticists is required for correlation of clinical and radiological findings. The few cases that survive infancy have progressing orthopaedic problems.

ABVD chemotherapy of Hodgkin's disease.

The authors report on their results in ABVD therapy, which was given by 91 patients with Hodgkin's disease as first-line treatment. 78 patients (85% achieved complete, 10 (11%) partial remission, 3 (4%) did not respond to therapy. In the follow-up period (36-223 months) 6 patients (7%) died because of the progression of Hodgkin's disease. Serious side effects or treatment-related death did not occur. Based on their results of ABVD, the authors find ABVD chemotherapy effective as first-line treatment of Hodgkin's disease.

Restriction endonuclease analysis of a porcine isolate of bovine herpesvirus type 1.

Deoxyribonucleic acid (DNA) was extracted from bovine herpesvirus type 1 (BHV-1) isolated from a stillborn porcine fetus, from the Cooper reference strain of BHV-1, and from an Ontario bovine respiratory isolate. Each DNA was digested with the restriction endonucleases HindIII, EcoRI, HpaI and BamHI. Except for very minor differences in the patterns produced after digestion with EcoRI and HindIII, the DNA of the porcine isolate reacted in a similar manner to the bovine viruses, and it was concluded that the porcine virus is genetically similar to bovine isolates of BHV-1.

Larsen syndrome in siblings with consanguineous parents.

Two siblings with Larsen syndrome and consanguineous parents are described, both with multiple joint dislocations and the typical facial appearance. Short stature is marked in both children and one had a diaphragmatic hernia which has not been previously described in this condition.

Neonatal audit in the United Arab Emirates: a country with a rapidly developing economy.

We aimed to determine whether birth-weight-specific mortality rates and causes of neonatal death could identify interventions needed to reduce neonatal mortality rates. Data were collected from three hospitals responsible for 99% of births in Al-Ain Medical District. There were 8083 live births weighing > or = 500 g, of which 54 (0.67%) died. The mortality rate among very low-birth-weight infants was higher in this district than from centres with more advanced neonatal technology and resources. Problems of preterm births, lethal malformations and asphyxia accounted for 95% of deaths and half of the malformations were autosomal recessive syndromes. Improved management of lower-birth-weight infants, asphyxia and genetic counselling could lead to a further decline in neonatal mortality rates.

[Second malignant disease in patients under treatment for malignant lymphoma]. / Malignus lymphoma miatt kezelt betegeink második malignus betegsége.

Between 1978 and 1993, 1013 patients 529 with Hodgkin's disease and 484 with non-Hodgkin's lymphomas--were treated in our department. Out of 1013 patients secondary neoplasms developed in 23 cases: 3 acute nonlymphocytic leukemia, 19 solid tumors and 1 secondary non-Hodgkin's lymphoma. The median time from diagnosis of malignant lymphoma was 7 years and their median age was 49 years. 11 patients with secondary tumor were treated with chemotherapy and 12 received combined (radio- and chemo-) therapy. Since alkylating agents increase the risk of leukemia and radiation contributes mainly to other cancer, future treatment protocols should attempt to reduce the most serious consequences of therapy without compromising the survival. Careful lifelong observation is indicated for patients with malignant lymphomas, with special attention given to new clinical signs or symptoms.

[CHOP-Bleo therapy in high-malignancy non-Hodgkin lymphoma]. / Kifejezett malignitasú non-Hodgkin lymphomás betegek CHOP-Bleo kezelése.

The records of 85 patients were reviewed in order to study the efficacy of CHOP-Bleo combination chemotherapy and to determine factors influencing prognosis. The patients were treated between 1978 and 1989, the median age was 44.4 (range, 15-68) years, the majority of them were in advanced clinical stages (92.9%). Systemic (B) symptoms were present in half of the patients and a quarter of them had bulky disease. Complete response was achieved in 36 patients (42.5%), partial response in 26 patients (30.5%), and primary treatment failure occurred in 23 patients (27.0%). The median survival was 92.6 months and 36.5% of the patients remained relapse-free at 10 years. The expected 5-year and 10-year survival rates were 47% and 39%, respectively. Using multivariate analysis B symptoms, involvation of two or more extralymphatic sites and lymphoblastic histologic subtype proved to be unfavourable prognostic factors. The most frequently observed side-effects were myelosuppression, neurotoxicity and gastrointestinal symptoms. No treatment-related death have occurred.

[Clinical features and guidelines of treatment of mucosa-associated lymphoid tissue lymphoma of stomach] / A primer gyomor MALT limfóma klinikuma és korszerû kezelésének irányelvei.

The mucosa-associated lymphoid tissue (MALT) lymphoma is a very indolent disease. Its most common site is the stomach. The lymphoma begins as a reactive lymphocyte accumulation mostly due to an infection of Helicobacter pylori (HP). Through repeated mutations this tissue is transformed into the characteristic MALT lymphoma. At the time of the diagnosis the lymphoma is usually localised, but in one third of the patients the disease has already been disseminated. There are not any commonly accepted guidelines of therapy concerning this primary gastric MALT lymphoma, but certain general tendencies have already been defined. In the early disease the aim of the treatment is curative with the preservation of the stomach as much as possible. In a considerable number of cases, when the surface of the stomach is affected by HP, one can achieve histological and molecular biologic remission after eliminating the bacteria. However, there is no such therapeutic consequence to be expected in case of a deeply invasive tumour. The optimal treatment of patients of this group as well as those whose disease is resistant to HP eradication treatment together with those who are HP negative is radiotherapy or surgery with chemotherapy. In this latter case quality of life becomes worse. In an advanced case cure is impossible and chemotherapy is the most effective to ease the patient's state.

Determination of normal ß values via radio frequency echo-tracking technique in a healthy Central European population.

The aim of our article is to report normal values of ß stiffness parameter and pulse wave velocity (PWV) determined via radio frequency echo-tracking technique first in different age groups of a healthy Central European population in both gender. Values of PWV and ß stiffness parameters increase normally during aging and in certain vascular diseases including atherosclerosis. Detection of significant deviation from the normal values provides opportunity to diagnose the early stage atherosclerosis before morphological changes become visible and while therapy is more efficient. Atherosclerosis is responsible great percentage of morbidity and mortality in western world population therefore the detection of these parameters could be important and useful in primary and secondary prevention.

Estimation of vessel age and early diagnose of atherosclerosis in progeria syndrome by using echo-tracking.

The stiffness of the arteries normally increases with age. Radiofrequency echo-tracking is a non-invasive ultrasound method which is able to detect the stiffness of the arteries, represented by the beta stiffness index. The estimation of biological age of vessels is possible on the basis of the normal age-group specific beta stiffness values. The beta stiffness index becomes higher in early stages of atherosclerosis as well, before any visible morphological changes. Hutchinson-Gilford progeria syndrome (HGPS) is rare genetic disorder resulting in accelerated aging including appearance of progressive atherosclerosis at an early age which determines the quality and term of life of these patients. Determination of vascular age and early diagnosis of atherosclerosis seems crucial. According to our knowledge, the estimation of vascular age detected with radiofrequency echo-tracking in HGPS patients, in contrast to the normal age-specific beta stiffness values, has not been published yet.

Radiological manifestations of the skeleton, lungs and brain in Stueve-Wiedemann syndrome.

A total of 25 patients with the rare skeletal dysplasia Stueve-Wiedemann syndrome (SWS) have been evaluated during the last 11 years. Of all patients with clinical suspicion of SWS, skeletal and chest radiographs were obtained for classification of the underlying skeletal dysplasia. In one case, CT was carried out for the first time for further investigation of the midface hypoplasia. Typical conventional radiological findings and CT features were analysed and compared with published data. Early diagnosis of SWS was made by correlating radiological and clinical findings. Follow-up radiological examinations of the skeleton and of the chest were carried out in six children surviving infancy for evaluation of progression. Clinically, they suffered from progressive orthopaedic problems, recurrent aspiration pneumonia and recurrent episodes of hyperthermia, as well as cutaneous infections. Radiologically progressive bowing of the long tubular bones and progressive metaphyseal decalcification were present on follow-up skeletal radiographs. Skeletal abnormalities in SWS are so characteristic that an early post-partum diagnosis can be made by correlation of typical clinical and radiological findings. Few cases survive infancy. First, these patients face progressive orthopaedic problems and respiratory infections.

Genetic contribution to high neonatally lethal malformation rate in the United Arab Emirates.

OBJECTIVES: We examined the contribution of genetic disorders to congenital anomalies (CA) causing neonatal deaths in the Al Ain Medical District (AMD) in the United Arab Emirates (UAE) because of the high consanguineous marriage rate in the community. METHODS: Charts of all neonatal deaths in the three perinatal units, which accounted for 99% of all births in AMD (1992-2000), were studied. Data regarding pregnancy, a family history including the level of parental consanguinity, the results of genetic evaluations and neonatal outcomes were recorded as part of an ongoing malformation surveillance system. Causes of death were based on clinical, laboratory and imaging findings. RESULTS: Of the 508 neonates who died, 212 (42%) had CA, which were the leading cause of death. Forty-four percent of the CA were due to definite genetic disorders and 75% of these were single gene defects. Multisystem malformations were the commonest congenital malformations. Parental consanguinity was associated with a 2-fold increased risk of non-chromosomal multisystem malformations. CONCLUSIONS: Lethal malformations were the leading cause of neonatal deaths, and parental consanguinity was associated with an increased risk of autosomal recessive disorders. The results underscore the importance of genetic screening and counseling in strategies for further significant reductions in the neonatal mortality rate in the UAE.