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Biomedical subjects

A Kreczy

Publications and source records attributed to A Kreczy.

At least 37 records · Page 2Linked to original sources

Topographic variations in anatomical structures of the anterior neck of children: an ultrasonographic study.

OBJECTIVE: Tracheostomies in children are frequently used for temporary airway support during surgical procedures. In pediatric patients with congenital craniofacial malformations, preoperative assessment of the delicate anatomy of the airway is necessary. The purpose of this study was to assess the ultrasonographic anatomy of the anterior neck with regard to the performance of tracheostomy. STUDY DESIGN: Ultrasonographic investigation was done in 50 pediatric patients (age range, 6 to 15 years) to analyze the relationships among the anatomical structures that are of practical interest with respect to tracheostomy. RESULTS: The data reveal that information concerning variations in anatomical structures lying in the immediate vicinity of the tracheostomy site was readily obtainable with the techniques used. CONCLUSIONS: In pediatric patients requiring tracheostomy for surgical treatment of severe congenital craniofacial malformations, preoperative ultrasonography may be used to diagnose individual anatomical variations at the tracheostomy site.

Adolescent↗

Epstein-Barr virus-negative boys with non-Hodgkin lymphoma are mutated in the SH2D1A gene, as are patients with X-linked lymphoproliferative disease (XLP).

X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency, which most often manifests itself after Epstein-Barr virus (EBV) infection. The main clinical phenotypes include fulminant or fatal infectious mononucleosis, dysgammaglobulinaemia and malignant lymphoma. We have recently cloned the SH2D1A gene, which has been shown to be mutated in approximately 70% of XLP patients. Now we report five novel SH2D1A mutations in patients from five unrelated XLP families. No mutations were found in another three XLP families. In three boys with early onset non-Hodgkin lymphoma (NHL) from two unrelated families a deletion of SH2D1A exon 1 and a splice site mutation were found, respectively. These patients did not show any laboratory or clinical signs of a previous EBV infection. A fourth EBV-uninfected and unrelated boy with a stop mutation in the SH2D1A gene shows only signs of dysgammaglobulinaemia. Development of dysgamma-globulinaemia and lymphoma without evidence of prior EBV infection in four of our patients suggests that EBV is unrelated to these phenotypes, in contrast to fulminant or fatal infectious mononucleosis. The role of SH2D1A as a putative tumour suppressor gene remains to be investigated.

Carrier Proteins↗

Necrotizing sialometaplasia in two patients with bulimia and chronic vomiting.

The purpose of this report was to present two unusual cases of necrotizing sialometaplasia of the hard palate, in which the patients were suffering from bulimia and chronic self-induced vomiting. It emphasizes the importance of confirming the diagnosis by incisional biopsy and discusses local mucosal trauma as a possible etiologic factor.

Adult↗

In-vitro testing of susceptibility to amphotericin B is a reliable predictor of clinical outcome in invasive aspergillosis.

Invasive aspergillosis is a life-threatening fungal infection which, in neutropenic patients, is associated with an extremely high mortality rate despite optimal treatment. In order to investigate microbiological risk factors for treatment failures in more detail, Aspergillus spp. obtained from 29 patients with haematological diseases after myelo-ablative chemotherapy and bone marrow transplantation were analysed for their susceptibility to amphotericin B in vitro and this was compared with clinical outcome to see if there was a correlation. Aspergillus flavus was present in 12 (41 %) of the 29 patients, Aspergillus terreus in nine (31%) and Aspergillus fumigatus in eight (28%). The susceptibility of these isolates to amphotericin B varied between and within the three species. A. terreus was the only organism against which the MIC was consistently high, A. fumigatus and A. flavus showing variation between isolates in the degree of resistance to amphotericin B. The degree of in-vitro resistance was the only parameter correlating with clinical outcome in a univariate analysis and the only prognostic value in a multivariate analysis considering known risk factors. Irrespective of the species, all six patients with isolates against which the MIC was <2 mg/L survived, whereas most (22/23) of those with isolates resistant to > or = 2 mg/L died. Infections among the six survivors were caused by amphotericin B-susceptible A. fumigatus and A. flavus, but not A. terreus. We conclude that the outcome of aspergillus infection depends on the in-vitro susceptibility of the isolates to amphotericin B. Survival was poor in patients with isolates resistant to amphotericin B and good in those with amphotericin B-susceptible specimens. A. terreus was always associated with high resistance to amphotericin B and with poor survival.

Amphotericin B↗

Adrenocortical carcinoma evolving after diagnosis of preclinical Cushing's syndrome in an adrenal incidentaloma. A case report.

A 43-year-old female patient underwent abdominal ultrasonography and CT scan because of uncharacteristic abdominal pain. A 3-cm homogeneous adrenal tumor was diagnosed. The endocrine tests revealed an adrenal preclinical Cushing's syndrome (PCS). Due to the latent hormone excess we decided to operate on the adrenal tumor. Since the tumor was small, laparoscopic adrenalectomy was performed. Histological evaluation showed an adrenocortical tumor of undetermined nature. Four months later the patient presented with a metastasizing cortisol- and androgen-producing adrenocortical carcinoma (ACC). After pretreatment with ketoconazole to suppress the biosynthesis of adrenal steroids under substitution with hydrocortisone, we reduced the tumor load by surgery. Postoperatively we continued ketoconazole and started o, p'-dichlorodiphenyldichloroethane as well as chemotherapy with doxorubicin and suramin. However, the patient died from ACC 7 months after adrenalectomy. It is known from several reports that PCS may persist clinically silently or may progress to full-blown Cushing's syndrome. This is the first time a malignant course of PCS is described. Independent of the initial therapeutic strategy of PCS, i. e. surgery or regular follow-up visits, we must be aware that also relatively small adrenal tumors can harbor malignancy.

Adrenal Cortex Neoplasms↗

Bone bruise of the knee: histology and cryosections in 5 cases.

We evaluated the histopathologic and cryosectional appearance of bone bruise injuries of the knee detected on MRI. Histologic evaluation of bone biopsies from 3 patients revealed microfractures of cancellous bone, edema and bleeding in the fatty marrow. Between intact lamellar bone trabecules, fragments of hyaline cartilage mixed with highly fragmented bone trabecules were found. Postmortem specimens were obtained from 2 more patients, killed in motor vehicle accidents. MRI revealed bone bruise injuries of the lateral femoral condyle and of the lateral tibial plateau in 1 knee and anterior cruciate ligament disruption, a medial meniscus tear and bone bruise injury of the tibial plateau and of the lateral femoral condyle in the other specimen. The specimens were embedded in physiologic saline solution and frozen to -30 degrees C. By rotationcryotomy, 1 mm slices were removed from the surface of the specimens and documented on photographs. Subchondral lesions and bleeding were found, corresponding to the MR images.

Adult↗

DNA ploidy determination of early molar pregnancies by image analysis: comparison to histologic classification.

BACKGROUND: To improve histologic diagnosis of molar pregnancies, updated guidelines have been proposed recently. These guidelines take into account that less developed molar gestations differ from their fully developed counterparts. OBJECTIVE: To test the validity of these criteria by correlating histologic diagnosis with ploidy determination accomplished by means of image analysis. DESIGN: Fifty archival cases of early molar pregnancy were reclassified according to the new criteria. The diagnosis had to be changed from partial to complete hydatidiform mole (PM to CM, respectively) in 9 cases and from CM to PM in 4 cases. DNA image cytometry could be performed in 40 cases (CM, n = 21; PM, n = 19). RESULTS: There was 100% agreement between histologic diagnosis and a diploid or polyploid histogram in CM and 79% agreement between triploidy and PM, when the updated diagnostic criteria were used. This represents an improvement compared with diagnoses made with former criteria. Nevertheless, problems of correct classification remain: In 3 cases classified as PMs, fetal remnants were accompanied by the histologic appearance of a CM. These 3 cases could represent either a true embryonic development in CM or a twin gestation with one normal pregnancy and one mole, or they could belong to a (very rare) third type of mole. All of them show the same risk of persistent trophoblastic disease observed in classic CM. CONCLUSIONS: As the groups at risk for developing persistent trophoblastic disease can be identified by their DNA histograms, ploidy analysis would be desirable in addition to histologic examination.

Adult↗

Partial nephrectomy in a cystic partially differentiated nephroblastoma.

Cystic partially differentiated nephroblastoma (CPDN) is a rare neoplastic disorder consisting of a well-demarcated cystic lesion of the kidney where blastemal or other embryonic cells are present in the septa of the cysts. Magnetic resonance imaging can detect the cystic character of the lesion and will produce imaging features that are highly suggestive of either CPDN or cystic nephroma (CN) (synonym: multilocular cyst of the kidney), a benign entity. Although malignant potential exists in CPDN, all cases reported to date have had a favorable prognosis after surgery alone. Partial nephrectomy is considered safe, and the treatment of choice in the newborn period. We report a case of CPDN in a newborn that was successfully treated with partial nephrectomy. More than five years after nephron sparing surgery, the involved kidney shows normal anatomical structure except for a diminished upper pole, no evidence of tumor recurrence and good renal function.

Cysts↗

Flexor tenosynovitis in the hand caused by Mycobacterium malmoense: a case report.

This report analyzes a rare case of flexor tenosynovitis caused by Mycobacterium malmoense. A synovectomy was carried out on the index finger (no other finger was afflicted) of a 66-year-old farmer, followed by antibiotic therapy with ethambutol, rifampin, and clarithromycin. Because of strong side effects, the treatment with ethambutol and rifampicin had to be discontinued after 4 months. There was no recurrence after 14 months, and the patient's finger had a full range of motion.

Aged↗

Is there a dysplasia-carcinoma sequence in rat gastric remnant?

Epithelial dysplasia in the gastric remnant is generally considered to have a positive predictive value for malignancy. Whether dysplasia progresses to carcinoma or whether both just have a common origin, is still a matter of controversy. The aim of the present study in rats was to investigate the natural history of epithelial lesions in the gastric remnant. A gastric resection was carried out in 50 male Wistar rats. Postoperatively the animals received N-methyl-N'-nitro-N-nitrosoguanidine orally. Gastroscopy was carried out monthly and biopsies were taken for histologic evaluation. The rats were killed after 12 months or if gastric cancer was found on gastroscopy. Twenty-four rats died postoperatively and were excluded from the study. A total of 228 gastroscopies was performed in the remaining 26 animals; 24 animals developed dysplastic lesions during the follow-up period. The rate of development of gastric cancer within one month increased with the stage of dysplasia at the previous examination (3% for mild, 48% for moderate, 100% for severe dysplasia). There was a strong correlation between the time period following gastric resection and grade of dysplasia and between the grade of dysplasia and development of cancer. Our study demonstrates that gastric stump cancer in rats develops from dysplastic lesions. A dysplasia-carcinoma sequence can therefore be assumed.

Animals↗

Deletion mapping on chromosome 17p in medulloblastoma.

Medulloblastoma is the most frequent paediatric brain tumour. Because of the uniform histology, a common genetic mechanism has been postulated. Loss of heterozygosity (LOH) studies support evidence that a candidate gene, which functions as a tumour-suppressor gene, is located in 17p13. Eighteen tumours were examined for loss of heterozygosity at 15 different loci at chromosome 17p. Nine of 18 (50%) tumours had allelic loss in 17p 13.3-13.2. The smallest region of overlap, which harbours the disease gene, includes markers from UT222 (D17S675) to UT49 (D17S731) and spans a region of less than 6 cM. Candidate genes within this region are HIC-1, a potential tumour-suppressor gene, and DPH2L, a gene that has been cloned from the ovarian critical region. The putative region excludes the p53 gene and the ABR gene, which have been favoured by others. LOH of chromosome 17p may be used as a new prognostic biological marker. Children with an allelic loss had a poorer prognosis than those patients without loss of heterozygosity (P<0.05).

Adolescent↗

Hydranencephaly with renal dysgenesis: a coincidental finding? Case report with review of the literature.

We present the case of a female fetus aborted in the 20th week of gestation due to severely dysplastic kidneys, anhydramnios and hydranencephalus. The combination of these malformations is extremely rare, resulting in only 4 cases described so far. Our case is the first ever presented in a female showing polycystic-dysplastic kidneys. Multiple multinucleated neurons were a remarkable finding in the remnants of the brain. The possibility of an underlying genetic disorder is discussed, together with a brief review of the literature to date.

Abortion, Induced↗

Gastric antral vascular ectasia with portal hypertension: treatment with TIPSS.

Gastric antral vascular ectasia, or "watermelon stomach," is a distinct clinical entity within the spectrum of upper gastrointestinal mucosal vascular abnormalities. The pathobiology of the disease is unclear. We here describe a case that emphasizes the importance of the "watermelon stomach" as differential diagnosis of occult gastrointestinal blood loss and report on its successful treatment through installation of a transjugular intrahepatic portosystemic stent shunt (TIPSS).

Aged↗

Isochromosome 12p and maternal loss of 1p36 in a pediatric testicular germ cell tumor.

Analysis of a pediatric germ cell tumor by conventional cytogenetic investigation and fluorescence in situ hybridization showed consistently the presence of two isochromosomes 12p, loss of the maternal band 1p36, and other numerical and structural chromosome changes. The rearrangements observed resulted mainly from breaks occurring at paracentromeric regions. This report represents the first description of i(12)(p10) in a pediatric testicular embryonal carcinoma.

Adolescent↗

Diagnostic value of different PCR assays for the detection of mycobacterial DNA in granulomatous lymphadenopathy.

Diagnosis of mycobacterial infection is made by assessment of characteristic histological features, staining of acid-fast bacilli, or agar culture. Recent advances in molecular biology have provided alternative approaches for the detection of mycobacteria, but only limited data are available dealing with the comparative evaluation of these methods. In order to determine the diagnostic applicability of polymerase chain reaction (PCR)-based assays, 20 formalin-fixed and paraffin-embedded lymph nodes with bacille Calmette-Guérin (BCG) lymphadenitis were investigated which in Löwenstein Jensen agar culture were either positive or negative (ten cases each); ten lymph nodes with non-specific lymphadenitis served as negative controls. Ziehl-Neelsen staining as well as three different PCR assays (including nested PCR), amplifying a specific sequence of the Mycobacterium tuberculosis complex or sequences of the 65 kD antigen gene, were performed. Positive culture was only obtained from lymph nodes which had been surgically removed within 20 weeks after vaccination (P < 0.001). In contrast to microscopic examination, which yielded no more information than agar culture, PCR detection of mycobacterial DNA was unrelated to culture findings. Combined use of different assays, as well as DNA extraction from at least three paraffin sections from each specimen, resulted in the detection of mycobacterial DNA in all lymph nodes with amplifiable DNA (18 out of 20 cases). Controls remained consistently negative. Thus, the combined use of different PCR assays is proposed as a rapid and sensitive technique for the detection of mycobacterial DNA in formalin-fixed and paraffin-embedded tissue.

Axilla↗

Comparison of different modes of high-frequency ventilation in surfactant-deficient rabbits.

Various modes of high-frequency ventilation (HFV) have been developed to avoid the disadvantages of conventional mechanical ventilation. In the present study, we examined the hypothesis that high-frequency oscillation (HFO) is superior to high-frequency positive pressure ventilation (HPPV) and combined high-frequency ventilation (CHFV) in surfactant-deficient rabbits. The aim of the ventilator strategy was to adjust the mean airway pressure to 2 cm above critical opening pressure of the inflation limb of the respiratory system pressure volume (P/V) curve, achieve a normal tidal volume (VT) (5 ml/kg body weight) and apply repeated sustained inflations. We studied the effect of these HFV modes on oxygenation, lung mechanics and lung histology in 15 New Zealand White rabbits during a 6-hour experiment. Statistically, the HFO group demonstrated significantly better oxygenation (P < 0.05), lung mechanics (lung stability index: P < 0.05), and better lung tissue histology compared to the HPPV and CHFV groups. In contrast to the HPPV and CHFV groups, the P/V curves of the HFO group showed significant recovery over the 6-hour period after lavage. The lungs of the HFO-treated group had a more uniform distribution of alveoli and less overdistention than the HPPV group (P < 0.002), and less atelectasis than the CHFV group (P < 0.05). The HFO group had less lung injury than the CHFV groups (P < 0.01) and its lungs contained significantly less water than both other groups (P < 0.05). We conclude that the relationship between mean and end-expiratory pressures impacts strongly on both oxygenation and the progression of injury during HFV at the same mean airway pressures. The HFO group showed less acute lung injury than the other ventilatory groups.

Animals↗