Severe Spontaneous Pneumomediastinum in a Girl with Cystic Fibrosis.

We report on an 11-year-old girl with cystic fibrosis who presented with thoracic pain and an extensive subcutaneous emphysema and subsequently developed progressive respiratory distress. The chest computed tomography revealed a huge pneumomediastinum. Due to the development of severe respiratory failure, urgent needle thoracocentesis was necessary that resulted in only temporary improvement. Therefore, under general anesthesia two mediastinal drains were introduced. Using active suction, the size of the pneumomediastinum decreased gradually and the drains were removed after 3 weeks. Here, we describe an extremely rare situation, when acute surgical intervention was necessary in a child with spontaneous pneumomediastinum.

Characterization of Asymptomatic Bacteriuria Escherichia coli Isolates in Search of Alternative Strains for Efficient Bacterial Interference against Uropathogens.

Asymptomatic bacterial colonization of the urinary bladder (asymptomatic bacteriuria, ABU) can prevent bladder colonization by uropathogens and thus symptomatic urinary tract infection (UTI). Deliberate bladder colonization with Escherichia coli ABU isolate 83972 has been shown to outcompete uropathogens and prevent symptomatic UTI by bacterial interference. Many ABU isolates evolved from uropathogenic ancestors and, although attenuated, may still be able to express virulence-associated factors. Our aim was to screen for efficient and safe candidate strains that could be used as alternatives to E. coli 83972 for preventive and therapeutic bladder colonization. To identify ABU E. coli strains with minimal virulence potential but maximal interference efficiency, we compared nine ABU isolates from diabetic patients regarding their virulence- and fitness-associated phenotypes in vitro, their virulence in a murine model of sepsis and their genome content. We identified strains in competitive growth experiments, which successfully interfere with colonization of ABU isolate 83972 or uropathogenic E. coli strain 536. Six isolates were able to outcompete E. coli 83972 and two of them also outcompeted UPEC 536 during growth in urine. Superior competitiveness was not simply a result of better growth abilities in urine, but seems also to involve expression of antagonistic factors. Competitiveness in urine did not correlate with the prevalence of determinants coding for adhesins, iron uptake, toxins, and antagonistic factors. Three ABU strains (isolates 61, 106, and 123) with superior competitiveness relative to ABU model strain 83972 display low in vivo virulence in a murine sepsis model, and susceptibility to antibiotics. They belong to different phylogroups and differ in the presence of ExPEC virulence- and fitness-associated genes. Importantly, they all lack marked cytotoxic activity and exhibit a high LD50 value in the sepsis model. These strains represent promising candidates for a more detailed assessment of relevant fitness traits in urine and their suitability for therapeutic bladder colonization.

Whole-Genome Draft Sequences of Nine Asymptomatic Escherichia coli Bacteriuria Isolates from Diabetic Patients.

Escherichia coli can colonize the urinary bladder without causing a disease response in the host. This asymptomatic bacteriuria (ABU) can protect against recurrent symptomatic urinary tract infection by virulent bacteria. Here, we report the whole-genome sequences of nine E. coli ABU isolates from diabetic patients.

Anti-N-methyl-D-aspartate receptor encephalitis and drug abuse - the probable role of molecular mimicry or the overstimulation of CB receptors in a 17-year-old adolescent - case report.

Anti-N-methyl-D-aspartate encephalitis is an autoimmune disorder characterized by autoantibodies produced against NMDA receptors. We report the case of a 17-year-old drug user teenager who presented with altered mental scale, psychiatric symptoms and autonomic dysfunction. In the background we diagnosed NMDA encephalitis. We supposed that synthetic cannabinoids/drugs may have lead to the of trigger NMDA encephalitis via the altered activation of the immune system and molecular mimicry mechanism.

[Autoimmune comorbidity and microvascular complications in childhood-onset type 1 diabetes after 20 years of diabetes duration: a questionnaire study]. / Autoimmun komorbiditás és microvascularis szövodmények gyermekkorban diagnosztizált 1-es típusú diabeteses fiatal felnottekben 20 éves betegségtartam után: Kérdoíves vizsgálat.

UNLABELLED: There are no population-based data on the autoimmune morbidity and vascular complications of young adults with childhood-onset type 1 diabetes in Hungary. AIMS: To assess the prevalence of these morbidities after 20 years of diabetes duration. METHOD: Postal questionnaire. RESULTS: 6.2% of the patients had celiac disease. Diabetes was diagnosed at a significantly earlier age in patients with diabetes and celiac disease as compared to those without celiac diasease. Thyroid autoimmunity was reported in 7.6% of cases. They were significantly older with longer duration of diabetes. Every fifth patients reported retinopathy, one sixth of patients was treated for hypertension. Neuropathy was found in 3.4% and kidney disease in 4.8% of the cases. CONCLUSIONS: Apart from retinopathy and hypertension, the prevalence of microvascular complications was relatively low. Considering the limitations of questionnaire studies, laboratory screening is warranted to assess the true prevalence of comorbidities and complications.

[Anti-NMDA-receptor encephalitis: description of the syndrome in connection with the first Hungarian patient]. / Anti-N-metil-D-aszpartát-receptor-encephalitis: a szindróma ismertetése az elsõ magyar beteg leírása kapcsán.

In the majority of cases, anti-NMDA (N-methyl-D-aspartate) receptor encephalitis is a severe, but treatable disorder, therefore early diagnosis and adequate therapy are very important. It should be suspected in children and young women, who develop acute psychiatric symptoms and seizures. During the course of the disease severe encephalopathy, agitation, hallucinations, orofacial dyskinesias, prolonged cognitive disturbance, autonomic symptoms can be observed and akinetic mutism develops. EEG shows diffuse slowing. Brain MRI is normal or unspecific. Elevated protein, pleiocytosis and oligoclonal bands can be present in the CSF Detection of NMDA-receptor antibodies in sera or CSF confirms diagnosis. We present the case of a 15-year-old girl, who fully recovered within two months after steroid treatment and repeated plasma exchange. Ovarian teratoma has not been detected.

Severe dystrophy in DiGeorge syndrome.

We present the case history of a 3-year-old girl who was examined because of severe dystrophy. In the background, cow's milk allergy was found, but her body weight was unchanged after eliminating milk from her diet. Other types of malabsorption were excluded. Based on nasal regurgitation and facial dysmorphisms, the possibility of DiGeorge syndrome was suspected and was confirmed by fluorescence in situ hybridization. The authors suggest a new feature associated with DiGeorge syndrome.

[Different clinical forms of adrenal abscess in two newborns]. / Mellékvesetályog különbözo megjelenési formái két ujszülöttben.

Adrenal abscess is a rare disease in the neonatal period. The classical symptoms are abdominal mass, anaemia and prolonged jaundice which are associated with fever, vomiting and feeding difficulties. The authors present the histories of two mature, male newborns with adrenal hemorrhage, where the size of the masses increased progressively and finally adrenal abscesses were developed. The first case showed the classical symptoms of adrenal abscess, however, the antibiotic treatment led to complete remission of the symptoms and laboratory infectious markers, therefore the baby was discharged. In a few day later he became septic and was admitted again. This time he was operated on and a large abscess was evacuated, which contained about 60 ml of pus. After this intervention multi-organ failure developed, but due to the intensive therapy the baby recovered. In the second newborn the increasing adrenal hemorrhage was accompanied by elevated infectious markers despite of the different antibiotic treatments, however, he showed no clinical signs of infection. Because of the discrepancies between the clinical and laboratory findings and also the imaging studies the possibility of neuroblastoma has also arisen. Urinary vanillylmandelic acid excretion was normal, but elevated neuron-specific enolase levels were measured. Surgical exploration was performed, pus was drained from the mass and anaerobic Bacteroides fragilis was cultured, therefore metronidazole therapy was started. After that the baby fully recovered.

Urinary cytokine response to asymptomatic bacteriuria in type 1 diabetic children and young adults.

It has been reported that urinary interleukin-6 (IL-6) and IL-8 levels are decreased in adult diabetic women with asymptomatic bacteriuria (ASB) when compared with non-diabetic women with ASB. Such impaired cytokine excretion might play a role in the higher prevalence of ASB among diabetic subjects. The aim of this study was to examine the urinary IL profile in children and young adults with type 1 diabetes mellitus (T1DM) with and without ASB. Midstream clean voiding urine samples were collected and cultured from 133 patients with T1DM (age: 15.6 +/- 5.7 yr) and 178 controls (14.1 +/- 4.7 yr) for two consecutive days. ASB was diagnosed in the case of >or=10(5) bacteria/mL. The urinary IL-6 and IL-8 concentrations were determined, and the presence of leukocyturia was also recorded. The prevalence of ASB was 16.5% in diabetic subjects and 2.8% in controls (p = 0.001). There was no difference between the diabetic and the control groups in the prevalence of 'IL-6-uria' (21.9 vs. 18.0%; p = 0.41), but IL-8 was more frequently detectable in the diabetic group (47.4 vs. 27.5%; p = 0.001). In individuals with ASB, the IL-8 level was similar in the diabetic (median: 70.0 pg/mg creatinine) and control group (42.3 pg/mg creatinine; p = 0.8). Indeed, the IL-8 levels were higher in diabetic subjects with ASB as compared with those without it (70.0 vs. <3.1 pg/mg creatinine; p = 0.001), and there was a significant association between the urinary IL-8 concentration and the bacterial count (p = 0.001). Diabetic patients with leukocyturia had higher IL-8 concentration than those without it (20.9 vs. <3.1 pg/mg creatinine; p = 0.003). Weak significant correlation was found between urinary IL-8 and hemoglobin A1c (HbA1c) (r = 0.4; p = 0.002). The sensitivity and specificity of leukocyturia were 50 and 89.9% in the whole population and those of IL-8 were 74.1 and 67.5%, respectively. In diabetic patients, 36.4% of the bacteriuria were gram-negative and 63.6% gram-positive. Our results suggest that diabetic children with ASB mount an IL-8 response to pathogens, which is comparable to non-diabetic children with bacteriuria. Thus, early in the natural history of diabetes, there are no significant changes in the IL response of children with ASB, as previously reported in adults.

[Ganciclovir treatment of congenital cytomegalovirus infection]. / Ujszülöttkori cytomegalovirus-fertozés kezelése ganciclovirrel.

The authors present the cases of two infants with congenital cytomegalovirus infection, who to the best of the authors' knowledge were the first ones to receive ganciclovir treatment in Hungary. Both infants were treated for symptomatic congenital cytomegalovirus infection affecting the central nervous system. Ganciclovir was given intravenously in two phases, for a total of 4 and 6 weeks, in a daily dose of 5-10 mg/kg. Diagnosis of infection and follow-up of treatment efficacy was based on the quantitative assessment by PCR assay of viral nucleic acid in blood and urine samples. Treatment resulted in substantial reduction of viral copy numbers in both infants' blood and urine samples. Improvement in the biochemical markers of disease activity was accompanied with spectacular improvement of clinical symptoms in the infant with severe liver involvement. Following the treatment viral loads increased in both infant but clinical symptoms did not reoccur. In one patient a considerable improvement of hearing loss was observed. The authors' first results indicate that ganciclovir treatment of neonatal cytomegalovirus infection represents a promising approach in preventing the progression of the disease and in ameliorating the consequences.