[Surgical urinary diversion in children from war and crisis areas-20-year experience report]. / Operative Harnableitung bei Kindern aus Kriegs- und Krisengebieten ­ 20-jähriger Erfahrungsbericht.

BACKGROUND: Because sick and injured children from war and crisis areas can often only be helped to a limited extent in their countries of origin, those children with the most complex diseases often receive medical treatment in Europe. MATERIALS AND METHODS: To evaluate the postoperative outcome of reconstructive interventions in children from war and crisis areas, an evaluation of the clinic's internal database between 1997 and 2017 was carried out. The operative indication, the surgical procedure, any revisions or conversions and the overall mortality were analyzed. RESULTS: During the above-mentioned period a total of 44 (male: 32, female: 12) children aged 3-14 years from Afghanistan, Angola, Central Asia and the Caucasus were treated. Indications for the reconstructive procedures were bladder exstrophy (n = 23), neurogenic bladder dysfunction (n = 9), penile partial amputation (n = 1) and traumatic bladder dysfunction (n = 11). In 26 children, a conduit was created, 11 children received a sigma rectum pouch and 1 child an ileocecal pouch, while 5 other children received bladder augmentation and 1 child an oral mucosal urethroplasty. In total, 7 revisions or conversions due to ureteral implantation stenoses and recurrent acidosis had to be performed. Five children died in their home country due to acidosis, sepsis, end stage renal failure, accident and social exclusion. CONCLUSION: In the long term 39 of the 44 (88.6%) children live socially integrated in their home country and are regularly monitored by local aid organizations. When choosing the reconstructive intervention, the local conditions must be considered.

Endothelial function in contemporary patients with repaired coarctation of aorta.

OBJECTIVE: Previous studies have suggested endothelial dysfunction in adult patients after repair of aortic coarctation (CoA). It has been proposed to play a key role in the pathogenesis of arterial hypertension in the absence of re-coarctation. We aimed to assess the presence of endothelial dysfunction, the number of endothelial progenitor cells (EPC), and the levels of proinflammatory cytokines associated with endothelial injury in contemporary patients after CoA repair. METHODS: For this prospective observational study, 20 CoA patients and 22 healthy controls were recruited. Digital reactive hyperaemia was measured by peripheral arterial tonometry. Flow cytometry was used to quantify EPCs, and a comprehensive panel of laboratory markers of endothelial dysfunction was measured. RESULTS: Half the patients had known arterial hypertension requiring medical treatment. Indices of reactive hyperaemia showed no significant difference between CoA patients (1.96±0.32) and controlss (1.765±0.48) (p=0.82). Circulating EPCs, defined by the number of CD34(+), CD34(+)/KDR(+), CD34(+)/AC133(+), CD34(+)/AC133(+)/KDR(+) or CD34(+)/CD45(-) labelled cells were equally not significantly different between the groups. Furthermore, plasma levels of inflammatory mediators and markers of endothelial function (IL-6, IL-8, ICAM1 and VCAM1) were not significantly different between the groups, as were vascular endothelial growth factor levels (p>0.05, for all). CONCLUSIONS: By contrast with earlier reports, no clinically significant difference in endothelial function between adult patients with coarctation repair and healthy controls could be demonstrated. Therefore, endothelial dysfunction may not necessarily be present in this population. Further studies are required to identify mechanisms and to develop strategies to avoid arterial hypertension in these patients.

Prevalence and associations of psychological distress in young adults with Type 1 diabetes.

AIMS: To determine the prevalence of psychological distress in young adults with Type 1 diabetes and to explore associated factors. METHODS: Ninety-two participants with Type 1 diabetes (46 male, 46 female) attending a young adult clinic completed two psychological self-report assessments; the Centre for Epidemiological Studies-Depression Scale (CES-D) and Adult Self-Report Scale (ASR). The mean age was 21.6 +/- 2.8 years (sd) and mean duration of diabetes was 9.3 +/- 5.4 years. A questionnaire identified the method of insulin delivery, the frequency of blood glucose monitoring and hypoglycaemia requiring third-party assistance. HbA(1c) was measured. RESULTS: Of the participants, 35.2% reported depressive symptoms (CES-D > or = 16), 23.1% indicating severe depressive symptoms (CES-D > or = 24), and 32.2, 40.4 and 35.5% of participants reported significant distress (ASR > or = 60) on the ASR total problem scales, ASR internalizing and ASR externalizing scores, respectively. Mean HbA(1c) levels were higher in participants with depressive symptoms compared with those with normal scores (CES-D > or = 16, HbA(1c)= 9.4% vs. CES-D < 16, HbA(1c)= 8.4%, P = 0.01). Factors associated with psychological distress included use of continuous subcutaneous insulin infusion (CSII) (P = 0.02) and increased frequency of hypoglycaemic episodes (P = 0.03). CSII users had higher CES-D (21.3 vs. 11.9, P = 0.001) and ASR-Total (59.7 vs. 53.0, P = 0.02) scores than non-CSII users. CONCLUSIONS: Approximately one-third of young adults with Type 1 diabetes experience psychological distress, which is associated with poorer glycaemic control. Psychological distress was related to frequency of hypoglycaemic episodes and method of insulin administration, with significantly greater distress being observed in those using CSII. These findings support inclusion of a psychologist in the diabetes team.

[Central pain processing in chronic low back pain. Evidence for reduced pain inhibition]. / Zentrale Schmerzverarbeitung bei chronischem Rückenschmerz. Hinweise auf verminderte Schmerzinhibition.

INTRODUCTION: A study of patients with low back pain (LBP) had revealed altered central pain processing. At an equal pain level LBP patients had considerably more neuronal activation in the somatosensory cortices than controls. In a new analysis of this dataset, we further investigated the differences in central pain processing between LBP patients and controls, looking for possible pathogenic mechanisms. METHODS: Central pain processing was studied by functional magnetic resonance imaging (fMRI), using equally painful pressure stimuli in a block paradigm. In this study, we reanalyzed the fMRI data to statistically compare pain-elicited neuronal activation of both groups. RESULTS: Equally painful pressure stimulation resulted in a significantly lower increase of regional cerebral blood flow (rCBF) in the periaqueductal gray (PAG) of the LBP patients. The analysis further revealed a significantly higher increase of rCBF in LBP than in HC in the primary and secondary somatosensory cortex and the lateral orbitofrontal cortex (LOFK), elicited by these same stimuli. CONCLUSIONS: These findings support a dysfunction of the inhibitory systems controlled by the PAG as a possible pathogenic mechanism in chronic low back pain.

Accidental tenfold overdose of propofol in a 6-month old infant undergoing elective craniosynostosis repair.

We report a 6-month-old male infant undergoing elective craniosynostosis repair who accidentally received a tenfold dose of propofol over a 4-h operative period. Myocardial dysfunction was observed after nearly 3 h of infusion; this could not solely be explained by the propofol overdose.

[The Lazarus phenomenon. Spontaneous return of circulation after unsuccessful intraoperative resuscitation in a patient with a pacemaker]. / Das Lazarus-Phänomen. Spontane Kreislaufstabilisierung nach erfolgloser intraoperativer Reanimation bei einem Patienten mit Herzschrittmacher.

We present a case of spontaneous recovery after failed intraoperative cardiopulmonary resuscitation in a patient with a cardiac pacemaker. Of the various mechanisms discussed in the literature, that which seems most relevant in our case of a Lazarus phenomenon, is that of impeded venous return in the course of positive pressure ventilation and hypovolemia.With the disconnection of ventilation, passive cardiac filling combined with electrical stimulation due to the cardiac pacemaker may have caused spontaneous myocardial electrical activity. Corresponding to previous authors,we recommend continued monitoring for 10 min after cessation of cardiopulmonary resuscitation. In addition, we propose a further attempt by disconnecting ventilation and external myocardial stimulation in the case of unresponsiveness to resuscitation efforts.

[Severe ethanol-intoxication mimics symptoms of intracranial hypertension]. / Schwere Ethanol-Intoxikation imitiert die Symptome einer intrakraniellen Drucksteigerung.

We report on a patient intoxicated with ethanol, who was found unconscious with fixed dilated pupils, deviation of eyes, absent cough and corneal reflexes, with her arms in decerebrate posture. Because of an orbital haematoma a traumatic intracranial hemorrhage with elevated intracranial pressure and compression or concussion of the brain stem was suspected. A CT-scan of the head did not reveal an intracranial mass or any radiographic signs of intracranial hypertension. Drug screening, blood gas analysis and other laboratory tests as well anamnestic data were not indicative of other intoxications or a metabolic decompensation. These unusual neurological findings were caused by a life-threatening ethanol intoxication with a blood-alcohol concentration of 700 mg/dl (151.8 mmol/l). The patient was extubated after several hours of mechanical ventilation in the intensive care unit and was discharged to a regular ward on the following day.

Effects of electrical stimulation of the Gasserian ganglion on regional cerebral blood flow after induced subarachnoid hemorrhage in pigs evaluated by 99mTc-HMPAO-SPECT.

It could be demonstrated that cervical spinal cord stimulation increases cerebral blood flow. The effects of electrical stimulation of the trigeminal ganglion in the acute phase of SAH in pigs were investigated. The experiments were carried out on 11 domestic pigs divided in two groups (group I: SAH [n = 5]; group II: SAH and trigeminal stimulation [n = 6]). In all animals a native SPECT was performed. The Gasserian ganglion was exposed for inserting the stimulation electrode. SAH was induced by injecting 10 ml autologous blood through a catheter placed in the suprasellar cistern. 30 minutes after SAH-induction electrical stimulation was started for two hours in group II (2.8-4.5 V, 50 Hz, 300 microseconds). 99mTc-HMPAO (400-540 MBq) was injected intravenously 110 minutes later. In group I 99mTc-HMPAO was applied after the same time interval. 80 minutes later SPECT was performed. Data were processed to calculate the uptake of radioactivity (%/kg tissue weight). The mean values were calculated for the different groups: native animal examination (%/kg tissue weight): 0.6343; group I: 0.468; group II: 0.6533. Comparing the mean values a highly significant difference between group I and group II (p < 0.01) and between native examination and group I (p < 0.01) could be found. No statistical significance could be detected on comparing the left/right-ratio in any ROI. The electrical stimulation of the Gasserian ganglion leads to a significantly increased uptake of 99mTc-HMPAO after induced SAH. Maybe the stimulation of the Gasserian ganglion constitutes a new therapeutic modality treating disturbed rCBF after SAH.

Huntington disease: DNA analysis in Brazilian population.

Huntington disease (HD) is associated with expansions of a CAG trinucleotide repeat in the HD gene. Accurate measurement of a specific CAG repeat sequence in the HD gene in 92 Brazilian controls without HD, 44 Brazilian subjects with clinical findings suggestive of HD and 40 individuals from 6 putative HD families, showed a range from 7 to 33 repeats in normal subjects and 39 to 88 repeats in affected subjects. A trend between early age at onset of first symptoms and increasing number of repeats was seen. Major increase of repeat size through paternal inheritance than through maternal inheritance was observed. Data generated from this study may have significant implications for the etiology, knowledge of the incidence, diagnosis, prognosis, genetic counseling and treatment of HD Brazilian patients.

[Standardized measurement of sound transmission of different middle ear prostheses]. / Standardisierte Messungen der Schallübertragung verschiedener Mittelohrprothesen.

The sound transmission properties of three different commercially available middle ear implants and the prototype of a complete middle ear prosthesis (CoMEP) were evaluated with a special measurement system that permits standardized conditions. This system uses a mechanical middle ear model (MMM) which approximates the impedances of the tympanic membrane and inner ear. The implants were fitted under defined conditions into the MMM. Displacement of the artificial stapes footplate of the MMM was measured with an optical probe. The measurements of different middle ear prostheses showed that the mass of the implant was the most important factor for optimum high frequency transmission. The lightest implant (4 mg, titanium) showed the best results. The CoMEP revealed the highest sensitivity because of a slight enlargement of the diameter of the artificial tympanic membrane. These findings show that the CoMEP is able to restore sound transmission to a normal range. In a second experiment one of the implants was fitted into the MMM with different forces. The increase of stiffness produced a slightly better high frequency transmission at the expense of low frequency sensitivity. Hence, the fitting of the implant may not be too stiff because of its loss of mobility within a significant frequency range.

[Using the Mongolian gerbil (Meriones unguiculatus) as an animal model in ontogenetic cochlear implant research]. / Einsatz der mongolischen Wüstenrennmaus (Meriones unguiculatus) als Tiermodell in der ontogenetischen Kochleaimplantatforschung.

One of the problems when providing prelingually deafened children with a cochlear implant is to find the optimal moment for the implantation in order to make optimal use of the sensitive phase in the maturation of the auditory system and to achieve the maximum improvement of hearing. Ontogenetic questions can be investigated in animal experiments under controlled conditions. In the present study an animal model (gerbil, Meriones unguiculatus) was established to simulate the situation of prelingually deafened children and to examine the influence of chronic electrostimulation on the development of the auditory pathway. We used species specific sounds for chronic stimulation to simulate significance of human language in our animal modell. A daily two-hour electrostimulation during the ontogenesis of early deafened animals lead to a partial compensation of the functional degeneration of the auditory pathway, which can be observed in totally deafened animals. This animal model provides an elaborate test design which can be used in CI research for the investigation of numerous problems.

Sequential hepatic and pulmonary resections for metastatic colorectal cancer.

BACKGROUND: Resection of pulmonary or hepatic colorectal metastases is associated with a 5-year survival rate of 25-40 per cent. This report analyses outcome following sequential resection of colorectal metastases to both organs. METHODS: Seventeen patients with histologically confirmed colorectal adenocarcinoma and resection of liver and lung metastases were identified from a prospective database. RESULTS: The median interval between resection of the primary tumour and first metastasis was 21 (range 0-64) months. The interval between resection of the first and subsequent metastases was 18 (range 1-74) months. No patient died in the postoperative period and there were two perioperative complications. The overall survival rate in 17 patients was 70 per cent at 2 years from resection of metastasis to the second organ, but the disease-free survival rate at 2 years was only 24 per cent. CONCLUSION: Although few long-term survivors were observed in this small series, sequential resection of hepatic and pulmonary metastases is warranted in a highly selected group of patients.

HLA-DQA1 and HLA-DQB1 alleles and haplotypes in two Brazilian Indian tribes: evidence of conservative evolution of HLA-DQ.

Nucleotide sequence polymorphism of the HLA-DQA1 and HLA-DQB1 class II genes was analyzed in the Kaingang and Guarani Amerindians from southern Brazil using PCR sequence-specific oligonucleotide typing methods. Four different DQA1-DQB1 haplotypes were found: DQA1*0401-DQB1*0402 (associated with DRB1*0802, DRB1*08041, and DRB1*0807), DQA1*0501-DQB1*0301 (associated with DRB1*1602, DRB1*1413, and DRB1*1402), DQA1*03-DQB1*0302 (associated with DRB1*0404 and DRB1*0411), and DQA1*03-DQB1*03032 (associated with DRB1*09012). These HLA-DQA1 and HLA-DQB1 alleles and haplotypes are common in many other populations of all major ethnic groups. Alleles and haplotypes introduced into the populations by post-Columbian admixture were seen at low frequency both in the Kaingang (3.2%) and in the Guarani (3.8%). No novel HLA-DQA1 and HLA-DQB1 alleles have thus far been identified in Amerindians. This differs from previous results for HLA-DRB1, another class II locus presenting novel alleles (i.e., alleles not found in other ethnic groups and probably generated after migration of paleo-Indians to the Americas) in the Guarani and in other South American Indian populations. The distribution of the HLA-DQ alleles and haplotypes in Amerindians indicates a weaker diversifying selective pressure on the HLA-DQ genes compared with HLA-DRB1 and HLA-B. The more conservative evolution of HLA-DQA1 and HLA-DQB1 compared with HLA-DRB1 is strong evidence of (still not well-defined) functional differences of these class II genes.

Allelic association of the cystic fibrosis locus and two DNA markers, XV2c and KM19, in 55 German families.

Allelic association between cystic fibrosis and two linked markers is demonstrated in a sample of 55 German families. It is shown by example how these observations can be used for genetic risk calculation.