[COVID-19: treatment strategies of German-speaking pediatric rheumatologists : Results of an online survey]. / COVID-19: Behandlungsstrategien der deutschsprachigen Kinderrheumatologen : Ergebnisse einer Online-Umfrage.

BACKGROUND: Reliable data on the course and treatment of pediatric COVID-19 ("corona virus disease 2019") in immunosuppressed patients with rheumatic diseases are missing. AIM: Delineation of individual strategies of the members of the Society for Pediatric Rheumatology (GKJR) in cases of COVID-19. METHODS: In May 2020 all GKJR members were invited to take part in an online survey. Opinion data regarding an approach using disease-modifying anti-rheumatic drugs (DMARD) in cases of COVID-19 as well as the readiness to use new therapeutic agents in patients in different stages of the disease were collected. RESULTS: A total of 71 respondents (27.3% of all contacted pediatric rheumatologists) took part in the survey. Of these 28.2% had treated patients with COVID-19. Over 95% of the respondents did not support a preventive adaptation of the anti-rheumatic treatment during the SARS-CoV­2 pandemic. In the case of outpatients under immunosuppression with proven COVID-19 more than 50% of the respondents would refrain from administering intravenous high-dose steroids, cyclophosphamide, anti-CD20 antibodies as well as BAFF, CTLA­4 and TNF-alpha blockades. Conversely, >70% of the respondents would continue the treatment with nonsteroidal anti-inflammatory drugs, hydroxychloroquine (HCQ), oral steroids, mycophenolate, IL­1 blockade and immunoglobulins (Ig). In the case of inpatients 74.6% of respondents would consider targeted COVID-19 treatment. In stable patients with oxygen treatment (stage I) HCQ (18.3%), azithromycin (16.9%) and Ig (9.9%) were most frequently used. In cases of early signs (stage II) or a manifest cytokine storm (stage III) anakinra (40.8% for stage II and 46.5% for stage III), tocilizumab (26.8% and 40.8%, respectively), steroids (25.4% and 33.8%, respectively) and remdesivir (29.6% and 38.0%, respectively) were most frequently used. The need for a personalized approach based on the current clinical situation was emphasized by many respondents. CONCLUSION: The currently low prevalence of COVID-19 in Germany limits the general clinical experience. Therefore, the presented results have to be interpreted with caution and mostly as hypothetical treatment considerations. It is to be expected that there will always be a limited amount of evidence on pediatric COVID-19; therefore, a continuous and critical exchange of expert opinions on the treatment strategies is important.

Disentangling the Free-Fall Arch Paradox in Silo Discharge.

Several theoretical predictions of the mass flow rate of granular media discharged from a silo are based on the spontaneous development of a free-fall arch region, the existence of which is still controversial. In this Letter, we study experimentally and numerically the particle flow through an orifice placed at the bottom of 2D and 3D silos. The implementation of a coarse-grained technique allows a thorough description of all the kinetic and micromechanical properties of the particle flow in the outlet proximities. Though the free-fall arch does not exist as traditionally understood--a region above which particles have negligible velocity and below which particles fall solely under gravity action--we discover that the kinetic pressure displays a well-defined transition in a position that scales with the outlet size. This universal scaling explains why the free-fall arch picture has served as an approximation to describe the flow rate in the discharge of silos.

X-linked agammaglobulinemia in community-acquired pneumonia cases revealed by immunoglobulin level screening at hospital admission.

In children with primary immunodeficiencies, the onset of symptoms precedes the diagnosis and the initiation of appropriate treatment by months or years. This delay in diagnosis is due to the fact that while these disorders are rare, some of the infections seen in immunodeficient patients are common. Defective antibody production represents the largest group among these disorders, with otitis, sinusitis and pneumonia as the most frequent initial manifestation. We performed a prospective study of humoral immunity in children hospitalized due to community-acquired pneumonia in tertiary care hospital. Out of 254 patients (131 boys, 123 girls, median age 4.5 years) recruited over 3 years, we found 2 boys (age 11 and 21 months) lacking serum immunoglobulins and circulating B cells. Subsequent genetic analysis confirmed diagnosis of X-linked agammaglobulinemia. Despite their immunodeficiency, the pneumonia was uncomplicated in both patients and did not call for immunological evaluation. However, the immunoglobulin screening at admission allowed for an early diagnosis of the immunodeficiency and timely initiation of immunoglobulin substitution, the key prerequisite for a favorable course of the disease.Simple and inexpensive immuno-globulin measurement during the manage-ment of hospitalized children with community-acquired pneumonia may help in early identification of patients with compromised humoral immunity and prevent serious complications.

[Care of infectious diseases in underage refugees exemplified by Ukraine]. / Infektiologische Versorgung minderjähriger Flüchtlinge am Beispiel der Ukraine: Vorgehen in Deutschland gemäß der S1-Leitlinine (AWMF-Register Nr. 048-017).

Background: By early June 2022, around 300,000 children and adolescents from Ukraine were registered in the German central registry for foreigners.The updated recommendations for action should provide the foundations for an evidence-based and targeted care for the diagnosis and prevention of infectious diseases in underage refugees and asylum seekers, exemplified by Ukraine. Objective: The recommendations for action are intended to support medical personnel in the care of minor refugees in order to1) ensure early recognition and completion of an incomplete vaccination status,2) diagnose and treat common infectious diseases,3) ensure early recognition and treatment of infectious diseases that are rare in the German healthcare system. Material and methods: The recommendations for action were drafted as level 1 (S1) guidelines coordinated by the Association of the Scientific Medical Societies in Germany (AWMF) and were adapted to the situation of refugees from Ukraine.The recommendations were compiled by a representative expert panel appointed by the participating professional societies in an informal consensus and finally officially adopted by the board of directors of all societies concerned. Results: Recommendations are given for the extent of the medical evaluation of minor refugees, including the medical history and physical examination, adapted to the situation of refugees from Ukraine. A blood count and screening for tuberculosis, hepatitis B and C as well as human immunodeficiency virus (HIV) infections are recommended for all minor refugees.For a rapid completion of the vaccination status, an age-related and indications-related prioritization of individual vaccinations will be undertaken. Conclusion: In view of the continuing high numbers of refugees not only from Ukraine, a further professionalization of medical health care is necessary. For this purpose, the necessary structural and personnel framework conditions need to be accomplished.

Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency.

Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαß+CD4-CD8- double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distribution of B cell subsets with raised transitional B cells and reduced marginal zone B cells, switched memory B cells and plasma blasts in most of 22 analyzed ALPS patients. Moreover, 5 out of 66 ALPS patients presented with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID). In patients presenting with lymphoproliferation, cytopenia, hypogammaglobulinemia and impaired B cell differentiation, serum biomarkers were helpful in addition to apoptosis tests for the identification of ALPS patients. Our observations may indicate a role for apoptosis defects in some diseases currently classified as CVID.

Flow-rate fluctuations in the outpouring of grains from a two-dimensional silo.

We present experimental results obtained with a two-dimensional silo discharging under gravity through an orifice at the flat bottom. High-speed measurements provide enough time resolution to detect every single bead that goes out and this allows the measurement of the flow rate in short-time windows. Two different regimes are clearly distinguished: one for large orifices, which can be described by Gaussian fluctuations, and another for small orifices, in which extreme events appear. The frontier between those two regimes coincides with the outlet size below which jamming events are frequent. Moreover, it is shown that the power spectrum of the flow-rate oscillations is not dominated by any particular frequency.

Prolonged impairment of polymorphonuclear cells functions in one infant with transient zinc deficiency: a case report.

BACKGROUND: Zinc is an essential trace element for the immune system. The zinc deficiency diminishes antibody- and cell-mediated responses in man. Lymphopenia and thymic atrophy are usually the early hallmarks of zinc deficiency. Surprisingly, only scarce data are available about polymorphonuclear cells (PMNs) functions in infants with zinc deficiency. We present the results of immunological analyses in one infant with transient zinc deficiency due to decreased zinc concentration in mother milk resulting in severe lactogenic acrodermatitis enteropathica. MATERIAL/METHODS: Nine repeated examination of oxidative burst of PMNs and immunoglobulin levels using nitroblue tetrazolium dye test, chemiluminescence, flow cytometry and nephelometry were performed in the infant with severe zinc deficiency during 28 months period. RESULTS: The unusual prolonged but transient impairment of PMNs respiratory burst accompanied with hypogammaglobulinaemia developed since the age of 2.5 months. Dramatic improvement of the skin was observed within days with total resolution of skin lesions on the 9th day of zinc therapy, but decreased PMNs respiratory burst persisted until the age of 23 months. CONCLUSIONS: We conclude that zinc deficiency may lead to prolonged impairment of polymorphonuclear cells functions and hypogammaglobulinaemia.

Mottled cyan opacification of the posterior cornea in contact lens wearers.

PURPOSE: We studied patients who had mottled cyan-colored opacities of the cornea to better understand the cause and prognosis of this entity. METHODS: We reviewed examinations of patients who had a mottled cyan opacification of the cornea. Risk factors, including contact lens wear and exposure to heavy metals, were analyzed. Clinical findings, pachymetry specular microscopy, and progression of the abnormality were noted. RESULTS: Six patients who had a mottled cyan opacification at the level of Descemet's membrane were identified. These opacities were located in the peripheral and midperipheral cornea. All patients had bilateral findings, had visual acuities of 20/20 or better, and were asymptomatic. All patients had worn soft contact lenses bilaterally for periods ranging from seven to 14 years. CONCLUSION: All patients had the similar clinical appearance of a mottled cyan opacification at the level of Descemet's membrane in the peripheral cornea. Long-term contact lens wear appears to be associated; however, the exact cause is unclear.

[Anesthesia adjacent to the spine in vascular surgery in high-risk patients]. / Rückenmarksnahe Anästhesieverfahren für gefässchirurgische Eingriffe bei Risikopatienten.

From 1978 to 1982, 515 patients underwent vascular surgery with 230 operations performed under epidural analgesia and 330 operations under spinal anaesthesia. In the case of vascular surgery on the lower limbs epidural analgesia was effective in 168 patients (97.1%) and ineffective in five (2.9%), spinal anaesthesia was effective in 312 patients (98.7%) and ineffective in four (1.3%). Epidural analgesia was effective for vascular surgery on the lower abdomen in 40 patients (70.2%), and inadequate in 17 patients (29.8%), whilst spinal anaesthesia was adequate in 9 patients (64%) and inadequate in 5 patients (36%). Nevertheless, lumbar spinal and epidural analgesia may be the method of choice in high-risk patients undergoing vascular surgery, especially on the lower limbs.

Ocular trauma. Triage and treatment.

Many types of ocular trauma can be diagnosed and treated in the primary care office, particularly if a slit lamp is available. Treatment for corneal abrasions consists of applying a cycloplegic medication, antibiotic ointment, and a patch (unless a corneal ulcer is suspected). Iritis can be treated with cycloplegics and topical corticosteroids; the prescribing physician should be familiar with the potential ocular side effects and complications. Hyphemas are treated with bed rest, topical atropine sulfate drops and topical corticosteroids, as well as measures to prevent rebleeding. A slit lamp, topical anesthesia, and a foreign-body spud greatly facilitate the removal of foreign bodies from the cornea. Acid and alkali burns should be irrigated until the pH is normal and then should be treated like a corneal abrasion. Lid lacerations must be repaired with care to preserve proper functioning of the lid. Ruptures of the globe are serious injuries requiring surgical repair and long-term follow-up.

Sudden nontraumatic visual loss. The challenge in primary care.

A number of causes need to be considered in the patient with acute, nontraumatic visual loss at or below the 20/200 level. Etiologic factors include several anterior segment diseases, as well as posterior segment (vitreoretinal) and retrobulbar (optic nerve) conditions. Normal slit-lamp and funduscopic findings make identification of the true cause of decreased visual acuity more difficult. The physician must not rush immediately to the diagnosis of functional disease without first ruling out the other causes.

[Lactate in the diagnosis of acute intestinal vascular occlusions]. / Lactat zur Diagnose akuter intestinaler Gefässverschlüsse.

The diagnostic value of lactate in acute occlusion of intestinal vessels was investigated in two groups of patients: Group 1 included 86 patients with reconstructions of aorta and intestinal arteries: The lactate-concentrations measured during uncomplicated postoperative course over 24 hours remained within the normal range of 4.31 +/- 1.06 mmol/l. Only in 3 patients with acute postoperative occlusion of intestinal arteries, lactate rose to high pathologic levels up to 59.94 mmol/l. In 10 patients after reconstruction of acute mesenteric artery occlusion the postoperative lactate levels enforced the decision in 9 cases against and in one patient to second look operation. Group 2 included 36 patients with acute abdomen: In 18 patients with intestinal vascular occlusions the mean value of lactate was 7.45 +/- 2.86 mmol/l, in 18 patients without intestinal ischemia lactate was only 1.94 +/- 1.02 mmol/l. The statistical difference is significant. In summary, the results of this study document the reliable value of lactate for the diagnosis of acute occlusions of intestinal vessels during pre- and postoperative course.

[Insertion and removal of intravaginal tampons during estrus synchronization in heifers]. / Aplikace a vyjímání intravaginálních tampónu pri synchronizaci ríje jalovic.

In eight trials, 197 heifers were synchronized with gestagen chlorsuperlutin (CSL). The intravaginal tampons soaked with chlorsuperlutin were introduced in the vagina by means of an applier by a vaginal speculum. Silon threads were attached to the tampons, by which they were pulled out after treatment. The mentioned method of application removes the great physical exertion needed for manual application and increases labour productivity in tampon application by about 200%. The hazard of tampon contamination is reduced to the minimum. Of the two tampon types tested (ester and ether type) the ester type was found to be better for not needing sterilization if the disinfectant Aseptin is dropped upon it prior to application. The retention of the tampons was 94.9%. Eighty-four per cent of the retained tampons could be removed by pulling the silon threads. About 40 to 60% of CSL is excreted from the tampons during the treatment. If the initial content of CSL is increased, its excretion is raised as well.

Evaluation of health care related web resources based on web citation analysis and other quality criteria.

Automatic system for ranking of educational health care related web resources based on quality criteria was developed. Automatic retrieval of new web resources from well-known directories followed by their automatic evaluation is a keystone of the system. The system provides this information: a) location of the resource and its author, b) number of back links to the resource, c) checks presence of electronic labels of quality. Currently the system encompasses more than 500 links to educational web resources divided into 47 medical specialities. The resources in all categories are ranked according to their link popularity, the electronic labels of quality are presented. History of link popularity is recorded and might be displayed at every resource. There are 2 language editions. The system operates automatically, the editors check and correct the retrieved values. The described system adds to webcite indexing criteria for objective evaluation of quality of webpages. It is useful for the selection of optimum education resources in health care.

[Compression of the trachea by a hematoma caused by an internal jugular vein catheter]. / Kompression der Trachea durch ein Hämatom nach Vena jugularis interna-Katheter.

In a patient with thrombocytopenia, respiratory obstruction because of a hematoma occurred following internal jugular vein cannulation. This patient recovered completely after surgical intervention. With the following precautions in mind, puncture of the internal jugular vein in patients with coagulopathies has a high success rate and does not result in severe complications: Internal jugular vein cannulation by an experienced physician; optimal conditions for puncture by increasing venous pressure and diameter with slight Trendelenburg position and Valsalva maneuver in patients with spontaneous breathing, or positive end-expiratory pressure in patients with artificial ventilation; catheter insertion by the Seldinger technique; manual compression of the puncture site for 10-15 min; and vein puncture with ultrasonographic aid if possible.

[Neurological complications secondary to subclavian venipuncture (author's transl)]. / Neurologische Komplikationen beim Vena subclavia-Katheterismus.

Report on 2 cases of transient phrenic nerve paresis respectively stationary brachial plexus paresis secondary to percutaneous subclavian venipuncture. In retrospect, the disregard of unfavourable anatomical conditions at the site of venipuncture was an important pathogenic factor for this neurological complications.

[Halothane-intoxication by failure of the halothane-vaporizer]. / Halothanüberdosierung durch defekten Halothanverdunster

Regular measurement of the Halothane-concentration delivered by the output of the vaporizer is necessary to avoid a clinically dangerous situation due to Halothane-overdosage.

[Dopamin-therapy for acute postoperative renal failure (author's transl)]. / Dopamin zur Therapie der akuten postoperativen Niereninsuffizienz

In 10 out 14 patients with acute postoperative renal failure the administration of a dopamin-drip (175-350 mcg/min) resulted in an increase in urinary output more than 30 ml/h. But there was no decrease of the high lethality by postoperative renal failure.

[Intraoperative cardiac arrest and postoperative coma secondary to flunitrazepam (Rohypnol) (author's transl)]. / Intraoperativer Herzstillstand und postoperatives Koma durch Flunitrazepam (Rohypnol).

Report on a case of cardiac arrest and coma lasting 10 hours secondary to the i.v. administration of 2 mg Flunitrazepam. The possible cardiorespiratory depressant respectively cerebral side-effects of Flunitrazepam are discussed. The high concentration of Flunitrazepam in the registered ampules is misleading to overdosage.