Juvenile ecthyma gangrenosum caused by Pseudomonas aeruginosa revealing an underlying neutropenia: case report and review of the literature.

BACKGROUND: Ecthyma gangrenosum (EG) is characterized by the occurrence of erythematous, violaceous or haemorrhagic macules and/or vesicles, often evolving into necrotic ulcers, with a central grey-black eschar. It is a rare skin condition, usually occurring in immunocompromised patients suffering from bacterial sepsis caused by Pseudomonas aeruginosa. However, seemingly healthy children have been diagnosed with this skin disease as well. OBJECTIVES: We report the workup of a case of vulvar EG caused by P. aeruginosa in a toddler, which led to a diagnosis of an underlying neutropenia. Moreover, we provide a brief literature review on those cases of EG where an underlying primary immunodeficiency, neutropenia in particular, was eventually diagnosed. METHODS: A one-and-a-half-year-old girl presented with a history of recurrent (respiratory) infections and the sporadic occurrence of purpuric, vulvar ulcers. Workup consisted of microbiological and haematological investigations, including repeated blood analyses. RESULTS: Bacterial swabs from the vulvar ulcers showed the growth of P. aeruginosa. No concomitant sepsis was present, but laboratory investigations pointed towards a cyclic neutropenia, coinciding with the occurrence of the EG lesions. Topical gentamicin ointment allowed the skin lesions to heal faster. Following the administration of granulocyte colony-stimulating factor (G-CSF), the girl experienced less infections in general and had no recurrence of EG lesions in particular. Treatment with G-CSF could eventually be stopped, and the neutropenia, ultimately transient in nature, completely resolved. CONCLUSION: Children presenting with (anogenital) EG should always alert a physician to consider a potentially underlying immunodeficiency, neutropenia in particular.

Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study.

BACKGROUND: The prevalence and causes of sensorineural hearing loss (SNHL) in children with Down syndrome (DS) are poorly delineated. OBJECTIVE: To describe the prevalence, severity, laterality and underlying etiology of SNHL in a cohort of children with DS. METHODS: A cross-sectional study was performed among all children with DS followed at the multidisciplinary Downteam of the Antwerp University Hospital. Patients' characteristics, risk factors for hearing loss, audiometric data and results of an etiological work-up were collected. RESULTS: Among 291 patients in follow-up, 138 patients (47.4%) presented with hearing loss. In the majority this was caused by middle ear effusion and only 13 patients (4.5%) had sensorineural hearing loss, 7 boys and 6 girls with a mean age of 14.4 ±â€¯7.4 years. Hearing loss was bilateral in 8 cases. Hearing loss severity was graded as mild in 38.5%, moderate in 30.8% and profound in 30.8% of the patients. An etiological work-up was completed in 9 children. Four patients presented with single sided deafness due to cochlear nerve deficiency. One patient had a genetic cause and in 2 patients the hearing loss was attributed to excessive noise exposure. The etiology of hearing loss was unknown in 6 patients. CONCLUSION: Sensorineural hearing loss is uncommon in children with DS with a prevalence of 4.5%. Etiological work-up may allow identifying a specific underlying cause. Cochlear nerve deficiency was found in 4 children with DS and single sided deafness.

Species boundaries in the Astragalus cusickii complex delimited using molecular phylogenetic techniques.

Understanding the source of phenotypic variability is a challenge in the biological sciences. Variation in phenotypes is the result of variation in the genetics and environment the organism experiences, but elucidating the relative contribution of these two parameters can pose problems, especially in the field of systematics. Systematists are challenged to classify biological diversity into groups that share common ancestry. Phenotypic variation can be useful to demonstrate common ancestry, but only when the primary contributor to the variation is under strong genetic control, and thus heritable. Cusick's milkvetch (Astragalus cusickii) is a perennial forb endemic to the northwestern intermountain region of the United States. The species currently comprises four varieties based on subtle morphological dissimilarities, such as leaf size and density, and the size and shape of the seed pods. The taxonomic organization of the varieties of A. cusickii and related species of Astragalus were reexamined through phylogenetic analysis of low copy nuclear, nuclear-ribosomal, and chloroplast gene regions. Maximum parsimony, maximum likelihood, Bayesian inference, the genealogical sorting index, and an approximately unbiased test were used to determine appropriate species boundaries under the phylogenetic species concept. The results support reclassification of A. cusickii var. packardiae and A. cusickii var. sterilis as separate species. Additionally, evidence suggests a chloroplast capture event may have occurred in one population of A. cusickii var. packardiae.

Correction: New tetranuclear manganese clusters with [MnII3MnIII] and [MnII2MnIII2] metallic cores exhibiting low and high spin ground state.

Correction for 'New tetranuclear manganese clusters with [MnII3MnIII] and [MnII2MnIII2] metallic cores exhibiting low and high spin ground state' by M. Sobocinska et al., Dalton Trans., 2016, 45, 7303-7311.

New tetranuclear manganese clusters with [MnMn(III)] and [MnMn] metallic cores exhibiting low and high spin ground state.

Two tetranuclear mixed-valent clusters, [MnMn(III)Cl(Ph3CCOO)4(CH3OCH2CH2O)4(CH3CN)]·0.4C6H5CH3·0.6CH3CN () with an unprecedented [MnMn(III)] core and [MnMnCl4(CH3OCH2CH2O)6] (), were synthesized and characterized by single-crystal X-ray diffraction and magnetic measurements. Their properties were analyzed in the framework of phenomenological modelling and DFT calculations, showing acceptable agreement between theory and experiment. Both building blocks [MnMn(III)] and [MnMn] provide good examples of bipartite systems with the lowest ST = 1/2 and highest ST = 9 magnetic ground states available for them. The topology of the magnetic interactions in the [MnMn(III)] core provides a suitable template for the molecular qubit implementation and the stability of the spin-1/2 ground state strongly depends on the antiferromagnetic Mn(II)-Mn(II) coupling.

(1)H NMR relaxometry and quadrupole relaxation enhancement as a sensitive probe of dynamical properties of solids--[C(NH2)3]3Bi2I9 as an example.

(1)H nuclear magnetic resonance relaxometry has been applied to reveal information on dynamics and structure of Gu3Bi2I9 ([Gu = C(NH2)3] denotes guanidinium cation). The data have been analyzed in terms of a theory of quadrupole relaxation enhancement, which has been extended here by including effects associated with quadrupole ((14)N) spin relaxation caused by a fast fluctuating component of the electric field gradient tensor. Two motional processes have been identified: a slow one occurring on a timescale of about 8 × 10(-6) s which has turned out to be (almost) temperature independent, and a fast process in the range of 10(-9) s. From the (1)H-(14)N relaxation contribution (that shows "quadrupole peaks") the quadrupole parameters, which are a fingerprint of the arrangement of the anionic network, have been determined. It has been demonstrated that the magnitude of the quadrupole coupling considerably changes with temperature and the changes are not caused by phase transitions. At the same time, it has been shown that there is no evidence of abrupt changes in the cationic dynamics and the anionic substructure upon the phase transitions.

Two cases of rickets presenting with poor growth, hypotonia, and respiratory problems.

Rickets is a rare disease in developed countries. In children, it is a disease which affects growing bone. Depending on the severity, it can present with a wide variety of symptoms. Because it is such a rare disease in developed countries, symptoms suggesting rickets are often not easily recognized. This can cause a delay in diagnosing and treating rickets. Often unnecessary and sometimes invasive investigations are performed. First leading clues to rickets on physical examination are poor growth, especially length, thickening of wrists, bow legs, and craniotabes. At further examination, special attention should be paid to osteopenia and cupping and fraying at the metaphyses on X-rays. Laboratory results suggestive for rickets are elevated alkaline phosphatase and disturbances in calcium and phosphate homeostasis. In this report, we present two cases presenting with poor growth, severe pain, and respiratory problems secondary to calcipenic rickets.

Orbital wall infarction in child with sickle cell disease.

We present the case of a 17-year-old boy, known with homozygous sickle cell disease, who was admitted because of generalised pain. He developed bilateral periorbital oedema and proptosis, without pain or visual disturbances. In addition to hyperhydration, oxygen and analgesia IV antibiotics were started, to cover a possible osteomyelitis. Patients with sickle cell disease are at risk for vaso-occlusive crises, when the abnormally shaped red blood cells aggregate and block the capillaries. Such a crisis typically presents at a location with high bone marrow activity, as the vertebrae and long bones. At an early age, the bone marrow is still active at other sites, for example the orbital wall, and thus infarction can also occur there. Thus, in young persons with sickle cell disease, it is important to consider orbital wall infarction in the differential diagnosis, since the approach is different from osteomyelitis. If the disease is complicated by an orbital compression syndrome, corticosteroids or surgical intervention may be necessary to preserve the vision. In our patient, an MRI of the orbitae demonstrated periorbital oedema with bone anomalies in the orbital and frontal bones, confirming orbital wall infarction. Ophthalmological examination revealed no signs of pressure on the nervus opticus. The patient recovered gradually with conservative treatment.

Dynamics of ferroelectric bis(imidazolium) pentachloroantimonate(III) by means of nuclear magnetic resonance 1H relaxometry and dielectric spectroscopy.

Some of haloantimonates(III) and halobismuthates(III) are ferroelectric. Bis(imidazolium) pentachloroantimonate(III), (C3N2H5)2SbCl5 (abbreviation: ICA) is the first example of such compounds with a one-dimensional anionic chain which exhibits ferroelectric properties. The relation between the ionic dynamics and network structure and the ferroelectric features is not clear. Here Nuclear Magnetic Resonance (NMR) (1)H spin-lattice relaxation experiments at 25 MHz are reported for ICA in the temperature range of 80 K-360 K, covering ferroelectric-paraelectric and structural phase transitions of the compound occurring at 180 and 342 K, respectively. The relaxation process is biexponential in the whole temperature range indicating two dynamically nonequivalent types of imidazolium cations. Temperature dependences of both relaxation contributions allow for identifying three motional processes. Two of them are cation-specific - i.e. they are attributed to the two types of imidazolium cations, respectively. The third process involves both types of cations, and it is characterized by much lower activation energy. Moreover, the relaxation data (combined with (1)H second moment measurements) show that the ferroelectric-paraelectric phase transition mechanism is governed, to a large extent, by the anionic network arrangement. The NMR studies are complemented by dielectric spectroscopy experiments performed in the vicinity of the Curie temperature, TC = 180 K, to get insight into the mechanism of the ferroelectric-paraelectric phase transition. The dielectric dispersion data show critical slowing down of the macroscopic relaxation time, τ, in ICA when approaching TC from the paraelectric side, indicating an order-disorder type of ferroelectrics.

A cross-sectional analysis of otitis media with effusion in children with Down syndrome.

UNLABELLED: Children with Down syndrome are at risk to develop otitis media with effusion (OME). We performed a retrospective and cross-sectional analysis to evaluate the prevalence of OME in children with Down syndrome (DS) for consecutive age categories between 6 months and 12 years. Clinical and audiometric data were available for 107 children followed in a multidisciplinary Down team. A high prevalence of OME was found at the age of 1 year (66.7 %), with a second peak prevalence of 60 % at 6-7 years. A declining trend was observed in children ≥8 years. Overall, 52.3 % of DS children had either OME or ventilation tubes at the time of evaluation. Hearing thresholds were significantly higher in children with bilateral OME (median 36.7 decibel hearing level (dB HL), range 26.7-46.1) compared to those with at least one normally ventilated middle ear (median 28.3 dB HL, range 22.8-3.3), p = 0.013. CONCLUSION: We found a high prevalence of OME in children with Down syndrome, with a peak of ≥60 % around 1 and 6-7 years. A declining trend is seen in older children. Mild to moderate hearing loss was present in children with bilateral OME.

Epidemiology of pertussis in children of Flanders Belgium: can healthcare professionals be involved in the infection?

Pertussis is a communicable disease whose registration incidence in the Flemish region of Belgium has increased since 2003. Originally, this increment was obvious only in the province of Antwerp, but since 2012, there has also been an increase in the registered cases of pertussis in other Flemish provinces. The overall registration incidence for pertussis in Flanders was 5·6 per 100 000 in 2012. The majority of these cases were identified among young children, but older children and adults were also affected. Increased awareness, new diagnostic tools, better registration, waning immunity, and circulation of new strains were most likely associated with the increase in reporting. Although many of the pertussis infections we studied occurred within family units, several healthcare workers who had been in contact with young children were also identified as sources of pertussis. A number of these were index cases, while others were secondary infections. Finally, a fatal neonatal pertussis case is presented to illustrate the severity of the disease in young unvaccinated children.

Ectopic pancreatic tissue at the umbilicus in childhood: a case report and review of the literature.

Ectopic pancreatic tissue at the umbilicus is very rare. To our best knowledge, only fourteen cases of ectopic pancreatic tissue at the umbilicus are reported. In this paper we present the case of a two-year-old boy with an abrasion at the umbilicus. He had a poorly healing scar that started bleeding after recurrent injuries. Abdominal ultrasound revealed an unclear cystic structure with no communication to intra-abdominal structures. Surgical resection was performed without complications. Histology diagnosed an ectopic pancreatic tissue with reactive epidermal changes. We present a review of the literature and the clinical manifestations and treatment of the previously reported fourteen cases.

Encapsulating peritoneal sclerosis in a peritoneal dialysis patient with prune-belly syndrome: a case report.

This case describes a prune-belly syndrome patient who had a kidney transplantation and was diagnosed with Encapsulating Peritoneal Sclerosis (EPS), a rare but potentially fatal condition, mostly associated with Peritoneal Dialysis (PD). The definition of EPS is based on the clinical findings linked to bowel obstruction and on the demonstration of peritoneal thickening. Surgical treatment is the only established basic treatment for the condition. Prune-belly syndrome is characterized by the triad of deficient abdominal musculature, urinary tract abnormality and cryptorchidism. Because it is often associated with end-stage renal disease, PD is essential in the treatment of patients with prune-belly syndrome. The aetiology of EPS follows a 'two-hit theory': the first 'hit' is peritoneal deterioration, caused by long-time exposure to PD. This causes peritoneal disruption which predisposes the patient to a second hit. In our patient, PD discontinuation and renal transplantation are possible 'second hits' that triggered the development of EPS. This case of prune-belly syndrome has all the necessary elements for the development of EPS, and we felt we should report it as the peroperative diagnosis was unexpected.

A solid pseudopapillary tumour of the pancreas presenting after an abdominal trauma.

We present the case of 12-year-old girl who was referred with persistent abdominal pain and a palpable mass after blunt trauma and whose final diagnosis was a solid pseudopapillary tumour of the pancreas. This is the second case in our hospital of a solid pseudopapillary tumour of the pancreas presenting after a blunt abdominal trauma. Solid pseudopapillary tumour of the pancreas is a rare neoplasm. It comprises 2 to 3% of primary pancreatic tumours occurring at all ages. It was first described in 1959 by Frantz. The presenting symptoms are usually a slow growing abdominal mass with vague abdominal discomfort. Sometimes they are discovered after a trauma. Solid pseudopapillary tumours of the pancreas have a fairly characteristic appearance on imaging but the final diagnosis depends on histological confirmation. After resection the prognosis is excellent.

Carbohydrate absorption by blackcap warblers (Sylvia atricapilla) changes during migratory refuelling stopovers.

Passerine birds migrating long distances arrive at stopover sites to refuel having lost as much as 50% of their initial body mass (mb), including significant losses to digestive organs that may serve as a reservoir of protein catabolised for fuel during flight. Birds newly arrived at a stopover show slow or no mb gain during the initial 2-3 days of a stopover, which suggests that energy assimilation may be limited by reduced digestive organs. Measurements of migrants and captive birds subjected to simulated migratory fasts have shown reductions in intestine mass, morphological changes to the mucosal epithelium, and reductions in food intake and assimilation rate upon initial refeeding. We found that blackcaps (Sylvia atricapilla, Linnaeus) newly arrived at a migratory stopover after crossing the Sahara and Sinai deserts had significantly increased paracellular nutrient absorption (non-carrier mediated uptake occurring across tight junctions between enterocytes) that may provide partial compensation for reduced digestive capacity resulting from changes to intestinal tissues. Indeed, newly arrived birds also had a slightly reduced capacity for absorption of a glucose analogue (3-O-methyl-D-glucose) transported simultaneously by both carrier-mediated and non-mediated mechanisms. Increased paracellular absorption coupled with extended digesta retention time may thus allow migratory blackcaps to maintain high digestive efficiency during initial stages of refuelling while digestive organs are rebuilt.

Intralobar bronchopulmonary sequestration with mixed venous drainage in a 10-year-old girl.

Bronchopulmonary sequestration consists of a mass of abnormal lung tissue that has no normal connection with the bronchial tree and is supplied with blood from an aberrant artery mostly originating in the thoracic aorta. Two forms are recognized: intralobar and extralobar sequestration. The first is localized within the normal visceral pleura and has a venous drainage into the pulmonary system; the latter is localized without the normal lung in its own pleura with venous drainage into the systemic venous system. Intralobar sequestration is the most common form accounting for 75% of the cases. Intralobar sequestration usually presents in adolescence or adulthood with signs of recurrent pneumonia. Extralobar sequestration presents early in life with respiratory distress or feeding difficulties and is frequently associated with other congenital malformations. The diagnosis is confirmed by CT scan of the lungs and magnetic resonance angiography as demonstration of the aberrant vascular supply is essential for the diagnosis. Therapy consists in surgical removal. We present a case of intralobar sequestration in a 10-year-old girl. The clinical symptomatology was typical. Arterial supply with two aberrant arteries and mixed venous drainage into the pulmonary and systemic systems were particular features.

Effects of confinement and crowding on folding of model proteins.

We perform molecular dynamics simulations for a simple coarse-grained model of crambin placed inside of a softly repulsive sphere of radius R. The confinement makes folding at the optimal temperature slower and affects the folding scenarios, but both effects are not dramatic. The influence of crowding on folding are studied by placing several identical proteins within the sphere, denaturing them, and then by monitoring refolding. If the interactions between the proteins are dominated by the excluded volume effects, the net folding times are essentially like for a single protein. An introduction of inter-proteinic attractive contacts hinders folding when the strength of the attraction exceeds about a half of the value of the strength of the single protein contacts. The bigger the strength of the attraction, the more likely is the occurrence of aggregation and misfolding.

Ca(2+) -dependent plant response to Pb(2+) is regulated by LCT1.

Tobacco plants transformed with TaLCT1 were cultured on Knop's medium with modified calcium concentrations (0.01-3 mM) in the presence of Pb(2+), and in soil contaminated by lead. A 4-5 microM Pb(2+) administered in the presence of 1 mM Ca(2+) inhibited the root growth of transgenic plants to much lesser degree than of control plants, whereas in the presence of 3mM Ca(2+) no differences were found between the studied lines. The reduction of Pb(2+) toxicity in the presence of 1 mM Ca(2+) was not accompanied by a change in the lead tissue concentration. However, when Ca(2+) level in the medium was lowered to 0.01 mM, several fold higher root/shoot Pb ratio in transgenic plants was observed, twofold increase in the total amount of metal accumulated, and lower concentration of Pb in the xylem sap. Results suggest the involvement of TaLCT1 in the regulation of Ca-dependent Pb-detoxification, and under conditions of low calcium in lead uptake and distribution.

[Minimally invasive unicondylar knee replacement with computer navigation]. / Minimal-invasive navigiert implantierte unikondyläre Knieendoprothese.

METHODS: From January 2003 to July 2005 a total of 50 St Georg medial knee monosleds with metal-backed tibial components in stably seated form and Uniglide prostheses (Alphanorm/Corin) with mobile bearing onlays were implanted in minimally invasive operations. Postoperatively the alignment-especially of the tibial components-was investigated, to check whether we had achieved the dorsal slope we had been aiming at, i.e. 5-7 degrees , in the region of these knee replacements. RESULTS: Before surgery there were initial malalignments of up to 10 degrees varus and 3 degrees valgus. All leg axes were restored to between -3 degrees and +3 degrees . The desired dorsal slope of 5-7 degrees for the knee monosleds relative to the tibial component was realized, the average slope being 5.3 degrees . The a-p alignment of the tibial component and of the femoral component was correct. CONCLUSIONS: Use of the navigation system leads to more accurate and reproducible results in terms of tibial dorsal slope, which is extremely important when these monosleds are used. Overcorrection of the leg axis is generally avoided. The use of too-high medial onlays is also reliably avoided by the navigation system's monitoring of the level of the cut.