Refining the Pediatric Multiple Organ Dysfunction Syndrome

Since its introduction into the medical literature in the 1970s, the term multiple organ dysfunction syndrome (or some variant) has been applied broadly to any patient with >1 concurrent organ dysfunction. However, the epidemiology, mechanisms, time course, and outcomes among children with multiple organ dysfunction vary substantially. We posit that the term pediatric multiple organ dysfunction syndrome (or MODS) should be reserved for patients with a systemic pathologic state resulting from a common mechanism (or mechanisms) that affects numerous organ systems simultaneously. In contrast, children in whom organ injuries are attributable to distinct mechanisms should be considered to have additive organ system dysfunctions but not the syndrome of MODS. Although such differentiation may not always be possible with current scientific knowledge, we make the case for how attempts to differentiate multiple organ dysfunction from other states of additive organ dysfunctions can help to evolve clinical and research priorities in diagnosis, monitoring, and therapy from largely organ-specific to more holistic strategies.

Molecular study of Pompe disease in Egyptian infants

Background Pompe disease (PD) is a serious genetic disorder caused by deficiency of acid α-glucosidase (GAA) and subsequent glycogen accumulation inside lysosomes. This study included a cohort of 5 Egyptian infants (1–8 months old) with far lower than average normal GAA activity and clinical signs of PD in 4 of the 5 cases. The fifth case was discovered by newborn screening (NBS). Molecular analysis of the GAA gene was performed to confirm the diagnosis and identify the underlying mutation. Results The study identified the causative mutations [c.1193T > C (p.Leu398Pro), c.1134C > G (p.Tyr378*) & c.1431del (p.Ile477Metfs*43)] in 4 cases. However, molecular analysis reversed the expected pathologic state in the fifth infant, where his reduced enzymatic activity was related to the presence of pseudodeficiency allele c.868A > G (p.Asn290Asp) in addition to heterozygous disease-causing mutation c.2238G > C (p.Trp746Cys). Conclusion This study presents the first molecular analysis of GAA gene in Egypt and has thrown some light on the importance of PD molecular diagnosis to provide precise diagnosis and enable therapeutic commencement in affected subjects.

Assessing the quality of root canal filling and instrumentation time using kedo-s files, reciprocating files and k-files

Objective: Pulpectomy is the conservative treatment approach that retains and preserves the primary tooth in the dental arch in its normal function and non-pathologic state until its exfoliation. Over the years, pulpectomy has been performed using various instrumentation techniques. The aim of this study was to compare the quality of root filing and instrumentation time using Kedo-S files, Reciprocating files and K-files in primary teeth. Material And Methods: A randomized clinical trial was performed on 45 primary molars equally distributed for instrumentation with Kedo-S files, reciprocating files and K-Files. Immediate post-operative digital radiographs were taken to evaluate the quality of root filing and root canal instrumentation time was also recorded. Results: Mean instrumentation time with Kedo-S was 75.6 seconds, reciprocating file was 190.6 seconds and K-file was 95.4 seconds. Highest optimal fill was obtained with Kedo-S file group, highest overfill was obtained with Kedo-S group and highest under fill was obtained with reciprocating file group. Conclusion: Kedo-S rotary system provides better quality of root canal filling in minimum instrumentation time.KEYWORDS Pulpectomy; Primary teeth; Root canal filling; Rotary files.

Staphylococcus által kiváltott, immunglobulin-E-alapú anafilaxiás reakciók Crohn-betegségben. Egy tünetegyüttes első leírása

Immunglobulin E (IgE)-based, irregularly recurring, severe anaphylactic reactions occurred in a 50-year-old European white male patient suffering also from Crohn’s disease. On the base of immunologic laboratory tests concerning the mechanism of the phenomenon, the idea arose whether molecules derived for certain microbial derivatives could enter the blood circulation via the damaged bowel walls in the patient with Crohn’s disease and they might act as allergens. The microbial analysis diagnosed atypical Staphylococcus in the stool. The serum level of IgE was very high. The concomitant use of targeted antibiotics and anti-allergy and immunosuppressive agents resulted in a complete remission during a couple of months. Not only Crohn’s disease has improved, but also the total serum IgE level has decreased significantly, and the unpredictable anaphylactic attacks have been completely eliminated. In Crohn’s disease, the anaphylactic complications induced by atypical microbial allergens (e.g., derivatives of Staphylococcus) can be effectively treated after the recognition of this pathological mechanism. This is the first description of such a pathologic state. Orv Hetil. 2019; 160(38): 1514–1518.

A simple approximation to bias in gene-environment interaction estimates when a case might not be the case

ABSTRACTCase-control genetic association studies are often used to examine the role of the genetic basis in complex diseases, such as cancer and neurodegenerative diseases. The role of the genetic basis might vary by non-genetic (environmental) measures, what is traditionally defined as gene-environment interactions (GxE). A commonly overlooked complication is that the set of clinically diagnosed cases might be contaminated by a subset with a nuisance pathologic state that presents with the same symptoms as the pathologic state of interest. The genetic basis of the pathologic state of interest might differ from that of the nuisance pathologic state. Often frequencies of the pathologically defined states within the clinically diagnosed set of cases vary by the environment. We derive a simple and general approximation to bias in GxE parameter estimates when presence of the nuisance pathologic state is ignored. We then perform extensive simulation studies to show that ignoring presence of the nuisance pathologic state can result in substantial bias in GxE estimates and that the approximation we derived is reasonably accurate in finite samples. We demonstrate the applicability of the proposed approximation in a study of Alzheimer’s disease.

Diffusion in Tube Dialyzer

Nowadays, kidney failure is a problem of many peoples in the world. We know that the main function of kidney is maintaining the chemical quality of blood particularly removing urea through urine. But when they malfunction, the pathologic state known as uremia results in a condition in which the urea is retained in the body. Failure of the kidney results in building up of harmful wastes and excess fluids in the body. Kidney diseases (failures) can be due to infections, high blood pressure (hypertension), diabetes, and/or extensive use of medication. The best form of treatment is the implantation of a healthy kidney from a donor. However, this is often not possible due to the limited availability of human organs. Chronic kidney failure requires the treatment using a tube dialyzer called dialysis. Blood is taken out of the body and passes through a special membrane that removes waste and extra fluids. The clean blood is then returned to the body. The process is controlled by a dialysis machine (tube dialyzer) which is equipped with a blood pump and monitoring systems to ensure safety. So this article investigates the real application of mathematics (diffusion) in medical science, and it also contains the mathematical formulation and interpretation of tube dialyzer in relation to diffusion.

The Prevalence of Vitreomacular Interface Pathology in a Spanish Tertiary Hospital

Purpose: To determine the prevalence of vitreomacular interface (VMI) pathology, using spectral-domain optical coherence tomography (SD-OCT). Methods: VMI status was classified into macular posterior vitreous detachment (PVD), focal vitreomacular adhesion (VMA; ≤1,500 μm), broad VMA (>1,500 μm), focal vitreomacular traction (VMT; ≤1,500 μm), broad VMT (>1,500 μm), full-thickness macular hole (FTMH) with the presence of VMT, and FTMH without the presence of VMT. Results: A total of 1,976 eyes were included. A nonpathologic VMI was observed in 1,875 eyes (94.8%), including 1,050 (53.1%) with PVD, 120 (6.1%) with focal VMA and 705 (35.6%) with broad VMA. A pathologic state of the VMI was diagnosed in 101 eyes (5.1%). Thirty-three eyes (1.7%) were classified as focal VMT, 29 (1.4%) as broad VMT, 39 (1.9%) as FTMH, resulting in 6 small, 12 medium and 21 large FTMHs, six eyes had VMT associated to FTMH. Conclusions: Even in a tertiary care, retinal referral practice, VMI pathology is a relatively rare condition. There was a higher prevalence in a tertiary hospital study compared to population-based studies.

Myeloid skewing in murine autoimmune arthritis occurs in hematopoietic stem and primitive progenitor cells

Skewing toward myeloid cell production is often observed in chronic inflammation and autoimmune diseases. Herein, we determined whether persistent myeloid activation and proinflammatory output occurring in pathologic conditions is at the level of hematopoietic stem and primitive progenitor cells (HSPPCs). By using a mouse arthritis model, we found that even though HSPPCs in arthritis still retained the capacity to differentiate into different lineages, they acquired enhanced in vitro and in vivo propensity in a disease-dependent manner to generate myeloid cells, the key perpetrators of tissue damage in arthritis. This myeloid skewing was cell intrinsic, as arthritic HSPPCs up-regulate myeloid-specific transcripts including S100a8. Exogenous S100a8 promoted myeloid cell output from wild-type HSPPCs, suggesting mechanistic involvement of this gene in the myeloid priming that occurs in arthritic HSPPCs. Therefore, our results indicate that in arthritic mice, HSPPCs adopt a pathologic state that favors disease persistence.

Linear and Nonlinear Heart Rate Variability Indexes in Clinical Practice

Biological organisms have intrinsic control systems that act in response to internal and external stimuli maintaining homeostasis. Human heart rate is not regular and varies in time and such variability, also known as heart rate variability (HRV), is not random. HRV depends upon organism's physiologic and/or pathologic state. Physicians are always interested in predicting patient's risk of developing major and life-threatening complications. Understanding biological signals behavior helps to characterize patient's state and might represent a step toward a better care. The main advantage of signals such as HRV indexes is that it can be calculated in real time in noninvasive manner, while all current biomarkers used in clinical practice are discrete and imply blood sample analysis. In this paper HRV linear and nonlinear indexes are reviewed and data from real patients are provided to show how these indexes might be used in clinical practice.