Two rare cases of cystic angiomatosis and a literature review

Cystic angiomatosis is a rare disease characterized by disseminated multifocal hemangiomatous and/or lymphangiomatous cystic lesions of the skeleton with possible visceral organ involvement. Only a few dozens of such patients worldwide have been described in the literature. This article presents two case reports of the patients admitted to the D. Rogachev NRMCPHOI with suspected Langerhans cell histiocytosis. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. During the investigation, multiple cysts of the skull bones, spine, pelvic bones and limbs, as well as of the spleen were found in both patients. A biopsy of the bone cysts of the skull revealed no data in favor of histiocytosis or other neoplasms. Cystic angiomatosis was diagnosed in both cases. This is a rare disease that should be kept in mind in the differential diagnosis in patients with cystic lesions of the bones and visceral organs.

Visceral organ morphogenesis via calcium-patterned muscle contractions

How organs achieve their final shape is a problem at the interface between physics and developmental biology. Organs often involve multiple interacting tissue layers that must be coordinated to orchestrate the complex shape changes of development. Intense study has uncovered genetic and physical ingredients driving the form of monolayer tissue. Yet, tracing dynamics across tissue layers and across scales -- from cell to tissue, to entire organs -- remains an outstanding challenge. Here, we study the midgut of Drosophila embryos as a model visceral organ to reconstruct in toto the dynamics of multi-layer organ formation in vivo. Using light-sheet microscopy, genetics, computer vision, and tissue cartography, we extract individual tissue layers to map the time course of shape across scales. We identify the kinematic mechanism driving the shape change due to tissue layer interactions by linking out-of-plane motion to active contraction patterns, revealing a convergent extension process in which cells deform as they flow into deepening folds. Acute perturbations of contractility in the muscle layer using non-neuronal optogenetics reveals that these contraction patterns are due to muscle activity, which induces cell shape changes in the adjacent endoderm layer. This induction cascade relies on high frequency calcium pulses in the muscle layer, under the control of hox genes. Inhibition of targets of calcium involved in myosin phosphorylation abolishes constrictions. Our study of multi-layer organogenesis reveals how genetic patterning in one layer triggers a dynamic molecular mechanism to control a physical process in the adjacent layer, orchestrating whole-organ shape change.

Growth performance, visceral organ weights, and gut health of weaned pigs fed diets with different dietary fiber solubility and lipid sources

Objective of this study was to determine the interactive effects of dietary fiber solubility and lipid source on growth performance, visceral organ weights, gut histology, and gut microbiota composition of weaned pigs. A total of 280 nursery pigs [initial body weight (BW) = 6.84 kg] weaned at 21 d were housed in 40 pens (7 pigs/pen). The pigs were fed four diets (10 pens/diet) in a randomized complete block design in two phases; Phase 1 from 0 to 2 weeks and Phase 2 from 2 to 5 wk. The diets were corn-soybean meal-based with either sugar beet pulp (SBP) or soybean hulls (SBH) as a fiber source and either soybean oil (SBO) or choice white grease (CWG) as a lipid source in a 2 × 2 factorial arrangement. The BW and feed intake were determined by phase, whereas visceral organ weights, intestinal histology, and gut microbial composition were determined at the end of the trial. Dietary fiber solubility and lipid source did not interact (P > 0.05) on average daily feed intake and average daily gain across all phases. However, the gain to feed ratio (G:F) for CWG-containing diets was lower (P < 0.05) than that for SBO-containing diets for Phase 1. Also, G:F for SBP-containing diets was lower (P < 0.05) than that for SBH-containing diets for Phase 1 and for the entire study period. Pigs fed SBP-containing diets had greater (P < 0.05) stomach weight, and tended to have greater (P < 0.10) small and large intestine weights relative to BW than those fed SBH-containing diets. Duodenal villous height to crypt depth ratio for CWG-based diets tended be greater (P = 0.09) than that for SBO-based diets. Fiber solubility and lipid source interacted (P < 0.05) on relative abundance of Bacteroides in the colon such that the relative abundance of the Bacteroides for CWG was greater (P < 0.05) than that for the SBO in SBP-based diet, but not in SBH-based diet. Relative abundance of Butyricicoccus in the colon for SBH-based diet was greater (P < 0.05) than that for SBP-based diet. In conclusion, inclusion of SBH instead of SBP in corn-soybean meal-based diets for weaned pigs can result in increased feed efficiency and relative abundance of Butyricicoccus in the colon, which is associated with improved gut health. Also, inclusion of SBO instead of CWG in the diets for weaned pigs can result in improved feed efficiency during Phase 1 feeding; however, the pigs may recover from the low feed efficiency induced by dietary inclusion of CWG instead of SBO after Phase 1 feeding.

THE WANDERING INTRA UTERINE DEVICE: A RARE CASE REPORT

Intra Uterine Device (IUD) is one of the widely used reversible methods of female contraception. There are many complications reported with IUD insertion, among which device migration and delayed perforation of viscera like small bowel is one of the rare and serious complication. Here we report a case of 29 year old female who presented with sudden onset of abdominal pain associated with vomiting. Her Computed Tomography (CT) scan of abdomen revealed IUD lying within peritoneal cavity causing terminal ileal perforation. She was treated surgically and she recovered well. Extra uterine visceral organ injury many months or years following IUD insertion is rare and should be considered as a differential in any female with IUD presenting with abdominal symptoms.

Less Known Gastrointestinal Manifestations of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Systematic Review of the Literature

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life threatening severe cutaneous drug reaction. Most patients develop eosinophilia, a rash, a fever, lymphadenopathy and variable visceral organ involvement 2–6 weeks following exposure to the inciting medication. Unlike other severe cutaneous drug reactions, internal organ involvement that leads to high mortality is a unique feature of DRESS syndrome. While the liver is the most common internal organ involved, literally every other visceral organ can be affected in this syndrome. The lesser-known gastrointestinal manifestations of this syndrome include esophagitis, gastritis, enteritis, colitis, pancreatitis and a late autoimmune sequela due to pancreatic injury such as fulminant type 1 diabetes mellitus, autoimmune type 1 diabetes mellitus and type 2 diabetes mellitus. While these entities are less common, they are associated with equally severe complications and adverse patient outcomes. In this review, we synthetize data on these rare manifestations using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The liver, the most common visceral organ involved, has been described as part of DRESS elsewhere and is not included in the scope of this article.

Diagnosis of Early Mycosis Fungoides

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphomas, generally has a favorable clinical course. Early MF typically presents erythematous patches and/or plaques and lasts for many years without affecting the life expectancy. Only limited cases progress to develop skin tumors, with subsequent lymph nodes and rarely visceral organ involvement. One of the clinical problems in early MF is the difficulty in differentiating the disease from benign inflammatory disorders (BIDs), such as atopic dermatitis, chronic eczema, and psoriasis. In some MF cases, clinical and pathological findings are similar to those of BIDs. However, the accurate diagnosis of early MF is quite important, as inappropriate treatment including immunosuppressants can cause unfavorable or even fatal outcomes. This article focuses on general methods and novel tools for diagnosis of early MF.

The Regulatory Mechanism of Feeding a Diet High in Rice Grain on the Growth and microRNA Expression Profiles of the Spleen, Taking Goats as an Artiodactyl Model

Several researchers have testified that feeding with diets high in rice grain induces subacute ruminal acidosis and increases the risk of gastrointestinal inflammation. However, whether diets high in rice grain affect spleen growth and related molecular events remains unknown. Therefore, the present study was conducted to investigate the effects of feeding a high-concentrate (HC) diet based on rice on the growth and microRNA expression profiles in goat spleen. Sixteen Liuyang black goats were used as an artiodactyl model and fed an HC diet for five weeks. Visceral organ weight, LPS (lipopolysaccharide) concentration in the liver and spleen, and microRNA expression were analyzed. The results showed that feeding an HC diet increased the heart and spleen indexes and decreased the liver LPS concentration (p < 0.05). In total, 596 microRNAs were identified, and twenty-one of them were differentially expressed in the spleens of goats fed with the HC diet. Specifically, several microRNAs (miR-107, miR-512, miR-51b, miR-191, miR-296, miR-326, miR-6123 and miR-433) were upregulated. Meanwhile, miR-30b, miR-30d, miR-1468, miR-502a, miR-145, miR-139, miR-2284f, miR-101 and miR-92a were downregulated. Additionally, their target gene CPPED1, CDK6, CCNT1 and CASP7 expressions were inhibited (p < 0.05). These results indicated that the HC diet promoted the growth of the heart and spleen. The HC diet also regulated the expression of miR-326, miR-512-3p, miR-30b, miR-30d, miR-502a and their target genes (CPPED1, CDK6 and CCNT1) related to the enhancement of splenocyte proliferation. The HC diet also modulated the expression of miR-15b-5p, miR-1468 and miR-92a, related to the suppression of splenocyte apoptosis.

A Case Report on Scleroderma: A Diagnostic Dilema

Scleroderma is a rare heterogenous group of autoimmune fibrosing disorder that mainly exists in two forms; localized scleroderma (LS) and systemic sclerosis (SSc). It involves thickening of the skin at fingers region extending from proximal to metacarpophalangeal joints. The diagnostic criteria of scleroderma include past history of patient, symptoms of patient, serology, and skin biopsy. The morbidity and mortality are much worse for SSc with the patients are at risk for life threatening lung, heart and other visceral organ fibrosis and vasculopathy. There is no drug that can cure or stop scleroderma over fibrosis, but certain drugs regulate the symptoms associated with it and boost the patient's quality of life, particularly steroidal creams that help alleviate swelling, joint pain, loosen tight skin; blood pressure drugs that dilate blood vessels; immunosuppressive agents. If the disease is severe amputation is necessary.