Microcephalic osteodysplastic primordial dwarfism (MOPD) type I with severe anemia and MRI brain findings of MOPD type II

We report a 4 month old male, 4th in order of birth of healthy consanguineous Egyptian parents with typical characteristics of microcephalic osteodysplastic primordial dwarfism most probably belongs to type I (MOPD I). The patient had intrauterine growth retardation, sparse scalp hair, sparse eyebrows and eyelashes, high arched palate, micrognathia, low set ears, short neck, clenched fists, groove between thumb and palm of hand, arachnodactyly, flexion contractures of elbow and knee. He also had thin dry skin with marked decreased subcutaneous fat and prominent superficial veins over chest and abdomen and mild hypertrichosis over lower back and buttocks. However, the patient had severe anemia and MRI brain findings revealed global hypovolemic brain changes in the form of dilated ventricles and widened cortical sulci, multiple old vascular insults and aneurismal dilatation of right internal carotid artery (ICA) which are consistent with MOPD II

Computed tomography independent quantitative determinants of CAD-RADS 4 versus CAD-RADS 3 for calcified coronary lesions

Purpose: An investigation of coronary computed tomography angiography (CCTA)-derived quantitative parameters to determine CAD-RADS 4 versus CAD-RADS 3 of coronary lesions with moderate to severe calcification. Material and methods: The study included 150 coronary lesions proven to have moderate or severe stenosis by invasive coronary angiography and showing moderate to severe calcification in CCTA. Various CCTA-quantitative parameters were correlated to the degree of stenosis (moderately versus severely stenosed lesions). Their sensitivity and specificity to detect severe stenosis (supposed to be corresponding to CAD-RADS 4) were examined at multiple cut-off points. Results: The calcification remodelling index (CRI) was the only statistically significant independent computed tomography angiography-derived predictor of severe stenosis versus moderate stenosis on multivariate regression analysis. The best cut-off value was ≤ 0.84, with 77.78% sensitivity and 86.46% specificity. Conclusions: From all quantitative-derived CCTA parameters, CRI ≤ 0.84 was the predictor with the highest diagnostic performance for severe versus moderate stenosis in moderately to severely calcified coronary lesions. Accordingly, CRI can help to determine CAD-RADS 4 versus CAD-RADS 3

Effects of Poly-MVA on the rheological properties of blood after in-vivo exposure to gamma radiation

AbstractThis study aims to examine the radio-prophylactic effects of poly-MVA against exposure to acute dose of gamma radiation. Adult male rats, weighing 200 gm, were exposed to 6 Gy gamma radiation from Cs-137 source. The animals received daily oral administration of 2 ml/kg body weight of poly-MVA for different time intervals. The prophylactic effect was examined by two modes of administration: two weeks before irradiation and another group which received continuous administration for two weeks before and two other weeks after irradiation (total time of administration 28 days). Different parameters were performed, which include determination of cellular antioxidant enzymes (Glutathione (GSH), catalase and superoxide dismutase) in hepatic cells, the rheological properties of blood, osmotic fragility and scanning electron microscope photography of red blood cells. Exposure to radiation resulted in a significant decrease in cellular antioxidant enzymes (GSH, Catalase and SOD) and decrease in Bingham viscosity, yield stress and aggregation index of blood. Furthermore it induced slightly increase in average osmotic fragility of red blood cells accompanied by decrease in osmotic dispersion and remarkable modification of red blood cell morphology. Administration of Poly-MVA showed markedly elevation in GSH, Catalase and SOD content in liver cells in all treated groups. It also showed improvement in all observed parameters. The obtained results showed that oral uptake of poly MVA posses a radio-prophylactic effect that might be used in planned radiation exposure in diagnosis and radiotherapy

Radio-mitigation effect of poly-MVA after exposure to an acute dose of gamma radiation

AbstractAdult male rats were exposed to a 6Gy single dose from a Cs-137 source. The radio-mitigation effect of poly-MVA was evaluated by daily administration of 2ml/kg of body weight immediately after irradiation for two weeks. The morphological changes in the red blood cells were studied. The osmotic fragility and rheological properties of blood, the alteration in the contents of antioxidant enzymes (glutathione, catalase and superoxide dismutase) and lipid peroxidation in hepatic cells were determined. The results showed that exposure to radiation resulted in significant changes in cellular antioxidant enzymes (GSH, catalase and SOD) and a decrease in the blood Bingham viscosity, yield stress and aggregation index. Furthermore, it induced a slight increase in the average osmotic fragility of red blood cells accompanied by a decrease in osmotic dispersion, as well as a modification of red blood cell morphology. It also caused a significant increase (75%) in the lipid peroxidation 1 day after exposure to radiation, which persisted until the 14th day recorded after irradiation. Oral administration of poly-MVA after irradiation reduced the radiation-induced damage, as seen in the non-significant change in lipid peroxidation compared to the control. It also resulted in improvement in the observed parameters

Trichorhinophalangeal syndrome II, expanding the clinical spectrum

AbstractWe report a 4.5year old Egyptian male child, fourth in the order of birth of healthy remote consanguineous parents. He has typical facial as well as skeletal features of Trichorhinophalangeal syndrome (TRPS) II. The facial features included bilateral downward slanting palpebral fissures, bulbous nose, long filtrum, retromicrognathia, sparse hair in the scalp and thick eyebrows. The skeletal features included retarded bone age, cone shaped epiphyses of the phalanges and multiple exostoses. The patient has also growth retardation, moderate mental retardation and hyperlaxity of the right knee joint. However our patient has some features not reported in TRPS II patients. These included bilateral partial ptosis, long eye lashes, preauricular skin tag, short 2nd right finger, short metacarpals of both thumbs. So we have to expand the clinical spectrum. Karyotype demonstrated 46,XY,del 8(q23.3-q24.1)

Cornelia-de Lange syndrome in an Egyptian infant with unusual bone deformities

AbstractWe report a 4month old female infant with the typical features of Cornelia-de Lange syndrome. What was striking in our patient was the presence of skeletal anomalies not reported previously. These included arachnodactly of both fingers and toes, flexion of thumbs at metacarpophalengeal joints, bilateral short big toes, angulation of the lower part of the bones of right forearm and both legs with multiple skin folds. Also biochemical and X-ray evidence of rickets was detected mostly due to malnutrition and failure to thrive. The patient died at the age of 5months with bronchopneumonia and gastroenteritis

Serum selenoprotein P in lean and obese Egyptian individuals and its relation to insulin resistance

Background: Obesity is a major health problem with increasing prevalence and directly contributes to the development of cardiovascular disease (CVD). Selenoprotein P (Se P) is a novel hepatokine that affects glucose metabolism, lipid metabolism, and correlating with insulin resistance, inflammation, and atherosclerosis. Plasma Se P level is one of the promising biomarkers for predictions or diagnoses/prognoses of the diseases.Objective: Evaluating the serum level of Selenoprotein P in overweight, obese, and lean individuals and its relation to insulin resistance and cardiometabolic parameters.Subjects and Methods: A cross-sectional study was conducted in the Outpatient Endocrinology Clinics, Internal Medicine Department, Menoufia University Hospitals on 90 individuals that were divided into two groups: (group I) 50 overweight and obese individuals (with body mass index (BMI) in the range 25.0–29.9 kg/m2 and ≥30.0 kg/m2, respectively) and (group II) 40 lean individuals with BMI in the range (18.5–24.9) kg/m2. All subjects were subjected to detailed history taking, clinical examination, and laboratory investigations including fasting and 2 hours postprandial blood sugar, lipid profile, fasting serum insulin level, Homeostatic Model Assessment of Insulin Resistance (HOMA-IR), and serum Se P.Results: Se P concentrations were significantly increased in overweight and obese group than lean group (p < 0.001). Se P concentration was related to various cardio-metabolic parameters. In overweight and obese group there was a significant positive correlation between serum Selenoprotein p level and waist circumference (WC) (r −0.453, p <0.001), hip circumference (HC) (r = 0.464, p < 0.001), waist-hip ratio (WHR) (r −0.506, p < 0.001), visceral adiposity index (VAI) (r = 0.323, p = 0.022), fasting serum insulin (r = 0.585, p < 0.001), and HOMA-IR (r = 0.570, p < 0.001).Conclusion: Circulating Se P concentrations is elevated in obesity and related to insulin resistance, visceral adiposity, and atherogenic index of plasma

Effect of Deficit Irrigation on the Productive Response of Drip-irrigated Onion (Allium cepa L.) in Mediterranean Conditions

[EN] Water is an essential resource for food production, and agriculture consumes close to 69% of total freshwater use. Water shortage is becoming critical in arid and semiarid areas worldwide; therefore, it is vital to use water efficiently. The objective of this research was to evaluate the response of onion growth, plant water status, bulb yield, irrigation water use efficiency and bulb quality using three continued deficit strategies, applying 100, 75, and 50% of the irrigation water requirements during three seasons. The yield response factor was 0.71, indicating that in the analysed conditions the crop was tolerant to a water deficit. Compared to full irrigation, deficit irrigation with 75% of the irrigation water requirements resulted in a low yield and profit reduction for the growers (10.3% and 10.9%, respectively), but also important water savings (26.6%), improving both the irrigation water use efficiency and water use efficiency. However, onion exposure to severe water deficits at 50% of the irrigation water requirements drastically reduced plant growth and bulb yield and growers' profits, although it did increase their soluble solid content. Irrigating at 75% of the irrigation water requirements could be an actionable strategy for onion production under water-limited conditions.Abdelkhalik, A.; Pascual-Seva, N.; Nájera, I.; Domene, MA.; Baixauli Soria, C.; Pascual España, B. (2019). Effect of Deficit Irrigation on the Productive Response of Drip-irrigated Onion (Allium cepa L.) in Mediterranean Conditions. Horticulture Journal. 88(4):488-498. https://doi.org/10.2503/hortj.UTD-081S48849888

Magnetic resonance imaging of head and neck vascular anomalies: pearls and pitfalls

Purpose: The aim of this study was to describe typical MRI features of the head and neck vascular anomalies and the possible diagnostic pitfalls.Patients and methods: Patients with extracranial vascular anomalies of the head and neck, who underwent MRI examinations between January 2013 and January 2016, were included in the study. Precontrast and postcontrast T1-WI,T2-WI, with and without fat saturation were acquired. When indicated, a noncontrast MR angiography was performed. Dynamic postcontrast MRI techniques were available in six children.Results: The study included 33 patients (age ranged from 10 to 20 years, mean: 49 months). MRI confirmed the clinical diagnosis in equivocal cases, and provided proper determination of lesion extension and/or associated intracranial anomalies. The study included 10 cases of vascular tumors (hemangioma), whereas the remaining 23 cases had the diagnosis of vascular malformations (one patient with arteriovenous malformation, one with capillary malformation, seven with venous, nine with macrocystic lymphatic, and five with microcystic lymphatic malformations).Conclusion: Vascular anomalies in the head and neck are mostly diagnosed on clinical basis; however, when the history is uncertain or the diagnosis is equivocal, a well-tailored MR examination can be a single valuable diagnostic tool providing structural and functional information.Keywords: children, head and neck, hemangioma, lymphatic malformation, magnetic resonance angiography, venous malformatio

Physical, metabolic, and microbial rumen development in goat kids: a review on the challenges and strategies of early weaning

The digestive system of newborn ruminant functions is similar to monogastric animals, and therefore milk flows into the abomasum instead of rumen for digestion. The rumen undergoes tremendous changes over time in terms of structure, function, and microbiome. These changes contribute to the smooth transition from the dependence on liquid diets to solid diets. Goat kids are usually separated at early ages from their dams in commercial intensive systems. The separation from dams minimizes the transfer of microbiota from dams to newborns. In this review, understanding how weaning times and methodologies could affect the normal development and growth of newborn goats may facilitate the development of new feeding strategies to control stress in further studies