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Lancet (London, England) Jun 2024
Topics: Humans; Carcinoma, Renal Cell; Kidney Neoplasms; Immune Checkpoint Inhibitors; Purpura Fulminans; Male; Middle Aged; Female; Aged
PubMed: 38944528
DOI: 10.1016/S0140-6736(24)01178-4 -
Advances in Pediatrics Aug 2024Pediatric immune thrombocytopenia (ITP) is a fairly common bleeding disorder PRESENTING with a decreased number of platelets. The typical clinical presentation involves... (Review)
Review
Pediatric immune thrombocytopenia (ITP) is a fairly common bleeding disorder PRESENTING with a decreased number of platelets. The typical clinical presentation involves mild bleeding symptoms with bruising and petechiae and occasional mucosal bleeding. ITP is thought to be an autoimmune disorder and more recently other mechanisms have been described. Most cases resolve spontaneously and can undergo watchful waiting as the platelet count improves. Initially, steroids or intravenous immunoglobulin G (IVIg) can be used to increase platelets. For those cases that do not resolve and become persistent or chronic, there are multiple treatment options, with new agents being studied in adults that will hopefully make it to clinical trials in pediatrics in the future.
Topics: Humans; Child; Purpura, Thrombocytopenic, Idiopathic; Immunoglobulins, Intravenous; Platelet Count
PubMed: 38944486
DOI: 10.1016/j.yapd.2024.02.007 -
Giornale Italiano Di Nefrologia :... Jun 2024IgA nephropathy (IgAN) is a fairly common association with alcoholic liver disease. However, IgA vasculitis (IgAV) is quite an uncommon association with alcoholic liver...
IgA nephropathy (IgAN) is a fairly common association with alcoholic liver disease. However, IgA vasculitis (IgAV) is quite an uncommon association with alcoholic liver cirrhosis and only a handful of cases have been reported in literature. Secondary IgAN usually presents in a docile manner, progressing slowly in about 5-25 years. It is usually responsive to steroid therapy, very rarely progressing to End-Stage Renal Disease. Here, we present a man in his late 50s, a known hypertensive and alcohol related liver-cirrhotic, who presented to our hospital with rash and rapidly progressive renal failure (RPRF). He was diagnosed with IgA nephritis with IgA vasculitis (IgAVN). His diagnosis was confirmed with skin and renal biopsy. He was started on renal replacement therapy for his renal failure and began oral steroid therapy. After administration of steroid therapy for 6 months, the patient recovered and was dialysis independent with stable renal parameters.
Topics: Humans; Male; Glomerulonephritis, IGA; Middle Aged; Disease Progression; Liver Diseases, Alcoholic; IgA Vasculitis; Vasculitis
PubMed: 38943330
DOI: No ID Found -
Revista de La Facultad de Ciencias... Jun 2024El cuy en el Ecuador es sinónimo de nuestra gastronomía ancestral y de tradición cultural, pero por la alimentación rica en L-canavanina (alfalfa) que reciben;...
El cuy en el Ecuador es sinónimo de nuestra gastronomía ancestral y de tradición cultural, pero por la alimentación rica en L-canavanina (alfalfa) que reciben; podría limitar su consumo en pacientes con trombocitopenia inmune primaria (PTI). La ingesta de alfalfa en humanos puede propiciar insuficiencia renal y síndrome lupus-like. El centro de Lupus John Hopkins recomiendan evitarla en la dieta de los pacientes con Lupus Eritematoso Sistémico (LES), al agravar la inflamación por estimulación de la actividad inmune (flares). Presentamos dos casos de pacientes con PTI vinculados con la ingesta de cuy. ¿Es probable?
Topics: Animals; Humans; Guinea Pigs; Female; Purpura, Thrombocytopenic, Idiopathic; Adult; Lupus Erythematosus, Systemic; Ecuador; Male; Middle Aged
PubMed: 38941218
DOI: 10.31053/1853.0605.v81.n2.44592 -
Immunology and Allergy Clinics of North... Aug 2024Urticarial vasculitis is a rare autoimmune disorder characterized by persistent edematous papules and plaques on the skin that last longer than 24 hours, often... (Review)
Review
Urticarial vasculitis is a rare autoimmune disorder characterized by persistent edematous papules and plaques on the skin that last longer than 24 hours, often accompanied by systemic symptoms such as joint pain and fever. Unlike common urticaria, this condition involves inflammation of small blood vessels, leading to more severe and long-lasting skin lesions with a tendency to leave a bruiselike appearance. Diagnosis is challenging and may require a skin biopsy. Associated with underlying autoimmune diseases, treatment involves managing symptoms with medications such as antihistamines and corticosteroids, addressing the immune system's dysfunction, and treating any concurrent autoimmune conditions.
Topics: Humans; Urticaria; Vasculitis; Skin; Diagnosis, Differential; Histamine Antagonists; Autoimmune Diseases; Biopsy; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 38937011
DOI: 10.1016/j.iac.2024.03.006 -
Blood Advances Jun 2024Hereditary thrombotic thrombocytopenic purpura (hTTP) is a rare autosomal recessive, life-threatening disorder caused by a severe deficiency of the plasma enzyme,...
Hereditary thrombotic thrombocytopenic purpura (hTTP) is a rare autosomal recessive, life-threatening disorder caused by a severe deficiency of the plasma enzyme, ADAMTS13. The current estimated prevalence of hTTP in different regions of the world, 0.5-2.0 patients/million, is determined by the frequency of diagnosed patients. To evaluate more accurately the worldwide prevalence of hTTP, and also the prevalence within distinct ethnic groups, we used data available in exome and genome sequencing of 807,162 (730,947 exomes, 76,215 genomes) subjects reported recently by the Genome Aggregation Database (gnomAD-v4.1). Among 1,614,324 analyzed alleles in the gnomAD population we identified 6,321 distinct ADAMTS13 variants. Of these 6,321 variants, 758 were defined as pathogenic; 140 (18%) variants had been previously reported and 618 (82%) were novel (predicted as pathogenic). 10,154 alleles (0.6%) were carrying the reported or predicted pathogenic variants; 7,759 (77%) with previously reported variants. Considering all 758 pathogenic variants and also only the 140 previously reported variants, we estimated a global hTTP prevalence of 40 and 23 cases/106, respectively. Considering only the 140 previously reported variants, the highest estimated prevalence was in East Asians (42/106). The estimated prevalences of other populations were: Finnish, 32/106; non-Finnish Europeans, 28/106; Admixed Americans, 19/106; Africans/African Americans, 6/106; and South Asians, 4/106. The lowest prevalences were Middle Eastern, 1/106 and Ashkenazi Jews, 0.7/106. This population-based genetic epidemiology study reports that hTTP prevalence is substantially higher than the currently estimated prevalence based on diagnosed patients. Many hTTP patients may not be diagnosed or may have died during the neonatal period.
PubMed: 38935915
DOI: 10.1182/bloodadvances.2024013421 -
Journal of Pediatric Hematology/oncology Jun 2024Immune thrombotic thrombocytopenic purpura (iTTP) in children is a rare, severe thrombotic microangiopathy. This condition is characterized by microangiopathic hemolytic...
BACKGROUND
Immune thrombotic thrombocytopenic purpura (iTTP) in children is a rare, severe thrombotic microangiopathy. This condition is characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia due to reduced activity of the von Willebrand factor-cleaving protease ADAMTS13.
METHODS
A retrospective case series evaluating data collected from the medical files of 4 children diagnosed with iTTP.
RESULTS
The presented case series depicts a variety of iTTP presentations: 1 case of primary iTTP, 1 case induced by Shiga toxin, 1 associated with RAS-associated autoimmune leukoproliferative disease (RALD), and 1 initial manifestation of systemic lupus erythematosus (SLE). Notably, 2 patients recovered without undergoing plasma exchange.
CONCLUSION
Early ADAMTS13 testing in children with unexplained hemolysis or thrombocytopenia is crucial. The diverse underlying causes, including infections and autoimmune disorders, underscore the complexity of iTTP in the pediatric population. These cases highlight the necessity for personalized treatment approaches that consider each patient's unique clinical situation and potential alternatives or modifications to conventional therapeutic regimens.
PubMed: 38934698
DOI: 10.1097/MPH.0000000000002914 -
Nutrients Jun 2024Ashwagandha has been reported to reduce stress and attenuate cognitive decline associated with inflammation and neurodegeneration in clinical populations. However, the... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Ashwagandha has been reported to reduce stress and attenuate cognitive decline associated with inflammation and neurodegeneration in clinical populations. However, the effects as a potential nootropic nutrient in younger populations are unclear. This study examined the effects of liposomal ashwagandha supplementation on cognitive function, mood, and markers of health and safety in healthy young men and women.
METHODS
59 men and women (22.7 ± 7 yrs., 74.9 ± 16 kg, 26.2 ± 5 BMI) fasted for 12 h, donated a fasting blood sample, and were administered the COMPASS cognitive function test battery (Word Recall, Word recognition, Choice Reaction Time Task, Picture Recognition, Digit Vigilance Task, Corsi Block test, Stroop test) and profile of mood states (POMS). In a randomized and double-blind manner, participants were administered 225 mg of a placebo (Gum Arabic) or ashwagandha () root and leaf extract coated with a liposomal covering. After 60-min, participants repeated cognitive assessments. Participants continued supplementation (225 mg/d) for 30 days and then returned to the lab to repeat the experiment. Data were analyzed using a general linear model (GLM) univariate analysis with repeated measures and pairwise comparisons of mean changes from baseline with 95% confidence intervals (CI).
RESULTS
Ashwagandha supplementation improved acute and/or 30-day measures of Word Recall (correct and recalled attempts), Choice Reaction Time (targets identified), Picture Recognition ("yes" correct responses, correct and overall reaction time), Digit Vigilance (correct reaction time), Stroop Color-Word (congruent words identified, reaction time), and POMS (tension and fatigue) from baseline more consistently with several differences observed between groups.
CONCLUSION
Results support contentions that ashwagandha supplementation (225 mg) may improve some measures of memory, attention, vigilance, attention, and executive function while decreasing perceptions of tension and fatigue in younger healthy individuals. Retrospectively registered clinical trial ISRCTN58680760.
Topics: Humans; Male; Female; Cognition; Double-Blind Method; Dietary Supplements; Young Adult; Adult; Affect; Plant Extracts; Adolescent; Reaction Time; Biomarkers; Liposomes; Plant Leaves; Plant Roots
PubMed: 38931168
DOI: 10.3390/nu16121813 -
Cureus May 2024Bacterial endocarditis is a rare infection that can present with variable clinical manifestations. Rarely, it can present as cutaneous vasculitis characterized by a...
Bacterial endocarditis is a rare infection that can present with variable clinical manifestations. Rarely, it can present as cutaneous vasculitis characterized by a purpuric rash mimicking immune-mediated vasculitis. There have been a few case reports of leukocytoclastic vasculitis (LCV) due to infectious endocarditis. It is important to recognize endocarditis as a potential cause of vasculitis because treatment with immunosuppressive agents can have devastating consequences. We report a case of a 53-year-old male with endocarditis who developed a palpable purpura of the bilateral lower extremities. A skin biopsy was performed, and histopathologic and immunofluorescence studies demonstrated LCV.
PubMed: 38916001
DOI: 10.7759/cureus.61021 -
Biomedical Reports Aug 2024During a routine ultrasound examination of the abdomen, a 60-year-old male patient was diagnosed with mass in the tail of the pancreas. However, computed tomography (CT)...
During a routine ultrasound examination of the abdomen, a 60-year-old male patient was diagnosed with mass in the tail of the pancreas. However, computed tomography (CT) suggested that the lesion was an intrapancreatic accessory spleen (IPAS). IPAS is a congenital anomaly, which usually does not present with symptoms. IPAS occurs during embryologic splenic development when a portion of the splenic tissue fails to fuse with the main body of the spleen. IPAS does not require treatment, except when it is combined with idiopathic thrombocytopenic purpura. In the present case, the diagnosis of IPAS was confirmed using magnetic resonance imaging (MRI). On CT and MRI, the IPAS had a density and intensity comparable with that of the spleen in all plain and contrast-enhanced phases. Due to comorbidities, the patient refused further evaluation or surgery. The lesion was periodically monitored using CT every 1-2 years. Since the tumour was stable during the 7-year follow-up, it was concluded that it was an IPAS. In patients that cannot undergo surgery, a characteristic location (near the spleen) and imaging features (such as a 'zebra-patterned' enhancement in the arterial phase on CT and high signal intensity on diffusion-weighted imaging sequences on MRI, which is comparative to that of the normal spleen) may allow for a diagnose of IPAS with a high level of certainty. Being aware of this condition could aid a correct diagnosis of IPAS and prevent unnecessary surgery.
PubMed: 38912170
DOI: 10.3892/br.2024.1801