INTRODUCTION: Immune thrombocytopenia (ITP) is the most common cause of acquired bleeding in childhood, but little is known about the clinical course and outcomes in infants with ITP. METHODS: This is a retrospective study of all infants (1-12 months of age) and toddlers (13-47 months of age) diagnosed with ITP from a single centre during a 13-year period. The following data were compared between the two patients groups: demographics, severity of bleeding, platelet counts, duration of illness, development of chronic ITP, treatment and association with recent vaccination. RESULTS: Twenty-two infants and 30 toddlers were diagnosed and followed up for ITP during the study period. Infants with ITP generally had minor or mild bleeding (19, 86.4%) and seldom required treatment (7, 31.8%), and their thrombocytopenia resolved at a mean of 1.90 months after diagnosis. Besides age, the sex ratio, severity of bleeding, platelet counts and proportion that required treatment were comparable between infants and toddlers. Fewer infants developed chronic ITP (1/22 vs. 9/30, P = 0.032), but more infants had a history of vaccination in the preceding 6 weeks prior to diagnosis of ITP (13/22 vs. 1/30, P < 0.001). CONCLUSION ITP in infants is almost always a self-limiting and transient illness, and the majority of cases do not require treatment.
Humans ; Retrospective Studies ; Infant ; Male ; Purpura, Thrombocytopenic, Idiopathic/complications* ; Female ; Child, Preschool ; Platelet Count ; Hemorrhage

A 39-year-old male patient was admitted to hospital with abdominal distension, unconsciousness, and anuria. Head computed tomography (CT) showed subarachnoid hemorrhage and diffuse cerebral edema. The high-density area of contrast accumulation region in the high-density CT plaque was 38 HU, and the preliminary diagnosis was SAH, incomplete intestinal obstruction, and sepsis caused by acute cerebrovascular disease. After admission, the patient displayed upturned eyes, limb convulsions, serum procalcitonin level exceeding 100 ng/mL, low blood pressure and septic shock. Imipenem was given for intensive anti-infection therapy. After treatment, procalcitonin levels showed a slow decline, renal function, and intra-abdominal pressure returned to normal, urine volume gradually increased, but platelets still showed a downward trend. Lumbar puncture showed colorless and clear cerebrospinal fluid, and the biochemical and routine results of cerebrospinal fluid were normal. SAH and intracranial infection were excluded, and it was considered that the head CT showed pseudo-subarachnoid hemorrhage. On the 3rd day of admission, laparoscopic exploratory laparotomy+appendectomy+abdominal drainage under general anesthesia were performed. During surgery, purulent gangrene in the appendix was found, with pus adhering to the surface of the intestines and a large amount of pus present in the abdominal cavity. Rhabdomyolysis syndrome developed after surgery. After continuous renal replacement therapy, the indicators gradually returned to normal. The patient was conscious, and the head CT results were normal. The patient was discharged from the hospital on the 19th day after surgery, and no special discomfort and abdominal pain and distension occurred during the 3-month follow-up.
Humans ; Male ; Adult ; Tomography, X-Ray Computed ; Sepsis/diagnostic imaging* ; Multiple Organ Failure/etiology* ; Subarachnoid Hemorrhage/complications*

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-system disease that presents significant diagnostic challenges due to its complexity and low incidence (White and Dubey, 2023). It affects males and females equally, though males may exhibit more active disease at diagnosis and often require more aggressive treatment (Liu et al., 2023). The hallmark features of EGPA include delayed-onset asthma, eosinophilia in tissues and blood, and vasculitis affecting small to medium-sized arteries (White and Dubey, 2023). EGPA falls under the category of antineutrophil cytoplasmic antibody (ANCA)‍-associated vasculitis (AAV), whereas only about half of EGPA patients test positive for ANCA (Khoury et al., 2023).
Humans ; Male ; Hemorrhage/etiology* ; Granulomatosis with Polyangiitis/complications* ; Heart Arrest/etiology* ; Early Diagnosis ; Eosinophilia/diagnosis* ; Kidney Diseases/etiology* ; Churg-Strauss Syndrome/complications* ; Middle Aged

The nod-like receptor family pyrin domain containing 3 (NLRP3) inflammasome plays a crucial role in the prognosis of subarachnoid hemorrhage (SAH). WNK1 kinase negatively regulates NLRP3 in various inflammatory conditions, but its role in early brain injury (EBI) after SAH remains unclear. In this study, we used an in vivo SAH model in rats/mice and AAV-WNK1 intraventricular injection to investigate its neuroprotective mechanisms. WNK1 expression was significantly reduced in SAH patient blood and SAH model brain tissue, correlating negatively with microglial activation. AAV-WNK1 alleviated brain edema, neuronal necrosis, behavioral deficits, and inflammation by inhibiting NLRP3 inflammasome activation. In hemin-stimulated BV-2 cells, WNK1 overexpression reduced NLRP3 activation and inflammatory cytokines. Chloride counteracted WNK1's inhibitory effects, and WNK1 suppressed P2X7R-induced NLRP3 activation. Mechanistically, WNK1 functioned via the OXSR1/STK39 pathway. These findings highlight WNK1 as a key regulator of intracellular chloride balance and neuroinflammation, presenting a potential therapeutic target for SAH treatment.
Animals ; NLR Family, Pyrin Domain-Containing 3 Protein/metabolism* ; Subarachnoid Hemorrhage/complications* ; Inflammasomes/metabolism* ; Rats ; Mice ; Neuroinflammatory Diseases/metabolism* ; WNK Lysine-Deficient Protein Kinase 1/genetics* ; Male ; Humans ; Chlorides/metabolism* ; Mice, Inbred C57BL ; Rats, Sprague-Dawley ; Brain Injuries/metabolism* ; Microglia/metabolism* ; Protein Serine-Threonine Kinases

Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain. The patient had elevated serum creatinine levels, prompting further investigation through immunofixation electrophoresis and bone marrow aspiration biopsy. Immunofixation electrophoresis showed IgA-λ-type monoclonal immunoglobulin, while the bone marrow aspiration biopsy suggested plasmacytosis. Kidney biopsy indicated membranous hyperplastic glomerulonephritis, light and heavy chain deposition, IgA-λ. The patient was diagnosed with monoclonal gammopathy of renal significance. In light of the elevated serum creatinine, the patient was treated with chemotherapy regimen (bortezomib +cyclophosphamide +dexamethasone). After chemotherapy, there was no significant improvement in the patient's renal function. Subsequently, the patient experienced abdominal pain, skin purpura, joint pain and severe gastrointestinal bleeding. Gastroenteroscopy did not find the exact bleeding position. Angiography revealed hyperplasia of left jejunal artery. Surgical operation found that the bleeding site was located between the jejunum and ileum, where scattered hemorrhagic spots and multiple ulcers were present on the surface of the small intestine, with the deepest ulcers reaching the serosal layer. And the damaged intestine was removed during the operation. Intestinal pathology showed multiple intestinal submucosal arteritis, rusulting in intestinal wall necrosis and multiple ulcers. Considering intestinal lesions as gastrointestinal involvement of IgA vasculitis, methylprednisolone was used continually after the operation, and the patient's condition was improved. However, after half a year, the patient suffered a severe respiratory infection and experienced a recurrence of serious gastrointestinal bleeding. It was considered that the infection triggered the activity of IgA vasculitis, accompanied by gastrointestinal involvement. Finally, the patient died from gastrointestinal bleeding. The present case represented a patient with monoclonal gammopathy of renal significance and IgA vasculitis, prominently presenting with renal insufficiency and severe gastrointestinal bleeding, making the diagnosis and treatment process complex. Patients with IgA monoclonal gammopathy who presented with abdominal pain, purpura, and arthralgia should be vigilant for the possibility of concomitant IgA vasculitis. The treatment of cases with IgA vasculitis combined with monoclonal gammopathy of renal significance was rather challenging. Plasma cell targeting therapy might be an effective regimen for IgA vasculitis with monoclonal gammopathy. However, patients with poor renal response to the treatment indicated poor prognosis.
Humans ; Cyclophosphamide/administration & dosage* ; Gastrointestinal Hemorrhage/etiology* ; IgA Vasculitis/complications* ; Immunoglobulin A ; Intestine, Small/pathology* ; Kidney/pathology* ; Kidney Diseases/pathology* ; Monoclonal Gammopathy of Undetermined Significance/complications* ; Necrosis ; Paraproteinemias/complications* ; Vasculitis/etiology*

Objective: To compare the safety and efficacy of transmesenteric vein extrahepatic portosystemic shunt (TEPS) and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of cavernous transformation of the portal vein (CTPV). Methods: The clinical data of CTPV patients with patency or partial patency of the superior mesenteric vein treated with TIPS or TEPS treatment in the Department of Vascular Surgery of Henan Provincial People's Hospital from January 2019 to December 2021 were selected. The differences in baseline data, surgical success rate, complication rate, incidence rate of hepatic encephalopathy, and other related indicators between TIPS and TEPS group were statistically analyzed by independent sample t-test, Mann-Whitney U test, and Chi-square test. Kaplan-Meier survival curve was used to calculate the cumulative patency rate of the shunt and the recurrence rate of postoperative portal hypertension symptoms in both groups. Results: The surgical success rate (100% vs. 65.52%), surgical complication rate (6.67% vs. 36.84%), cumulative shunt patency rate (100% vs. 70.70%), and cumulative symptom recurrence rate (0% vs. 25.71%) of the TEPS group and TIPS group were statistically significantly different (P < 0.05). The time of establishing the shunt [28 (2141) min vs. 82 (51206) min], the number of stents used [1 (12) vs. 2 (15)], and the length of the shunt [10 (912) cm vs. 16 (1220) cm] were statistically significant between the two groups (t = -3.764, -4.059, -1.765, P < 0.05). The incidence of postoperative hepatic encephalopathy in the TEPS group and TIPS group was 6.67% and 15.79% respectively, with no statistically significant difference (Fisher's exact probability method, P = 0.613). The pressure of superior mesenteric vein decreased from (29.33 ± 1.99) mmHg to (14.60 ± 2.80) mmHg in the TEPS group and from (29.68 ± 2.31) mmHg to (15.79 ± 3.01) mmHg in TIPS group after surgery, and the difference was statistically significant (t = 16.625, 15.959, P < 0.01). Conclusion: The best indication of TEPS is in CTPV patients with patency or partial patency of the superior mesenteric vein. TEPS improves the accuracy and success rate of surgery and reduces the incidence of complications.
Humans ; Portal Vein/surgery* ; Portasystemic Shunt, Transjugular Intrahepatic/methods* ; Hepatic Encephalopathy/etiology* ; Treatment Outcome ; Hypertension, Portal/complications* ; Retrospective Studies ; Gastrointestinal Hemorrhage/etiology*

Viper bite envenomation represents a significant occupational hazard among agricultural workers in India. The viper bite envenomation is usually suspected when a patient presents with predominant local symptoms at the bitten site, including pain, swelling, and necrosis. Further, systemic findings such as diffuse intravascular coagulation, hypotension, and shock may alert physicians of viper bite envenomation rather than a neurotoxic snake bite. However, cerebral complications are rare in viper bites but may potentially fatal. Central nervous system involvement in a viper bite is either due to neurotoxins or hemorrhagins present in the venom, which may induce cerebral thrombosis, ischemia, infarction, and hemorrhage. Here we present a case of a previously healthy adult male who succumbed to extensive subarachnoid, intracerebral, and intraventricular hemorrhages involving bilateral cerebral hemispheres following viper snake bite envenomation. This report highlights the importance of anticipating cerebral complications in viper bite envenomation, a rare occurrence. It also emphasizes the need for early antisnake venom administration to prevent and control systemic envenomation and its complications.
Adult ; Humans ; Male ; Snake Bites/complications* ; Hemorrhage/etiology* ; Intracranial Hemorrhages ; Shock ; India

Liver disease-associated thrombocytopenia syndrome refers to thrombocytopenia caused by liver disease or the treatment of liver disease, and its incidence rate is related to the duration and severity of liver disease. The direct effect of thrombocytopenia on clinical outcomes is an increased risk of bleeding in patients with liver disease, whereas the indirect effect involves delay or termination of treatment due to the potential risk of bleeding. Liver disease-associated thrombocytopenia pathophysiological mechanisms involve decreased platelet production, abnormal distribution, destruction, or increased consumption. Presently, treatment strategies targeting different mechanisms include platelet-stimulating drugs, surgery, immunosuppressive drugs, and platelet transfusion, but the clinical application needs to be standardized further. The National Clinical Research Center for Infectious Diseases organized experts to discuss and formulate consensus with reference to the latest evidence-based medical evidence in the field so as to improve the clinical management level of liver disease-associated thrombocytopenia syndrome in China in terms of diagnosis, typing, and reasonable selection of treatment schemes.
Humans ; Consensus ; Thrombocytopenia/complications* ; Liver Diseases/complications* ; Hemorrhage/etiology* ; Blood Platelets

OBJECTIVE: To compare and analyze the feasibility of autologous facet joint bone block as an alternative to polyetheretherketone (PEEK) cage in lumbar intervertebral fusion surgery for patients with osteoporosis. METHODS: From December 2018 to June 2021, the case data of patients with osteoporosis (T value ≤ -2.5 on dual energy X-ray bone density) who underwent posterior lumbar interbody fusion in the Fourth Medical Center, Chinese PLA General Hospital were retrospectively reviewed. All the cases were followed up for no less than 12 months and were divided into two groups according to the differences of interbody fusion materials: the autologous facet joint bone block group (autogenous bone group) and the PEEK cage group (PEEK group). The general data [such as age, gender, body mass index (BMI), primary diagnosis, distribution of fusion segments, bone mineral density of lumbar (BMD), incidence of preoperative complications], the perioperative data (such as duration of operation, intraoperative blood loss, postoperative drainage, perioperative allogeneic blood transfusion rate), and the incidence of postoperative complications were compared between the two groups. Imaging parameters (disc height, lumbar lordosis angle, segment lordosis angle, segmental lordosis angle, disc height improvement rate, and fusion rate) and lumbar functional scores [visual analogue scale (VAS), Oswestry disability index (ODI), Japanese Orthopedics Association (JOA) score for lower back pain] were compared to evaluate the clinical efficacy between the kinds of intervertebral fusion materials 1 week, 3 months and 6 months postoperative and at the last follow-up. RESULTS: A total of 118 patients were enrolled, including 68 cases in the autogenous bone group and 50 cases in the PEEK group, there were no statistical differences in age, gender, BMI, primary diagnosis, distribution of fusion segments, BMD, incidence of preoperative complications, duration of operation, intraoperative blood loss, postoperative drainage, perioperative allogeneic blood transfusion rate, incidence of postoperative complications, all the preoperative imaging parameters and all the lumbar function scores between the two groups (P>0.05). Postoperative superficial surgical site infections occurred in 3 patients in the autogenous bone group and 2 patients in the PEEK group. At the last follow-up, 3 cases of intervertebral graft collapse occurred in the autogenous bone group and 5 cases in the PEEK group, 1 case of graft subsidence in the autogenous bone group and 1 case in the PEEK group. All the imaging parameters showed significant differences between postoperation and preoperation (P < 0.05), and all the imaging parameters showed significant differences between 1 week and 3 months postoperative in both groups (P < 0.05). The height, angle of fusion gap in the autogenous bone group were lower than those in the PEEK group 1 week postoperatively (P < 0.05), and the fusion gap height improvement rate in the autogenous bone group was lower than that in the PEEK group (P < 0.05). The cases in both groups started to show final fusion 3 months after surgery, and the fusion rate in the autogenous bone group was 75% 6 months postoperatively, which was significantly higher than the rate of 56% in the PEEK group (P < 0.05), and there was no statistically significant difference in the final fusion rate between the two groups (P>0.05). The ODI, the postoperative VAS score was significantly lower than that in preoperation, while the postoperative JOA score was significantly higher than that in preoperation (P < 0.05). The ODI was lower while the JOA score was higher of the autogenous bone group than that of the PEEK group 6 months postoperatively (P < 0.05). CONCLUSION In osteoporosis patients, good interbody fusion rate and improvement of lumbar vertebral function can be obtained by using autologous facet joint bone block or PEEK cage, while the fusion rate and the improvement of lumbar function with autologous facet joint bone block are better than those with PEEK cage 6 months post-operatively. PEEK cage is superior to autologous facet joint bone block in intervertebral distraction and improvement of lumbar lordosis. Significant disc space subsidence occurred in osteoporotic patients within 3 months after lumbar interbody fusion, and the subsidence of PEEK cage was more obvious than that of autologous facet joint bone block.
Humans ; Retrospective Studies ; Lordosis ; Zygapophyseal Joint ; Spinal Fusion/methods* ; Polyethylene Glycols/therapeutic use* ; Treatment Outcome ; Ketones ; Lumbar Vertebrae/surgery* ; Osteoporosis ; Blood Loss, Surgical ; Postoperative Complications ; Postoperative Hemorrhage

Objective: To explore the effects of pregnancy complicated with moyamoya disease on maternal and fetal outcomes. Methods: The general clinical data and maternal and fetal outcomes of 20 pregnancies of 15 patients with moyamoya disease admitted to the First Affiliated Hospital of Zhengzhou University from January 2012 to October 2022 were retrospectively analyzed. Results: (1) General information: among the 20 pregnancies of 15 clearly diagnosed pregnant women complicated with moyamoya disease, 12 were diagnosed before pregnancy (60%, 12/20), 3 were diagnosed during pregnancy (15%, 3/20), and 5 were diagnosed during puerperal period (25%, 5/20). There were 7 cases of primipara (35%, 7/20) and 13 cases of multipara (65%, 13/20). (2) Pregnancy complications and maternal and infant outcomes: among the 20 pregnancies of 15 pregnant women with moyamoya disease, there were 9 pregnancy complications (45%, 9/20), including 5 gestational hypertension (25%, 5/20), 2 severe pre-eclampsia (10%, 2/20), 1 hyperlipidemia and 1 gestational diabetes mellitus (5%, 1/20). There were 2 case of drug abortion in the first trimester, 3 cases of labor induction in the second trimester, and 15 cases of delivery during the third trimester. All the 15 deliveries were cesarean section, of which 11 (11/15) were cesarean sections with medical indications, and 4 (4/15) were cesarean sections caused by personal factors. General anesthesia was used in 5 cases (5/15), epidural block anesthesia in 7 cases (7/15), and combined spinal and epidural anesthesia in 3 cases (3/15). The median gestational age of 15 neonates was 37.2 weeks (34.0 to 40.8 weeks), with 10 cases (10/15) were full-term infants, and 5 (5/15) were preterm infants (3 of which were associated with hypertensive disorder complicating pregnancy). The birth weight of 15 neonates was (2 853±454) g. Four neonates were admitted to neonatal intensive care unit (NICU), of which 3 cases were admitted to NICU due to premature delivery and 1 case was admitted to NICU due to neonatal jaundice. There was no neonatal asphyxia or death. All neonates were followed up from 4 months to 6 years after birth, and all grew well. (3) Neurological symptoms during pregnancy: 8 cases (40%, 8/20) had neurological symptoms during pregnancy, and 6 cases (30%, 6/20) had hemorrhagic symptoms, of which 3 cases occurred during the puerperal period (3/6). There were 2 cases of ischemic symptoms (10%, 2/20), all of which occurred during the puerperal period (2/2). (4) Analysis of factors related to the occurrence of cerebral hemorrhage: the incidence of cerebral hemorrhage in patients with moyamoya disease diagnosed before pregnancy was significantly lower than that in those without a clear diagnosis, and the incidence of cerebral hemorrhage in women with moyamoya disease was lower than that in primipara (all P<0.01). The incidence of cerebral hemorrhage in moyamoya patients without hypertensive disorder complicating pregrancy was lower than that in patients with hypertensive disorder complicating pregrancy, but the difference was not statistically significant (P>0.05). Conclusions: Pregnancy combined with moyamoya disease has adverse effects on maternal and infant outcomes, and the incidence of pregnancy complications increases. Cerebral hemorrhage occurres in prenatal and puperium, while cerebral ischemia occurres mainly in puperium.
Infant ; Pregnancy ; Infant, Newborn ; Female ; Humans ; Pregnancy Outcome ; Cesarean Section ; Pregnant Women ; Infant, Premature ; Moyamoya Disease/complications* ; Retrospective Studies ; Pregnancy Complications/epidemiology* ; Cerebral Hemorrhage