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European Journal of Pediatrics Sep 2023Necrotizing enterocolitis (NEC) is associated with significant morbidity and mortality in preterm infants. Early recognition and treatment of NEC are critical to...
UNLABELLED
Necrotizing enterocolitis (NEC) is associated with significant morbidity and mortality in preterm infants. Early recognition and treatment of NEC are critical to improving outcomes. Enteric nervous system (ENS) immaturity has been proposed as a key factor in NEC pathophysiology. Gastrointestinal dysmotility is associated with ENS immaturity and may serve as a predictive factor for the development of NEC. In this case-control study, preterm infants (gestational age (GA) < 30 weeks) were included in two level-IV neonatal intensive care units. Infants with NEC in the first month of life were 1:3 matched to controls based on GA (± 3 days). Odds ratios for NEC development were analyzed by logistic regression for time to first passage of meconium (TFPM), duration of meconial stool, and mean daily defecation frequency over the 72 h preceding clinical NEC onset (DF < T0). A total of 39 NEC cases and 117 matched controls (median GA 27 + 4 weeks) were included. Median TFPM was comparable in cases and controls (36 h [IQR 13-65] vs. 30 h [IQR 9-66], p = 0.83). In 21% of both cases and controls, TFPM was ≥ 72 h (p = 0.87). Duration of meconial stool and DF < T0 were comparable in the NEC and control group (median 4 and 3, resp. in both groups). Odds of NEC were not significantly associated with TFPM, duration of meconial stools, and DF < T0 (adjusted odds ratio [95% confidence interval]: 1.00 [0.99-1.03], 1.16 [0.86-1.55] and 0.97 [0.72-1.31], resp.).
CONCLUSION
In this cohort, no association was found between TFPM, duration of meconium stool, and DF < T0 and the development of NEC.
WHAT IS KNOWN
• Necrotizing enterocolitis (NEC) is a life-threatening acute intestinal inflammatory disease of the young preterm infant. Early clinical risk factors for NEC have been investigated in order to facilitate early diagnosis and treatment. • Signs of disrupted gastrointestinal mobility, such as gastric retention and paralytic ileus, have been established to support the diagnosis of NEC. Nevertheless, defecation patterns have insufficiently been studied in relation to the disease.
WHAT IS NEW
• Defecation patterns in the three days preceding NEC did not differ from gestational age-matched controls of corresponding postnatal age. Additionally, the first passage of meconium and the duration of meconium passage were comparable between cases and controls. Currently, defecation patterns are not useful as early warning signs for NEC. It remains to be elucidated whether these parameters are different based on the location of intestinal necrosis.
PubMed: 37349579
DOI: 10.1007/s00431-023-05035-8 -
Travel Medicine and Infectious Disease 2023Several gastrointestinal complications have been reported in patients with COVID-19, including motility disorders, such as acute colonic pseudo-obstruction (ACPO). This...
BACKGROUND
Several gastrointestinal complications have been reported in patients with COVID-19, including motility disorders, such as acute colonic pseudo-obstruction (ACPO). This affection is characterized by colonic distention in the absence of mechanical obstruction. ACPO in the context of severe COVID-19 may be related to neurotropism and direct damage of SARS-CoV-2 in enterocytes.
METHOD
We conducted a retrospective study of patients who were hospitalized for critical COVID-19 and developed ACPO between March 2020 and September 2021. The diagnostic criteria to define ACPO was the presence of 2 or more of the following: abdominal distension, abdominal pain, and changes in the bowel movements, associated with distension of the colon in computed tomography. Data of sex, age, past medical history, treatment, and outcomes were collected.
RESULTS
Five patients were detected. All required admission to the Intensive Care Unit. The ACPO syndrome developed with a mean of 33.8 days from the onset of symptoms. The mean duration of the ACPO syndrome was 24.6 days. The treatment included colonic decompression with placement of rectal and nasogastric tubes, endoscopy decompression in two patients, bowel rest, fluid, and electrolytes replacement. One patient died. The remaining resolved the gastrointestinal symptoms without surgery.
CONCLUSIONS
ACPO is an infrequent complication in patients with COVID-19. It occurs especially in patients with critical condition, who require prolonged stays in intensive care and multiple pharmacological treatments. It is important to recognize its presence early and thus establish an appropriate treatment, since the risk of complications is high.
Topics: Humans; Colonic Pseudo-Obstruction; Argentina; Retrospective Studies; COVID-19; SARS-CoV-2; Syndrome
PubMed: 37315827
DOI: 10.1016/j.tmaid.2023.102604 -
European Journal of Case Reports in... 2023Adult-onset Still's disease (AOSD) is a rare autoinflammatory disorder that can lead to a cytokine storm, causing a range of symptoms. Acute intestinal...
UNLABELLED
Adult-onset Still's disease (AOSD) is a rare autoinflammatory disorder that can lead to a cytokine storm, causing a range of symptoms. Acute intestinal pseudo-obstruction is another rare condition that results in intestinal obstruction without anatomical cause. Although the two conditions are rarely reported together, we present the case of a 62-year-old male who developed acute intestinal pseudo-obstruction in the context of an AOSD flare. This led to severe hypokalaemia and a critical condition. Other symptoms included a high-spiking fever lasting for weeks, polyarthralgias and a typical salmon-coloured rash. After ruling out other potential causes, the patient was diagnosed with AOSD. Our findings suggest that the cytokine storm associated with this disease triggered the acute intestinal pseudo-obstruction and life-threatening hypokalaemia, establishing a causal relationship. Only four other cases of AOSD complicated by intestinal pseudo-obstruction have been reported, and this is the first to present with life-threatening hypokalaemia. This case serves as a crucial reminder that, despite being a diagnosis of exclusion, Still's disease should be considered as a potential cause of intestinal pseudo-obstruction, as prompt recognition and treatment of the underlying cause is crucial in managing this potentially life-threatening condition.
LEARNING POINTS
Acute intestinal pseudo-obstruction is one of the systemic complications that can occur in autoinflammatory diseases such as AOSD, although it has rarely been described.If AOSD presents with abdominal presentation, such as acute intestinal pseudo-obstruction, then amyloidosis should always be carefully considered.
PubMed: 37304998
DOI: 10.12890/2023_003887 -
Journal of Rare Diseases (Berlin,... 2023The visceral myopathies (VM) are a group of disorders characterised by poorly contractile or acontractile smooth muscle. They manifest in both the GI and GU tracts,...
OBJECTIVES/AIMS
The visceral myopathies (VM) are a group of disorders characterised by poorly contractile or acontractile smooth muscle. They manifest in both the GI and GU tracts, ranging from megacystis to Prune Belly syndrome. We aimed to apply a bespoke virtual genetic panel and describe novel variants associated with this condition using whole genome sequencing data within the Genomics England 100,000 Genomes Project.
METHODS
We screened the Genomics England 100,000 Genomes Project rare diseases database for patients with VM-related phenotypes. These patients were screened for sequence variants and copy number variants (CNV) in , , , , , , , and by analysing whole genome sequencing data. The identified variants were analysed using variant effect predictor online tool, and any possible segregation in other family members and novel missense mutations was modelled using in silico tools. The VM cohort was also used to perform a genome-wide variant burden test in order to identify confirm gene associations in this cohort.
RESULTS
We identified 76 patients with phenotypes consistent with a diagnosis of VM. The range of presentations included megacystis/microcolon hypoperistalsis syndrome, Prune Belly syndrome and chronic intestinal pseudo-obstruction. Of the patients in whom we identified heterozygous variants, 7 had likely pathogenic variants including 1 novel likely pathogenic allele. There were 4 patients in whom we identified a heterozygous variant of uncertain significance which leads to a frameshift and a predicted protein elongation. We identified one family in whom we found a heterozygous variant of uncertain significance in which in silico models predicted to be disease causing and may explain the VM phenotype seen. We did not find any CNV changes in known genes leading to VM-related disease phenotypes. In this phenotype selected cohort, is the largest monogenic cause of VM-related disease accounting for 9% of the cohort, supported by a variant burden test approach, which identified variants as the largest contributor to VM-related phenotypes.
CONCLUSIONS
VM are a group of disorders that are not easily classified and may be given different diagnostic labels depending on their phenotype. Molecular genetic analysis of these patients is valuable as it allows precise diagnosis and aids understanding of the underlying disease manifestations. We identified as the most frequent genetic cause of VM. We recommend a nomenclature change to 'autosomal dominant ACTG2 visceral myopathy' for patients with pathogenic variants in and associated VM phenotype.
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s44162-023-00012-z.
PubMed: 37288276
DOI: 10.1007/s44162-023-00012-z -
Journal of Minimal Access Surgery 2023Hysterectomy is the most common gynaecological surgery and there are different techniques of performing hysterectomy. With the advent of laparoscopic technology,...
UNLABELLED
Hysterectomy is the most common gynaecological surgery and there are different techniques of performing hysterectomy. With the advent of laparoscopic technology, laparoscopic hysterectomy (LH) is rapidly gaining its ground. However, every surgery has its complications which are specific but also depends on various factors such as surgical sk[ills and experience of surgeons, levels of operative laparoscopy and patient populations.
AIMS AND OBJECTIVE
In this study, we evaluated the complications of total laparoscopic hysterectomy (TLH) and analysed the trend of complications, intraoperative and post-operative, over a period of time.
METHODS
It was a retrospective study conducted in the private care setting. All women who underwent hysterectomy for benign conditions from a 1 January 2003 to 31 December 2017, (15 years) were included in this study. A total of 3272 patients were operated during this period. All surgeries were performed by a single surgeon.
RESULTS
Intraoperative complications that occurred during surgery during the study period were 3 cases (0.09%) had bladder injury, 3 cases (0.09%) had bowel injury, 1 case (0.03%) had internal iliac vessel bleeding and 1 case(0.03%) needed conversion to vaginal hysterectomy due to cautery failure and post operative complications were 90 cases (2.75%) had vault bleeding, 2 cases (0.06%) had intestinal obstruction, 5 cases (0.15%) had paralytic ileus, 1 case (0.03%) had vesicovaginal fistula, 1 case(0.03%) had ureterovaginal fistula and 1 case (0.03%) had peritonitis.
CONCLUSIONS
TLH is a very effective, patient-friendly and safe technique in the hands of experienced surgeons giving good quality of life to patients postoperatively.
PubMed: 37282419
DOI: 10.4103/jmas.jmas_148_22 -
Nigerian Journal of Clinical Practice Apr 2023Postoperative paralytic ileus refers to the disruption of the normal coordinated propulsive motor activity of the gastrointestinal system following surgery. Surgery...
BACKGROUND
Postoperative paralytic ileus refers to the disruption of the normal coordinated propulsive motor activity of the gastrointestinal system following surgery. Surgery causes inflammation in the muscle walls of organs with an intestinal lumen that, in turn, leads to a decrease in intestinal motility.
AIM
The aim of this study was to investigate the efficacy of gastrografin, neostigmine, and their combined administration in patients diagnosed with paralytic ileus in the postoperative period.
PATIENTS AND METHODS
One-hundred twelve patients were included from January 2017 and November 2019. The retrospective study is involving prolonged postoperative ileus cases following colorectal surgery. The effect of gastrografin, neostigmine, and gastrografin neostigmine combination was compared retrospectively in the treatment of prolonged ileus after surgery.
RESULTS
The study covered 112 patients. Gastrografin was administered to 63 patients; neostigmine was administered to 29, while 20 patients received the combination of the two. Data pertaining to the comparison of the two groups revealed that patients in the gastrografin group were discharged earlier than those in the neostigmine group. Further, patients in the combined group had earlier gas and/or stool discharge and were also discharged from the hospital earlier than those in the neostigmine group.
CONCLUSION
Gastrografin and combined use of gastrografin and neostigmine are effective and viable methods for postoperative ileus cases. Gastrografin can safely be used in patients with anastomoses.
Topics: Humans; Neostigmine; Retrospective Studies; Diatrizoate Meglumine; Ileus; Postoperative Complications; Intestinal Pseudo-Obstruction
PubMed: 37203116
DOI: 10.4103/njcp.njcp_618_22 -
Therapeutic Advances in Urology 2023Urothelial carcinoma can arise from the urinary bladder or from the upper urinary tract. In some instances, urinary bladder cancer (UBC) and upper tract urothelial... (Review)
Review
INTRODUCTION
Urothelial carcinoma can arise from the urinary bladder or from the upper urinary tract. In some instances, urinary bladder cancer (UBC) and upper tract urothelial carcinoma (UTUC) can be concurrently diagnosed, necessitating a combined radical cystectomy (RC) with radical nephroureterectomy (RNU). A systematic review was done on the combined procedure exploring outcomes and indications, in addition to a comparative analysis between the combined procedure and cystectomy alone.
METHODS
For the systematic review, three databases (Embase, PubMed, and Cochrane) were queried, selecting only studies that included intraoperative and perioperative data. For the comparative analysis, using the NSQIP database, CPT codes for RC and RNU were used to identify two cohorts, one with RC and RNU and one with RC alone. A descriptive analysis was performed on all preoperative variables, and propensity score matching (PSM) was performed. Postoperative events were then compared between the two matched cohorts.
RESULTS
For the systematic review, 28 relevant articles were included amounting to 947 patients who underwent the combined procedure. The most common indication was synchronous multifocal disease, the most common approach was open surgery, and the most common diversion technique was using an ileal conduit. Almost 28% of patients required blood transfusion and remained in the hospital for an average of 13 days. The most common postoperative complication was prolonged paralytic ileus. For the comparative analysis, 11,759 patients were included of which 97.5% underwent RC only and 2.5% underwent the combined procedure. After PSM, the cohort that had undergone the combined procedure showed an increased risk of renal injury, increased readmission rates, and increased reoperation rates. Whereas the cohort that had undergone RC only showed an increased risk of deep venous thrombosis (DVT), sepsis, or septic shock.
CONCLUSION
A combined RC and RNU is a treatment option for concurrent UCB and UTUC that should be cautiously utilized as it is associated with high morbidity and mortality. Patient selection, discussion of the risks and benefits of the procedure, and explanation of the available treatment options remain the most important pillars in managing patients with this complex disease.
PubMed: 37188157
DOI: 10.1177/17562872231171757 -
Journal of Clinical Medicine Apr 2023People with hemophilia (PWH), especially severe hemophilia, often experience bleeding episodes, which occur mostly at major joints. Intramural hematoma of the... (Review)
Review
People with hemophilia (PWH), especially severe hemophilia, often experience bleeding episodes, which occur mostly at major joints. Intramural hematoma of the gastrointestinal (GI) tract is a rare, potentially life-threatening clinical bleeding manifestation in PWH. Prompt identification and timely administration of clotting factor concentrates are of utmost importance for effective management and optimal patient outcomes. In this report, we present the case of a 48-year-old male with severe hemophilia A. The patient developed a spontaneous intramural hematoma of the jejunum, leading to signs of acute abdomen, bloody stool, and paralytic ileus. Conservative management with factor VIII (FVIII) infusion was successfully administered. However, within a span of three months, the patient suffered from a recurrent episode of intramural hematoma, which was again effectively treated with conservative therapy. Subsequently, prophylactic FVIII therapy was administered to the patient, resulting in the absence of recurrence for over three years. Inspired by this case, we conducted a comprehensive review of the relevant literature and gathered data from 79 reported cases of intramural hematoma that were documented between the years 1956 and 2022. We classified these cases based on the site affected within the gastrointestinal (GI) tract (spread across five different locations) and proceeded to conduct a simple pooling analysis on the data collected, which subsequently revealed that the overall mortality rate of intramural hematoma in people with hemophilia (PWH) was found to be 12.2%, while children have a higher mortality rate (23.3%) than adults (4.9%). We hope this case report and literature review increase awareness of this rare bleeding manifestation in PWH, the effectiveness of conservative treatment, and the possibility of prophylaxis against recurrence.
PubMed: 37176534
DOI: 10.3390/jcm12093093 -
JPGN Reports Nov 2022Pediatric intestinal pseudo-obstruction (PIPO) is a heterogeneous condition characterized by impaired gastrointestinal propulsion, a broad clinical spectrum, and...
UNLABELLED
Pediatric intestinal pseudo-obstruction (PIPO) is a heterogeneous condition characterized by impaired gastrointestinal propulsion, a broad clinical spectrum, and variable severity. Several molecular bases underlying primary PIPO have been identified, of which autosomal dominant ACTG2-related visceral myopathy is the most common in both familial or sporadic primary PIPO cases. We present a family with autosomal recessive ACTG2-related disease in which both parents have mild gastrointestinal symptoms and sons have severe PIPO and bladder dysfunction.
METHODS
Clinical genome sequencing was performed on the patients and the mother. Immunohistochemistry was performed on intestinal tissue from the patients to show expression levels of the .
RESULTS
Genome sequencing identified a 6.8 kb 2p13.1 loss that includes the gene and a maternally inherited missense variant p.Val10Met in the gene.
DISCUSSION
This case demonstrates that monoallelic hypomorphic variants may underly mild primary gastrointestinal symptoms, while biallelic mild variants can cause severe diseases. The Deletions of the noncoding exon can be an under-recognized cause of mild gastrointestinal symptoms unidentifiable by exome sequencing, explaining some instances of interfamilial variability with an apparent autosomal dominant inheritance. Genome sequencing is recommended as a genetic work-up for primary or idiopathic PIPO because of genetic heterogeneity.
PubMed: 37168481
DOI: 10.1097/PG9.0000000000000258 -
Archives of Gynecology and Obstetrics Feb 2024
Topics: Humans; Female; Pregnancy; Colonic Pseudo-Obstruction; Cesarean Section; Postoperative Complications
PubMed: 37156907
DOI: 10.1007/s00404-023-07052-2