Hereditary spherocytosis before and after splenectomy and risk of hospitalization for infection.

BACKGROUND: The infectious burden in hereditary spherocytosis (HS) children before splenectomy has rarely been reported and the risk of severe postsplenectomy infection is controversial. METHODS: We conducted a retrospective study of pediatric patients with HS to evaluate the risk of infection presplenectomy and postsplenectomy. The primary outcome was any bacterial, Mycoplasma, or fungal infection that required hospitalization. The secondary outcomes were sepsis and septic shock. Appendectomized children were matched on age at surgery and enrolled as controls. RESULTS: In all, 232 patients were included. Before splenectomy, the primary outcome was identified in 51 (22.0%) patients, and the secondary outcome was identified in 1 (0.4%) patient. After splenectomy, the primary and secondary outcomes were detected in 8 (4.1%) and 1 (0.5%) patients, respectively. The risk of infection was higher presplenectomy than postsplenectomy (OR, 6.6; 95% CI, 3.0-14.2). HS patients had a higher risk of infection than the controls before surgery (OR, 3.7; 95% CI, 2.3-5.9) but not after surgery (OR, 1.4; 95% CI, 0.6-3.6). CONCLUSIONS: HS patients who require splenectomy later in life had a high incidence of hospitalization for infections. In contrast, postsplenectomy risk of hospitalization involving infection or severe infection was low. IMPACT: Patients with hereditary spherocytosis who require splenectomy later in life have a high risk of hospital admission for infections, especially those with severe hereditary spherocytosis. With vaccines or postoperative antibiotics, splenectomy does not increase the risk of infection or severe infections. Splenectomy may reduce the risk of hospitalization for infections by alleviating the complications of hereditary spherocytosis. With vaccines, prophylaxis, or advanced antibiotics, the benefits of splenectomy in children with hereditary spherocytosis and a heavy disease burden may outweigh the risks.

Ileocecal duplication in children: a single-center experience of 115 cases.

To evaluate the clinical features, surgical management, and prognosis of ileocecal duplication in children. A total of 115 patients diagnosed with ileocecal duplication at Beijing Children's Hospital between January 2010 and June 2021 were retrospectively reviewed. Ileocecal duplications were divided into ileal intraluminal (n = 41), ileal extraluminal (n = 24), ileocecal valve (n = 11), cecal intraluminal (n = 18), and cecal extraluminal (n = 3) types according to their locations. Median age at diagnosis was 9.5 (0.1-169.2) months. Intussusception was only observed preoperatively in patients with the ileal intraluminal (8/41), ileocecal valve (4/11), and cecal intraluminal (7/18) types (P = 0.004). Ileocecal resection and ileocolostomy and cyst excision without ileocecal resection were performed in 41 (35.7%) and 74 (64.3%) patients, respectively. The proportions of cyst excision without ileocecal resection performed in patients with different types were 78.0% (32/41), 91.7% (22/24), 27.3% (3/11), 27.8% (5/18), and 100.0% (3/3) (P < 0.001). Time of oral intake (P = 0.003) and hospital stay after surgery (P < 0.001) were significantly shorter in patients undergoing cyst excision without ileocecal resection. There were no significant differences in the complications, growth, and stool frequency (older than 4 years) between patients undergoing different surgical procedures. Regarding the stool consistency (older than 4 years), there was a lower proportion of dry stool in patients undergoing cyst excision (P = 0.008). CONCLUSIONS: Ileocecal duplications at specific locations are prone to intussusception and can influence the surgical procedure choice. At mid-term follow-up, the children's growth and defecation patterns do not seem to be affected by ileocecal resection. WHAT IS KNOWN: • How to address ileocecal duplication has always been challenging in clinical management. • Children who have an ileocecal resection can develop some early postoperative complications. WHAT IS NEW: • Ileocecal duplications at specific locations are prone to intussusception and can influence the surgical procedure choice. • Children's growth and defecation patterns do not seem to be affected by ileocecal resection.

Surgical treatment of benign splenic lesions in pediatric patients: a case series of 30 cases from a single center.

BACKGROUND: Benign splenic lesions are rarely encountered. This study aimed to review the clinical characteristics and surgical outcomes in a case series of 30 pediatric patients. METHODS: From January 1st, 2001 to December 31st, 2021, 30 pediatric patients from a single center were consecutively included. Electronic medical records were reviewed and patients were followed up. Clinical presentations, imaging features, surgical procedures, pathological diagnoses, and prognoses were summarized. The lesion locations and 7-day postoperative platelet levels were compared between total and partial splenectomy patients. RESULTS: Eighteen males and twelve females were included, with mean age at surgery 116.4 ± 43.6 months. The clinical presentations included abdominal pain (16/30), splenomegaly (6/30), skin petechia (2/30), hemolytic jaundice (1/30), and no symptoms (5/30). Pathological diagnoses included congenital epithelial cyst (CEC, 17/30), vascular malformation (8/30), sclerosing angiomatoid nodular transformation (SANT, 3/30), hamartoma (1/30), and leiomyoma (1/30). Patients undergone total splenectomy were more likely to have a lesion involving the hilum than those undergone partial splenectomy (68.4% vs 31.6%, P = 0.021). The 7-day postoperative platelet level was higher in total splenectomy patients than partial splenectomy patients (adjusted means 694.4 × 109/L vs 402.4 × 109/L, P = 0.002). CONCLUSIONS: Various clinical characteristics of pediatric benign splenic lesions are summarized. The most common pathological diagnoses are congenital epithelial cyst and vascular malformation. Partial and total splenectomy result in good prognosis with a low recurrence rate, and the former is preferred to preserve splenic function if possible.

Diagnosis of liver cirrhosis with two-dimensional shear wave elastography in biliary atresia before Kasai portoenterostomy.

PURPOSE: To evaluate the application value of two-dimensional shear wave elastography (2D-SWE) for non-invasive diagnosis of liver cirrhosis (LC) in patients with biliary atresia (BA) before Kasai portoenterostomy (KP), and the cutoff value of liver stiffness measurement (LSM) for diagnosing LC. METHODS: The clinical data of 51 patients with BA who were diagnosed via surgery and pathological results from May 2017 to December 2018 in the department of general surgery, Beijing Children's Hospital, Capital Medical University, were retrospectively analyzed. The liver tissue specimens obtained during KP were evaluated according to the METAVIR criteria. The LSM was obtained using the 2D-SWE technique before KP. RESULTS: There was a grade positive correlation between LSM and METAVIR staging, and the Spearman correlation coefficient was 0.432 (P = 0.002). The AUC for 2D-SWE diagnosing LC (METAVIR score S = 4) in patients with BA before KP was 0.843 (95% confidence interval 0.736 ~ 0.950). The best cutoff value was 16.05 kPa, and the corresponding sensitivity was 75.0%, specificity was 83.7%, positive predictive value (PPV) was 46.1%, negative predictive value (NPV) was 94.7%, and the accuracy was 82.4%. CONCLUSION: 2D-SWE can be used to noninvasively diagnose LC in patients with BA before KP, and the cutoff value is 16.05 kPa.

Risk factors for therapy failure after incision and drainage alone for perianal abscesses in children.

Background: It is well known that recurrent perianal abscesses (PAs) and fistula-in-ano (FIA) are the main causes of therapy failure following incision and drainage (I&D) for PAs. But few studies have focused on the risk factors for therapy failure after I&D for PAs in children. In this study, we retrospectively examine the risk factors for therapy failure after I&D for PAs in children in a pediatric tertiary care institution. Methods: A retrospective review of all outpatient children with PA treated by I&D at Beijing Children's Hospital between January 2021 and December 2022 was performed. A follow-up was conducted in October 2023. Patients with other predisposing factors for perianal infection, such as inflammatory bowel disease, hematologic tumor, and anorectal surgery, were excluded from this study. Logistic regression yielding odds ratios (ORs) was used to assess the significance of variables for therapy failure. Results: Of 160 children initially identified, follow-up was available for 146, with a total of 172 treatments. A total of 91% of children were male. The median (interquartile range) age at I&D was 2 (1, 15) months. The median follow-up duration was 20 (14, 25) months. Therapy failure occurred in 25 (15%) treatments performed for the prevention of recurrence of PA and in 35 (20%) treatments for the prevention of development of FIA. In the univariate analysis, a history of PA (P = 0.001), history of I&D (P = 0.014), and multilocal occurrence (P = 0.003) were associated with therapy failure. A sitz bath after I&D (P = 0.016) and regular cleaning of the wound after I&D (P = 0.024) were associated with therapy success. In the multivariate analysis, a history of PA (P = 0.015, OR = 3.374) and multilocal occurrence (P = 0.012, OR = 4.649) were independently associated with therapy failure. Regular cleaning of the wound (P = 0.017, OR = 0.341) and sitz bath (P = 0.001, OR = 0.128) after I&D were independently associated with therapy success. Conclusions: A history of PA and multilocal occurrence were predictor factors for therapy failure before I&D. Regular cleaning of the wound and sitz bath after I&D were protective factors for therapy success. Therefore, regular cleaning of the wound and sitz bath after I&D should be emphasized in all children with PAs, especially in those with a history of PA and multilocal occurrence.

Associations of COVID-19 vaccination during pregnancy with adverse neonatal and maternal outcomes: A systematic review and meta-analysis.

Objectives: The low COVID-19 vaccine uptake rate among pregnant women is mainly due to safety concerns about COVID-19 vaccines due to limited safety evidence. Our goal was to evaluate the safety of COVID-19 vaccination during pregnancy with up-to-date evidence. Methods: A comprehensive search of MEDLINE, EMBASE, the Cochrane Library, and clinicaltrials.gov was performed on April 5th, 2022, and updated on May 25th, 2022. Studies evaluating the association of COVID-19 vaccination during pregnancy with adverse maternal and neonatal outcomes were included. Two reviewers independently performed the risk of bias assessment and data extraction. Inverse variance random effect meta-analyses were performed to pool outcome data. Results: Forty-three observational studies were included. COVID-19 vaccination [96,384 (73.9%) BNT162b2, 30,889 (23.7%) mRNA-1273, and 3,172 (2.4%) other types] during pregnancy [23,721 (18.3%) in the first trimester, 52,778 (40.5%) in the second trimester, and 53,886 (41.2%) in the third trimester].was associated with reduced risks of stillbirth or neonatal death (OR, 0.74; 95% CI, 0.60-0.92). Sensitivity analysis restricted to studies in participants without COVID-19 showed that the pooled effect was not robust. COVID-19 vaccination during pregnancy was not associated with congenital anomalies (OR, 0.83; 95% CI, 0.63-1.08), preterm birth (OR, 0.98; 95% CI, 0.90-1.06), NICU admission or hospitalization (OR, 0.94; 95% CI, 0.84-1.04), an Apgar score at 5 min <7 (OR, 0.93; 95% CI, 0.86-1.01), low birth weight (OR, 1.00; 95% CI, 0.88-1.14), miscarriage (OR, 0.99; 95% CI, 0.88-1.11), cesarean delivery (OR, 1.07; 95% CI, 0.96-1.19), or postpartum hemorrhage (OR, 0.91; 95% CI, 0.81-1.01). Conclusions: COVID-19 vaccination during pregnancy was not associated with any of the adverse neonatal or maternal outcomes studied. Interpretation of study findings is limited by the types and timing of vaccination. The vaccinations in our study received during pregnancy were primarily mRNA vaccines administered in the second and third trimester. Future RCTs and meta-analysis are warranted to evaluate the efficacy and long-term effects of the COVID-19 vaccines. Systematic review registration: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022322525, identifier: PROSPERO, CRD42022322525.

Effects of vitamin D supplementation during pregnancy on offspring health at birth: A meta-analysis of randomized controlled trails.

BACKGROUND & AIMS: Vitamin D deficiency during pregnancy is common across the world. The effects of maternal vitamin D supplementation on offspring health were contradict from previous studies. This study was conducted to update the effects of vitamin D supplementation during pregnancy on offspring health with new evidence. METHODS: A systematic search of Medline, Embase, the Cochrane Database of Systematic Reviews, ClinicalTrials.gov and ChiCTR.org.cn through July 2021 were conducted. Studies were included if they reported randomized clinical trials comparing vitamin D supplementation with placebo, no supplementation (400 IU/day or less) during pregnancy, and included birth outcomes. Pooled analyses were performed using random-effects models. RESULTS: Forty-two RCTs recruiting 11,082 participants were included. Vitamin D supplementation during pregnancy was associated with a lower risk of intrauterine or neonatal death (RR, 0.69; 95% CI, 0.48-0.99) in 13 RCTs with 6238 participants. We also found prenatal vitamin D supplementation was associated with an increased offspring length at birth (MD, 0.27 cm; 95% CI, 0.02-0.51), increased neonatal vitamin D concentration (MD, 27.72 nmol/L; 95% CI, 20.51-34.92), and reduced risk of vitamin D insufficiency (RR of 0.51; 95% CI, 0.38-0.67), but not associated with birth weight (MD, 37.07 g; 95% CI, -9.67 to 83.80), head circumference (MD, 0.15 cm; 95% CI, -0.02 to 0.32), preterm birth (RR, 0.93; 95% CI, 0.79-1.09), or low birth weight (RR, 0.90; 95% CI, 0.66-1.24). Supplementation initiated before the 20th week of gestation was associated with a decreased risk of small for gestational age (RR, 0.46; 95% CI, 0.24-0.90). CONCLUSIONS: Vitamin D supplementation during pregnancy was associated with improved offspring vitamin D sufficiency status, improved fetal linear growth, and reduced fetal or neonatal mortality. No effect was demonstrated for vitamin D supplementation on birth weight, risk of low birth weight, and risk of preterm birth.

Clinical features of colorectal duplication in children: A study of 25 cases.

PURPOSE: To describe the clinical features and surgical management of pediatric colorectal duplication. METHODS: Twenty-five patients with colorectal duplication at Beijing Children's Hospital between January 2010 and December 2020 were reviewed. Colorectal duplications were divided into cystic, diverticular, tubular and mixed types according to shape. RESULTS: Of the patients, 64% were female, and 44% had accompanying malformations. Abnormal prenatal ultrasound findings (24%), abnormalities immediately found after birth (28%), and postnatal subjective symptoms (48%) were the main clinical presentations. Cystic lesions were observed in 16 patients (64%), including 12 in the colon and 4 in the rectum. Diverticular lesions were observed in 4 patients (16%), and all originated from the sigmoid colon. Among the 4 patients (16%) with tubular lesions, 3 involved the total colon, and the other originated from the splenic flexure to the proximal sigmoid colon. The patient with mixed duplication presented an intact enteric cyst within tubular duplication of the total colon. The mean age at surgery was 20 (0.3-130) months. Except for 4 total colon duplications, complete surgical resection of duplication lesions was performed in all patients. The "distal cross-section, mucosa stripping, and closure" procedure was performed in 3 patients with total colon duplication. Eighteen patients (72%) were followed up for a median time of 54.5 (5-129) months and recovered uneventfully. CONCLUSIONS: Owing to various clinical presentations, colorectal duplication requires careful preoperative differential diagnosis. Complete surgical resection is the preferred radical method for treating cystic, diverticular and short tubular colorectal duplications, except for total colon duplications. LEVEL OF EVIDENCE: Level IV.