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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.
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Esferocitosis Hereditaria , Esplenectomía , Niño , Humanos , Hospitalización , Estudios Retrospectivos , Esferocitosis Hereditaria/complicaciones , Esferocitosis Hereditaria/cirugía , Esplenectomía/efectos adversosRESUMEN
BACKGROUND: Gallstones are common in hereditary spherocytosis (HS) and other chronic hemolytic diseases, with most affected patients being asymptomatic. Whether and how asymptomatic gallstones should be treated is controversial. METHODS: We conducted a retrospective cohort study of pediatric patients with HS and asymptomatic gallstones to compare the clinical outcomes between the observation group (followed up with gallstones in situ) and the intervention group (cholecystectomy or cholecystolithotomy). The primary outcome was the composite of adverse outcomes, including gallstone-associated, gallbladder surgery-associated, and splenectomy-associated events. Secondary outcomes included the changes in the size and number of gallstones and the recurrence of gallstones. RESULTS: Fifty-two patients were included (38 in the observation group and 14 in the intervention group), with a mean follow-up length of 5.2 years. Patients in the intervention group had a lower incidence of primary outcomes (7.1% vs. 42.1%) than patients in the observation group (OR, 0.12; 95%CI, 0.01-0.99). Patients with gallstones >5 mm in diameter had the highest risk of adverse events (47.4%). In the observation group, gallstone growth, disappearance, and stability were observed in 19.4%, 29.0%, and 51.6% of splenectomized HS patients, respectively. Small gallstones (diameter ≤5 mm) or sludge were more likely to be associated with disappearance (P = 0.04). CONCLUSIONS: Most asymptomatic gallstones grow or persist in splenectomized HS patients for an extended period. Surgical treatment of asymptomatic gallstones in HS patients requiring splenectomy is associated with a lower risk of adverse outcomes. LEVELS OF EVIDENCE: III (retrospective comparative study).
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Cálculos Biliares , Esferocitosis Hereditaria , Niño , Humanos , Cálculos Biliares/complicaciones , Cálculos Biliares/cirugía , Estudios Retrospectivos , Esplenectomía/efectos adversos , Esferocitosis Hereditaria/complicaciones , Esferocitosis Hereditaria/cirugía , Colecistectomía , Enfermedad CrónicaRESUMEN
BACKGROUND: Currently, chemotherapy combined with immunotherapy is the established first-line standard treatment for advanced gastric cancer (GC). In addition, the combination of radiotherapy and immunotherapy is considered a promising treatment strategy. CASE SUMMARY: In this report, we present a case of achieving nearly complete remission of highly advanced GC with comprehensive therapies. A 67-year-old male patient was referred to the hospital because he presented with dyspepsia and melena for several days. Based on fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT), endoscopic examination and abdominal CT, he was diagnosed with GC with a massive lesion and two distant metastatic lesions. The patient received mFOLFOX6 regimen chemotherapy, nivolumab and a short course of hypofractionated radiotherapy (4 Gy × 6 fractions) targeting the primary lesion. After the completion of these therapies, the tumor and the metastatic lesions showed a partial response. After having this case discussed by a multidisciplinary team, the patient underwent surgery, including total gastrectomy and D2 lymph node dissection. Postoperative pathology showed that major pathological regression of the primary lesion was achieved. Chemoimmunotherapy started four weeks after surgery, and examination was performed every three months. Since surgery, the patient has been stable and healthy with no evidence of recurrence. CONCLUSION: The combination of radiotherapy and immunotherapy for GC is worthy of further exploration.
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BACKGROUND: Timely discriminating biliary atresia (BA) from other causes of cholestasis is important but challenging. AIMS: To develop a useful diagnostic nomogram and a simplified scoring system to diagnosing BA. STUDY DESIGN: All medical records of the patients who were consecutively admitted to our institution with cholestasis from March 2016 to December 2020 were retrospectively searched. The patients were allocated to the derivation cohort (n = 343) and the validation cohort (n = 246). Multivariable logistic regression models were used to construct the nomogram. The nomogram was validated in both cohorts. The simplified risk score was derived from the nomogram. RESULTS: The nomogram was constructed based on presence of clay stool, gallbladder length, gallbladder emptying index, shear wave elastography value, and gamma-glutamyl transferase level. This model showed good calibration and discrimination ability, with the C-index of 0.968 (95% CI: 0.951-0.984). The discriminating ability is most prominent in the 61-90 days group, with AUC of 0.982 (95% CI: 0.955-1.000). The simplified risk score identified most patients with very high or low risk of BA, and was capable of exempting 64.3% non-BA patients from intraoperative cholangiogram procedure. CONCLUSIONS: This novel diagnostic nomogram had good discrimination and calibration abilities. The simplified scoring system showed significant clinical utility.
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Atresia Biliar , Colestasis , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico , Colestasis/diagnóstico , Colestasis/etiología , Humanos , Modelos Logísticos , Nomogramas , Estudios Retrospectivos , Factores de RiesgoRESUMEN
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.