Spleen size evaluation in children: Time to define splenomegaly for pediatric surgeons and pediatricians

Pelizzo, Gloria; Guazzotti, Marinella; Klersy, Catherine; Nakib, Ghassan; Costanzo, Federico; Andreatta, Erika; Bassotti, Gabrio; Calcaterra, Valeria

doi:10.1371/journal.pone.0202741

Cited by 37 publications

(29 citation statements)

References 45 publications

(51 reference statements)

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“…Second, high concentration of serum copper might also have toxic effects on the spleen. Third, with increasing age, the size and function of the spleen are easily affected[38], and the younger age among WD-associated cirrhosis patients might also lead to a higher sensitivity of the spleen to all kinds of stimulation.…”

Section: Discussionmentioning

confidence: 99%

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis

Zhong

Sun

Xue

et al. 2019

WJG

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BACKGROUNDCirrhosis is a chronic late stage liver disease associated with hepatitis viruses, alcoholism, and metabolic disorders, such as Wilson disease (WD). There are no clear markers or clinical features that define cirrhosis originating from these disparate origins. We hypothesized that cirrhosis is not one disease and cirrhosis of different etiology may have differential clinical hepatic features.AIMTo delineate the liver features between WD-associated cirrhosis and hepatitis B-associated cirrhosis in the Chinese population.METHODSIn this observational study, we reviewed the medical data of consecutive inpatients who had WD-associated cirrhosis or hepatitis B-associated cirrhosis from January 2010 to August 2018, and excluded patients who had carcinoma, severe heart or pulmonary diseases, or other liver diseases. According to the etiology of cirrhosis, patients were divided into two groups: WD-associated cirrhosis group (60 patients) and hepatitis B-associated cirrhosis group (56 patients). The liver fibrosis degree, liver function indices, and portal hypertension features of these patients were compared between the two groups.RESULTSNo inter-group differences were observed in the diagnostic liver fibrosis markers, however, clinical features clearly defined the origin of cirrhosis. WD-associated cirrhosis patients (16-29 years) had lower levels of alanine transaminase, aspartate transaminase, and bilirubin, lower prothrombin time, lower incidence of hepatic encephalopathy, and lower portal vein diameter (P < 0.05), compared to cirrhosis resulting from hepatitis B in older patients (45-62 years). Importantly, they had decreased risks of progression from Child-Pugh grade A to B (odds ratio = 0.046, 95% confidence interval: 0.006-0.387, P = 0.005) and of ascites (odds ratio = 0.08, 95% confidence interval: 0.01-0.48, P = 0.005). Conversely, WD-associated cirrhosis patients had a higher risk of splenomegaly (odds ratio = 4.15, 95% confidence interval: 1.38-12.45, P = 0.011).CONCLUSIONWD-associated cirrhosis presents a higher risk of splenomegaly associated with leukopenia and thrombocytopenia, although revealing milder liver dysfunction and portal hypertension symptoms, which recommends WD patients to be monitored for associated complications.

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Section: Discussionmentioning

confidence: 99%

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis

Zhong

Sun

Xue

et al. 2019

WJG

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“…In order to assess organomegaly in ASMD, and other diseases such as Gaucher, spleen and liver volume are usually expressed as multiples of normal based on calculations that assume normal spleen volume is 0.2% of body weight and normal liver volume is 2.5% of body weight [ 15 , 31 , 32 ]. In healthy individuals, in addition to a high correlation with body weight, spleen size is also highly correlated with height and body surface area in children [ 33 , 34 ], and height and sex in adults [ 35 ]. Therefore, in serial long-term assessments of pediatric patients with ASMD such as this 11-year analysis, during which almost all patients who were children at baseline became adults by their final assessment, multiple body parameters may factor into variation in spleen size relative to body size over time.…”

Section: Discussionmentioning

confidence: 99%

Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation

McGovern

Wasserstein

Bembi

et al. 2021

Orphanet J Rare Dis

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Background Acid sphingomyelinase deficiency (ASMD) (also known as Niemann-Pick disease types A and B) is a rare and debilitating lysosomal storage disorder. This prospective, multi-center, multinational longitudinal study aimed to characterize the clinical features of chronic forms of ASMD and disease burden over time in children and adults. Results Fifty-nine patients (31 males/28 females) ranging in age from 7 to 64 years with chronic ASMD types A/B and B and at least two disease symptoms participated from 5 countries. Disease characteristics were assessed at baseline, after 1 year, and at the final visit (ranging from 4.5 to 11 years). Thirty patients (51%) were < 18 years at baseline (median age 12 years), and 29 were adults (median age 32 years). Overall, 32/59 patients completed the final visit, 9 died, 9 discontinued, and 9 were lost to follow up. Common clinical characteristics that tended to worsen gradually with time were splenomegaly, hepatomegaly, interstitial lung disease, lung diffusion capacity (DLCO), and dyslipidemia. Spleen volumes ranged from 4 to 29 multiples of normal at baseline, and splenomegaly was moderate or severe in 86%, 83%, and 90% of individuals at baseline, year 1, and final visits, respectively. The proportion of all individuals with interstitial lung disease was 66% (39/59) at baseline and 78% (25/32) at the final visit, while median % predicted DLCO decreased by > 10% from baseline to the final visit. Nine patients died (15%), eight of causes related to ASMD (most commonly pneumonia); of these eight patients, five (63%) had symptom onset at or before age 2. Overall, six of the nine deaths occurred before age 50 with three occurring before age 20. Individuals with either severe splenomegaly or prior splenectomy were ten times more likely to have died during the follow-up period than those with smaller or intact spleens (odds ratio 10.29, 95% CI 1.7, 62.7). Most children had growth deficits that persisted into adulthood. Conclusions This study provides important information about the natural history of chronic ASMD and provides a longitudinal view of the spectrum of disease manifestations and major morbidities in children and adults and supports the selection of clinically meaningful endpoints in therapeutic trials.

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“…Consistent with other studies, the splenic length increased rapidly until 24 months, while the growth rate was reduced thereafter. 6 , 14 , 18 …”

Section: Discussionmentioning

confidence: 99%

“… 17 An assessment of organ size is an integral part of the evaluation and treatment of disease, particularly before splenectomy. 18 …”

Section: Introductionmentioning

confidence: 99%

Sonographic measurements for spleen size in healthy Saudi children and correlation with body parameters

et al. 2021

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BACKGROUND: Ultrasound is commonly the first-line imaging modality for assessing the visceral organ dimensions without any risk of radiation. An abnormal size of the spleen may indicate disease, but the evaluation is challenging because of the normal size changes with age. In addition, published normal value charts for children may vary by population and methods. OBJECTIVES: Determine normal growth curves for splenic length in relationship to sex, age, body weight, height, body mass index, and body surface area of healthy children in Saudi Arabia. DESIGN: A retrospective review of ultrasonography images SETTING: Tertiary referral hospital SUBJECTS AND METHODS: We included all normal ultrasonography exams of splenic length (from full-term neonates to 16 years of age) performed between 2003 and 2018. The greatest longitudinal distance of the spleen from the dome to the tip (splenic length) measured at the hilum in the coronal plane was obtained by ultrasonography. MAIN OUTCOME MEASURES: The relationship between the longitudinal length of the spleen and sex, age, height, weight, body mass index, and body surface area. SAMPLE SIZE: 1028 healthy children RESULTS: Height had the most significant correlation with the length of the spleen ( P <.001). There was a consistent difference in splenic length according to sex. The spleen was longer in males than in females in children older than 2 years. In terms of splenic growth according to age, our data showed a statistically significant correlation between the ages of 0-24 months and 2-16 years. There was a significant difference between cases from Saudi Arabia, the United States ( P =.023) and India ( P =.045). CONCLUSION: We developed scatter plots between splenic lengths and body parameters among Saudi children for children from 0 day to 24 months and children from 2 years to 16 years for reference. LIMITATION: Retrospective study. Ultrasound techniques in patient positioning, and cursor placement differed from other studies, which can affect measurements of splenic length. CONFLICTS OF INTEREST: None.

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Spleen size evaluation in children: Time to define splenomegaly for pediatric surgeons and pediatricians

Cited by 37 publications

References 45 publications

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis

Prospective study of the natural history of chronic acid sphingomyelinase deficiency in children and adults: eleven years of observation

Sonographic measurements for spleen size in healthy Saudi children and correlation with body parameters

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