Coronary artery calcium (CAC), which is detected using computed tomography scanning, is a well-established indicator of subclinical atherosclerosis. The CAC score is independently associated with atherosclerotic cardiovascular disease (ASCVD) outcomes and provides improved predictive values for estimating the risk of ASCVD beyond traditional risk factors. Thus, CAC is considered to have important implications for reclassification as a decision aid among individuals in the preclinical phase and as the primary prevention of ASCVD. This review is focused on epidemiological evidence on CAC in asymptomatic population-based samples from Western countries and Japan. We also discuss the usability of CAC as a tool for assessing ASCVD risk and its role in the primary prevention of ASCVD. A lack of evidence for the CAC score in ASCVD risk assessment beyond traditional risk factors in populations other than those in Western countries (including Japan) warrants further investigation. Clinical trials are also necessary to demonstrate the usefulness and safety of CAC screening in the primary prevention of ASCVD.

Aims: Patients with familial hypercholesterolemia (FH) are known to have higher exposure to coronary risk than those without FH with similar low-density lipoprotein cholesterol (LDL-C) level. Lipid-lowering medications (LLMs) are the mainstay treatments to lower the risk of premature coronary artery disease in patients with hypercholesterolemia. However, the LLM prescription pattern and its effectiveness among Malaysian patients with FH are not yet reported. The aim of this study was to report the LLM prescribing pattern and its effectiveness in lowering LDL-C level among Malaysian patients with FH treated in specialist hospitals.

Methods: Subjects were recruited from lipid and cardiac specialist hospitals. FH was clinically diagnosed using the Dutch Lipid Clinic Network Criteria. Patients’ medical history was recorded using a standardized questionnaire. LLM prescription history and baseline LDL-C were acquired from the hospitals’ database. Blood samples were acquired for the latest lipid profile assay.

Results: A total of 206 patients with FH were recruited. Almost all of them were on LLMs (97.6%). Only 2.9% and 7.8% of the patients achieved the target LDL-C of ＜1.4 and ＜1.8 mmol/L, respectively. The majority of patients who achieved the target LDL-C were prescribed with statin–ezetimibe combination medications and high-intensity or moderate-intensity statins. All patients who were prescribed with ezetimibe monotherapy did not achieve the target LDL-C.

Conclusion: The majority of Malaysian patients with FH received LLMs, but only a small fraction achieved the therapeutic target LDL-C level. Further investigation has to be conducted to identify the cause of the suboptimal treatment target attainment, be it the factors of patients or the prescription practice.

Aim: Although recent advances in endovascular devices have markedly improved clinical outcomes of femoropopliteal endovascular therapy, lesions located in the popliteal artery are still a major challenge. This study aimed to determine the association of cardiovascular risk factors, including smoking, diabetes mellitus, and dialysis-dependent renal failure, with the location of atherosclerotic lesions in femoropopliteal artery disease.

Methods: We used a multicenter prospective study database registering patients with symptomatic femoropopliteal artery disease undergoing drug-coated balloon treatment. The analysis included 1912 patients with de novo femoropopliteal lesions. The association of clinical characteristics with popliteal lesions was investigated using the logistic regression model. In addition, the femoropopliteal artery was divided into six segments (the proximal, middle, and distal portions of the superficial femoral artery and P1, P2, and P3 segments of the popliteal artery), and the association of clinical characteristics with the presence of atherosclerotic lesions in the respective arterial segments was investigated.

Results: Smoking and dialysis-dependent renal failure showed a statistically significant inverse and positive association with the presence of popliteal lesions, respectively (adjusted odds ratio, 0.66 [95% confidence interval, 0.51–0.85] and 2.01 [1.62–2.49]; P=0.001 and P＜0.001), whereas diabetes mellitus did not (P=0.17). The subsequent per-segment analysis presented similar results.

Conclusions: Smoking was inversely associated with popliteal lesions, whereas renal failure on dialysis was positively associated in patients with symptomatic femoropopliteal artery disease who underwent drug-coated balloon treatment. Diabetes mellitus was not significantly associated.

Aim: Blood cholesterol absorption and synthesis biomarkers predict cardiovascular risk. This study aimed to determine the values of serum non-cholesterol sterol markers [lathosterol (Latho), campesterol (Campe), and sitosterol (Sito)] in healthy individuals and factors affecting these markers.

Methods: The CACHE Consortium compiled clinical data, including serum Latho (cholesterol synthesis marker), and Campe and Sito (cholesterol absorption markers), by a gas chromatography method in 2944 individuals. Healthy subjects were selected by excluding those with prior cardiovascular disease, diabetes mellitus, hypertension, chronic kidney disease, familial hypercholesterolemia, sitosterolemia, current smokers, those with low (＜17 kg/m²) or high (≥ 30 kg/m²) body mass index (BMI), and those with treatment for dyslipidemia or hyperuricemia. Nonlinear regression stratified by sex was used to examine the associations of cholesterol metabolism markers with age, BMI, and serum lipid levels.

Results: Of 479 individuals selected, 59.4% were female; the median age was 48 years in females and 50 years in males. The three markers showed positively skewed distributions, and sex differences were present. Age was associated positively with Latho, inversely with Campe, but not significantly with Sito. BMI was associated positively with Latho, but not significantly with Campe or Sito. High-density lipoprotein cholesterol (HDL-C) was positively associated with Campe and Sito, but not significantly with Latho. Non-HDL-C was positively associated with the three markers.

Conclusion: Our study results in the healthy subjects help to interpret the non-cholesterol sterol markers for cardiovascular risk assessment in patients with cardiovascular risk factors.

Aims: This study aimed to clarify the relationships among tooth loss, periodontal condition, and subclinical atherosclerosis from the aspect of intensity, extent, and duration of inflammation.

Methods: This cross-sectional study included 9,778 people from the Nagahama Study, a large-scale, general population-based study conducted in Japan. The number of teeth and periodontal status, including the attachment level (AL) and pocket depth (PD) of representative teeth from six regions, were evaluated by dentists. The maximum intima-media thickness (IMT) of the common carotid artery was used as an index of atherosclerosis.

Results: In the multivariate analysis adjusted for conventional risk factors, a large number of missing teeth (＜9 remaining teeth), which related to long-lasting inflammation indicative of the highest stage of periodontitis, was identified as an independent determinant of IMT in a general population (coefficient: 0.042; 95% confidence interval [CI]: 0.016 to 0.068). The presence of two or more regions with an AL ≥4 mm, which is indicative of the progressing, long-lasting stages of periodontal inflammation, was also independently associated with IMT (coefficient: 0.016; 95% CI: 0.004 to 0.028). On the contrary, PD, a measure of the early and reversible phases of periodontal inflammation, and loss of AL in the group without tooth loss were not significantly associated with IMT, because of the limited degree of accumulated periodontitis.

Conclusion: The present results suggest that the association between periodontitis and atherosclerosis depends on the inflammation intensity, extent, and duration.

Aims: The long-term prognostic value of the bioavailability of L-arginine, an important source of nitric oxide for the maintenance of vascular endothelial function, has not been investigated fully. We therefore investigated the relationship between amino acid profile and long-term prognosis in patients with a history of standby coronary angiography.

Methods: We measured the serum concentrations of L-arginine, L-citrulline, and L-ornithine by high-speed liquid chromatography. We examined the relationship between the L-arginine/L-ornithine ratio and the incidence of all-cause death, cardiovascular death, and major adverse cardiovascular events (MACEs) in 262 patients (202 men and 60 women, age 65±13 years) who underwent coronary angiography over a period of ≤ 10 years.

Results: During the observation period of 5.5±3.2 years, 31 (12%) patients died, including 20 (8%) of cardiovascular death, while 32 (12%) had MACEs. Cox regression analysis revealed that L-arginine/L-ornithine ratio was associated with an increased risk for all-cause death (unadjusted hazard ratio, 95% confidence interval) (0.940, 0.888–0.995) and cardiovascular death (0.895, 0.821–0.965) (p＜0.05 for all). In a model adjusted for age, sex, hypertension, hyperlipidemia, diabetes, current smoking, renal function, and log₁₀-transformed brain natriuretic peptide level, cardiovascular death (0.911, 0.839–0.990, p=0.028) retained an association with a low L-arginine/ L-ornithine ratio. When the patients were grouped according to an L-arginine/L-ornithine ratio of 1.16, the lower L-arginine/L-ornithine ratio group had significantly higher incidence of all-cause death, cardiovascular death, and MACEs.

Conclusion: A low L-arginine/L-ornithine ratio may be associated with increased 10-year cardiac mortality.

Aims: Skipping breakfast is known as a cardiovascular disease (CVD) risk. Recently, eating and dietary patterns have been varied in many countries, however, the mechanisms of promoting CVD are still unclear. Our goal was to assess the effects of eating and dietary patterns on CVD risk factors, with a focus on lipid parameters including serum concentration of small dense low-density lipoprotein cholesterol (sdLDL-C).

Methods: Subjects consisted of 27,997 Japanese men and women, who had medical check-up. Lipid parameters including sdLDL-C levels in breakfast skippers were compared with those in breakfast eaters. Lipid parameters in staple foods skippers were also compared with those in staple foods eaters.

Results: Breakfast skippers had significantly (p＜0.01) higher serum levels of median sdLDL-C than breakfast eaters in both genders (34.7 versus 32.0 mg/dL in men, 25.4 versus 24.9 mg/dL in women, respectively), and the same was true for sdLDL-C/LDL-C ratio (0.276 versus 0.260 in men, 0.218 versus 0.209 in women, respectively). Similarly, staple foods skippers had significantly higher sdLDL-C level than staple foods eaters in both genders (34.1 versus 31.6 mg/dL in men, 25.8 versus 24.7 mg/dL in women), and the same went for sdLDL-C/LDL-C ratio (0.278 versus 0.256 in men, 0.215 versus 0.208 mg/dL in women, respectively).

Conclusion: Our data indicate that both skipping breakfast and having meals without staple foods increase serum sdLDL-C concentration and cause unfavorable lipid profiles, and then may promote CVD. These finding provide evidence for the importance of having breakfast and meals with staple foods for preventing CVD.

Aim: A cluster randomized trial was conducted within 41 Japanese municipalities (21 intervention and 22 usual care) to examine whether the standardized health counseling for individuals at high cardiovascular risk screened at community sites accelerates clinic visits to strengthen the primary health care system.

Methods: Among high-risk individuals aged 40–74 years screened by health checkups, 8,977 and 6,733 were allocated to the intervention and usual care groups, respectively, who were not under medical treatment but had high levels of blood pressure (systolic/diastolic ≥ 160/100 mmHg), hemoglobin A1c or glucose (≥ 7.0% or corresponding glucose levels), LDL-cholesterol (≥ 180 mg/dL for men), and/or proteinuria of ≥ 2+. The intervention was performed from May 2014 to March 2016 under a standardized health counseling program based on the health belief model primarily by public health nurses. The usual care group was provided with local counseling protocols.

Results: The cumulative proportions of clinic visits for 12 months after health checkups were 58.1% (95% confidence interval, 57.0%, 59.3%) versus 44.5% (43.2%, 45.8%), with the probability ratio of clinic visits between the groups being 1.46 (1.24, 1.72). The between-group differences between the baseline and 1-year surveys were −1.50 (−2.59, −0.41) mmHg for diastolic blood pressure in the hypertension category, −0.30% (−0.53%, −0.07%) for HbA1c in the diabetes category, −0.37 (−0.48, −0.27) mmol/L for LDL-cholesterol in the dyslipidemia category, and none for proteinuria.

Conclusion: Standardized health counseling for high-risk individuals accelerated clinic visits, with larger reductions in blood pressure, HbA1c, and LDL-cholesterol levels. The nationwide use of counseling after health checkups for high-risk individuals could help in controlling risk factors and in preventing lifestyle-related diseases.

Aim: To investigate the incidence and in-hospital mortality of acute myocardial infarction (AMI) by conforming to the Universal Definition of Myocardial Infarction (UDMI) in a population-based registry.

Methods: The Shiga Stroke and Heart Attack Registry is a multicenter, population-based registry in the Shiga Prefecture, designed to evaluate the incidence and prognosis of acute cerebro-cardiovascular diseases. We registered patients with AMI as defined by the UDMI, who developed AMI from January 2014 to December 2015 in the Shiga Prefecture. The incidence rate of AMI was calculated and standardized for age by a direct method using the 2015 Japanese population. We also evaluated the in-hospital mortality among hospitalized patients with ST-elevation myocardial infarction (STEMI) and non-STEMI (NSTEMI).

Results: A total of 1,587 patients were diagnosed with AMI, and the age-adjusted incidence rate was 61.9 per 100,000 person-years. The proportions of patients with STEMI, NSTEMI, and type 3 myocardial infarction were 57%, 30%, and 12%, respectively. The incidence rates of AMI increased with age regardless of sex, which was the highest in those 85 years and older, with 389.7 per 100,000 person-years in men and 221.8 per 100,000 person-years in women. Age-adjusted in-hospital mortality among hospitalized patients with STEMI and NSTEMI was 12.3% and 5.8%, respectively.

Conclusions: This population-based registry clarified the age-adjusted incidence rate of AMI under the application of the UDMI, highlighting that in-hospital mortality is still high among patients with STEMI in Japan.

Aim: Hypertensive disorders of pregnancy (HDP) are estimated to occur in up to 10% of all pregnancies and are associated with an increased risk of future cardiovascular disease (CVD) and chronic hypertension (HT). Therefore, we examined the impact of a history of HDP on CVD possibility in middle- and older-aged Japanese women.

Methods: We used the Tohoku Medical Megabank database to obtain the data of 26,024 menopausal women who were aged ≥ 50 years, had children, did not smoke, and did not have chronic kidney disease and to analyze the relationship between HDP history and CVD.

Results: A history of HDP was found in 4.6% of women. We divided the women into four groups according to the presence or absence of HDP and HT. The percentage of women with dyslipidemia, diabetes mellitus, and body mass index of ≥ 25 kg/m² was the highest in the HDP＋ HT＋ group compared to the other groups (43.4%, 24.0%, and 45.2%, respectively). Adjusted odds ratio (OR) for the combined six CVD categories was higher for those with a history of HDP alone (OR [95% confidence interval [CI]]: 1.61 [1.03–2.53]). Moreover, the OR was significantly higher for those with combination with HDP history and HT (OR [95% CI]: 4.11 [3.16–5.35]). The prevalence of individual CVD was also the highest in the HT＋ HDP＋ group.

Conclusion: An HDP history can influence the risk of CVD in Japanese women, indicating the importance of information about pregnancy outcomes in health management.

Aims: The association between dietary patterns and serum low density lipoprotein (LDL) cholesterol would be changing in recent dietary habits in Japan. We investigated the relationship between dietary patterns and serum LDL cholesterol in a large general population.

Methods: From the baseline survey of Japan Multi-Institutional Collaborative Cohort Study between 2005 and 2013, 27,237 participants (13,994 were women) aged 35–69 years were cross-sectionally analyzed. Using a semi-quantitative food frequency questionnaire, five major sex-specific dietary patterns were identified using factor analysis. We assessed serum LDL cholesterol by quintiles of dietary pattern factor score.

Results: We identified dietary patterns; “vegetable rich pattern” , “meat and fried food rich pattern” and “high bread and low rice pattern” in women and men; “fish and shellfish rich pattern” and “high confectioneries and low alcohol pattern” in men; “healthy Japanese diet pattern” and “high alcohol and low rice pattern” in women. Serum LDL cholesterol in men was associated with “high bread and low rice pattern” score (Q5 was 4.2 mg/dL higher than Q1, p for trend ＜0.001) and “high confectioneries and low alcohol pattern” scores (Q5 was 9.5 mg/dL higher than Q1, p for trend ＜0.001). In women, serum LDL cholesterol was associated with “high bread and low rice pattern” score (Q5 was 7.1 mg/dL higher than Q1, p for trend ＜0.001).

Conclusion: Some recent dietary patterns in Japan were associated with serum LDL cholesterol. Serum LDL cholesterol was associated with high bread and low rice pattern in both sex, and high confectioneries and low alcohol pattern in men.

Aim: Intracranial atherosclerotic stenosis (ICAS) is the leading cause of ischemic stroke worldwide. Hyperlipidemia is a major contributor to atherosclerosis. However, the effect of hyperlipidemia on the evolution of intracranial atherosclerotic plaques and downstream ischemic episodes remains unclear. In this study, we aimed to assess the radiological features of ICAS plaques and to explore the relationship between hyperlipidemia and plaque progression.

Methods: We included people with ICAS (≥50% stenosis) undergoing high-resolution magnetic resonance imaging. The culprit plaque was defined as the sole, or in case of multiple stenosis, the narrowest plaque on the intracranial artery responsible for acute ischemic stroke. Demographic, clinical data, plaque features on MRI, and lipid parameters were compared between culprit and non-culprit plaques. Plaque enhancement was graded as Grade 0, 1 and 2 by comparing to the adjacent normal vessel wall and pituitary funnel after contrast enhancement on T1-weighted sequences.

Results: 162 patients were included (mean age 57.7±12.1 years, male 61.6%), 110 of whom were identified as culprit plaque with an ipsilateral acute stroke. High-grade enhancement was the most prominent MRI feature of the culpable plaque (Grade-2: OR 6.539, 95%CI 1.706-23.707, p=0.006). LDL cholesterol was significantly associated with overall acute ischemic stroke caused by culprit plaque. After stratification by enhancement grading LDL was independently associated with ischemic events in Grade-1 enhancement plaques (OR 6.778, 95%CI 2.122-21.649, p=0.001). In patients with Grade-2 enhancement plaques, however, LDL was not associated with ischemic event; in contrast, Neutrophil/Lymphocyte ratio was independently associated with ischemic events caused by Grade-2 enhancement plaques (OR 2.188, 95%CI 1.209-3.961, p=0.010).

Conclusions: LDL was related with ischemia events in intermediate stage of intracranial atherosclerotic plaque progression, an excellent period for intensive lipid-lowering treatment. In advanced stage, inflammatory agents maybe the main contributor to ischemic events.

Aim: The present study investigated the effects of additional cilostazol administration on the 12-month risk of restenosis after femoropopliteal heparin-bonded stent graft implantation.

Methods: This study was a sub-analysis of the Viabahn stent graft placement for femoropopliteal disease reQUIring endovaScular tHerapy (VANQUISH) study, which was a prospective multicenter study investigating patients who received Viabahn stent graft (W.L. Gore & Associates, Flagstaff, AZ, USA) and dual-antiplatelet therapy with aspirin and a thienopyridine. The comparison of clinical outcomes between subgroups with and without cilostazol treatment were performed using the propensity score-matching method to minimize the intergroup differences in baseline characteristics.

Results: Cilostazol-treated patients had a lower 12-month proportion of restenosis than cilostazol-free patients (8.2% vs 27.3%). The odds ratio of cilostazol for the 12-month restenosis was 0.27 [95% CI, 0.08 to 0.97] (p=0.045). Furthermore, the cumulative incidence rates of surgical reconstruction, target lesion revascularization and acute thrombotic occlusion (p values by the log-rank test) were 2.6% versus 1.8% (P=0.43), 5.3% versus 20.5% (P=0.067), and 0.0% versus 11.8% (P=0.0499), respectively. The rates of surgical reconstruction and target lesion revascularization (TLR) were not significantly different between both groups.

Conclusions: Our study revealed the clinical impact of additional cilostazol treatment on the risk of restenosis and acute thrombotic occlusion following heparin-bonded stent graft implantation, while TLR and surgical reconstruction were not significantly different.

Aim: We examined the association between the carotid intima medica thickness (cIMT) and risk factors for atherosclerosis based on the Japan Atherosclerosis Society 2022 Atherosclerosis Prevention Guideline.

Methods: Using data from the Tohoku Medical Megabank Community-based Cohort Study, we performed a cross-sectional study that enrolled 13,366 participants (age ≥ 20 years) with an analysis of covariance to assess associations between cIMT and risk factors for atherosclerosis. The maximum common carotid artery was measured using high-resolution B-mode ultrasound. Analysis was conducted in the model adjusted for age, sex, smoking status, drinking status, body mass index (BMI), systolic blood pressure (SBP), glycated hemoglobin (HbA1c), high-density lipoprotein-cholesterol (HDL-C), non-high-density lipoprotein-cholesterol (non-HDL-C), and height.

Results: In this study cohort, the average age and cIMT were 57.3 (13.8) years and 0.61 (0.13) mm, respectively, which included 3,988 males (29.8%). Males had a higher cIMT than did the females. Age, height, BMI, SBP, HbA1c, and non-HDL-C were positively associated with cIMT. HDL-C was inversely associated with cIMT. Compared with never drinkers, current drinkers (≥ 46.0 g/day) had a significantly decreased cIMT.

Conclusions: The cIMT was associated with atherosclerosis risk factors including age, sex, BMI, SBP, HbA1c, non-HDL-C, and HDL-C, and adequate control of risks in high-risk individuals might be required to prevent atherosclerotic cardiovascular diseases.

Aim: We aimed to examine sex-specific risk factors for hyperuricemia or gout in Japanese cohorts.

Methods: We followed up 3,188 men (mean age, 55.6 years) and 6,346 women (mean age, 54.1 years) without hyperuricemia, gout, or elevated liver enzymes at baseline from 1986 to 1990 for a median of 14.6 years. The participants were considered as having hyperuricemia or gout if their serum uric acid levels were ≥ 7.0 mg/dL or they were receiving treatment for hyperuricemia or gout during annual health checkups. The sex-specific multivariable hazard ratios (HRs) of hyperuricemia or gout incidence were calculated after adjustment for smoking and drinking status, body mass index, hypertension, diabetes, hypercholesterolemia, and hypertriglyceridemia using the Cox proportional-hazard model.

Results: During follow-up, 733 men and 355 women had hyperuricemia or gout. Among men, the multivariable HRs (95% confidence intervals) of hyperuricemia or gout were 1.23 (1.00–1.52) and 1.41 (1.13–1.75) for drinkers of ＜46 and ≥ 46 g ethanol/day, respectively, compared with non-drinkers; 1.00 (0.81–1.24) and 1.18 (0.93–1.50) for smokers of 1–19 and ≥ 20 cigarettes/day, respectively, compared with never smokers; and 1.41 (1.20–1.65) for hypertensive compared with non-hypertensive participants. The HRs for women were 1.02 (0.70–1.48), 1.66 (1.05–2.63), and 1.12 (0.88–1.42) for current drinkers, current smokers, and hypertensive participants, respectively. For both men and women, body mass index, diabetes, hypercholesterolemia, and hypertriglyceridemia were not associated with hyperuricemia or gout incidence.

Conclusions: Hypertension and alcohol drinking are risk factors for hyperuricemia or gout among men and smoking among women.

Aims: To examine the trajectory of white blood cell (WBC) and their potential impacts on cardiovascular disease (CVD) and all-cause mortality (ACM) risks.

Methods: This prospective cohort included 61,666 participants without CVD on or before June 1, 2012. Latent mixture modeling was used to identify WBC trajectories in 2006-2012 as predictors of CVD and ACM. Incident CVD and ACM in 2012-2019 were the outcomes. Cox proportional hazards models were fitted to analyze the risks of incident CVD and ACM.

Results: According to WBC ranges and dynamics, five distinct WBC trajectories were identified: low-stable (n=18,432), moderate-stable (n=26,656), elevated-stable (n=3,153), moderate-increasing (n=11,622), and elevated-decreasing (n=1,803). During 6.65±0.83 years of follow-up, we documented 3773 incident CVD cases and 3304 deaths. Relative to the low-stable pattern, the moderate-increasing pattern was predictive of an elevated risk of CVD (HR=1.36, 95% CI: 1.24-1.50), especially acute myocardial infarction (AMI) (HR=1.91, 95% CI: 1.46-2.51), while the elevated-stable pattern was predictive of an elevated risk of ACM (HR=1.77, 95% CI: 1.52-2.06). Among participants with hs-CRP ＜2 mg/L or ≥2 mg/L, similar associations were observed between the moderate-increasing pattern with CVD (HR=1.41, 95% CI: 1.24-1.61) and ACM (HR=1.54, 95% CI: 1.18-2.01, HR=1.89, 95% CI: 1.57-2.29, respectively).

Conclusions: We found that distinct WBC trajectories were differentially associated with CVD and ACM risks in Chinese adults.

A one-year-and-nine-month-old Japanese boy was admitted with hypertriglyceridemia (fasting triglycerides 2548 mg/dL). After close examination, he was diagnosed with lipoprotein lipase (LPL) deficiency (compound heterozygous) and was immediately started on a fat-restricted dietary therapy. He responded well to the regimen (1200 kcal/day, 20 g fat/day) and his triglycerides decreased to 628 mg/dL within 7 days of starting the dietary therapy. It was decided to manage his illness without using any drugs because he was still an infant and responded well to a fat-restricted diet. During his hospital stay, dietitians provided him with nutritional counseling using a food exchange list, which was designed to easily calculate the fat content by including foods that are commonly served. His family quickly learned the skills to prepare a fat-restricted diet. Moreover, since dietary restrictions may have impaired the child’s growth and development, the dietitians continued to intervene regularly after the child was discharged from the hospital. The dietitians confirmed that the patient was receiving nutritional intake appropriate for his growth and discussed the dietary concerns in his daily life and how to participate in school events that involved eating and drinking. Nutritional counseling was provided every 3–4 months from disease onset to age 23 years, except for a 14-month break at age 20 years. The patient grew up without developing acute pancreatitis, a serious complication of LPL deficiency. The long-term intervention of dieticians is necessary to achieve a balance between living on a strict diet for disease management and ensuring appropriate nutritional intakes for growth/development.