Intensive Care Therapy Research Articles

Association of Body Mass Index and Outcome in Acutely Ill Patients With Chronic Kidney Disease Requiring Intensive Care Therapy

An association of body mass index (BMI) and outcome, the "obesity paradox," has been described in patients with chronic kidney disease (CKD) and end-stage renal disease. We sought to assess whether a potential beneficial effect of a high body mass is also seen in CKD patients with critical illness. In a retrospective analysis of a prospectively collected database of 123,416 patients from 107 Austrian intensive care units (ICUs) in whom BMI was available, the association of 6 groups of BMI and hospital mortality was assessed in 12,206 patients with CKD 3-5 by univariate and multivariate logistic regression analyses. Patients with CKD were sicker, had a longer ICU stay, and had a higher ICU and hospital mortality than those without. The association of BMI and outcome in CKD patients indicated a U-shaped curve with the highest mortality in patients with BMI <20 and ≥40, and the lowest with a BMI between ≥25 and <40. This relationship was also significant in a multivariate analysis adjusted for severity of illness assessed by Simplified Acute Physiology Score III score, age, gender, admission diagnosis, and pre-existing comorbidities. It was not found in patients with CKD 5 on renal replacement therapy, in patients below 60years of age, and those with diabetes mellitus requiring insulin treatment. BMI is associated with better outcomes in CKD 3-5 patients who have acquired acute intermittent diseases and are admitted to an ICU, but not those requiring renal replacement therapy. This higher tolerance to acute disease processes may in part explain the "obesity paradox" observed in CKD patients.

Fluid resuscitation after severe trauma injury : U-shaped associations between tetrastarch dose and survival time or frequency of acute kidney failure.

Using tetrastarch for fluid resuscitation after asevere trauma injury may increase risks of death and acute kidney injury. The importance of tetrastarch dose, however, is unknown. Aretrospective observational study was performed in two trauma centres using data on type and amount of fluids (balanced crystalloids or tetrastarch) used for pre- and acute in-hospital shock management. We evaluate independent associations between the relative and absolute volumes of tetrastarch and 90-day survival time or the frequency of severe acute kidney failure (AKF). We studied 271 patients who had sustained asevere blunt trauma injury (average predicted mortality according to the Revised Injury Severity Classification Score (RISC) 15.1 ± 1.4% [mean, standard deviation]), and who had required more than 2days of intensive care therapy. In all, 75.3% of patients had received tetrastarch with acrystalloid/colloid ratio of 2.93 ± 2.60. The 90-day mortality was 11.1%, and 7.8% of the patients developed severe AKF. After adjusting for confounders, we found aU-shaped, nonlinear association between absolute or relative volumes of tetrastarch and survival time (p = 0.003 and 0.025, respectively). Optimal relative volumes of tetrastarch approximately ranged from 20 to 30% of total fluids. Giving less than about 1000 ml, or more than about 2000 ml tetrastarch was significantly associated with an increased risk of developing severe AKF (p = 0.023). There was acomplex U‑shaped association between the tetrastarch dose and morbidity/mortality of patients after asevere trauma injury. The optimal crystalloid/tetrastarch ratio for acute shock management appears to range from about 2.5 to 4.0.

Socioeconomic status and brain injury in children born preterm: modifying neurodevelopmental outcome.

Improved intensive care therapies have increased the survival of children born preterm. Yet, many preterm children experience long-term neurodevelopmental sequelae. Indeed, preterm birth remains a leading cause of lifelong neurodevelopmental disability globally, posing significant challenges to the child, family, and society. Neurodevelopmental disability in children born preterm is traditionally linked to acquired brain injuries such as white matter injury and to impaired brain maturation resulting from neonatal illness such as chronic lung disease. Socioeconomic status (SES) has long been recognized to contribute to variation in outcome in children born preterm. Recent brain imaging data in normative term-born cohorts suggest that lower SES itself predicts alterations in brain development, including the growth of the cerebral cortex and subcortical structures. Recent evidence in children born preterm suggests that the response to early-life brain injuries is modified by the socioeconomic circumstances of children and families. Exciting new data points to the potential of more favorable SES circumstances to mitigate the impact of neonatal brain injury. This review addresses emerging evidence suggesting that SES modifies the relationship between early-life exposures, brain injury, and neurodevelopmental outcomes in children born preterm. Better understanding these relationships opens new avenues for research with the ultimate goal of promoting optimal outcomes for those children born preterm at highest risk of neurodevelopmental consequence.

Therapeutic Management of Trauma-related Acute Pancreatitis in a Heart Transplant Recipient

Acute pancreatitis is a very mortal disease, mortality that increases even more in patients with cardiac transplantation. Medical-surgical management of acute pancreatitis in transplanted patients can make the difference between life and death. The aim of this paper was to highlight the severity of this pathology especially because the patient is immunosuppressed after cardiac transplant. A case of 36-year-old man, known with heart transplant, immunosuppressive treatment and chronic renal frailer, who arrived to Emergency Department, with severe abdominal pain and abdominal distention which started after a traumatic accident. Investigations revealed acute pancreatitis that needed three surgeries for acute necrotic hemorrhagic pancreatitis, acute bleeding, left subphrenic abscess and intensive care therapy. With favorable postoperative evolution, patient is discharged 60 days later He�s follow up reveled no gastrointestinal or cardiac complication with an improved quality of life.

Early Multidisciplinary Intensive-care Therapy can Improve Outcome of Severe Anti-NMDA-receptor Encephalitis Presenting with Extreme Delta Brush.

Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDARE) is a synaptic autoimmune encephalitis syndrome mainly affecting young females. An underlying tumor, most commonly ovarian teratomas in young females, may indicate a paraneoplastic syndrome. Prognostic factors of the clinical course of disease and outcome play a central role in view of early administration of second-line immunotherapy and intensive-care therapy. We report a case of severe Anti-NMDARE associated with unfavorable predictors including an extreme delta brush (EDB) electroencephalographic-pattern and high anti-NMDAR-antibody titers in the cerebral spinal fluid (CSF), which necessitated the admission to an intensive care unit. In spite of the poor prognosis, the patient completely recovered; we attribute this to an early escalation to second-line immunotherapy with rituximab and multidisciplinary intensive-care therapy. The present case underlines the relevance of multidisciplinary management for individuals with Anti-NMDARE.

Research protocol for mechanical complications after central venous catheterisation: a prospective controlled multicentre observational study to determine incidence and risk factors of mechanical complications within 24 hours after cannulation

IntroductionCentral venous catheterisation is a common procedure in intensive care therapy and the use of central venous catheters is essential for treatment of many medical disorders. Although rare, central venous...

Diagnostics and treatment of selected clinically relevant, acute drug intoxications

Acute drug poisoning due to accidental or self-damaging overdoses is responsible for 5-10% of emergency medical interventions in Germany. The treatment of asymptomatic to life-threatening courses requires extensive expertise. On the basis of aselective literature search, this article gives an overview of selected clinically relevant, acute drug poisonings with regard to epidemiology, symptomatology, diagnostics, and therapy.Intoxications with psychotropic drugs are the most common drug intoxications. Poisoning with tricyclic antidepressants causes anticholinergic, central nervous, and cardiovascular symptoms. Less toxic are selective serotonin reuptake inhibitors (SSRIs); the intoxication may be characterized by serotonin syndrome. Malignant neuroleptic syndrome is asevere complication of neuroleptic poisoning.Poisoning with analgesics is clinically relevant due to its high availability. For paracetamol poisoning, intravenous acetylcysteine is available as an antidote. Hemodialysis may be indicated for severe salicylate intoxication. Poisoning with nonsteroidal anti-inflammatory drugs is usually only associated with mild signs of intoxication.Poisoning with cardiac drugs (β-blockers and calcium antagonists) can cause life-threatening cardiovascular events. In addition to symptomatic therapy, insulin glucose therapy also plays an important role.The majority of acute drug poisonings can be treated adequately by symptomatic and partly intensive care therapy - if necessary with the application of primary and secondary toxin elimination. Depending on the severity of the intoxication, pharmacology-specific therapy must be initiated.

Expert Workshop COPD: Lungs and Heart - Quite Often Ill Together

When caring for patients with respiratory diseases, always think of the heart! This is especially important for COPD patients, but also for a variety of other disorders of the respiratory system. At the workshop "Luftschlösser", held once more at Wiesbaden, Germany in February 2019 the many and important interactions of the lungs and the heart as well as the therapeutic implications were discussed. Based on pathophysiology, the psycho-social consequences of dyspnea, the leading symptom in patients with lung and heart disease became apparent. A particularly demanding diagnostic and therapeutic situation occurs in patients suffering simultaneously of lung and heart disease. It has been shown how frequently the diagnosis myocardial infarction is missed in COPD patients - and vice versa. Surprisingly, this is also the case in asthmatics with coronary heart disease or heart failure, a fact not readily known in clinical practice. In patients with emphysema and no apparent heart disease, hyperinflation leads to significantly restricted heart function. Reducing hyperinflation by inhaling broncholytics thus improves heart function. Biomarkers are increasingly being used for diagnostic purposes. Their role is being investigated in the large German COPD cohort COSYCONET. Lung patients suffering from more severe heart diseases pose a challenge for therapy in intensive care, especially when ventilated, and weaning from the ventilator is prolonged. Lung vessel diseases are "classic" examples of the intimate interaction of the lungs and the heart. In pulmonary arterial hypertension as well as in chronic thrombo-embolic pulmonary hypertension the lag time between the first symptoms and the definite diagnosis is often unacceptably long. For both diseases of the lung vessels therapeutic options have improved significantly over the last years. Pulmonologists should take care of this increasingly important patient group. Sleep-related breathing disorders and heart function are closely intertwined. Both conditions need special attention after the results of the SERVE-HF trial have been published. But there is no doubt that obstructive sleep apnea represents an independent and important risk factor for cardiovascular disease and needs to be treated according to existing guidelines.This workshop demonstrated impressively the multiple interactions of the respiratory system with cardiac function, resulting diagnostic and therapeutic problems, and means to overcome these problems. Guidelines for respiratory diseases should appropriately address cardiac comorbidity.

PT01.4: Efficiency of Early Enteral Nutrition with Augmented Protein-Caloric Value in the Complex of Intensive Care Therapy of Polytrauma Patients

PT01.4: Efficiency of Early Enteral Nutrition with Augmented Protein-Caloric Value in the Complex of Intensive Care Therapy of Polytrauma Patients

Haematocrit heterogeneity in blood flows past microfluidic models of oxygenating fibre bundles

Blood oxygenators act as an extracorporeal artificial lung during certain types of cardiac surgery and intensive care therapies. Inside these devices, blood is forced to flow across an oxygenating bundle, encountering interstitial gaps comparable to those typical of the microvasculature. Despite the well-known effects of such length scales on haemorheology and red blood cell (RBC) behavior, these are generally overlooked in oxygenator modeling and design; it is persistently assumed that RBCs are homogeneously distributed throughout the oxygenating bundle, independently of their microstructure arrangement or main flow directions.The goal of this study is to provide preliminary experimental evidence of heterogeneous RBC distributions inside oxygenating fibre bundles. To this end, a number of microchannels were manufactured inspired by actual oxygenating devices, considering simplified versions of their microstructure. These comprise staggered arrays of micro pillars, which were perfused with RBC suspensions, with feed haematocrit (Ht) and velocities relevant for clinical use. The microchannels were imaged using a microscope and high-speed camera to accurately capture cell distribution.The imaged blood flows revealed the non-uniform nature of RBC distributions in the arrays, characterized by local Ht gradients particularly around the O2 sources inside the bundle. These heterogeneous distributions should be accounted for during oxygenator design, as RBC concentration plays a key role in O2 transport and, ultimately, overall device performance.

Secondary cholangitis of the critically ill

Sclerosing Cholangitis of the Critically Ill (SC-CIP) is a relatively new entity within the spectrum of secondary cholangitis that develops in the wake of intensive care therapy with mechanical ventilation and catecholamine treatment. It is caused by ischemic or immunologic injury to small bile ducts that becomes self-aggravating and persists beyond the end of the intensive care stay. Early clinical and laboratory findings show acute cholangitis with elevated CRP, gamma GT, AP, and bilirubin. ERCP shows damaged intrahepatic bile ducts with irregular calibers and biliary casts. The following phase is chronic and oligosymptomatic. Still, all laboratory parameters will stay mildly elevated and ERCP and MRCP will show progressive loss of small bile ducts. Long-term prognosis is poor. Even with UDCA therapy, most patients will develop liver cirrhosis within months or years.

Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study

BackgroundMeconium ileus (MI) is a risk factor for poor outcomes in cystic fibrosis (CF) patients. The aim of this study was to identify the risk factors for poor 12-month clinical outcomes in MI-CF newborns. MethodsThis retrospective, multicentre, observational study of MI-CF infants born 2009–2015 recorded their pre- and neonatal histories, intestinal occlusion treatments, post-surgical history, nutrition, CF diagnosis, and compared the patients with 12-month faltering growth or chronic Pseudomonas aeruginosa respiratory infection (cases) with the others (controls). ResultsAbout 25% of the 85 patients enrolled by 13 Italian CF centres (24% premature, 18% of low birth weight) had prenatally diagnosed bowel obstruction, and 39% had complex MI. Seventy-one required surgery (the 33 with complex MI and 38 with simple MI), of whom 58 (82%) required post-surgical intensive care, including 25 (35%) needing ventilatory support. Forty-six (54%) were breastfed; exclusively parenteral nutrition was started in 52 (61%). Cholestasis was diagnosed in 21%.Thirty-one (37%) experienced negative outcomes: the only risk factors were prenatally diagnosed intestinal obstruction and a need for intensive care and oxygen therapy. The cases had significantly higher first blood immunoreactive trypsinogen (b-IRT) levels (P = .008).Logistic regression showed that the probability of having negative outcome is decreased in the absence of cholestasis (Odds Ratio = 0.125) and a need for intensive therapy (OR = 0.141), and increased by not having been breastfed (OR = 2.921). ConclusionsHigh b-IRT levels, prenatally diagnosed intestinal obstruction, a severe post-surgical clinical picture and early liver disease are risk factors for negative outcomes. Breastfeeding may be protective.

Refining the dermatological spectrum in primary immunodeficiency: mucosa‐associated lymphoid tissue lymphoma translocation protein 1 deficiency mimicking Netherton/Omenn syndromes

The proteinase mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1), which forms part of the caspase recruitment domain-containing protein 11-B-cell lymphoma 10-MALT1 signalosome complex, plays a direct role in nuclear factor kappa B activation. Here, we describe the case of a female infant with severe immune dysregulation leading to recurrent systemic infections, failure to thrive and severe crises of ichthyosiform erythroderma with high levels of serum IgE. Hence, initial symptoms indicated Netherton syndrome or Omenn syndrome. Surprisingly, sequence analyses of SPINK5 and RAG1/RAG2, respectively, excluded these diseases. During the hospital stay the patient's health deteriorated, despite intensive care therapy, and she died. In order to delineate the diagnosis, whole-exome sequencing was performed. Two compound heterozygous mutations in MALT1 were found and verified by Sanger sequencing (exon 2 c.245T>C, exon 2 c.310dup), which led to a MALT1 deficiency at the protein level. Based on these results, an immunological analysis was performed, as was immunofluorescence staining of key skin proteins, to confirm a diagnosis of MALT1 deficiency. This case report provides a closer description of the clinical and histological skin phenotype of MALT1 deficiency, and we conclude that MALT1 deficiency must be considered a possible differential diagnosis of Netherton and Omenn syndromes. What's already known about this topic? Mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1) deficiency is a combined immunodeficiency. MALT1 is part of the caspase recruitment domain-containing protein 11-B-cell lymphoma 10-MALT1 signalosome complex, which is essential for nuclear factor kappa B activation. Current publications describe a phenotype of recurrent systemic infections; only in a few cases has an inflammatory involvement of the integument been described. What does this study add? A closer description of the cutaneous phenotype of MALT1 deficiency in a patient with two novel MALT1 mutations. Immune mapping of follicular epidermis shows lympho-epithelial Kazal-type-related inhibitor is reduced in MALT1 deficiency and absent on interfollicular staining. Clinically, MALT1 deficiency mimics Netherton syndrome and Omenn syndrome, and should be considered a differential diagnosis.

Speckle Tracking Echocardiography - a New Tool for the Intensive Care Unit?

The noninvasive evaluation of cardiac morphology and function by echocardiography is an essential part of modern intensive care therapy. However, this procedure can be challenging and beginners often lack the ability to objectively state the correct global and regional myocardial function. Recent developments allow a semi-automatic deformation (strain) analysis by a couple of more objective respective parametric techniques. Strain describes the change in length of a myocardial segment during the cardiac cycle. While this is primarily a regional analysis, an insight into the global left ventricular deformation is possible by averaging all relevant segments. Speckle tracking echocardiography (STE) is actually the only clinically relevant technique and is well scientifically and clinically approved. The advantages of STE are the angle-independency, the ease and fastness of its use, the availability at the bedside and low costs. Through proven good reproducibility it should be a good method for repeated analysis even by different echocardiographers. However, actually the greatest disadvantage is the variation of measures between different vendors of ultrasound machines and software-packages. At the moment, a task force of leading echocardiography experts and industry personal is working on a solution. Normal values have been published for healthy collectives and STE has been in use in the majority of cardiac diseases. Besides from a few research studies, the usage in critically ill patients actually is still limited.

Quantifying neonatal pulmonary mechanics in mechanical ventilation

BackgroundMechanical Ventilation (MV) is an important intensive care therapy. It is often used in neonatal intensive care units (NICUs) to treat respiratory distress syndrome. This paper uses model-based methods in a first in-depth attempt to quantify the underlying lung mechanics in NICU patients. MethodsUp to 24 h of airway flow and pressure data were recorded in 10 mechanically ventilated patients. A single compartment model with added term for the pressure drop across the endotracheal tube is used to identify breath-specific elastance and resistance. ResultThe model was fitted to 422,475 (79%) breaths of 535,428, with the remainder comprising a range of asynchronies. Elastance was median 1.62 [IQR: 0.85–2.25] cmH2O/ml and resistance 5.22 [0.00–33.85] cmH2O.s/ml. Patients treated with surfactant therapy had significantly lower specific elastance (adjusting for weight) than those without (p ≤ 0.01). A decrease in elastance with increasing weight was also noted. ConclusionThe single compartment model was successfully fit with low error. The subgroup cohorts showed expected trends and further validates the identified model values. There was significant breath-to-breath variability in elastance within and between patients.

Intraabdominal continuous negative pressure therapy for secondary peritonitis: an observational trial in a maximum care center

Background: Acute secondary peritonitis is afflicted with a high morbidity and mortality. Intensive care therapy, antibiotics and surgical procedures are mandatory. Continuous negative pressure therapy (cNPT) seems to be beneficial but it is unclear which patients will benefit most from this procedures.Methods: We performed a prospective observational trial including all patients that needed to undergo an exploratory laparotomy for the suspicion of acute secondary peritonitis and were treated with cNPT in one year.Results: Thirty nine patients fitted the criteria. Median hospitalization length was 40 days. The vacuum therapy treatment was applied for a median of 4 days. The subgroup analysis between patients, who received the cNPT-dressing for one time (Group A) and patients, in whom the cNPT was continued after first relaparotomy (Group B) showed no differences concerning patients’ characteristics. The Mannheimer Peritonitis Index (MPI) during the first operation was significantly correlated with the number of dressing changes (Spearman’s rho 0.518, p = .002).Conclusions: Fast acting in acute secondary peritonitis for elimination of the source, abdominal lavage, derivation of the exsudat and interdisciplinary treatment is the treatment of choice. The MPI could be beneficial for the decision process of using cNPT.

The Effects of Genetic 3-Mercaptopyruvate Sulfurtransferase Deficiency in Murine Traumatic-Hemorrhagic Shock.

Hemorrhagic shock is a major cause of death after trauma. An additional blunt chest trauma independently contributes to mortality upon the development of an acute lung injury (ALI) by aggravating pathophysiological consequences of hemorrhagic shock. The maintenance of hydrogen sulfide availability is known to play an important role during hemorrhage and ALI. We therefore tested the impact of a genetic 3-mercaptopyruvate sulfurtransferase mutation (Δ3-MST) in a resuscitated murine model of traumatic-hemorrhagic shock. Anesthetized wild-type (WT) and Δ3-MST mice underwent hemorrhagic shock with/without blunt chest trauma. Hemorrhagic shock was implemented for 1 h followed by retransfusion of shed blood and intensive care therapy for 4 h, including lung-protective mechanical ventilation, fluid resuscitation, and noradrenaline titrated to maintain a mean arterial pressure at least 50 mmHg. Systemic hemodynamics, metabolism, and acid-base status were assessed together with lung mechanics and gas exchange. Postmortem tissue samples were analyzed for immunohistological protein expression and mitochondrial oxygen consumption. 3-MST-deficient mice showed similar results in parameters of hemodynamics, gas exchange, metabolism, acid base status, and survival compared with the respective WT controls. Renal albumin extravasation was increased in Δ3-MST mice during hemorrhagic shock, together with a decrease of LEAK respiration in heart tissue. In contrast, mitochondrial oxygen consumption in the uncoupled state was increased in kidney and liver tissue of Δ3-MST mice subjected to the combined trauma. In summary, in a resuscitated murine model of traumatic-hemorrhagic shock, 3-MST deficiency had no physiologically relevant impact on hemodynamics and metabolism, which ultimately lead to unchanged mortality regardless of an additional blunt chest trauma.

Renal Replacement Therapy in Intensive Care Unit at a Tertiary Care Center in Nepal

Acute kidney injury is a major complication in intensive care unit patients. It is associated with increased in-hospital mortality and length of stay. The provision of renal replacement therapy in intensive care is not widely available in resource poor countries like Nepal. The study aims to look into clinical profile and outcome of patients who received renal replacement therapy in intensive care unit. It was an observational study done from 1st October 2016 till 30th September 2017. Patient’s demographic data, indications, biochemical tests, outcomes, modality of renal replacement therapy were recorded. Statistical package for the social sciences version 17 was used for statistical analysis. There were total of 649 admissions in intensive care, among which 148 had kidney related complications. Of 148 patients, 69 (47%) received renal replacement therapy. Mean age, urea and creatinine on admission were 50.17 ± 18.42 years, 174.54 ± 63.46 mg/dl and 8.05 ± 3.49 mg/ dl respectively. They underwent 4.32 ± 3.09 sessions and 14.94 ± 10.88 hours of renal replacement therapy. Total 42 (61%) had septic shock on admission and underwent sustained low efficiency dialysis as the modality of renal replacement therapy. In-hospital mortality was 19 (28%). Presence of septic shock on admission and mean number of ionotropes required 2.05 ± 1.12 was statistically significant for in-hospital mortality (p=0.01). About half of the patients were on mechanical ventilation which was statistically significant for in-hospital mortality (p&lt;0.001). Sustained low efficiency dialysis can be done in patients on ionotropes and patients can be switched over to intermittent hemodialysis.

Acute-on-chronic liver failure: Objective admission and support criteria in the intensive care unit.

SummaryCirrhosis is a leading cause of morbidity and mortality throughout the world. Significant complications include variceal bleeding, hepatic encephalopathy, hepatorenal syndrome, and infection. When these complications are severe, admission to the intensive care unit (ICU) is often required for organ support and management. Intensive care therapy can also serve as a bridge to liver transplantation. Along with decompensation of cirrhosis, the concept of acute-on-chronic liver failure (ACLF) has emerged. This involves an acute precipitating event, such as the development of infection in a patient with cirrhosis, which leads to acute deterioration of hepatic function and extrahepatic organ failure. Extrahepatic complications often include renal, cardiovascular, and respiratory failures. Patients with significant extrahepatic and hepatic failures need ICU admission for organ support. Again, in patients who are deemed suitable liver transplant candidates, intensive care management may allow bridging to liver transplantation. However, patients with a Chronic Liver Failure Consortium ACLF score greater than 70 at 48 to 72 hours post-ICU admission do not seem to benefit from ongoing intensive support and a palliative approach may be more appropriate.

Hypertriglyceridaemia; a rare cause for severe acute pancreatitis

Acute pancreatitis is a common cause for acute abdominal pain. Its aetiology is multifactorial with gallstone disease and alcohol abuse being common causative factors. Hypertriglyceridaemia (HTG) is a rare yet well known cause for acute pancreatitis. HTG pancreatitis (HTGP) is associated with severe multi-organ involvement increasing the morbidity and mortality. Prompt lipid lowering therapy and multi-organ support in intensive care is essential for such patients to achieve a good outcome. However, randomised clinical trials evaluating the efficacy of specific lipid lowering measures for HTGP is lacking due to its relative rarity.