Long-Term Outcomes of Tuberous Sclerosis Complex-Associated Non-functional Pancreatic Neuroendocrine Tumors: Should We Be More Conservative?

BACKGROUND: Hereditary syndromes such as tuberous sclerosis complex (TSC) account for 10% of pancreatic neuroendocrine tumors (PNETs). Surgical intervention is the current standard of care for sporadic PNETs (spPNETs) that are >2 cm in size. We compared the long-term outcomes of resected TSC-PNETs with patients with spPNETs. METHODS: We conducted a retrospective review of perioperative data and outcomes of TSC-PNETs compared with spPNETs. Inclusion criteria involved selecting patients whose tumors were no larger than 5.1 cm, the maximum size observed in the TSC-PNET group. RESULTS: Of the 347 patients resected for PNETs, 14 were TSC-PNETs and 241 were non-functional spPNETs. The median age for the whole cohort was 56 years (interquartile range [IQR] 21.0) and 47% were female. The median follow-up was 103.8 months (95% confidence interval [CI] 89.2-118.6). Specifically, 14 patients with TSC-PNETs and 194 patients with spPNETs were included. Compared with spPNETs, patients with TSC-PNETs were operated on at a younger age (24.0 vs. 57.5 years; p < 0.001), were more frequently multifocal (28.5% vs. 0.0%; p < 0.001), were more likely to undergo minimally invasive operations (78.6% vs. 24.3%; p < 0.001), and had more R1 resections (28.6% vs. 5.7%; p = 0.006). Local and distant tumor recurrence was only observed in the spPNET group. The 5-year mortality rates for the spPNET and TSC-PNET groups were 6.2% and 0.0%, respectively. No PNET-related deaths were observed among TSC-PNETs. CONCLUSION: None of the TSC-PNET patients recurred after a median follow-up of 78.0 months. The risk-benefit of aggressive pancreatic operations in TSC-PNET patients is still unclear and our findings suggest a conservative approach should be considered.

Brain and/or Spinal Cord Tumors Accompanied with Other Diseases or Syndromes.

Several medical conditions that interest both the brain and the spinal cord have been described throughout the history of medicine. Formerly grouped under the term Phacomatosis because lesions of the eye were frequently encountered or genodermatosis when typical skin lesions were present, these terms have been progressively discarded. Although originally reported centuries ago, they still represent a challenge for their complexity of cure. Nowadays, with the introduction of advanced genetics and the consequent opportunity of whole-genome sequencing, new single cancer susceptibility genes have been identified or better characterized; although there is evidence that the predisposition to a few specific tumor syndromes should be accounted to a group of mutations in different genes while certain syndromes appeared to be manifestations of different mutations in the same gene adding supplementary problems in their characterization and establishing the diagnosis. Noteworthy, many syndromes have been genetically determined and well-characterized, accordingly in the near future, we expect that new targeted therapies will be available for the definitive cure of these syndromes and other gliomas (Pour-Rashidi et al. in World Neurosurgery, 2021). The most common CNS syndromes that will be discussed in this chapter include neurofibromatosis (NF) types 1 and 2, von Hippel-Lindau (VHL) disease, and tuberous sclerosis complex (TSC), as well as syndromes having mostly extra-neural manifestations such as Cowden, Li-Fraumeni, Turcot, and Gorlin syndromes.

QEEG characteristics associated with malnutrition-inflammation complex syndrome.

End-stage renal disease (ESRD) has been linked to cerebral complications due to the comorbidity of malnutrition and inflammation, which is referred to as malnutrition-inflammation complex syndrome (MICS). The severity of this condition is clinically assessed with the malnutrition-inflammation score (MIS), and a cutoff of five is used to optimally distinguish patients with and without MICS. However, this tool is still invasive and inconvenient, because it combines medical records, physical examination, and laboratory results. These steps require clinicians and limit MIS usage on a regular basis. Cerebral diseases in ESRD patients can be evaluated reliably and conveniently by using quantitative electroencephalogram (QEEG), which possibly reflects the severity of MICS likewise. Given the links between kidney and brain abnormalities, we hypothesized that some QEEG patterns might be associated with the severity of MICS and could be used to distinguish ESRD patients with and without MICS. Hence, we recruited 62 ESRD participants and divided them into two subgroups: ESRD with MICS (17 women (59%), age 60.31 ± 7.79 years, MIS < 5) and ESRD without MICS (20 women (61%), age 62.03 ± 9.29 years, MIS ≥ 5). These participants willingly participated in MIS and QEEG assessments. We found that MICS-related factors may alter QEEG characteristics, including the absolute power of the delta, theta, and beta 1 bands, the relative power of the theta and beta 3 subbands, the coherence of the delta and theta bands, and the amplitude asymmetry of the beta 1 band, in certain brain regions. Although most of these QEEG patterns are significantly correlated with MIS, the delta absolute power, beta 1 amplitude asymmetry, and theta coherence are the optimal inputs for the logistic regression model, which can accurately classify ESRD patients with and without MICS (90.0 ± 5.7% area under the receiver operating characteristic curve). We suggest that these QEEG features can be used not only to evaluate the severity of cerebral disorders in ESRD patients but also to noninvasively monitor MICS in clinical practice.

Malnutrition Inflammation Complex Syndrome in Pre-dialysis Chronic Kidney Disease Patients in a Nigerian Tertiary Hospital.

BACKGROUND: Malnutrition Inflammatory Complex Syndrome (MICS) is a non-traditional cardiovascular risk factor that is associated with poor overall outcomes in chronic kidney disease (CKD). However, it has not been well studied among Nigerian CKD population despite its potential for response to therapeutic intervention. OBJECTIVES: To determine the prevalence and severity of MICS and some of its associated factors among pre-dialysis CKD patients. METHODS: This was a cross-sectional study that involved 51 predialysis CKD patients and 51 healthy controls. MICS was assessed using malnutrition inflammation scores (MIS) among the participants. MIS of > 6 was used as the criterion for diagnosis of MICS. RESULTS: The mean ages of the CKD and control groups were 50.96 ± 11.42 years and 48.31±9.83 years, respectively. The prevalence of MICS was significantly higher in the CKD group compared to the control group (54.90% vs 7.8%; P =<0.001). MICS was mild and moderate to severe in 64.3% and 35.7% of the predialysis CKD participants, respectively. Among all the study participants, lower educational level, low estimated glomerular filtration rate, (P<0.001), dyslipidemia (P =<0.001), anaemia (P=<0.001) and hypertension (P=0.033) were significantly associated with MICS. There was significant negative correlation between the MIS and estimated glomerular filtration (r = -0.73, P <0.001), and haematocrit (r = -0.335, p =0.016). CONCLUSION: MICS was common in pre-dialysis CKD population. It was significantly associated with hypertension, dyslipidemia, educational level, anaemia and estimated glomerular filtration rate in pre-dialysis CKD patients. Early diagnosis and treatment may reduce poor cardiovascular outcomes in them.

CONTEXTE: Le syndrome du complexe inflammatoire de la malnutrition (MICS) est un facteur de risque cardiovasculaire non traditionnel qui est associé à de mauvais résultats globaux dans la maladie rénale chronique (MRC). Cependant, il n'a pas été bien étudié parmi la population nigériane atteinte de MRC, malgré son potentiel d'intervention thérapeutique. OBJECTIFS: Déterminer la prévalence et la gravité de la MICS et certains de ses facteurs associés chez les patients atteints d'IRC en pré-dialyse. MÉTHODES: Il s'agissait d'une étude transversale portant sur 51 patients atteints d'IRC en prédialyse et 51 témoins sains. Le MICS a été évalué en utilisant les scores d'inflammation de malnutrition (MIS) parmi les participants. Un indice de malnutrition supérieur ou égal à 6 a été utilisé comme critère de diagnostic de la MICS. RÉSULTATS: L'âge moyen des groupes de personnes atteintes de NC et de témoins était de 50,96 ± 11,42 ans et de 48,31±9,83 ans, respectivement. La prévalence des MICS était significativement plus élevée dans le groupe IRC que dans le groupe témoin (54,90 % contre 7,8 % ; P =<0,001). La MICS était légère et modérée à sévère chez 64,3% et 35,7 % des participants à l'IRC avant dialyse, respectivement. Parmi l'ensemble des participants à l'étude, un niveau d'éducation plus faible, un faible débit de filtration glomérulaire estimé. (P<0,001), la dyslipidémie (P =<0,001), l'anémie (P=<0,001) et l'hypertension (P=0,033) étaient significativement associés au MICS. Il y avait une corrélation négative significative entre le MICS et la filtration glomérulaire estimée (r = -0,73, P <0,001), et l'hématocrite (r=-0,335, p =0,016). CONCLUSION: Le MICS était fréquent dans la population IRC pré-dialyse. Il était significativement associé à l'hypertension, la dyslipidémie, le niveau d'éducation, l'anémie et le taux de filtration glomérulaire estimé chez les patients IRC pré-dialysés. Un diagnostic et un traitement précoces pourraient réduire les mauvais résultats cardiovasculaires chez ces patients. Mots clés: Le syndrome du complexe inflammatoire de la malnutrition, Pré-dialyse, maladies rénal chronique, Titre courant proposé : Le MICS chez les patients IRC pré-dialysés.

A rare case of limb body wall complex.

Limb body wall complex (LBWC) is a complex and rare poly-malformative syndrome. We report a case of this syndrome diagnosed antenatally in a 37-year-old primiparous woman, by a first obstetrical ultrasound performed at 22 weeks of amenorrhea and one day. After termination of pregnancy, macroscopic examination revealed a male newborn with all the diagnostic criteria of LBWC.

Malnutrition-Inflammation Complex Syndrome and Bone Fractures and Cardiovascular Disease Events in Patients Undergoing Hemodialysis: The Q-Cohort Study.

Rationale & Objective: Malnutrition-inflammation complex syndrome (MICS) is common in patients receiving hemodialysis and increases the risks of morbidity and mortality. However, few studies have examined the overall impact of MICS on disorders of the bone-cardiovascular axis. Study Design: Prospective, multicenter, observational cohort study. Setting & Participants: A total of 3,030 patients receiving maintenance hemodialysis registered in the Q-Cohort Study. Predictors: A newly developed score for MICS composed of elements chosen from 8 baseline parameters related to nutrition and inflammation by bootstrap resampling, multivariable-adjusted Cox proportional hazard risk analysis for all-cause mortality, and the risk prediction rule. ß-coefficients of each element analyzed in the multivariable-adjusted model were used for the creation of the MICS score. Outcomes: Bone fractures, cardiovascular disease events, and the composite outcome of bone fractures and cardiovascular disease events. Analytical Approach: Cox proportional hazard regression and Fine-Gray proportional subdistribution hazards regression. Results: During a median follow-up of 4 years, 140 patients developed bone fractures and 539 developed cardiovascular disease events. Age; serum levels of creatinine, albumin, and C-reactive protein; and body mass index were selected for the creation of the MICS score. The median (IQR) MICS score was 196 (181-212). The multivariable-adjusted Cox proportional hazard risk model and the competing risk model showed that a higher MICS score was incrementally associated with elevated risks of bone fractures, cardiovascular disease events, and the composite outcome; hazard risks (95% CIs) of fractures, cardiovascular disease events, and the composite outcome for each 10-point increase in the MICS score were 1.18 (1.01-1.38), 1.16 (1.07-1.26), and 1.15 (1.07-1.24), respectively. Limitations: One-time measurement of the parameters used for the creation of the MICS score. Conclusions: Malnutrition and inflammation represented by the MICS score were associated with increased risks of bone-cardiovascular axis disorders in patients receiving maintenance hemodialysis.

Carney complex syndrome manifesting as cardioembolic stroke: a case report and review of the literature.

BACKGROUND: "Carney Complex (CNC) is a familial lentiginosis syndrome, caused by PRKAR1A mutations that lead to cyclic AMP-dependent protein kinase (PKA) signaling pathway abnormalities, predisposing to a variety of skin tumors, myxomas and endocrine tumors. METHODS/RESULTS: We describe a Greek family diagnosed with CNC after recurrent embolic strokes, secondary to left-sided atrial myxomas. There are limited cases in the literature describing this type of presentation for CNC; typically, most cases present with an endocrine syndrome. Our case serves as a reminder of this rare, underdiagnosed syndrome and its wide phenotypic spectrum. It is followed by a review of the current literature on cases with cerebrovascular disease as a manifestation of CNC. CONCLUSION: The co-occurrence of emboligenic cardiac myxomas and skin lesions should be an indication for screening for CNC.

A novel homozygous variant of COL2A1 in a Chinese male with type II collagenopathy: a case report.

BACKGROUND: Type II collagenopathies are a spectrum of diseases and skeletal dysplasia is one of the prominent features of collagenopathies. Molecular defects of the COL2A1 gene cause type II collagenopathies that is mainly an autosomal dominant disease, whereas some rare cases with autosomal recessive inheritance of mode have also been identified. CASE PRESENTATION: The patient was a 5-year-old male with a short neck, flat face, epiphyseal dysplasia, irregular vertebral endplates, and osteochondritis. Sequencing result indicated NM_001844.4: c.3662C > T; p. (Ser1221Phe) a novel missense variant, leading to a serine-to-phenylalanine substitution. Sanger sequencing confirmed the variant compared to his parents and brother. CONCLUSIONS: We identified a novel homozygous variant of the COL2A1 gene as the cause of type II collagenopathies in a Chinese male, enriching the spectrum of genotypes. This is the first case of type II collagenopathies inherited in an autosomal recessive manner in China and East Asia, and it is the first case that resulted from serine substitution in the world.

A proposal for a new clinical entity: transient epileptic amnesia complex syndrome (TEACS).

Transient epileptic amnesia (TEA) is a special type of temporal lobe epilepsy, the main symptom of which is recurrent amnesia attacks. In the late 1990s, Zeman et al. developed the following diagnostic criteria for TEA: (i) recurrent, witnessed episodes of amnesia; (ii) other cognitive functions remain intact during attacks; and (iii) evidence of epilepsy. It was subsequently reported that patients with TEA almost always demonstrate two other types of memory symptoms: accelerated long-term forgetting (ALF) and autobiographical amnesia (AbA). As a result, it has been recognised that TEA causes at least three characteristic types of amnesia, that is, amnesia attacks, ALF, and AbA. In this report, we present two clinical cases, in which the patients showed symptoms of ALF and/or AbA without suffering any type of epileptic seizure, including amnesia attacks, for a long time. We discuss a syndrome associated with TEA, particularly the relationship between TEA and ALF/AbA, based on our two cases and a review of the literature. In addition, we propose a new clinical entity, which we named 'transient epileptic amnesia complex syndrome (TEACS)' and will help to ensure that physicians recognise the existence of such cases and do not overlook this condition. Furthermore, the following diagnostic criteria for TEACS are proposed. (i) The patient is middle-aged to elderly at onset and has no history of epilepsy. (ii) ALF and/or AbA have been definitively diagnosed. (iii) The ALF and/or AbA precede TEA attacks and/or other epileptic seizures. (iv) Except for the ALF/AbA, the patient's cognitive functions are confirmed to be intact via clinical examinations. (v) There is evidence for a diagnosis of epilepsy. Such evidence can include: (i) wake or sleep electroencephalography; or (ii) a clear response to anti-epileptic drugs. Furthermore, we describe our hypotheses regarding the pathogenesis of ALF/AbA and discuss the relationships between TEACS and other epileptic amnesia-related syndromes.

EEG Delta/Theta Ratio and Microstate Analysis Originating Novel Biomarkers for Malnutrition-Inflammation Complex Syndrome in ESRD Patients.

The Malnutrition-Inflammation Score (MIS) was initially proposed to evaluate malnutrition-inflammation complex syndrome (MICS) in end-stage renal disease (ESRD) patients. Although MICS should be routinely evaluated to reduce the hospitalization and mortality rate of ESRD patients, the inconvenience of the MIS might limit its use. Cerebral complications in ESRD, possibly induced by MICS, were previously assessed by using spectral electroencephalography (EEG) via the delta/theta ratio and microstate analysis. Correspondingly, EEG could be used to directly assess MICS in ESRD patients, but the relationships among MICS and these EEG features remain inconclusive. Thus, we aimed to investigate the delta/theta ratio and microstates in ESRD patients with high and low risks of MICS. We also attempted to identify the correlation among the MIS, delta/theta ratio, and microstate parameters, which might clarify their relationships. To achieve these objectives, a total of forty-six ESRD subjects were willingly recruited. We collected their blood samples, MIS, and EEGs after receiving written informed consent. Sixteen women and seven men were allocated to low risk group (MIS ≤ 5, age 57.57 ± 14.88 years). Additionally, high risk group contains 15 women and 8 men (MIS > 5, age 59.13 ± 11.77 years). Here, we discovered that delta/theta ratio (p < 0.041) and most microstate parameters (p < 0.001) were significantly different between subject groups. We also found that the delta/theta ratio was not correlated with MIS but was strongly with the average microstate duration (ρ = 0.708, p < 0.001); hence, we suggested that the average microstate duration might serve as an alternative encephalopathy biomarker. Coincidentally, we noticed positive correlations for most parameters of microstates A and B (0.54 ≤ ρ ≤ 0.68, p < 0.001) and stronger negative correlations for all microstate C parameters (-0.75 ≤ ρ ≤ -0.61, p < 0.001). These findings unveiled a novel EEG biomarker, the MIC index, that could efficiently distinguish ESRD patients at high and low risk of MICS when utilized as a feature in a binary logistic regression model (accuracy of train-test split validation = 1.00). We expected that the average microstate duration and MIC index might potentially contribute to monitor ESRD patients in the future.

Malnutrition-Inflammation Complex Syndrome: A Cause of Low Parathyroid Hormone in Patients With Chronic Kidney Disease.

Secondary hyperparathyroidism is commonly seen in patients with chronic kidney disease (CKD) due to hypocalcemia, hyperphosphatemia and low vitamin D levels and is associated with high-turnover bone disease. In contrast, some patients with advanced CKD, including those requiring dialysis (end-stage renal disease [ESRD]), develop adynamic bone disease with features of low-turnover bone disease. Low serum parathyroid hormone (PTH) has been used as a biochemical marker of adynamic bone disease. Low PTH levels may not necessarily be due to adynamic bone disease but could be a manifestation of the malnutrition inflammation complex syndrome (MICS). The optimal management of hypoparathyroidism associated with MICS is not well known. Currently, there is insufficient evidence to suggest if there is any role in improving nutritional and inflammatory status among patients with CKD and MICS. Furthermore, it also remains unclear whether these changes will help address low PTH levels seen in these patients. We report three patients with advanced CKD who had very low PTH levels possibly attributed to MICS. In addition, we briefly discuss other characteristics and pathophysiology of MICS.

Prevalence of malnutrition inflammation complex syndrome among patients on maintenance haemodialysis at Muhimbili National Hospital in Tanzania: a cross-sectional study.

BACKGROUND: Malnutrition, inflammation, and the combination thereof are predictors of poor outcomes in haemodialysis patients. Malnutrition Inflammation Complex Syndrome (MICS) is an accelerator of atherosclerosis and portends high mortality. Early recognition and treatment of MICS may help to improve the clinical outlook of such patients. This study investigated the prevalence of MICS and its associated factors among patients on maintenance haemodialysis at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania. METHODS: This was a prospective cross-sectional observational study done among 160 adult patients on maintenance haemodialysis at MNH in 2019. All participants provided written informed consent. Questionnaires were used to collect data and patients' blood was tested for complete blood count (CBC), C-reactive protein (CRP), ferritin, transferrin, creatinine, urea, total cholesterol, and albumin. The Malnutrition Inflammation Score was used to assess MICS and its severity. Data analysis was done using the SPSS 20 software. RESULTS: Of the 160 patients included in the study, 111 (69.4%) were male. The mean age (±SD) of patients and mean duration (±SD) on haemodialysis were 52.2(13.3) years and 22(18) months respectively. MICS was prevalent in 46.3% (mild in 24.4% and moderate to severe in 21.9%). Long-term haemodialysis (> 4 years) was an independent predictor of MICS [Adjusted Odds Ratio, AOR 5.04 (95% CI: 1.33-19.2), p < 0.05]. Hypercholesterolaemia was a negative predictor of MICS [AOR 0.11 (95% CI: 0.01-0.97), p < 0.05]. Patients with MICS had significantly lower mean body mass index, serum albumin, total cholesterol, transferrin, haemoglobin, and creatinine levels. The presence of MICS was higher in underweight patients and those who had inflammation. Haemodialysis adequacy did not correlate with MICS. CONCLUSION: Malnutrition Inflammation Complex Syndrome is relatively common among patients on haemodialysis in Dar es Salaam, Tanzania. Our study has shown a longer duration on haemodialysis to be associated with the occurrence of MICS; on the contrary, having hypercholesterolaemia seems to be protective against MICS consistent with the concept of reverse epidemiology. Patients on haemodialysis should be assessed regularly for malnutrition and inflammation and should receive appropriate and timely treatment to reduce the burden of associated morbidity, and mortality to these patients.

Perspective: Current Pitfalls in the Search for Future Treatments and Prevention of Parkinson's Disease.

We are gradually becoming aware that there is more to Parkinson's disease (PD) than meets the eye. Accumulating evidence has unveiled a disease complexity that has not (yet) been incorporated into ongoing efforts aimed at slowing, halting or reversing the course of PD, likely underlying their lack of success. There is a substantial latency between the actual onset of PD pathology and our ability to confirm diagnosis, during which accumulating structural and functional damage might be too advanced for effective modification or protection. Identification at the earliest stages of the disease course in the absence of Parkinsonism is crucial if we are to intervene when it matters most. Prognostic and therapeutic inferences can only be successful if we are able to accurately predict who is at risk for developing PD and if we can differentiate amongst the considerable clinicopathologic diversity. Biomarkers can greatly improve our identification and differentiation abilities if we are able to disentangle cause and effect.

The Incidence and Pathophysiology of the Obesity Paradox: Should Peritoneal Dialysis and Kidney Transplant Be Offered to Patients with Obesity and End-Stage Renal Disease?

PURPOSE OF REVIEW: To educate nephrologists and primary-care physicians about the incidence, pathophysiology, and survival benefits of the obesity paradox in end-stage renal disease (ESRD). This review also discusses the future of kidney transplant and peritoneal dialysis in obese dialysis patients. RECENT FINDINGS: Obesity paradox in ESRD was first reported three decades ago, and since then, there have been several epidemiological studies that confirmed the phenomenon. Regardless of the anthropometric indices used to define obesity in ESRD patients, these markers serve to predict the dialysis patient's survival. The pathophysiology of obesity paradox tends to be multifactorial. Recent cohort studies demonstrated a survival benefit in all race and ethnic groups, but Hispanics and blacks experienced increased survival rates when compared to non-Hispanic whites. Obese dialysis patients should be offered peritoneal dialysis, especially if they are new to dialysis and have an adequate renal residual function. Several studies have shown that the benefit of receiving kidney transplant in obese patients exceeds the risks. The robotic-assisted kidney transplant (RAKT) procedure is the latest innovation that could offer hope for obese dialysis patients who have been denied or are waiting for kidney transplant. The obesity paradox phenomenon in ESRD is a unique illustration of survival benefit in a population that has a high overall annual mortality. Peritoneal dialysis should be encouraged for obese patients who have preserved residual renal function. Kidney transplant centers should encourage RAKT utilization in obese dialysis patients instead of denying them a kidney transplant.

Novel variant in the FGD1 gene causing Aarskog-Scott syndrome.

Aarskog-Scott syndrome (ASS) is a rare, X-linked recessive inherited disorder. Affected individuals may develop short stature and exhibit distinctive skeletal and genital development. Mutations in the FYVE, rhogef and pleckstrin homology domain-containing protein 1 (FGD1) gene, located within the Xp11.21 region, are responsible for the occurrence of ASS. Since it is rare and complex, it can take a long time to obtain a definitive clinical diagnosis unless clinicians are familiar with the disease. In the present study, whole-exome sequencing (WES) was performed to screen for causal variants in a Chinese pediatric patient who exhibited a number of clinical symptoms of ASS, including short stature, facial abnormalities, stubby metacarpals and swollen testis. DNA sequencing revealed a novel c.1270 A>G mutation in exon 6 of the FGD1 gene, which led to an amino acid conversion of asparagine to aspartic acid on codon 424 and in silico analysis indicated that this novel missense mutation was pathogenic. The present study identified a novel variant of the FGD1 gene and to the best of our knowledge, is the first report of ASS in a Chinese individual. The results indicated that WES is an effective tool for the diagnosis of rare and complex syndromes such as ASS.

Hand and Wrist Injuries in Golfers and Their Treatment.

A thorough understanding of the swing phases and mechanisms of injury in golf allows accurate diagnosis, treatment, and future prevention of injuries. Recommended initial treatment starts with cessation of practice to rest the wrist, a splint or orthotic brace, and nonsteroidal antiinflammatory drug medication with corticosteroid injection and swing modification. Pisiform excision is the best treatment of the most severe chronic cases of pisiform ligament complex syndrome. Delayed diagnosis of hook of hamate fracture may lead to complications, including flexor tendon rupture. Prompt surgical resection is recommended to hasten return to sport and to prevent further complications.

MICS, an easily ignored contributor to arterial calcification in CKD patients.

In chronic kidney disease (CKD), simultaneous mineral and skeleton changes are prevalent, known as CKD-mineral bone disorder (CKD-MBD). Arterial calcification (AC) is a clinically important complication of CKD-MBD. It can increase arterial stiffness, which leads to severe cardiovascular events. However, current treatments have little effect on regression of AC, as its mechanisms are still unclear. There are multiple risk factors of AC, among which Malnutrition-Inflammation Complex Syndrome (MICS) is a new and crucial one. MICS, a combined syndrome of malnutrition and inflammation, generally begins at the early stage of CKD and becomes obvious in end-stage renal disease (ESRD). It was linked to reverse epidemiology and associated with increased cardiovascular mortality in ESRD patients. Recent data suggest that MICS can trigger CKD-MBD and accelerate the course of AC. In this present review, we summarize the recent understanding about the aggravating effects of MICS on AC and discuss the possible underlying mechanisms. A series of findings indicate that targeting MICS will provide a potential strategy for treating AC in CKD.

Intradialytic Oral Protein Supplementation and Nutritional and Inflammation Outcomes in Hemodialysis: A Randomized Controlled Trial.

BACKGROUND: Malnutrition is a common finding in hemodialysis patients and can increase oxidative stress and inflammation levels. STUDY DESIGN: A randomized, controlled, nonblinded, parallel trial. SETTING & PARTICIPANTS: 92 hemodialysis patients from a single center with malnutrition according to subjective global assessment (SGA) score (SGA score > 7). INTERVENTION: 3 treatment groups (23 patients each) received 220mL of fermented vitamin E-fortified whey beverage (15g of whey protein concentrate + 600IU of vitamin E) or 220mL of fermented whey beverage (15g of whey protein concentrate) or vitamin E (600IU) 3 times a week for 8 weeks. The control group (23 patients) received no intervention. OUTCOME & MEASUREMENTS: Primary outcomes were change in SGA score and malnutrition-inflammation score (MIS) from baseline to the end of the trial. RESULTS: At the end of the study, 83 patients were analyzed (2, 3, 1, and 3 patients left the study in the vitamin E-fortified whey beverage, whey beverage, vitamin E, and control groups, respectively). Changes in SGA scores were -3.48 (95% CI, -4.90 to -2.00), -3.22 (95% CI, -4.13 to -2.30), -1.70 (95% CI, -3.20 to -0.24), and 1.56 (95% CI, 0.60 to 2.50) for the vitamin E-fortified whey beverage, whey beverage, vitamin E, and control groups, respectively (overall P<0.001; P≤0.001 for each treatment group vs control). Changes in MISs were -3.17 (95% CI, -4.40 to -1.90), -1.83 (95% CI, -2.50 to -1.10), -2.30 (95% CI, -3.50 to -1.10), and 1.48 (95% CI, 0.65 to 2.30) for the vitamin E-fortified whey beverage, whey beverage, vitamin E, and control groups, respectively (overall P<0.001; P<0.001 for each treatment group vs control). Few adverse effects were reported in any group. LIMITATIONS: Lack of blinding, small sample size, and short duration. CONCLUSIONS: Whey protein in the form of a new fermented whey beverage and vitamin E supplementation may improve SGA score and MIS in the short term.

Transoral resection of stylopharyngeus calcification: a unique manifestation of a stylohyoid complex syndrome.

BACKGROUND: Eagle syndrome is often considered in the work-up of odynophagia and neck pain. Classically, this is manifested by ossification or calcification of the stylohyoid ligament or styloid process. There are no reported cases of stylopharyngeal calcification leading to these symptoms. CASE: We describe a patient with a suspected submucosal pharyngeal foreign body who was found to have a calcified stylopharyngeus muscle and tendon during surgery. The patient experienced full resolution of symptoms after transoral robotic resection. This diagnosis was initially missed because the radiology was inconsistent with Eagle syndrome. CONCLUSION: This is the first report of isolated stylopharyngeal calcification, and this unique manifestation of a stylohyoid complex syndrome should be considered in patients with symptoms of Eagle syndrome without styloid elongation.

The malnutrition inflammation complex syndrome-the micsing factor in the perio-chronic kidney disease interlink.

The patients who undergo Maintenance Haemodialysis (MHD) have a high prevalence of Protein-Energy Malnutrition (PEM) and inflammation. Because these two conditions often occur concomitantly in the MHD patients, they have been referred to together as the Malnutrition-Inflammation Complex Syndrome (MICS) or Malnutrition-inflammation atherosclerosis, to underscore the atherosclerotic complications of this entity. MICS is also reported to correlate with a poor outcome, including a decreased quality of life, refractory anaemia and significantly greater rates of hospitalization and mortality in the MHD patients .Indeed, MICS may be the major cause of the paradoxical exposure-outcome association, which is also known as reverse epidemiology of the cardiovascular disease risk factors in the maintenance dialysis patients. Periodontitis is a chronic inflammatory disease of the supporting tissues from the dentition, which results from the infection of and the interaction of selected bacterial species with the components of the host response in disease-susceptible individuals as the haemodialysis (HD) patients. Only in recent years, did an emerging evidence link the dental infection, especially periodontitis, to an increased risk of atherosclerosis and thrombosis. In the HD patients, studies have been showing a positive link between periodontal disease and systemic inflammation on correlation between the levels of CRP and immunoglobulin G of Porphyromonas gingivalis. Recent researches have confirmed that the periodontal health is poor in haemodialysis patients and that it correlates with the markers of malnutrition and inflammation.