Development and validation of the Unmet Needs Questionnaire for young stroke survivors.

OBJECTIVE: Owing to the lack of a suitable tool for detecting the unmet needs of young stroke survivors, this study aims to develop a validated questionnaire for evaluating these unmet needs. DESIGN: A cross-sectional, observational research design. SETTING: Chang Gung Memorial Hospital Linkou and Taoyuan branches in Taiwan. PARTICIPANTS: A total of 211 participants (average age 53 years; within 6 months post-stroke) completed the questionnaire. MAIN MEASURES: A qualitative approach was used to create an item pool. Experts verified item suitability, and content validity was evaluated using the item content validity index. Item analysis was applied to determine item quality, and factor analysis was used to explore construct validity. In addition, parallel analysis was employed to ascertain the optimal number of factors. RESULTS: The scale development procedure resulted in a 27-item questionnaire that assesses the unmet needs of young stroke survivors after a stroke. The item content validity index was 1.0. The Unmet Needs Questionnaire has five factors: restoring prestroke abilities and life, rehabilitation-related resources, social support and self-adjustment, economic and post-stroke life adjustment, and stroke-related information. These five factors accounted for 54% of the variance. Cronbach's alpha for the total scale was 0.91, while the alpha for the subscales ranged from 0.74 to 0.88. CONCLUSIONS: The Unmet Needs Questionnaire showed acceptable reliability and validity. It can help clinical professionals and government agencies identify stroke survivors' unmet needs and develop tailored care plans. Future research should explore the trajectory of post-stroke unmet needs using this tool.

Evaluating the combination of in-person and electronic social networking services for family caregivers of stroke survivors: A quasi-experimental analysis.

INTRODUCTION: The effectiveness of health interventions delivered via a combination of in-person and electronic social networking services for caregivers of stroke survivors remains uncertain. This study evaluates the feasibility of implementing educational and peer support programs for these caregivers through such platforms. DESIGN: Quasi-experimental design. METHODS: This study included 105 caregiver-survivor dyads, with 54 dyads allocated to the intervention group and the remaining 51 to the control group. The LINE intervention comprised a combination of in-person and electronic social networking services including stroke and rehabilitation education, problem-solving skills training, long-term care information support, and 24-h peer and professional support for caregivers. The outcomes were assessed at baseline, after 1 month, and after 3 months, and encompassed caregivers' care burden, depressive symptoms, perceived social support, and quality of life, as well as the rehabilitation adherence and depressive symptoms of stroke survivors. Generalized estimating equations were used to examine group differences. The data were collected between August 2021 and October 2022. RESULTS: The average age of the caregivers was 48.3 years. Caregivers in the intervention group reported reduced care burdens and enhanced perceptions of social support and quality of life as compared to those in the control group. Additionally, stroke survivors in the intervention group were less likely to exhibit high-risk depressive symptoms. CONCLUSION: Delivering a stroke caregiver support intervention via in-person and electronic social networking services, such as LINE, effectively reduced the care burden for caregivers of stroke survivors. Additionally, it enhanced caregivers' perceived social support and quality of life. CLINICAL RELEVANCE: This study demonstrated that caregiver education and peer support programs administered through a combination of in-person and electronic social networking services can serve as an effective support system for the psychosocial health of stroke caregivers. These findings support the integration of such interventions into standard clinical practice by healthcare providers or governmental bodies.

Can rehabilitation adherence among stroke patients be measured using a single item?

AIMS AND OBJECTIVES: To assess the concurrent validity between logbooks and a single-item rehabilitation adherence measurement for patients with stroke. Agreement between caregivers and patients and between caregivers and physical therapists regarding a single-item measurement was investigated, and its predictive validity was explored. BACKGROUND: Adherence to therapy is a primary determinant of treatment success. There are no standard instruments for measuring rehabilitation adherence available for stroke patients. DESIGN: Prospective longitudinal study. METHODS: Seventy-five patients with stroke were recruited, measured four times and followed for 6 months. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist was used to ensure comprehensive reporting. Adherence was documented in logbooks, and single-item measurements were compared. Predictive validity was explored by assessing associations between adherence levels, self-care ability and health-related quality of life. The Spearman's correlation coefficients, weighted kappa, and generalised estimating equations statistics were used to explore the concurrent validity, measurement agreement, and predictive validity, respectively. RESULTS: Logbook records had a fair correlation (rs  = .23, p = .04) with the single-item rehabilitation adherence measurements. There was moderate agreement (kappa = 0.42, p < .001) between caregiver and patient assessments and fair agreement (kappa = 0.29, p = .017) between caregiver and physical therapist assessments of patients' rehabilitation adherence levels. Perfect rehabilitation adherence, based on the logbook and single-item measurements, predicted better scores for self-care ability and quality of life than imperfect rehabilitation adherence during 6 months after inclusion. CONCLUSIONS: There was fair concurrent validity between logbooks and single-item rehabilitation adherence measurements and moderate and fair adherence measure agreement between caregivers and patients and caregivers and physical therapists, respectively. Logbooks and single-item rehabilitation adherence measurements had adequate predictive validity. RELEVANCE TO CLINICAL PRACTICE: Single-item rehabilitation adherence measurement is a workable and straightforward method to assess stroke patients' rehabilitation adherence in busy clinical care settings. Caregivers can represent stroke patients regarding their reported rehabilitation adherence. PATIENT OR PUBLIC CONTRIBUTION: Patients were diagnosed with stroke in the study hospital. Rehabilitation physicians transferred patients to a research nurse who then screened them for the inclusion criteria and invited them and their family caregivers to participate in this study if they met the requirements. We also recruited seven physical therapists responsible for the physical therapy of the study participants. After participants signed informed consent, the research nurse encouraged participants to respond to research questions face to face, including rehabilitation adherence data, daily physical function, and quality of life. Each participant was measured four times at baseline and at 1, 3, and 6 months after inclusion in this study. Physical therapists had to score their patients' rehabilitation adherence levels before discharge. TRIAL REGISTRATION DETAILS: Not applicable.

Design, Synthesis and Evaluation of Novel Derivatives of Curcuminoids with Cytotoxicity.

Curcumin and curcuminoids have been discussed frequently due to their promising functional groups (such as scaffolds of α,ß-unsaturated ß-diketone, α,ß-unsaturated ketone and ß'-hydroxy-α,ß-unsaturated ketone connected with aromatic rings on both sides) that play an important role in various bioactivities, including antioxidant, anti-inflammatory, anti-proliferation and anticancer activity. A series of novel curcuminoid derivatives (a total of 55 new compounds) and three reference compounds were synthesized with good yields using three-step organic synthesis. The anti-proliferative activities of curcumin derivatives were examined for six human cancer cell lines: HeLaS3, KBvin, MCF-7, HepG2, NCI-H460 and NCI-H460/MX20. Compared to the IC50 values of all the synthesized derivatives, most α,ß-unsaturated ketones displayed potent anti-proliferative effects against all six human cancer cell lines, whereas ß'-hydroxy-α,ß-unsaturated ketones and α,ß-unsaturated ß-diketones presented moderate anti-proliferative effects. Two potent curcuminoid derivatives were found among all the novel derivatives and reference compounds: (E)-5-hydroxy-7-phenyl-1-(3,4,5-trimethoxyphenyl)hept-1-en-3-one (compound 3) and (1E,4E)-1,7-bis(3,4,5-trimethoxyphenyl)hepta-1,4-dien-3-one (compound MD12a). These were selected for further analysis after the evaluation of their anti-proliferative effects against all human cancer cell lines. The results of apoptosis assays revealed that the number of dead cells was increased in early apoptosis and late apoptosis, while cell proliferation was also decreased after applying various concentrations of (E)-5-hydroxy-7-phenyl-1-(3,4,5-trimethoxyphenyl)hept-1-en-3-one (compound 3) and (1E,4E)-1,7-bis(3,4,5-trimethoxyphenyl)hepta-1,4-dien-3-one (compound MD12a) to MCF-7 and HpeG2 cancer cells. Analysis of the gene expression arrays showed that three genes (GADD45B, SESN2 and BBC3) were correlated with the p53 pathway. From the quantitative PCR analysis, it was seen that (1E,4E)-1,7-bis(3,4,5-trimethoxyphenyl)hepta-1,4-dien-3-one (compound MD12a) effectively induced the up-regulated expression of GADD45B, leading to the suppression of MCF-7 cancer cell formation and cell death. Molecular docking analysis was used to predict and sketch the interactions of the GADD45B-α,ß-unsaturated ketone complex for help in drug design.

Stereoselective Synthesis of Spiro Bis-C,C-α-arylglycosides by Tandem Heck Type C-Glycosylation and Friedel-Crafts Cyclization.

Spiro bis-C,C-α-arylglycosides were synthesized in three steps in 78-85% overall yields starting from exo-glycals. The initial Heck type C-aryl addition of exo-glycals with arylboronic acids afforded α-aryl-ß-substituted C-glycosides with exclusive α-stereoselectivity. Among the products, ß-ethanal α-aryl C-glycosides further reacted with alkylthiol in the presence of InCl3, followed by in situ Friedel-Crafts cyclization to yield the desirable final products. We proposed a mechanism to explain how the α-aryl group serves as a main determinant of the cyclization.

Low-level laser irradiation stimulates tenocyte proliferation in association with increased NO synthesis and upregulation of PCNA and cyclins.

Low-level laser therapy is commonly used to treat tendinopathy or tendon injury. Tendon healing requires tenocyte migration to the repair site, followed by proliferation and synthesis of the extracellular matrix. There are few evidence to elucidate that low-level laser promote tenocyte proliferation. This study was designed to determine the effect of laser on tenocyte proliferation. Furthermore, the association of this effect with secretion of nitric oxide (NO) and the expressions of proliferating cell nuclear antigen (PCNA) and cyclins D1, E, A, and B1 was investigated. Tenocytes intrinsic to rat Achilles tendon were treated with low-level laser (660 nm). Tenocyte proliferation was evaluated by MTT assay and immunocytochemistry with Ki-67 stain. NO in the conditioned medium was measured by ELISA. Western blot analysis was used to evaluate the protein expressions of PCNA and cyclins D1, E, A, and B1. The results revealed that tenocytes proliferation was enhanced dose dependently by laser. NO secretion was increased after laser treatment. PCNA and cyclins E, A, and B1 were upregulated by laser. In conclusion, low-level laser irradiation stimulates tenocyte proliferation in a process that is mediated by upregulation of NO, PCNA, and cyclins E, A, and B1.

Impact of rehabilitation adherence and depressive symptoms on post-stroke self-care ability and quality of life: a longitudinal study.

BACKGROUND: Good rehabilitation adherence leads to effective post-stroke recovery. However, some recovering patients experience post-stroke depressive symptoms, which can affect post-stroke health outcomes. Previous studies have not examined the effect of a combination of rehabilitation adherence and depressive symptoms on recovery after a stroke. OBJECTIVES: This study explored the combined predictive influence of rehabilitation adherence and post-stroke depressive symptoms on self-care abilities and quality of life in patients with stroke. METHODS: This prospective longitudinal study analyzed data from 75 stroke patients. We examined rehabilitation adherence (self-reported, five-point scale), post-stroke depressive symptoms (Taiwanese Depression Scale), self-care ability (Chinese versions of the Barthel Index and Lawton - Brody Instrumental Activities of Daily Living Scale), and post-stroke quality of life (World Health Organization Quality of Life-BREF). Patients were followed up for six months after inclusion. The influence of rehabilitation adherence and post-stroke depressive symptoms on post-stroke self-care abilities and quality of life was examined using generalized estimating equations. RESULTS: The sample's mean age was 60.85 (±12.9) years. Patients with perfect rehabilitation adherence had better self-care abilities and quality of life than those with imperfect rehabilitation adherence. Patients without post-stroke depressive symptoms had a better quality of life than their counterparts. Patients with perfect rehabilitation adherence and no post-stroke depressive symptoms had better self-care abilities and quality of life than those with imperfect rehabilitation adherence and post-stroke depressive symptoms. CONCLUSION: Both depressive symptoms and rehabilitation adherence behavior impacted the rehabilitation effect among patients who are recovering from a stroke.

Hepatitis C virus-associated type II mixed cryoglobulinemia vasculitis complicated with membranous proliferative glomerulonephritis.

Essential mixed cryoglobulinemia (type II) has turned out to be secondary to hepatitis C virus (HCV) infection in the large majority of patients. Interferon might be anticipated to be effective only in HCV-associated cryoglobulinemias. We found that interferon was highly effective in an HCV-positive patient with true essential type II mixed cryoglobulinemia. The patient presented with symptomatic cryoglobulinemic vasculitis without underlying immunologic, infectious, or neoplastic diseases. Tests for HCV viremia, a reverse-transcriptase-polymerase-chain-reaction (RT-PCR) assay, and anti-HCV antibodies (third-generation assays) were positive before therapy. The patient had severe cryoglobulinemic vasculitis with purpura, peripheral neuropathy, and membranous proliferative glomerulonephritis. The cryocrit before therapy was 6 percent in the patient. Recombinant interferon alfa-2a (Roferon-A, Hoffmann-LaRoche, Basel, Switzerland) was administered at a dose of 3 million units per day for three months and 3 million units every other day for the subsequent nine months, a protocol adopted for HCV-associated cryoglobulinemia. The patient had a complete clinical response, with the disappearance of serum cryoglobulins and all signs of cutaneous vasculitis and with the normalization of kidney-function results and urinary values in the patient with nephropathy. The patient has remained in complete remission for more than one year since the withdrawal of therapy. True essential mixed cryoglobulinemia with HCV infection complicated with glomerulonephritis represents a therapeutic challenge.

Valproate-induced hyperammonemic encephalopathy treated by hemodialysis.

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that may occur in people with normal liver-associated enzyme levels, despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl who had absence seizure and complained about progressive dizziness and general malaise several days after restarting valproate. Then, she presented vomiting and decreased consciousness three weeks after valproate use. Notably, her serum ammonia level was five times the upper limit of normal (184 micrommol/L), with normal liver-associated enzyme and supra-therapeutic valproate level. EEG showed continuous generalized slowing. The tandem mass analysis revealed carnitine deficiency. Consciousness improved after emergent hemodialysis. Ammonia level and EEG also returned to normal. Possible mechanisms, risk factors and the treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.

Renal carcinosarcoma: case report and review of literature.

Carcinosarcoma is a malignancy that occurs very rarely in the renal pelvis, and only a very limited number of cases has been documented. These tumors are composed of both malignant mesenchymal and epithelial elements. Unlike the cases described so far, this carcinosarcoma of the renal pelvis was characterized by quick recurrence, following complete surgical resection. Carcinosarcomas of the renal pelvis are known to be rapid in progression and associated with a poor prognosis. They usually metastasize soon after surgery, but very little information is available on the histologic types that characterize the recurrent tumors. We presented a histologically proven case of renal carcinosarcoma extending from renal pelvis. Convalescence was uneventful, and fifteen months after the operation, he is alive with no recurrence or metastasis. If a rapidly growing tumor is detected that seems to originate from the kidney with extension to ureter and bladder, carcinosarcoma should be included in the differential diagnosis.

Adjuvant hyperbaric oxygen therapy in the treatment of hemodialysis patients with chronic osteomyelitis.

BACKGROUND: Hemodialysis dependence is an independent risk factor for hematogenous complication, including distant metastatic infection and osteomyelitis. Chronic osteomyelitis is a serious disease that fails to respond to aggressive medical and surgical treatment. Hyperbaric oxygen therapy has been proved to enhance bone and soft tissue healing in many studies. This article presents the preliminary result of hyperbaric oxygen therapy in hemodialysis-dependent patients with chronic osteomyelitis. MATERIALS AND METHODS: Ten hemodialysis dependent patients who were diagnosed with chronic diffuse osteomyelitis were treated prospectively with adjunctive hyperbaric oxygen therapy, in addition to aggressive surgical debridement and antibiotic treatment. RESULTS: The hyperbaric oxygen therapy averaged 20 daily sessions. Successful treatment was achieved in eight patients (80%). The mean length of treatment was 21 days. The preliminary results are comparable with other series. CONCLUSION: Hyperbaric oxygen is effective as an adjunct to aggressive medical and surgical treatment in chronic refractory osteomyelitis among hemodialysis-dependent patients.

Sjogren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis.

We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.

Proteomic analysis of peritoneal dialysate fluid in patients with dialysis-related peritonitis.

The prognosis of uremia patients on continuous ambulatory peritoneal dialysis (CAPD) is related to frequent peritonitis rate. Frequent peritonitis will lead to peritoneum failure, making CAPD unfeasible. We have performed proteomic profiling of peritoneal dialysis effluent samples from a cross-section of CAPD patients with and without peritonitis in order to identify biomarkers of peritonitis. We performed 2D gel electrophoresis and surface-enhanced laser esorption/ionization time of flight mass spectrometry (SELDI-TOF MS) on peritoneal dialysis effluent from 16 subjects with peritonitis. A genetic algorithm search of principal component space revealed a group of a peak distinguishing peritonitis-positive subjects, with mass/charge (m/z) values of 11,117.4. Our analyses identified the peak at m/z 11,117.4 with an accuracy of 95% for classifying peritonitis. Mass spectrometric analysis of peritonitis PDE samples identified the 11,117.4 protein as beta2-microglobulin (B2M). Using an unbiased protein profiling approach, we have validated previously reported findings of B2M as a biomarker associated with CAPD peritonitis. Prospective studies are warranted to establish additional biomarkers that would be predictive of peritoneal dialysis peritonitis. Besides, extending the study to a larger number of patients with subgroup analyses may yield additional information of the peritoneal dialysate proteins in association with dialysis adequacy, residual renal function, nutritional status, and risk of peritoneal infection.

Paraneoplastic polymyositis associated with crescentic glomerulonephritis.

A female concurrently developed polymyositis (PM), lung cancer, and nephrotic range proteinuria. Renal biopsy revealed crescentic glomerulonephritis. Pathology of lung cancer was proved to be adenocarcinoma. After surgical treatment of lung cancer, the symptoms of PM-associated crescentic glomerulonephritis disappeared. PM is associated with a higher risk of malignancy, though renal involvement in patients with PM is thought to be uncommon. In patients with PM, there have been few reports concerning the coexistence of glomerular disease, including crescent glomerulonephritis. Herein we report a case of crescentic glomerulonephritis-associated PM that was successfully treated after the surgical removal of lung cancer. We consider that such association of PM and crescent glomerulonephritis is rare in adults. Careful evaluation of underlying malignancy is important. The definite treatment is adequate management of underlying malignancy.

Fas expression on peritoneal macrophages during continuous ambulatory peritoneal dialysis peritonitis.

BACKGROUND: Peritonitis is a common complication of end stage renal failure (ESRF) patients receiving continuous ambulatory peritoneal dialysis (CAPD). Peritoneal macrophage may participate in the activation of specific T cells and in the generation of local cell-mediated immunity to various pathogens. The purpose of this study is to investigate the possible role of macrophage in CAPD patients with peritonitis. METHODS: We evaluated the expression of Fas receptor (CD95), ICAM-1 (CD54), CD25, and CD69 by two-color flow cytometry on extravasted macrophages from 16 ESRF patients on CAPD with peritonitis (peritonitis-positive) and compared them to 11 ESRF patients on CAPD without peritonitis (peritonitis-negative) and normal controls. RESULTS: We found an increased expression of CD95, CD54, and CD25 on macrophage in peritonitis-positive group compared to controls (all p < 0.001). In the peritonitis-positive group, the CD95 expression was significantly higher than that of the peritonitis-negative group (p < 0.001). The expression of CD54, CD25, and CD69, however, was not significantly different between the peritonitis-positive and peritonitis-negative CAPD subgroups. CONCLUSION: We found an abnormally increased percentage of macrophage-expressing Fas receptor and ICAM-1, and the percentage of CD95+ macrophage, but not those of other markers, were increased among the subset of CAPD patients with peritonitis. The later finding suggests that this macrophage phenotype is associated with peritonitis occurring in CAPD.

Is rehabilitation intervention during hospitalization enough for functional improvements in patients undergoing lumbar decompression surgery? A prospective randomized controlled study.

OBJECTIVE: Rehabilitation has been reported to improve pain and disability for patients after lumbar surgery. However, studies to investigate the rehabilitation intervention for lumbar decompression surgery during hospitalization are scarce. The aim of this study was to examine outcomes of perioperative rehabilitation intervention for patients who underwent lumbar decompression surgery (LDS). METHODS: Patients aged 18-65 years old who received their first LDS were randomized into the perioperative rehabilitation group (PG) or control group (CG). The PG received rehabilitation intervention during hospitalization for lumbar decompression surgery. Pain, functional capacity, Roland-Morris Disability Questionnaire (RMDQ), and Short-Form Health Survey (SF-12) were assessed on admission, at discharge, and at follow-ups one month, three months, and six months after surgery. Two-way repeated measures ANOVAs were used for statistical analysis. RESULTS: A total of 60 patients scheduled for decompression surgery for lumbar stenosis were enrolled into the study. After surgery, the PG showed significant pain relief and improvement of disability as well as quality of life, but there were no significant functional improvements compared with the CG. CONCLUSIONS: The findings of this study indicate that the rehabilitation intervention during hospitalization improves pain intensity as well as disability and quality of life, yet has limited effects on the functional performance over time up to six months post-surgery in patients who received LDS. The study suggest that rehabilitation interventions during hospitalization must include regular support for patient adherence to the intervention program and focus on task-oriented programs for lower extremities such as closed-chain exercises in functional postures.

Stereoselective glycosylation of exo-glycals accelerated by Ferrier-type rearrangement.

[reaction: see text] Owing to the driving force of the Ferrier-type rearrangement, the exo-glycals are highly reactive with various alcohols to afford glycosides and glycoconjugates with exclusive alpha-configuration. The resulting vinyl group in these glycosylation products can be further elaborated for general applications, including the synthesis of spiro derivatives and glycosylation of 2-ketoaldonic acids.

Unexpected IgA nephropathy during the treatment of a young woman with idiopathic dermatomyositis: case report and review of the literature.

This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates that the most common pattern seen in idiopathic polymyositis is mesangial proliferative glomerulonephritis. However, both membranous and mesangial proliferative glomerulonephritis are often seen in idiopathic dermatomyositis. It is still not clear, however, whether the humorally- mediated immune process in dermatomyositis and the cell-mediated immune process in polymyositis can explain the different patterns of occurrence of glomerular lesions in these two closely related disease entities.

Aminograms during continuous hemodiafiltration in the treatment of hyperammonemia due to ornithine transcarbamylase deficiency.

BACKGROUND: Hyperammonemia caused by ornithine transcarbamylase (OTC) deficiency can be properly managed by continuous arteriovenous hemodiafiltration (CAVHDF). Removal of amino acids (AA) during CAVHDF has not been thoroughly investigated. AA losses in patients with urea cycle defects due to ornithine transcarbamylase deficiency treated by CAVHDF were analyzed. METHODS: Twelve neonates with elevated serum ammonia levels, confirmed through urine organic acid analysis and serum amino acid studies, were documented to have OTC deficiency. CAVHDF was administered in an attempt to lower serum ammonia concentration. Amino acid analysis of ultrafiltration and serum were performed by liquid chromatography. RESULTS: Serum levels of leucine, isoleucine, methionine, phenylalanine, and tyrosine were significantly lower than acceptable in these patients. Glutamine was the only amino acid that increased significantly, which is clinically relevant to OTC enzyme deficiency. Although the mean serum concentrations of tyrosine and glutamine concentrations were lower in the dialysate, the serum and dialysate concentrations of other amino acids did not differ. CONCLUSION: CAVHDF may induce changes in amino acid metabolism and distribution as well. The requirement of aminogram monitor for amino acid supplementation in urea cycle defect patients is important.

Continuous hemodiafiltration in the treatment of hyperammonemia due to methylmalonic acidemia.

Methylmalonic academia (MMA) is a rare inborn error of branched-chain amino acid metabolism. Therapy consists of a special formulated protein diet, carnitine supplementation, and emergent detoxification during acute decompensation. Continuous hemodiafiltration is a modality choice to treat acute metabolic decompensation in inborn error of metabolism. We report the successful use of continuous hemodiafiltration in the management of acute decompensation in patients with methylmalonic academia. Three male patients were diagnosed with methylmalonic academia with the initial presentation of hyperammonemia. Continuous hemodiafiltration was utilized to treat acute decompensation of metabolic crisis. This approach results in a rapid reduction of systemic toxin levels. Continuous hemodiafiltration should be considered as a treatment modality for symptomatic neonates with MMA where hemodialysis is not feasible.