Substitution of soybean meal for yellow mealworm meal in the diet of slow-growing chickens provides comparable carcass traits and meat quality.

1. This study investigated the effects of incorporating yellow mealworm (Tenebrio molitor) larval meal as a partial and/or complete substitute for soybean meal on carcass and meat quality in slow-growing chickens.2. A total of 256 one-day-old male broilers were randomly allocated to 1 of 32 experimental units distributed among four treatments (n = 8): the control treatment (C), where soybean (SB) meal was the protein source, and three experimental treatments, in which SB meal was replaced by Tenebrio molitor (TM) larval meal at levels of 50% (T1), 75% (T2) and 100% (T3), respectively. Three different feed phases (1-29; 29-57 and 57-92 d of age) were used for each treatment. All chickens were slaughtered at 92 d of age, with eight animals per treatment randomly selected to assess carcass and meat quality. Near-infrared reflectance spectroscopy (NIR) was used to classify meat quality.3. Carcass traits were not significantly different between treatments, except for head and thigh weight, which were higher in the control group (p < 0.01). In terms of physicochemical characteristics, treatment T2 showed less yellowness (p < 0.05), while water and cooking losses were lower in treatments T1 and T2 (p < 0.01). Meat from both T1 and T2 groups had lower shear forces (p < 0.01), higher moisture content (p < 0.01) and less protein (p < 0.05) compared to treatments C andT3. Birds fed T3 had the highest meat ash content (p < 0.01). Chickens consuming TM had higher monounsaturated fatty acid (MUFA) levels and lower polyunsaturated fatty acid (PUFA) and n-6 acidsPUFA (p < 0.01).4. Substitution of SB with TM is a protein alternative for slow-growing chickens that supports carcass and meat quality comparable to those fed a conventional diet.

How to detect non-institutionalized older patients at risk of malnutrition during their hospitalization? Comparison of 8 screening tools for malnutrition or nutritional risk.

BACKGROUND: The prevalence of malnutrition is high among the elderly population. Hospital admission is a window of opportunity for its detection. OBJECTIVE: To assess the concordance of different nutritional scales in hospitalized patients. METHODS: Prospective study in non-institutionalized patients over 65 years of age admitted to an internal medicine department. Five malnutrition screening surveys (MNA, MST, MUST, NRS-2000 and CONUT) and three nutritional risk screening surveys (SCREEN 3, 8 and 14) were compared. As gold standard we use the Global Malnutrition Leadership Initiative for Malnutrition (GLIM) definition of malnutrition. RESULTS: Eighty-five patients (37% female, median age 83 years) were included. Forty-eight percent (95% CI 38-59%) of patients were classified as malnourished according to GLIM criteria. The SCREEN 3 scale was the most sensitive (93%; 95% CI 87-98) and MUST the most specific (91%; CI 85-99). The most effective scale for excluding suspected malnutrition was SCREEN 3 (LR- 0.17; 95% CI 0.05-0.53) and the best for confirming it was MST (LR+ 7.08; 95% CI 3.06-16.39). Concordance between the different scales was low or very low with kappa indices between 0.082 and 0.465. CONCLUSIONS: A comprehensive approach is needed to detect malnutrition in hospitalized patients. More sensitive scales are more useful in initial screening. Nutritional risk tools could be effective at this stage. In a second step, malnutrition should be confirmed according to established criteria such as GLIM.

Immune thrombotic thrombocytopenic purpura in older patients: Results from the Spanish TTP Registry (REPTT).

Immune thrombotic thrombocytopenic purpura (iTTP) is an ultra-rare disease that seldom occurs in the elderly. Few reports have studied the clinical course of iTTP in older patients. In this study, we have analysed the clinical characteristics at presentation and response to therapy in a series of 44 patients with iTTP ≥60 years at diagnosis from the Spanish TTP Registry and compared them with 209 patients with <60 years at diagnosis from the same Registry. Similar symptoms and laboratory results were described in both groups, except for a higher incidence of renal dysfunction among older patients (23% vs. 43.1%; p = 0.008). Front-line treatment in patients ≥60 years was like that administered in younger patients. Also, no evidence of a difference in clinical response and overall survival was seen in both groups. Of note, 14 and 25 patients ≥60 years received treatment with caplacizumab and rituximab, respectively, showing a favourable safety and efficacy profile, like that observed in patients <60 years.

Características radiográficas de la neumonía adquirida en la comunidad en el adulto inmunocompetente hospitalizado según el agente causal / Radiographic characteristics of community-acquired pneumonia in the immunocompetent adult hospitalized according to the etiologic agent

Resumen En la práctica clínica, la radiografía de tórax permite confirmar el diagnóstico y la extensión de la neumonía adquirida en la comunidad (NAC). Objetivos: Examinar las características radiográficas de la NAC según el agente causal y el grado de concordancia interobservador (CI) en la descripción de los hallazgos radiográficos. Métodos: Se evaluaron las radiografías de tórax de 300 pacientes adultos inmunocompetentes hospitalizados por NAC, tres residentes de radiología consignaron el patrón de los infiltrados pulmonares, su localización anatómica y extensión, la presencia de derrame pleural y otros hallazgos radiográficos. Se realizaron cultivos de esputo, hemocultivos, pruebas serológicas y técnicas de biología molecular de hisopado nasofaríngeo para identificar los principales patógenos respiratorios. Resultados: Las manifestaciones clínicas y los hallazgos de la radiografía de tórax fueron similares en las neumonías causadas por diferentes patógenos respiratorios: bacterias clásicas, virus respiratorios y microorganismos atípicos. En las neumonías bacterianas predominó el patrón de relleno alveolar de distribución lobar, en las neumonías vírales y atípicas predominó el patrón intersticial o mixto alvéolo-intersticial con opacidades en vidrio esmerilado. La CI fue satisfactoria (kappa > 0,6) para determinar el patrón principal de los infiltrados pulmonares, su localización anatómica y la presencia de derrame pleural, su localización y extensión. La CI fue moderada (kappa 0,4-0,6) para definir la extensión de la neumonía y detectar signos radiológicos asociados a congestión pulmonar. Conclusión: Los hallazgos de la radiografía de tórax no permitieron identificar con precisión el agente causal de la neumonía, siendo útil en la caracterización de los infiltrados pulmonares y para detectar complicaciones como el derrame paraneumónico.

In a clinical setting the chest radiograph is the reference standard in establishing the diagnosis of community-acquired pneumonia (CAP). Objectives: This study aimed to assess interobserver reliability (IR) of radiographic findings and the relationship to different respiratory pathogens in CAP. Methods: Chest radiographs of 300 immunocompetent adult patients hospitalized with pneumonia, obtained from a database, were reviewed by three residents of radiology without specific clinical information. Main pattern of pulmonary infiltrates, topographic localization, extent of pneumonia, presence of pleural fluid, thickened bronchial walls, lymphadenopathy and air bronchogram were scored. Sputum and blood cultures, serological tests and nasopharyngeal swab for respiratory virus detection by molecular diagnostic techniques were performed to identify the causative pathogen. Results: Clinical manifestations and chest X-ray findings were similar in pneumonias caused by different respiratory pathogens: classic bacteria, respiratory viruses and atypical microorganisms. The alveolar pattern of lobar distribution predominated in bacterial pneumonia; meanwhile, interstitial or mixed alveolar-interstitial pattern with ground glass opacities predominated in viral and atypical pneumonias. IR was fair to good (kappa > 0.6) for determining the main pattern of infiltrates, anatomical location and the presence of pleural effusion, their anatomical location and extension. IR was moderate (kappa 0.4-0.6) for determining the extent of pneumonia and signs of congestive heart failure. Conclusion: Simple features such as main pattern description, anatomical location, identifying the involved lobes and pleural fluid recognition showed fair to excellent interobserver reliability. Chest radiographs was of limited value in predicting the causative pathogen but were of beneficial use to characterize pulmonary infiltrates and to detect complications such as parapneumonic effusion.

CYB5R3 homozygous pathogenic variant as a rare cause of cyanosis in the newborn.

Detailed below is a very illustrative case of a rare pathology of recessive congenital methemoglobinemia. The patient, a newborn female, was homozygous for c.535G > A, p.(Ala179Thr) a pathogenic variant in the CYB5R3 gene. The reported population frequency of the allele is 0.853%, demonstrating why it is remarkable to find both parents are heterozygous carriers without consanguinity. A brief review of previously published cases is also presented.

Identification and antimicrobial susceptibility of Streptomyces and other unusual Actinobacteria clinical isolates in Spain.

Two hundred and eighty-six isolates from human clinical samples were identified between 1996 and 2019 as belonging to 8 families, 19 genera and 88 species of Actinobacteria. The most identified genera were Streptomyces (182 strains from 45 species), Actinomadura (29 strains, 5 species), Nocardiopsis (21 strains, 6 species) and Dietzia (18 strains, 5 species). The rest of the identified genera (15) contained 27 species with 36 isolates. Of the species studied, only 13/88 had been documented previously as isolates from clinical samples, and in some cases, as true pathogens. In this sense, a literature review of the species found in infections or in clinical samples without clear involvement in pathology has been carried out. Finally, the susceptibility to 8 antimicrobial agents has been studied. Streptomyces showed high resistance (80.8%) against cefotaxime and cotrimoxazole (55.5%), and no isolate resistance to amikacin and linezolid have been found. Lower percentages of resistance have been found in other genera, except in Dietzia (100% against cotrimoxazole and 44.4% against erythromycin). The greatest resistance in these genera was to cotrimoxazole (29.8) and erythromycin (27,9%), and no resistance to linezolid has been found in these genera. In Microbispora, Nonomuraea and Umezawaea, no resistant isolates have been found against any antibiotic studied. Only 3/104 isolates were resistant to amikacin in Amycolatopsis, Crossiella, and Micromonosopora. One isolate of Amycolatopsis was resistant to imipenem.

Power-series solution of compartmental epidemiological models.

In this work, power-series solutions of compartmental epidemiological models are used to provide alternate methods to solve the corresponding systems of nonlinear differential equations. A simple and classical SIR compartmental model is considered to reveal clearly the idea of our approach. Moreover, a SAIRP compartmental model is also analyzed by using the same methodology, previously applied to the COVID-19 pandemic. Numerical experiments are performed to show the accuracy of this approach.

Differential diagnosis of adipocytic differentiation in androgen-secreting mature ovarian teratoma with Leydig cell hyperplasia.

Mature cystic teratomas (dermoid cysts) of the ovary are very rarely associated with androgen production. The source of androgens in these cysts may be tumours such as Sertoli-Leydig cell tumour or Leydig cell hyperplasia. In this study, we present a case of virilisation in a postmenopausal female patient, where Leydig cell hyperplasia in a mature cystic teratoma was found to be responsible for the production of testosterone. In addition, extensive areas of lipomatous differentiation were identified. These areas showed significant alterations in adipocytic morphology, and differential diagnoses such as spindle cell lipoma (SCL) and atypical lipomatous tumour (ALT) were excluded after additional workup. Adipose tissue is traditionally described as an energy reservoir, but recently it has become clear that adipose tissue is a complex endocrine organ with additional metabolic roles in whole body homeostasis. Exuberant proliferation of lipomatous tissue in this teratoma raises the possibility of a synergistic role of Leydig cells and adipocytes in the development of hyperandrogenism.

"You Don't Have a Normal Life": Coping with Chagas Disease in Los Angeles, California.

Chagas disease is the neglected tropical disease of greatest public health impact in the United States, where it affects over 300,000 people. Diverse barriers limit healthcare access for affected people; fewer than 1% have obtained testing or treatment. We interviewed 50 people with Chagas disease in Los Angeles, California, and administered a cultural consensus analysis questionnaire. Participants were asked about their experiences and perceptions of Chagas disease, access to healthcare, and strategies for coping with the disease. In participants' narratives, the physical and emotional impacts of the disease were closely interwoven. Participant explanatory models highlight difficulties in accessing care, despite a desire for biomedical treatment. Obtaining testing and treatment for Chagas disease poses substantial challenges for US patients.

Concordancia diagnóstica del sistema de graduación Chisholm Mason y Focus Score en la sialoadenitis linfocítica focal como aproximación al síndrome de Sjögren / Diagnostic agreement of the Chisholm Mason and Focus Score grading system in focal lymphocytic sialadenitis in patients with dry symptoms

RESUMEN Introducción: Existen diferentes sistemas de graduación para interpretar la biopsia de glándula salival menor (BGSM) en pacientes con síntomas secos. En la actualidad la metodología recomendada (Focus Score [FS]), aunque no se emplea de forma rutinaria, conlleva discrepancias diagnósticas. Objetivo: Determinar la concordancia de la lectura histopatológica de BGSM entre las técnicas FS y Chisholm Mason (CM) para el diagnóstico de sialoadenitis linfocítica focal en el hospital de San José, entre enero de 2017 y junio de 2018. Métodos: Estudio de concordancia. Se incluyeron BGSM de pacientes con síntomas secos. Se excluyó material no apto para el estudio, o con menos de 4 lóbulos o menos de 8 mm de área. Se clasificaron utilizando las técnicas FS y CM por 2 evaluadores independientes. Se evaluó el acuerdo interobservador, intraobsevador y global entre las pruebas mediante el coeficiente de Kappa de Cohen. Resultados: Se evaluaron 130 biopsias. Los valores K intraobservador entre FS y CM fueron 0,42 para el observador 1 y 0,23 para el observador 2. Los valores K del acuerdo interobservador fueron 0,47 para FS y 0,65 para CM. La concordancia entre los 2 sistemas de puntuación fue K de 0,13. Conclusiones: Se evidencia una probable sobreestimación de focos con CM, que resalta sus debilidades, y mayor precisión con FS. Por ende, la concordancia entre las 2 pruebas es débil. Es necesario un mayor esfuerzo para el uso del método estandarizado de lectura de BGSM con el FS para una correcta interpretación y beneficio en la clasificación de pacientes con síndrome seco.

ABSTRACT Introduction: There are different grading systems for interpreting the minor salivary gland biopsy (MSGB) in patients with dry symptoms (Sicca). The recommended methodology is currently the Focus Score (FS), which, although not performed routinely, results in diagnostic discrepancies. Objective: To determine the concordance of the histopathological reading of MSGB between the FS and Chisholm Mason (CM) techniques for diagnosis of focal lymphocytic sialadenitis at the San José Hospital between January 2017 and June 2018. Methods: Concordance study. MSGB of patients with sicca symptoms were included. Material not suitable for study and / or with less than 4 lobules and / or less than 8 mm of surface area was excluded. They were classified, using the FS and CM techniques, by two independent evaluators. The inter-observer, intra-observer, and overall agreement between the tests were evaluated using Cohen's Kappa coefficient. Results: A total of 130 biopsies were evaluated. The intra-observer K values between FS and CM were 0.42 for observer 1, and 0.23 for observer 2. The K values of the inter-observer agreement were 0.47 for FS, and 0.65 for CM. The concordance between the two scoring systems was K = 0.13. Conclusions: There is evidence of a probable over-estimation of foci with CM, highlighting its weaknesses and greater precision with FS technique, resulting in the weak concordance between the two tests. A greater effort is necessary for the use of the standardised MSGB FS reading method for a correct interpretation and benefit in the classification of patients with dry syndrome.

Molecular identification of Leishmania tropica and L. infantum isolated from cutaneous human leishmaniasis samples in central Morocco.

BACKGROUND & OBJECTIVES: Cutaneous leishmaniasis (CL) in Marrakesh-Safi region located in the central-south part of Morocco is a public health problem. This study assessed the efficiency of a microscopic examination method in establishing the diagnosis of CL and PCR for the characterization and identification of the circulating Leishmania strains in different CL foci of the study area. METHODS: A total of 297 smears obtained from cutaneous lesions of suspected patients with CL were stained with May-Grünwald Giemsa (MGG) for microscopic examination. For each positive smear, genomic DNA was extracted and PCR-analysed, targeting the small subunit ribosomal ribonucleic acid (ssu rRNA) gene to detect Leishmania DNA. Then, the internal transcribed spacer 1 (ITS1) was amplified and sequenced in order to identify the Leishmania species. The sensitivity and specificity of the conventional microscopy with ssu rRNA gene were compared by Leishmania nested PCR (LnPCR) and ITS1 gene by ITS-PCR. RESULTS: A total of 257 smears were positive in the microscopic examination, i.e. the detection rate of amastigotes by optical microscopy was 86.53% (257/297). The LnPCR was found to have a specificity and a sensitivity of 100%, each. Interestingly, the sequencing results showed that 99.61% (256/257) of the isolates had Leishmania tropica and 0.39% (1/257) had L. infantum infection. INTERPRETATION & CONCLUSION: Though, classical microscopic examination is useful and economical, it is not sensitive enough, especially in endemic regions where several Leishmania species coexist. In such situations, PCR constitutes a complementary method for the identification of the causal species. The results indicate that both the L. tropica (dominant) and L. infantum are the causative agents of CL in the Marrakesh-Safi region. The rate of CL infection is high in Imintanout, and Chichaoua provinces. Hence, early diagnosis and prompt treatment of CL patients is necessary to prevent its extension to neighboring localities.

Causes and impacts of a mine water spill from an acidic pit lake (Iberian Pyrite Belt).

In May 2017, a spill from La Zarza pit lake (SW Spain) resulted in the release of approximately 270,000 m3 of extremely acidic waters to the Odiel River. Around 780 × 103 kg of Fe, 170 × 103 kg of Al, 2.15 × 103 kg of As and high amounts of other trace metals and metalloids were spilled. The purpose of this study is to explain the causes, consequences and impacts of the mine spill on the receiving water bodies. To this end, an extensive sampling along the mine site, river and estuary as well as a hydrological model of the pit lake was performed. Around 53 km of the Odiel River's main course, which was already contaminated by acid mine drainage (AMD), were affected. The mine spill resulted in an incremental impact on the Odiel River water quality. Thus, dissolved concentrations of some elements increased in the river up to 450 times; e.g. 435 mg/L of Fe and 0.41 mg/L of As. Due to low pH values (around 2.5), most metals (e.g., Cu, Zn, Mn, Cd) were transported in the dissolved phase to the estuary, exhibiting a conservative behavior and decreasing their concentration only due to dilution. However, dissolved concentrations of Fe, Cr, Pb, Se, Sb, Ti, V and especially As decreased significantly along the river due to Fe precipitation and sorption/coprecipitation processes. At the upper zone of the estuary, a noticeable increment of metal concentrations (up to 77 times) was also recorded. The water balance illustrates the existence of groundwater inputs (at least 16% of total) to the pit lake, due probably to local infiltration of rainwater at the mining zone. The probable existence of an ancient adit connected to the pit lake indicates that potential releases could occur again if adequate prevention measures are not adopted.

Prognosis of venous thromboembolism in orthopaedic surgery or trauma patients and use of thromboprophylaxis. / Pronóstico de la enfermedad tromboembólica venosa en cirugía ortopédica o pacientes traumatológicos y uso de tromboprofilaxis.

BACKGROUND: There is scarce evidence about the prognosis of venous thromboembolism in patients undergoing orthopedic surgery and in patients suffering non-surgical trauma. METHODS: We used the RIETE database (Registro Informatizado de pacientes con Enfermedad Trombo Embólica) to compare the prognosis of venous thromboembolism and the use of thromboprophylaxis in patients undergoing different orthopedic procedures and in trauma patients not requiring surgery. RESULTS: From March 2001 to March 2015, a total of 61,789 patients were enrolled in RIETE database. Of these, 943 (1.52%) developed venous thromboembolism after elective arthroplasty, 445 (0.72%) after hip fracture, 1,045 (1.69%) after non-major orthopedic surgery and 2,136 (3.46%) after non-surgical trauma. Overall, 2,283 patients (50%) initially presented with pulmonary embolism. Within the first 90 days of therapy, 30 patients (0.66%; 95% CI 0.45-0.93) died from pulmonary embolism. The rate of fatal pulmonary embolism was significantly higher after hip fracture surgery (n = 9 [2.02%]) than after elective arthroplasty (n = 5 [0.53%]), non-major orthopedic surgery (n = 5 [0.48%]) or non surgical trauma (n = 11 [0.48%]). Thromboprophylaxis was more commonly used for hip fracture (93%) or elective arthroplasty (94%) than for non-major orthopedic surgery (71%) or non-surgical trauma (32%). Major bleeding was significantly higher after hip fracture surgery (4%) than that observed after elective arthroplasty (1.6%), non-major orthopedic surgery (1.5%) or non-surgical trauma (1.4%). CONCLUSIONS: Thromboprophylaxis was less frequently used in lower risk procedures despite the absolute number of fatal pulmonary embolism after non-major orthopedic surgery or non-surgical trauma, exceeded that observed after high risk procedures.

Determination of denudation rates by the measurement of meteoric 10Be in Guadiana river sediment samples (Spain) by low-energy AMS.

The concentration of meteoric 10Be in estuarine sediment samples has been measured by Spanish Accelerator for Radionuclides Analysis (SARA) at CNA and subsequently used to assess the denudation rate in Guadiana river basin together with the sediment budget method, on both sides of the frontier between Spain and Portugal. The two methods yielded coincident results. The estimation by the 10Be method gave the denudation rate of (0.76 ±â€¯0.10) × 10-2 cm/y. After correcting for an approximate 80% attenuation of the sediment discharge into the ocean, caused by the river dams, the sediment budget method yielded the rate of (0.77 ±â€¯0.17) × 10-2 cm/y.

Amendment of the OMERACT ultrasound definitions of joints' features in healthy children when using the DOPPLER technique.

BACKGROUND: Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler findings in the evaluation and definition of normal joint features that can be visualized in healthy children at different age groups. METHODS: A multistep approach was used. Firstly, new additional definitions of joint components were proposed during an expert meeting. In the second step, these definitions, along with the preliminary B-mode-US definitions, were tested for feasibility in an exercise in healthy children at different age groups. In the last step, a larger panel of US experts were invited to join a web-based consensus process in order to approve the developed definitions using the Delphi methodology. A Likert scale of 1-5 was used to assess agreement. RESULTS: Physiological vascularity and fat pad tissue were identified and tested as two additional joint components in healthy children. Since physiological vascularity changes over the time in the growing skeleton, the final definition of Doppler findings comprised separate statements instead of a single full definition. A total of seven statements was developed and included in a written Delphi questionnaire to define and validate the new components. The final definitions for fat pad and physiological vascularity agreed by the group of experts reached 92.9% and 100% agreement respectively in a web survey. CONCLUSION: The inclusion of these two additional joints components which are linked to detection of Doppler signal in pediatric healthy joints will improve the identification of abnormalities in children with joint pathologies.

Asymptomatic carriers of Leishmania infantum in patients infected with human immunodeficiency virus (HIV) in Morocco.

In Morocco, visceral leishmaniasis (VL) is a parasitic disease caused by the flagellated protozoan parasite Leishmania infantum. L. infantum is transmitted by the bite of female phlebotomine sandflies, and its main reservoir hosts are domestic dogs. Asymptomatic infection with L. infantum is more frequent than clinically apparent disease. In HIV-infected patients, the risk of clinical VL is increased due to immunosuppression that may reactivate latent infections. However, coinfected subjects do not necessarily develop VL and may remain as asymptomatic carriers depending on their immune status. The present study investigates the asymptomatic carriers of L. infantum in HIV-infected patients in central Morocco, where human cases of visceral leishmaniasis by L. infantum have been reported. A total of 200 HIV-infected patients attending the Infectious Diseases Unit of the Ibn Zohar Hospital of Marrakech participated in the study. Parasitological and serological blood analyses included a direct microscopic examination (DME), culture in Novy-McNeal-Nicolle (NNN) medium, and serology by indirect immunofluorescence (IFI). We found prevalence rates of 5% (10/200) by IFI, 3% (6/200) by DME, and 2.5% (5/200) by culture. The parasite was identified as L. infantum by PCR from positive cultures.

"It's Like a Phantom Disease": Patient Perspectives on Access to Treatment for Chagas Disease in the United States.

Chagas disease (CD) affects > 6 million people globally, including > 300,000 in the United States. Although early detection and etiological treatment prevents chronic complications from CD, < 1% of U.S. cases have been diagnosed and treated. This study explores access to etiological treatment from the perspective of patients with CD. In semi-structured interviews with 50 Latin American-born patients of the Center of Excellence for Chagas Disease at the Olive View-UCLA Medical Center, we collected demographic information and asked patients about their experiences managing the disease and accessing treatment. Patients were highly marginalized, with 63.4% living below the U.S. poverty line, 60% lacking a high school education, and only 12% with private insurance coverage. The main barriers to accessing health care for CD were lack of providers, precarious insurance coverage, low provider awareness, transportation difficulties, and limited time off. Increasing access to diagnosis and treatment will not only require a dramatic increase in provider and public education, but also development of programs which are financially, linguistically, politically, and geographically accessible to patients.

International practices in the dietary management of fructose 1-6 biphosphatase deficiency.

BACKGROUND: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. METHODS: A cross-sectional questionnaire (13 questions) was emailed to all members of the Society for the Study of Inborn Errors of Metabolism (SSIEM) and a wide database of inherited metabolic disorder dietitians. RESULTS: Thirty-six centres reported the dietary prescriptions of 126 patients with FBPase deficiency. Patients' age at questionnaire completion was: 1-10y, 46% (n = 58), 11-16y, 21% (n = 27), and >16y, 33% (n = 41). Diagnostic age was: <1y, 36% (n = 46); 1-10y, 59% (n = 74); 11-16y, 3% (n = 4); and >16y, 2% (n = 2). Seventy-five per cent of centres advocated dietary restrictions. This included restriction of: high sucrose foods only (n = 7 centres, 19%); fruit and sugary foods (n = 4, 11%); fruit, vegetables and sugary foods (n = 13, 36%). Twenty-five per cent of centres (n = 9), advised no dietary restrictions when patients were well. A higher percentage of patients aged >16y rather than ≤16y were prescribed dietary restrictions: patients aged 1-10y, 67% (n = 39/58), 11-16y, 63% (n = 17/27) and >16y, 85% (n = 35/41). Patients classified as having a normal fasting tolerance increased with age from 30% in 1-10y, to 36% in 11-16y, and 58% in >16y, but it was unclear if fasting tolerance was biochemically proven. Twenty centres (56%) routinely prescribed uncooked cornstarch (UCCS) to limit overnight fasting in 47 patients regardless of their actual fasting tolerance (37%). All centres advocated an emergency regimen mainly based on glucose polymer for illness management. CONCLUSIONS: Although all patients were prescribed an emergency regimen for illness, use of sucrose and fructose restricted diets with UCCS supplementation varied widely. Restrictions did not relax with age. International guidelines are necessary to help direct future dietary management of FBPase deficiency.