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Background: Succinyl-CoA:3 oxoacid CoA transferase deficiency (SCOTD) is a rare autosomal recessive disease, characterized by altered utilization of ketone bodies, with acute episodes of ketoacidosis. Clinical case: It is presented the case of a patient with SCOTD, with a first atypical episode accompanied by hyperglycemia, with 4 subsequent episodes with classic manifestations of the disease, presenting with a biochemical pattern of permanent ketonuria with marked elevation of ketone bodies (acetoacetate, 3 beta-hydroxybutyrate) in the study of urinary organic acids by gas chromatography and mass spectrometry, together with the clinical picture granting the diagnosis. It was started a maintenance therapy with a characteristic feeding plan; it was shown an adequate response to treatment, and the absence of permanent ketosis was surmised. Conclusion: Being a rare disease, the categorization of these patients as diabetic ketoacidosis is frequent. The clinical and biochemical characteristics with ketosis or persistent ketonuria should be analyzed very carefully, especially in patients presenting with hyperglycemia, which is an atypical manifestation of the disease, in order to make an early diagnosis and treatment, positively impacting the prognosis of patients.

Introducción: la deficiencia de succinil-CoA acetoacetato transferasa (SCOT) es una enfermedad rara, autosómica recesiva, caracterizada por alteración en la utilización de cuerpos cetónicos, con episodios agudos de cetoacidosis. Caso clínico: se presenta el caso de un paciente con deficiencia de SCOT, con un primer episodio atípico acompañado con hiperglucemia, con 4 episodios posteriores con manifestaciones clásicas de la enfermedad, que presentó patrón bioquímico de cetonuria permanente con marcada elevación de cuerpos cetónicos (acetoacetato, 3 beta-hidroxibutirato) en estudio de ácidos orgánicos urinarios por cromatografía de gases y espectrometría de masas, aunado a cuadro clínico que otorgó el diagnóstico. Se inició terapia de mantenimiento con plan de alimentación característico; se demostró una adecuada respuesta al tratamiento, y se infirió una ausencia de cetosis permanente. Conclusiones: al ser una enfermedad rara, la categorización de estos pacientes como cetoacidosis diabética es frecuente. Se deben analizar de forma muy minuciosa las características clínicas y bioquímicas con cetosis o cetonuria persistente, sobre todo en pacientes que se presenten con hiperglucemia, que es una manifestación atípica de la enfermedad, para realizar un diagnóstico y tratamiento temprano que impacte de forma positiva el pronóstico de los pacientes.

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RESUMEN Introducción: La cetoacidosis en el embarazo es una emergencia médica que requiere tratamiento en Unidad de Cuidados Intensivos debido a su asociación con morbimortalidad maternofetal. Las gestantes pueden presentar una forma atípica del cuadro llamada cetoacidosis normoglicémica, siendo muy infrecuente en pacientes sin antecedente de diabetes. Caso Clínico: Se presenta una gestante cursando tercer trimestre de embarazo, sin antecedente de diabetes, ingresada en Unidad de Paciente Crítico debido a neumonía por COVID-19 y acidosis metabólica con anión gap aumentado. Se realizó diagnóstico de cetoacidosis normoglicémica posterior al ingreso, iniciándose tratamiento intensivo de trastorno ácido-base con buena evolución. Conclusión: La infección por SARS-CoV-2 puede causar cetoacidosis normoglicémicas en embarazadas no diabéticas; se requiere una alta sospecha clínica para realizar el diagnóstico y tratamiento oportuno.

ABSTRACT Introduction: Ketoacidosis in pregnancy is a medical emergency that requires treatment in an intensive care unit due to its association with maternal-fetal morbimortality. Pregnant women may present an atypical form of the condition called normoglycemic ketoacidosis, being very rare in patients with no history of diabetes. Clinical Case: We present a pregnant woman in the third trimester of pregnancy, without history of diabetes, admitted to a critical patient unit due to COVID-19 pneumonia and metabolic acidosis with an increased anion gap. A diagnosis of normoglycemic ketoacidosis was made after admission, and intensive treatment of acid-base disorder was initiated, with good evolution. Conclusion: SARS-CoV-2 infection can cause normoglycemic ketoacidosis in non-diabetic pregnant women; is required a high clinical suspicion to make the diagnosis and appropriate treatment.

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Spinal muscular atrophy is an uncommon cause of ketoacidosis, where there is a decrease in muscle mass, an abnormal metabolism of glucose and fatty acids, and changes in neuroendocrine function. These conditions favor the accumulation of keto acids and the development of metabolic acidosis. We report a 26-year-old female, with a history of spinal muscular atrophy type III, consulting for abdominal pain and vomiting lasting one week. She was admitted to the emergency service somnolent and poorly perfused. She had a pH of 6.98, HCO3- of 3.8 mmol/L, pCO2 of 16.4 mmHg, BE of -26 mmol/L, delta ratio of 1.05, anion gap of 31 mEq/L, creatinine of 0.37 mg/dL, sodium of 147 mEq/L, potassium of 3.7 mEq/L, chloride of 112 mEq/L, lactate of 1.2 mmol/L, glucose of 108 mg/dL, albumin of 4.2 g/dL, ketonemia +++, ketonuria +, measured plasma osmolality of 322 mOsm/kg, estimated osmolality of 314 mOsm/kg, toxilab negative, salicylate levels < 3 µg/mL, acetaminophen levels < 1.2 µg/mL. Intravenous hydration and bicarbonate were started, without satisfactory response. Interpreting the clinical picture as a ketoacidosis induced by stress in a patient with spinal muscular atrophy, it was handled with glucose, amino acids, vitamins and trace elements, with a favorable response.

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Spinal muscular atrophy is an uncommon cause of ketoacidosis, where there is a decrease in muscle mass, an abnormal metabolism of glucose and fatty acids, and changes in neuroendocrine function. These conditions favor the accumulation of keto acids and the development of metabolic acidosis. We report a 26-year-old female, with a history of spinal muscular atrophy type III, consulting for abdominal pain and vomiting lasting one week. She was admitted to the emergency service somnolent and poorly perfused. She had a pH of 6.98, HCO3- of 3.8 mmol/L, pCO2 of 16.4 mmHg, BE of -26 mmol/L, delta ratio of 1.05, anion gap of 31 mEq/L, creatinine of 0.37 mg/dL, sodium of 147 mEq/L, potassium of 3.7 mEq/L, chloride of 112 mEq/L, lactate of 1.2 mmol/L, glucose of 108 mg/dL, albumin of 4.2 g/dL, ketonemia +++, ketonuria +, measured plasma osmolality of 322 mOsm/kg, estimated osmolality of 314 mOsm/kg, toxilab negative, salicylate levels < 3 µg/mL, acetaminophen levels < 1.2 µg/mL. Intravenous hydration and bicarbonate were started, without satisfactory response. Interpreting the clinical picture as a ketoacidosis induced by stress in a patient with spinal muscular atrophy, it was handled with glucose, amino acids, vitamins and trace elements, with a favorable response.

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Background: Cows in perinatal period are prone to ketosis, characterized by increased ketone bodies and decreased bloodsugar. Study found that cows with type II ketosis had higher blood glucose levels. This type of ketosis exhibits insulinresistance and impaired glucose tolerance that are similar to type 2 diabetes. The purpose of this study was to investigatethe role of insulin resistance(IR) in type II ketosis in dairy cows.Materials, Methods & Results: In one intensive cattle farm, eight cows with ketosis and eight healthy cows that were 14-21 days postpartum were selected. The plasma of experimental dairy cows was collected and tested for the concentrationof glucose concentration. Glucose tolerance of blood glucose levels in 120 min was used as the cut-off point to divide thedairy cows into three groups: the abnormal glucose tolerance ketosis group (TH), the normal glucose tolerance ketosis(TL), and the healthy control group. Simultaneously, the indexes of plasma liver function, oxidative stress, and insulinresistance were detected. The results showed that after intravenous glucose injection, there was no significant differencein blood glucose concentration between the TL and control group, while the blood glucose levels in the TH group werealways higher than in the other two groups. The insulin sensitivity index (RQUICKI) value was significantly lower in theTH group than in the other two groups. Liver function abnormalities in the TH group were more severe than in the TL andcontrol group. Oxidative stress was higher in the TH group than in the TL and control groups...(AU)

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Ketosis is a metabolic disorder that has an important impact on health and productivity in dairy cattle during early lactation. In Colombia, the incidence and risk factors for ketosis development have not been studied. The objectives of the present study were to determine the incidence of subclinical (SCK) and clinical ketosis (CK) and to determine the risk factors associated with ketosis within the first 6 weeks of lactation in dairy cattle in Colombia. A prospective cohort study was carried out. A convenience sample of 150 lactating dairy cows from ten commercial dairy farms in the Cundi-Boyacencian Andean plateau were tested weekly using a point of care device to measure ß-hydroxybutyrate during the first 6 weeks of lactation. Incidence of SCK and CK was calculated. Risk factors were evaluated using a survey for each animal and for each farm. Potential risk factors were evaluated using a X2 test, Relative Risk was calculated for significant variables by the X2 test (P ≤ 0.05) and these variables were included in the logistic regression model. Cumulative incidence of SCK was 25.33%, 3.33% for CK, and overall incidence of ketosis was 26%. In the logistic regression model, parity 3 or more, herd size > 150 animals, body condition score ≥ 3, retained placenta, and metritis were determined as risk factors for ketosis. The results of this study confirm previous reports in which a high body condition score, increased parity, herd size, retained placenta, and metritis are associated with an increased risk of ketosis in stabled and grazing cattle.

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Background: Cows in perinatal period are prone to ketosis, characterized by increased ketone bodies and decreased bloodsugar. Study found that cows with type II ketosis had higher blood glucose levels. This type of ketosis exhibits insulinresistance and impaired glucose tolerance that are similar to type 2 diabetes. The purpose of this study was to investigatethe role of insulin resistance(IR) in type II ketosis in dairy cows.Materials, Methods & Results: In one intensive cattle farm, eight cows with ketosis and eight healthy cows that were 14-21 days postpartum were selected. The plasma of experimental dairy cows was collected and tested for the concentrationof glucose concentration. Glucose tolerance of blood glucose levels in 120 min was used as the cut-off point to divide thedairy cows into three groups: the abnormal glucose tolerance ketosis group (TH), the normal glucose tolerance ketosis(TL), and the healthy control group. Simultaneously, the indexes of plasma liver function, oxidative stress, and insulinresistance were detected. The results showed that after intravenous glucose injection, there was no significant differencein blood glucose concentration between the TL and control group, while the blood glucose levels in the TH group werealways higher than in the other two groups. The insulin sensitivity index (RQUICKI) value was significantly lower in theTH group than in the other two groups. Liver function abnormalities in the TH group were more severe than in the TL andcontrol group. Oxidative stress was higher in the TH group than in the TL and control groups...

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The case of a patient that bleed approximatelly 40 lts during a liver and kidney transplant is reported. Strategies directed to maintain hemodynamic and hemostatic conditions are discussed. Elective surgery in patients that may require massive transfusion allow to planify a strategy directed to avoid hypothermia, appearence of metabolic acidosis and coagulopathy. All of these conditions have been related to the appearence and maintenance of bleeding. Maintenance of hemostatic condition appears particularly important requiring a strategy of early administration of blood components directed to preserve an adequate level of clotting factors and platelets. The same considerations must be kept in mind when the requirement of massive transfusion is not expected and the coagulation condition must be preserved or restored.

Se reporta el caso de un paciente que sangró 40 litros durante un trasplante hepático y renal, discutiéndoselas estrategias utilizadas, mantener adecuadas condiciones hemodinámicas y hemostáticas.La cirugía electiva en pacientes que pueden requerir transfusión masiva permite planificar la implementación de medidas dirigidas a prevenir la aparición de hipotermia, acidosis metabólica y coagulopatía, los cuales han sido identificados, relacionados a la aparición y mantención de un estado de mayor sangrado. La mantención de adecuadas condiciones hemostáticas aparece como un factor de gran importancia, requiriendo una estrategia que requiere la precoz administración de hemocomponentes, con la idea de mantener adecuados niveles de factores de coagulación y plaquetas. Estas mismas consideraciones deben tenerse presente cuando el requerimiento de transfusión masiva es imprevisto, y las condiciones de hemostasia deben ser mantenidas o restauradas.

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The purpose of this study was to estimate the prevalence of Subclinical ketosis (SCK) between 4 and 19 days in milk (DIM) in a grazing production system and investigate the importance of potential risk factors for SCK. This cross-sectional study was conducted in dairy cows (n = 107), which had more of two parities. The concentration of ß-hydroxybutyric (BHB) in blood was quantified through a hand-held meter. Potential risk factors evaluated were calving interval (CI), milk yield in previous lactation, metritis, dystocia, calf sex (male), parity (≤3 vs. ≥4) and pre-partum body condition score (BCS ≤ 3.5 vs. ≥3.75). Prevalence of SCK was 10.3% (95% CI 4.7-15) between 4 and 19 DIM. Risk factors identified were the occurrence of both metritis and pre-partum BCS ≥ 3.75. Cows with metritis had 4.9 (95% CI 1.17-20.98) times more risk of developing SCK than cows without metritis. And the cows with pre-partum BCS ≥ 3.75 had 5.25 (95% CI 1.32-21.11) times more risk of developing SCK than cows with pre-partum BCS ≤ 3.5. Metritis could induce a lower feed intake and promote the development of SCK. High pre-partum BCS could induce a greater mobilization of body reserves altering liver function and aggravating post-partum NEB. The results are indicative of the expected prevalence of SCK in grazing production system. Factors associated could help to identify cattle at risk of SCK and improve the management of strategies to limit the effects.

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The effects of linseed oil (LO) and macadamia oil (MO) on the metabolic changes induced by a high-fat diet (HFD) rich in saturated fatty acid were investigated. For the purpose of this study, the vegetable oil present in the HFD, i.e. soybean oil (SO) was replaced with LO (HFD-LO) or MO (HFD-MO). For comparative purposes, a group was included, which received a normal fat diet (NFD). Male Swiss mice (6-week old) were used. After 14 days under the dietary conditions, the mice were fasted for 18 h, and experiments were then performed. The HFD-SO, HFD-LO and HFD-MO groups showed higher glycaemia (p < 0.05 versus NFD). However, no significant effect was observed on glycaemia, liver gluconeogenesis and liver ketogenesis when SO was replaced by either LO or MO. The body weight and the sum of epididymal, mesenteric, retroperitoneal and inguinal fat weights were higher (p < 0.05) in the HFD-SO and HFD-MO groups as compared with the NFD group. However, there was no significant difference in these parameters between the NFD and HFD-LO groups. Thus, the protective role of LO on lipid accumulation induced by an HFD rich in saturated fatty acid is potentially mediated by the high content of É·-3 polyunsaturated fatty acid in LO.

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