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Severe Metabolic Acidosis (severe + metabolic_acidosis)
Selected AbstractsMethanol outbreak in Norway 2002,2004: epidemiology, clinical features and prognostic signsJOURNAL OF INTERNAL MEDICINE, Issue 2 2005K. E. HOVDA Abstract. Objectives., Knowledge on methanol poisoning does mainly come from clinical studies. We therefore report epidemiological, clinical and prognostic features from the large methanol outbreak in Norway in 2002,2004 where the new antidote fomepizole was the primary antidote in use. Design and subjects., Combined prospective and retrospective case series study of 51 hospitalized patients who were confirmed poisoned with methanol, of whom nine died. In addition, eight patients died outside hospital. Most patients were admitted in a late stage and because of symptoms. Treatment consisted of alkali, fomepizole (71%) and haemodialysis (73%). Results., The median serum methanol was 25.0 mmol L,1 (80 mg dL,1) (range 3.1,147.0 mmol L,1), median pH was 7.20 (6.50,7.50), and median base deficit 22 mmol L,1 (range 0,31). The most frequent clinical features reported were visual disturbances (55%), dyspnoea (41%), and gastrointestinal symptoms (43%). Twenty-four per cent were comatose on admission, of whom 67% died. There was a trend towards decreasing pCO2 with decreasing pH amongst the patients surviving. The opposite trend was demonstrated in the dying; the difference was highly significant by linear regression analyses (P < 0.001). Conclusions., Methanol poisoning still has a high morbidity and mortality, mainly because of late diagnosis and treatment. Respiratory arrest, coma and severe metabolic acidosis (pH < 6.90, base deficit >28 mmol L,1) upon admission were strong predictors of poor outcome. Early admission and ability of respiratory compensation of metabolic acidosis was associated with survival. [source] A fatal case of cutaneous zygomycosis in a patient with severe metabolic acidosisMYCOSES, Issue 4 2009C. Kosmidis Summary We present a case of cutaneous zygomycosis in a patient with an ureteroileostomy and severe metabolic acidosis, but without diabetes. The patient died despite multiple aggressive surgical interventions and antifungal therapy with liposomal amphotericin B. Ureteroileostomy-related acidosis can be a predisposing factor for zygomycosis. Metabolic acidosis can have a role in the severity of cutaneous disease. [source] Holocarboxylase Synthetase Deficiency Presenting as IchthyosisPEDIATRIC DERMATOLOGY, Issue 2 2006H. Alan Arbuckle M.D. Clinical manifestations usually present within the first few days of life and include severe acidosis, feeding difficulties, breathing abnormalities, vomiting, seizures, progressive loss of consciousness, coma, and death. Skin findings, when present, usually develop within the first weeks of life and are described as an erythroderma-like dermatitis involving the eyebrows, eyelashes, and scalp. We were asked to consult on a newborn with a collodion membrane and severe metabolic acidosis who was eventually diagnosed with holocarboxylase synthetase deficiency and ichthyosis. The diagnosis of holocarboxylase synthetase deficiency might be considered in a newborn with collodion membrane, ichthyosis, and acidosis. [source] Renal Fanconi syndrome and myopathy after liver transplantation: Drug-related mitochondrial cytopathy?PEDIATRIC TRANSPLANTATION, Issue 1 2008Umut Selda Bayrakci Abstract:, Advances in the field of transplantation provide a better quality of life and allow more favorable conditions for growth and development in children. However, combinations of different therapeutic regimens require consideration of potential adverse reactions. We describe a 15-yr-old girl who had orthotopic liver transplantation because of Wilson's disease. Tacrolimus, MMF, and steroids were given as immunosuppressant. Lamivudine was added because of de nova hepatitis B infection during her follow-up. Three yr after transplantation she developed renal Fanconi syndrome with severe metabolic acidosis, hypophosphatemia, glycosuria, and aminoaciduria. Although tacrolimus was suspected to be the cause of late post-transplant renal acidosis and was replaced by sirolimus, acidosis, and electrolyte imbalance got worse. Proximal muscle weakness has developed during her follow-up. Fanconi syndrome, as well as myopathy, is well recognized in patients with mitochondrial disorders and caused by depletion of mtDNA. We suggest that our patient's tubular dysfunction and myopathy may have resulted from mitochondrial dysfunction which is triggered by tacrolimus and augmented by lamivudine. [source] Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growthPEDIATRIC TRANSPLANTATION, Issue 6 2007Sema Aydogdu Abstract:, In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score <2), and four patients had weight SD score <2. Liver transplantation were performed at the age of 43.2 ± 27 months (range 9 to 96 mfonths), 6.5 ± 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score <2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis. [source] | ||||||||||