1/148. Metabolic acidosis and thiamine deficiency.We describe a 19-year-old patient who was receiving home parenteral nutrition in whom lactic acidosis developed. A review of her home parenteral nutrition formula revealed the absence of multivitamins, most significantly thiamine. After thiamine administration, the acidosis resolved, and the patient experienced pronounced clinical improvement. Clinicians must be aware that thiamine is essential for normal glucose metabolism and that thiamine deficiency can lead to lactic acidosis. thiamine deficiency should be included in the differential diagnosis of lactic acidosis. The recent shortage of intravenous multivitamin preparations has led to documented cases of lactic acidosis as a result of thiamine deficiency, and a previous shortage led to several deaths due to lactic acidosis as a consequence of thiamine deficiency. All patients receiving parenteral nutrition must also receive adequate vitamin supplementation.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/148. Metabolic acidosis in restraint-associated cardiac arrest: a case series.The mechanism of death in patients struggling against restraints remains a topic of debate. This article presents a series of five patients with restraint-associated cardiac arrest and profound metabolic acidosis. The lowest recorded pH was 6.25; this patient and three others died despite aggressive resuscitation. The survivor's pH was 6.46; this patient subsequently made a good recovery. Struggling against restraints may produce a lactic acidosis. Stimulant drugs such as cocaine may promote further metabolic acidosis and impair normal behavioral regulatory responses. Restrictive positioning of combative patients may impede appropriate respiratory compensation for this acidemia. Public safety personnel and emergency providers must be aware of the life threat to combative patients and be careful with restraint techniques. Further investigation of sedative agents and buffering therapy for this select patient group is suggested.- - - - - - - - - - ranking = 6.5498794759653keywords = acidemia (Clic here for more details about this article) |
3/148. Abnormal ductus venosus blood flow: a clue to umbilical cord complication.We report a case of umbilical cord complication causing, fetal hypoxemia and acidemia. At 30 weeks of gestation, the patient was referred because of slightly increased amniotic fluid volume and a non-reactive cardiotocogram. biometry was appropriate for gestational age. Umbilical artery and fetal aortic Doppler findings were normal, whereas diastolic blood flow velocities in the middle cerebral artery were increased and the ductus venosus showed severely abnormal flow velocity waveforms with reversal of flow during atrial contraction. Since other reasons for fetal hypoxemia could be excluded, careful examination of the umbilical cord was performed. traction of the hypercoiled umbilical cord due to its course around the fetal neck and shoulders was suspected. cesarean section confirmed the sonographic findings and fetal blood gases revealed fetal acidemia. This case indicates that investigation of fetal venous blood flow may also help to identify fetal jeopardy due to reasons other than increased placental vascular resistance.- - - - - - - - - - ranking = 13.099758951931keywords = acidemia (Clic here for more details about this article) |
4/148. Hypoketotic hypoglycemic coma in a 21-month-old child.We present the case of a 21-month-old child with hypoketotic hypoglycemic coma. The differential diagnosis initially included metabolic causes versus a toxicologic emergency (unripe ackee fruit poisoning). Using information obtained from the emergency department, the diagnosis was confirmed as the late-onset form of glutaric acidemia type II. This case illustrates the importance of emergency physicians in the diagnosis and management of children with inborn errors of metabolism.- - - - - - - - - - ranking = 322.76341601844keywords = ketotic, acidemia (Clic here for more details about this article) |
5/148. Fetal arrhythmia is not always a simple vagotonia.Fetal arrhythmia (extrasystoles) is usually caused by vagotonia and thought to be innocuous. Four case reports are presented in which arrhythmia accompanied myocardial localization of tuberous sclerosis, fetal sepsis, fetal hypoxia and acidemia.- - - - - - - - - - ranking = 6.5498794759653keywords = acidemia (Clic here for more details about this article) |
6/148. delayed diagnosis of fatal medium-chain acyl-coa dehydrogenase deficiency in a child.A 5-year-old white female presented with coma and died unexpectedly. She had a history of recurrent episodes of febrile illnesses associated with lethargy and coma. Postmortem investigation revealed a fatty liver, leading to a suspicion of inborn error of fatty acid oxidation. The diagnosis of medium-chain acyl-coa dehydrogenase (MCAD) deficiency was suggested by abnormal acylcarnitine profile with increased octanoylcarnitine in the blood, and confirmed by fatty acid oxidation studies and mutation analysis in skin fibroblast cultures. This case emphasizes the need to consider fatty acid oxidation disorders in all children who present with hypoglycemia with absent or mild ketones in the urine and high anion gap metabolic acidosis.- - - - - - - - - - ranking = 0.625keywords = deficiency (Clic here for more details about this article) |
7/148. Graft failure of autologous peripheral blood stem cell transplantation due to acute metabolic acidosis associated with total parenteral nutrition in a patient with relapsed breast cancer.A 32-year-old female had been diagnosed as having relapsed breast cancer and liver metastasis. She underwent high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (PBSCT) with 5.8 x 10(6)/kg CD34 cells. She was supported by total parenteral nutrition (TPN) without vitamins throughout these therapies. Hematopoietic recovery was not observed by day 28 after PBSCT, necessitating a second PBSCT on day 29 using the back-up material of 4.4 x 10(6)/kg CD34 cells. On the next day, she suddenly developed severe metabolic acidosis, heart failure and deep coma. After immediate infusion of thiamine, heart failure and coma rapidly improved. The neutrophil count reached 0.5 x 10(9)/l on day 9 and the platelet count 50 x 10(9)/l on day 15 after the second PBSCT. This is a rare graft failure due to acute metabolic acidosis or thiamine deficiency associated with TPN.- - - - - - - - - - ranking = 0.125keywords = deficiency (Clic here for more details about this article) |
8/148. Severe lymphopenia in tuberculosis. A mere coincidence or a significant association?A variety of infectious agents can cause secondary immunodeficient states. We herein present a one-year-old patient, admitted to the hospital with severe lymphopenia, who was subsequently diagnosed as tuberculosis. After the antituberculosis (anti TB) therapy was started, the clinical condition and the immunologic findings of the patient improved. We have thus concluded that the transient lymphopenia of the patient was due to mycobacterium tuberculosis. We suggest that immunodeficiency should be investigated more often in children with tuberculosis and that further studies will shed light on the pathogenesis of this aspect of the disease.- - - - - - - - - - ranking = 0.125keywords = deficiency (Clic here for more details about this article) |
9/148. Pyroglutamic acidemia: a cause of high anion gap metabolic acidosis.OBJECTIVE: To report four cases of pyroglutamic acidemia in adults causing clinically significant acidosis. DATA SOURCES: patients admitted to the intensive care units of the Alfred Hospital (a quaternary referral center) and Geelong Hospital (a major regional center) with an unexplained high anion gap acidosis. CONCLUSIONS: Pyroglutamic acidemia (5-oxoprolinemia) is a rare cause of high anion gap metabolic acidosis that should be suspected in patients presenting with sepsis, hepatic, and/or renal dysfunction who are receiving drugs such as acetaminophen, flucloxacillin, and vigabatrin after the more common causes of a high anion gap acidosis have been excluded. Should pyroglutamic aciduria be present, known precipitants should be ceased, infection should be managed aggressively, and supportive management should be instituted.- - - - - - - - - - ranking = 39.299276855792keywords = acidemia (Clic here for more details about this article) |
10/148. propionic acidemia in a four-month-old male: a case study and anesthetic implications.propionic acidemia is a rare genetic disease associated with significant medical problems. When patients with this disease present for surgery, their anesthetic must be tailored to meet their special needs. This case report provides information regarding propionic acidemia and its anesthetic management.- - - - - - - - - - ranking = 107.08113266387keywords = propionic acidemia, propionic, acidemia (Clic here for more details about this article) |
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