1/14. Post-transplant diabetic ketoacidosis--a possible consequence of immunosuppression with calcineurin inhibiting agents: a case series.Post-transplant diabetes mellitus, a complication due to corticosteroids and the calcineurin inhibitors, cyclosporine and tacrolimus (FK506), is commonly regarded as a form of type-2 (adult-onset) diabetes mellitus. diabetic ketoacidosis, which requires relative insulin deficiency to impair fatty acid metabolism, is a complication of type-1 diabetes mellitus. We report three patients who presented with diabetic ketoacidosis post-transplant. All three patients presented with severe hyperglycemia, significant ketosis and metabolic acidosis of variable severity. One patient was a renal transplant recipient on a cyclosporine-based regimen. The other two patients were liver transplant recipients receiving either cyclosporine or tacrolimus-based immunosuppression. Both of the liver transplant recipients were found to have moderate to high serum levels of calcineurin inhibitors on presentation. The liver recipient on cyclosporine (Neoral) had a 4 hour post-dose level of 388 ng/ml and the patient on tacrolimus was found to have a trough level of 21.2 ng/ml. Our experience suggests that post-transplant diabetes mellitus, in association with calcineurin inhibition, may result in ketoacidosis either secondary to relative beta cell dysfunction, peripheral insulin resistance, or a combination of the two effects. Post-transplant diabetes mellitus can be an atypical form of adult-onset diabetes with features of both type I and type II diabetes mellitus.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/14. hypoglycemia and resistance to ketoacidosis in a subject without functional insulin receptors.humans with congenital absence of the islets of langerhans and mice rendered null for the insulin receptor rapidly develop severe hyperglycemia and ketoacidosis and, if untreated, die in the early neonatal period. In contrast, children with homozygous or compound heterozygous mutations of the insulin receptor gene, although hyperglycemic postprandially, survive for many months without developing ketoacidosis. Paradoxically, they often develop hypoglycemia. The rarity of the condition and the difficulties of undertaking metabolic studies in ill infants have limited the physiological information that might explain the clinical features. We studied a boy with Donohue's syndrome who represents a further example of the null phenotype, with two different and novel nonsense mutations in the alpha-subunit of the receptor. He survived for 8 months without developing ketoacidosis, and fasting hypoglycemia was a frequent problem. Despite the complete absence of insulin receptors, evidence for persistent insulin-like effects on fat and liver was seen; fasting plasma beta-hydroxybutyrate and nonesterified fatty acid levels were low, fell further during the early postprandial period, and failed to rise in response to hypoglycemia. The inverse relationships between plasma insulin and insulin-like growth factor-binding protein-1 levels were maintained, suggesting persistent hepatic effects of insulin. GH levels measured over a 6.5-h period were low throughout. Thus, the differences between congenital insulin deficiency vs. insulin receptor deficiency in humans may be explained by persistent insulinomimetic activity of the grossly elevated plasma insulin presumably being mediated through the type 1 insulin-like growth factor receptor. As GH plays a critical role in the regulation of ketogenesis during insulinopenia in humans, but not in rodents, this may contribute to the distinct phenotype of human vs. mouse insulin receptor knockouts.- - - - - - - - - - ranking = 2keywords = deficiency (Clic here for more details about this article) |
3/14. Cerebral venous thrombosis during diabetic ketoacidosis.Neurological deterioration during an episode of diabetic ketoacidosis is usually assumed to be caused by cerebral oedema. We present a case of cerebral venous sinus thrombosis presenting in a similar manner, also associated with severe iron deficiency anaemia. Computed tomography scanning provided the correct diagnosis and allowed institution of anticoagulation with improvement in neurological outcome. neuroimaging should always be performed in suspected cerebral oedema associated with diabetic ketoacidosis in order to exclude other pathologies.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
4/14. Hypothalamopituitary deficiency and precocious puberty following hyperhydration in diabetic ketoacidosis.We report on a 5-year-old child who survived an intracerebral crisis, following ketoacidosis-revealing diabetes (DKA), with visual impairment due to a vascular occipital lesion. Two and 4 months after the initial episode, a unique hypothalamopituitary disorder consisting in GH, ACTH, TSH deficiencies and central precocious puberty, was detected. Cranial magnetic resonance images showed no visible lesion in the hypothalamopituitary region. The most likely hypothesis is the ischemia of hypothalamopituitary and occipital regions following possible cerebral edema after hyperhydration. She survived with low visual acuteness and received a combined replacement therapy for the neuroendocrinological deficiencies. This case emphasizes that the rehydration at the initial period of DKA is critical, especially when risk factors for cerebral edema are present (young age, marked hyponatremia). The neuroendocrinological consequences of acute cerebral edema are rare, but physicians must be attentive in survivors of these accidents.- - - - - - - - - - ranking = 4keywords = deficiency (Clic here for more details about this article) |
5/14. Biotin responsive multiple carboxylase deficiency presenting as diabetic ketoacidosis.multiple carboxylase deficiency (MCD) is a rare inherited metabolic disease of biotin dependency due to deficiency of holocarboxylase synthetase (HCS) or biotinidase deficiency. A 30-month-old female patient who presented with the initial features of diabetic ketoacidosis (severe metabolic acidosis, ketosis, and hyperglycemia), lactic acidemia, moderate hyperammonemia, and generalized organic aciduria is described. Associated symptoms and signs included erythematous skin rashes, alopecia and developmental delay. The patient responded dramatically to treatment with biotin (10 mg/day) showing normalization of clinical symptoms and most biochemical abnormalities. Based on the urine organic profile by gas chromatography/ mass spectrometry (GC/MS), the diagnosis of MCD was made. A plasma tandem mass study confirmed this diagnosis. The biotinase activity in serum was normal, indicating that this was a rare case of late-onset HCS deficiency.- - - - - - - - - - ranking = 8keywords = deficiency (Clic here for more details about this article) |
6/14. Fatal cerebral infarctions in diabetic ketoacidosis in a child with previously unknown heterozygosity for factor v Leiden deficiency.An 11-year-old boy with poorly controlled diabetes had sudden collapse after the development of lower extremity deep venous thrombosis, with fatal cerebral infarctions. He was heterozygous for factor v Leiden deficiency. This case emphasizes the value of cranial imaging after initial resuscitative treatment of neurologic collapse in diabetic ketoacidosis.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
7/14. 'Disappearing diabetes'--resolution of apparent Type 1 diabetes in a patient with AIDS and cytomegalovirus (CMV) infection.A 30-year-old African female with established acquired immunodeficiency syndrome (AIDS) and no history of diabetes, presented in severe diabetic ketoacidosis (DKA). Blood pH was 6.96, serum bicarbonate 5 mmol/l, plasma glucose (PG) 33.0 mmol/l, and urine heavily positive for ketones. She responded to standard treatment and was established on twice-daily subcutaneous insulin. Four months later her insulin was stopped because of hypoglycaemic attacks on small doses. A glucose tolerance test (GTT) at 6 months postdiagnosis was normal (fasting PG 4.4 mmol/l and 2 h PG 7.5 mmol/l), and at 12 months random PG was 4.1 mmol/l and HbA1c 4.3%. The onset of her apparent Type 1 diabetes coincided with an hiv-associated cytomegalovirus (CMV) infection, and a reversible 'CMV insulitis' may be an explanation. Alternatively, the patient may have had what has recently been described as 'atypical diabetes' in African or Afro-Caribbean diabetic patients. Here resolution of diabetes may occur after presentation, though complete return to normoglycaemia after true DKA is very unusual.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
8/14. Profound hypokalemia in diabetic ketoacidosis: a therapeutic challenge.OBJECTIVE: To describe profound hypokalemia in a comatose patient with diabetic ketoacidosis. methods: We present a case report, review the mechanisms for the occurrence of hypokalemia in diabetic ketoacidosis, and discuss its management in the setting of hyperglycemia and hyperosmolality. RESULTS: A 22-year-old woman with a history of type 1 diabetes mellitus was admitted in a comatose state. Laboratory tests revealed a blood glucose level of 747 mg/dL, serum potassium of 1.9 mEq/L, pH of 6.8, and calculated effective serum osmolality of 320 mOsm/kg. She was intubated and resuscitated with intravenously administered fluids. Intravenous administration of vasopressors was necessary for stabilization of the blood pressure. Intravenous infusion of insulin was initiated to control the hyperglycemia, and repletion of total body potassium stores was undertaken. A total of 660 mEq of potassium was administered intravenously during the first 12.5 hours. Despite such aggressive initial repletion of potassium, the patient required 40 to 80 mEq of potassium daily for the next 8 days to increase the serum potassium concentration to normal. CONCLUSION: Profound hypokalemia, an uncommon initial manifestation in patients with diabetic ketoacidosis, is indicative of severe total body potassium deficiency. Under such circumstances, aggressive potassium repletion in a comatose patient must be undertaken during correction of other metabolic abnormalities, including hyperglycemia and hyperosmolality. Intravenously administered insulin should be withheld until the serum potassium concentration is (3)3.3 mEq/L.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
9/14. Wernicke's encephalopathy with hyperemesis and ketoacidosis.BACKGROUND: avitaminosis can result from the acute malnutrition associated with prolonged pregnancy-related hyperemesis. Serious complications may arise from thiamine deficiency under these circumstances. CASE: We review the relevant literature and describe a case with central nervous system involvement presenting with typical manifestations of Wernicke's encephalopathy, apparently precipitated by a combination of hyperemesis gravidarum, diabetic ketoacidosis, and intravenous glucose administration. CONCLUSION: While this life-threatening complication is rare, it is important for all who care for obstetric patients to be aware of it and alert to its development. Early recognition is critical given the need to treat affected women expeditiously to help avoid potentially fatal adverse consequences. Prophylactic thiamine supplementation should be considered in the care of gravidas with hyperemesis.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
10/14. Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.A 4-year-old girl with pyruvate dehydrogenase deficiency, static encephalopathy, and seizure disorder treated with the ketogenic diet presented in severe diabetic ketoacidosis. Pyruvate dehydrogenase deficiency is a rare genetic defect of mitochondrial energy metabolism that leads to inefficient glucose use and lactic acidosis. The ketogenic diet provides the brain with an alternate fuel source, but its implementation opposes traditional diabetes management. Faced with this therapeutic dilemma, we aimed to maintain ketosis without compromising safety to optimize neurologic function and quality of life. This is the first report, to our knowledge, of a child simultaneously treated with the ketogenic diet and exogenous insulin. A 28-month follow-up revealed excellent glycemic control, improved activity level, significant developmental achievements, and, perhaps most striking, catch-up linear growth from < 5th percentile to the 50th percentile. Her progress to date indicates that diabetes does not preclude use of the ketogenic diet.- - - - - - - - - - ranking = 6keywords = deficiency (Clic here for more details about this article) |
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