Tuesday, May 20, 2008

116 - hypoglycemia causes list

Recently there was a question in the medical sciences paper of the UPSC prelims examination conducted on 18 may 2008 regarding hypoglycemia. Let us look into this question. The question was as follows :

Which of the following conditions is associated with hypoglycemia?

a- VIPoma

b- Phaeochromocytoma

c- Fibrosarcoma

d- Glucaganoma

The answer is : c . fibrosarcoma . surprised ? I was too , but this is the answer , because all the other three do not cause hypoglycemia and only fibrosarcoma causes hypoglycemia. Check out the tumors that cause hypoglycemia and also all the other causes that cause hypoglycemia. I know it is an exhaustive list but read once so that atleast a few of them will remain in ur brain .

Extrapancreatic Tumors

  • Mechanisms
    • Increased utilization by large or active tumor
    • Cachexia and depletion of glycogen reserves
    • Insulin-like peptides, especially IGF-II (Doege-Potter syndrome)
    • Inhibited hepatic gluconeogenesis or glycogenolysis
    • Blunted counterregulatory hormone responses
    • Autoimmune (see below)
  • Tumors associated with hypoglycemia
    • Benign and malignant mesothelioma
    • Neurofibroma
    • Fibrosarcoma
    • Rhabdomyosarcoma
    • Leiomyosarcoma
    • Liposarcoma
    • Sarcomatous dysembryoma
    • Reticulum cell sarcoma
    • Hemangiopericytoma
    • Spindle cell sarcoma
    • Pseudomyxoma peritonei (Rosenfeld syndrome)
    • Hepatoma (Nadler Wolfer Elliott syndrome)
    • Adrenal carcinoma (Anderson syndrome)
    • Non-islet pancreatic carcinoma
    • Hypernephroma
    • Adenocarcinomas of prostate, colon, bile duct, breast, stomach
    • Simple fibroma
    • Leukemia
    • Lymphomas and Hodgkin's disease
    • Multiple myeloma
    • Wilms' tumor
    • Apudoma
    • Carcinoid tumors
    • Pheochromocytoma (especially after removal)
    • Melanoma
    • Teratoma
    • Neuroblastoma
    • Paraganglioma

Causes of Transient Neonatal Hypoglycemia

  • Prematurity
  • Intrauterine growth retardation
    • Smaller of twins
  • Severe infant respiratory distress syndrome
  • Maternal toxemia
  • Perinatal asphyxia
  • Critical illness

Starvation, Inadequate Intake Or Absorption

  • Fasting, e.g. preoperative
  • Severe malnutrition
  • Kwashiorkor
  • Anorexia nervosa
  • Pyloric stenosis
  • Vitamin B6 (pyridoxine) deficiency
  • Biotin deficiency
  • Hypomagnesemia
  • Diarrhea
  • Renal glycosuria
  • Combined intestinal monosaccharide transport defect

Major Organ Failure & Critical Illness

  • Congestive heart failure
  • Cyanotic congenital heart disease
  • Renal failure, especially chronic
    • Dialysis
  • Central nervous system disorders
    • Head trauma
    • Stroke
    • Severe encephalopathies
    • Hypothalamic tumor
  • Liver disorders
    • Severe acute hepatitis due to infection or toxins
    • Chronic liver failure
    • Reye's syndrome
    • HELLP syndrome (hemolysis, elevated liver enzymes, thrombocytopenia)
  • Chronic pancreatitis
  • Sepsis
  • Hypothermia
  • Malignant hyperthermia

Hyperinsulinism

  • Hypoglycemia due to endogenous insulin
    • Congenital hyperinsulinism
      • Transient neonatal hyperinsulinism
        • Due to maternal factors
          • Maternal diabetes
          • Erythroblastosis fetalis
          • Excessive intravenous glucose during labor
          • Maternal sulfonylurea
          • Sympathomimetic tocolytic agents (e.g. ritodrine)
        • Due to infant factors
          • Intrauterine growth retardation
          • Perinatal asphyxia
          • Idiopathic transient hyperinsulinism
        • Iatrogenic
          • Malposition of umbilical catheter
      • Focal congenital hyperinsulinism
        • Paternal SUR1 mutation with clonal loss of heterozygosity of 11p15
        • Paternal Kir6.2 mutation with clonal loss of heterozygosity of 11p15
      • Diffuse congenital hyperinsulinism
        • Autosomal recessive forms
          • SUR1 mutations
          • Kir6.2 mutations
        • Autosomal dominant forms
          • Glucokinase gain-of-function mutations
          • Hyperammonemic hyperinsulinism (glutamate dehydrogenase gain-of-function mutations)
          • Loss of heterozygosity of 11p15 (Beckwith-Wiedemann syndrome)
      • Donohue syndrome (leprechaunism)
    • Acquired tumors and hyperplasias of pancreatic beta cells
      • Islet cell adenoma
      • Islet cell carcinoma
      • Multiple endocrine adenomatosis syndrome
      • Pluriglandular syndrome of islet, pituitary, parathyroid hyperplasia
    • Autoimmune insulin syndrome
    • Reactive hypoglycemia (postprandial hypoglycemia syndrome)
    • Dumping syndrome
  • Drug induced hyperinsulinism
    • Oral hypoglycemic agents, especially Sulfonylureas
      • Treatment of diabetes
      • Treatment of diabetes insipidus
      • Ingestion by child
    • Aspirin overdose
    • Acetaminophen overdose
    • Pentamidine
    • Quinine
    • Disopyramide
    • Bordetella pertussis vaccine or infection
  • Hypoglycemia due to exogenous (injected) insulin
    • Insulin self-injected for treatment of diabetes
      • Excessive insulin dosage or accelerated absorption
      • Excessive activity
      • Inadequate food or delayed or decreased absorption
      • Alcohol
      • Drugs which contribute synergistically
      • Development of concurrent disease
      • Acquired endocrinopathies
      • Renal, cardiac or liver failure
    • Factitious & malicious insulin injection
      • Insulin self-injected surreptitiously (e.g., Munchausen syndrome)
      • Munchausen by proxy
    • Insulin tolerance test for pituitary or adrenergic response assessment
    • Treatment of hyperkalemia
    • Insulin potentiation treatment (cancer quackery)
    • Insulin-induced coma for depression or psychosis treatment (insulin shock)

Hormone Deficiencies

  • Cortisol
    • Addison's disease (acquired adrenal destruction)
    • ACTH deficiency
    • ACTH unresponsiveness
    • Congenital adrenal hypoplasia
    • Congenital adrenal hyperplasia
  • Growth hormone
    • Isolated growth hormone deficiency
    • Laron dwarfism (GH unresponsiveness)
  • Epinephrine and catecholamines
    • Adrenomedullary unresponsiveness
  • Glucagon
  • Combined deficiencies
    • Congenital hypopituitarism (various causes)
    • Psychosocial deprivation syndrome (hypothalamic)
    • Thyroid hormone (depresses GH and ACTH)

Metabolic Defects

  • Defective glycogenolysis or glycogen accumulation
    • Glucose-6-phosphatase deficiency (glycogenosis type I, von Gierke dis)
    • Pseudoglycogenosis type I
    • Amylo-1,6-glucosidase (debrancher) deficiency (glycogenosis type III)
    • Hepatic phosphorylase deficiency (glycogenosis type VI)
    • Hepatic phosphorylase kinase deficiency (glycogenosis type IXb)
    • Glycogen synthase deficiency (glycogenosis type 0)
  • Galactose-1-phosphate uridyl transferase deficiency (galactosemia)
  • Defects of gluconeogenesis or substrate supply
    • Fructose-1,6-diphosphatase deficiency
    • Isovaleric acidemia
    • Hypoalaninemia
    • Phosphoenolpyruvate carboxykinase deficiency
    • Pyruvate carboxylase deficiency (Leigh syndrome)
    • Fructose-1-phosphate aldolase deficiency
  • Defects of mitochondrial beta-oxidation and fatty acid metabolism
    • Systemic carnitine deficiencies
      • Enzyme deficiencies
      • Carnitine palmitoyltransferase I
      • Carnitine palmitoyltransferase II
      • Carnitine acyltransferase
    • Butyryl CoA dehydrogenase
    • Hydroxymethylglutaryl CoA lyase
    • Methylcrotonyl CoA carboxylase
    • Medium chain acyl CoA dehydrogenase
    • Short chain acyl CoA dehydrogenase
    • Long chain acyl CoA dehydrogenase
    • Multiple acyl CoA dehydrogenase (glutaric aciduria type II)
    • Long-chain 3-hydroxyacyl-CoA dehydrogenase
    • Short-chain 3-hydroxyacyl CoA dehydrogenase
    • Carnitine/acylcarnitine translocase
  • Enoyl CoA hydratase
  • Ketothiolase
  • Succinyl CoA:acetoacetate transferase
  • Defects of amino acid metabolism
    • Maple syrup urine disease
    • Methylmalonic acidemia
    • [[hydroxy-methylglutaric aciduria]]
    • Tyrosinosis
    • Phenylketonuria
    • Propionic acidemia
  • Miscellaneous metabolic defects
    • Defective type I glucose transporter in brain
    • Methylglutaconic aciduria
    • Sucrosuria
    • Glycerol intolerance
    • Rare variants of galactose intolerance
    • Other rare or poorly defined congenital metabolic defects

Drugs And Toxic substances

  • Insulin, antidiabetic agents (see above)
  • Drugs associated with hypoglycemia alone
    • Ethanol
    • Beta blockers, e.g. propranolol
    • Salicylates
    • Acetaminophen
    • Acetazolamide
    • Aluminium hydroxide
    • Chloroquine
    • Chlorpromazine
    • Cimetidine, ranitidine
    • Diphenhydramine, other antihistamines
    • Propoxyphene
    • Disopyramide
    • Doxepin
    • Golytely (in distal intestinal obstruction syndrome of cystic fibrosis)
    • Imipramine
    • Indometacin
    • Isoxsuprine
    • Insulin-like growth factor 1
    • Lidocaine
    • Lithium
    • Pentamidine
    • Propranolol, nadolol, labetalol, metoprolol
    • Orphenadrine
    • Oxytetracycline
    • Quinine, quinidine
    • Perhexiline
    • Ritodrine
    • Haloperidol
    • Chelating agents (BAL and EDTA)
    • THAM
    • Colchicine
    • Para amino benzoic acid, para-amino salicylic acid
    • Cholestyramine added during glucocorticoid therapy (reduces absorption)
  • Drugs which lower glucose in diabetics
    • Enalapril and captopril
    • Coumarin
    • Phenylbutazone
    • Antihistamines
    • Sulfa antibiotics, including SMX/TMP (especially in renal failure)
    • Monoamine oxidase inhibitors
    • Medicines not available in U.S.
      • Azapropazone, buformin, carbutamide, cibenzoline, cycloheptolamide, glibornuride, gliclazide, mebanazine, metahexamide, perhexiline, sulphadimidine, sulphaphenazole, Nigerian cow urine medicine
  • Environmental toxins
    • Amanita phalloides toxin
    • Abractylis gummifera (Mediterranean plant)
    • Hypoglycin from unripe Ackee fruit (Jamaican vomiting illness)
    • Parathion
    • Vacor rat poison

Idiopathic And Miscellaneous

  • Ketotic hypoglycemia
    • Identifiable hormone and enzyme deficiencies
  • Idiopathic
    • Idiopathic hypoglycemias, etiologies undetermined
    • Autoimmune
    • Antibodies to insulin
    • Antibodies to insulin receptor
    • Stimulating antibodies to islet cells
    • Thyrotoxicosis (extremely rare)
    • Infection
      • Mumps
      • Varicella
      • Pertussis
      • Measles
      • Malaria
  • Extreme exercise
  • Artifactual
    • In vitro glucose consumption after blood drawing
    • Leukemic WBC's may consume glucose in vitro
    • Polycythemia of infancy (RBCs consume glucose in vitro)
    • Inaccuracies of blood drop strips
    • Inherent variation inaccuracy at low end
    • Inadequate drop
    • Excessive wiping
    • Short time interval

Reactive, Functional, Postprandial, Etc.

  • Prediabetes (both categories controversial & may not be valid)
    • Juvenile diabetes (rare, anecdotal reports)
    • Adult onset diabetes (in early stages)
  • After intravenous glucose load
  • Abrupt discontinuation of parenteral nutrition or i.v. glucose
  • After exchange transfusion with ACD preserved blood in neonate
  • Alimentary (rapid jejunal emptying with exaggerated insulin response)
  • Post fundoplication for gastroesophageal reflux
  • Post gastrectomy dumping syndrome
  • Short bowel syndrome
  • Idiopathic gastrointestinal motility disturbance
  • Alternate day growth hormone therapy
  • Idiopathic reactive or postprandial hypoglycemia (hypoglycemia documented at time of symptoms: rare)
  • Idiopathic postprandial syndrome (hypoglycemia never documented: common)

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