Hypoglycemia: A pathophysiologic approach

Senior, Boris; Sadeghi‐Nejad, Abdollah

doi:10.1111/j.1651-2227.1989.tb11227.x

Cited by 20 publications

(9 citation statements)

References 193 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…While the plasma amino acid profile in our patients was not totally typical for protein-calorie malnutrition (21,22), their general hypoaminoacidaemia and deficiency of important substrates of ghicogenesis, especially alanine (23,24), are seen as signs of severe protein-calorie deficiency (20,22,25), possibly due to hypermetabolism and poor nutrient absorption (26,27). We have no evidence that their taurine deficit resulted from dietary amino acid deficiencies as suggested by others (12).…”

Section: Discussionmentioning

confidence: 64%

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

Byrd

Wiltfang²,

Rodeck³

et al. 1993

Clinical Chemistry and Laboratory Medicine

View full text Add to dashboard Cite

Summary:The absolute and relative concentrations of 16 plasma amino acids in 48 mostly dystrophic infants and children (median of age 1 1 A years) with extrahepatic biliary atresia and mainly stable preterminal cirrhosis were compared with those of controls. Patient plasma amino acid data were analysed statistically for diagnostic usefulness and correlated with standard biochemical quantities of liver function and of liver perfusion. In the patients the total amounts of non-essential and essential amino acids were reduced by 19%, and with the same significance (p < 0.0005). Plasma tyrosine was increased (+40%), while taurine (-44%) and branched chain amino acids (+28.8% to -34.7%) were decreased. Methionine values varied widely. In the molar fractional plasma amino acid profile, only alanine, valine, and leucine were decreased, while threonine, methionine, tyrosine, phenylalanine, ornithine, and serine were increased. Discriminate function analysis showed that the plasma amino acid data discriminated 93.8% of the patients from controls. The concentrations of some amino acids in plasma seemed to have been influenced by protein-calorie deficiency in the patients. The valine/tyrosine ratio and the Fischer index (ratio branched chain/aromatic amino acids) were significantly reduced in the patients versus controls (1.54 + 0.55 vs 3.08 + 0.55 and 1.66 + 0.39 vs 3.00 + 0.48). A number of significant correlations (range of r: 0.37 -0.59, p < 0.05, 30-48 data pairs) were calculated between plasma amino acid data and several standard biochemical quantities of liver function. The statistical analyses also showed that the Fischer index began to decrease gradually and linearly early in the progression of liver failure. It is concluded that plasma amino acid data can be useful in the evaluation of the progression of liver failure and possibly of the nutritional status in liver transplant candidates with biliary atresia.

show abstract

Section: Discussionmentioning

confidence: 64%

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

Byrd

Wiltfang²,

Rodeck³

et al. 1993

Clinical Chemistry and Laboratory Medicine

View full text Add to dashboard Cite

show abstract

“…A deficiency or excess of any of these can result in a homeostatic imbalance and lead to hypoglycemia. 1 In this case, the onset of hyperinsulinemic hypoglycemia immediately after birth rules out all primary defects in carbohydrate and lipid metabolism, as well as growth hormone or cortisol deficiency. Because all these disorders result in the depletion or unavailability of liver glycogen, the marked rise in glucose after the administration of glucagon supports this conclusion.…”

Section: T Able 2 B Lood C Hemicalmentioning

confidence: 98%

“…The glucose level remained low despite vigorous treatment with intravenous glucose and pharmacologic agents; the clinical effect of low blood glucose in children depends on the child's age, the availability of alternative fuels, and the duration of the metabolic derangement. 1,2 Many factors can contribute to hypoglycemia in infants and young children. Newborns, particularly those born prematurely, may have a developmental immaturity of the enzymes required for gluconeogenesis and ketogenesis.…”

Section: T Able 2 B Lood C Hemicalmentioning

confidence: 99%

Case 39-2001

Sadeghi‐Nejad¹,

Graeme‐Cook²

2001

N Engl J Med

View full text Add to dashboard Cite

A two-day-old girl was admitted to the hospital because of persistent hypoglycemia.The infant had been born at 34 weeks' gestation to a 35-year-old mother after an uneventful pregnancy. The birth weight was 3510 g. The Apgar score was 4 at one minute and 7 at five minutes. There was no maternal history of gestational or insulin-dependent diabetes mellitus. The blood glucose level, measured at the hospital where the child was born, was less than 10 mg per deciliter (0.6 mmol per liter). An umbilical arterial catheter was inserted, and a bolus of glucose was given, followed by a 22-ml infusion of glucose, for one hour, and then by a 10-ml infusion, given hourly. At three hours, the glucose level was 24 mg per deciliter (1.3 mmol per liter). During the next several hours, repeated boluses and a continuous infusion of glucose were administered; the glucose level ranged between 21 and 34 mg per deciliter (1.2 and 1.9 mmol per liter). At 14 hours, a seizure occurred, and the dose of glucose was increased.The results of laboratory tests (except for glucose and insulin), performed at 12 hours and subsequently, are shown in Tables 1, 2, and 3. During the next few hours, the glucose level varied; a level of approximately 60 mg per deciliter (3.3 mmol per liter) was common, but the level repeatedly dropped to 25 mg per deciliter (1.4 mmol per liter). The infant had six seizures, each of which lasted for 25 to 30 seconds and was characterized by facial grimaces, twitching of the left eye, and a cat-like scream, followed by tonicclonic movements of the arms and legs and lethargy of a few minutes' duration. The oxygen saturation fell to 80 to 90 percent during these episodes. Phenobarbital was administered after the third seizure.Two hours later, a 45-second episode of apnea responded to continuous positive airway pressure. Shortly after birth, the infant had been placed in a hood that provided 40 percent oxygen; between seizures, the oxygen saturation ranged from 95 to 100 percent. Ampicillin and gentamicin were administered. On the second day of life, the infant was transferred to this hospital. An umbilical venous catheter was inserted en route.The infant had two normal siblings; the third sibling had cerebral palsy.The temperature was 36.7°C, the pulse was 144, and the respirations were 60. The mean arterial pressure was 45 mm Hg. The weight was 3460 g.On examination, the infant had a plethoric appearance and was large for her gestational age. The Moro and grasp reflexes were present; there was no sucking reflex.The urine was normal. The phenobarbital level was in the therapeutic range. The insulin level was 105.5 µU per milliliter (757.0 pmol per liter). A thoracic radiograph showed hyperexpanded lungs with possible pneumonia.The glucose infusion was increased to 90 mg per kilogram of body weight per minute at a concentration of 22.5 percent glucose. Ampicillin and gentamicin were continued, and a central venous line was inserted. The ionized calcium level was normal.Hypoglycemia persisted. By the second hospital day, the...

show abstract

“…a 62-86 h fast, accounts for 4.5% and 21.6% of the glucose output, respectively (15). Senior and Sadeghi-Nejad have stressed the fact that glycerol is the most important precursor to new glucose formed in the liver (18) and Wolfe and coworkers speculated that "the physiological role of lipolysis in fasting is the provision of gluconeogenetic substrate" (15). In the GKD patient, glycerol, which in the normal subject is used for either gluconeogenesis or direct esterification of FFA, has to be replaced by glucose.…”

Section: Glycerol Metabolismmentioning

confidence: 99%

“…Children with GKD have a symptomatology very similar to that of ketotic hypoglycaemia. In a review of the pathophysiology and aetiology of hypoglycaemia, Senior and Sadeghi-Nejad include GKD as a cause of ketotic hypoglycaemia (18), but this is not mentioned even in recently updated reference works in paediatrics (32).…”

Section: Diagnosis and Treatmentmentioning

confidence: 99%