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,,>> low ammonia can occur when kids are acidotic.....

When you say Acidotic?? What kind of acid?? Lactic?

He did not have a lacate for some reason.Tell me more about the acidotic/low ammonia level.

His creatine was .2 normal and the ref. range is .20 - .40

WBC were 14.5 normal and ref. range is 6 - 17.50

Thank you VERY much for helping out!

Krystena

Re: lab test

Krystena,The high platelets are usually from infection or inflammation, the high BUN/creatinine ratio can occur in dehydration, low ammonia can occur when kids are acidotic. Evan also had low sodium when his ammonia was low and he was sick and acidoitc. High lymphs usually mean it is a viral infection. The reference range for anion gap, RDW, and albumin seem different than the ones that I have seen on pediatric labs. What was his white blood cell count? What was his creatinine?Krystena s wrote:

I took Carsen to the dr today since I was worried due to him only urinating 1 time in 16 hrs.

He did finally pee but he threw up twice while there.

Not done it since ????

Should I be concerned about the following labs?

High Platelets 568 (150-400)

High ALK phos 298 (40-230)

High Lymp 69 (15-40)

Low Sodium 133 (139-146)

High BUN/CREAT 40 (6-25)

LOW ANION GAP 5 (10-18)

LOW ALBUMIN 3.6 (3.8-5.40)

LOW RDW 11.3 (11.5-14.5)

LOW Band 1 (2-21)

Get this.....his ammonia was LOW @ 6 (19-60)

How is his BUN/CREAT high and not his BUN OR CREATININE????Please contact mito-owner with any problems or questions.

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Krystena,

Did they check urine for ketones? My son showed really high ketones

during that episode and ketones are acidic. It is very common for kids

with mito to go into ketosis when they are sick. Do you have any WBC

testing on Carsen when he was not sick? Carsen is very young and WBC

levels are higher in infants, but we always know with Evan that anytime

his WBC count is too far from his norm to watch for illness and

ketosis.

Evan's labs from that day showed sodium 136 and ammonia 9. In urine

his ketones were 100 times the top of the reference range. He wasn't

even that sick at the time.

Here is some good info--

13.28.02 Differential Diagnosis of an Infant or Child Presenting

with Acidosis and/or Ketosis

Overview:

An infant or child presenting with acidosis and/or ketosis may have

an inborn error of metabolism or simply a physiologic response to

fasting,

vomiting or diet. By looking at the pattern of laboratory test findings

some possible explanations for the patient's condition can be

identified.

Presenting groups:

(1) metabolic acidosis with ketosis

(2) metabolic acidosis without ketosis

(3) ketosis without metabolic acidosis

Metabolic acidosis:

(1) pH < 7.30

(2) PCO2 < 30 mm Hg

(3) serum bicarbonate < 15 mmol/L (mEq/L)

Ketosis: ketonuria

increase in ketone bodies

Metabolic Acidosis with Ketosis

Glucose Levels

Additional Testing

Diagnostic Considerations

increased

high ammonia

ketolytic defects

increased

normal or low ammonia

branched chain organic acidurias

normal

high lactate

congenital lactic acidosis

respiratory chain disorders

organic acidurias

normal

normal lactate

maple syrup urine disease

late onset form

ketolytic defects

organic acidurias

short chain acylCoA dehydrogenase

decreased

high lactate often with hepatomegaly

gluconeogenesis defects

respiratory chain defects

decreased

normal lactate

maple syrup urine disease

late onset form

branched chain organic acidurias

acetoacetyl CoA thiolase

adrenal insufficiency

Metabolic Acidosis without Ketosis

Lactate

Glucose

Diagnostic Considerations

increased

normal

pyruvate dehydrogenase deficiency

increased

decreased

fatty acid oxidation disorders

hydroxymethylglutaryl-CoA lyase

glucose 6 phosphatase deficiency

fructose biphosphatase deficiency

normal

normal

renal tubular acidosis I and II

pyroglutamic aciduria

Ketosis without Metabolic Acidosis

Glucose Levels

Additional Testing

Diagnostic Considerations

normal glucose

intermittent ketosis

fasting

catabolism

recurrent vomiting (physiologic ketonuria)

short chain acyl CoA dehydrogenase

hydroxy short chain acyl dehydrogenase

normal glucose

permanent ketosis

medium chain triglyceride enriched diet and ketotic diet

ketolytic defects

fasting hypoglycemia

with hepatomegaly and post-prandial hyperlactacidemia

glycogenosis type III

glycogen synthetase deficiency

fasting hypoglycemia

without hepatomegaly

recurrrent ketotic hypoglycemia

hypoglycemia due to adrenal disorders

hydroxy short chain acyl dehydrogenase

medium chain acyl CoA dehydrogenase

glycogen synthetase deficiency

ketolytic defects

ketolytic defects: oxoacyltransferase deficiency

thiolase deficiency

branched chain organic acidurias: methylmalonic acid (MMA)

propionic acid (PA)

isovaleric acid (IVA)

congenital lactic acidosis: pyruvate carboxylase (PC) deficiency

multiple carboxylase deficiencies

ketoglutarate dehydrogenase (KGDH) deficiency

lipoamide oxidoreductase (E3) deficiency

gluconeogenesis defects: fructose diphosphatase (FDP) deficiency

glucose 6 phosphatase (G6P) deficiency

glycogen synthase deficiency

organic acidurias: 3-hydroxyisobutyric aciduria

others

References:

Saudubray J-M

Charpentier C. Chapter 66: Clinical phenotypes: Diagnosis/algorithms.

pages 1327-1403 (Table 66-7 page 1342

Table 66-9 page 1344). IN: Scriver CR

Beaudet AL

et al (editors). The Metabolic & Molecular Bases of Inherited

Disease

Eighth Edition. McGraw-Hill. 2001

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