Table 2.
SELECTED REPORTS OF HYPERMANGANESEMIA IN PARENTERALLY FED CHILDREN AND ADOLESCENT PATIENTS
| Reference | n | Age Range | Disease | Mn daily dose and duration of intake | Laboratory Findings | Radiological Findings | Clinical Symptoms |
|---|---|---|---|---|---|---|---|
|
| |||||||
| Fell et al. (1992) | 57 | 1–162 mo | Numerous 46/57 w/o cholestasis | wt<10 Kg-1 μmol/Kg wt>10 Kg-0.8 μmol/Kg (median PN duration 1.25 mo) |
45/57 Incr WB Mn 615–1840 nmol/L (normal range 72–210 nmol/L); Higher Mn associated w/cholestasis. | 2 selected cases of abnormal MRI Incr MRI signal (globus pallidus, nuclei subthalamic) |
2 pts w/dystonic limb movements and abnormal posturing; 4 pts died |
| Reynolds et al. (1994) | 1 | 7 mo | SBS, PN associated liver disease | 44–55 μg/Kg (PN duration 17 mo) | Incr WB Mn 1740 nmol/L (normal range 73–210 nmol/L). | MRI abnormalities compatible w/deposition of a paramagnetic metal | Developmental delay, abnormal dystonic movements of both arms, microcephaly |
| 53 | NR | numerous | 44–55 μg/Kg (PN duration > 6 wk) | Incr WB Mn in 35/53 w/evidence of cholestatic liver disease > 360 nmo/L | NR | NR | |
| Ono et al. (1995) | 1 | 5 y | Intractable diarrhea and recurrent pancreatitis | 10 μmol (PN duration > 2y) | Incr WB Mn 135 μg/dL (normal range 14.6 + 4.7), 5 mo after d/c Mn, decr WB Mn to 20 μg/dL | Incr MRI signal (basal ganglia) | Nonspecific headache and amnesia |
| Komaki et al. (1998) | 1 | 2 y | Intractable vomiting and diarrhea | 82 μg/Kg (PN duration 14 mo) | Incr WB Mn 9.7 μg/dL (normal range < 2.5 μg/dL), 3 mo after d/c Mn, decr WB Mn to normal levels | Incr MRI signal (globus pallidus, thalamus), 3 mo after d/c Mn MRI abnormalities regressed | Tremor and generalized tonic seizures, psychomotor retardation, hyperactivity; 3 mo after d/c Mn, seizures and tremor completely disappeared |
| Hsieh et al. (2007) | 1 | 10 y | SBS | 8 μg/kg (PN duration 3 mo) | Incr WB Mn 3.7 μg/dL (normal range 0.4 – 1.4 μg/L) | Incr MRI signal (basal ganglia) | Tonic-clonic seizures |
| Iinuma et al. (2003) | 7 | 41–249 mo (mean 93 mo) | numerous | 15.7–91.5 μg/kg (PN duration 18–37 mo) | 4/7 Incr WB Mn 3.1–5.1 μg/dL (normal range 1.8–2.4) | 6/7 pts incr MRI signal, 12 mo after d/c Mn, 5/7 pts decr MRI signals | No symptoms |
Conc-concentration, d/c - discontinuation, Decr - decreased, GI - gastrointestinal, h-hours, Incr - increased, mo - month, MRI - magnetic resonance imaging, NR - not reported, PN - parenteral nutrition, pts-patients, RBC - red blood cells, SBS - short-bowel disease, suppl. - supplementation, wk - weeks, w/o - without, w/- with, wt - weight, y-years.