Acute and Long-Term Treatment of Children with Maple Syrup Urine Disease

2015 ◽  
pp. 335-347
Author(s):  
U. Wendel
Keyword(s):  
Maple Syrup Urine Disease ◽  
Term Treatment ◽  
Maple Syrup ◽  
Long Term Treatment ◽  
Urine Disease

Outcome of Early and Long-Term Management of Classical Maple Syrup Urine Disease

PEDIATRICS ◽  
1981 ◽  
Vol 68 (6) ◽  
pp. 856-862
Author(s):  
Carol L. Clow ◽  
Theresa M. Reade ◽  
Charles R. Scriver
Keyword(s):  
Acute Phase ◽  
Maple Syrup Urine Disease ◽  
Home Visiting ◽  
Clinical Signs ◽  
Maple Syrup ◽  
Long Term Treatment ◽  
Urine Disease ◽  
The Mean ◽  
Plasma Leucine

The outcome of 8,400 treatment days in the lives of four patients with classical maple syrup urine disease (MSUD) (present ages: 13½, 57½, 71½, and 811½ years) are described. Each diagnosis was made by clinical signs rather than by newborn screening. Acute-phase treatment beginning on the 11th day of life comprised peritoneal dialysis, intravenous lipid, and early intestinal alimentation. Mean age at discharge from hospital was 29 days. There were 16 readmissions to the hospital for the group (89 days, 1.05% treatment days) without any serious neurologic symptoms. The mean level of plasma leucine for the group (for levels below 1 mM) during treatment was 0.42 mM (normal for age range, 0.077 ± 0.021 mM [mean ± SD]). Plasma leucine exceeded 1 mM during 1.02% of treatment days (representing 8.6% of 1,042 measurements. Mean levels of plasma valine and isoleucine were 60% and 70% of the plasma leucine value for the group. Tolerance for dietary leucine did not exceed 620 mg/day in any patient. Somatic growth was normal and the mean current IQ/development quotient (DQ) score is 101 (range 89 to 117); the three oldest patients attend regular schools. A characteristic EEG pattern resembling the teeth of a comb was observed in three patients during the acute phase in the newborn period but not during long-term treatment. These results were obtained in an ambulatory program with home visiting.

scholarly journals 709 OUTCOME OF EARLY AND LONG-TERM MANAGEMENT OF CLASSICAL MAPLE SYRUP URINE DISEASE

1981 ◽  
Vol 15 ◽  
pp. 560-560
Author(s):  
Carol L Clow ◽  
Theresa H Reade ◽  
Charles R Scriver
Keyword(s):  
Maple Syrup Urine Disease ◽  
Maple Syrup ◽  
Urine Disease ◽  
Term Management

Maple Syrup Smell in the Intensive Care Unit

PEDIATRICS ◽  
1982 ◽  
Vol 69 (4) ◽  
pp. 506-507
Author(s):  
Angela E. Lin
Keyword(s):  
Intensive Care ◽  
Maple Syrup Urine Disease ◽  
Neonatal Intensive Care ◽  
Premature Labor ◽  
Baby Girl ◽  
Maple Syrup ◽  
White Mother ◽  
Urine Disease ◽  
Term Management

The article on maple syrup urine disease (Clow CL, Reade TM, Scriver CR: Outcome of early and long-term management of classical maple syrup urine disease. Pediatrics 68:856, 1981) has prompted me to report our neonatal intensive care unit's experience with an "outbreak" of maple syrup odor. Baby girl M. was the 1,730-gm product of a 32-week gestation of a 29-year-old white mother. Pregnancy was uncomplicated except for premature labor.

Long-term metabolic follow-up and clinical outcome of 35 patients with maple syrup urine disease

2017 ◽  
Vol 40 (6) ◽  
pp. 783-792 ◽  
Author(s):  
Marie-Thérèse Abi-Wardé ◽  
Célina Roda ◽  
Jean-Baptiste Arnoux ◽  
Aude Servais ◽  
Florence Habarou ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Maple Syrup Urine Disease ◽  
Maple Syrup ◽  
Urine Disease

scholarly journals Liver Transplantation for Classical Maple Syrup Urine Disease: Long-Term Follow-Up in 37 Patients and Comparative United Network for Organ Sharing Experience

2012 ◽  
Vol 160 (1) ◽  
pp. 116-121.e1 ◽  
Author(s):  
George V. Mazariegos ◽  
D. Holmes Morton ◽  
Rakesh Sindhi ◽  
Kyle Soltys ◽  
Navdeep Nayyar ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Maple Syrup Urine Disease ◽  
Maple Syrup ◽  
Long Term Follow Up ◽  
Urine Disease ◽  
United Network

Hypnotherapy is effective in the long-term treatment of functional dyspepsia

2001 ◽  
Vol 120 (5) ◽  
pp. A115-A115 ◽  
Author(s):  
E CALVERT ◽  
L HOUGHTON ◽  
P COOPER ◽  
P WHORWELL
Keyword(s):  
Functional Dyspepsia ◽  
Term Treatment ◽  
Long Term Treatment

1608: Long-Term Treatment with High Doses of Sildenafil Does Not Up-Regulate the Levels of Phosphodiesterase 5 (Pde5) in the Rat Penis

2004 ◽  
Vol 171 (4S) ◽  
pp. 424-424 ◽  
Author(s):  
Monica G. Ferrini ◽  
Eliane G. Valente ◽  
Jacob Rajfer ◽  
Nestor F. Gonzalez-Cadavid
Keyword(s):  
Term Treatment ◽  
Long Term Treatment ◽  
High Doses ◽  
Phosphodiesterase 5

Long-Term Treatment Outcomes for Heroin Dependence: The 11-Year Follow-Up of the ATOS Study

2013 ◽  
Author(s):  
Christina Marel ◽  
Maree Teesson ◽  
Shane Darke ◽  
Katherine Mills ◽  
Joanne Ross ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Term Treatment ◽  
Heroin Dependence ◽  
Long Term Treatment
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