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1.
Klin Padiatr ; 234(4): 221-227, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35114702

RESUMEN

BACKGROUND/AIM: There have been no studies to date examining the effect of metformin treatment on vitamin B12 status in children and adolescents. In this prospective study, the effects of metformin on blood vitamin B12, serum methylmalonic acid (MMA), homocysteine and holo-transcobalamin-II (holo-TC-II) levels were assessed in pediatric age group. MATERIALS AND METHODS: This prospective study was conducted at the Pediatric Endocrinology and Adolescent Department between January 2017 and March 2019. Metabolic syndrome and polycystic ovary syndrome diagnosed patients with insulin resistance and/or impaired glucose tolerance, patients with type 2 diabetes mellitus (DM) treated with metformin were enrolled in study. Blood vitamin B12, MMA, homocysteine, holo-TC-II levels and hemogram values were evaluated. RESULTS: Twenty-four patients were enrolled in study. Among these, 15 (62.5%) were female. The mean age of patients was 13.7±2.3 (10-19) years. Sixteen patients were diagnosed with metabolic syndrome and 8 patients were type 2 DM. At 6-month follow-up of all patients, there was no statistically significant difference in terms of vitamin B12, homocysteine, MMA and holo-TC-II levels. A 0.6% decline in vitamin B12 levels were revealed. At 12-month follow-up of 11 patients (45.8%) (6 Type 2 DM, 5 metabolic syndrome), no statistically significant difference was determined in vitamin B12, homocysteine, MMA and holo-TC-II levels. There were 6% decline in vitamin B12 levels and 10.9% increase in homocysteine levels, 5.4% decrease was detected in holo-TC-II level. CONCLUSION: Although no significant changes in the serum vitamin B12, homocysteine, MMA or holo-TC-II levels with metformin therapy were detected, long-term prospective studies with high-dose metformin treatment in pediatric population are needed to confirm our results.


Asunto(s)
Diabetes Mellitus Tipo 2 , Síndrome Metabólico , Metformina , Deficiencia de Vitamina B 12 , Adolescente , Niño , Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/tratamiento farmacológico , Femenino , Homocisteína , Humanos , Masculino , Síndrome Metabólico/diagnóstico , Síndrome Metabólico/tratamiento farmacológico , Metformina/efectos adversos , Ácido Metilmalónico , Estudios Prospectivos , Transcobalaminas , Vitamina B 12 , Deficiencia de Vitamina B 12/diagnóstico , Deficiencia de Vitamina B 12/tratamiento farmacológico
2.
Gene ; 528(2): 364-6, 2013 Oct 10.
Artículo en Inglés | MEDLINE | ID: mdl-23892085

RESUMEN

Very early onset Toni-Debré-Fanconi Syndrome, a disorder of proximal renal tubules of the kidney which results in the increased urinary excretion of glucose, amino acids, uric acid, phosphate and bicarbonate, could be the manifestation of various inborn errors. Defects of oxidative phosphorylation are a heterogeneous group of disorders with various clinical presentations. Recently, patients with early liver failure, renal tubulopathy and encephalopathy due to the mutations in the BCS1L gene coding for a structural protein in mitochondrial complex III have been described. Ten-day-old female newborn was referred to our clinic because of intractable acidosis. Physical examination revealed severe hypotonia, and hepatomegaly. The laboratory examinations revealed lactic acidosis, increased blood alanine, alanine aminotransferase and aspartate aminotransferase levels, generalized aminoaciduria and glucosuria. The tubular reabsorption of phosphate was reduced. Because of multisystem involvement, mitochondrial disease was suspected and the mutational analysis of the BCS1L gene revealed homozygous P99L mutation. As the patient was unresponsive to bicarbonate replacement, oral dichloroacetate and peritoneal dialysis, continuous high dose intravenous sodium bicarbonate therapy with a dose up to 1.25 mEq/kg/h was started. The patient got on well until the age of 9 months when she died of sepsis. It was stressed that high dose intravenous continuous sodium bicarbonate therapy could be an alternative treatment option in patients with severe acidosis and renal tubulopathy resistant to dichloroacetate and peritoneal dialysis. Patients with BCS1L mutations should be considered in the differential diagnosis of severe tubulopathy in the newborn period.


Asunto(s)
Complejo III de Transporte de Electrones/genética , Síndrome de Fanconi/diagnóstico , ATPasas Asociadas con Actividades Celulares Diversas , Consanguinidad , Síndrome de Fanconi/genética , Síndrome de Fanconi/terapia , Resultado Fatal , Femenino , Humanos , Recién Nacido , Infecciones por Pseudomonas/diagnóstico , Sepsis/diagnóstico
3.
J Pediatr Endocrinol Metab ; 24(9-10): 843-5, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-22145490

RESUMEN

Total parenteral nutrition (TPN) is a revolution in neonatal intensive care unit (NICU) care, but this therapy is not without problems. A 35-week-old, 1300 g female infant was transferred to our NICU because of bilious vomiting and feeding problems. When enteral feeding was started again, a severe condition similar to the previous one developed. On the 24th day, the patient underwent surgery with a diagnosis of Hirschprung's disease. One week before surgery, the parenteral solutions were composed without vitamins because intravenous vitamin supplements suitable for infants were not available. Thereafter, the patient suffered from severe hypoglycaemia, and sepsis started to develop, accompanied by a large anion gap and metabolic acidosis which is severe lactic acidosis refractory to massive doses of bicarbonate. The acidosis improved significantly when the patient was treated with thiamin. Although TPN is life saving in the NICU, meticulous attention must be paid while treating a patient with TPN, and all possible nutrients should be provided. In this report, a case of a preterm newborn requiring a prolonged period of TPN and complicated by serious lactic acidosis is presented and discussed.


Asunto(s)
Acidosis Láctica/etiología , Enfermedad de Hirschsprung/dietoterapia , Nutrición Parenteral Total/efectos adversos , Deficiencia de Tiamina/etiología , Femenino , Humanos , Lactante , Trastornos de la Nutrición del Lactante/etiología , Recién Nacido , Recien Nacido Prematuro , Índice de Severidad de la Enfermedad
4.
Pediatr Int ; 53(6): 968-73, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21988338

RESUMEN

BACKGROUND: The aim of this study was to establish the frequencies of vitamin D deficiency and insufficiency among healthy children aged 1-16 years and also to determine the factors affecting the levels of vitamin D in Turkey. METHODS: A total of 849 healthy individuals whose ages ranged from 1 to 16 years were included in the study. Serum 25(OH)D, calcium, phosphorous and alkaline phosphatase l levels were measured at the end of the winter period. The approximate daily calcium intake was calculated by using a 1-week diet history. RESULTS: We determined that the prevalence of vitamin D deficiency (<20 ng/mL) was 8% and that of vitamin D insufficiency (20-29 ng/mL) was 25.5% in the population investigated. The average daily intake of calcium was especially low in the >8-year-old age group (<1300 mg/day). CONCLUSION: Vitamin D insufficiency was found to be very common in the population investigated. The daily calcium intake was below the adequate levels especially in school children. Vitamin D supplementation after the first year of life could be beneficial especially for school children and adolescents. The government must develop public policies for the fortification of milk, milk products, and fruit juices with vitamin D.


Asunto(s)
Estaciones del Año , Deficiencia de Vitamina D/epidemiología , Vitamina D/sangre , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Prevalencia , Estudios Retrospectivos , Turquía/epidemiología , Deficiencia de Vitamina D/sangre
5.
Pediatr Hematol Oncol ; 23(4): 277-85, 2006 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-16621768

RESUMEN

In recent years, there have been reports that glutamine support improves immune functions in adult patients with malignancy, but there is a lack of data in children. Oral glutamine support of 4 g/m2/day was given to 21 children with various solid tumors, aged 1-17 years (9.86 +/- 5.38) for all 5 days of a chemotherapy course. The same parameters in another course of the same protocol without glutamine supplementation were considered as controls. There were significant improvements of some nutritional and immunological parameters in the glutamine-supplemented course. Also glutamine seemed to reduce antibiotic necessity. Oral glutamine supplementation could be considered in children with solid tumors receiving chemotherapy.


Asunto(s)
Glutamina/administración & dosificación , Neoplasias/tratamiento farmacológico , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Pesos y Medidas Corporales , Niño , Preescolar , Femenino , Glutamina/uso terapéutico , Humanos , Sistema Inmunológico/efectos de los fármacos , Factores Inmunológicos , Lactante , Masculino , Estado Nutricional/efectos de los fármacos , Nutrición Parenteral , Resultado del Tratamiento
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