Andreina Cattani O

Recambio óseo en nodrizas adolescentes: evaluación al término de la gestación, lactancia y postdestete

BACKGROUND There is paucity of information about bone metabolism during pregnancy or breast feeding in teenagers. AIM To study bone turnover at the end of pregnancy and during breast feeding in teenagers and correlate it with environmental, hormonal or nutritional variables. SUBJECTS AND METHODS Thirty teenagers during their breast feeding period after a first pregnancy and 30 nulliparous girls matched for age, age of menarche and body mass index were assessed three weeks after delivery (period 1), at six months of breast feeding (period 2) and one year after the lactating period (period 3). Calcium intake and plasma calcium, phosphorus, alkaline phosphatases, parathormone, estradiol and prolactin were measured. Calcium, creatinine and hydroxyproline were also measured in a morning urine sample. RESULTS Lactating and control girls were aged 16.3 +/- 0.8 and 16.1 +/- 0.7 years old respectively. Calcium intake in lactating and control girls was 798 +/- 421 and 640 +/- 346 g/day respectively in period 1, 612 +/- 352 and 592 +/- 309 mg/day in period 2 and 495 +/- 180 and 456 +/- 157 g/day in period 3. During periods 1 and 2, lactating girls had higher alkaline phosphatases (161 +/- 37 compared to 119 +/- 28 U/l and 149 +/- 37 compared to 106 +/- 23 U/l), parathormone (4.3 +/- 2.6 compared to 2.8 +/- 0.8 ng/dl and 3.6 +/- 1.6 compared to 3.0 +/- 0.9 ng/dl) and urinary hydroxyproline (95 +/- 16 compared to 63 +/- 15 mg/g creatinine and 84 +/- 19 compared to 59 +/- 15 mg/g creatinine). No differences were observed in period 3. No correlation between bone turnover variables, body mass index or hormonal parameters, was observed. CONCLUSIONS In teenagers, there is an increase in bone turnover at the end of pregnancy, that persists during the lactating period. These changes are not related to nutritional or hormonal variables.

Enfermedad de Basedow Graves en pacientes pediátricos

Graves-Basedow disease in pediatric patients Objective: Determine differences in the epidemiology, clinical features and diagnosis of Graves-Basedow disease (GBD) in prepubertal and pubertal patients. Method: Retrospective study analyzing medical records of 38 patients with GBD at Pontificia Universidad Catolica de Chile between 1992-2007. Statistical analysis was performed with non parametric test of Mann-Whitney U and proportions difference with Fisher Test (SPSS 10.0 for Windows and Graphpad Prism 4). Results: 21 patients were prepubertal and 17 were pubertal, with ages between 3 and 15,9 years. There were more girls than boys in both groups (5:2 and 15:2, respectively; p = 0.2). The most common clinical presentations were diffuse goiter, hyperactivity, frequent bowel movements, insomnia and heat intolerance. The prepubertal group had a taller stature (+2.4 SDS) compared with the pubertal group (+0.2 SDS; p = 0.03) and the most frequent ocular manifestation was exophthalmus in both groups. Conclusions: We did not find any differences in the clinical presentations of Graves-Basedow disease among prepubertal and pubertal patients. Neuropsychiatric symptoms such as hyperactivity and insomnia, together with tall stature are common features in children with GBD. (Key words: Hyperthyroidism, thyroid, Graves-Basedow, autoinmunity, hyperactivity). Rev Chil Pediatr 2008; 79 (1): 26-35

Hiperinsulinismo neonatal persistente: análisis del diagnóstico diferencial a propósito de dos casos clínicos

Persistent neonatal hyperinsulinism is the most common cause of refractory hypoglycemia during the first year of life. Inadequate insulin secretion is associated to mutations of four different genes, that can be diagnosed to orient patient management. We report two patients: a female newborn that presented a hypoglycemia of 16 mg/dl two hours after birth, was subjected to a subtotal pancreatectomy that did not correct hypoglycemia, requiring a total pancreatectomy. Pathological study of the pancreas showed a focal adenomatous hyperplasia. At the present time, she is three years of age and maintains euglycemia with fractionated feeding. A male newborn that had seizures at 28 hours of life and a hypoglycemia of 15 mg/dl was detected. He was also subjected to a subtotal pancreatectomy, that did not correct hypoglycemia and had to be extended to a total pancreatectomy. At the present time, he is 3 years and 11 months of age and has a normal psychomotor development (Rev Med Chile 2004; 132: 995-1000). (Key Words: Endogenous hyperinsulism; Hyperinsulism; Hypoglicemia)Persistent neonatal hyperinsulinism is the most common cause of refractory hypoglycemia during the first year of life. Inadequate insulin secretion is associated to mutations of four different genes, that can be diagnosed to orient patient management. We report two patients: a female newborn that presented a hypoglycemia of 16 mg/dl two hours after birth, was subjected to a subtotal pancreatectomy that did not correct hypoglycemia, requiring a total pancreatectomy. Pathological study of the pancreas showed a focal adenomatous hyperplasia. At the present time, she is three years of age and maintains euglycemia with fractionated feeding. A male newborn that had seizures at 28 hours of life and a hypoglycemia of 15 mg/dl was detected. He was also subjected to a subtotal pancreatectomy, that did not correct hypoglycemia and bad to be extended to a total pancreatectomy. At the present time, he is 3 years and 11 months of age and has a normal psychomotor development.

Pubertad precoz periférica por tumor ovárico de las células de la granulosa juvenil, con manifestaciones iso y heterosexuales en una niña de 6 años: Report of one case

A six years old girl consulted due to mammary development. On physical examination, clitoris enlargement and a tumor localized in the abdominal-pelvic region were observed. Hormonal study disclosed elevated testosterone and estradiol levels. On exploratory laparotomy, a right ovarian tumor was observed and a right salpingo-oophorectomy was performed. The contemporary biopsy informed a disgerminoma, leading to a surgical staging of the tumor. The definitive pathological diagnosis was a juvenile granular cell tumor, limited to the ovary. In the postoperative period, estradiol and testosterone levels returned to normal values and the pseudopuberty reverted. The patient did not receive adjuvant treatment and after three years of follow up, there is no evidence of tumor recidivism

Diabetes Insípida en Pediatría: Serie Clínica y Revisión de la Literatura

Introduccion: La diabetes insipida (DI) se caracteriza por poliuria y polidipsia, secundario a una disminucion de la secrecion o accion de la hormona antidiuretica. Su diagnostico precoz es fundamental. Objetivo: Describir las caracteristicas clinicas, radiologicas y tratamiento inicial de una serie de ocho pacientes con DI. Diseno: Estudio descriptivo-restrospectivo. Universo: Pacientes con DI evaluados en la Universidad Catolica de Chile y Hospital Dr. Sotero del Rio entre 1998-2008. Pacientes y Metodos: Desde la ficha clinica se analizaron variables clinicas, epidemiologicas, bioquimicas e imagenes. Se determino Osmolaridad serica (OsmS) y urinaria (OsmU). Se considero DI si la OsmS > 300 mOsm con OsmU 50% o > 600 mOsm, de los contrario se clasifico como nefrogenica (DIN). Resultados: Se reclutaron ocho pacientes con DI (5 varones), fueron DIN 3/8. El motivo de consulta fue: polidipsia-poliuria (5/8), hipertermia (2/8) y talla baja (1/8). La RNM mostro lesion intracraneana en todos los pacientes con DIC: nodulo hipofisiario, aracnoidocele selar, Histiocitosis X, germinoma y un paciente sin senal de neurohipofisis. Los sujetos con DIC usaron DDAVP inhalatoria (4) y oral (1). Los sujetos con DIN usaron hidroclorotiazida. Conclusion: Polidipsia, poliuria, hipertermia con hipernatremia y falla de medro en lactantes son sugerentes de DI. La prueba de deprivacion hidrica es fundamental en la diferenciacion de DIC y DIN. La RNM cerebral es una herramienta diagnostica imprescindible en la DIC. El tratamiento de estos pacientes debe ser multidiciplinario interactuando pediatra, nefrologo, endocrinologo y nutricionista.Separate mechanisms supply and eliminate water to maintain fluid homeostasis. A variety of sensors modulate thirst to drive water-seeking behavior and water intake. The elimination of water is achieved via hormonal control of the kidney. To ensure that a healthy fluid balance is maintained, the mechanisms that accrue water can compensate for defects in the mechanisms that eliminate water and vice versa. Clinical problems arise when the capacity for this compensation is exceeded or when such compensation becomes unduly demanding.

Insuficiencia suprarrenal primaria de etiología autoinmune: Dos casos clínicos

Introduccion: La insuficiencia suprarrenal primaria (ISRP) es producida por diversas etiologias, congenitas o adquiridas. Su sintomatologia es poco especifica, exigiendo un alto indice de sospecha. Objetivo: Presentacion de dos casos clinicos y revision de ISRP. Casos Clinicos: Dos ninos de 9 y 6 anos, ambos con astenia y adinamia, dolor abdominal, baja de peso y vomitos, con avidez por la sal, con mal estado general, lipotimia, hiperpigmentacion de piel y mucosas. Se confirmo el diagnostico de ISRP con compromiso en la secrecion de cortisol, y mineralocorticoides. Ambos tuvieron anticuerpos antiadrenales positivos. Conclusiones: La ISRP es poco frecuente en pediatria, sin embargo, es potencialmente de riesgo vital, de manera que reconocer precozmente sus sintomas permitira realizar un diagnostico y tratamiento oportuno