1/14. Transient hyperinsulinism in an asphyxiated newborn infant with hypoglycemia.We describe a case of transient hyperinsulinism in an asphyxiated newborn infant with hypoglycemia. Although hypoglycemia in birth asphyxiated infants is generally attributed to depletion of glycogen stores, we observed severe hyperinsulinism associated with the hypoglycemia. The hypoglycemia was refractory to high glucose delivery rates (25 mg/kg/min), but responded to a combination of octreotide and diazoxide. At 3 weeks of age, the medications were discontinued and infant was normoglycemic on glucose infusion rates of 5-8 mg/kg/min. Subsequent insulin studies were within normal limits. Transient hyperinsulinism should be considered in asphyxiated infants with protracted hypoglycemia and medications designed to suppress insulin secretion may be useful in refractory cases.- - - - - - - - - - ranking = 1keywords = hyperinsulinism (Clic here for more details about this article) |
2/14. Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage.We describe a 4-month-old Japanese infant with West syndrome with gelastic seizures in clusters. Smile developed around 3 months and gradually increased in frequency and intensity. Positron emission tomography showed hypoperfusion in bilateral hypothalamus. Interictal electroencephalogram (EEG) showed hypsarrythmia. Simultaneous video/EEG monitoring was performed. At first, a smile-like episode developed every several seconds, gradually increased to an abrupt flexion of the neck and extremities, and gradually decreased to a smile-like episode at the end. Ictal EEG revealed desynchronization. ACTH was effective. Smiles are common emotional responses in infancy. However, EEG and neuroimaging should be considered in a case of perinatal asphyxia and delayed development.- - - - - - - - - - ranking = 4.4353811577556E-5keywords = infancy (Clic here for more details about this article) |
3/14. Multiple cystic and focal encephalomalacia in infancy and childhood with brain stem damage.Two cases are described in which damage to the brain stem was associated with extensive necrosis of the cerebral hemisphere. In the first case--a monochorionic twin--there was clear evidence that injury of an ischaemic or hypoxic type had occurred during fetal life and some evidence that an inadequate share of the placental circulation was an important aetiological factor. In the second case death occurred 4 yr after an asphyxial episode at birth. The lesions in the hemispheres and brain stem were extensive, although less than in the first example. The lesions are discussed in the context of our knowledge of the anatomy and physiology of the developing nervous system. Although they cannot as yet be fitted into the concepts of "critical periods" and "vulnerable periods" of development, this is perhaps because observations on human cases are scanty in comparison with the extensive animal studies which have been reported. The lesions are contrasted and compared with those seen in animals.- - - - - - - - - - ranking = 0.00017741524631022keywords = infancy (Clic here for more details about this article) |
4/14. Neonatal hyperinsulinemic hypoglycemia. Two case reports.Neonatal hyperinsulinemic hypoglycemia must be suddenly and appropriately diagnosed and treated to prevent any further neurological dysfunction and damage. Therefore, we report two cases of our observation. Case 1: birth asphyxia, episodes of hypoglycemia after delivery, hyperinsulinism and reduced IGFBP1 blood concentration. Clinical and laboratory pictures resolved progressively after 8 days of life, perfusions were stopped and the neonate began to suck breast milk. Case 2: negative familial and perinatal history. On the 3rd day of life he developed cyanosis, hypotonia, tremors and hypoglycemia. He was discharged with a diagnosis of cerebral injury and neonatal hypoglycemia. At 1 year of life the child showed progressive and heavy neurological damage. The RMN of the brain showed: enlarged ventricles and liquor spaces around the brain, particularly in the frontal region. hyperinsulinism was diagnosed in our Clinic. He began pharmacological treatment with diazoxide that permitted euglycemia. The ammonium was normal and excluded glutamate dehydrogenase deficiency (mutation of GLUD1 gene); diazoxide responsivity excluded mutations of SUR1 and KIR6.3 genes. At 9 years of life he showed motor and language retardation. Newborns with perinatal history of asphyxia may develop transient hyperinsulinism with absent neurological consequences. Persistent hypoglycemic or epileptic-like episodes, in particular on waking up, after meals or during banal infections, must be studied to reveal hyperinsulinism.- - - - - - - - - - ranking = 0.42857142857143keywords = hyperinsulinism (Clic here for more details about this article) |
5/14. thoracoplasty for treatment of asphyxiating thoracic dysplasia in a newborn.In our case report and literature review, we report about a female newborn with severe asphyxiating thoracic dysplasia of the spondylocostal dysostosis classification to whom an expandable thoracoplasty with metal implants offered survival and discharge at home from newborn to infancy.- - - - - - - - - - ranking = 4.4353811577556E-5keywords = infancy (Clic here for more details about this article) |
6/14. subcutaneous fat necrosis of the newborn: be aware of hypercalcaemia.subcutaneous fat necrosis of the newborn is an uncommon, self-limiting panniculitis that usually occurs in full-term infants as a consequence of perinatal asphyxia. The cutaneous involvement may be associated with metabolic complications such as hypoglycaemia, thrombocytopenia, hypertriglyceridemia, anemia and hypercalcaemia. The delayed onset of hypercalcaemia, 1-6 months after the development of the skin manifestations, imposes a prolonged follow-up to avoid its acute toxic effects on cardiovascular and renal systems and the more durable metastatic calcifications.- - - - - - - - - - ranking = 0.0016846718231987keywords = hypoglycaemia (Clic here for more details about this article) |
7/14. Transient hyperinsulinism in asphyxiated newborn infants.hypoglycemia in birth asphyxiated infants is attributed to glycogen depletion. We observed three term AGA (Appropriate for gestational age) infants with birth asphyxia, who developed hyperinsulinemic hypoglycemia postnatally. All had inappropriately high serum insulin concentrations for their blood glucose levels, and needed glucose infusion rates of greater than 8 mg/kg/min for several days to maintain normoglycemia. All infants recovered spontaneously.- - - - - - - - - - ranking = 0.57142857142857keywords = hyperinsulinism (Clic here for more details about this article) |
8/14. Prolonged hyperinsulinism and hypoglycemia. In an asphyxiated, small for gestation infant. case management and literature review.The authors describe a term female, asphyxiated, small for gestational age (SGA) infant with documented hyperinsulinism and hypoglycemia occurring at approximately 45 hours of age. The hypoglycemia was refractory to a high rate glucose infusion and steroid administration but responded to diazoxide. The subsequent hospital course was complicated by right-sided heart failure and sepsis. With the onset of sepsis, a transient hyperglycemia was noted that required intermittent insulin therapy for 10 days. hypoglycemia and hyperinsulinism reemerged and responded to diazoxide therapy. An attempt to discontinue diazoxide at age 6 months was aborted at 2 weeks when hyperinsulinism and hypoglycemia recurred. The infant required diazoxide for 7 more months, then she recovered without having any sequelae. The review of this uncommon hypoglycemia etiology in an SGA and asphyxiated infant and the merits of long-term diazoxide treatment are discussed.- - - - - - - - - - ranking = 1keywords = hyperinsulinism (Clic here for more details about this article) |
9/14. Direct hyperbilirubinemia and hepatic fibrosis: a new presentation of Jeune syndrome (asphyxiating thoracic dystrophy).Jeune syndrome (asphyxiating thoracic dystrophy) is an autosomal recessive skeletal dysplasia with multisystem involvement. The disorder is usually considered lethal, with death resulting from respiratory insufficiency during infancy, or from renal failure during childhood. Hepatic dysfunction, diagnosed on the basis of direct hyperbilirubinemia and hepatic fibrosis, led to early diagnosis of Jeune syndrome in a newborn infant. review of the literature indicates that the specific pathologic lesion (biliary dysgenesis with portal fibrosis and bile duct proliferation) has been observed as an incidental finding at autopsy, but that clinically significant liver dysfunction has not been appreciated as an important manifestation in this disorder. The characteristic histopathologic abnormality has been present in every patient studied, and may advance during infancy; in contrast, clinical liver dysfunction resolves over the first few months of life. Whereas many patients with Jeune syndrome die early, several have survived into adolescence, suggesting that aggressive treatment is appropriate, especially for those with minimal respiratory and renal involvement. Longitudinal evaluation of liver function and pathology in these survivors is needed to define the long-term prognosis.- - - - - - - - - - ranking = 8.8707623155111E-5keywords = infancy (Clic here for more details about this article) |
10/14. Jeune syndrome associated with pancreatic fibrosis.Jeune syndrome, a rare autosomal recessive disorder characterized by skeletal abnormalities of the thorax and extremities, is associated with respiratory distress in infancy, hepatic fibrosis in some instances, and renal failure that may develop later. We report here one additional feature, pancreatic fibrosis. The association of pancreatic fibrosis with Jeune syndrome has been previously described, but it has not been appreciated as a stable and important manifestation of this disorder.- - - - - - - - - - ranking = 4.4353811577556E-5keywords = infancy (Clic here for more details about this article) |
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