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nesidioblastosis/seizures

L'enllaç es desa al porta-retalls
Pàgina 1 des de 71 resultats
The long-term follow-up of chronic hyperinsulinemic seizures, epileptogenesis and other neurological complications in five patients who were treated with conservative therapy followed by pancreatectomy during the neonatal period and infancy, who were confirmed to have diffuse nesidioblastosis are

Diffuse nesidioblastosis of the pancreas in a neonate with seizures.

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Minimally invasive surgical interventions in the treatment of primary persistent hyperinsulinemic hypoglycemia of infancy.

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Hyperinsulinemia, diagnosed by laboratory tests, should be diagnosed and treated as soon as possible to prevent fatal complications such as neurological damage. Patients who are resistant to medical therapy should be treated surgically. Minimally invasive surgery, a newly developed approach, is a

Screening for Mutations in ABCC8 and KCNJ11 Genes in Saudi Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI) Patients.

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The autosomal recessive form of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is associated with mutations in either ABCC8 or KCNJ11 genes. In the present study, we describe the clinical features and results of genetic analysis of 13 Saudi Arabian patients with PHHI. Clinically, most

[Persistent hyperinsulinemic hypoglycemia of infancy: case report]

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OBJECTIVE: To report a case of Persistent Hyperinsulinemic Hypoglycemia in twins which is a situation not yet reported in the literature. METHODS: Report of seizures in identical twins, from consanguineous parents, with persistent hypoglycemia as cause of the seizures. Laboratory tests, performed

A Rare Cause of Hyperinsulinemic Hypoglycemia: Kabuki Syndrome

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Kabuki syndrome (KS) is a disease characterized by distinctive facial features, skeletal anomalies and delay in neuromotor development. KS 1 is an autosomal dominant condition caused by mutations in the KMT2D gene, whereas KS 2 is an X-linked disorder caused by mutations in the KDM6A

A study on nesidioblastosis in hyperinsulinemic hypoglycemia--diagnosis, treatment, and neurologic sequelae.

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The medical records of six cases of nesidioblastosis were examined to determine the diagnostic approach, treatment, and neurologic sequelae. All six patients were male, and their ages at the onset of the disease ranged from one day to six months (mean 3.36 +/- 2.5 mo.). Initial clinical features

Anaesthetic Management Of Nesidioblastosis In Two Infants.

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Nesidioblastosis is the most common cause of non-transient, recurrent and persistent hypoglycaemia in neonates and infants. It is a disorder of diffuse proliferation of beta cells of the pancreas leading to hyperinsulinemia and hypoglycaemia. The main aim is to prevent the severe episodes of
BACKGROUND Adult nesidioblastosis, a rare form of abnormal islet cell proliferation arising from the pancreatic ductal epithelium, is usually associated with severe hyperinsulinemic hypoglycemia. Overall, seventy-three cases of nesidioblastosis have been described in the English literature since the

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI): Long-term outcome following 95% pancreatectomy.

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OBJECTIVE To evaluate the outcome of neonates and infants with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) who had undergone 95% pancreatectomy, with special emphasis on development of diabetes mellitus (DM). METHODS Ten infants diagnosed according to the established criteria of PHHI,

[Clinical aspects, diagnosis and therapy of nesidioblastosis].

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Hyperinsulinemia due to an excessive secretion of insulin independent on normal regulation is the most frequent cause of persistent neonatal hypoglycemia. We report on clinical course, diagnostic procedures and treatment of nesidioblastosis in three patients. Main symptoms observed in newborn period

Persistent hyperinsulinemic hypoglycemia of infancy.

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OBJECTIVE To study the nature and clinical course of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) due to nesidioblastosis. METHODS Clinical, laboratory and therapeutic evaluation of infants with this disorder and study the outcome. METHODS Hospital born neonates and infants referred

Persistent hyperinsulinemic hypoglycemia of infancy in 38 children.

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OBJECTIVE To describe the clinical, biochemical, radiological and electrophysiological features of 38 Saudi children with persistent hyperinsulinemic hypoglycemia of infancy that have been followed since 1983. METHODS Data from 38 patients followed at King Faisal Specialist Hospital and Research

Metastatic insulinoma in an adult patient with underlying nesidioblastosis.

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OBJECTIVE To report a case of metastatic insulinoma presenting 11 yr after enucleation of an isolated insulinoma, and 5 yr after distal pancreatectomy for nesidioblastosis. METHODS We present the clinical, laboratory, radiological and pathological findings in a 34 yr-old-man with recurrent

Hyperinsulinemic hypoglycemia: experience in a series of 17 cases.

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OBJECTIVE Hyperinsulinemic hypoglycemia (HIH) is a genetically heterogeneous disorder with both familial and sporadic variants. Patients with HIH may present during the neonatal period, infancy, or childhood and may show transient, prolonged, and persistent features. In this study, we aimed to
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