BECKWITH'S SYNDROME WITH EXTREME ORGAN HYPERPLASIA

PEDIATRICS ◽  
1973 ◽  
Vol 52 (3) ◽  
pp. 372-381
Author(s):  
Thomas F. Roe ◽  
Ann K. Kershnar ◽  
Jordan J. Weitzman ◽  
Luis Salinas Madrigal
Keyword(s):  
Adrenal Gland ◽  
Abdominal Mass ◽  
Severe Hypoglycemia ◽  
Immunoreactive Insulin ◽  
Left Adrenal Gland ◽  
Adrenal Steroid ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Pedunculated Tumor ◽  
The Right

A large newborn infant with hemihypertrophy, omphalocele, hepatomegaly, left upper quadrant abdominal mass, and zoster-like skin rash had severe hypoglycemia at 4 hours of age. Serum immunoreactive insulin levels were markedly elevated. Hypoglycemia was not controlled by vigorous medical therapy but blood glucose levels returned toward normal following subtotal excision of the markedly hyperplastic pancreas at 24 days of age. At 4½ months of age, a right upper quadrant abdominal mass was noted and urinary adrenal steroid levels were elevated. The right adrenal gland was found to be markedly hyperplastic and it was removed; the left adrenal gland was slightly hyperplastic. Between the ages of 5 and 8 months the umbilical stump enlarged progressively forming a large pedunculated tumor which was removed. This patient presents an unusually severe example of Beckwith's syndrome with both prenatal and postnatal organ overgrowth, severe hypoglycemia and hyperinsulinism.

Effective Therapy of Insulinoma by Using Long-Acting Somatostatin Analogue. A Case Report and Literature Review

2011 ◽  
Vol 120 (02) ◽  
pp. 68-72 ◽  
Author(s):  
A. Jawiarczyk ◽  
M. Bolanowski ◽  
J. Syrycka ◽  
G. Bednarek-Tupikowska ◽  
M. Kałużny ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Somatostatin Receptors ◽  
Subcutaneous Administration ◽  
Pancreatic Neuroendocrine Tumor ◽  
Somatostatin Analogue ◽  
Left Adrenal Gland ◽  
Glucose Levels ◽  
Long Acting ◽  
The Right

AbstractWe are reporting a case of 68-year-old woman with insulinoma, after a non-successful tumor surgery and a long-term diazoxide treatment. She had a lot of hypoglycemia cases, and a weight gain of 50 kg. An abdominal CT scan demonstrated a tumor 28 mm in the diameter, in the head of the pancreas. The patient did not agree for the repeated insulinoma surgery. Furthermore, we found a lesion in the left adrenal gland (14 mm in the diameter) and in the right lung (8 mm in the diameter). Pheochromocytoma was diagnosed on the basis of hypertension, elevated levels of normetanephrine in the 24-h urine collection, and an elevated level of norepinephrine in a plasma sample. After the left adrenal gland removal we observed lower blood pressure. Since we had revealed the presence of somatostatin receptors by the somatostatin receptors scintigraphy, we decided to control hypoglycemia by a monthly subcutaneous administration of the long-acting lanreotide. Because of higher glucose levels (300–400 mg/dl) we started an intense insulin therapy. Nowadays, the patient feels better, she has lost 20 kg of her body weight, and we have observed normal blood glucose levels during the long-term lanreotide treatment. We have noticed neither side effects nor hypoglycemic episodes and we have reduced the dose of insulin. The presented case can be an evidence of the effective treatment of the pancreatic neuroendocrine tumor of insulinoma type, with somatostatin analogue.

scholarly journals Giant adrenal hemangioma: Unusual cause of huge abdominal mass

2015 ◽  
Vol 9 (11-12) ◽  
pp. 834 ◽  
Author(s):  
Mohamed Tarchouli ◽  
Adil Boudhas ◽  
Moulay Brahim Ratbi ◽  
Mohamed Essarghini ◽  
Noureddine Njoumi ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Abdominal Mass ◽  
Abdominal Distension ◽  
Microcytic Anemia ◽  
Hypochromic Microcytic Anemia ◽  
Normal Limits ◽  
Laboratory Examinations ◽  
Right Lobe ◽  
The Right

Adrenal hemangioma is an extremely rare benign and non-functioning neoplasm of the adrenal gland. We report a case of a 71-year-old woman admitted for intermittent abdominal pain and abdominal distension associated with vomiting and chronic constipation for 5 years. Physical examination revealed a large abdominal mass. Both computed tomography scan and magnetic resonance imaging suggested hemangioma in the right lobe of the liver. Laboratory examinations and tumour markers were within normal limits, except for hypochromic microcytic anemia. The mass was removed intact by conventional surgery and histopathology revealed a cavernous hemangioma of the adrenal gland with no signs of malignancy. Surgical resection was curative, with no recurrence at the 2-year follow-up.

scholarly journals Glucose-Responsive Insulin Through Bioconjugation Approaches

2019 ◽  
Vol 14 (2) ◽  
pp. 198-203 ◽  
Author(s):  
Maria M. Disotuar ◽  
Diao Chen ◽  
Nai-Pin Lin ◽  
Danny Hung-Chieh Chou
Keyword(s):  
Glycemic Control ◽  
Severe Hypoglycemia ◽  
Therapeutic Window ◽  
Insulin Analogs ◽  
Future Directions ◽  
Major Health ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Glucose Responsiveness ◽  
Reduced Risk

Although insulin analogs have markedly improved glycemic control for people with diabetes, glycemic excursions still cause major health problems and complications. In particular, the narrow therapeutic window of current insulin therapy makes it extremely difficult to maintain normoglycemia without risking severe hypoglycemia. Currently, there are no FDA-approved insulin therapeutics whose bioactivity is regulated by blood glucose levels. This review discusses recent progress on developing glucose-responsive insulin (GRI) bioconjugates without the need of exogenous matrices. Through this approach, tremendous efforts have been made over the years to demonstrate the promise of better glycemic control and reduced risk of hypoglycemia. Last, we discuss future directions of GRI development with a goal to maximize the glucose responsiveness.

scholarly journals Clinical and genetic characterization of congenital hyperinsulinism in Spain

2016 ◽  
Vol 174 (6) ◽  
pp. 717-726 ◽  
Author(s):  
R Martínez ◽  
C Fernández-Ramos ◽  
A Vela ◽  
T Velayos ◽  
A Aguayo ◽  
...  
Keyword(s):  
Mutational Analysis ◽  
Genetic Diagnosis ◽  
Clinical Information ◽  
Severe Hypoglycemia ◽  
Compound Heterozygous ◽  
Congenital Hyperinsulinism ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Lower Blood ◽  
Design And Methods

Context Congenital hyperinsulinism (CHI) is a clinically and genetically heterogeneous disease characterized by severe hypoglycemia caused by inappropriate insulin secretion by pancreatic β-cells. Objective To characterize clinically and genetically CHI patients in Spain. Design and methods We included 50 patients with CHI from Spain. Clinical information was provided by the referring clinicians. Mutational analysis was carried out for KCNJ11, ABCC8, and GCK genes. The GLUD1, HNF4A, HNF1A, UCP2, and HADH genes were sequenced depending on the clinical phenotype. Results We identified the genetic etiology in 28 of the 50 CHI patients tested: 21 had a mutation in KATP channel genes (42%), three in GLUD1 (6%), and four in GCK (8%). Most mutations were found in ABCC8 (20/50). Half of these patients (10/20) were homozygous or compound heterozygous, with nine being unresponsive to diazoxide treatment. The other half had heterozygous mutations in ABCC8, six of them being unresponsive to diazoxide treatment and four being responsive to diazoxide treatment. We identified 22 different mutations in the KATP channel genes, of which ten were novel. Notably, patients with ABCC8 mutations were diagnosed earlier, with lower blood glucose levels and required higher doses of diazoxide than those without a genetic diagnosis. Conclusions Genetic analysis revealed mutations in 56% of the CHI patients. ABCC8 mutations are the most frequent cause of CHI in Spain. We found ten novel mutations in the KATP channel genes. The genetic diagnosis is more likely to be achieved in patients with onset within the first week of life and in those who fail to respond to diazoxide treatment.

Adrenal Insufficiency in the Term and Preterm Neonate

1999 ◽  
Vol 18 (5) ◽  
pp. 21-28 ◽  
Author(s):  
Catherine Witt
Keyword(s):  
Adrenal Gland ◽  
Adrenal Insufficiency ◽  
Preterm Neonate ◽  
Endocrine System ◽  
Signs And Symptoms ◽  
The Body ◽  
Electrolyte Balance ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Appropriate Treatment

The adrenal gland is part of the endocrine system and produces hormones that are essential for survival. The adrenal cortex, the largest part of the adrenal gland, produces three major hormones. Glucocorticoids regulate metabolism of glucose, protein, and fat and allow the body to respond to stress by increasing blood glucose levels and cardiac output. Mineralocorticoids regulate fluid and electrolyte balance and help maintain blood pressure. Androgens are responsible for sexual differentiation in the fetus.Insufficient production of these hormones can occur for many reasons and can have a profound effect on the newborn. It is important for neonatal nurses to be familiar with signs and symptoms and appropriate treatment of adrenal insufficiency. This article explores causes, signs and symptoms, and treatment of adrenal insufficiency in the newborn.

Adrenal Pheochromocytoma With Contralateral Adrenocortical Adenoma in a Cat

2010 ◽  
Vol 46 (1) ◽  
pp. 36-42 ◽  
Author(s):  
Jon David R. Calsyn ◽  
Rebecca A. Green ◽  
Garrett J. Davis ◽  
Christopher M. Reilly
Keyword(s):  
Adrenal Gland ◽  
Low Dose ◽  
Clinical Signs ◽  
Adrenocortical Adenoma ◽  
Left Adrenal Gland ◽  
Adrenal Pheochromocytoma ◽  
History Of ◽  
Suppression Test ◽  
The Right ◽  
Dexamethasone Suppression

A 7-year-old, neutered male cat was presented with a 6-month history of progressive polyuria, polydipsia, polyphagia, aggression, and weight gain. Previous blood work, urinalysis, and radiographs did not delineate a cause for the clinical signs. An ultrasound revealed bilateral adrenal gland enlargement. A low-dose dexamethasone suppression test was consistent with hyperadrenocorticism. Based on these findings, bilateral adrenalectomy was attempted and successfully performed. Histopathology was consistent with a cortical adenoma in the right adrenal gland and a pheochromocytoma in the left adrenal gland. This association has never been reported in the cat.

scholarly journals The diagnosis and treatment of primary adrenal lipomatous tumors in Chinese patients: a 31-year follow-up study

2014 ◽  
Vol 8 (3-4) ◽  
pp. 132 ◽  
Author(s):  
Fukang Sun ◽  
Juping Zhao ◽  
Xiaolong Jing ◽  
Wenlong Zhou ◽  
Xin Huang ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Diagnosis And Treatment ◽  
Chinese Patients ◽  
Preoperative Imaging ◽  
Left Adrenal Gland ◽  
Single Centre ◽  
Follow Up Study ◽  
The Right ◽  
Lipomatous Tumors

Introduction: Adrenal lipomatous tumours (ALTs) are rarely encountered in clinical practice and consequently little is known about their clinical features.Methods: We analyze the clinical features, diagnosis and treatment of ALTs based on cases presenting at a single centre over a 31-year period. We reviewed clinical data from patients with primary adrenal tumours treated at the Ruijin Hospital, Shanghai between January 1980 and December 2010.Results: A total of 73 cases of primary ALTs in 22 men and 51 women (mean age 51.1±14.2 years) were reviewed. The ALTs included 65 myelolipomas (89.0%), 3 lipomas (4.1%), 2 angiomyolipomas (2.7%), 2 teratomas (2.7%), and 1 liposarcoma (1.4%). Of the total 73 patients, 24 of them had tumours in the left adrenal gland, 47 in the right gland and 2 had bilateral tumours. In total, 51 patients underwent open surgery and 22 laparoscopic surgery.Conclusion: Myelolipoma is predominant among the various types of lipomatous adrenal gland tumours; it accounts for about 90% of all cases. Surgery is recommended for tumours ≥3.5 cm in diameter, for all cases of symptomatic tumour, and for cases of teratoma or liposarcoma identified by preoperative imaging.

scholarly journals VIRUS-INDUCED DIABETES MELLITUS

1973 ◽  
Vol 137 (5) ◽  
pp. 1226-1239 ◽  
Author(s):  
D. Wark Boucher ◽  
Abner Louis Notkins
Keyword(s):  
Blood Glucose ◽  
Encephalomyocarditis Virus ◽  
Immunoreactive Insulin ◽  
Free Access ◽  
Abnormal Glucose Tolerance ◽  
Male Mice ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Access To Food ◽  
Rapid Drop

Infection of DBA/2N male mice with encephalomyocarditis virus resulted in a diabeteslike syndrome characterized by hyperglycemia, glycosuria, hypoinsulinemia, polydipsia, and polyphagia. Blood glucose levels were elevated within 4 days after infection and reached a maximum mean level of 320 mg/100 ml within 12 days. Approximately 60–80% of the animals developed a transient hyperglycemia while 10–15% of the animals remained hyperglycemic for well over 6 mo. The remaining animals failed to become hyperglycemic but many had abnormal glucose tolerance curves. Hyperglycemia was most pronounced when animals were allowed free access to food, and the incidence of byperglycemia was related both to the strain and sex of the animals, with few females developing hyperglycemia. The amount of immunoreactive insulin in the plasma of infected hyperglycemic mice was significantly lower than in appropriate controls, and injection of exogenous insulin resulted in a rapid drop in the blood glucose levels. Despite the fact that certain animals were hyperglycemic for many months, virus could not be recovered from the pancreas after the first 10 days of the infection.

scholarly journals Therapeutic Use of Intranasal Glucagon: Resolution of Hypoglycemia

2019 ◽  
Vol 20 (15) ◽  
pp. 3646 ◽  
Author(s):  
Antonio E. Pontiroli ◽  
Elena Tagliabue
Keyword(s):  
Severe Hypoglycemia ◽  
Well Being ◽  
Glucose Levels ◽  
Fear Of Hypoglycemia ◽  
Blood Glucose Levels ◽  
Increase Blood Glucose ◽  
Level Of Consciousness ◽  
Patients With Diabetes ◽  
Adults And Children ◽  
First Time

Episodes of hypoglycemia are frequent in patients with diabetes treated with insulin or sulphonylureas. Hypoglycemia can lead to severe acute complications, and, as such, both prevention and treatment of hypoglycemia are important for the well-being of patients with diabetes. The experience of hypoglycemia also leads to fear of hypoglycemia, that in turn can limit optimal glycemic control in patients, especially with type 1 diabetes. Treatment of hypoglycemia is still based on administration of carbohydrates (oral or parenteral according to the level of consciousness) or of glucagon (intramuscular or subcutaneous injection). In 1983, it was shown for the first time that intranasal (IN) glucagon drops (with sodium glycocholate as a promoter) increase blood glucose levels in healthy volunteers. During the following decade, several authors showed the efficacy of IN glucagon (drops, powders, and sprays) to resolve hypoglycemia in normal volunteers and in patients with diabetes, both adults and children. Only in 2010, based on evaluation of patients’ beliefs and patients’ expectations, a canadian pharmaceutical company (Locemia Solutions, Montreal, Canada) reinitiated efforts to develop glucagon for IN administration. The project has been continued by Eli Lilly, that is seeking to obtain registration in order to make IN glucagon available to insulin users (children and adolescents) worldwide. IN glucagon is as effective as injectable glucagon, and devoid of most of the technical difficulties associated with administration of injectable glucagon. IN glucagon appears to represent a major breakthrough in the treatment of severe hypoglycemia in insulin-treated patients with diabetes, both children and adults.

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