Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature

Existing Reviews

Please note, new claims can take a short while to show up.

No claims yet.

Annotation Summary

Term Occurence Count Dictionary
hyperinsulinemia 1 endocrinologydiseases
hypoglycemia 30 endocrinologydiseases
cholic acid 1 endocrinologydiseasesdrugs
diazoxide 5 endocrinologydiseasesdrugs
galactosemia 3 endocrinologydiseases
hyperglycemia 1 endocrinologydiseases

Graph of close proximity drug and disease terms (within 200 characters).

Note: If this graph is empty, then there are no terms that meet the proximity constraint.

Review

Having read the paper, please pick a pair of statements from the paper to indicate that a drug and disease are related.

Select Drug Character Offset Drug Term Instance
cholic acid 4140 phototherapy. He also developed a clinically relevant neonatal cholestasis, which was treated with ursodeoxy cholic acid , L-carnitine, and liposoluble vitamins (higher administration of these vitamins is required since the
diazoxide 5880 vena cava inferior (white thin arrow in f)Due to these findings, an orally administered therapy with diazoxide was started to suppress insulin secretion. Simultaneously starch was added to the newborn’s milk feeding.
diazoxide 6499 (mg/dl)614.5Maltodextrin35–441018.4Maltodextrin FM85 5%19–382822Maltodextrin 200 ml/kg Milk uptake30–443623.2+ Diazoxide28–428017.5Starch diazoxide 34–4410017.7Starch diazoxide41–44We decided to close the shunts via interventional radiology due
diazoxide 6529 5%19–382822Maltodextrin 200 ml/kg Milk uptake30–443623.2+ Diazoxide28–428017.5Starch diazoxide34–4410017.7Starch diazoxide 41–44We decided to close the shunts via interventional radiology due to the recurrent episodes of hypoglycemia,
diazoxide 7373 measure blood sugar levels and instructed to do so three times a day to make sure the therapy with diazoxide and Glycosade® (modified cornstarch)-enhanced mother’s milk was sufficient. The diazoxide therapy
diazoxide 7466 therapy with diazoxide and Glycosade® (modified cornstarch)-enhanced mother’s milk was sufficient. The diazoxide therapy showed no side effects. Close follow-up examinations including an ultrasound of his liver were
Select Disease Character Offset Disease Term Instance
galactosemia 10602 proposed screening for CPSS via ultrasound if, for example, neonatal conjugated hyperbilirubinemia or hyper galactosemia is present. We propose to add “persistent hypoglycemia” to the reasons for screening for CPSS. When
galactosemia 12597 patient, serum levels for ammonia or galactose were normal. One should expect hyperammonemia and/or hyper galactosemia due to the increased blood volume bypassing the liver and thus the hepatic metabolism. Why both hyperammonemia
galactosemia 12730 blood volume bypassing the liver and thus the hepatic metabolism. Why both hyperammonemia and hyper galactosemia were absent in our patient cannot be conclusively explained. This probably would require experimental
hyperglycemia 11996 et al. postulated an insufficient postprandial hepatic glucose uptake, resulting in early systemic hyperglycemia , leading to an exaggerated insulin secretion [[9]]. In addition, the insulin then bypasses the hepatic
hyperinsulinemia 8337 to secondary hyperinsulinismAuthorTitleShunt anatomyTherapyBas et al., 2015 [[9]]Premature pubarche, hyperinsulinemia and hypothyroxinemia: novel manifestations of congenital portosystemic shunts (Abernethy malformation)
hypoglycemia 84 Title: Journal of Medical Case ReportsCongenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literatureAlexander WeigertJeanette
hypoglycemia 593 other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have been reported so far and our case, to the best of our knowledge, describes the most severe extent
hypoglycemia 712 been reported so far and our case, to the best of our knowledge, describes the most severe extent of hypoglycemia .Case presentationWe describe the case of a newborn Arab boy with two intrahepatic portosystemic shunts,
hypoglycemia 864 of a newborn Arab boy with two intrahepatic portosystemic shunts, resulting in severe and persistent hypoglycemia , due to which one of the shunts was closed by interventional radiology whereas the other shunt had already
hypoglycemia 1401 insights into glucose regulation mechanisms of the liver and we suggest a consistent screening for hypoglycemia in patients with congenital portosystemic shunts.IntroductionCongenital portosystemic shunts (CPSS)
hypoglycemia 2650 a newborn with two congenital intrahepatic portosystemic shunts with concomitant, severe persistent hypoglycemia due to secondary hyperinsulinism. We describe the clinical course and treatment and review the literature.Case
hypoglycemia 4733 throughout the clinical course. From the second day of life, our patient presented with clinically relevant hypoglycemia (blood sugar below 45 mg/dl) which was treated with intravenously administered glucose substitutions.
hypoglycemia 5034 unsuccessful. Even with an oral glucose uptake of 25 g per kilogram body weight per day episodes of hypoglycemia reoccurred. Increased oral glucose uptake was achieved both by adding glucose and starch to our patient’s
hypoglycemia 6056 was added to the newborn’s milk feeding. As a result, blood sugar levels stabilized and periods of hypoglycemia were scarce, but still present. A scheme of oral glucose uptake and resulting hypoglycemia is presented
hypoglycemia 6147 periods of hypoglycemia were scarce, but still present. A scheme of oral glucose uptake and resulting hypoglycemia is presented in Table 1.Table 1Scheme of oral glucose uptake and resulting hypoglycemiaAge (days)Glucose
hypoglycemia 6236 resulting hypoglycemia is presented in Table 1.Table 1Scheme of oral glucose uptake and resulting hypoglycemia Age (days)Glucose content of nutriment (g/kg per day)Medication/dietary supplementRange of hypoglycemia
hypoglycemia 6339 hypoglycemiaAge (days)Glucose content of nutriment (g/kg per day)Medication/dietary supplementRange of hypoglycemia (mg/dl)614.5Maltodextrin35–441018.4Maltodextrin FM85 5%19–382822Maltodextrin 200 ml/kg Milk uptake30–443623.2+
hypoglycemia 6638 diazoxide41–44We decided to close the shunts via interventional radiology due to the recurrent episodes of hypoglycemia , insufficient production of coagulation factors, and the elevated levels of ALT and AST. When he was
hypoglycemia 7827 [[2]].Literature reviewWe searched the literature (www.ncbi.nlm.nih.gov; April 30, 2017) for reports of hypoglycemia in patients with intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism using
hypoglycemia 7892 (www.ncbi.nlm.nih.gov; April 30, 2017) for reports of hypoglycemia in patients with intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism using the following search terms: “intrahepatic,” “portocaval,”
hypoglycemia 8048 following search terms: “intrahepatic,” “portocaval,” “portosystemic,” “shunts,” “ hypoglycemia ,” and “hyperinsulinism.” The results are summarized in Table 2.Table 2Case reports of hypoglycemia
hypoglycemia 8154 “hypoglycemia,” and “hyperinsulinism.” The results are summarized in Table 2.Table 2Case reports of hypoglycemia in patients with intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinismAuthorTitleShunt
hypoglycemia 8219 in Table 2.Table 2Case reports of hypoglycemia in patients with intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinismAuthorTitleShunt anatomyTherapyBas et al., 2015 [[9]]Premature pubarche,
hypoglycemia 8586 childrenExtrahepaticDietetic (rich in complex carbohydrates)Duprey et al., 1985 [[7]]Glucose intolerance and post-stimulatory hypoglycemia secondary to a probably congenital intrahepatic portocaval anastomosisData not availableData not availableGouin
hypoglycemia 8917 abnormalitiesData not availableData not availableSenniappan et al., 2015 [[10]]Postprandial hyperinsulinemic hypoglycemia secondary to a congenital portosystemic shuntExtrahepaticDiazoxide, surgical closureYoshii et al., 2017
hypoglycemia 9102 surgical closureYoshii et al., 2017 [[11]]Portosystemic shunt as a cause of congenital hyperinsulinemic hypoglycemia Data not availableData not availablePresent caseCongenital intrahepatic portocaval shunts and hypoglycemia
hypoglycemia 9208 hypoglycemiaData not availableData not availablePresent caseCongenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: presentation of a new case and review of the literatureIntrahepaticInterventional
hypoglycemia 10216 intrahepatic and extrahepatic shunts have different chances of spontaneous closure [[3]].Complications such as hypoglycemia due to hyperinsulinism as reported in our case seem to be rare [[1], [6], [7]] and, until now, never
hypoglycemia 10659 neonatal conjugated hyperbilirubinemia or hypergalactosemia is present. We propose to add “persistent hypoglycemia ” to the reasons for screening for CPSS. When CPSS is diagnosed, Sokollik et al. suggested screening
hypoglycemia 11101 complication, to add a consequent and regular screening for low blood sugar levels to screen for persisting hypoglycemia to these algorithms. This can be achieved either through regular measurement of blood glucose levels
hypoglycemia 11345 life or by using a continuous glucose monitoring system.The episodes of hyperinsulinism with secondary hypoglycemia reported here might result from a reduced hepatic degradation of insulin due to the high blood volume
hypoglycemia 12138 insulin secretion [[9]]. In addition, the insulin then bypasses the hepatic metabolism, causing late hypoglycemia due to the prolonged insulin effect [[7]].Since our patient also presented with elevated levels for
hypoglycemia 12972 would be beyond the scope of this case report. It is worth noting that in other cases of CPSS with hypoglycemia due to hyperinsulinism, levels of ammonia were also normal [[7], [8]].ConclusionsThe blood volume bypassing
hypoglycemia 13262 it also, as our case shows, has a strong influence on metabolic homeostasis. Although it seems that hypoglycemia is a rare complication of CPSS, we suggest a persistent screening for hypoglycemia in those patients.
hypoglycemia 13345 Although it seems that hypoglycemia is a rare complication of CPSS, we suggest a persistent screening for hypoglycemia in those patients. If CPSS are diagnosed prenatally, then regular measurement of blood sugar levels

You must be authorized to submit a review.