- Heating of Newborn Infants due to Blue Light-Emitting Diode Fibreoptic Phototherapy Pads. [Journal Article]
- NNeonatology 2017 Apr 27; 112(2):103-109
- CONCLUSIONS: LED fibreoptic phototherapy pads are heated by high-intensity blue light. The thermal environment and temperature of babies should be monitored closely during LED fibreoptic phototherapy. A temperature probe placed between the skin and the pad will not accurately reflect the core temperature during fibreoptic phototherapy.
- Cholestasis caused by panhypopituitarism and acquired cytomegalovirus infection in a 2-month-old male infant: A case report. [Journal Article]
- MMedicine (Baltimore) 2017; 96(17):e6757
- CONCLUSIONS: Although an endocrine disorder such as panhypopituitarism is rare in the cause of neonatal or infantile cholestasis, we must keep this reason in mind.
- Different clinical presentations of choledochal cyst among infants and older children: A 10-year retrospective study. [Journal Article]
- MMedicine (Baltimore) 2017; 96(17):e6679
- Choledochal cyst is a rare and often benign congenital cystic dilation throughout the biliary tree. Due to the benign nature of choledochal cyst among early-diagnosed patients, the clinical assumptio...
Choledochal cyst is a rare and often benign congenital cystic dilation throughout the biliary tree. Due to the benign nature of choledochal cyst among early-diagnosed patients, the clinical assumption and diagnosis seem to be of utmost significance. Therefore, we sought to assess different clinical manifestations of choledochal cyst and relevant laboratory findings in infants and older children.Retrospectively, medical records of all patients with the diagnosis of choledochal cyst between 2005 and 2015 were reviewed. Demographic data, initial clinical presentation, positive findings on physical examination, history of any remarkable behavior such as persistent and unexplained crying and poor feeding, and diagnostic imaging modalities were listed. In addition, laboratory values for total and direct bilirubin, alkaline phosphatase, alanine transaminase, aspartate transaminase, prothrombin time, and partial thromboplastin time (PTT) were recorded for each patient. Patients were divided into 2 groups; younger than 1-year-old (infants), and 1 year to 18 years old (older children). Demographic data, clinical data, and laboratory values were compared between the infants and older children.Thirty-two patients with a diagnosis of choledochal cyst were included in the study: 9 patients (28.12%) were infants and 23 patients (71.87%) were older children. Abdominal pain was the most common presenting symptom (62.5%), followed by nausea/vomiting (59.4%) and jaundice (28.1%). None of the patients presented with the classic triad of abdominal pain, jaundice, and right upper quadrant mass. Seventeen older children (73.91%) presented with nausea and vomiting, while 2 subjects (22.22%) in the infantile group presented with this feature (P = .01). Similarly, abdominal pain was found in 20 older children (86.95%); however, none of the infants presented with abdominal pain at diagnosis (P < .001). By contrast, the abdominal mass was more detected in infants than the older children (33.33% vs. 0%, P = .01). In terms of laboratory values, the median PTT was 44 and 36 s in infants and older children, respectively (P = .04).Infants were more likely to present with abdominal mass and older children were more likely to have nausea, vomiting, and abdominal pain. Furthermore, infants had more prolonged PTT than older children, implying a potential bleeding tendency.
- [Acute liver failure]. [Journal Article]
- MKMed Klin Intensivmed Notfmed 2017 Apr 25
- Acute liver failure (ALF) is a rare, but life-threatening disease that is characterized by the acute onset of jaundice, coagulopathy, and hepatic encephalopathy (HE) in patients without pre-existing ...
Acute liver failure (ALF) is a rare, but life-threatening disease that is characterized by the acute onset of jaundice, coagulopathy, and hepatic encephalopathy (HE) in patients without pre-existing liver disease. Main causes in Germany are drug toxicity, acetaminophen overdose, and viral hepatitis (A, B, E). For the initial assessment of patients with ALF and the diagnostic algorithm, the early detection of HE, exclusion of liver cirrhosis, immediate diagnosis of the underlying etiology, and evaluation for the necessity of liver transplantation (LT) are critical. Intensive care therapeutic measures aim at preventing or treating complications of ALF. Potentially, plasmapheresis (full plasma exchange) offers a survival benefit for ALF patients who do not undergo LT. The King's College criteria and the Clichy criteria are used as prognostic tools for the indication for LT.
- A rare association of central hypothyroidism and adrenal insufficiency in a boy with Williams-Beuren syndrome. [Journal Article]
- APAnn Pediatr Endocrinol Metab 2017; 22(1):65-67
- Primary hypothyroidism related to morphological and volumetric abnormalities of the thyroid gland is one of the commonest of several endocrine dysfunctions in Williams-Beuren syndrome (WBS). We repor...
Primary hypothyroidism related to morphological and volumetric abnormalities of the thyroid gland is one of the commonest of several endocrine dysfunctions in Williams-Beuren syndrome (WBS). We report a 10-month-old boy with WBS who presented with central hypothyroidism. During the neonatal period, he had prolonged jaundice, feeding difficulties and episodes of colic that continued during early infancy. Additionally, there was slowing of growth and mild developmental delay. He underwent surgical repair for supravalvular aortic stenosis at 6 months of age. An evaluation done to exclude cortisol deficiency before initiating levothyroxine lead to the detection of secondary adrenal insufficiency, unreported previously in WBS. In addition, insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 levels were low. This report of hypopituitarism in WBS indicates a need for complete evaluation of pituitary dysfunction in children with WBS.
- The Natural History of Severe Acute Liver Injury. [Journal Article]
- AJAm J Gastroenterol 2017 Apr 25
- CONCLUSIONS: A majority of ALI cases are due to APAP, 93% of whom will improve rapidly and fully recover, while non-APAP patients have a far greater risk of poor outcome and should be targeted for early referral to a liver transplant center.Am J Gastroenterol advance online publication, 25 April 2017; doi:10.1038/ajg.2017.98.
- Endoscopic ultrasound-guided choledochoduodenostomy for obstructive jaundice with venous collaterals around the bile duct wall (with video). [Journal Article]
- EUEndosc Ultrasound 2017 Mar-Apr; 6(2):140-141
- Significant Radiologic Response of Pancreatic Metastasis After Targeted Therapy of Ceritinib (LDK378) for ALK-Rearranged Lung Adenocarcinoma Presenting With Hyperglycemia. [Journal Article]
- OROncol Res 2017 Apr 14; 25(4):545-550
- Pancreatic metastasis from non-small cell lung cancer (NSCLC) is usually asymptomatic or presents with abdominal pain, acute pancreatitis, or jaundice. A lung primary is associated with worse surviva...
Pancreatic metastasis from non-small cell lung cancer (NSCLC) is usually asymptomatic or presents with abdominal pain, acute pancreatitis, or jaundice. A lung primary is associated with worse survival compared to pancreatic metastases from other organs. Surgical treatment of solitary metastasis to the pancreas from NSCLC has been reviewed in several studies, one of which had a notable disease-free interval. To our knowledge, there are no prior reports of targeted therapy of pancreatic metastasis of NSCLC followed by a significant response. Herein we report the case of a 31-year-old female with a solitary pancreatic metastasis from ALK-rearranged lung adenocarcinoma despite treatment with chemotherapy and crizotinib; she presented with symptoms of hyperglycemia. Targeted therapy with ceritinib (LDK378) led to symptomatic improvement and a significant radiologic response in the lung and pancreas, but not in the brain.
- Pathology of Naturally Occurring Bacillary Hemoglobinuria in Cattle. [Journal Article]
- VPVet Pathol 2017; 54(3):457-466
- Clostridium haemolyticum causes bacillary hemoglobinuria (BH), an infectious and usually fatal disease that occurs mostly in cattle, which is clinically characterized by jaundice, hemoglobinuria, and...
Clostridium haemolyticum causes bacillary hemoglobinuria (BH), an infectious and usually fatal disease that occurs mostly in cattle, which is clinically characterized by jaundice, hemoglobinuria, and anemia. The trematode Fasciola hepatica has been commonly reported as the main predisposing factor that triggers this condition. The authors evaluated 20 naturally occurring cases of bovine BH to characterize the pathology and pathogenesis of the disease. Grossly, the most consistent finding was a large, frequently single focus of necrosis surrounded by a red to purple halo, observed most frequently on the parietal surface of the right and left hepatic lobes. Other findings were jaundice, dark-brown discoloration of kidneys, and red urine in the urinary bladder. Microscopically, characteristic lesions were locally extensive, necrotizing hepatitis with thrombosis and numerous intralesional Gram-positive rod-shaped bacteria, and acute renal tubular necrosis. By immunohistochemistry, many hepatocytes outside the necrotic focus in the liver were positive for activated caspase 3, suggesting that those cells were undergoing apoptosis. Ultrastructural evaluation revealed hepatocyte necrosis, hemolysis, and clumps of vegetative and sporulating bacilli within the liver. Polymerase chain reaction for the C. haemolyticum beta toxin gene was positive in randomly selected liver samples. No gross or microscopic lesions indicative of fascioliasis were detected in the liver of any animal, suggesting that other yet undetermined predisposing factors were associated with these cases of BH.
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- A 6-year-old boy with Wilson disease-A diagnostic dilemma. [Congresses]
- IJIndian J Gastroenterol 2017 Apr 24
- A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, an...
A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, and coagulopathy. Viral markers and slit lamp examination for Kayser-Fleischer ring were negative. Serum ceruloplasmin and 24-h urinary copper post-D-pencillamine challenge were normal. Anti-smooth muscle antibody was positive 1:20, and liver biopsy showed micronodular cirrhosis with abundant Mallory hyaline and stainable copper deposits. The liver histology was indicative of Indian childhood cirrhosis, whereas the presence of autoantibodies, elevated transaminases, and increased globulin was suggestive of autoimmune hepatitis. Gene studies identified p.R969Q mutation in ATP7B gene, which solved the dilemma and confirmed the diagnosis of Wilson disease (WD). We report a clinicopathological conference of this boy to highlight the challenges faced by pediatricians in the diagnosis of Wilson disease. ᅟ.