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Biliary atresia in premature infants
S240
Abstracts
Case report: A 54 year-old man with history of hypertension, coronary artery disease, congestive heart failure, antiphospholipid syndrome, and mesangiocapillary nephritis with end-stage renal disease on hemodialysis was hospitalized with decompensated heart failure. His medications included warfarin, steroids and ASA. After responding to initial treatment, he developed a sudden episode of massive hematemesis and subsequently became hypotensive with systolic blood pressure of 60mmHg. Aggressive volume resuscitation was initiated. An emergent gastroscopy was performed which showed an actively spurting fundic vessel with normal surrounding mucosa, consistent with a Dieulafoy lesion. The bleeding site was injected with five mls of epinephrine 1/10,000 followed by band ligation. This provided immediate hemostasis and improved hemodynamics. Repeat endoscopy demonstrated a non-bleeding banded site and no additional pathology. A total of eight units of packed RBC’s were required to bring the hemogram back to baseline. Patient remained stable throughout hospitalization with no further evidence of bleeding. Conclusion: Early diagnosis and prompt endoscopic intervention is critical for successful management of Dieulafoy lesions. Combination therapy with epinephrine injection and band ligation may be an effective modality.
767 Biliary atresia in premature infants Samra Sarigol-Blanchard M.D., Marla Gerrek C.F.N.P., Gisela Chelimsky M.D., Judy Splawski Steve Czinn M.D.*. 1Pediatric Gastroenterology, Case Western Reserve University, Cleveland, OH, United States. Purpose: Biliary atresia as a cause of chronic cholestasis in full term infants is well described in the literature. Affected infants are usually born at term and are of normal birth weight. We present 2 cholestatic premature infants, both of whom presented as a diagnostic dilemmas who were ultimately diagnosed with biliary atresia. Results: The first patient was born at 31 weeks of gestation. He was noted to have indirect hyperbilirubinemia, which peaked on day 5 of life and received phototherapy for 5 days. On day of life 35 this infant was noted to have a direct bilirubin (DB) of 2.0 mg/dl and a total bilirubin (TB) of 2.4mg/dl. In response to the direct hyperbilirubinemia an ultrasound examination was performed and found to be normal. The metabolic disease and infectious work up were also negative. A HIDA scan demonstrated no excretion and finally he had open liver biopsy which was consistent with biliary atresia. The diagnosis was confirmed with an operative cholangiogram, which revealed no excretion. He had Kasai procedure at DOL 65 at which time the TB was 3.4 mg/dl, DB was 2.7 mg/dl, and GGT 73 U/L, AST 96 U/L, ALT 58U/L. The second patient was also born at 31 weeks of gestation. She also had an initial indirect hyperbilirubinemia requiring phototherapy for 8 days. She initially also had gradual decline in bilirubin levels but DB continued to be greater than 20% of total (TB of 6.7 mg/dl and DB of 2.0 mg/dl) at DOL 17 which warranted a full work-up. She had negative infectious and metabolic panel. The gallbladder was not visualized in ultrasound. A HIDA scan showed no excretion. She underwent open liver biopsy at DOL 30, which revealed bile duct proliferation with cholestasis. She had cholangiogram with no excretion and underwent Kasai at DOL 55 at which time the TB was 6.4 mg/dl, DB was 4.8 mg/dl and ALT 62 U/L, AST 138 U/L, GGT 202 U/L. Conclusions: Biliary atresia can occur in premature infants despite being primarily a disease of full-term newborn infants. The insidious onset of direct hyperbilirubinemia in the premature infant may overlap with the duration of physiologic jaundice, sepsis or TPN related cholestatsis. (Both of these infants were on TPN for a short period). Despite the small size of the pre-term infant sometimes making it impossible to perform a percutaneous liver biopsy, the same aggressive approach to evaluate direct hyperbilirubinmia in full-term infants should apply to preterm infants to prevent delay in diagnosis. Open liver biopsy with intraoperative cholangiogram is a safe method to evaluate premature infants with direct hyprbilirubinemia.
AJG – Vol. 96, No. 9, Suppl., 2001
768 Elevation of liver enzymes in pediatric oncology patient: a case report Samra Sarigol-Blanchard M.D.1*, Kemal Sarman M.D.2, Metin Vural M.D.1 and Leyla Agaoglu M.D.1. 1Pediatric Gastroenterology, Hematology and Radiology, American Hospital, Istanbul, Turkey; and 2 Sarman Pathology Laboratories, Istanbul, Turkey. Purpose: We report a 3-year-old girl diagnosed with infantile Acute Lymphocytic Leukemia at age 3 months who presented with a six month history of elevated liver enzymes. Results: At the time of presentation she was in remission but required intense high dose maintenance chemotherapy including vincristine, cyclophosphamide, high dose methoteraxate, 6-MP, thioguanine and cytosine arabinoside. The physical exam revealed a pleasant girl with significant growth delay. The abdominal exam was remarkable for hepatosplenomegaly with no ascites. Laboratory studies at the time of presentation demonstrated an albumin of 4.2 g/dL, total bilirubin 6.1 mg/dL, direct bilirubin 3.6 mg/dL, aspartate aminotransferase 54 IU/L, alanine aminotransferase 236 IU/L, glutamyl transferase 1187 IU/L, and a protrombin time of 10.2 sec. Serologic tests for viral and autoimmune disease were negative. An Ultrasound of the abdomen revealed moderate hepatomegaly and splenomegaly with no evidence of ascites or enlarged lymph nodes. A percutaneous liver biopsy was performedand was remarkable for the the presence of significant hemosiderosis in macrophages, Kupffer cells and hepatocytes. Although there was a slight increase in collagen deposition particularly in the portal regions, the limiting plates were intact with no evidence of fibrosis. Iron studies and an MRI of the abdomen performed after the liver biopsy were remarkable for a significantly elevated ferritin level with a transferrin saturation of 60%. An MRI of the abdomen showed diminished signal intensity on T2-weighted images of liver and spleen with diffuse hepatosplenomegaly. Desferoxamine treatment rather than phlebotomy was initiated following consultation with her pediatric oncologist. Conclusions: Abnormal liver function tests are common in Acute Lymphocytic Leukemia patients and are usually attributed to hepatotoxic chemotherapy agents, viral hepatitis or recurrence of disease in the liver. It is important to remember that patients, receiving high dose chemotherapy, may also require significant transfusions of packed red blood cells. Therefore, in addition to the routine evaluation of elevated liver enzymes in such a patient, iron studies, liver biopsy and possibly an MRI of the abdomen, a useful, non-invasive diagnostic tool for evaluation of iron deposition should be considered.
769 Unusual endoscopic and histologic findings in teenagers presenting with constipation and rectal bleeding Samra Sarigol-Blanchard M.D., Gisela Chelimsky M.D., Judy Splawski M.D., Fred Rothstein M.D. and Steve Czinn M.D.*. 1Pediatric Gastroenterology, Case Western Reserve University, Cleveland, OH, United States. Purpose: Constipation is a common symptom in pediatric population and usually never requires an extensive work-up. We present two patients who presented with constipation and rectal bleeding and had an uncommon pathology in their colonoscopic evaluation. Results: A 14 year-old male presented with an 8-month history intermittent rectal bleeding with and without stooling. The past medical history was remarkable for intermittent episodes of constipation. On physical exam the perianal area was normal and the rectal exam was notable for grossly bloody mucous with no palpable lesions. Laboratory evaluation was remarkable only for an elevated sedimentation rate. Colonoscopy revealed raised mucosal folds with swollen, edematous, friable ulcerated mucosa at 10 cm from the anal verge. The biopsies demonstrated focal ulcerations and changes indicative of a solitary rectal ulcer syndrome. Despite the benign nature of the biopsy a CT of the abdomen and pelvis was performed to rule
Abstracts
Case report: A 54 year-old man with history of hypertension, coronary artery disease, congestive heart failure, antiphospholipid syndrome, and mesangiocapillary nephritis with end-stage renal disease on hemodialysis was hospitalized with decompensated heart failure. His medications included warfarin, steroids and ASA. After responding to initial treatment, he developed a sudden episode of massive hematemesis and subsequently became hypotensive with systolic blood pressure of 60mmHg. Aggressive volume resuscitation was initiated. An emergent gastroscopy was performed which showed an actively spurting fundic vessel with normal surrounding mucosa, consistent with a Dieulafoy lesion. The bleeding site was injected with five mls of epinephrine 1/10,000 followed by band ligation. This provided immediate hemostasis and improved hemodynamics. Repeat endoscopy demonstrated a non-bleeding banded site and no additional pathology. A total of eight units of packed RBC’s were required to bring the hemogram back to baseline. Patient remained stable throughout hospitalization with no further evidence of bleeding. Conclusion: Early diagnosis and prompt endoscopic intervention is critical for successful management of Dieulafoy lesions. Combination therapy with epinephrine injection and band ligation may be an effective modality.
767 Biliary atresia in premature infants Samra Sarigol-Blanchard M.D., Marla Gerrek C.F.N.P., Gisela Chelimsky M.D., Judy Splawski Steve Czinn M.D.*. 1Pediatric Gastroenterology, Case Western Reserve University, Cleveland, OH, United States. Purpose: Biliary atresia as a cause of chronic cholestasis in full term infants is well described in the literature. Affected infants are usually born at term and are of normal birth weight. We present 2 cholestatic premature infants, both of whom presented as a diagnostic dilemmas who were ultimately diagnosed with biliary atresia. Results: The first patient was born at 31 weeks of gestation. He was noted to have indirect hyperbilirubinemia, which peaked on day 5 of life and received phototherapy for 5 days. On day of life 35 this infant was noted to have a direct bilirubin (DB) of 2.0 mg/dl and a total bilirubin (TB) of 2.4mg/dl. In response to the direct hyperbilirubinemia an ultrasound examination was performed and found to be normal. The metabolic disease and infectious work up were also negative. A HIDA scan demonstrated no excretion and finally he had open liver biopsy which was consistent with biliary atresia. The diagnosis was confirmed with an operative cholangiogram, which revealed no excretion. He had Kasai procedure at DOL 65 at which time the TB was 3.4 mg/dl, DB was 2.7 mg/dl, and GGT 73 U/L, AST 96 U/L, ALT 58U/L. The second patient was also born at 31 weeks of gestation. She also had an initial indirect hyperbilirubinemia requiring phototherapy for 8 days. She initially also had gradual decline in bilirubin levels but DB continued to be greater than 20% of total (TB of 6.7 mg/dl and DB of 2.0 mg/dl) at DOL 17 which warranted a full work-up. She had negative infectious and metabolic panel. The gallbladder was not visualized in ultrasound. A HIDA scan showed no excretion. She underwent open liver biopsy at DOL 30, which revealed bile duct proliferation with cholestasis. She had cholangiogram with no excretion and underwent Kasai at DOL 55 at which time the TB was 6.4 mg/dl, DB was 4.8 mg/dl and ALT 62 U/L, AST 138 U/L, GGT 202 U/L. Conclusions: Biliary atresia can occur in premature infants despite being primarily a disease of full-term newborn infants. The insidious onset of direct hyperbilirubinemia in the premature infant may overlap with the duration of physiologic jaundice, sepsis or TPN related cholestatsis. (Both of these infants were on TPN for a short period). Despite the small size of the pre-term infant sometimes making it impossible to perform a percutaneous liver biopsy, the same aggressive approach to evaluate direct hyperbilirubinmia in full-term infants should apply to preterm infants to prevent delay in diagnosis. Open liver biopsy with intraoperative cholangiogram is a safe method to evaluate premature infants with direct hyprbilirubinemia.
AJG – Vol. 96, No. 9, Suppl., 2001
768 Elevation of liver enzymes in pediatric oncology patient: a case report Samra Sarigol-Blanchard M.D.1*, Kemal Sarman M.D.2, Metin Vural M.D.1 and Leyla Agaoglu M.D.1. 1Pediatric Gastroenterology, Hematology and Radiology, American Hospital, Istanbul, Turkey; and 2 Sarman Pathology Laboratories, Istanbul, Turkey. Purpose: We report a 3-year-old girl diagnosed with infantile Acute Lymphocytic Leukemia at age 3 months who presented with a six month history of elevated liver enzymes. Results: At the time of presentation she was in remission but required intense high dose maintenance chemotherapy including vincristine, cyclophosphamide, high dose methoteraxate, 6-MP, thioguanine and cytosine arabinoside. The physical exam revealed a pleasant girl with significant growth delay. The abdominal exam was remarkable for hepatosplenomegaly with no ascites. Laboratory studies at the time of presentation demonstrated an albumin of 4.2 g/dL, total bilirubin 6.1 mg/dL, direct bilirubin 3.6 mg/dL, aspartate aminotransferase 54 IU/L, alanine aminotransferase 236 IU/L, glutamyl transferase 1187 IU/L, and a protrombin time of 10.2 sec. Serologic tests for viral and autoimmune disease were negative. An Ultrasound of the abdomen revealed moderate hepatomegaly and splenomegaly with no evidence of ascites or enlarged lymph nodes. A percutaneous liver biopsy was performedand was remarkable for the the presence of significant hemosiderosis in macrophages, Kupffer cells and hepatocytes. Although there was a slight increase in collagen deposition particularly in the portal regions, the limiting plates were intact with no evidence of fibrosis. Iron studies and an MRI of the abdomen performed after the liver biopsy were remarkable for a significantly elevated ferritin level with a transferrin saturation of 60%. An MRI of the abdomen showed diminished signal intensity on T2-weighted images of liver and spleen with diffuse hepatosplenomegaly. Desferoxamine treatment rather than phlebotomy was initiated following consultation with her pediatric oncologist. Conclusions: Abnormal liver function tests are common in Acute Lymphocytic Leukemia patients and are usually attributed to hepatotoxic chemotherapy agents, viral hepatitis or recurrence of disease in the liver. It is important to remember that patients, receiving high dose chemotherapy, may also require significant transfusions of packed red blood cells. Therefore, in addition to the routine evaluation of elevated liver enzymes in such a patient, iron studies, liver biopsy and possibly an MRI of the abdomen, a useful, non-invasive diagnostic tool for evaluation of iron deposition should be considered.
769 Unusual endoscopic and histologic findings in teenagers presenting with constipation and rectal bleeding Samra Sarigol-Blanchard M.D., Gisela Chelimsky M.D., Judy Splawski M.D., Fred Rothstein M.D. and Steve Czinn M.D.*. 1Pediatric Gastroenterology, Case Western Reserve University, Cleveland, OH, United States. Purpose: Constipation is a common symptom in pediatric population and usually never requires an extensive work-up. We present two patients who presented with constipation and rectal bleeding and had an uncommon pathology in their colonoscopic evaluation. Results: A 14 year-old male presented with an 8-month history intermittent rectal bleeding with and without stooling. The past medical history was remarkable for intermittent episodes of constipation. On physical exam the perianal area was normal and the rectal exam was notable for grossly bloody mucous with no palpable lesions. Laboratory evaluation was remarkable only for an elevated sedimentation rate. Colonoscopy revealed raised mucosal folds with swollen, edematous, friable ulcerated mucosa at 10 cm from the anal verge. The biopsies demonstrated focal ulcerations and changes indicative of a solitary rectal ulcer syndrome. Despite the benign nature of the biopsy a CT of the abdomen and pelvis was performed to rule