Papers by William Berquist
Transplantation, 2014
small-for-size problems in LDLT, or long-lasting intractable infections, will be those. Maternal ... more small-for-size problems in LDLT, or long-lasting intractable infections, will be those. Maternal microchimerism is proposed as the etiologic factor for BA, but mother as the donor did not infl uence the result of LDLT for BA. Conclusion: Current result of LTx for BA in Japan is satisfactory in the unique background, in contrast to western countries. The reason of the relatively poor outcome of older patients should be clarifi ed to improve the result more.
PubMed, Jul 28, 2015
Background: In solid organ transplant patients, non-participation in all aspects of the medical r... more Background: In solid organ transplant patients, non-participation in all aspects of the medical regimen is a prevalent problem associated with adverse consequences particularly in the adolescent and young adult (AYA) age group. This study is the first to evaluate the feasibility, utility and impact of a text messaging (TM) intervention to improve participation in laboratory testing in adolescent liver transplant patients. Methods: AYA patients, aged 12 to 21 years, were recruited for a prospective pilot trial evaluating a TM intervention delivered over a 1-year period. The intervention involved automated TM reminders with feedback administered according to a prescribed laboratory testing frequency. Participation rate in laboratory testing after the intervention was compared to the year prior. Patient responses and feedback by text and survey were used to assess feasibility, acceptability and use of the intervention. Results: Forty-two patients were recruited and 33 patients remained enrolled for the study duration. Recipients of the TM intervention demonstrated a significant improvement in participation rate in laboratory testing from 58% to 78% (P<.001). This rate was also significantly higher than in non-intervention controls (P=.003). There was a high acceptability, response rate and a significant correlation with reported versus actual completion of laboratory tests by TM. Conclusions: TM reminders significantly improved participation in laboratory testing in AYA liver transplant patients. The intervention demonstrated feasibility, acceptability, and use with a high proportion of patients who engaged in and perceived a benefit from using this technology.
Pediatric Transplantation, Jun 14, 2014
In the majority of children with ALF, the etiology is unknown and liver transplantation is often ... more In the majority of children with ALF, the etiology is unknown and liver transplantation is often needed for survival. A patient case prompted us to consider that immune dysregulation may be the cause of indeterminate acute hepatitis and liver failure in children. Our study includes nine pediatric patients treated under a multidisciplinary clinical protocol to identify and treat immune-mediated acute liver injury. Patients with evidence of inflammation and no active infection on biopsy received treatment with intravenous immune globulin and methylprednisolone. Seven patients had at least one positive immune marker before or after treatment. All patients had a CD8+ T-cell predominant liver injury that completely or partially responded to immune therapy. Five of the nine patients recovered liver function and did not require liver transplantation. Three of these patients subsequently developed bone marrow failure and were treated with either immunosuppression or stem cell transplant. This series highlights the importance of this tissue-based approach to diagnosis and treatment that may improve transplant-free survival. Further research is necessary to better characterize the immune injury and to predict the subset of patients at risk for bone marrow failure who may benefit from earlier and stronger immunosuppressive therapy.
Journal of Pediatric Gastroenterology & Nutrition, 2021
BACKGROUND Endoscopic retrograde cholangiopancreatography (ERCP) is a fluoroscopy and endoscopy-b... more BACKGROUND Endoscopic retrograde cholangiopancreatography (ERCP) is a fluoroscopy and endoscopy-based procedure important for diagnosis and management of pediatric pancreaticobiliary disorders. Patient, procedure, endoscopist and facility characteristics have been shown to influence ERCP complexity and procedure outcomes as well as fluoroscopy utilization in adults, however the extent to which this is true in pediatric patients remains under-studied and there are minimal data regarding fluoroscopy utilization in pediatric ERCP. METHODS We retrospectively analyzed ERCPs performed on patients < 18 years of age at our tertiary care children's hospital from 2002-2017 using our institution's paper and electronic medical record system along with a prospectively-maintained radiation exposure database. Procedure complexity was graded using the Stanford Fluoroscopy Complexity Score and the American Society of Gastrointestinal Endoscopy Complexity scale. High volume endoscopists (HVE) were defined as having a cumulative annual ERCP volume > 100 and low volume endoscopists (LVE) as < 100 (pediatric + adult) ERCPs/year. RESULTS 385 ERCPs performed on 321 patients were included in this analysis. The mean patient age was 13.4 years (+/- 4.2 years), 77% were index ERCPs (native ampullas) and 81% were performed with therapeutic intent (87% for biliary indication and 13% for pancreatic indication). Fluoroscopy times varied dramatically between procedures and providers. Median fluoroscopy time was 4.85 (+/- 2.68) minutes. Endoscopist annual ERCP volume was the strongest predictor of fluoroscopy time (p < 0.001). In addition to endoscopist volume, procedure-specific predictors of increased fluoroscopy time included pancreatic indication for the procedure, biliary or pancreatic duct stricture, patient age < 4 years or > 16 years at the time of ERCP (p < 0.01 for each), and native ampulla. ERCP complexity rating based on the Stanford Fluoroscopy Complexity Score correlated with fluoroscopy time. CONCLUSIONS Radiation exposure is higher than desirable for pediatric ERCP and varies with endoscopist as well as patient and procedure-specific factors. HVE perform ERCP with lower fluoroscopy time relative to LVE even though HVE procedure complexity was higher. The Stanford Fluoroscopy Score predicted fluoroscopy time for pediatric ERCP, but the ASGE ERCP complexity scale did not. Adaptation and refinement of pediatric-specific ERCP complexity scales including factors such as patient size and age and indications/interventions more consistent with those encountered in pediatrics could be beneficial.
Pediatrics, 1983
Clinical features, radiographic and esophageal manometry findings, and treatment results in 16 pa... more Clinical features, radiographic and esophageal manometry findings, and treatment results in 16 patients less than 15 years old with achalasia are described. Esophageal manometry performed in 15 patients showed results similar to those found in adults: (1) increased resting lower esophageal sphincter pressure, (2) incomplete or failure of relaxation of the lower esophageal sphincter on swallowing, and (3) ineffective or absence of peristalsis in all. The most common symptoms in the 16 patients were: dysphagia in 15, postprandial vomiting in 13, and retrosternal pain in five. The average duration from onset of symptoms to diagnosis was 28 months. The esophagram was diagnostic in all patients. Pneumatic dilation was the initial treatment in eight and was successful for more than 1 year in five. Two patients required two dilations and were then symptom-free for more than 1 year, but required a Heller myotomy. The remaining patients underwent Heller myotomy following failure of the secon...
Blood, 2006
Following liver transplantation, patients require immunosuppressive medications to prevent graft ... more Following liver transplantation, patients require immunosuppressive medications to prevent graft rejection. However, these drugs can have significant side effects including severe anemia. Tacrolimus is now commonly used in pediatrics patients after liver transplantation. Case reports indicate that tacrolimus rarely can cause microangiopathic hemolytic anemia and also hemolytic anemia due to cold reactive IgM antibodies (cold agglutinin disease). We have seen 4 patients with symptomatic cold agglutinins thought to have been triggered by tacrolimus. Four patients, 6 to 26 months post-ABO-compatible liver transplant, presented with severe anemia (hemoglobin < 6g/dL), indirect hyperbilirubinemia, reticulocytosis and low serum haptoglobin (Table 1). Peripheral blood smear exhibited marked autoagglutination but no evidence of microangiopathic changes. The direct antiglobulin test (DAT) initially was strongly positive in all 4 patients (Table 2). After warm saline washes, the DAT remain...
Molecular Genetics and Metabolism, 2019
et al., Long-term safety and efficacy of glycerol phenylbutyrate for the management of urea cycle... more et al., Long-term safety and efficacy of glycerol phenylbutyrate for the management of urea cycle disorder patients, Molecular Genetics and Metabolism,
Digestive Diseases and Sciences, 2017
A 4-week-old ex-33 male infant, born to a 27-year-old G3P3 Ethiopian mother through normal vagina... more A 4-week-old ex-33 male infant, born to a 27-year-old G3P3 Ethiopian mother through normal vaginal spontaneous delivery, was transferred to our facility for liver transplant evaluation. His overall birth was unremarkable except for mild transient tachypnea of the newborn and a brief oxygen requirement. Laboratory studies were notable at onset for significant thrombocytopenia (platelets 10 K/lL) requiring ongoing platelet transfusions; hepatitis (AST 500 U/L, ALT 90 U/L); stable coagulopathy (International normalized ratio [INR] 1.9); cholestasis (total bilirubin 24.5 mg/dL, direct bilirubin 12.4 mg/dL); mild hypoalbuminemia (3.2 g/dL); and hyperammonemia (70-100 lmol/L). The infant was otherwise stable and off pressors or antibiotics. Social and family histories were unremarkable except for parental Ethiopian descent, without consanguinity. Ursodiol, lactulose, and multivitamin drops were prescribed for the presumed liver failure. His examination was notable for jaundice and a very prominent raised ''blueberry muffin'' rash that was present throughout the face and body (Fig. 1). His abdomen was distended with a liver edge palpable 6 cm below the right costal margin and spleen palpable to 5 cm. The infant was arousable and crying, with no clear facial dysmorphism. He
Journal of pediatric gastroenterology and nutrition, Jan 21, 2018
Molecular genetics and metabolism, Mar 1, 2018
Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia durin... more Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death. Long-term prognosis despite optimal pharmacological and dietary therapy is still poor. The combination of intravenous sodium phenylacetate and sodium benzoate (Ammonul®) can eliminate nitrogen waste independent of the urea cycle. We report attempts to improve outcomes for males with severe ornithine transcarbamylase deficiency (OTCD), a severe X-linked condition, via prenatal intravenous administration of Ammonul and arginine to heterozygous carrier females of OTCD during labor. Two heterozygote OTCD mothers carrying male fetuses with a prenatal diagnosis of OTCD received intravenous Ammonul, arginine and dextrose-containing fluids shortly before birth. Maintenance Ammonul and arginine infusions and high-caloric enteral nutrition were started immediately after birth. Ammonul metabolites were measured in umbi...
Digestive Diseases and Sciences, 2017
A 5-month-old female was observed to have massive hematemesis 48 h after surgical closure of a pa... more A 5-month-old female was observed to have massive hematemesis 48 h after surgical closure of a patent ductus arteriosus. Her prior medical history was significant for: intrauterine growth retardation, with a birth weight of 2 kg; delivery through a scheduled Cesarean section due to breech presentation; and a maternal history of antiphospholipid antibodies and thrombophilia with six prior miscarriages. An esophagogastroduodenoscopy (EGD) was performed emergently at the bedside; although the examination was limited due to respiratory instability, it was grossly normal except for a large portion of the stomach being obscured by a clot. Although she was initially stabilized, she had an acute drop in hemoglobin four days later. An abdominal ultrasound demonstrated cavernous transformation of the porta hepatis without visualization of portal vein, corroborated by magnetic resonance imaging (MRI), which additionally revealed esophageal and rectal varices and splenomegaly (Fig. 1a). Repeat EGD demonstrated multiple grade 4 varices in the distal esophagus (Fig. 2a). After receiving sclerotherapy to the largest varices, she had no further bleeding and was discharged home. Since an evaluation for hypercoagulability during admission was negative, it was presumed that at some point she may have developed a portal vein thrombus from maternal transfer of antiphospholipid antibodies with subsequent portal hypertension, though no thrombus was ever visualized by imaging. The patient then underwent four scheduled, monthly sclerotherapy sessions in the outpatient setting, without improvement in her varices (Fig. 2b). At age 9 months, two weeks after her last sclerotherapy, she again was in hemorrhagic shock from a massive gastrointestinal bleeding. Given the lack of improvement with sclerotherapy, the decision was made for her to undergo a modified Sugiura procedure (ligation of gastric varices and splenectomy) [1] as well as a proximal spleno-renal shunt. An intraoperative liver biopsy demonstrated moderate steatosis of unknown etiology but was otherwise normal (Fig. 3a). Her postoperative course was significant for prolonged need for oxygenation and aspiration requiring a nasogastric (NG) tube, but otherwise she did well and was discharged with nasal oxygen and NG feedings. A repeat outpatient endoscopy showed the varices greatly decreased in size. As an outpatient, she was noted to have poor weight gain, with intermittent need for NG feedings, as well as supplemental oxygen thought to be secondary to air trapping from her aspiration history. She also underwent scheduled ultrasounds for shunt patency surveillance. When she was 2 years old, she was diagnosed with a liver & Christine Yang [email protected]
Pediatric Transplantation, 2016
Transplantation, Nov 1, 1998
Molecular Genetics and Metabolism Reports, 2016
F1000 - Post-publication peer review of the biomedical literature, 2006
Seminars in pediatric surgery, 1995
The physiological rationale, methodology, and results of biofeedback therapy in the management of... more The physiological rationale, methodology, and results of biofeedback therapy in the management of anorectal disorders in children are presented. Balloon manometry and surface electromyography (EMG) are current biofeedback methods used to teach improved recognition of rectal distension and coordination of muscle function to either maintain fecal continence or to allow proper defecation. These techniques have permitted a success rate of over 70% in the management of children with functional or organic anorectal disease refractory to conventional medical and behavioral therapy. Biofeedback therapy is recommended to assist in the successful rehabilitation of these patients.
NeoReviews, 2001
After completing this article, readers should be able to: 1. Describe the indications for liver t... more After completing this article, readers should be able to: 1. Describe the indications for liver transplantation in neonates and young infants. 2. Delineate major considerations that affect the timing of liver transplantation in young infants. 3. Characterize major medical complications of liver transplantation in young infants and approaches to their treatment. 4. Describe issues unique to the neonate and young infant that affect successful liver transplantation and approaches taken to address these issues. Prior to 1980, liver transplantation in the pediatric age group was characterized by poor outcomes, with high rates of mortality and complications and poor quality of life for the survivors. With improved surgical techniques and immunosuppressive medications, the average survival rate for children has increased to 85% at 1 year and 77% at 5 years after surgery. In addition, complications have become less frequent and are less severe. Because of these advancements, liver transplantation has become standard therapy for life-threatening liver disease in children. Nonetheless, liver transplantation in neonates and young infants remains challenging due to factors such as paucity of donor organs and technical difficulties related to small body size and small graft size. Recent data from United Network of Organ Sharing (UNOS) show that although infants younger than 1 year of age comprise a relatively small group of children waiting on the liver transplant list (<10%), they have the longest waiting times and highest mortality rates while awaiting an organ. This review focuses on the indications, timing, and common complications of liver transplantation pertinent to this high-risk population of children as well as a discussion of recent clinical advances that have resulted in improved outcomes. The most common indications for liver transplantation in the pediatric patient are biliary atresia (43%), metabolic liver disease (13%), and fulminant hepatic failure due to acute hepatic necrosis (11%). Other, less common liver diseases …
Genetics in medicine : official journal of the American College of Medical Genetics, Jan 11, 2014
Purpose:The aim of this study was to examine predictors of ammonia exposure and hyperammonemic cr... more Purpose:The aim of this study was to examine predictors of ammonia exposure and hyperammonemic crises in patients with urea cycle disorders.Methods:The relationships between fasting ammonia, daily ammonia exposure, and hyperammonemic crises were analyzed in >100 patients with urea cycle disorders.Results:Fasting ammonia correlated strongly with daily ammonia exposure (r = 0.764; P < 0.001). For patients with fasting ammonia concentrations…
Digestive Diseases and Sciences, 2014
A 16-year-old previously healthy girl was referred to a community-based pediatric gastroenterolog... more A 16-year-old previously healthy girl was referred to a community-based pediatric gastroenterologist with a 6-month history of epigastric pain, nausea, and nonbilious emesis. The pain and vomiting worsened with eating, occurred mostly at nighttime, but did not wake her from sleep. During this time, she also complained of abdominal distention, bloating, and heartburn. Two months prior to the onset of these symptoms, she experienced a presumed viral illness with fever, sore throat, and fatigue lasting 3 days. Testing for streptococcal pharyngitis and infectious mononucleosis was negative at that time. Her past medical history was only significant for migraine headaches. She was an honor student and cheerleader at a competitive high school. Her physical examination was normal as were a comprehensive metabolic panel, measurement of serum inflammatory markers, and serum amylase and lipase levels. A normal abdominal ultrasound and a gastric emptying study with markedly prolonged gastric emptying time with (79 % retention after 240 min [normal\ 30 %]) were obtained as part of her initial evaluation, for which she was prescribed erythromycin, ondansetron, and small frequent meals. After 4 weeks adhering to this regimen, minimal symptom improvement was noted; coffee ground emesis also became evident. An endoscopy revealed a stomach filled with small pieces of food, a narrowed antrum with a nodular mass extending into the pylorus with a 1-cm-wide cavity in the inferior curve of the antrum and an enlarged mound proximal to the antral mass (Fig. 1a–c). The pyloric channel was only wide enough to allow the passage of an Olympus N30 ultrathin endoscope (OD 5.3 mm). No other abnormalities of the duodenal bulb and duodenum were noted although the examination was limited. Histologic examination of tissue obtained by a grasp forceps biopsy of the mass was reported as a mild increase in lymphoplasmocytic cells in the lamina propria of the antrum and degenerative bacterial forms suggestive of Helicobacter pylori (H. pylori). She was treated for H. pylori for 2 weeks with triple therapy (amoxicillin, clarithromycin, and omeprazole), but she continued to have persistent abdominal pain and nausea. Due to minimal improvement in her symptoms after receiving medical management, surgical intervention was considered. Endoscopy 6 weeks after the initial procedure was unchanged with no evidence of H. pylori. She underwent exploratory laparotomy due to her endoscopic findings and clinical symptoms of gastric outlet obstruction, which revealed a very firm circumferential mass measuring 1.5 cm 9 1.5 cm 9 1.5 cm extending from the distal antrum through the pylorus to the proximal duodenum. The mass was resected with reconstruction using a Billroth II loop gastrojejunostomy. Submucosal pancreatic tissue was apparent when the mass was examined histologically, consistent with Type I pancreatic heterotopia (Fig. 2). Modest symptom improvement was apparent immediately postoperatively, although she continued to experience less severe but persistent nausea and epigastric abdominal pain, which has been managed with lifestyle modification including small, frequent meals, and ondansetron. FollowA. A. Rodriguez (&) W. Berquist Division of Pediatric Gastroenterology, Hepatology and Nutrition, Lucile Packard Children’s Hospital, Stanford University Medical Center, Stanford, CA, USA e-mail: [email protected]
Pediatric Transplantation, 2012
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Papers by William Berquist