Hepatopulmonary syndrome associated with nodular regenerative hyperplasia after liver transplantation in a child

HPS is a significant complication of portal hypertension in children with chronic liver disease and is an established indication for LT. It is characterized clinically by the triad of pulmonary vascular dilatation causing hypoxemia in the setting of advanced liver disease. NRH, a cause of non‐cirrhotic portal hypertension, is characterized by diffuse benign transformation of the hepatic parenchyma into small regenerative nodules with minimal or no fibrosis. Development of NRH and HPS in pediatric LT recipients has not been reported, although occasional cases have been reported in adult LT recipients. In this report, we discuss a case of a three‐yr‐old male who developed HPS, two yr after LT. Pulmonary and cardiac causes for hypoxemia were ruled out by appropriate investigations including a chest X ray, echocardiogram, cardiac catheterization, and a CT angiographic study. The diagnosis of HPS was confirmed via bubble echocardiogram that demonstrated intrapulmonary shunting. Open liver biopsy revealed marked NRH. The patient underwent liver retransplantation that resulted in complete reversal of his pulmonary symptoms and normal oxygen saturations within three months after LT.     

Pediatric living donor liver transplantation for biliary atresia with hepatopulmonary syndrome: the gift of a second wind

Purpose

Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13?C47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe hypoxemia before transplantation, but the course of HPS after living donor liver transplantation (LDLT), especially for biliary atresia (BA) patients is not well established.

Patients and methods

The present study evaluated 122 patients who received an LDLT for BA and of these, 3 patients had HPS at the time of LDLT in a single-center series.

Results

Two patients of the HPS patients them had biliary and/or vascular complications, but they recovered uneventfully with interventional treatment. None of the patients required supplemental oxygen and had no residual cardiopulmonary abnormalities at a follow-up of more than 24?months.

Conclusion

Although a series of three patients is too small for definitive conclusion and further investigations must be conducted, pediatric LDLT can be a favorable therapeutic option for HPS.     

Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post‐liver transplant respiratory recovery

Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre‐ or post‐liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post‐operative recovery.     

Pediatric split liver transplantation after Fontan procedure in left isomerism combined with biliary atresia: A case report

LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI and CHD is a challenging issue for a transplant surgeon. Herein, we report a successful split LT on a three‐yr‐old boy with LI who survived after a Fontan procedure due to single ventricle, but who suffered from HPS associated with BA.     

Use of airway pressure release ventilation in a child with refractory hepatopulmonary syndrome after liver transplantation

HPS is a life‐threatening condition in patients with end‐stage liver disease, in which intrapulmonary vascular dilatations result in intrapulmonary shunts and hypoxemia. The only successful treatment is liver transplantation. Hypoxemia may be severe prior to transplantation; however, it can worsen or become refractory after liver transplantation and result in increased post‐operative mortality. Here, we present the case of a 10‐month‐old female infant with progressive end‐stage liver disease and severe HPS, who developed refractory hypoxemia after a successful liver transplantation. After 19 days of unsuccessful attempts to reverse the hypoxemia using conventional mechanical ventilation and HFOV, the patient responded dramatically to APRV, with rapid improvement in her PaO₂ and sharp decline in her OI. She was able to begin weaning from APRV two days later and was extubated within seven days. APRV was successful in treating refractory hypoxemia in this patient with severe HPS after liver transplantation, possibly by modifying distribution of pulmonary blood flow. Although we cannot rule out coincidental natural resolution of the HPS, APRV could be a useful rescue therapy in patients with HPS and refractory hypoxemia.     

Nitric oxide for post‐liver‐transplantation hypoxemia in pediatric hepatopulmonary syndrome: Case report and review

Schiller O, Avitzur Y, Kadmon G, Nahum E, Steinberg RM, Nachmias V, Schonfeld T. Nitric oxide for post‐liver‐transplantation hypoxemia in pediatric hepatopulmonary syndrome: Case report and review.
Pediatr Transplantation 2011: 15: E130–E134. © 2010 John Wiley & Sons A/S. Abstract: HPS is rare in the pediatric population. Liver transplantation is the ultimate treatment for severe HPS. There are only a few case reports and one series of children in whom HPS was the main indication for liver transplantation. Outcome was good in most of them, with full regression of the pulmonary process. However, hypoxemia in the early post‐operative course can have severe consequences, and effective treatment modalities are needed. There are rare instances of the use of iNO for the treatment of post‐operative hypoxemia. We describe a 10.5‐yr‐old boy with severe HPS owing to chronic liver disease after bone marrow transplantation. Liver transplantation from a living related donor (the same sister who donated the bone marrow) was complicated by severe hypoxemia on POD 2. iNO was administered via the ventilator circuit and, after extubation, through nasal prongs. It was slowly tapered down and stopped on POD 10. The child had an otherwise uneventful course and was discharged home on POD 21 with normal oxygen saturation. Liver transplantation should be offered to children with severe HPS. iNO can reverse the hypoxemia that may occur after the operation.     

Successful liver retransplantation for recurrent hepatopulmonary syndrome

HPS is defined as arterial hypoxemia because of pulmonary vasodilation as a result of cirrhotic or non-cirrhotic portal hypertension. This report describes a teenager with HPS because of primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome requiring OLT. HPS resolved completely within three months of OLT, but recurred again at 12 months post-OLT following liver dysfunction secondary to a biliary stricture. She underwent a second OLT successfully and remains well two yr and three months post-second OLT. Recurrent HPS after OLT may occur because of graft dysfunction and as this novel case illustrates, retransplantation may lead to a successful outcome.     

Liver transplantation in infants and children. Evaluation of the first 40 cases (March 1991-March 1997)]

BACKGROUND: Liver transplantation (LT) is the treatment of end-stage liver disease in children. We report our experience with LT using grafts from living related (LRD) and cadaver donors (CD). POPULATION: From March 1991 to March 1997, 40 children and infants received a total of 42 liver grafts. A reduced-size liver was used in 28 cases. We studied pre-transplantation status, survival rate, and medical and surgical complications in these patients. RESULTS: The survival rate in our series was respectively 85 and 80% at 1 and 7 years after LT. Low weight infants required a prolonged ventilatory assistance. Five of the six deaths noticed during the first three months after LT occurred in children weighing less than 12 kg. One year after LT, no significant difference in the incidence of rejection was found, neither between low-weight children and the others, nor between patients transplanted from CD or LRD. Biliary tract stricture was the major surgical complication. CONCLUSION: This series consisted of a majority of low-weight children. The survival rate in the patients weighting less than 12 kg is lower than in the others. This may be explained by the nutritional status of these patients and early postsurgical complications. The use of grafts from living donors offers more flexibility since the operation is performed electively, but it did not seem to modify the incidence of acute rejections and surgical complications.     

Efficacy of plasma exchange in children with severe hemophagocytic syndrome: a prospective randomized controlled trial北大核心CSCD

目的 探讨血浆置换在辅助救治儿童重症噬血细胞综合征（hemophagocytic syndrome,HPS）中的疗效和应用价值。 方法 采用前瞻性随机对照研究方法,选取2018年10月至2020年10月在湖南省儿童医院儿童重症监护室（pediatric intensive care unit,PICU）接受救治的重症HPS患儿40例为研究对象,将患儿随机分为置换组和常规组（n=20）,常规组患儿进行病因和对症常规支持治疗,置换组在常规治疗的基础上加以血浆置换辅助治疗。对两组患儿的基本情况、治疗前后的临床症状体征、主要实验室检查指标、疗效和预后情况进行比较分析。 结果 两组患儿治疗前在性别、年龄、入院前病程、病因构成、小儿危重病例评分、器官或系统功能累及情况等指标上比较差异均无统计学意义（P>0.05）,在实验室指标的比较上差异亦均无统计学意义（P>0.05）。两组患儿治疗7 d后,临床症状体征均有所缓解和改善。治疗后置换组C反应蛋白、降钙素原、血清铁蛋白、丙氨酸氨基转移酶、总胆红素水平均低于常规组（P<0.05）。置换组的PICU住院时间较常规组明显缩短,总有效率较常规组明显提高（P<0.05）,但两组总的住院时间和3个月病死率比较差异无统计学意义（P>0.05）。 结论 血浆置换在辅助治疗儿童重症HPS上的疗效优于常规治疗,能改善患儿的临床症状体征和部分实验室指标,缩短PICU住院时间,可能成为治疗儿童重症HPS的一项有效辅助治疗方法。     

Liver transplantation for homozygote familial hypercholesterolemia: the only curative treatment

FH is an autosomal dominant genetic disorder characterized by increased TC and LDL level, which leads to xanthomas, atherosclerosis, and cardiac complications even in childhood. The treatment options are diet, medical treatment, lipid apheresis, and LT. The aim of our study was to analyze our data of patients with FH. Between 2004 and 2015, there were 51 patients who underwent pediatric LT at our center. All patients with FH were identified, and the data were retrospectively analyzed. There were eight patients with homozygous FH in the median age of 10 years (IQR 6–12) who underwent LT. The median pre‐operative TC and LDL levels were 611 mg/dL (IQR: 460–844) and 574 mg/dL (IQR: 398–728) and decreased to normal levels 1 week after LT (TC: 193 mg/dL and LDL: 141 mg/dL). Two patients died two and 18 months after LT due to sudden cardiac arrest. Both patients were diagnosed with cardiovascular disease pre‐operatively. The LT is the only curative treatment for this disease. To achieve an excellent outcome, it should be performed before the development of cardiovascular disease, because the regression of severe cardiovascular disease after transplantation is limited.     

Current role of liver transplantation for methylmalonic acidemia: a review of the literature

Abstract:  To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six. Three hospital mortalities were noted, because of metabolic decompensation, sepsis and aspergillosis. Although mean postoperative serum MMA level decreased to 13.8% ± 9.2% (range 1.25–26.1%) of preoperative levels, four patients (22.2%) had renal insufficiency after isolated LT and three (16.7%) had postoperative neurological disability. Continuing metabolic damage to the kidney and brain may occur even after successful LT. Further evaluation is required to determine the long-term suitability of this treatment modality.     

Plasma Big Gastrin and Gastric Secretion in Newborns and Infants:Part 2: Plasma Big Gastrin and Gastric Secretion in Hypertrophic Pyloric Stenosis

To elucidate the etiological role of big gastrin in hypertrophic pyloric stenosis (HPS), the pattern of plasma gastrins, the response of plasma big gastrin aftermilk feeding and the gastric secretion were investigated in 22 infants with HPS. In addition, histochemical study of the antral gastrin cells was carried out. The pattern of plasma gastrins in HPS was closely similar to that in the normal infant and big gastrin was a dominant component of circulating gastrins. The mean big gastrin level in HPS was significantly higher than that of the controls both fasting and after feeding. The HPS also had hypersecretion, of gastric juice, acid and pepsin before and after pentagastrin stimulation, Therefore, the HPS had both hypersecretion of gastric acid and hypergastrinemia. The number and the distribution of gastrin cells in the antral mucosa in HPS were similarly found by immunostainings with two antisera, anti-big gastrin serum and anti-gastrin serum. These gastrin cells were not different in number from those of infants and children with other diseases. These data suggest that the HPS has an incomplete negative feedback mechanism between plasma gastrin level and gastric acid secretion, and generally has hypergastrinemia whose trophic effect may play a role in the pathogenesis of this disease     

Living donor liver transplantation for ornithine transcarbamylase deficiency

Ornithine transcarbamylase deficiency, the most common urea cycle disorder, causes hyperammonemic encephalopathy and has a poor prognosis. Recently, LT was introduced as a radical OTCD treatment, yielding favorable outcomes. We retrospectively analyzed LT results for OTCD at our facility. Twelve children with OTCD (six boys and six girls) accounted for 7.1% of the 170 children who underwent LDLT at our department between May 2001 and April 2010. Ages at LT ranged from nine months to 11 yr seven months. Post-operative follow-up period was 3-97 months. The post-operative survival rate was 91.7%. One patient died. Two patients who had neurological impairment preoperatively showed no alleviation after LT. All patients other than those who died or failed to show recovery from impairment achieved satisfactory quality-of-life improvement after LT. The outcomes of LDLT as a radical OTCD treatment have been satisfactory. However, neurological impairment associated with hyperammonemia is unlikely to subside even after LT. It is desirable henceforth that more objective and concrete guidelines for OTCD management be established to facilitate LDLT with optimal timing while avoiding the risk of hyperammonemic episodes.     

Pediatric liver transplantation for neonatal‐onset Niemann‐Pick disease type C: Japanese multicenter experience

Niemann‐Pick disease type C (NPC) is a rare autosomal recessive inherited disease characterized by lysosomal accumulation of free cholesterol in macrophages within multiple organs. Infantile‐onset NPC often presents with jaundice and hepatosplenomegaly from birth, but these symptoms usually improve during early childhood, and it rarely progresses to liver failure. We report three cases from different hospitals in Japan; the patients developed neonatal‐onset NPC, and liver transplantation (LT) was performed as a life‐saving procedure. LT was performed at 19 days, 59 days, and 4 months of age, respectively. The last patient was diagnosed with NPC before LT, while the first two patients were diagnosed with neonatal hemochromatosis at LT. In these two patients, the diagnosis of NPC was made more than a year after LT. Even though oral administration of miglustat was started soon after the diagnosis of NPC, all patients showed neurological regression and required artificial respiratory support. All patients survived more than one year after LT; however, one patient died due to tracheal hemorrhage at 4.5 years of age, and another one patient was suspected as recurrence of NPC in liver graft. In conclusion, while LT may be a temporary life‐saving measure in patients with neonatal‐onset NPC leading to liver failure, the outcome is poor especially due to neurological symptoms. A preoperative diagnosis is thus critical.     

Brain abscess in hepatopulmonary syndrome associated with biliary atresia

The first‐choice therapy for biliary atresia (BA) is Kasai hepatoportoenterostomy, which has been shown to greatly improve outcome. Various long‐term complications, however, such as portal hypertension and hepatopulmonary syndrome (HPS), can occur in patients with native liver. A rare case of brain abscess in an 11‐year‐old girl with HPS associated with BA is reported. The patient underwent hepatoportoenterostomy for BA at 53 days of age, with resolution of hyperbilirubinemia. At 10 years of age, she was diagnosed with severe HPS with right‐to‐left shunting, and preparations for liver transplantation proceeded. Three months after the diagnosis, she had a right parietal brain abscess. Given that the brain abscess enlarged in size, surgical drainage of the brain abscess was performed. The postoperative course was uneventful, but a slight left hemiplegia remained at discharge. The presumed mechanism of abscess formation in HPS may be right‐to‐left bacterial transit through intrapulmonary vascular dilatations and/or arteriovenous fistulae.     

Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child

PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein-losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the jejunal loop used for the cholangiojejunostomy. Diarrhea resolved rapidly after surgery. He remained well after six months following biliary diversion, with normal stool output and no protein loss. We documented a dramatic improvement of graft steatosis at histology as well as normalization of liver function test. EBD can be considered a valuable treatment option to avoid organ disfunction and loss in PFIC 1 transplanted patients who develop graft steatohepatitis.     

Pretransplant trends in α‐fetoprotein levels as a predictor of recurrence after living donor liver transplantation for unresectable hepatoblastoma: A single‐institution experience

LT is a practical therapeutic alternative for unresectable hepatoblastoma; however, deciding when to perform LT is difficult. The aim of this study was to optimize the timing of LT for hepatoblastoma using pretransplant trends in AFP levels. Trends in pretransplant AFP levels and their influence on post‐transplant outcomes were retrospectively evaluated. All patients who underwent living donor LT for hepatoblastoma in our institution since 2002 were included. Variables analyzed included history of prior tumor resection, pretransplant AFP responses to chemotherapy, metastatic disease at diagnosis, and post‐transplant chemotherapy. Eight patients (seven boys and one girl; median age, 35 months; range, 15 months‐12 years) were transplanted. The overall post‐transplant recurrence‐free survival rate was 62.5% (5/8) with a mean follow‐up of 77 months. Patients with post‐transplant recurrence showed a 0.573 log increase in AFP levels after the last chemotherapy session before LT. This was significantly higher than the 0.279 log decrease observed in patients without post‐transplant recurrence (P = .024). Because the AFP response cannot be accurately predicted before each cycle of chemotherapy, it may be appropriate to perform LT when AFP levels do not decrease after the last cycle and before they are found to be elevated again.     

Kwalifikacja chorych na mukowiscydozę do transplantacji płuc – doświadczenia własne

Background

Lung transplantation is a recognized treatment option for selected patients with cystic fibrosis in the end stage of bronchopulmonary disease. This is, however, a major challenge associated with the right choice of recipients proceedings before, around and after surgery, many complications and psychosocial factors.

Materials and methods

This article presents six patients aged 14–19 years who have been referred for LT from the Institute of Mother and Child. The eligibility criteria, the risk factors of death, which must be taken into account in the care of patients in the end stage of bronchopulmonary disease, were analyzed.

Results

Six patients out of 350 treated in the Outpatient Clinic of Cystic Fibrosis were qualified for the lung transplantation from 2008 to 2012. One patient died 22 months after LT, the remaining survival time is between 2 and 5 years. They are in good general condition under the strict control of transplantation centers.

Conclusions

The exact determination of the time to put a CF patient on the waiting list for transplantation is very difficult. But it is also of paramount importance to minimize mortality before and maximize survival after LT. Multidisciplinary team experienced in the treatment of CF, caring for a patient with transplant centre should make such difficult decisions. The principle of treatment of patients with CF before LT is to maximize all available and relevant aspects of care. The procedure should be tailored to the patient's needs and capabilities of the center.     

CT findings in unilateral hepatopulmonary syndrome after the Fontan operation

Background  Patients with complex congenital heart defects palliated by connecting the systemic veins directly to the pulmonary circulation are known to develop hepatopulmonary syndrome (HPS). Although rare, HPS can develop following the Fontan operation. Objective  To present and analyse the CT findings of HPS in patients with a Fontan circulation. Materials and methods  From May to December 2005, six patients with HPS following the completion of a Fontan circulation were evaluated. CT findings were reviewed and were compared with angiographic findings. Results  All six patients showed unilateral involvement. All patients except one had inferior vena cava (IVC) interruption with azygos continuation. CT scans showed abnormal vascular dilatation in one lung, and properly demonstrated the anatomy causing the hepatic venous blood to flow preferentially into one lung. These CT findings correlated well with the angiography findings. Conclusion  HPS that develops after the Fontan procedure is typically unilateral and is often associated with IVC interruption and azygos or hemiazygos continuation. CT demonstrates dilatation of pulmonary vessels in the affected lung and may be able to demonstrate the underlying anatomical cause for the predilection of hepatic venous flow to the contralateral lung.