Infantile giant cell hepatitis with autoimmune hemolytic anemia

Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.

A Study of the Mechanism of Yinzhihuang（菌栀黄）Injection in the Treatment of Infantile Hepatitis Syndrome

The content of serum total bilirubin, serum alanine aminotransferase and hepatic enlarge-ment were observed before and affer the treatment for intantile hepatitis syndrome by using injections ofYinzhihuang. Peripheral blood T cell subproportions and levels of duodenal bilirubin were also detected insome patients. The results of this study suggest the injections of Yinzhihuang had a significant effect in re-solving jaundice, but no effect in improving the disturbance of cellular immunity.

Current State of Clinical Diagnosis and Treatment of Infantile Cytomegaloviral Hepatitis

Cytomegaloviral hepatitis is an infantile liver disease commonly encountered in China, which could be differentiated into 4 patterns with different clinical conditions. Along with the progress of laboratory diagnostic techniques, multiple diagnostic approaches are available for this disease, but accurate diagnosis can only be made when individual patients＇ realities are taken into consideration. Clinical treatments are various, and the Western medicine used is mainly anti-viral agents such as Ganciclovir, and so far no unified therapeutic program has been formed. More and more ways of regarding Chinese medicine treatment of cytomegaloviral hepatitis have been published increasingly in recent years, though further research to seek preferable treatment programs is still expected.

Clinical Observations on Treatment of Infantile Cholestatic Hepatitis Syndrome by Rhubarb

Objective: To evaluate the role of rhubarb in treating infantile cholestatic hepatitis syndrome (ICHS).Methods: Forty-four patients of the treatment group were treated with rhubarb taken orally and (or) by duodenal infusion, 20 cases of the control group were treated with general liver protecting therapy such as inosine, vitamin C and glucose. Serum bilirubin, alanine aminotransferase and γ-glutamyl transpeptidase level, bilirubin in duodenal juice and liver size were observed before and after treatment.Results: In the treatment group, 18 cases were markedly effective, 21 effective, 3 ineffective and 2 deteriorated, while in the control group, the respective numbers were 0, 4, 13 and 3. Conclusion: Rhubarb was markedly effective in cholagoging and abating jaundice, it could promote the recovery of liver function, dredge the cholestasis in cholangiole and micro-cholangiole so as to promote the bile secretion.

Pediatric vascular tumors of the liver:Review from the pathologist’s point of view

Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature,histologic overlap and the rarity of some entities.Here we give an up-to-date overview of the most important entities.We discuss the clinic,histology and pathophysiology of hepatic congenital and infantile heman-gioma,hepatic epithelioid hemangioendothelioma and hepatic angio-sarcoma.

A Homozygotic Mutation in KDSR may Cause Keratinization Disorders and Thrombocytopenia: A Case Report

Pathogenic mutations in 3-keto-dihydrosphingosine reductase(KDSR)gene are associated with keratinization disorders and impaired platelet function.However,no case with both homozygotic mutation of KDSR and hepatic hemangioendothelioma has ever been reported due to its low prevalence.Here we report a seven months old Chinese boy with a homozygotic missense mutation in KDSR and both of his parents carry a same heterozygous mutation.He was born with thick plate-like scales overlying erythrodermic skin,but the skin symptoms were resolved spontaneously over the first month of his birth.He was also diagnosed with hepatic hemangioendothelioma at birth and accepted a resection surgery at 2 months old.At birth,his platelet count was severely low(10-20×10~9/L)with recurrent skin and gingival bleeding.Meanwhile,he suffered a mild normocytic,normochromic anemia with normal iron and hematinic levels.The anemia spontaneously recovered over the first 6 months,while the platelet count keeped at a low level(4-20×10~9/L).Treatment with corticosteroids,immunoglobulin or thrombopoietin was all suboptimal.

Infantile hepatic hemangiomas:looking backwards and forwards

Infantile hepatic hemangiomas(IHHs)are common benign tumors seen in the liver of infants.IHHs are true infantile hemangiomas(IHs)and have phases of proliferation and involution parallel to those of cutaneous IHs.The definition and classification of IHH are still confusing in the literature.The mechanisms during the pathogenesis of IHH have yet to be discovered.The clinical manifestations of IHH are heterogeneous.Although most IHH lesions are asymptomatic,some lesions can lead to severe complications,such as hypothyroidism,consumptive coagulopathy,and high-output congestive cardiac failure.Consequently,some patients can possibly encounter a fatal clinical condition.The heterogeneity of the lesions and the occurrence of disease-related comorbidities can make the treatment of IHH challenging.Oral propranolol is emerging as an effective systemic approach to IHH with obvious responses in tumor remission and symptom regression.However,the precise clinical characteristics and treatment strategies for patients with severe IHH have not yet beenwell established.Here,we summarize the epidemiology,pathogenicmechanism,clinical manifestations,diagnosis,and treatment of IHH.Recent updates and future perspectives for IHH will also be elaborated.

Imaging findings of multiple infantile hepatic hemangioma associated with cardiac insufficiency

Background:Infantile hepatic hemangioma(IHH)as a benign liver tumor in infancy and childhood is commonly associated with high output cardiac failure.The present study aims to describe the imaging findings in a patient who was diagnosed as having multiple IHH with congestive cardiac insuffi ciency.Methods:The imaging findings and clinical manifestations of the patient with multiple IHH associated with cardiac insuffi ciency were retrospectively reviewed.Results:Ultrasonography showed multiple intrahepatic lesions with mixed echoes and markedly expanded hepatic veins and the inferior vena cava of the patient.Echocardiography revealed right heart insufficiency and pulmonary hypertension.Contrast-enhanced MRI showed early mild enhancement of lesions and more obvious delayed enhancement.The patient died after combined therapy of surgery and hormone.Conclusions:The imaging findings of multiple IHH associated with cardiac insufficiency are typical and diagnostic.Early imaging assessment may facilitate the diagnosis and treatment of the disease.

The subspecialty of pediatric infectious diseases is growing in China

Although infectious diseases are still common in Chinese children, great changes have taken place in the spectrum of the diseases. Two severe contagious diseases, smallpox and poliomyelitis, were eliminated many years ago, and other infectious diseases under effective control are measles, diphtheria, whooping cough, neonatal tetanus, epidemic cerebrospinal meningitis, epidemic encephalitis type B, typhoid, scarlet fever, dysentery, malaria, viral hepatitis, tuberculosis, syphilis,