To compare clinical features, diagnosis and therapeutic effect between pulmonary histoplasmosis and progressive disseminated histoplasmosis.
 Methods: A retrospective analysis for 12 cases of hospitalized patients with histoplasmosis, who was admitted in Xiangya Hospital, Central South University during the time from February 2009 to October 2015, was carried out. Four cases of pulmonary histoplasmosis and 8 cases of progressive disseminated histoplasmosis were included. The differences of clinical features, imaging tests, means for diagnosis and prognosis were analyzed between the two types of histoplasmosis.
 Results: The clinical manifestations of pulmonary histoplasmosis were mild, such as dry cough. However, the main clinical symptoms of progressive disseminated histoplasmosis were severe, including recurrence of high fever, superficial lymph node enlargement over the whole body, hepatosplenomegaly, accompanied by cough, abdominal pain, joint pain, skin changes, etc.Laboratory examination showed pancytopenia, abnormal liver function and abnormal coagulation function. One pulmonary case received the operation of left lower lung lobectomy, 3 cases of pulmonary histoplasmosis and 6 cases of progressive disseminated histoplasmosis patients were given deoxycholate amphotericin B, itraconazole, voriconazole or fluconazole for antifungal therapy. One disseminated case discharged from the hospital without treatment after diagnosis of histoplasmosis, and 1 disseminated case combined with severe pneumonia and active tuberculosis died ultimately.
 Conclusion: As a rare fungal infection, histoplasmosis is easily to be misdiagnosed. The diagnostic criteria depends on etiology through bone marrow smear and tissues biopsy. Liposomeal amphotericin B, deoxycholate amphotericin B and itraconazole are recommended to treat infection for histoplasma capsulatum.
Abdominal Pain ; etiology ; Amphotericin B ; therapeutic use ; Antifungal Agents ; therapeutic use ; Biopsy ; Cough ; epidemiology ; Death ; Deoxycholic Acid ; therapeutic use ; Diagnostic Errors ; Drug Combinations ; Fever ; etiology ; Hepatomegaly ; etiology ; Histoplasma ; Histoplasmosis ; complications ; diagnosis ; mortality ; therapy ; Humans ; Invasive Fungal Infections ; complications ; diagnosis ; therapy ; Itraconazole ; therapeutic use ; Lung ; microbiology ; surgery ; Lung Diseases, Fungal ; diagnosis ; surgery ; therapy ; Pneumonia ; complications ; mortality ; Recurrence ; Retrospective Studies ; Splenomegaly ; etiology ; Treatment Outcome ; Tuberculosis ; complications ; mortality

We herein report the case of a 14-year-old girl with Epstein-Barr virus (EBV) infectious mononucleosis who developed prolonged hepatitis and jaundice. At presentation, she had tender hepatomegaly with a markedly deranged liver function test. Abdominal ultrasonography showed hepatomegaly and a thickened gallbladder wall. During the subsequent 11 weeks, her transaminases showed two further peaks, which corresponded with clinical deterioration. Her highest alanine transaminase level was 1,795 µ/L and total bilirubin level was 154 µmol/L. She recovered fully with conservative management. EBV-related liver involvement is typically mild and self-limiting. We believe that tender hepatomegaly and gallbladder thickening may be important predictors of significant liver involvement. Although multiple transaminase peaks may occur, we do not consider this an indication for antiviral or immunosuppressive therapy. In the absence of strong evidence supporting the use of any specific therapy, we recommend a conservative approach for an immunocompetent patient.
Adolescent ; Alanine Transaminase ; blood ; Antiviral Agents ; therapeutic use ; Bilirubin ; blood ; China ; Epstein-Barr Virus Infections ; complications ; Female ; Gallbladder ; pathology ; Hepatitis ; complications ; Hepatomegaly ; complications ; Humans ; Immunosuppressive Agents ; therapeutic use ; Jaundice ; complications ; Liver ; diagnostic imaging ; Treatment Outcome ; Ultrasonography ; gamma-Glutamyltransferase ; blood

Hepatic glycogenosis in type 1 diabetes mellitus (DM) can be caused by poor glycemic control due to insulin deficiency, excessive insulin treatment for diabetic ketoacidosis, or excessive glucose administration to control hypoglycemia. Mauriac syndrome, which is characterized by hepatomegaly due to hepatic glycogenosis, growth retardation, delayed puberty, and Cushingoid features, is a rare diabetic complication. We report a case of hepatic glycogenosis mimicking Mauriac syndrome. A 14-year-old girl with poorly controlled type 1 DM was admitted to The Catholic University of Korea, Seoul St. Mary's Hospital for abdominal pain and distension. Physical examination revealed hepatomegaly and a Cushingoid face. The growth rate of the patient had decreased, and she had not yet experienced menarche. Laboratory findings revealed elevated liver enzyme levels. A liver biopsy confirmed hepatic glycogenosis. Continuous glucose monitoring showed hyperglycemia after meals and frequent hypoglycemia before meals. To control hyperglycemia, we increased insulin dosage by using an insulin pump. In addition, we prescribed uncooked cornstarch to prevent hypoglycemia. After strict blood glucose control, the patient's liver functions and size normalized. The patient subsequently underwent menarche. Hepatic glycogenosis is a complication of type 1 DM that is reversible with appropriate glycemic control.
Abdominal Pain ; Adolescent ; Biopsy ; Blood Glucose ; Diabetes Complications ; Diabetes Mellitus, Type 1* ; Diabetic Ketoacidosis ; Female ; Glucose ; Glycogen Storage Disease* ; Hepatomegaly ; Humans ; Hyperglycemia ; Hypoglycemia ; Insulin ; Korea ; Liver ; Meals ; Menarche ; Physical Examination ; Puberty, Delayed ; Seoul ; Starch

Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months.
Adrenal Cortex Hormones/therapeutic use ; Female ; Hepatomegaly/complications/*diagnosis/pathology ; Humans ; Liver/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Peliosis Hepatis/complications/*diagnosis/pathology ; Purpura, Thrombocytopenic, Idiopathic/complications/*diagnosis/drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo investigate the computed tomography (CT) manifestations of liver involvement in children with Langerhans cell histiocytosis (LCH).

METHODSRetrospective analysis was performed on 9 LCH children with liver involvement confirmed by clinical, laboratory and pathological examinations to investigate the CT manifestations of this condition. These children, including 6 males and 3 females, had undergone both plain CT scan and dual-phase (the arterial and portal venous phases) contrast-enhanced CT scan.

RESULTSThe main CT manifestations included hepatomegaly (8 cases); periportal dendritic hypodense lesions or "periportal halo sign" (7 cases) which were mildly or moderately enhanced in the arterial phase; intrahepatic bile duct dilatation (5 cases); lymphadenopathy in the hepatic hilar or retroperitoneal region (4 cases); and diffuse small hypodense nodules (3 cases), which showed annular enhancement on the contrast-enhanced CT scan.

CONCLUSIONSCT findings may be helpful in the early diagnosis and treatment of LCH in children.

Child ; Child, Preschool ; Female ; Hepatomegaly ; diagnostic imaging ; Histiocytosis, Langerhans-Cell ; complications ; diagnostic imaging ; Humans ; Infant ; Liver ; diagnostic imaging ; pathology ; Male ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

Glycogenic hepatopathy (GH) is an uncommon cause of serum transaminase elevation in type I diabetes mellitus (DM). The clinical signs and symptoms of GH are nonspecific, and include abdominal discomfort, mild hepatomegaly, and transaminase elevation. In this report we describe three cases of patients presenting serum transaminase elevation and hepatomegaly with a history of poorly controlled type I DM. All of the cases showed sudden elevation of transaminase to more than 30 times the upper normal range (like in acute hepatitis) followed by sustained fluctuation (like in relapsing hepatitis). However, the patients did not show any symptom or sign of acute hepatitis. We therefore performed a liver biopsy to confirm the cause of liver enzyme elevation, which revealed GH. Clinicians should be aware of GH so as to prevent diagnostic delay and misdiagnosis, and have sufficient insight into GH; this will be aided by the present report of three cases along with a literature review.
Acute Disease ; Adult ; Alanine Transaminase/blood ; Aspartate Aminotransferases/blood ; Delayed Diagnosis ; Diabetes Mellitus, Type 1/complications/*pathology ; Diagnostic Errors ; Female ; Glycogen Storage Disease/complications/*diagnosis/ultrasonography ; Hepatitis/diagnosis ; Hepatomegaly/complications/*diagnosis/ultrasonography ; Humans ; Liver/pathology ; Recurrence ; Young Adult

Amyloid ; metabolism ; Amyloidosis ; complications ; diagnosis ; pathology ; Biopsy, Fine-Needle ; Congo Red ; Female ; Hepatomegaly ; etiology ; Humans ; Liver ; metabolism ; pathology ; Liver Diseases ; diagnosis ; etiology ; pathology ; Liver Function Tests ; Middle Aged ; Tomography, X-Ray Computed

Hepatitis A is generally known as a mild, self-limiting disease of the liver, but in rare instances it can progress to fulminant hepatitis, which may require liver transplantation for recovery. Such cases are known to be related to old age and underlying liver disease. We report four cases of hepatitis A in which patients presented with fulminant hepatitis and acute renal failure and underwent liver transplantation. The following common features were observed in our cases: (1) occurrence in relatively old age (> or =39 years old), (2) association with acute renal failure, (3) presence of hepatomegaly, and (4) microscopic features of submassive hepatic necrosis.
Adult ; Age Factors ; Female ; Hepatitis/complications/*diagnosis/therapy ; Hepatitis A/complications/*diagnosis ; Hepatomegaly/diagnosis/etiology ; Humans ; Kidney Failure, Acute/complications/*diagnosis ; Liver/pathology ; Liver Cirrhosis/diagnosis/etiology ; *Liver Transplantation ; Male ; Tomography, X-Ray Computed

Fundoplication is accepted as an effective treatment of gastroesophageal reflux disease. The recent results of laparoscopic fundoplication demonstrated safety and less morbidity, shorter hospital stay and less pulmonary complication compared to the open operation. Laparoscopic fundoplication has been our first choice of operation for gastroesophageal reflux disease since 2003. Among 29 cases, there were 2 conversion cases because of severe distension of transverse colon and hepatomegaly. We studied 27 consecutive patients operated upon from January 2003 through December 2004. There were 15 boys and 12 girls, ages from 1.5 months to 12 years (median 25.3 months). Body weight ranged from 2.9 kg to 37 kg (median 9.8 kg). Neurological abnormalities were present in 23 patients. Indications for surgery included medically refractory reflux associated with vomiting, pneumopathy, otorhinolaryngologic pathology, failure to thrive, esophagitis, apnea and bradycardia. We used 4-5 trocars of 5 mm or 12 mm with 30degrees telescope and performed the Nissen technique in all patients. In neurological impaired patients, gastrostomy tube was placed at the time of fundoplication. Median operative time was 130 minutes (70 . 300 minutes). There was no mortality nor intraoperative complication. Twenty-six patients were followed for median of 19 months (8 . 31 months). Four patients (15.4 %), who were all neurological impaired, developed recurrent symptoms of gastroesophageal reflux disease. Two of these patients had reoperation (1 laparoscopic approach, 1 open method). There were significant increases in body weight in 11 patients after fundoplication. Laparoscopic fundoplication is acceptable as a safe and effective method for gastroesophageal reflux disease.
Apnea ; Body Weight ; Bradycardia ; Child* ; Colon, Transverse ; Esophagitis ; Failure to Thrive ; Female ; Fundoplication* ; Gastroesophageal Reflux* ; Gastrostomy ; Hepatomegaly ; Humans ; Intraoperative Complications ; Laparoscopy ; Length of Stay ; Mortality ; Operative Time ; Pathology ; Reoperation ; Surgical Instruments ; Telescopes ; Vomiting

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
Alanine Transaminase/metabolism ; Alkaline Phosphatase/metabolism ; Antigens, CD/blood ; Antigens, Differentiation, Myelomonocytic/blood ; Arthritis, Juvenile Rheumatoid/blood/*complications/pathology ; Aspartate Aminotransferases/metabolism ; Blood Cell Count ; Hepatomegaly/*etiology/pathology ; Humans ; Infant ; Liver/enzymology/pathology ; *Macrophage Activation ; Male ; Partial Thromboplastin Time ; Prothrombin Time ; Splenomegaly/*etiology/pathology ; Syndrome ; gamma-Glutamyltransferase/metabolism