Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A short survey is given on description and evaluation of progress and developmental trend of laparoscopy. Important technical improvements are the cold light via the glass fibre light conductor, optics of high value, the colour photography with electronic flash-light, colour films and colour television, new accessory instruments and belongings. Now as ever hepatomegaly and splenomegaly, jaundice, ascites, portal hypertension, suspicion of cirrhosis and metastases are regarded as main indications. The laparoscopy deserves a greater consideration in unclear abdominal symptoms, in gynaecological diseases, for the proof of the affection of liver and spleen in lymphogranulomatosis, sarcoidosis, tuberculosis (with aimed liver biopsy). The endoscopic retrograde cholangiopancreatography in the differential diagnosis of the jaundice competes with the laparoscopy, but it is not able to supersede it. The laparoscopic judgment of the pancreas and the importance of visible changes of the fine structed. Among the contraindications of the laparoscopy the hiatal hernia has lost its significance.
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PMID:[Laparoscopy--current aspects]. 13 32

Twenty infants and young children with hereditary fructose intolerance (HFI) were admitted to hospital. None was diagnosed at admission. Referals were for vomiting of unknown aetiology (16X), pyloric stenosis or hiatus hernia (5X), toxic condition (3X), and hepatomegaly of unknown origin (5X). Feeding difficulties (20X), vomiting (18X), and failure to thrive (16X) were leading symptoms. The most frequent clinical findings were hepatomegaly (18X), pallor (14X), haemorrhages (13X). Ascites, oliguria, tachypnoea, fever, splenomegaly and rickets were less frequent. Laboratory findings were indicative of disturbed hepatic and renal tubular function and also of disturbed intermediary metabolism (hypokaliaemia, hypophosphataemia). However, hypoglycaemia was found in only 4 out of 15 patients tested. Differential diagnosis after hospital admission centered on metabolic disorders such as glycogenoses, galactosaemia, tyrosinosis, or Wilson's disease. Hepatitis, toxic hepatosis, liver tumour, intrauterine infection and sepsis were also considered. Eleven children had first ingested fructose within the first 6 weeks of life. The diagnosis was usually established only many weeks or months after first fructose intake and appearance of symptoms. This documents how difficult the diagnosis of this disease can be both in practice and in hospital. The course was severe in 11 children and lethal in 4. In only 5 patients was the course mild. The 16 survivors are doing well under fructose-exclusion diet. Irreversible visual impairment after intraocular haemorrhage occurred once. In each case HFI could have been suspected immediately, had a detailed nutritional history been taken. Practising paediatricians should know the composition of commonly used infant formulae. They should never prescribe sugared condensed milk for intractable vomiting prior to excluding HFI. Solution for intravenous infusion containing fructose and sorbitol are life-threatening for undiagnosed HFI patients.
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PMID:Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. 73

Although laparoscopic fundoplication is now performed commonly in children, its long-term results in neurologically impaired (NI) children is unknown. We present a single surgeon's experience. During an 8.5 year period, 54 consecutive NI children (age 5 months to 16 years; weight 2.7 to 42 kg) who had failed medical treatment for severe gastroesophageal reflux (GER) underwent laparoscopic Nissen fundoplication without (7) or with (47) gastrostomy. Indications for surgery included failure to thrive and feeding difficulties in all, major vomiting in 42, recurrent chest infections in 44, and inability to take oral medication in 14. Hiatus hernia was present in 14 and delayed gastric emptying in 6 patients. Eight (15%) had undergone previous abdominal surgery. Access was modified according to individual anatomy and 4 or 5 cannulae were used in each patient. Postoperative epidural/morphine analgesia was used in the first 12 to 24 hours, and fluid intake and feeding were started on day 1 and 2, respectively. The average operating time for fundoplication was 2.2 hours (range 1.05 to 3) and for fundoplication and gastrostomy 2.3 hours (range 1.22 to 4.10). Three patients had conversion to open surgery (1 perforated esophagus, 1 hypercarbia and hepatomegaly, 1 camera failure). There were no other operative complications or mortality. One child with Down syndrome developed a food bolus obstruction 3 days postoperatively. The vast majority of patients were discharged home 3 to 4 days following fundoplication and 5 to 7 days following fundoplication and gastrostomy. Postoperative gas bloat was common, diarrhea developed in 4, dumping in 3, and major gastrostomy infection in 1 case. During follow-up (median 5.2, range 3 months to 8.6 years), 9 (16%) children showed signs of persistent/recurrent problems. Investigations showed a recurrent hiatus hernia in 1 (requiring re-operation) and minor reflux in 3 patients. To date 6 (11%) children have died of their background conditions. In NI children, laparoscopic fundoplication is safe and successful. Awareness of the differences in access and risks for NI and normal children is important. Compared with historical data for open technique, laparoscopic fundoplication produces lower mortality and morbidity and similar intermediate and long-term results.
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PMID:Results of laparoscopic antireflux procedures in neurologically impaired children. 1240 29

Golden Retriever muscular dystrophy is an inherited, degenerative myopathy due to the absence of dystrophin and is used as a model of Duchenne muscular dystrophy of young boys. This report describes the radiographic abnormalities of Golden Retriever muscular dystrophy in 26 dogs. The thoracic abnormalities included diaphragmatic asymmetry (18/26), diaphragmatic undulation (18/26), and gastro-esophageal hiatal hernia (6/26). Pelvic abnormalities included narrowing of the body of the ilia (14/19), ventral deviation and curvature of the tuber ischii (14/19), elongation of the obturator foramen with a decrease in opacity of the surrounding bone (12/19), and lateral flaring of the wings of the ilia (12/19). Abdominal abnormalities consisted of hepatomegaly (14/22) and poor serosal detail (12/22). The unique thoracic abnormalities were a consistent finding in affected Golden Retriever muscular dystrophy dogs. The diagnosis of muscular dystrophy should be included in the differential list if the combination of diaphragm undulation and asymmetry, and gastro-esophageal hiatal hernia are identified. These diaphragmatic abnormalities are related to hypertrophy and hyperplasia of the diaphragm. Additionally, the skeletal changes of pelvic tilt, elongation of the pelvis, widening of the obturator foramina and thinning of the ischiatic tables appear to be specific to Golden Retriever muscular dystrophy in dogs. These pelvic abnormalities are most likely secondary to bone remodeling associated with the progressive skeletal myopathy and subsequent contracture/fibrosis.
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PMID:Radiographic features of Golden Retriever muscular dystrophy. 1715 67