[Clinic and therapy of idiopathic hemochromatosis. Report of 51 cases (author's transl)]. 1976

R Fischer

Idiopathic hemochromatosis is a genetical defect leading to continuous accumulation of iron. Symptomatology is caused by iron deposition in different organs. Phlebotomy is by far the most efficient therapy. Case reports are given of 51 cases in different stages of the disease, all of which were proved by biopsy. Taking into account data of these cases some aspects of heredity, history and clinical symptomatology of this metabolic disease are discussed; histological and peritoneoscopic findings during the course of the disease under management by phlebotomy are demonstrated. On 46 patients treated in this was 25 were freed of iron deposits and 5 improved. In most cases there was a reduction of collagenous tissue in the liver. In some, and even in far advanced cases, almost complete anatomical and clinical normalization could be reached. Prognosis depends upon early diagnosis and consequent long-term treatment by phlebotomy.

UI MeSH Term Description Entries
D007501 Iron A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. Iron-56,Iron 56
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010535 Laparoscopy A procedure in which a laparoscope (LAPAROSCOPES) is inserted through a small incision near the navel to examine the abdominal and pelvic organs in the PERITONEAL CAVITY. If appropriate, biopsy or surgery can be performed during laparoscopy. Celioscopy,Laparoscopic Surgical Procedures,Peritoneoscopy,Surgical Procedures, Laparoscopic,Laparoscopic Assisted Surgery,Laparoscopic Surgery,Laparoscopic Surgical Procedure,Procedure, Laparoscopic Surgical,Procedures, Laparoscopic Surgical,Surgery, Laparoscopic,Surgical Procedure, Laparoscopic,Celioscopies,Laparoscopic Assisted Surgeries,Laparoscopic Surgeries,Laparoscopies,Peritoneoscopies,Surgeries, Laparoscopic,Surgeries, Laparoscopic Assisted,Surgery, Laparoscopic Assisted
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D001815 Bloodletting Puncture of a vein to draw blood for therapeutic purposes. Bloodletting therapy has been used in Talmudic and Indian medicine since the medieval time, and was still practiced widely in the 18th and 19th centuries. Its modern counterpart is PHLEBOTOMY.
D002614 Chelating Agents Chemicals that bind to and remove ions from solutions. Many chelating agents function through the formation of COORDINATION COMPLEXES with METALS. Chelating Agent,Chelator,Complexons,Metal Antagonists,Chelators,Metal Chelating Agents,Agent, Chelating,Agents, Chelating,Agents, Metal Chelating,Antagonists, Metal,Chelating Agents, Metal
D005260 Female Females
D006432 Hemochromatosis A disorder of iron metabolism characterized by a triad of HEMOSIDEROSIS; LIVER CIRRHOSIS; and DIABETES MELLITUS. It is caused by massive iron deposits in parenchymal cells that may develop after a prolonged increase of iron absorption. (Jablonski's Dictionary of Syndromes & Eponymic Diseases, 2d ed) Diabetes, Bronze,Bronze Diabetes,Bronzed Cirrhosis,Familial Hemochromatosis,Genetic Hemochromatosis,Haemochromatosis,Hemochromatoses,Iron Storage Disorder,Pigmentary Cirrhosis,Primary Hemochromatosis,Troisier-Hanot-Chauffard Syndrome,Von Recklenhausen-Applebaum Disease,Bronzed Cirrhoses,Cirrhoses, Bronzed,Cirrhoses, Pigmentary,Cirrhosis, Bronzed,Cirrhosis, Pigmentary,Disease, Von Recklenhausen-Applebaum,Diseases, Von Recklenhausen-Applebaum,Disorder, Iron Storage,Disorders, Iron Storage,Familial Hemochromatoses,Genetic Hemochromatoses,Haemochromatoses,Hemochromatose,Hemochromatoses, Familial,Hemochromatoses, Genetic,Hemochromatosis, Familial,Hemochromatosis, Genetic,Iron Storage Disorders,Pigmentary Cirrhoses,Recklenhausen-Applebaum Disease, Von,Recklenhausen-Applebaum Diseases, Von,Storage Disorder, Iron,Storage Disorders, Iron,Syndrome, Troisier-Hanot-Chauffard,Syndromes, Troisier-Hanot-Chauffard,Troisier Hanot Chauffard Syndrome,Troisier-Hanot-Chauffard Syndromes,Von Recklenhausen Applebaum Disease,Von Recklenhausen-Applebaum Diseases

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