ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00001.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00002.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00003.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractRational management of pulmonary artery hypertension (PAH) in the newborn infant must be based on an understanding of underlying causes. When a specific diagnosis can be made, empirical treatment gives way to specific therapy. For every pathophysiologic abnormality potentially resulting in PAH, clinical entities can be identified and key therapeutic interactions can be considered.1. Pulmonary Venous Hypertension: PAH is secondary to obstruction of blood flow through the left heart. If based on anatomic abnormality (mitral or left atrial), surgical relief of obstruction is required. If PAH is the result or functional abnormality of the left ventricle (transient left ventricular ischemia), medical treatment of left ventricular failure is most important.2. Functional Obstruction of Pulmonary Vascular Bed: Hyperviscosity of blood (polycythemia ‐ HCT>65) results in increased pulmonary vascular resistance. Treatment consists of reduction of red blood cell volume.3. Pulmonary Vascular Constriction: Hypoxemia with or without pulmonary parenchymal disease (primary or secondary persistent fetal circulation [PFC] syndrome). Therapy is directed at improvement of oxygenation and dilatation of pulmonary arteriolar bed (e.g. O2hyperventilation and/or pulmonary vasodilator therapy).4. Decreased Pulmonary Vascular Bed: If primary pulmonary hypoplasia is present, no treatment is available. If secondary form (diaphragmatic hernia), surgical repair and pulmonary vasodilatation of contralateral lung is indicated. Extracorporeal membrane oxygenation (ECMO) can be used in severe cases.5. Increased pulmonary blood flow: Causes in the neonate include complex heart disease without pulmonary stenosis; and peripheral A—V fistula. Treatment: Surgical limitation of pulmonary blood flow may be required.Even when PAH is the result of combined etiologies, a prompt well‐organized clinical management plan can follow from understanding of pathologic princ

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00004.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00005.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00006.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00007.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractThe clinical appearance in the early phase of two cases of juvenile IDDM detected by urine glucose screening are described. Their postprandial blood glucose and HbAj were maintained without insulin therapy for several months below 180 mg/dl and 8.7% respectively. At three and six months after diagnosis both cases became ketoacidotic and their glucose tolerance deteriorated with a marked decrease of postprandial serum CPR level.There was no high insulin requirement with low CPR level at the time of diagnosis in 12 such patients with the above mentioned slow onset type IDDM. The insulin requirement steadily increased and serum CPR decreased from 12 months after diagnosis. The so‐called honeymoon period was not observed in this group.However, at the time of diagnosis in 24 patients with typical abrupt onset IDDM, there was a high insulin dose requirement with a low level of postprandial CPR value followed by an early decline in the insulin requirement with a temporary increase of CRP value.HLA‐DR4 or DRW9 or both were more freqently observed in patients with abrupt onset type IDDM than in slow onset type IDDM. It may probably be that genetic background as well as environmental factors affect the clinical picture in the early phase of IDDM in child

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00008.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00009.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractThe electro‐clinical characteristics of childhood epilepsy are known to appear during the development of the brain and to change their features with age. Accordingly, developmental viewpoints are indispensable to researches on childhood epilepsy.Age‐dependent epileptic encephalopathy has the most remarkable developmental characteristics among childhood epilepsies.Early‐infantile epileptic encephalopathy with suppression‐burst (EIEE), the West syndrome and the Lennox‐Gastaut syndrome, which constitute age‐dependent epileptic encephalopathy, have many common features. Each of these three syndromes, however, has its own distinctive clinical and electro‐encephalographic specificities, and shows mutual transition with age.Results of developmental studies on the West syndrome are described, confirming it as one type of age‐dependent epileptic encephalopathy.1) On the basis of the long‐term follow‐up study of 14 cases of EIEE, sequential transition from EIEE to the West, and further to the Lennox‐Gastaut syndrome was observed, with reference to critical changing period, transitional patterns and prognoses.2) Analytical follow‐up study of 83 cases of the West syndrome, divided into idiopathic and symptomatic groups showed its evolution into the Lennox‐Gastaut syndrome to be important for its prognosis, with reference to the various factors re

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb00010.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY