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Biomedical subjects

G Harmat

Publications and source records attributed to G Harmat.

At least 19 recordsLinked to original sources

Sex hormones in hereditary angioneurotic oedema.

OBJECTIVE: The fluctuations in sex hormone levels at the beginning of adolescence, in the perimenopausal period, during pregnancy or during the use of oral contraceptives can precipitate oedematous attacks in hereditary angioneurotic oedema (HANO). Attacks usually disappear after the onset of menopause. This study was undertaken to establish any relationship between the serum levels of sex hormones and the incidence of HANO attacks. PATIENTS AND MEASUREMENTS: Serum levels of LH, FSH, progesterone, oestradiol, testosterone, PRL and SHBG were measured in 78 patients [mean age 30.3 years (range 4-70 years)] with HANO. A questionnaire was used to explore the medical history of adult patients to characterize the evolution and the characteristics of attacks. RESULTS: The number of attacks was significantly higher [odds ratio (OR) 6.36 (1.31-30.81); P = 0.022] in females with high progesterone levels (> or = 4 nmol/l), irrespective of age, menstrual cycle and danazol dose. The OR was even higher [13.4 (2.2-81.4); P = 0.005] when only subcutaneous attacks were considered. Multiple logistic regression analysis demonstrated a significantly lower attack frequency during 1-year follow-up in patients with a higher (40 nmol/l) SHBG level (OR 0.25 (0.07-0.90); P = 0.034). This difference existed independently of age and danazol dose. CONCLUSION: In view of these results, the monitoring of progesterone and SHBG levels can prove useful in the prediction of attacks in hereditary angioneurotic oedema.

Adolescent↗

Acute abdominal attack of hereditary angioneurotic oedema associated with ultrasound abnormalities suggestive of acute hepatitis.

UNLABELLED: Hereditary angioneurotic oedema (HANO) is an autosomal dominant disorder caused by a deficiency of the inhibitor protein Cl-esterase. Recurrent subcutaneous and/or submucosal oedema formation is a hallmark of this disease. HANO is a rare, but potentially life-threatening disorder with a mortality around 20-30%. Acute oedematous abdominal attacks of HANO can mimic a surgical emergency; this is exemplified by the case of a 14-y-old male patient with HANO admitted for such clinical manifestations. CONCLUSION: Diagnostic clues include ascites and abnormalities of hepatic structure visible with ultrasound during the oedematous attack. The importance of appropriate treatment is emphasized.

Abdomen, Acute↗

Coordinated ultrasound screening of infants: Hungary experience.

OBJECTIVE: In 1990, three hospitals (the Madarász Street Children's Hospital in Budapest and the general hospitals in Cegléd and Zalaegerszeg) organised a coordinated ultrasound (US) screening programme. The screening was aimed at detecting a predefined series of developmental and acquired disorders. MATERIAL: In 9 years, a total of 46,858 infants were screened by the three hospitals: 20,185 in Budapest, 13,258 in Cegléd and 12,415 in Zalaegerszeg. RESULTS: Disorders were detected in 4068 cases. Most (2885) involved abdominal or renal disorders; intracranial anomalies were found in 1062, and the remaining discoveries were of cysts or tumours in the liver, spleen or ovaries. CONCLUSIONS: Many disorders were detected (8.7%) needing either follow-up, or some form of treatment.

Congenital Abnormalities↗

Ultrasonography in the diagnosis and monitoring of ascites in acute abdominal attacks of hereditary angioneurotic oedema.

BACKGROUND: Hereditary angioneurotic oedema (HAE) is a rare cause of ascites. As acute abdominal attacks of the disease can mimic surgical emergencies, prompt and accurate diagnosis is essential. This study was undertaken to evaluate the usefulness of serial abdominal ultrasound (US) examinations. PATIENTS AND METHODS: Seventy patients with HAE were followed up for almost a decade. All patients presenting with an acute oedematous attack underwent abdominal US, which was then repeated 24 and 48 h after appropriate therapy. RESULTS: Twenty-two acute oedematous attacks with abdominal complaints severe enough to justify hospital admission occurred in the study population. Abdominal US performed during the attack showed oedematous thickening of the intestinal wall in 80% of cases and invariably demonstrated the presence of free peritoneal fluid in all patients. Rapid symptomatic relief achieved by treatment was accompanied by the significant regression of US abnormalities. CONCLUSIONS: Transitory ascites demonstrated by abdominal US is a clue to the diagnosis of an acute abdominal attack of HAE. The possibility of HAE should always be considered whenever unexplained abdominal pain recurs with or without ascites.

Abdomen, Acute↗

[Hereditary angioneurotic edema in children].

Hereditary angioneurotic edema results from the deficiency of C1-esterase inhibitor. The clinical picture of this autosomal dominant disorder is characterized by recurrent attacks of edema formation in the subcutis and/or the submucosa. The clinical records of 21 children with established hereditary angioneurotic edema were reviewed. Follow-up care included laboratory check-ups and abdominal ultrasound. Clinical manifestations of the disease first occurred in 2.5 to 12 years of age. Mechanical trauma was the most common precipitating factor. Pedigree-analysis revealed 19 patients with afflicted relatives. Long-term prophylaxis was initiated with tranexamic acid and danazol in 10 cases; 2 children required short-term prophylaxis. Therapy improved serum complement parameters significantly and reduced the frequency and severity of clinical manifestations. Acute, life-threatening edematous attacks were treated by the administration of C1-inhibitor concentrate, which achieved the resolution of the edema within several hours. Abdominal ultrasonography performed during the attack invariably demonstrated transitory ascites that resolved spontaneously after treatment. Adequate prophylaxis and follow-up care can spare pediatric patients from edematous attacks. Undesirable adverse effects can be avoided and the patient's quality of life can be enhanced considerably by administering the lowest effective drug dose.

Adolescent↗

[Skull base fractures in childhood. The role of CT in the accuracy of diagnosis].

The authors treated 51 patients suffering from basilar skull fractures between January 1995 and June 1997 following a newly initiated protocol. The effectiveness of recognizing these fractures increased to twice and a half after applying a complex (neurologic, otolaryngologic, ophthalmologic, X-ray and cranial CT) diagnostic examination. CT scans were performed at the slightest suspicion of basilar skull fracture since they provide far more information than the native skull X-ray. A close coherence was observed concerning clinical symptoms, potential complications and the manifested fractures, which helped to draw an exact therapeutical strategy and to prevent potential complications. The authors found X-ray gave satisfactory information only about fractures starting on the vault. In the studied period CT proved the basilar skull fracture in 40 (78%) cases out of the total 51. X-ray did it in only 4 (8%) cases and raised the suspicion of the vault fracture spreading to the cranial floor in 20 (39%) cases. CT did not prove the clinically supposed basilar skull fractures in only 11 (22%) cases compared to 27 (53%) by X-ray. After having consultation with radiologists examination methods were always determined by the clinical picture and the available technical conditions (helical technique, multiplan or 3D-reconstruction).

Child↗

[Polysomnography in the prevention of crib death].

The authors stress the importance of polysomnography--a new electrophysiologic method--in the prevention of SIDS (cot death). SIDS is the most important and frequent cause of infant mortality between 1 and 12 months of age in western countries (nowadays between 1-2/1000!). In Hungary the frequency is not so high. In the last few years the incidence declined after the "back to sleep" campaigns, but to reach further success, it is very important to seek the so called "risk" babies. The unique cause of cot death is not yet understood exactly, but some instability in respiration (mostly during the sleep) is one of the accepted principal basic factors. The mentioned new method helps in choosing the SIDS risk infants from the "normal" population, allows to examine their respiratory irregularity or even disorders during the sleep and gives possibility for the prevention of lethal apneas. The authors describe the details of the prevention in case of abnormal polysomnography in their other publications.

Birth Weight↗

A rare case of infantile meningioma.

This paper reports the use of ultrasound and computed tomography in combination to diagnose a case of meningioma in a 3-month-old infant, and also describes the treatment.

Female↗

Intracranial sonography in infancy.

Neonates and older infants were subjected to sonography through the anterior fontanelle with grey-scale compound scanner. The patients were referred to us from neurosurgery or showed clinical symptoms of perinatal CNS damage (ventricular or intracerebral haemorrhage, subdural effusion, etc.) or malformations (meningomyelocele, encephalocele, cystic brain, cerebral dysgenesis, etc.). Sonography is the simplest non-invasive diagnostic method to exclude suspected hydrocephalus in cases of macrocephalus, or when the head of premature infants grows more rapidly than the rest of the body. When screening showed alterations, sonography was performed weekly or fortnightly according to the extent of deviation from the normal. In cases of progressive hydrocephalus, when shunt surgery was indicated, ultrasound was used to monitor the operation of the shunt. In early age, ultrasound is a tool equal in value to CT, but is far less expensive and the examination can safely be repeated at any time.

Brain↗

[Vaginal delivery following active transabdominal treatment of a prenatally diagnosed fetal hydrocephalus].

In a case of fetal hydrocephalus the authors repeatedly removed cerebrospinal fluid from the ventricles, transabdominally, by continuous pressure monitoring. Their aim was partly to improve the future functional prospects of the fetus, partly, by reducing the head circumference, to make possible a spontaneous and rapid deliver. After the period of adaptation a ventriculo-peritoneal shunt was implanted.

Adult↗