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A L Hoffmann

Publications and source records attributed to A L Hoffmann.

8 recordsLinked to original sources

Childhood sarcoidosis in Denmark 1979-1994: incidence, clinical features and laboratory results at presentation in 48 children.

AIM: To describe the incidence, clinical presentation and paraclinical findings in childhood sarcoidosis in Denmark, 1979-1994. METHODS: Patients (n = 5536) with a diagnosis of sarcoidosis were drawn from the nationwide Patient Registry; 81 patients were < or = 15 y of age. The diagnosis of sarcoidosis was reconfirmed in 48/81 (59%) patients. In 35/48 (73%) patients, diagnosis was verified by histology, and in 13 it was substantiated by paraclinical/clinical findings. RESULTS: The series comprised 26 boys and 22 girls (male/female ratio 1.18). Median age at diagnosis was 13 y (range 0.7-15). In 1979-1994 the incidence was 0.29 per 100000 person-years < or = 15 y of age. The incidence was 0.06 in children < or = 4 y of age and increased gradually with age to 1.02 in children aged 14-15 y. General malaise, fever, weight loss, abdominal discomfort, respiratory symptoms, lymphadenopathy and central nervous system symptoms were common; 31% of patients had erythema nodosum, 12.5% sarcoid skin lesions, 25% uveitis/iridocyclitis and 4.2% sarcoid arthritis. Chest X-rays were normal (stage 0) in 10% of patients, and showed pulmonary infiltrates stage I in 71%, stage II in 8.3% and stage III in 8.3%. Lung function tests were examined in 13 patients: 50% had decreased FEV1 and vital capacity, 80% decreased DLCO. Haemoglobin values were normal. Some patients had mild leukopenia, some moderate leukocytosis and a few had moderate eosinophilia. Erythrocyte sedimentation rate was elevated in 40% of the patients. Plasma calcium was elevated in 30% of the patients; 4 patients had severe hypercalcaemia and elevated plasma creatinine, and 1 patient had nephrocalcinosis. Serum angiotensin-converting enzyme was elevated in 55% of the patients. Liver function tests were normal with no sarcoid hepatitis. Urinary examination (glucose, albumin, haemoglobin) was normal in 96% of the patients; the patient with nephrocalcinosis had albuminuria and haematuria. CONCLUSION: The incidence of sarcoidosis in Danish children is low and increases with age. Sarcoidosis in young children may present clinical features that are different from the appearance of those in older children and often constitute a diagnostic challenge. In older children, the clinical appearance has many features in common with the presentation in adults.

Adolescent↗

Intraprostatic temperature monitoring during transurethral microwave thermotherapy: status and future developments.

Transurethral microwave thermotherapy is being applied as a minimally invasive treatment for alleviating the symptoms of urinary outlet obstruction associated with benign prostatic hyperplasia. Treatment progress has traditionally been guided in its effective power by rectally and urethrally measured temperatures, whereas intraprostatic temperatures would be preferred for feedback purposes. A critical evaluation is presented of intraprostatic thermometry techniques that have been suggested, the techniques currently being used and investigated, and the problems that remain to be solved. Techniques for noninvasive temperature measurement and detecting tissue response during thermal therapy are discussed in more detail. Results presented in the literature have shown magnetic resonance imaging and ultrasonic imaging to be adequate thermometry modalities. For treatment monitoring of transurethral microwave thermotherapy, ultrasonic imaging is especially promising. Future research will indicate whether the promise evolves into a sound clinical technique.

Body Temperature↗

Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis.

This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.

Age Factors↗

[Sarcoidosis in children. Clinical manifestations, epidemiology, treatment and prognosis].

Sarcoidosis is a granulomatous disease of unknown aetiology, which may affect various organs. The diagnosis is obtained by the demonstration of epytheloid cell granulomas in an affected organ. The incidence of sarcoidosis in Danish children less than 15 years of age is 0.22-0.27/100.000 children/year, corresponding to approximately three new cases in Denmark each year. The disease often takes an asymptomatic course. During the period 1980-1992, three cases of paediatric sarcoidosis were observed in Copenhagen Country. All three had pulmonary involvement, and one had severe hypercalcaemia. In children less than five years of age, the disease is characterized by involvement of lungs, lymph nodes and eyes. Treatment, which is symptomatic, consists of systemic steroids. Due to the risk of growth retardation, the indication for treatment should be carefully considered and steroids administered at the lowest effective dose. Due to the lack of follow-up studies, the long term prognosis is unclarified.

Adolescent↗

Childhood sarcoidosis presenting with hypercalcaemic crisis.

We report a case of hypercalcaemic crisis due to sarcoidosis in a 15-year-old boy. The clinical suspicion of sarcoidosis was confirmed by a liver biopsy. At admission serum calcium, 1,25(OH)2 and ACE were elevated and iPTH was suppressed. The levels of serum total and ionized calcium, iPTH, ACE, 1,25(OH)2 and 25-OH were followed and chest X-ray and pulmonary function tests were performed during systemic steroid treatment. The clinical condition improved during treatment and the paraclinical measurements normalised within 5 weeks. The mechanism whereby hypercalcaemia occurs in childhood sarcoidosis is clarified.

Adolescent↗

Bladder diverticula in patients with prostatism.

The incidence of bladder diverticula was investigated in a patient material referred for prostatism. Out of 104 referred patients 69 were examined by cystography and urography. Fifty-one per cent had diverticula on cystography but only 30% on urography. Diverticula were significantly related to age. They were significantly associated with upper tract dilatation and tended to relate to residual urine volume and bladder instability. There was no relation to the bladder neck-verumontanum distance, maximal flow rate or bladder capacity.

Age Factors↗

[Malaria and pregnancy].

In regions where malaria is endemism, the disease is a recognised cause of complications of pregnancy such as spontaneous abortion, premature delivery, intrauterine growth retardation and foetal death. Malaria is seldom seen in pregnant women in Denmark but, during the past two years, the authors have treated four patients in the University Hospital in Copenhagen. These pregnancies were all successful but two of the mothers required emergency Caesarean section on account of threatening intrauterine asphyxia. The patients came relatively late for treatment which may be because not only the patients but also their practitioners were unaware that malaria can occur several years after exposure. Three out of the four patients had employed malaria prophylaxis. As resistance to malarial prophylactics in current use is increasing steadily, chemoprophylaxis should be supplemented by mechanical protection against malaria and insect repellents. As a rule, malaria is treated with chloroquine. In cases of Falciparum malaria in whom chloroquine resistance is suspected, treatment with mefloquine may be employed although this should only be employed in cases of dire necessity in pregnant patients during the first trimester. Severe cases should be treated with infusion of quinine. During pregnancy, benign malaria may run a violent course and pregnant women with malaria should be monitored in maternity departments and should be treated in cooperation with specialists in tropical medicine.

Adult↗

The relationship between Apgar score, umbilical artery pH and operative delivery for fetal distress in 2778 infants born at term.

A total of 2778 infants born at term were studied to determine the relationship between Apgar scores after 1 min, umbilical artery pH values, mode of delivery, a diagnosis of fetal distress leading to operative delivery, and sex. Eighty-three percent of the population had normal Apgar scores (greater than or equal to 8) and normal pH values (greater than 7.15) in which 10% were operatively delivered for fetal distress (ODFD). Sixty-one percent of the children with low Apgar scores (less than or equal to 7) had normal pH values, and 74% of the infants with acidosis (pH less than or equal to 7.15) had normal Apgar score. Twenty-four percent of the infants with a low Apgar score and/or acidosis were ODFD (sensitivity). Ninety percent of the infants who had Apgar scores and pH values were not ODFD (specificity). The predictive value (a low Apgar score and/or acidosis) of ODFD was 33%, and the negative predictive value (normal Apgar score and a normal pH) of ODFD was 85%. A significantly higher incidence of ODFD and acidosis was found in boys.

Acidosis↗