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Biomedical subjects

B Simma

Publications and source records attributed to B Simma.

36 records · Page 2Linked to original sources
PubMed1996

Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve.

We report a case of neonatal lupus erythematosus (NLE) with congenital heart block and severe myocardial failure, which was followed from the 25th week of gestation because of fetal bradycardia. The child was delivered at the 37th week of gestation by elective cesarean section because of echocardiographically documented heart enlargement, pericardial effusion and moderate insufficiency of the mitral and tricuspid valves. In spite of immediate pacing, intubation and supportive treatment, the newborn developed progressive heart failure. Echocardiography showed endocarditis of the mitral valve and diffuse myocarditis. The heart failure resolved under steroid treatment. Our experience supports the early use of steroids in treating myocarditis due to NLE. Intrauterine steroid treatment in the presence of fetal hydrops and congenital heart block is discussed.

Adult↗
PubMed1996

[Transoral CO2 laser resection of extensive nasal and oropharyngeal teratoma].

BACKGROUND: Teratomas are quite rare congenital tumours, especially originating from the skull base in the region of the nasopharynx (1:40,000). PATIENTS: We report on a premature 36-week old girl with an extensive nasopharyngeal and oral teratoma. METHOD: With CO2 laser and micro-endoscopic techniques the tumour was successfully resected directly via the transoral route without needing external incisions. RESULTS AND CONCLUSIONS: These techniques, and particularly the use of CO2 laser, allow gentle resection of nasopharyngeal teratomas with only moderate bleeding. General pathological aspects, clinical symptoms and update management or teratomas in the head and neck area are discussed, based on this case. CO2 laser in conjunction with microscope and videoendoscope seems to offer an advantage compared to resection with conventional instruments alone.

Adult↗
PubMed1995

Fulminant hepatic failure in a child as a potential adverse effect of trimethoprim-sulphamethoxazole.

UNLABELLED: Trimethoprim-sulphamethoxazole (TMP-SMZ) is considered a safe drug for treatment of infectious bacterial diseases in children. Side-effects are rare and generally take the form of a hypersensitivity reaction to the sulphamethoxazole component of the drug. Hepatic injury usually presents as a transient elevation of liver enzymes, which is of little clinical relevance. Fulminant liver failure due to TMP-SMZ has been reported in only six adults and never in children. We here report a 5-year-old girl who developed fulminant liver failure 3 weeks after her third exposure to TMP-SMZ. After a biphasic clinical course she underwent successful liver transplantation. CONCLUSION: Trimethoprim-Sulphamethoxazole may cause fulminant liver failure in children. The disease can run a biphasic clinical course and liver transplantation must be considered as the therapeutic option for these patients.

Biopsy↗
PubMed1994

Tracheostomy in children.

We reviewed the records of 108 patients who had a tracheostomy performed over a 10-year period from July 1979 to April 1989. Median age at tracheostomy was 6 months (1 week-15 years). Indications for surgery were acquired subglottic stenosis (31.4%), bilateral vocal cord paralysis (22.2%), congenital airway malformations (22.2%) and tumours (11.1%). No epiglottis and no emergency situation had to be managed by tracheostomy. Operation was uneventful in all, but 8 patients (7.4%) developed a pneumothorax in the postoperative period. Twenty-one (19.5%) had severe complications during the cannulation period (tube obstruction in 11 patients with cardiorespiratory arrest in 4; dislocation of the tube in 6 patients). Fifteen patients (13.8%) had severe complications after decannulation (2 had a cardiorespiratory arrest); all 15 had to be recannulated. At the end of the study period 85 patients (78.7%) were successfully decannulated with a median period of tracheostomy of 486 days (8 days-6.6 years). The median hospital stay was 159 days (13 days-2.7 years). All patients could be discharged. Eight patients (7.4%) died but no death was related to tracheostomy. In summary the mortality rate is lower than reported in previous reviews and tracheostomy is a safe operation even in small children but cannula-related complications may lead to life-threatening events. The management of tracheostomized small children and infants in a highly staffed and monitored intensive care unit has allowed better handling of complications and has resulted in a reduction in cannula-related deaths.

Adolescent↗
PubMed1993

Resuscitation after severe hypoxia in a young child: temporary isoelectric EEG and loss of BAEP components.

EEG and multimodality evoked potentials are currently used to ascertain brain death. We have observed electrocerebral silence (ECS) and loss of wave IV and V in BAEP in a 28-month-old child, 2 h after resuscitation from severe hypoxia, followed by reappearance of EEG activity and of waves IV and V (latencies and amplitudes within normal range) 18 h later. Temporary loss of EEG activity has been observed occasionally, while rapid reoccurrence of BAEP waves IV and V was unexpected and in contrast to the common observation, that rostro-caudal loss of BAEP waves is irreversible. This observation suggests that temporary loss of BAEP waves IV and V may occur and that caution is necessary in the interpretation of BAEP recorded immediately after a resuscitation.

Brain Death↗
PubMed1993

Squamous-cell carcinoma arising in a non-irradiated child with recurrent respiratory papillomatosis.

We describe a patient with recurrent respiratory papillomatosis (RRP) associated with human papilloma virus (HPV), who developed a fatal squamous cell carcinoma of the lung. At the age of 1 year he presented with hoarseness, dyspnoea and inspiratory stridor but the diagnosis of RRP was made only 1 year later. At the age of 4 years he was tracheostomized because of upper airway obstruction. In spite of multiple surgical excisions and topic treatment with 5-fluorouracil the papillomata extended to the lung parenchyma. At the age of 16 years he developed a squamous-cell carcinoma of the lung and died 4 months later. Transformation to pulmonary carcinoma is a rare complication in non-irradiated patients with lung papillomatosis. We found only 11 similar cases in the literature.

Adolescent↗
PubMed1993

T cell activation and cytokine release in streptococcal toxic shock-like syndrome.

A 5-year-old girl with streptococcal toxic shock-like syndrome during varicella infection had high levels of tumor necrosis factor alpha and interleukin-6 but no interleukin-1 or interleukin-2 in the serum. Intravenous administration of gamma-globulin coincided with clinical improvement and with reduction of the levels of tumor necrosis factor alpha and interleukin-6. The data suggest that streptococcal pyrogenic exotoxins trigger synthesis of tumor necrosis factor alpha and interleukin-6 in vivo; intravenously administered gamma-globulin may down-regulate the cytokine response.

Arthritis, Infectious↗
PubMed1993

[The psychosocial status of HIV-positive mothers and their children].

We studied the psycho-social circumstances of HIV-positive women and their children, who were born between January 1, 1984 and June 30, 1991 and are being treated at the Department of Pediatrics. We interviewed 19 women out of 21 families using the problem-centered interview technique according to Witzel. 29 out of 30 children were older than 18 months. Four children were HIV-positive and the serology of one patient aged 12 months who had been perinatally exposed was yet unclarified. The study showed massive problems within these families, such as considerable partner problems, drug abuse and social isolation. Furthermore, there was a lack of child care to relieve the mothers. The predominance of drug problems and insufficient experience of social institutions with the problematic nature of HIV-infection in mothers and children require more efficient preventive measures.

Child↗
PubMed1991

Bacille Calmette-Guérin--associated neonatal hepatitis.

We describe a full-term immunocompetent neonate who developed jaundice at 3 weeks of age. Physical examination disclosed no abnormalities. Laboratory investigations showed direct reacting hyperbilirubinaemia and elevated liver enzymes. Liver biopsy revealed a non-caseating granulomatous hepatitis. The patient made an uneventful recovery within 4 weeks without therapy. Bacille Calmette-Guérin hypersensitivity reaction is suggested as the reason for this neonatal hepatitis.

BCG Vaccine↗
PubMed1989

[Maple syrup urine disease and cystathioninemia].

We report of our experience a case of a patient with the classic type of Maple Syrup Urine Disease (MSUD) and the rare combination with a secondary Cystathioninemia. The screening of newborns in terms of looking for MSUD has been terminated in 1979 because the number of cases was too small. An 8 days old boy was admitted to our hospital in al lifethreatening state with unspecific neurological symptoms. We were able to diagnose the MSUD in 36 hours. Although we succeeded in decreasing the plasma leucin level by an exchange transfusion and a continuous arteriovenous hemofiltration over 3 days, the leucin level again increased to neurotoxic levels. Finally we managed the leveling by applying a high caloric parenteral and enteral intake. Also a substitution of valin and isoleucin was necessary. The goal of the long-term treatment with a life-long diet with fixed quantities of BCCAs is to adjusted the plasma leucin level to 100-700 mumol/l, the valin and isoleucin level to 200-300 mumol/l. In summary we want to point out, that the diagnosis for the MSUD should be done early enough, to start the successfull therapy, as described above, and to improve the prognosis.

Amino Acid Metabolism, Inborn Errors↗
PubMed1988

[Juvenile nonspecific spondylodiscitis].

In the case of an 11-years old boy, diagnosis "Juvenile Spondylodiscitis" finally was established by MRI. X-Ray investigation was negative and bone scintigraphy was interpreted negative or not conclusive. In recently published studies using MRI for diagnosis of spondylodiscitis in children younger than six years, a typically MCR signal of involved disc and vertebral body was found at a stage of investigation when bone scintigraphy had already also been conclusive. In our case, the patient was a little bit older and may be because of this, scintigraphy was negative. The different vertebral blood perfusion situation of the older child seems to disadvantage scintigraphy to MRI in diagnosis of spondylodiscitis.

Child↗
PubMed1988

[Exogenous allergic alveolitis (bird fancier's lung)].

A 13 year old boy was admitted to our clinic because of caugh, dyspnoe, tachypnoe and bilateral fine moist rales since more than 6 months. Pulmonary function studies showed a restrictive lung disease. Lung biopsy revealed an interstitial pneumonitis. These findings together with a hypergammaglobulinemia and precipitating antibodies were highly suggestive of an extrinsic allergic alveolitis i.e. pigeon breeder's disease. The patient was treated with corticosteroids and is well after 1 year without any therapy except prevention of further exposure.

Alveolitis, Extrinsic Allergic↗
PubMedDate not supplied

[GM1 gangliosidosis and dilated cardiomyopathy].

We report a child with a rare cardiac involvement in GM1 gangliosidosis. At the age of 9 months a secondary dilatative cardiomyopathy due to the metabolic disorder was diagnosed. During 2 years the functional echocardiographic signs became normal, though the ECG still was not normal. Our report is the first longterm observation and investigation of cardiac involvement in GM1 gangliosidosis.

Cardiomyopathy, Dilated↗
PubMedDate not supplied

Constitutional interstitial deletion of 17(p11.2) (Smith-Magenis syndrome): a clinically recognizable microdeletion syndrome. Report of two cases and review of the literature.

We present two patients, a 12 year old Turkish and a 7 year old Italian girl, with severe mental retardation, multiple congenital malformations and a constitutional interstitial deletion of the short arm of chromosome 17, del(17) (11.2). The main clinical features of this syndrome which is also referred to as the Smith-Magenis syndrome consist of a broad flat midface with brachycephaly, broad nasal bridge, brachydactyly, speech delay, hoarse deep voice and peripheral neuropathy. Behavioural abnormalities include hypermotility, self mutilation and sleep disturbances. The remarkable consistency of the main clinical features of the 59 patients documented so far permits the clinical diagnosis of this syndrome; a fact which is of importance, since many of the reported patients--including ours--were considered normal on earlier cytogenetic studies.

Abnormalities, Multiple↗