Pulmonary involvement in tuberous sclerosis

The authors present a case of tuberous sclerosis with marked pulmonary involvement, confirmed by both radiological and pathological studies. The radiological manifestations and basic pathology of this rare condition are reviewed with emphasis on differential diagnosis.     

Donor conditions and graft survival - a retrospective study

In a retrospective study (one centre) the influence of donor and recipient factors were evaluated (n = 308). Head injury as the cause of death and anastomotic time less than 35 min were associated with a significantly better graft survival rate (P < 0.05). Although some of the donor factors influence graft survival, a stricter selection of grafts is not advisable, firstly because fewer kidneys would then be offered, and secondly because even comparatively bad graft survival rates are still better than dialysis.     

Cytomegalie der Kopfspeicheldrüsen bei SIDS

Zusammenfassung Es erfolgte eine retrospektive mikromorphologische Analyse der Glandula parotis und/oder der Glandula submandibularis von 180 plötzlichen Kindstodesfällen in Berlin und 75 Fällen in Hamburg. Erfaßt wurden Fälle der letzten 10 Jahre mit einem Alter zwischen 2 Wochen und einem Lebensjahr. — Typische CMV-Einschlußkörper wurden im Berliner Material in 18 Fällen (10%; Mädchen mehr als Jungen) und im Hamburger Material in 6 Fällen (7%; Jungen mehr als Mädchen) diagnostiziert. Während die meisten SIDS-Fälle sich im zweiten und dritten Lebensmonat ereigneten, war bei den CMV-Infektionen kein Altersgipfel festzustellen. Virusmaterial war mit immunhistochemischen Unterschungen sowie in-situ-Hybridisierung auch in histologisch unauffälligen Zellen nachzuweisen. — Unter Berücksichtigung der bekannten klinischen und epidemiologischen Daten zur Cytomegalie stellt die Diagnose einer lokalisierten Infektion der Kopf-speicheldrüsen keine befriedigende Erklärung für den plötzlichen Tod der Säuglinge dar; diese Befunde sind jedoch besonders beachtenswert als Hinweis auf eine Schwäche des Immunsystems.     

Platelet membrane and calcium control abnormalities in essential hypertension

The mechanisms whereby intracellular calcium concentration is controlled are briefly reviewed. With the current knowledge of both calcium homeostasis and the function and properties of cellular Ca2+-target proteins/signal transduction systems, a dysfunction of cellular calcium metabolism is considered in relation to the pathogenesis of hypertension. Although the enhanced peripheral vascular resistance characteristic of hypertension is ultimately a function of Ca2+ availability for smooth muscle cell contraction, the platelet possesses many parallel biochemical and physiologic properties. Therefore, we have utilized the platelet as the cell model for investigating the role of Ca2+ in hypertension disorders. An overview of Ca2+-linked platelet processes altered in essential hypertension is presented, and an attempt is made to integrate these multiple aberrations in a fundamental membrane lesion.     

Prophylactic antibiotic use in pediatric burn units.

Prophylactic antibiotic use in childhood burns is controversial. The efficiency of antibiotic prophylaxis in 77 pediatric burn patients was evaluated. Forty-seven patients received prophylactic antibiotics (Group AP), while 30 patients received no prophylaxis (Group NP). Age, wound depth, day of admission, mechanism of burn injury, type of dressings were similar for both groups (p > 0.05). Wound infection rates were 21.3 % in Group AP and 16.7 % in Group NP (p > 0.05). S. aureus, Enterobacter spp., P. aeruginosa, and E. coli were the most common microorganisms. Patients with wound colonization and infection had a larger burned total body surface area (BTBSA) in both groups (p < 0.01). Eight patients had clinical sepsis. All but one of the septic patients were from Group AP. Associated infections of the upper and lower respiratory tract (16), urinary tract (7), and otitis media (2) were more common in Group AP. One patient died from sepsis in Group AP. Hospital stays were longer in Group AP (21.7 +/- 16.4 vs. 13.5 +/- 10 days; p < 0.05). Antibiotic prophylaxis in childhood burns does not reduce the rate of wound infection. Age, wound depth and BTBSA are not critical variables for prophylaxis. Reinforcing the use of culture-specific antibiotics for more beneficial and cost-effective results in the treatment of childhood burns is recommended.     

A transmembrane protein-tyrosine phosphatase receptor type C (CD45) exon A point mutation (77 C to G) is not associated with the development of type 1 diabetes mellitus in a German population.

To investigate whether a C to G transversion at position 77 in exon A of the CD45 gene is associated with the development of diabetes mellitus type 1 (T1D), we studied 165 patients and 220 control individuals. The frequency of the 77G allele in the control group was 1.1%, which was not significantly different from the 1.2% found in the patient group (P = 0.922). The C to G transversion does not seem to be associated with susceptibility for T1D.     

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures.

Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.     

A practical method of fabricating a lingual retainer.

Retention is a major part of orthodontic treatment, not an optional secondary protocol. In recent years, studies investigating dentofacial changes during and after the growth period have led most clinicians to use fixed retention appliances after treatment. Fixed retainers can be attached to the teeth directly or indirectly. We present a practical, indirect method for bonding fixed retainers, using Sondhi Rapid-set Indirect Bonding Adhesive (3M Unitek, Monrovia, Calif).