Climatic droplet keratopathy with corneal amyloidosis

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.     

Inhibition of Ca2+ transport associated with cAMP-dependent protein phosphorylation in rat cardiac sarcoplasmic reticulum by triorganotins

Organotin compounds have been shown to interfere with cardiovascular system. We have studied the in vitro and in vivo effects of tributyltin bromide (TBT), triethyltin bromide (TET) and trimethyltin chloride (TMT) on the cardiac SR Ca²⁺ pump, as well as on protein phosphorylation of SR proteins, in order to understand the relative potency of these tin compounds. All the three tin compounds inhibited cardiac SR⁴⁵Ca uptake and Ca²⁺-ATPase in vitro in a concentration-dependent manner. The order of potency for Ca²⁺-ATPase as determined by IC₅₀, is TBT (2 M) > TET (63 M) > TMT (280 M). For⁴⁵Ca uptake, it followed the same order i.e., TBT (0.35 M) > TET (10 M) > TMT (440 M). In agreement with the in vitro results, both SR Ca²⁺-ATPase and⁴⁵Ca uptake were significantly inhibited in rats treated with these tin compounds, indicating that these tin compounds inhibit cardiac SR Ca²⁺ transport. cAMP significantly elevated (70–80%) the³²P-binding to SR proteins in vitro in the absence of any organotin. In the presence of organotins, cAMP-stimulated³²P-binding to proteins was significantly reduced, but the decrease was concentration dependent only at lower concentrations. The order of potency is TBT > TET > TMT. In agreement with in vitro studies, cAMP-dependent³²P bound to proteins was significantly reduced in rats treated with TBT, TET and TMT. SDS-polyacrylamide gel electrophoresis of the cardiac SR revealed at least 30 Coomassie blue stainable bands ranging from 9 to 120 kDa. Autoradiographs from samples incubated in the presence of cAMP indicated³²P incorporation in seven bands. Of these, the band corresponding to about 24 kDa molecular weight protein decreased in its intensity with the treatment of organotins. These results suggest that triorganotins may be affecting Ca²⁺ pumping mechanisms through the alteration of phosphorylation of specific proteins in rat cardiac SR.This work has been presented in part at the Annual meeting of Society of Toxicology, 1990 at Miami Beach, FL. The Toxicologist 10: 35 & 108 (1990).     

Design rationale of a small ingrowth acetabular component: two to three years' results

It is possible to use a noncemented porous metal-backed acetabular component with an outer diameter as small as 39mm with safety. A two to three year follow-up of the first 40 cases in which this type of acetabular component was used has revealed no particular problems. These small acetabular components have considerably reduced the necessity for acetabular bone grafting in patients with hip dysplasia.     

Tricalcium phosphate as a bone graft substitute

A clinical trial is reported that was conducted in an attempt to establish a timeframe for replacement of tricalcium phosphate by bone. In 20 cases, an 8.5mm by 3mm disc of tricalcium phosphate was implanted into the cut surface of the tibia at the time of total knee replacement. When followed radiographically, the disc of tricalcium phosphate generally could not be identified at six months. At two years, no untoward effects have occurred and no vestigial shadows remain, indicating that the block has fully incorporated and largely been absorbed and replaced by host bone. The experience in this series of cases indicates that tricalcium phosphate is a useful resorbable bone filler material.     

Tubular and interstitial factors in the progression of glomerulonephritis

All recent studies of the outcome of different forms of progressive glomerulonephritis concur that a major factor, apparently determining outcome, is the presence and severity of tubulointerstitial changes, and not the degree of glomerular alteration. Moreover, at the time of biopsy, tubulointerstitial changes correlate much better with the glomerular filtration rate. These at first surprising findings are not only useful clinically, but should make us think about our models of how progression takes place in so-called glomerular nephritides. In fact, a major tubulointerstitial infiltrate of immune-competent cells is present in all forms of progressive glomerulonephritis, and again correlates with outcome. In addition, it is now clear the tubular epithelium is capable of synthesising and secreting a number of factors important in fibrogenesis, and of displaying major histocompatibility complex class II antigens and leucocyte-adhesion molecules. Tubular cells could thus present peptides to T helper cells and amplify, or maybe even initiate, immune reactions. Finally, fibrogenesis within the kidney is at last being studied, long after studies have been performed on liver and lung. In the past, too much attention has been paid to reversible inflammation and not enough to irreversible cirrhosis of the kidney.Abbreviations used Ig immunoglobulin, e. g. IgA, IgG, IgM etc. - TBM tubular basement membrane - GBM glomerular basement membrane - WHO World Health Organization - MHC major histocompatibility complex - CD cluster determinant - NK natural killer - IL-1 interleukin-1 - IL-2 interleukin-2 - TNF- tumour necrosis factor alpha - ADCC antibody-dependent complement mediated cytolysis - ACE angiotensin-converting enzyme - m macrophage - ICAM-1 intercellular adhesion molecule-1 - ELAM-1 endothelial leucocyte adhesion molecule-1 - VCAM-1 vascular cell adhesion molecule-1 - LFA leucocyte function associated molecule, e. g. LFA-1, LFA-3 - C complement e. g. C3=third component of complement, etc. - TGF- transforming growth factor beta - TGF- transforming growth factor alpha - PDGF platelet derived growth factor - PAS periodic acid Schiff - TCR T cell receptor - PTEC proximal tubular epithelial cell - GM-CSF granulocyte colony stimulating factor - M-CSF monocyte colony stimulating factor - FGF fibroblast growth factor     

Use of a distal trochanteric osteotomy in hip revision

Revision of some Moore stems with a straight lateral shoulder where the stem has subsided distally and laterally may be difficult and can result in fracture of the greater trochanter. A trochanteric osteotomy that extends a long way down the lateral cortex of the femur facilitates this type of revision. A similar approach simplifies revision of proximally-fixed noncemented stems.     

Multiple mononeuropathy as the initial presentation of systemic lupus erythematosus — nerve biopsy and response to plasma exchange

Summary A total of 15 patients affected by idiopathic dystonia (7 with generalized and 8 with focal or segmental dystonia) were subjected to therapy with bromocriptine at low doses, pimozide and trihexyphenidyl. The symptoms were evaluated by giving a progressive score in relation to the intensity of the dystonic symptom to each of the body segments involved by the dystonia. Bromocriptine did not significantly modify the dystonia. Pimozide showed a slight nonsignificant improvement of the dystonic symptoms. Trihexyphenidyl was effective in the generalized dystonias, in agreement with previous reports in the literature. The variation in the pharmacological results could be due to the diversity of the dystonic syndromes, which comprise cases that are different in age at onset, site of dystonic symptoms, and evolution.

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Zusammenfassung Fünfzehn von idiopathischer — und zwar 7 von generalisierter und 8 von fokaler und segmentarischer — Dystonie befallenen Patienten unterzogen sich verschiedenen pharmakologischen Behandlungen mit kleinen Mengen Bromocriptine, Pymozide und Triesifenidile. Die Symptome wurden durch eine fortlaufende Punktzahl bezeichnet, so daß deren Schätzung von der Intensität des Symptoms Dystonie in jedem einzelnen befallenen Körperteil abhing. Die Dystonien wurden durch Bromocriptine nicht bedeutend geändert.Pymozide führte zu einer geringeren, doch unbedeutenden, Besserung der dystonischen Symptome.Triesifenidile wirkte auf die generalisierten Dystonien, in Übereinstimmung mit einigen Literaturangaben.Die Veränderlichkeit der pharmakologischen Ergebnisse wurde auf die Verschiedenheit der dystonischen Syndrome zurückgeführt, unter denen man Fälle versammelt, die sich durch Anfangsalter, Sitz der dystonischen Symptome und Entwicklungsart voneinander unterscheiden.

     

Plasma gonadotrophic hormones, testicular biopsy and seminal analysis in the men of infertile marriages.

The first step in the investigation of infertile men is to obtain 2 seminal analyses 3 days after the previous ejaculation. Clinical assessment of testicular size is an unreliable means of assessing spermatogenesis and is best done by performing bilateral testicular biopsies. Azoospermic men with grossly elevated FSH levels should be advised to consider AID or adoption. Those with normal or mildly elevated FSH levels should undergo testicular exploration in an attempt to correct an obstructive lesion. Azoospermic men with subnormal FSH levels may have an isolated hormone defect which will respond to treatment with Pergonal. Different treatment programmes for oligozoospermic men depending on the results of the FSH and LH assays may lead to a more rational approach to therapy. Infertility associated with varicocele may possibly be associated with a local disturbance of "inhibin" and FSH concentrations.     

A prospective study of serum pseudocholinesterase levels in patients with chronic spinal pain: a preliminary study

STUDY DESIGN: One-dimensional polyacrylamide gel electrophoresis was used to study serum esterase enzymatic activity in three groups of patients and one group of normal volunteers. OBJECTIVES: To determine whether there is a statistically significant correlation between variations of serum pseudocholinesterase and the perception of pain in patients with chronic spinal pain. SUMMARY OF BACKGROUND DATA: Changes in levels of cholinesterase in the extracellular space of the brain and in the cerebral spinal fluid have been found to be associated in animal pain experimentation. METHODS: Ninety-three surgical patients with chronic spinal pain, six surgical control subjects operated for conditions not associated with pain, 21 normal control volunteers, and nine disabled patients receiving monetary benefits were studied. The patients were analyzed for a period of time by rating the perception of their pain with a visual assessment score at the time venous blood was drawn. Serum samples were prepared, serum pseudocholinesterase was monitored, separated, and quantified according to Allen et al.5 Paired sample t tests were used to statistically evaluate the data. RESULTS: A trend of correlation was noted between preoperative serum pseudocholinesterase levels and visual assessment score: serum pseudocholinesterase levels increased as visual assessment score increased. The mean preoperative serum pseudocholinesterase level of chronic spinal pain patients (1313; SE = 26), which was significantly higher than the mean levels of the normal control volunteers (941; SE = 24; P<0.001) and that of surgical control subjects (1018; SE = 63; P <0.01), decreased significantly with anesthesia (P<0.005). The mean preoperative serum pseudocholinesterase level of the surgical controls, however, remained unchanged with anesthesia. A correlation demonstrated between visual assessment score and serum pseudocholinesterase in chronic spinal pain patients was not observed in six of nine patients receiving disability payments for more than a year. CONCLUSIONS: Measurements of quantitative alterations of serum pseudocholinesterase levels may be useful in the treatment of patients with chronic spinal pain.