Craniofacial morphology/phenotypes influence on mandibular range of movement in the design of a mandibular advancement device.

BACKGROUND: The mandibular opening path movements have different directions according to the craniofacial morphology of the patient but always downward and backward, therefore increasing the collapse of the upper airway. The aim of this work is to determine if there is a relationship between the craniofacial morphology and the mandibular movement to help understand the impact on the mandibular position. METHODS: 52 students with full permanent dentition aged 19 to 23 years (mean 21.3 SD 1.7; 29 females and 23 males), participated in the study. Each subject had a lateral cephalometric radiograph taken. The opening angle was determined for two levels of vertical openings at 5 and 10 mm. RESULTS: The opening angle showed a greater variability between subjects ranging from 63.15 to 77.08 for 5 mm angle and from for 61.65 to 75.72 for the 10 mm angle. Differences of facial phenotypes was evident when comparing the individual dissoccluding angle of the low angle horizontal pattern and high angle vertical pattern. CONCLUSIONS: The opening angle is related to craniofacial morphology with higher vertical anterior and shorter anteroposterior faces having a more horizontal path of mandibular movement than shorter vertical anterior and longer anteroposterior subjects who have a more vertical path.

[Baclofen-associated encephalopathy in a hemodialysis patient with hiccups]. / Encefalopatía asociada a baclofeno como tratamiento del hipo refractario en un paciente en hemodiálisis.

Baclofen is a centrally acting gamma-ammino butyric acid agonist that is used like muscular relaxant in disorders with spasticity and intractable hiccups. Although encouraging and safe results were provided 5 mg/day in hemodialysis patients, his pharmacokinetic and pharmacodinamic properties are not well known in end stage renal disease. We present here the case of a hemodialysis patient with intractable hiccups who developed baclofen-associated encephalopathy with this recommended dose.

Familial IgA nephropathy: report of two cases and brief review of the literature.

Immunoglobulin A nephropathy developed in two siblings who shared the DRw4 antigen but who were otherwise HLA-A, -B, and -C unrelated. It is suggested that a genetically induced alteration in the immune response can cause some of the cases of mesangial IgA glomerulopathy and that close blood reltaives of patients with this disease should be checked for the possible existence of renal abnormalities.

Electron-dense deposits in the renal arterioles of two patients with hypersensitivity vasculitis.

Immune complex deposition in the vessel wall is presumed to be the major pathogenetic mechanism leading to hypersensitivity vasculitis. Despite this, histologic evidence of vascular immune complex deposition such as that provided by electron-dense deposits has been reported only rarely in dermal vessels and never in visceral organs. The cases of two patients with hypersensitivity vasculitis affecting primarily the skin and the kidney are reported. Clinical renal involvement was manifested by proteinuria, hematuria, and a moderate increase in serum creatinine in one case. Renal biopsy showed minimal glomerular changes in one patient and focal necrotizing glomerulitis in the other. The arterioles appeared normal on light microscopic examination. However, obvious electron-dense deposits in the arteriolar wall could be demonstrated electron microscopically in both cases. This observation lends strong support to the theory of immune-complex-mediated vascular damage as the main pathophysiologic mechanism in vasculitis with visceral involvement.

High-dose busulfan and melphalan before bone marrow transplantation for acute nonlymphoblastic leukemia.

Fourteen patients with acute nonlymphoblastic leukemia (ANLL) (n = 13) or juvenile chronic myelomonocytic leukemia (n = 1) were transplanted after conditioning with high-dose busulfan (4 mg/kg daily on days -7 to -4) and melphalan (180 mg/m2 on day -2). This protocol was designed for patients considered unable to receive standard conditioning regimens with cyclophosphamide and/or TBI. Five patients (4 children and 1 adult) received a second allogeneic BMT in untreated early marrow relapse after a first BMT. There were 3 procedure-related deaths (PRD), 2 during aplasia and 1 from acute GVHD. Two patients survived the procedure; 1 relapsed at 6 months and 1 is alive at 43+ months. Nine subjects (8 children and 1 adult) received an autologous BMT, 7 in first and 2 in second complete remission (CR). Of the 7 patients grafted in first CR, there was 1 PRD, 2 relapses at 3 and 15 months, and four are alive at 38 to 82+ months. One patient grafted in second CR relapsed at 7 months and 1 is alive at 67+ months. Toxicities were mild or moderate in autologous BMT recipients, mainly affecting the gastrointestinal tract. In the allogeneic BMT group, there were more moderate to severe toxicities, including 3 cases of moderate-severe renal toxicity; no cases of such toxicity were seen in ABMT recipients. Two cases of HVOD occurred, 1 in each group. These results are encouraging, although the small patient group does not allow any firm conclusions.

Autologous hemopoietic reconstitution after fetal liver infusion in patients with bone marrow failure: consequence or coincidence?

In the past 4 years we have treated four patients with a total of 19 fetal liver infusions (FLI). Two cases of refractory anemia with excess blasts in transformation (RAEB-t) were conditioned with cyclophosphamide and total body irradiation (1400 cGy) and were treated with FLI. In spite of such intensive conditioning, one patient recovered autologous hemopoiesis 3 weeks later, remaining in remission 4 years after this procedure. The second patient died with aplastic marrow on day 154, and the third suffered from severe aplastic anemia refractory to several types of conventional treatment. After FLI and without previous conditioning therapy a partial fetal engraftment was documented. This was transient and followed by autologous hemopoietic recovery and cure of the disease. The fourth patient had bone marrow failure in the setting of a severe pneumonia following autologous bone marrow transplantation. Ten days after FLI the hematological parameters dramatically improved and the pneumonia resolved. Autologous reconstitution of hemopoiesis was demonstrated. These experiences suggest that FLI might stimulate autologous hemopoiesis. This therapeutic approach may be useful to treat bone marrow failure when there is no response to first-line therapy. In hematologic malignancies with an indication for stem cell transplantation, other sources such as allogeneic or autologous bone marrow seem preferable to fetal liver cells.

Acute and rapidly progressive forms of glomerulonephritis in the elderly.

This article presents a comparison of acute glomerular nephritis (AGN) with rapidly progressive glomerular nephritis (RPGN) in patients aged 60 or older. In 7 elderly patients with AGN, the renal disease was preceded by skin infection (4 cases), sore throat (2 cases), or pneumonia (1 case). The 7 patients with RPGN had no history of prior infection. Both AGN and RPGN were manifested clinically as acute renal failure, but the RPGN patients had significantly higher serum creatinine levels and lower hematocrit readings. Hypocomplementemia was a feature only of AGN. The biopsy specimens from patients with RPGN showed crescents in 50--100 percent of the glomeruli, whereas specimens from patients with AGN showed no significant extracapillary proliferation. Six AGN patients recovered and 1 died. Despite dialysis, 4 RPGN patients died and the remaining 3 require maintenance dialysis. It is concluded that AGN in the elderly is more common than previously believed, frequently follows skin infections, and has a reasonably good prognosis. In contrast, RPGN, also not rare in the elderly, has a much worse prognosis.

Cardiovascular risk factors and the long-term outcome of lupus nephritis.

We evaluated cardiovascular risk factors, morbidity and mortality in patients with lupus nephritis (LN). We prospectively studied 70 consecutive patients with LN, and 70 age- and sex-matched controls with systemic lupus erythematosus (SLE) but no evidence of nephropathy, from 1988 to 1998. Patients were evaluated at entry for hypertension, diabetes, hyperlipidaemia, smoking, menopause and antiphospholipid syndrome. The LN patients (64 women, 6 men) had a mean age of 35 years (SE 1.7, range 11-67). During the 10 years, 15 (21%) LN patients and 18 (25%) of the controls were lost to follow-up. Compared with controls, LN patients had a higher prevalence of hyperlipidaemia (44% vs. 2%, p<0.001), hypertension (44% vs. 9%, p<0.001) and antiphospholipid antibodies (45% vs. 22%, p=0.01) at study onset. At the last visit, 37 (67%) LN patients had normal plasma creatinine, 13 (24%) had renal failure and only five (9%) end-stage renal failure. Hyperlipidaemia (78% vs. 27%, p<0.001) and hypertension (67% vs. 32%, p=0.01) at study onset were associated with development of renal failure. Nine LN patients and one control died (16% vs. 2%, p=0.02). These patients showed more antiphospholipid syndrome (56% vs. 17%, p=0.03) and hyperlipidaemia (78% vs. 37%, p=0.03) at study onset. The main causes of death in LN patients were vascular complications (cardiovascular or cerebrovascular events) in five patients (four of whom had antiphospholipid antibodies) and sepsis in three.

Renin-induced massive proteinuria in man.

A 30 year old man developed renovascular hypertension and extreme elevation of plasma renin activity. Daily proteinuria ranged from 13 to 31 g. There were no criteria for the diagnosis of malignant hypertension. A primary glomerulopathy was excluded by a basically normal renal biopsy from the unprotected kidney. On electron microscopy the epithelial cell foot processes were not fused, thus ruling out simultaneous lipoid nephrosis. The source of renin was removed by means of a left nephrectomy. Following the procedure the patient became normotensive, the renin values normalized and the proteinuria disappeared. The results suggest that renin can cause significant proteinuria in man.

Long-term evolution of patients with isolated C3 mesangial glomerulonephritis.

Isolated mesangial C3 proliferative glomerulonephritis is a nephropathy poorly defined among glomerular diseases. Clinical picture is characterized by episodes of gross hematuria and/or persistent or recurrent microhematuria and/or proteinuria. Short-term prognosis (less than 3 years) is considered to be benign, although not much information is available in reference to long-term follow-up. We reviewed all renal biopsies performed in our center between 1978 and 1992 (n = 2,200) in order to study clinical course of these patients. Isolated mesangial C3 deposition was found in 11 cases (0.5%). Isolated proteinuria (> 0.5 g/d) was present in 3 patients and nephrotic syndrome in 2. Hematuria with proteinuria was detected in 5 patients. In one patient hematuria was the only clinical finding. Arterial hypertension was observed in 4 cases. At the time of renal biopsy, renal function was accurately normal in all but one patient. In our series, renal function showed no changes during the first 3 years, accordingly to data referred to in the literature. After 7 years of follow-up, however, renal function was declining in 4 of 5 patients who developed terminal renal failure up to requiring hemodialysis. Therefore, initial benign prognosis ascribed to this glomerulonephritis would not be confirmed in a long-term follow-up.

Silent renal disease in systemic lupus erythematosus.

Several recent studies have focused on the discrepancy between lupus nephropathy and clinical renal involvement and, consequently, question the relevance of renal biopsy in these patients. We analyze the clinical characteristics, histological renal findings and subsequent course of patients with silent renal disease. Renal biopsy was performed in 15 patients with systemic lupus erythematosus (SLE) who had no clinical signs of renal involvement (no urinary sediment abnormalities, absence of proteinuria and serum creatinine less than 1.3 mg/dl). All biopsies were classified according to a modified classification proposed by the WHO. Six cases (40%) showed no histological or immunofluorescence changes (type I), 7 (47%) had mesangial nephropathy (3 type IIa and 4 type IIb) and 2 (13%) had focal proliferative glomerulonephritis (type III). None of the patients had previous evidence of neurological abnormalities. Patients with type I only had arthritis, skin lesions and Raynaud's phenomenon. By contrast, 7 patients with histological renal involvement had serositis or hemolytic anemia. All cases with silent nephropathy were treated with steroids and showed a benign clinical course with stable renal function and absence of urinary abnormalities during follow-up. We concluded that in the absence of clinical renal abnormalities, renal involvement is not uncommon in SLE. We believe that a renal biopsy should be performed mainly in those SLE patients presenting with clinical manifestations other than arthritis or cutaneous lesions since this policy may allow detection of significant silent renal injury.

Acute visual and auditory neurotoxicity in patients with end-stage renal disease receiving desferrioxamine.

We report 5 cases of acute visual and auditory neurotoxicity in patients with end-stage renal disease receiving desferrioxamine. Four patients complained of visual toxicity characterized by a decrease in visual acuity, loss of color vision and night blindness. In these patients there was a decrease in visual acuity, and the Farnsworth's test revealed a tritanopy (yellow-blue axis impairment); 1 patient also showed retinal pigmentary changes. A fifth patient complained of bilateral and severe neurosensorial deafness. Patients improved clinically upon drug-withdrawal. We recommend careful monitoring of audiovisual toxicity in patients with end-stage renal disease receiving this drug.

Fibrillary glomerulonephritis and pulmonary hemorrhage in a patient with renal transplantation.

Fibrillary glomerulonephritis is an unusual kidney disease characterized by the deposition of immunoglobulins in a fibrillar pattern. Until recently it has been considered to involve the kidneys alone. We describe a patient who underwent renal transplantation and developed fibrillary glomerulonephritis with rapidly progressive renal failure and severe pulmonary hemorrhage two years and a half after transplantation. Nephropathy prior to transplantation was thought to be focal and segmental glomerulosclerosis. Diagnosis of fibrillary glomerulonephritis in renal allograft was confirmed by postmortem examination. 50% of the glomeruli with extracapillary crescents were observed on light microscopy. By immunofluorescence main deposition of IgA was detected in the glomerular capillar walls and the mesangium. Electron microscopy showed fibrillo-reticular deposits in the same place. Lung histology showed both old and recent areas of alveolar hemorrhage. Granular staining for IgA was observed in the alveolar walls by immunofluorescence. Ultrastructural analysis of the lung made evident fibrillo-reticulary deposits in the interstitium, similar than those observed in the glomeruli. The presence of these deposits in both renal and pulmonary tissues indicates the possibility of systemic involvement in fibrillary glomerulonephritis. In our case it could be related to the recurrence of this glomerulopathy in renal allograft.

Adverse effects secondary to the treatment with plasma exchange.

Side effects and their relationship with the material used were analyzed in 748 plasma exchanges (PE) performed in 75 patients. The total incidence of acute and mild adverse effects (chills and/or fever, paraesthesias, allergic reactions, acute anaemia, vasovagal reactions, abdominal pain and hypotension) was 18.04%. Two patients developed an episode of left cardiac insufficiency. One patient in whom all PE were performed with fresh frozen plasma (FFP) developed metabolic alkalosis. Three patients developed sepsis during treatment with repeated PE and immunosuppressive drugs; in these three patients a permanent vascular inlet was used (shunt or catheter). All patients in whom only FFP was used as replacement solution developed non-A, non-B hepatitis. Neither haemorrhagic nor thrombotic episodes were observed in these patients. It is of the greatest importance to choose the most suitable material for each patient and to develop a careful technique in order to avoid these complications during treatment with PE.

[Hemolytic-uremic syndrome in the adult (author's transl)]. / Síndrome urémico-hemolítico del adulto.

This article presents six cases of hemolytic-uremic syndrome, defined as the combination of acute renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia, in six adult patients, two men and four women, between 20 and 52 years old. Three of the cases were considered idiopathic, two secondary to the use of oral contraceptives, and one appeared after an abortion. All of the patients presented severe hypertension, frequently accompanied by increased renin levels; in no cases was there any important coagulation disorder. In all of the biopsies there were lesions denoting intravascular thrombosis in the arterioles and medium-sized arteries of the kidney, as well as thickening of the glomerular basal membrane. Immunofluorescence was positive for fibrinogen and C3 in the majority of biopsies examined. Two patients suffered acute pancreatitis, hypertension having perhaps been an important factor in this complication. One of the two patients died as a result of acute hemorrhagic pancreatitis and was the only death in the series. Of the five remaining subjects, three required periodic hemodyalisis and the other two presented a considerable degree of chronic renal failure, which confirms that the prognosis for the hemolytic-uremic syndrome is much worse for the adult than for the child.

[Lupus nephritis: clinicomorphological manifestations (author's transl)]. / Nefropatía lúpica: manifestaciones clínicas y morfológicas.

Forty patients with systemic lupus erythematosus have been evaluated for clinicopathological evidence of renal involvement. Average age at the onset of the disease was 23 years; 90 percent of the patients were women. Indications for renal biopsy included clinical symptoms of nephropathy (34 patients) and signs of immunological activity (6 patients(. All biopsied specimens were examined under light microscope; 28 of them under immunofluorescence, and 18 under electron microscope. Histological classification was as follows: I) Normal kidney (2 patients); II) mesangial glomerulonephritis (6 patients); III) focal glomerulonephritis (10 patients); IV) diffuse proliferative glomerulonephritis (19 patients), and V) membranous glomerulonephritis (3 patients). Six patients had no clinical nephritis, two of them had normal kidneys and four had a mesangial glomerulonephritis. Patients in groups III, IV, and V showed clinical and biological evidence of renal involvement. Highest incidence of renal failure, nephrotic syndrome or high blood pressure was found among cases in group IV. Group IV accounted also for the highest mortality rate. Clinical course of 25 cases (1 of type II, 8 or type III, and 16 of type IV) on corticosteroid therapy for an average follow-up of 22.8 months has showed greater clinical improvement (proteinuria and renal function) of nephropathy type III than nephropathy type IV. Renal biopsy is a useful diagnostic and prognostic method in patients with systemic lupus erythematosus, even in those cases without clinical evidence of renal disease.

[Incidence and characteristics of glomerular diseases after kidney transplant]. / Incidencia y características de las enfermedades glomerulares postrasplante renal.

The incidence of glomerular diseases was retrospectively reviewed in our population of renal transplants performed during the period 1972-1988. Overall, 42 instances of post-transplant glomerular disease were observed in 39 patients. The most common type of glomerular disease was transplant glomerulopathy in 13 cases, followed by membranous glomerulonephritis in 8. Regarding etiology, relapse of the original nephropathy was considered to be present in 15 patients and de novo glomerular disease in the remaining 27. In our series, the incidence of clinical relapses was 12%, ranging between 75% in hemolytic-uremic syndrome and 8% in mesangiocapillary glomerulonephritis. 50% of those patients who developed glomerular disease after transplant had a loss of their grafts during the follow up period. The survival of the graft, from 6.5 years after transplant onwards, was significantly lower than that seen in the group of transplanted patients without glomerular disease.

[Subacute kidney insufficiency in exclusively vascular kidney amyloidosis]. / Insuficiencia renal subaguda en la amiloidosis renal exclusivamente vascular.

Two patients are reported with secondary amyloidosis in whom renal biopsy disclosed massive amyloid deposition with exclusively extravascular localization. In one case, autopsy showed selective amyloid deposition in the vessel walls of most examined organs. In both patients, the clinical presentation consisted of subacute renal failure without proteinuria. A possible reduction of renal perfusion, secondary to a depleted blood volume in one case and to pharmacological inhibition of prostaglandin synthesis in the other, could enhance renal dysfunction in both patients, who, on the other hand, previously had mild renal failure. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.

[Nephropathy in human immunodeficiency virus infection]. / Nefropatía en la infección por el virus de la inmunodeficiencia humana.

BACKGROUND: Patients with the human immunodeficiency virus (HIV) infection may present different types of nephropathy with focal segmentary glomerulosclerosis (FSGS) being the most specific lesion associated to HIV (HIVN). The prevalence of this entity varies between 1 to 23%. These differences have been attributed to ethnic factors and/or intravenous drug addiction (IVDA). The renal alterations presented in these patients with HIV infection were analyzed. METHODS: Two groups of patients were studied: Group I with 300 consecutive patients with advanced HIV infection (complex related with AIDS) treated with zidovudine without initial kidney infection followed for a mean of 19 +/- 11 months (99% caucasian, 65% IVDA, 66% AIDS); and group II with 11 patients with HIV infection remitted for kidney involvement (4 on hemodialysis). RESULTS: Group I: 26 cases (9%) presented acute reversible kidney failure (12 prerenal, 9 nephrotoxicity and 5 mixed). No patient presented HIVN criteria. Group II: one black patient developed HIVN and had no known risk factors. The remaining patients presented glomerular changes different from those of HIVN. CONCLUSIONS: The scarce prevalence of human immunodeficiency virus nephropathy in the present series is of note despite the high number of intravenous drug addicts included suggesting that ethnic factors determine the differences observed in different series. The most frequent kidney disturbances seen are those related with infectious complications associated to the acquired immunodeficiency syndrome and the treatment of the same.

[Systemic lupus erythematosus: a clinical and immunological study of 300 patients]. / Lupus eritematoso sistémico: estudio clínico e inmunológico de 300 pacientes.

BACKGROUND: The aims of the present study were to analyze the clinical and immunologic characteristics of a wide group of patients with systemic lupus erythematosus (SLE) and define homogeneous subgroups with their own characteristics. METHODS: A prospective study of 300 patients diagnosed of SLE were studied. These patients were subdivided according to sex, age at the onset of the disease and immunologic profile. The statistical study was carried out by the chi (2), Fisher, Student's t and Mann-Whitney U tests. RESULTS: The series was made up of 266 (89%) females and 34 (11%) males. The mean age at onset of the disease was 31.8 +/- 14.6 years. In 48 (16%) patients the first manifestations appeared after the age of 50. Males were shown to present a lower prevalence of arthritis (59% vs 82% in woman, p < 0.005) and malar rash (29% vs 50%, p < 0.05), but had more cutaneous discoid lesions (18% vs 3% p < 0.001). In patients in whom the disease appeared after the age of 50 a lower prevalence of arthritis was presented (67% vs 82% in patients of less than 50 years of age, p < 0.005), malar rash (23% vs 53%, p < 0.001) and nephropathy (21% vs 41%, p < 0.05), but had greater myositis (17% vs 6%, p < 0.01). The absence of antinuclear antibodies (ANA) and the presence of anti-ds DNA and anti-ENA antibodies were associated with differences in the prevalence of different clinical manifestations. CONCLUSIONS: Sex, age and immunologic pattern in systemic lupus erythematosus permit the definition of homogeneous subgroups with their own characteristics: a) males present a lower prevalence of arthritis and malar rash, but a greater prevalence of cutaneous discoid lesions; b) patients over the age of 50 develop arthritis, malar rash and nephropathy with a lower prevalence but have a greater prevalence of myositis; c) patients without antinuclear antibodies and those with anti-ds DNA and anti-ENA antibodies present differences in the prevalences of different clinical manifestations.