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Biomedical subjects

J H Ehrich

Publications and source records attributed to J H Ehrich.

At least 19 recordsLinked to original sources

Ultrasonography in a Senegalese community recently exposed to Schistosoma mansoni infection.

Inhabitants of Ndombo (n = 614), a village in an area recently infected with Schistosoma mansoni in Northern Senegal, were examined clinically, parasitologically, and ultrasonographically to investigate the presence and degree of S. mansoni-related hepatosplenic morbidity after a few years of exposure to schistosomal infection of regional canals. Despite previous praziquantel treatment of 56% of the inhabitants prior to our investigation, the prevalence of S. mansoni infection in 1993 was 90%, and 42% of the villagers excreted more than 1,000 eggs per gram of stool. Previously untreated individuals were found to have significantly higher egg counts than treated ones. Despite the high intensities of infection, ultrasonographically detected severe periportal thickening of the liver was infrequent. Grading according to body length-dependent normal values of cross-section diameter of peripheral portal vein branches of a European control group correlated with intensities of infection. Of the total group of patients, 30% (n = 182) had more severe thickening of portal vein branch diameters above the 97th percentile and 70% of these had a splenomegaly. The highest egg counts and the most frequent development of periportal thickening were found in 11-20 year-old individuals. Periportal thickening was less frequent in praziquantel-treated adolescents than in untreated ones. This suggests that early antischistosomal medication may be useful to limit schistosomiasis-induced hepatic morbidity especially in children, even though reinfection seems inevitable.

Adolescent

Clinical course of hypophosphatemic rickets in 23 adults.

Twenty-three adult patients (19 females, 4 males) with x-linked hypophosphatemic rickets (HPR) underwent a retrospective evaluation of the clinical course and a clinical examination by a nephrologist, orthopedic surgeon and dentist. Blood and urine analysis, bone density measurements with QCT and DEXA, ultrasonic examination of the kidneys were performed and the patients were asked to fill in a standardized questionnaire on pain and psychosocial rehabilitation. Mean final height was 152.4 cm +/- 8.5 SD in females and 157.3 cm +/- 8.9 SD in males. Decreased joint mobility was seen in all patients, deviations of the normal leg axis in 18/23 patients in spite of 69 correcting osteotomies in the past. Dental (n = 14) and psychosocial problems were associated with inability to work (n = 8). There was a trend that patients with a very low Tp/GFR had a more severe course of the disease. Early therapy with vitamin D metabolites and phosphate had a beneficial effect on growth, bone density and deformations. Eight patients had nephrocalcinosis due to vitamin D and phosphate therapy and had normal kidney function. Four patients had urinary tract abnormalities. We conclude that patients with HPR should receive continuous interdisciplinary care given by nephrologists, orthopedic surgeons, physiotherapists and dentists not only during childhood but also as adults.

Adult

Repeated successful pregnancies after kidney transplantation in 102 women (Report by the EDTA Registry).

BACKGROUND: Between 1967 and 1990, 820 successful pregnancies in 718 women on renal replacement therapy (RRT) were reported to the EDTA Registry. METHODS: This study analyses data on repeated successful pregnancies in 102 of these women, of whom 99 had two and three had three pregnancies. RESULTS: Primary renal diseases were mainly glomerulonephritis (41%), pyelonephritis (32%), and congenital malformations such as cystic diseases and hypoplasia or dysplasia (3%). Mean age at start of RRT was 21 years +/-5 SD. Ninety-four per cent of the women had the same transplant during the first and second pregnancies; 85% of these were alive with their first graft and 9% with a second graft; 4% were retransplanted after the first pregnancy and 2% were back on dialysis during the second pregnancy. Of the mothers with two successful pregnancies, two-thirds had a serum creatinine below 121 micromol/l after the first or after the second pregnancy. Six mothers lost their first graft after the first pregnancy. None of the mothers had died after delivery of the second or third baby. Several features of the first and the second pregnancy in these mothers were quite similar. Mean gestational age was 36 weeks+/-3SD during first and second pregnancy. Mean birth weight (height) of the first child was 2490 g+/-660 SD (48 cm+/-4 SD) and 2587 g+/-639 SD (50 cm+/-3 SD) of the second child (NS). Neonatal mortality was 4% after the first and second delivery; congenital abnormalities were found in five and three children respectively. CONCLUSIONS: Fourteen per cent of mothers who had a successful pregnancy on RRT subsequently had a second baby. Repeated pregnancies should not adversely affect graft function and/or fetal development provided that graft function was well preserved at the time of conception.

Adult

Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia.

Focal segmental glomerulosclerosis, nephrotic syndrome and chronic renal failure were associated with spondyloepiphyseal dysplasia, growth failure, lymphopenia and transient ischemic attacks leading to severe neurological symptoms in three children. Two boys and one girl developed the full syndrome at the age of 5, 6 and 10 years. Positron emission tomography revealed perfusion defects of both cerebral and cerebellar arteries. A variant of the disease was found in two other children who had a nephrotic syndrome and terminal renal failure with only mild spondyloepiphyseal dysplasia, impaired growth and a normal cerebral function. It is concluded that there may be a close association between focal segmental glomerulosclerosis and spondyloepiphyseal dysplasias.

Brain

Oxygen transport properties in malaria-infected rodents--a comparison between infected and noninfected erythrocytes.

This study was performed to investigate oxygen transport properties in whole blood (WB) of malaria-infected rats as well as in infected erythrocytes (IE) and noninfected erythrocytes (NIE) separated by density centrifugation. One week after inoculation with Plasmodium berghei, mean parasitemia was 26.5% and high correlations were found between parasitemia and hemoglobin concentration ([Hb]; r = -.902), mean cellular Hb concentration (MCHC; r = -.712), MetHb (r = .923), and base excess (r = -.922). Compared with control animals (C), the oxygen affinity was lower in WB under standard (pH 7.40) and simulated "in vivo" (pH 7.00) conditions (difference in P50, 5.7 and 5.1 mm Hg, respectively; 2P < .01, 2P < .05). In IE Hb and 2,3-biphosphoglycerate (2,3-BPG) concentrations were decreased (MCHC: IE 14.6 +/- 1.0, NIE 33.1 +/- 1.7 g/100 mL; [2,3-BPG]: IE 2.0 +/- 0.6, NIE 7.6 +/- 1.8 mmol/L), whereas [MetHb] and [ATP] were increased ([MetHb]: IE 19.0 +/- 3.7, NIE 0.7% +/- 0.8%; [ATP]: IE 33.5 +/- 2.4, NIE 6.2 +/- 1.0 mumol/g Hb). At pH 7.40, half-saturation oxygen tension (P50) was reduced in IE (29.6 +/- 2.6, NIE 39.2 +/- 5.4 mm Hg, 2P < .001), which correlates with lower [2,3-BPG], increased MetHb content, and higher intrinsic Hb-O2 affinity. However, at pH 7.00, the oxygen affinity was lower in IE when compared with NIE, which was most likely due to high [ATP] in IE. The resulting Bohr coefficients (BC) calculated for CO2 and lactic acid were extremely high in IE and low in NIE (at 50% O2-saturation BCCO2: IE -1.04 +/- 0.06, NIE -0.26 +/- 0.10, 2P < .001; BCLac: IE -0.82 +/- 0.16, NIE -0.47 +/- 0.07, 2P < .001), which was caused by different [2,3-BPG] and [ATP] as well as probably by structural changes of the Hb molecule. The O2 capacity was 14.1 mL per 100 mL erythrocytes in IE compared with 44.4 mL/100 mL in NIE. On the basis of the calculated arterio-venous O2 difference under "in vivo" conditions, the infected red blood cell fraction transports 30% of the O2 amount delivered to the tissues by the noninfected cells (IE 8.0, NIE 26.9 mL/100 mL red blood cells). We conclude that the O2 transport in malaria infected blood is not only affected by the degree of anemia but also by the percentage of infected erythrocytes.

Adenosine Triphosphate

Estimation of ifosfamide/cisplatinum-induced renal toxicity by urinary protein analysis.

Ifosfamide (IFO) chemotherapy has been reported to result in deToni-Debré-Fanconi syndrome in a minority of patients only, but evaluation of tubular transport capacities has identified a substantial number of patients as having subclinical tubular dysfunction. After completion of combination chemotherapy employing IFO (n = 37) or IFO plus cisplatinum (CPL) (n = 27), glomerular and tubular function was assessed in 64 patients by the urinary excretion of transferrin, IgG, albumin, alpha 1-microglobulin (A1M) and N-acetyl-beta-D-glucosaminidase. Sodium dodecyl sulphate polyacrylamide gel electrophoresis was performed in 21 patients. The determination of urinary marker proteins was compared with the glomerular filtration rate, the fractional phosphate and percent amino acid reabsorption. A reduced glomerular filtration rate was observed in 9.8% of patients. Tubular dysfunction was frequent, with a predominance of renal amino acid (57%) and A1M (48%) loss. IFO-mediated renal toxicity was dose dependent. CPL treatment resulted in significant enhancement of tubular toxicity induced by IFO, whereas concomitant gentamicin therapy did not affect tubular function. Measurement of urinary protein cannot replace other tests for tubular dysfunction in IFO-treated patients, because the spectrum of IFO-induced nephrotoxicity includes dysfunction of different and independent transport mechanisms of the proximal tubular system. Increased urinary A1M excretion is an important indicator of impaired tubular protein reabsorption.

Adolescent

Ultrasonography of ureteric abnormalities induced by Schistosoma haematobium infection before and after praziquantel treatment.

OBJECTIVE: To describe the ultrasonographic appearance of ureteric lesions due to Schistosoma haematobium infection and to report prevalence rates of such lesions in endemic communities in Mali before and after praziquantel treatment. SUBJECTS AND METHODS: The total population (n = 961) of three S. haematobium-endemic villages in Mali was examined ultrasonographically to detect urinary tract morbidity. Urine was tested for S. haematobium ova and for haematuria. An identical follow-up study was performed 1 year after mass treatment with praziquantel. RESULTS: Prevalence rates of S. haematobium infection in the three villages were 53%, 72% and 73%. Ultrasonography revealed dilatation of the ureters in 143 subjects (15%). In 20 of these, mainly children and adolescents, thickening and irregularities of the ureteric wall with distal ureter obstruction were directly visualized on ultrasonography. Within the bladder of two others, an intraluminal structure of ureterocele-like appearance was seen. None of these alterations has been previously described as an ultrasonographic feature of schistosomal uropathy. Active S. haematobium infection and microhaematuria were found in 21 and 20 of these 22 subjects respectively. Significant renal obstruction was present in 10 of them. One year after treatment, an entirely normal urinary tract was found in 85% of 104 re-examined individuals who had had ureter dilatation. Ureteric wall thickening or ureterocele-like lesions had disappeared in 95% of subjects. CONCLUSIONS: The role of ultrasound in the evaluation of schistosomal uropathy is confirmed. Ureteric wall abnormalities causing strictures and ureterocele-like lesions of the ostium must be regarded as further ultrasonographic features of this condition. The study documents the excellent reversibility of ureteric abnormalities after antischistosomal treatment in children and adolescents, thus emphasizing the need for early and efficient medical treatment of the infection.

Adolescent

[Why compare hepatosplenic bilharziasis in six African countries with the help of ultrasonography? Presentation of a WHO project including Madagascar].

During a morbidity survey in two endemic foci of hepatosplenic schistosomiasis in the malagasy Hauts Plateaux (Tetikanana, 80 km East of Ambositra and Lohanosy, in the vicinity of Antananarivo), the objectives of a comparative pathology WHO project are presented. The survey in Madagascar was concurrently organized by the Ministry of Health (Bilharziasis central laboratory, DLMT), the Parasitology Unit of the local Pasteur Institute and the Medical Parasitology Institute of Bonn University, in Germany. This was the fourth part of this investigation of the morbidity variation due to Schistosoma mansoni infection in six african countries, a project sponsored by the WHO within its TDR programme. This comparison was based on the standardized description of morbidity as visualized by echography, following the "Cairo-1990" and the "Hannover-Managil-1989" classifications, and on coprology. One of the main objectives of this study was to demonstrate a morbidity variation at the scale of the African region (due to different S. mansoni strains or to a variable genetic background within the studied human populations), while participating to an international standardization effort for field echography. The project was conducted since July 1991 by a constant medical staff from Bonn and Hannover german universities. Eight foci in four african countries (Senegal, Mali, Uganda and Madagascar) are still analyzed and we are presenting here the first results. The data of an independent ultrasonographic investigation, organized by the Blair Institute and the Danish Bilharziasis Laboratory in Zimbabwe, was kindly proposed for this comparison. This project will end with the study of two supplementary foci in Tanzania and Burundi.

Africa

Sonographical morphometrical findings of the liver and spleen in Sudanese patients with Schistosoma mansoni induced periportal fibrosis.

Sonographic measurements of the liver, spleen and related blood vessels were performed in 87 healthy controls and in 50 patients with periportal fibrosis (pF) as well as in 52 patients with pF and at least one episode of gastro-oesophageal bleeding due to chronic Schistosoma mansoni infection. There was a progressing shrinkage of the liver paralleled by an increasing rate of patients with a larger left than right liver lobe as involvement of pF due to S. mansoni infection progressed. This was accompanied by an increasing percentage of pathological gallbladder wall enlargement, splenomegaly and an increasing portal vein diameter. It is suggested that organomorphometrical parameters of the liver and spleen can be used for sonographical evaluation of patients with S. mansoni infection as these may be useful for their clinical assessment of the progression of disease.

Adult

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