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Biomedical subjects

H Zappel

Publications and source records attributed to H Zappel.

14 recordsLinked to original sources

Bladder dysfunction in children with bilateral single ectopic ureters.

BACKGROUND: Single ectopic ureters are a rare malformation in children. Therapy consists of ureteral reimplantation. However, in case of bilateral single ectopic ureters, subsequent malformation of the bladder trigone and bladder neck may result in additional voiding dysfunction, and ureteral reimplantation alone may not solve the urologic problems. METHODS: The authors report their experience with 2 girls, in whom bilateral single ectopic ureters were treated by ureteral reimplantation in early childhood and who did not gain adequate bladder control during following years. RESULTS: Videourodynamic evaluation was done in both girls. No bladder overactivity was found during the urodynamic studies. However, cystography showed a widely open bladder neck during filling with no sufficient bladder neck closure shown by urethral pressure profile studies. When blocking the bladder outlet by balloon catheters, adequate bladder filling volume was achieved. Incontinence was cured by implantation of an AMS 800 artificial sphincter system in a 10-year-old girl. A 7-year-old girl was regarded to be too young for sphincter implantation and is waiting for surgery within the next years. CONCLUSION: Insufficient development of trigone and bladder neck with subsequent urinary incontinence has to be kept in mind when deciding on surgical procedures in children with bilateral single ectopic ureters.

Abnormalities, Multiple↗

Effective aromatase inhibition by anastrozole in a patient with gonadotropin-independent precocious puberty in McCune-Albright syndrome.

Testolactone is used to treat conditions with excessive estrogen synthesis, e.g. gonadotropin-independent precocious puberty in McCune-Albright syndrome (MAS). Unfortunately, daily treatment with testolactone requires 3 to 4 doses (10-20 tablets) and even at these doses it is sometimes ineffective. We treated a patient with MAS (café-au-lait spots; thelarche at age 2- 6/12 yr; menarche at 5- 5/12 yr; accelerated bone age [BA 10 yr]) with the highly selective aromatase inhibitor anastrozole (1 mg once per day). Tamoxifen 1 mg/kg per day was added for 1 year but was discontinued when an ovarian cyst developed with markedly elevated estradiol levels. Estradiol levels returned to normal after resuming anastrozole-only treatment and accelerated BA progressed only 6 months during 2 1/2 years of treatment. The potent estrogen suppressive action and simple dosage regimen of anastrozole suggest it may be advantageous compared to other aromatase inhibitors such as testolactone or anti-estrogens.

Anastrozole↗

[The iodine supply of newborns. Comparison of iodine absorption and iodine excretion of mother and child].

BACKGROUND: Since 1989 the use of iodized salt has been allowed in Germany, additional supplementation with iodide tablets has been recommended during pregnancy and lactation. This study was undertaken to clarify whether the iodine intake of neonates and young infants improved since then. PATIENTS AND METHODS: In the first part of the study the urinary iodine excretion of 52 newborns and their mothers in 1998 was compared to data of similar studies 1983 in the area of Göttingen and 1982 in the areas of Heidelberg and Rothenburg, Germany. All these are geographically low-iodine areas. In the second part the iodine supply of infants in 1998-1999 under feeding with mother's milk or formulas in 1998 and 1999 was obtained by measuring iodide concentrations in urine and milk using a high pressure liquid chromatography (HPLC) method. RESULTS: 45% of pregnant women were without iodide supplementation in 1998. In 1998 the median urinary iodide concentration during the first week of life was 4.3 micrograms/dl, which was more than twice that found in 1983 (1.75 micrograms/dl). Infants feeding by mother's milk without maternal iodine supplementation or by semi-elementary diet had the lowest urinary iodine excretion, whereas significantly higher values were measured when feeding formulas for term or preterm infants. CONCLUSIONS: The iodine intake of newborns has markedly improved during 15 years. The WHO criterias for adequate iodine supply (TSH < 5 microU/ml and urinary iodine >/ = 10 micrograms/dl) were only partly fulfilled in Göttingen indicating that a mild iodine deficiency still exists with the risk of iodine deficiency disorders.

Bottle Feeding↗

Immunoscintigraphy (BW 250/183) in neonates and infants with fever of unknown origin.

Fever of unknown origin is defined as a temperature above 39.0 degrees C together with a white blood cell count > or = 15,000 mm-3, the duration of fever exceeding 2 weeks and a correct diagnosis not being obtained in the first week of hospitalization. In neonates and infants with fever of unknown origin, the localization of the infectious focus is often difficult and unsatisfactory. In this retrospective study, the clinical value of 99Tcm-labelled antigranulocyte antibodies for this group of patients was investigated. Thirty-two immunoscintigrams were performed using 185-259 MBq 99Tcm-labelled antigranulocyte antibodies (BW 250/183) in 30 neonates and infants (21 boys, 9 girls, mean age 29.4 +/- 2 months), who had fever of unknown origin. Immunoscintigraphy was carried out as whole-body images (n = 7) or single planar images (n = 25) 4 h and 24 h post-injection. In children with known cardiac failure, single photon emission tomography of the thorax was performed to diagnose endocarditis (n = 2). For verification, the results of the immunoscintigrams were compared with radiology (conventional radiography = 14, MRI = 5, CT = 3), biopsy (n = 2), blood culture (n = 10) and clinical follow-up after specific therapy. In 11 of 30 children (36%), the diagnosis of an infective focus was possible with immunoscintigraphy. The sensitivity and specificity of diagnosing infective foci was 72% and 95% respectively (n = 11; colitis = 2, infection of the central permanent catheter tip = 2, middle ear infection = 1, spondylitis/discitis = 3, osteomyelitis = 2, umbilical infection = 1). In vertebral body infections, all lesions were photopenic. In 18 children (60%), no infective focus was found on immunoscintigraphy. In this group of children, the main reason (n = 5) for fever of unknown origin was chronic juvenile rheumatoid arthritis. No uptake was seen in two infants with cardiac failure and suspected endocarditis on SPET. In 3 of the 18 patients (17%), localization of an infective focus was not possible with immunoscintigraphy or on other examinations. In these patients, the fever disappeared spontaneously after a few days of antibiotic therapy. In conclusion, we have shown that 99Tcm-anti-NCA-95 scanning is a safe method with a high sensitivity and specificity for detecting infectious foci in neonates and infants with fever of unknown origin. Furthermore, this method is easy to perform, since no withdrawal of blood is necessary.

Antibodies, Monoclonal↗

[123I-scintigraphy and perchlorate depletion test in the diagnosis of congenital hypothyroidism].

AIM: Thirty eight children suffering from congenital primary permanent hypothyroidism were studied to determine the diagnostic impact of 123I scintigraphy in comparison to laboratory findings and ultrasonography. METHODS: In all patients 123I scintigraphy was performed after intravenous administration of 3.7 MBq 123I. If accumulation of the radiotracer in thyroid tissue occurred a perchlorate discharge test was performed subsequently. RESULTS: Scintigraphy revealed athyrosis in 7 children. In 9 children a lingual thyroid was observed. Deficiency in iodine organification was diagnosed by a significant discharge of 123I in 15 patients. In four of these children the diagnosis of Pendred's syndrome could be established. Ectopic thyroid tissue could be demonstrated only by scintigraphy where clinical examination and sonography failed in the diagnosis in all cases. Hypoplasia of the thyroid gland as it was diagnosed in 2 cases by ultrasonography appeared to be unlikely because a normal 123I uptake was seen in these patients. In 2 patients with scintigraphic proven athyrosis an orthotopic gland had been falsely considered by ultrasound. In 44% of our patients the final diagnosis could only be established if 123I scintigraphy and perchlorate discharge test were performed. CONCLUSION: This findings suggest that scintigraphy is indispensible in the correct diagnostic work up of congenital hypothyroidism.

Child↗

[Microproteinuria and enzymuria in fever and pyelonephritis in childhood. A prospective study of 180 children].

In 180 children (87 children belonging to a control group, 68 with fever of non-renal origin, and 25 with pyelonephritis) albumin and immunoglobulin G (markers for glomerular dysfunction), alpha-1-microglobulin and beta-NAG (markers for proximal tubular dysfunction) and apolipoprotein A1 (marker of "postrenal' dysfunction) were measured in second-voided morning urine. In children with fever of non-renal origin, glomerular dysfunction was encountered in 8.8%, tubular dysfunction in 17.6% and mixed glomerular-tubular dysfunction in 14.7% of cases. Among children with pyelonephritis, 28% revealed glomerular dysfunction and 44% mixed glomerular-tubular dysfunction. No case of solitary proximal tubular dysfunction was observed in children with pyelonephritis. There were highly significant differences in presence and expression of glomerular dysfunction between children with fever of non-renal origin and children with pyelonephritis (P < 0.0001), whereas with regard to proximal tubular dysfunction, the differences were only moderately significant (beta-NAG: P < 0.01) or of low significance (alpha-1-microglobulin: P < 0.05). This may indicate that morphologic changes occur during interstitial pyelonephritis due to inflammation of glomeruli, resulting in glomerular dysfunction, while proximal tubular dysfunction may additionally be due to fever-associated function processes.

Acetylglucosaminidase↗

Diagnostic value of 123iodine scintigraphy and perchlorate discharge test in the diagnosis of congenital hypothyroidism.

AIM: Forty children suffering from congenital primary permanent hypothyroidism were studied to determine the diagnostic impact of 123I scintigraphy in comparison to laboratory findings and ultrasonography. METHODS: In all patients 123I scintigraphy was performed after intravenous administration of 3.7 MBq 123I. If accumulation of the radiotracer in thyroid tissue occurred a perchlorate discharge test was performed subsequently. RESULTS: Scintigraphy revealed athyrosis in 7 children. In 11 children a lingual thyroid was observed. Deficiency in iodine organification was diagnosed by a significant discharge of 123I in 15 patients. In four of these children the diagnosis of Pendred's syndrome could be established. Ectopic thyroid tissue could be demonstrated only by scintigraphy where clinical examination and sonography failed in the diagnosis in all cases. Hypoplasia of the thyroid gland as it was diagnosed in 2 cases by ultrasonography appeared to be unlikely because a normal 123I uptake was seen in these patients. In 2 patients with scintigraphic proven athyrosis an orthotopic gland had been considered by ultrasound. In 50% of our patients the final diagnosis could only be established if 123I scintigraphy and perchlorate discharge test were performed. CONCLUSION: This findings suggest that scintigraphy is indispensible in the correct diagnostic work up of congenital hypothyroidism.

Child↗

Autoimmune thyreoiditis in childhood--epidemiology, clinical and laboratory findings in 61 patients.

Autoimmune thyroiditis (AT) is the most common cause of nonendemic goiter and acquired hypothyroidism in children and adolescents. In 61 patients AT was diagnosed following clinical, ultrasonographic, biochemical and cytological examinations. Girls were more often affected than boys, the female/male ratio in our patients was 9:1. Clinically the vast majority of patients had a goiter. Approximately half of the patients were euthyroid (n = 29). In the other patients hyperthyrotropinaemia (n = 16), hypothyroidism (n = 9) and hyperthyroidism (n = 7) were evident. Thyroid microsomal and peroxidase antibodies were elevated in most of the patients, while thyroglobin antibodies were less frequently elevated. As AT without detectable thyroid antibodies can occur, a fine-needle aspiration cytology of the thyroid is important in nontypical cases. This type of cytologic examination should also be considered in patients with euthyroid nonendemic goiter, thyroid nodules and hyperthyroid goiter.

Adolescent↗

[Imaging and functional parameters in diagnosis of obstructive nephropathy].

Obstruction of the kidney leads to terminal kidney failure within a few years. Therefore, early recognition of such obstruction is of importance. Non-invasive diagnostic ultrasound examination now allows intrauterine visualization of a suspected obstruction. However, the implications of such a dilated ureteral pelvic system are obscure. Whether there is obstruction or dilatation can only be evaluated postnatally by a nuclear technique. The aim of our study was to measure the recovery of kidney function. We investigated 13 kidneys of 9 newborns or small infants (up to 2 years). The follow-up was continuous for up to 29 days. The parameters were: urine output (24-h clearance), glomerular filtration rate, fractional excretion of sodium and potassium, free water clearance, total protein excretion, albumin and alpha 1 microglobulin excretion. The urine output fell from 0.3 to 0.12 ml/min within 14 days after relief of obstruction. The glomerular filtration rate rose from nearly 30 ml/min to about 50 ml/min within a week. The fractional excretion of sodium and potassium indicated recovery of the proximal tubli. The fractional sodium excretion fell below 1% within 4 days. The free water clearance reflects the concentrating ability of the kidney, and in kidneys from newborns it had only positive values, while in kidneys of children older than 6 months there were also negative values. The protein excretion and the albuminuria showed recovery of the glomerular as well as the tubular system.(ABSTRACT TRUNCATED AT 250 WORDS)

Alpha-Globulins↗

[Extracorporeal shockwave lithotripsy in the treatment of pediatric urolithiasis].

The 2nd generation lithotripter LITHOSTAR plus was used for extracorporeal shock wave lithotripsy of 9 kidney stones, 2 ureteral calculi and 2 bladder stones in 10 children 15 months to 17 years old. Ultrasonic stone localization was used in 2 children, x-ray guided stone localization in 8 children. General anesthesia was necessary in 6 children. A stone free rate of 60% was achieved after 1 treatment. Except for one 14-year old boy no adjuvant procedures like percutaneous nephrostomy or ureteral stents were applied. No major complications were encountered.

Adolescent↗

Choledochal cyst type I: successful endoscopic balloon dilatation of the distal common bile duct and sphincter of Oddi: a case report.

A 12-year-old boy with a choledochal cyst type I is reported with clinical features of abdominal pain, postprandial vomiting, weight loss and fatigue since 3 months. Diagnosis was made by ultrasound and confirmed by computer-assisted tomography (CAT) and endoscopic retrograde cholangiography (ERC). Instead of the usual surgical treatment balloon dilatation of the sphincter of Oddi and the distal common bile duct was performed by endoscopic means. The patient was relieved from his complaints, gained weight and felt healthy again. Sonographic and endoscopic reexamination had proven that the diameter of the common bile duct had diminished from 3,0 to 2,6 cm. Endoscopic balloon dilatation of choledochal cysts type I may be a valuable alternative to surgical treatment.

Ampulla of Vater↗

[Chronic neutropenia (author's transl)].

Chronic neutropenia was demonstrated in 6 members of a family including 3 children. Patient suffered from recurrent infections of the throat and upper respiratory tract and from lymphadenitis. There were no life-threatening diseases in any of the patients. In the bone marrow myelopoiesis showed arrested maturation. The previously reported forms of chronic neutropenia with mild clinical course are not differentiated either by haematological or by clinical data. We therefore suggest that they should be considered as one disease entity for the time being. This results in classification of childhood chronic neutropenia according to the clinical picture: a serious form (Kostmann) and cyclic neutropenia must be differentiated from the benign form described here.

Agranulocytosis↗

[Significance of the antibacterial agent assay of urine for bacteriological diagnosis and control of chemotherapy of urinary tract infections (author's transl)].

The disc agar-diffusion-test using Bacillus subtilis ATCC 6051 as test organism is a simple and rapid method for routine testing of antibacterial agents in urine specimens. The test records urine levels which are expected under medium dosage, and in many cases even lower concentrations of renal excreted antibiotics. Out of 5655 analysed urine samples 22% contain antibacterial substances. In urine specimens over which information was volunteered that either no chemotherapy had been administered or that more than a three day's interval free of therapy existed, inhibitory substances are found in 8% and 27% respectively. Urine specimens which are supposedly collected from patients under current chemotherapy do not show therapeutic relevant antibiotic levels in 26%. Between urine specimens with and without antibacterial activity there is no significant difference in the incidence of viable counts of 10-4-10-5/ml and 10-5/ml. From urine samples with antibacterial content increases in the numbers of multiple resistant strains of E. coli, Proteus spp., Pseudom. aerug. and Enterobacter spp. together with high numbers of Candida spp. are observed.

Anti-Bacterial Agents↗

[Ektopic ureter - a not considered cause of persisting enuresis in girls].

BACKGROUND: Ectopic ureter - especially in a duplicated collecting renal system - is a specific cause of incontinence in young girls. Although the symptom of continuous dribbling of urine is characteristic for this malformation, diagnosis is often delayed, as the possibility of ectopic ureters is not considered in treating girls with delayed toilet training. CASE REPORT: We present the case of a young girl with a variety of unnecessary invasive diagnostic and therapeutic procedures due to a misunderstood "enuresis", before incontinence was cured by upper pole heminephrectomy. CONCLUSIONS: Girls, who never have been dry and who loose urine all the time do have an ectopic ureter, unless evidence to the contrary has been put forward. Only considering the possibility of ectopic ureters will avoid unnecessary diagnostic and therapeutic procedures in these girls.

Child↗