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Inherited susceptibility to autoimmune Addison's disease is linked to human leukocyte antigens-DR3 and/or DR4, except when associated with type I autoimmune polyglandular syndrome.

The inherited susceptibility to autoimmune Addison's disease was found to be strongly associated with human leukocyte antigens (HLA)-DR3 and DR4 alleles. In a study of 45 white patients from the United States with the disease, the relative risks (the number of times that an individual is at risk for Addison's disease if they had a marker, compared to those without such marker) were found to be 6.0, 4.6, and 26.5 for the DR3 allele, the DR4 allele, and for DR3/DR4 heterozygotes, respectively. Frequencies of DR2, DR5, and DR7 in the patients with Addison's disease were significantly decreased in comparison to 265 individuals in the control population. These HLA-DR frequencies in patients with Addison's disease were similar to those for 723 patients with insulin-dependent diabetes (IDD). However, the above HLA-DR associations persisted even when only data from the 37 patients with Addison's disease who did not have IDD were considered. Adrenocortical autoantibodies in 23 patients with IDD who did not have Addison's disease were equally frequent among those with DR4 and DR3 alleles. In contrast, HLA-DR frequencies in 17 patients with type I autoimmune polyglandular syndrome (chronic mucocutaneous moniliasis, hypoparathyroidism, Addison's disease, etc.) were not different from control. We conclude that genetic susceptibility to autoimmune Addison's disease may involve the same HLA-associated genetic determinants as IDD, except when Addison's disease occurs as part of type I autoimmune polyglandular syndrome.

Addison Disease↗

[Familial idiopathic hypoparathyroidism associated with progressive sensorineural deafness(1)].

A 29-year-old man came to our hospital with tetany and sensorineural deafness. Routine laboratory testing revealed a serum calcium of 5.5 mg/dl and a serum phosphate of 6.1 mg/dl. The level of circulating immunoreactive parathyroid hormone was undetectable, and he showed hyperresponsiveness to the exogenous parathyroid hormone (synthetic hPTH (1-34), 100U). Thus, he was diagnosed as having idiopathic hypoparathyroidism. The other endocrine functions were normal. The parathyroid antibody was negative and no moniliasis was found. On the examination of his family, it was found that his male sibling and one of his female siblings also had hypocalcemia (6.4 mg/dl and 6.0 mg/dl) and undetectable levels of circulating immunoreactive parathyroid hormone, and all his siblings, his father and one of his nieces had sensorineural deafness, so he was diagnosed as as having familial idiopathic hypoparathyroidism and familial progressive sensorineural deafness. In addition to this case, we have experienced two siblings of another family having the same combination of these disorders (unpublished observation). Since familial idiopathic hypoparathyroidism is not common, it seems that the present combination of these disorders is not a chance association.

Adult↗

Diffuse finely nodular lesions of the esophagus.

Double contrast study of the esophagus revealed a variety of diffuse finely nodular lesions, some of which have not been adequately reported. Acanthosis nigricans, superficial spreading esophageal cancer, leukoplakia, moniliasis, and esophagitis were found to produce this appearance. Typical cases of the first three diseases are demonstrated and the differential diagnosis discussed.

Acanthosis Nigricans↗

Herpes esophagitis.

Herpes simplex virus is a well recognized cause of opportunistic esophageal infection, yet there have been few descriptions of the radiographic features of this entity. This report describes the radiographic and clinical findings in six patients with herpes esophagitis. Each patient had clinical evidence of immunosuppression. Acute odynophagia was the most common presenting complaint. Double-contrast esophagrams revealed discrete, widely separated ulcers, plaquelike defects without ulceration, or a combination of ulcers and plaques. Plaquelike lesions in the esophagus can be associated with herpes or moniliasis. However, discrete ulcers on an otherwise normal background mucosa are strongly suggestive of herpes esophagitis. Increased awareness of the radiographic findings should lead to better diagnosis of this entity, so that potentially toxic antifungal drugs can be avoided in patients with herpes esophagitis.

Adult↗

Glycogenic acanthosis of the esophagus.

A nodular appearance of the esophageal mucosa was observed in 28.3% of 300 consecutive double-contrast esophagrams. This most commonly appeared as numerous uniformly sized, usually less than 3 mm, subtle, round elevations involving the entire esophageal surface. When carefully performed, endoscopy will almost always confirm these findings. Endoscopic biopsies performed in 10 patients demonstrated the nodules to represent glycogenic acanthosis--a combination of cellular hyperplasia and increased cellular glycogen. The radiographic appearance of these nodules, while usually characteristic, may vary and they may simulate pathologic processes, particularly moniliasis. Distinction can usually be made by clinical and radiologic criteria. Although the etiology is unknown, this seems to be of no clinical significance.

Adult↗

Histoplasmosis of the colon in patients with AIDS: imaging findings in four cases.

OBJECTIVE: The purpose of this study was to describe and illustrate the radiographic findings of colonic histoplasmosis in patients with AIDS. SUBJECTS AND METHODS: Four HIV-positive patients were admitted because of fever, abdominal pain, tenderness, weight loss, and diarrhea. One patient had known disseminated histoplasmosis, one had esophageal moniliasis, and two had no other known opportunistic infections or tumors. All four patients had barium enemas, and two had abdominal CT examinations. The diagnosis was established via colonoscopic biopsy in three patients and with surgery in one patient. RESULTS: Barium enema in two patients showed separate, short, apple-core lesions in the transverse and descending colon. In one patient, a single lesion in the ascending colon mimicked colonic carcinoma. One patient had segmental involvement of the ascending colon, compatible with inflammatory bowel disease. CT examination revealed circumferential thickening of the wall of the colon with adjacent lymphadenopathy of mixed attenuation. Three patients responded to medical therapy, and one patient remained symptomatic and was found on a follow-up CT examination to have significant recurrence of retroperitoneal lymphadenopathy. CONCLUSION: Colonic histoplasmosis in patients with AIDS has protean radiographic features and can mimic colonic carcinoma. In these patients, histoplasmosis should be part of the differential diagnosis, particularly when several colonic lesions are detected, associated regional and retroperitoneal lymphadenopathy is present, and the patient is living or has lived in an endemic area.

AIDS-Related Opportunistic Infections↗

The causal agents of witches' broom and frosty pod rot of cacao (chocolate, Theobroma cacao) form a new lineage of Marasmiaceae.

The two most devastating diseases of cacao (Theobroma cacao)--the source of chocolate--in tropical America are caused by the fungi Crinipellis perniciosa (witches' broom disease) and Moniliophthora roreri (frosty pod rot or moniliasis disease). Despite the agricultural, socio-economic and environmental impact of these fungi, most aspects of their life cycles are unknown, and the phylogenetic relationships of M. roreri have yet to be conclusively established. In this paper, extensive phylogenetic analyses of five nuclear gene regions (28S rDNA, 18S rDNA, ITS, RPB1, and EF1-alpha) confirm that C. perniciosa and M. roreri are sister taxa that belong in the Marasmiaceae (euagarics). Furthermore, these taxa form part of a separate and distinct lineage within the family. This lineage includes the biotrophic fungi Moniliophthora perniciosa comb. nov. and M. roreri, as well as one undescribed endophytic species. The sister genera to Moniliophthora are Marasmius, Crinipellis and Chaetocalathus, which consist mainly of saprotrophic litter fungi.

Agaricales↗

[Diffuse plane xanthoma with arthritis, serositis, erythema nodosum, vasculitis and myelomonocytic leukemia. Description of a case with autopsy].

A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with Ménière-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.

Arthritis↗

Immunologic reconstitution in the DiGeorge syndrome by fetal thymic transplant.

An infant with idiopathic hypoparathyroidism was found to have no evidence of thymus by radiologic examinations, including pneumomediastinum. Immunoglobulin concentrations were normal but there was clear evidence of inadequate cellular immunity including gradually developing lymphopenia. The infant suffered recurrent diarrhea and pneumonia, chronic rhinorrhea and moniliasis. At age 7 months fetal thymic tissue was implanted in the rectus abdominis muscle. There was immediate increase in lymphocyte counts which have remained normal on most determinations. Other aspects of immunologic function were restored and remain intact. The child, now 6 years of age, although mentally retarded, has no unusual infections. Immunologic reconstitution was thus achieved by transplant of fetal thymus.

Antibody Formation↗

[Side-effects of ovulation inhibitors].

The discussion about the side-effects of oral contraceptives received new aspects through the publication of the prospective British Study "Oral Contraceptives and Health" (by the Royal College of General Practitioners) in 1974. This article is divided into the following sections: Beneficial side-effects (elimination of symptoms in dysmenorrhea, endometriosis, acne vulgaris, menorrhagia and anemia). Harmless side-effects (harmless symptoms not implying illness). Clinically important side-effects (menstrual disorders, facilitation of moniliasis and trichomoniasis, diseases of the circulatory system, thrombo-embolism). Oral contraceptives have no adverse effects on the fertility of women. Significant is the conclusion that pregnancy and children are normal after the use of oral contraceptives. There are no indications of an increased risk of cancer. The results of the British study confirm a reduced incidence of benign breast tumors in women using oral contraceptives. The suggestion to select a pill-type considering the hormonal constitution of a woman did not prove to be of help. The original proposal of changing the pill-type according to the observed side-effects therefore remains valid. A classification of the used oral contraceptives into the groups "with gestagen accentuation", "hormonally balanced" and "with estrogen accentuation" will help the physician to prescribe the most useful type. An extensive table summarizes the most frequent side-effects, explains their causes and recommends ways of eliminating the symptoms by changing the pill-type.

Breast↗

[Clotrimazol in the treatment of candida infections in premature and newborn children (author's transl)].

The early infancy is especially affected by moniliasis in pediatrics. The causes are: broad spread of Candida albicans in the environment of these children, a growing immunity, the immaturity of the skin and an insufficient candida-static activity of the serum. The prematures and the newborns with an irregular birth are especially endangered. This danger is increased by simultaneous antibiotic therapy. The arising mycosis can reach from the relative harmless mouth- and diaper-thrush to serious septic diseases. An effective therapy therefore is very much desired. As new efficacious medicaments the derivates of imidazol, Clotrimazol, have proved satisfactory for the treatment of mycoses. It is as Canesten marketed. The drug possesses a strong activity against numerous fungi, some protozoa, and bacteria. In this paper it is reported on own pharmacokinetic and therapeutic investigations with Clotrimazol. The substance has been very useful for the treatment of mouth- and diaper-thrush. Clotrimazol was administered as BAYb 5097 peroral and local as Canesten -solution 1% and -cream 1%. There were 171 treatments carried out. Essential side-effects were not observed.

Administration, Oral↗

Comparative trials of cefaclor AF in uncomplicated cystitis and asymptomatic bacteriuria.

Two different doses of cefaclor advanced formulation (AF), a new sustained-release formulation of cefaclor, were compared with the regular formulation of cefaclor for efficacy and safety in the treatment of uncomplicated cystitis and asymptomatic bacteriuria. A 7-day course of treatment was used, and the trials were double-dummy and double-blind. In one trial, cefaclor AF 500 mg once daily (at night) was compared with cefaclor 250 mg three times a day. Satisfactory clinical and bacteriological responses were found in 179/189 (94.7%) and 160/191 (83.8%) patients, respectively, given cefaclor AF and in 82/87 (94.3%) and 74/90 (82.2%) patients given cefaclor, 5-9 days after the end of treatment. In the other trial, cefaclor AF 375 mg twice daily was compared with cefaclor 250 mg three times a day. Satisfactory clinical and bacteriological responses were obtained in 164/180 (91.1%) and 156/184 (84.8%) patients, respectively, given cefaclor AF, and in 86/92 (93.5%) and 81/93 (87.1%) patients taking cefaclor, 5-9 days after the end of treatment. Very similar results were found in both studies in those patients who were assessable 3-5 weeks later. Only 4.3% and 2.4% of patients treated with cefaclor AF (375 mg and 500 mg, respectively) and 2.2% of cefaclor patients discontinued therapy due to adverse events. The three most commonly reported events were vaginal moniliasis or vaginitis (8.6%), headache (5.0%) and nausea (4.8%). No significant differences were found between clinical efficacy and safety parameters in the different study groups, and it was concluded that cefaclor AF in a twice-daily or once-daily dosage is as effective and as safe as the currently recommened three-times-a-day dosage of cefaclor.

Adolescent↗

Comprehensive safety profile of cefaclor AF in respiratory, urinary tract and skin infections.

The safety of cefaclor advanced formulation (cefaclor AF) was evaluated in 3,272 patients participating in 11 controlled clinical trials in comparison with cefaclor (2,210 patients) for a variety of infectious illnesses. Daily doses of cefaclor AF ranged from 500 to 1500 mg, with a mean duration of treatment of 8.1 days (range 1-18 days). There were no significant differences between the cefaclor AF- and cefaclor-treated groups in the frequency of adverse events by body system for all events reported. The majority of adverse events related to therapy were mild and transient. Severe adverse events occurred in 2.1% of the cefaclor AF group and 2.7% of the cefaclor group. The most frequently reported adverse events for cefaclor AF were diarrhoea (3.4%), headache (3.2%), nausea (2.5%) and vaginal moniliasis (2.5% of females). Drug-related adverse events led to early discontinuations in 1.7% of cefaclor AF-treated patients and 1.6% of cefaclor-treated patients. Overall, there were few significant differences in the frequency of adverse reactions between older and younger patients. Notably, elderly patients reported significantly less diarrhoea and fewer hypersensitivity-type reactions. There were, however, more therapy discontinuations due to adverse effects in patients aged 65 years or older than in those less than 65 years of age. Many of the discontinuations were thought to be unrelated to therapy. Alterations in laboratory values in patients treated with cefaclor AF were similar to those seen with other beta-lactam antibiotics. The comprehensive data indicate that cefaclor AF is a safe therapeutic option for a variety of common bacterial infections.

Adolescent↗

Treatment of superficial fungous infections. Value and limitations of systemic administration of griseofulvin.

Comprehensive studies and numerous clinical reports have shown that griseofulvin orally in a dose of 1 gm. daily is an effective treatment for superficial fungous infections of the skin, hair and nails. The drug is not effective against yeast infections (moniliasis), bacterial infections or most of the deep fungous infections. Duration of treatment varies with the site of infection, glabrous skin, crotch and scalp responding within four to five weeks. Infections of palms, soles and nails require a considerably longer time, palms healing more quickly than soles and fingernails more quickly than toenails, which may require up to a year of continuous treatment. Auxiliary measures such as clipping hair, removing infected nail tissue and topical fungicides shorten the duration of treatment. No serious side effects have been reported. Minor discomforts such as headaches and mild rashes occur in some cases. Observations of a series of 49 patients with superficial fungous infections, especially hand, foot and nail infections due to Trichophyton rubrum, confirmed these reports taken from the literature. Attempts to use a reduced dosage schedule did not prove satisfactory.

Arecaceae↗

The role of microorganisms in diaper dermatitis.

A quantitative microbiological survey of multiple sites in the diaper area in 40 normal infants and 100 infants with various forms of diaper dermatitis showed (1) no significant differences between normal infants and those with common chafing form of diaper dermatitis except for a low number of Staphylococcus aureus organisms in 50% of those with chafing dermatitis compared to no S aureus in normals; (2) S aureus occurred in high numbers in every case of atopic dermatitis and was frequently found at much lower levels in psoriasis, seborrheic dermatitis, and chafing dermatitis; (3) Candida albicans occurred in 80% of cases clinically diagnosed as moniliasis localized to the diaper area and in 33% of those suspected of Candida infection with dissemination beyond the diaper area, while C albicans was found in only five of 145 culture sites in 40 normal infants.

Candida albicans↗

Vaginal discharge: perceptions and health seeking behavior among Nepalese women.

OBJECTIVES: To understand women's perceptions and health seeking behavior and the association between vaginal discharge, clinical signs and laboratory findings as a presentation of sexually transmitted diseases (STD). METHODS: We conducted five focus group discussions with women attending the outpatient department in a large public hospital in Katmandu, Nepal, during May-June 1997. We also interviewed seventy women presenting with vaginal discharge to the same hospital, through structured questionnaire. Women presenting with discharge were also examined and investigated for six common sexually transmitted diseases and reproductive tract infections. RESULTS: In the focus groups vaginal discharge was identified as a common disease distinct from STDs, for which women can seek treatment. STDs were considered as social diseases transmitted to women through multiple sexual partners and not from husband. Patients with vaginal discharge preferred traditional healers and pharmacist. Clinical signs were inconclusive for type of infection. Simple laboratory tests identified etiologic agent in 64 (91%) patients and the three commonest infections were Moniliasis (78%), Bacterial Vaginosis (25%) and Trichomoniasis (17%). CONCLUSION: Vaginal discharge may be used as a risk marker for identification of STDs by Primary Health Workers. Low cost investigations should be made available at the secondary care level for identification of most common Reproductive Tract Infections. Communication campaigns should target the misconceptions that exist in the communities local context related to the prevention, treatment and control of vaginal discharge and STDs.

Adult↗

Surgical treatment of anterolateral tongue carcinoma.

AIM: The aim of this study was to present the results of the surgical treatment of patients over and under 40, diagnosed with anterolateral carcinoma of the tongue. The tongue is the most frequent site of malignancies, squamous cell carcinoma in particular, of the oral cavity. Recently, cases with such localization have become more frequent in Bulgaria due probably to the increased consumption of low-quality alcoholic beverages, smoking or other risk factors. METHODS: The study included patients with diagnosed squamous-cell carcinoma in the tongue anterolateral region. In all patients with anterolateral tongue carcinoma the treatment included mandibulotomy with or without partial resection of the lower jaw, total or two-third glossectomy with partial resection of the oral cavity floor and cervical dissections. A total of twelve patients (three younger than 40) with anterolateral carcinoma of the tongue were treated between 2002 and 2004. RESULTS: There was one lethal outcome (8.3%) for the period observed. Squamous-cell carcinoma was verified in 11 (91.7%) and rhabdomyosarcoma in 1 (8.3%) of the operated patients. One patient (8.3%) complained of persistent dysphagia for 7 months. In the patient with rhabdomyosacroma total laryngectomy was required 6 months after the operation because of secondary involvement of the larynx. In three patients (25%) moniliasis occurred after the course of radiotherapy, which necessitated antimycotic therapy. In one patient (8.3%) the cancer recurred and in another one (8.3%) metastasis developed. CONCLUSIONS: The results of the treatment, including surgery and postoperative radiotherapy, depend on the stage of the disease and extent of surgery. There is no evidence of poorer outcome of the disease in younger patients.

Adult↗

[Severe mixed immunodeficiency. Report of a case].

The case of a 3 month old child with severe combined sex linked immunodeficiency is presented. The diagnosis was well doccumented, during his life. The child presented as a case of mucocutaneous moniliasis resistant to treatment. There was a history of similar cases in the family; diagnosis was made at post-mortem in one cousin and death occurred at early age in all kins so affected. Blood marrow transplant was not feasible in our case because histocompatibility was lacking in the kins studied. Three units of transfer factor were given as well as hyperimmune plasma but the child died in respiratory failure. Autopsy demonstrated pulmonary infection by Pneumocystic carinii and generalized citomegalic inclussion virus infection; almost complete absence of immune tissue was also demonstrated.

Autopsy↗