Progressive tubulointerstitial nephritis and chronic cholestatic liver disease

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenel involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.     

Incidence of IDDM during 1988–1992 in Zagreb,Croatia

Summary The objective of this study was to determine the incidence of insulin-dependent diabetes mellitus (IDDM) in the population of Zagreb, Croatia, during 1988–1992. A centralized diabetes registry was the primary source of data, while secondary sources were used to assess ascertainment. A total of 282 new cases of IDDM were diagnosed in the study period, the primary and secondary sources identifying annually 93–100% of the cases. The annual incidence rate ranged from 5.6 per 100,000 to 6.6 per 100,000. Early fatality in persons older than 50 years was the major cause of underascertainment. The incidence peaked in the 10–14 years age group (12.4 per 100,000), and remained stable after age 24 years. Males had a significantly higher incidence in the 5–9 and 24–44 years age groups. In the 45–54 years age group, females had a significantly higher incidence. No seasonality was observed. Despite the war conditions in Croatia, the low overall IDDM incidence rates did not change significantly during the study period.Abbreviations IDDM Insulin-dependent diabetes mellitus - DKA diabetic ketoacidosis     

An external approach without tracheotomy for submucosal cordectomy with laterofixation for bilateral abductor paralysis of the vocal folds

There are numerous surgical procedures now available to manage bilateral abductor paralyses of the vocal folds. These procedures have various success rates but mostly do not offer reliable and predictable postoperative results, and usually require ,tracheotomy. The technique described in this report is based on a trial study to obtain a safe airway for an affected patient and avoid tracheotomy, if possible. Submucosal cordectomy was used with lateral fixation of one vocal fold and preservation of the arytenoid. The procedure was performed by using continuous intravenous anesthesia, which does not require intubation of the patient's airway. Seven patients were operated with this technique with excellent postoperative results. The glottal airway was largely improved in all patients, with only minor temporary complications.     

Isolated hydatid cyst of the adrenal gland

Hydatide disease of the adrenal gland is extremely rare, even in disseminated disease. Isolated cyst of the gland is even more rare. Only 9 of such cases seem to have been described so far. We present a 49 year old woman in whom a hydatide cyst of the right gland was found during the investigation for the dull pain in the area. She was successfully operated. Hydatide etiology was confirmed at operation and by histology. She had an uneventful recovery. The preoperative pain disappeared postoperatively.     

Diverticulum of the stomach

Gastric diverticula are very rare and usually asymptomatic diseases. Clinical symptoms may include haemorrhage, mild discomfort and pain in upper abdomen. They are most frequently diagnosed incidentally during X-Ray examinations. In symptomatic diverticulum of gastric fundus was diagnosed roentgenographically and endoscopically and was successfully cured surgically.     

Vaginoplasty in male to female sex change operations

Vaginoplasty presents the main procedure in one-stage male to female sex reassignment surgery. Our new vagina consist of two parts: a long vascularized urethral flap and a pedicled island tube skin flap serrated from the penile skin. The urethral segment of the vagina plays a major role, giving the vagina depth, width and providing moisture. In the period from January 1989 to August 1995, 89 patients aged 18 to 56 years underwent surgery. The follow-up ranged from 3 months to 6 years (mean 4.1 years). Satisfactory cosmetic and functional results were obtained in 86% of cases.     

Surgical treatment of toxic adenoma

In the period ranging from 1985. to 1994. a total number of 86 patients have been treated for toxic adenoma (TA). Hyperthyroidism was more often present in males (70.83% versus 51.61%) and in enlarged adenomas (more then 2 cm in diameter). Pathohistologic type of adenoma influenced degenerative changes. Three carcinoma have been observed, two papillary and one follicular. Surgical treatment is the method of choice. Complications are rare (3.49%) and mild.