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Background: Patients with complex congenital heart disease frequently undergo a life-long ambulatory therapy with the need for repeated hospital interventions. To optimize this manifold interplay, we designed and implemented a tele-medical service, the Congenital Cardiology Cloud (CCC). This study aims to analyse the requirements for its implementation through the comprehensive assessment of design, installation and impact on patient´s care., Methods: CCC's development comprised the analysis of historically raised admission and discharge management and the definition of technical and organizational requirements. Elaboration of procedural flow charts, description of data formats and technical processes as well as distribution of patient structure formed part of this process., Results: Analysis of existing workflows uncovered a need for the rebuilding of admission and discharge process and decision making for further treatment. The CCC reduces conference-meetings in general and repetitive meetings up to less than a third. Real-time dispatch of discharge documents guarantees an instantaneous access to patient-related data. Comparative analyses show a more complex patient group to be involved in tele-medical services., Conclusions: The CCC enables the sharing of complex clinical information by overcoming sectoral barriers and improves mutual patient advice. Implementation of a tele-medical network requires willingness, perseverance and professional engagement. Future application analysis and possible introduction of refinancing concepts will show its long-term feasibility., Competing Interests: The authors declare that they have no conflict of interest., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Background: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has improved survival in high-risk childhood leukemia but is associated with long-term sequelae such as impaired pulmonary function and reduced exercise capacity impacting quality of life., Methods: A convenience sample of 17 patients after allo-HSCT (HSCT-12 male, age 15.7±6.7 years, time after HSCT 5.3±2.8 years) underwent pulmonary function testing, echocardiography, and an incremental exercise test on a bike. Physical activity and health-related quality of life were assessed by questionnaires (7-day physical activity recall, PEDS-QL). Seventeen healthy age- and gender-matched controls served as control group (CG) for results of pulmonary function and exercise testing., Results: HSCT showed reduced pulmonary function (HSCT vs. CG: FEV1 90.5±14.0 vs. 108.0±8.7%pred; FVC 88.4±19.3 vs. 107.6±6.9%pred, DLCO 75.3±23.6 vs. 104.9±12.8%pred) and exercise capacity (VO 2 peak 89±30.8%pred, CG 98±17.5%pred; Wmax 84±21.7%pred, CG 115±22.8%pred), but no relevant cardiac dysfunction and a good quality of life (PEDS-QL mean overall score 83.3±10.7). Differences in peak oxygen uptake between groups were mostly explained by 5 adolescent patients who underwent total body irradiation for conditioning. They showed significantly reduced diffusion capacity and reduced peak oxygen uptake. Patients reported a mean time of inactivity of 777±159min/day, moderate activity of 110±107 min/day, hard activity of 35±36 min/day, and very hard activity of 23±22 min/day. A higher amount of inactivity was associated with a lower peak oxygen uptake (correlation coefficient tau -0.48, p=0.023)., Conclusions: This pilot study shows that although patients after allo-HSCT reported a good quality of life, regular physical activity and exercise capacity are reduced in survivors of stem cell transplantation, especially in adolescents who are treated with total body irradiation for conditioning. Factors hindering regular physical activity need to be identified and exercise counseling should be part of follow-up visits in these patients., (© 2021. The Author(s).)

The neonatal bacterial infection is a potentially life-threatening condition that justifies intravenous antibiotic therapy. However, clinical symptoms are often unspecific. Particularly in the absence of a response to antibiotic therapy, various differential diagnoses can be considered. We report the clinical presentation, the diagnostic steps, the therapy as well as the long-term progression of a preterm infant who acquired a perinatal enterovirus infection including viral myocarditis. The case underlines the clinical relevance of enterovirus infections in newborns. The current literature, however, describes only single aspects and lacks, in particular, accurate data on both epidemiology and morbidity., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

We report a male newborn who became symptomatic with supraventricular tachycardia on the first day of life. Neither adenosine nor electric cardioversion could terminate the tachycardia, therefore intravenous esmolol (β-receptor blocker) was initiated. Inspite of subsequent administration of various antiarrhythmic medications in increasingly higher doses, repeated supraventricular tachycardic episodes occurred. The electrocardiogram showed typical findings of a multifocal atrial tachycardia as the underlying cause. When tachycardic episodes occurred, they also presented as atrial flutter at 460 bpm and a 2:1 block. Finally, high dosage of amiodarone (10 mg/kgbw/d) led to continuous control of the heart rate without tachycardic episodes. To date our patient is mostly in sinus rhythm but without tachycardic episodes and doing well., (© Georg Thieme Verlag KG Stuttgart · New York.)

Despite antibiotic therapy, infections with Neisseria meningitidis still demonstrate a high rate of morbidity and mortality even in developed countries. The fulminant septicaemic course, named Waterhouse-Friderichsen syndrome, with massive haemorrhage into the adrenal glands and widespread petechial bleeding suggest pathophysiological inhibition of platelet function. Our data show that N. meningitidis produces the important physiological platelet inhibitor and cardiovascular signalling molecule nitric oxide (NO), also known as endothelium-derived relaxing factor (EDRF). N. meningitidis -derived NO inhibited ADP-induced platelet aggregation through the activation of soluble guanylyl cyclase (sGC) followed by an increase in platelet cyclic nucleotide levels and subsequent activation of platelet cGMP- and cAMP- dependent protein kinases (PKG and PKA). Furthermore, direct measurement of horseradish peroxidase (HRP) passage through a vascular endothelial cell monolayer revealed that N. meningitidis significantly increased endothelial monolayer permeability. Immunfluorescence analysis demonstrated NO dependent disturbances in the structure of endothelial adherens junctions after co-incubation with N. meningitidis . In contrast to platelet inhibition, the NO effects on HBMEC were not mediated by cyclic nucleotides. Our study provides evidence that NO plays an essential role in the pathophysiology of septicaemic meningococcal infection.

Accidental ingestion of foreign bodies is a common problem in infants and childhood, but ingestion of magnetic construction toys is very rare. In the case of ingestion of multiple parts of these magnetic construction toys, they may attract each other through the intestinal walls, causing pressure necrosis, perforation, fistula formation or intestinal obstruction. A 20-month-old boy presented with a three-day history of abdominal pain and bilious vomiting. Physical examination revealed a slighted distended abdomen. The -white blood cell count was increased, but the C-reactive protein was normal. Ultrasound and X-ray of the abdomen showed a distended bowel loop in the right upper quadrant, a moderate amount of free intraperitoneal liquid and 4 foreign bodies. Emergency laparotomy was performed and 2 perforations in the ileum were detected. The perforations were caused by a magnetic construction toy and 2 iron globes. The fourth foreign body was a glass marble. The foreign bodies were removed, both perforations were primarily sutured. The child was discharged on postoperative day 10 after an uneventful recovery. Parents should be warned against the potential dangers of children's constructions toys that contain these kinds of magnets., (Georg Thieme Verlag Stuttgart, New York.)

Extremely immature preterm infants rarely present with a leukocytosis exceeding 30,000/microL. The pathogenetic sequence leading to leukemoid reactions in non-malignant diseases remains to be elucidated. Potential triggers for leukemoid reactions in premature infants include prenatal corticosteroids, chorioamnionitis and funisitis or systemic infection. In the two-year period from 2006 to 2007 all infants with a gestational age of less than 26 weeks were screened for leukocytosis. Among our cases, one preterm infant presented with a leukocyte count of 229,300/microL at the age of 48 hours, lasting throughout the first three weeks of life. Impairment of microcirculation and resulting organ dysfunction were not observed. Thus, invasive therapeutic procedures, which are routinely initiated in hyperleukocytosis in accompanying malignant diseases, may not have the same significance in extremely immature preterm infants and should be executed in these patients on an individual basis and with extreme caution.

Infection with group B streptococcus (GBS) is the most common cause of early onset neonatal sepsis in many countries, leading to neonatal morbidity and mortality. There is much evidence for a direct involvement of platelets in the pathogenesis of inflammation and sepsis. Several bacteria are known to directly interact with platelets leading to activation and aggregation, a phenomenon also observed with GBS. Here, we demonstrate that GBS rapidly bound to platelets; however, only strains isolated from septic patients bound fibrinogen on their surface and induced platelet thromboxane synthesis, platelet aggregation, and P-selectin (CD62P) expression. In contrast, GBS strains isolated from healthy newborns or healthy pregnant women induced only shape change, but not platelet thromboxane synthesis, platelet aggregation, or CD62P expression. All GBS strains investigated were able to activate FcgammaRIIA receptor signaling pathways including phospholipase C gamma2 (PLCgamma2), as well as calcium/calmodulin-dependent myosin kinase II (CaMKII) and phosphorylation of myosin light chain (MLC). In contrast, protein kinase C (PKC) was exclusively activated by GBS strains isolated from septic patients, and p38 mitogen activated protein kinase (p38 MAP kinase) was preferentially activated by septic GBS strains. Furthermore, stress signaling kinase SEK1/MKK4 and focal adhesion kinase (FAK) were activated by all tested GBS strains in a FcgammaRIIA-independent way. This study demonstrates that septic, but not colonizing, GBS strains bind fibrinogen on their surface, and that septic GBS strains influence platelet function not only via the FcgammaRIIA receptor, but also via pathways distinct from IgG-mediated signalling. These mechanisms lead to platelet aggregation and secretion, thereby possibly modulating the pathophysiologic course of GBS infections.

Endoscopic polypectomy of a large polyp can be difficult due to inability to snare the polyp. The difficulty may increase when the polyp is located at turning corner of the bowel. We presented a case of a 3 cm-sized large pedunculated polyp located at the superior duodenal angle that was not amenable to conventional snare polypectomy, but was instead successfully resected by hemoclip-assisted and needle knife method. Such experience has not been reported in the English literature.

Background: Endoscopic hemostasis of gastrointestinal (GI) bleeding is a widely accepted modality of treatment, and endoscopic hemoclipping has been reported to cause fewer complications., Methods: Forty patients with gastrointestinal bleeding (active bleeding or non-bleeding visible vessel), 30 men and 10 women with a mean age of 59.1 +/- 14.4 (28-86) years were treated with endoscopic hemoclipping. After panendoscopy or colonoscopy, a local epinephrine injection was routinely given in the initial 20 cases, followed by hemoclipping. For the latter 20 cases, local epinephrine was given only to those with active bleeding. If there was adherent blood clot, irrigation with 3% H2O2, and removal of the blood clot with forceps or basket were done. Six cases with bleeding at technically difficult locations were managed with a new method; a transparent cap (Olympus EMRC) fitted with a 2-channel endoscope for hemoclipping., Results: There were 35 patients with peptic ulcer, 2 with post endoscopic papillotomy bleeding, 1 with duodenal Dieulafoy's lesion, 1 with Mallory Weiss syndrome, and 1 with rectal ulcer. The types of bleeding were spurting in 7, oozing in 12, and non-bleeding visible vessel in 21 cases. The average number of clips used was 3.1 +/- 1.7 (1-9) and the average clip loss was 0.6 +/- 0.9 (0-4) per patient. The success rate for hemostasis using the transparent cap-fitted endoscope was 100% and the overall success rate was 85% with no complications related to the procedure., Conclusion: Endoscopic hemoclip treatment for GI bleeding is safe and effective. The transparent cap-fitted endoscope is a new method for hemoclipping in technically difficult lesions.

Objective: Endoscopic retrograde cholangiopancreatography (ERCP) in post-Billroth II (BII) gastrectomy is more difficult due to anatomical changes. The difficulties include entrance to the afferent loop and selective cannulation. Our aim here is to report the success rate and special manipulations and techniques of this procedure., Methods: A retrospective review of 56 ERCP procedures in post-BII gastrectomy patients was performed. There were 43 male and 13 female patients with a mean age of 63 yr (range, 32-78 yr). All cases were tried with forward-viewing endoscope first. Of the failed cases, 10 were retried by side-view duodenoscope. The entrance to the afferent loop was attempted by starting from the upper opening at the anastomosis site and, if this failed, then using the lower opening; presence of bile; and air-contrasted afferent loop under fluoroscopy. If failure of afferent loop entrance resulted, hand compression over the mid-abdomen, or polypectomy snare in the working channel of the endoscope, was tried. For failure of common bile duct cannulation with straight catheters, techniques of pushing the catheter against the duodenal wall and bending the tip of the endoscope or guidewire were used., Results: The success rate of afferent loop entrance was 76.7% (43 of 56 cases). The afferent loop was identified in the upper orifice of the anastomosis in 93% (40 of 43) of the cases. Eight cases of afferent loop entrance could be facilitated by hand compression, and three by polypectomy snare in the working channel of the endoscope. The success rate of ERCP cannulation in those successful afferent loop intubation cases was 81.3% (35/43 cases). Most of the selective common bile duct (CBD) cannulation was achieved by straight (new) catheter and an additional six cases were successful using the techniques mentioned. No serious complications were encountered, except three cases of submucosal hemorrhage., Conclusion: The overall success rate of BII ERCP was 62.5% (35 of 56 cases). The special manipulations mentioned in BII ERCP can be helpful in certain cases.

In Taiwan, numbers of patients with the acquired immunodeficiency syndrome (AIDS) have been increasing in recent years. We present esophageal disease of different causes in 5(16%) heterosexual men among 31 AIDS patients over a 5-year period. Major symptoms included mild dysphagia in 4 (80%) patients and odynophagia in 3 (60%) patients. The duration of symptoms varied from 3 days to 6 months. The symptoms occurred before the diagnosis of AIDS in 3 patients. At esophagogastroduodenoscopy (endoscopy), all 5 patients had esophagitis and/or esophageal ulcers proved by histopathologic evaluation. Four had Candida esophagitis, 3 had cytomegalovirus esophagitis/ulcers and 2 had idiopathic esophageal ulcerations (IEU). Three patients had different esophagitis/ulcers at the same time or during follow-up. The median CD4 lymphocyte count at the time of diagnosis of esophageal disease was 12.2 cells/mm3 (range, 3 to 35 cells/mm3). The endoscopic pictures of the different causes of esophagitis/ ulcers lack uniformity in number, size and appearance. These observations make a conclusion that all AIDS patients with an esophageal disease should undergo endoscopy with biopsy to obtain a definitive diagnosis.

The diagnosis of hypoaldosteronism usually depends upon a combination of abnormal clinical and laboratory findings. The most common abnormality in hypoaldosteronism is hyperkalemia, which may be combined with sodium depletion. In the present study, 5 of 16 patients diagnosed with isolated hypoaldosteronism (IHA) had sodium depletion due to renal salt wasting, and four patients had normokalemia. Of these 16 IHA patients, 70% had subnormal baseline and stimulated plasma renin activity (PRA). Six patients diagnosed with type I pseudohypoaldosteronism (PHA) had normal or high PRA and plasma aldosterone concentrations (PAC). In 11 control subjects, supine PAC correlated positively with serum potassium (SK), and PAC stimulated by furosemide and ambulation correlated with the 24-hour urinary potassium excretion (UK). However, these correlations were not found in IHA and PHA patients. The ratio of UK/UNa+K and UNa/UK correlated with the stimulated PAC when the IHA and control subjects were taken as a whole. However, these electrolyte excretion parameters bore no relationship to the supine PAC. The stimulated PAC/SK ratio was used to discriminate the three groups; all IHA patients had a ratio below 3. The results indicate that stimulated PAC reflects the bioactivity of aldosterone on the collecting tubule, and the stimulated PAC/SK ratio is useful for the diagnosis of hypoaldosteronism and pseudohypoaldosteronism.

The clinical and pathological features of 14 cases of acute drug-induced liver disease (DILD) were analyzed using the French group method for drug reaction assessment. Among them, 8 were of the cytotoxic type, 4 were of the cholestatic type and 2 were of the mixed type. Serum alkaline phosphatase (ALP) levels of the cytotoxic type DILD were all < 1.8 times the normal value, while those of the cholestatic type DILD > 1.8 times (P < 0.05). The alanine aminotransaminase and aspartate aminotransaminase (ALT and AST) levels of the cholestatic type were all < 13.1 times the normal value, while those of the cytotoxic type varied from 2.2 to 118 times the normal value. We found that steatosis was the major feature in the cytotoxic type with ALT and AST < 2.5 times the normal value. Piecemeal necrosis was noted only in all the cases with ALT and AST > 20 times the normal value. In the cholestatic type, the pathological features of the oral contraceptive-related DILD showed mainly cholestasis, whereas chlorpromazine-related DILD revealed additional portal inflammation. Meticulous taking of patient history and clinical assessment are mandatory for the diagnosis of DILD. The ALP levels were helpful in distinguishing different types of DILD. There are some correlations between biochemical changes and pathological features, and both are helpful in distinguishing different etiologies of DILD when the inciting drug is in doubt.

Congestive gastropathy is a common cause of gastrointestinal bleeding in cirrhotic patients. Forty-six patients with cirrhosis of the liver and 225 control subjects matched in age and sex without cirrhosis of the liver entered the study. We studied the prevalence of congestive gastropathy in cirrhotic patients, and the relationship between endoscopic and histological findings. Congestive gastropathy seen endoscopically was found to be more common in the cirrhotic group than in the control (85% vs. 5%, P < 0.05). The sensitivity, specificity and positive predictive value were 85%, 95% and 76%, respectively. The presence of endoscopic congestive gastropathy was correlated with the severity of liver disease (P < 0.05), but not to the etiology of cirrhosis and the size of esophageal varices with or without red color sign. Endoscopic congestive gastropathy showed no correlation with the histological features including gastritis, interstitial edema, vessel ectasia/congestion and hemosiderin in endoscopic biopsy specimens. In conclusion, congestive gastropathy is a common finding in cirrhotic patients. Its appearance is closely correlated with the severity of liver disease. There is no good correlation between endoscopic congestive gastropathy and mucosal histology.

We report an adult case of asymptomatic Bartter's syndrome with the first presentation of hypokalemic paralysis triggered by gentamicin injection. Marked hypokalemia and hypomagnesemia associated with excessive kaliuresis and magnesiuria were found. Plasma renin activity and aldosterone concentration were high, but blood pressure was normal. Renal biopsy revealed hypercellularity of the renin-producing cell of the juxtaglomerular apparatus. Muscular paralysis subsided after potassium chloride supplementation. Hypokalemia was corrected with potassium and magnesium supplements and the use of diclofenac. To the best of our knowledge, there have been no reports of muscular paralysis associated with gentamicin in Bartter's syndrome.

A rapid, simple, and nonradioactive method for diagnosing point mutations of c-K-ras oncogenes in gastroenterologic cancers is described. This method involved the selective amplification of DNA fragments from cancer tissues of surgical specimens with specific oligonucleotide primers, followed by digestion with restriction enzymes that recognized artificially created or naturally occurring restriction sites. To detect codon 12 mutations, an artificial Msp I site was created by introducing a single nucleotide mismatch into the 5' mutagenesis primer. Using a similar approach, an Hae III site was created to detect codon 13 mutations. Bal I and MBo II sites were used to detect codon 61 mutations. A total of 61 gastroenterologic cancer cases were studied. Of 35 cases of colorectal cancer, 7 showed mutations: 6 at codon 12 and 1 at codon 13. In 1 of 2 cases of cholangiocellular carcinoma, point mutation at codon 12 was found. One case of duodenal cancer showed point mutation at codon 12. No mutations were found in the cases of hepatocellular carcinoma (4), gastric cancer (12), esophageal cancer (3), or pancreatic cancer (2).

The "eradication of malaria" in Taiwan was announced by WHO in 1965. From 1966 to 1989, 919 malaria cases were detected in Taiwan. Of these cases, 803 were classified as imported malaria. During 1977 to 1989, our hospital collected 11 cases of imported malaria, 6 of Plasmodium falciparum (PF), including 1 suspicious case, 2 of Plasmodium vivax (PV), 1 of mixed infection (PF plus PV), and 2 unclassified. Most of the patients presented clinically with fever and chills. Hepatosplenomegaly was the most common abnormal finding during the physical examination. Jaundice and anemia occurred in the more severe cases. No cases had lymphadenopathy which is helpful in making a differential diagnosis. Six cases had thrombocytopenia which may be considered as an indirect sign in the diagnosis. The MCV levels were within normal limits in all of the cases. This may indirectly imply a potential protective effect against malaria infection in cases of congenital hemoglobinopathy such as thalassemia or G6PD deficiency. Initially, 10 cases were given "standard treatment", which consisted of chloroquine 450 mg qd for 2 days then 300 mg qd for 2 days and primaquine 15 mg qd for 2 weeks. Four cases of chloroquine resistance were encountered, all in cases with PF infection. Two cases were grade I delayed type resistance and were successfully treated with Fansidar, tetracycline and quinine. Two cases were grade II resistance and presented clinically as cerebral malaria. Intravenous quinine was given plus Fansidar and tetracycline. The cases were resolved without sequele or recurrence. None of the cases, except for 2, received chemoprophylaxis.(ABSTRACT TRUNCATED AT 250 WORDS)

Eosinophilic gastroenteritis is a relatively uncommon disease of unknown etiology. Eosinophilic ascites resulting from significant serosal involvement is the rarest clinical subtype. The case reported here is of a 30-year-old male presenting with abdominal pain, diarrhea, and ascites. His personal history included childhood asthma, allergic rhinitis, and recurrent urticaria. The clinical picture was characterized by peripheral eosinophilia and eosinophilic infiltrates of the stomach and small bowel. Computed tomogram (CT) of the abdomen showed generalized thickening of the gastric and small bowel wall. Paracentesis revealed exudative ascites rich in eosinophils. The patient experienced an impressive response to steroid therapy.

The most common extrahepatic metastasis of primary carcinoma of the liver is pulmonary metastasis. The discrepancy in the incidence of metastasis between clinical and postmortem series is quite obvious. The rapidly fatal course of hepatoma, the limitations of the chest x-ray film in detecting the small implantation, and incomplete follow-up of patients lead to the lower incidence in clinical series. From January 1974 to December 1982, there were 470 cases of primary carcinoma of the liver at Taipei Municipal Jen-Ai Hospital. Among them, we found 439 cases of hepatocellular carcinoma and 31 cases of cholangiocellular carcinoma. We also found 50 cases (11 percent) of hepatocellular carcinoma with pulmonary metastasis and ten cases (2 percent) of hepatocellular carcinoma with bone metastasis. Seven of the ten cases of bone metastasis were intrathoracic. Additionally, we found that it was rare to have pulmonary metastasis in cholangiocellular carcinoma; it only occurred once. Hematogenous and lymphatic spread and direct invasion are the principal ways for metastasis in primary hepatoma. Multiple nodulation and pleural effusion are the main manifestations in the chest x-ray film. Additionally, we saw lymphangitic carcinomatosis and miliary lesions in some of our patients. The common characteristic of these metastases is that most of the lesions arise from or are prominent in the right lower pulmonary field. Osteolysis appears in the patients with bone metastasis. Most of the metastases form a big protruding mass of tumor.

In 20% of patients with duodenal ulcers, treatment is ineffective. Ulcers are considered resistant when they fail to heal following 8-12 weeks of treatment with full dose duodenal ulcer therapy. The present study reviewed the records and endoscopic findings of 73 patients who were considered by their physicians to have resistant duodenal ulcers. From this population, 15 patients with documented, active duodenal ulcers were chosen to participate in this study. Each had failed treatment with cimetidine or ranitidine at recommended doses administered for from 56 days to more than 2 years. After up to 6 weeks' treatment with 20 mg famotidine given twice daily, eight patients showed endoscopic evidence of healing (S1 or S2) based on the criteria established by the Japanese Society of Digestive Endoscopy. Five others showed moderate to marked improvement H1-H3). No patient experienced side-effects during famotidine treatment. The greater antisecretory effect of famotidine may be useful in treating duodenal ulcer patients who fail to heal with cimetidine or ranitidine.

Thirty-four cases of SLE treated during the past seven years (1974-1981) in Taipei Municipal Jen-Ai Hospital are reported and analyzed. Diagnosis of SLE was based on ARA preliminary criteria and Hahn's preliminary criteria. There were 32 females (94.2%) and 2 males (5.8%). The mean age at diagnosis was 28.5 years (range 14-51). Clinical manifestations were as follows: facial erythema 24 cases (70.6%), Raynaud's phenomenon 4 cases (11.4%), oral or nasopharyngeal ulceration 7 cases (20.6%), arthritis without deformity 22 cases (64.7%), proteinuria 21 cases (61.8%), pleural or pericardial effusions 13 cases (38.2%), psychosis or convulsions 9 cases (26.5%), hematological abnormalities 25 cases (73.5%). Laboratory findings were as follows: positive ANA test 33/34 (97.0%), hypocomplementemia 10/13 (76.9%), direct Coombs' test 4/18 (22.2%), indirect Coombs' test 1/13 (7.6%), LE cell 19/34 (55.9%), RA Latex 7/17 (41.7%), polyclonal gammopathy 15/17 (88.2%), anemia 25/34 (73.5%), leukopenia 12/34 (35.3%), thrombocytopenia 10/34 (29.4%). Three cases were complicated by herpes zoster, one by hyperthyroidism, and one by autoimmune thyroiditis. Ten cases died, including 4 renal failure, 2 heart failure, 2 cases of committed suicide and 1 case of CNS involvement.