Clinical reasoning and decision-making in practice. A man with hip pain and fever

A 42-year-old man was admitted to the hospital because of pain in the left hip. On examination he was febrile at 38 degrees C and he walked with a limp. The chest, abdomen and extremities were normal. Laboratory tests showed an elevated ESR and CRP. The ANA test was positive. CT-scan of the abdomen revealed a mass in the psoas region and some dilatation of the left renal pelvis. Following the histological results of the first and second diagnostic percutaneous biopsies, the clinicians suspected idiopathic retroperitoneal fibrosis. They treated the patient with corticosteroids for a period of 4 weeks. After a short interval of improvement this treatment failed and a third biopsy was taken. Subsequently, the diagnosis of anaplastic large cell lymphoma (ALCL) was made. The patient was successfully treated with combination chemotherapy. Usually, in practice, clinical reasoning and decision-making is carried out in accordance with Bayes' theorem. But when the a priori probability of disease is unknown and the likelihood ratio of a diagnostic test unavailable, one has to combine the available 'evidence' with critical thinking, interdisciplinary communication, judgement, intuition and common sense.     

Clinical reasoning and decision-making in practice. A woman with hypokalaemia

A 39-year-old woman had been investigated elsewhere due to symptomatic hypokalaemia, renal potassium wasting and metabolic alkalosis. Vomiting was considered to be the underlying cause but the patient repeatedly denied this behaviour. After extensive investigations, Bartter's syndrome was finally diagnosed. Eighteen months later the patient was readmitted due to progressive renal insufficiency. On the basis of a very low urinary chloride excretion and the aforementioned laboratory results, it was concluded that the metabolic disturbances were due to vomiting and the diagnosis of Bartter's syndrome was rejected. Upon being confronted with these findings, the patient finally admitted to vomiting, but stated that this happened involuntarily after meals. She had a stenotic ulcer in the pylorus and a gastric biopsy demonstrated the presence of Helicobacter pylori associated gastritis. Eradication therapy was given and the symptoms disappeared. The renal insufficiency was partly accounted for by hypovolaemia and partly by tubulointerstitial nephropathy (demonstrated by kidney biopsy) which was probably due to the chronic hypokalaemia. This case illustrates the difficulty of establishing the differential diagnosis of hypokalaemia, especially in the case of denied vomiting. However, it also shows that in such cases the correct diagnosis can be made if objective parameters are used.     

Clinical reasoning and decision-making in practice. A man with inexplicable joint pain and subsequent poor clinical condition

A 52-year-old man presented with polyarthritis and was negative for rheumatoid factor, anti-CCP and ANA. He was treated with low-dose methotrexate, the drug of first choice in rheumatoid arthritis. The arthritis disappeared, but the patient developed fever, progressive dyspnoea, appetite loss and weight loss. Upon hospital admission his medication was stopped and community-acquired pneumonia was diagnosed. The fever persisted despite antibiotic treatment. The tentative diagnosis of rheumatoid arthritis was changed to systemic lupus erythematosus, based on the change in clinical condition that could not be explained by polyarthritis and seroconversion to ANA- and anti-dsDNA-positive. The patient was treated with high-dose steroids and azathioprine and remained in remission for more than 1 year after treatment. The ANA test remained strongly positive, whereas anti-dsDNA was no longer detectable. This case stresses the limited value of classification criteria for the diagnosis of rheumatoid arthritis. To differentiate between rheumatoid arthritis and systemic lupus erythematosus, tests for autoantibodies against citrullinated peptides can be used. To differentiate between systemic lupus erythematosus and infection, tests for anti-dsDNA antibodies, antinuclear antibodies, C-reactive protein and complement can be used.     

Clinical reasoning and decision-making in practice. A patient with fever and pancytopenia

An 82-year-old man was admitted with a 1-week history of chilling fever and dry cough. Laboratory tests revealed pancytopenia and elevated levels of C-reactive protein and lactic dehydrogenase (LDH). Screening for infectious diseases was negative. A bone marrow biopsy showed aspecific findings. The combination of pancytopenia, persistent fever, elevated LDH and hepatomegaly (demonstrated by ultrasound examination of the abdomen) was suggestive of the haemophagocytic syndrome. This was confirmed by very high levels of ferritin and soluble interleukin-2 receptor in the blood. In addition, re-examination of the bone marrow showed several haemophagocytic histiocytes. A polymerase chain reaction for Epstein-Barr virus (EBV) revealed a very high viral load. Since the patient had a history of an increased level of anti-EBV immunoglobulin-G, this was explained by a reactivation of the EBV infection. On the sixth day in hospital the patient developed signs of bilateral pneumonia and subsequent multiple organ failure. Despite intensive treatment the patient died. Autopsy revealed no haematological or other malignancies, but did show haemophagocytosis in many organs. It was then concluded that the patient had a virus-associated haemophagocytic syndrome, due to a reactivation of EBV, for which no underlying cause was found.     

Clinical reasoning and decision-making in practice. A patient with oliguria following prostatectomy

A 70-year-old man with clinically localised prostate carcinoma underwent extraperitoneal endoscopic radical prostatectomy. His medical history revealed hypertension, renal colic, hypogonadotropic hypogonadism and recurrent deep venous thrombosis in the legs. The operation was uneventful with 500 ml blood loss and no periods ofhypotension. The patient developed oliguria within 12 h after surgery. A hypovolemic state was initially suggested to explain the oliguria and increasing amounts of intravenous fluids were administered. The oliguria persisted, however, and the patient did not respond to a diuretic. There was no fluid loss in the drain. Blood pressure, pulse and temperature were normal. Peritonitis and bowel perforation were excluded. Ultrasound examination of the bladder and kidneys revealed an empty bladder and no dilatation of the upper urinary tract, which excluded a post-renal obstruction. The clinical situation deteriorated within hours as the patient developed anuria, bowel distension, metabolic acidosis with progressive renal failure and signs of respiratory distress for which mechanical ventilation was needed. A chest X-ray prior to intubation did not show pneumonia or signs indicating pulmonary embolism. CT of the abdomen was performed to evaluate urinary leakage but revealed no fluid collection or urinoma. Thus pre- and post-renal causes of oliguria were excluded. In view of the systemic symptoms, intra-abdominal pressure was measured using a bladder catheter; it varied between 25 and 35 cm water. Together with the clinical situation, a diagnosis of abdominal compartment syndrome was made and coeliotomy was performed immediately. Within 10 min after decompression of the peritoneal cavity, diuresis started spontaneously. Renal function was restored to preoperative levels in 3 weeks. Abdominal compartment syndrome is a potentially life-threatening cause of anuria. The syndrome should be part of the differential diagnosis for patients with postoperative anuria, including those who underwent extraperitoneal minimally invasive procedures.     

Clinical reasoning and decision-making in practice. A patient with shortness of breath and hyponatraemia

A 61-year-old man with no significant medical history was referred by the general practitioner with progressive dyspnoea despite treatment with steroids and antibiotics. The patient had severe hyponatraemia. Eventually hypervolemic hyponatraemia due to ischemic cardiac failure was diagnosed. After recompensation by fluid restriction and lisdiuretics coronary bypass surgery was carried out. The patient was discharged free of symptoms. This case is a reminder that heart failure may not present itself in a classical manner but as hypervolemic hyponatraemia.     

Clinical reasoning and decision-making in practice. An older man with prostate carcinoma and a painless paraparesis of the legs

A 78-year-old man with metastasised prostate carcinoma presented with a painless paraparesis. His cerebrospinal fluid showed elevated protein and a mononuclear pleiocytosis, but cytology investigations of 5 separate samples revealed no malignant cells in the cerebrospinal fluid. Extensive viral and bacterial tests (including ELISA for Borrelia burgdorferi) of serum and cerebrospinal fluid were negative. On the day radiation therapy for presumed leptomeningeal metastases was due to start the IgG and IgM Western blot for Borrelia were found to be positive, indicating neuroborreliosis. Soon after the start of antibiotic therapy the paraparesis began to improve and after four weeks the patient had made a complete recovery. In patients with a progressive paraparesis, neuroborreliosis should be considered even in the absence of pain.     

Clinical thinking and decision-making in the practice. A young man in shock

A previously healthy male aged 32 years with noticeably long extremities went into shock whilst straining to defaecate. He did not lose consciousness and reported pain high in the back and in the abdomen. Transoesophageal echocardiography revealed no abnormalities. Ultrasonography of the abdomen showed blood in the abdominal cavity, following which laparotomy was performed. An aneurysm of the splenic artery was found to have ruptured. The aneurysm and the spleen were removed. Morbid-anatomical examination showed no vascular abnormalities. The postoperative course was uneventful.     

Clinical reasoning and decision-making in practice. A patient with loss of vision and painful legs

A 69-year-old man was admitted to the Department of Ophthalmology with bilateral loss of vision. For a few months he had also had shooting pains in both legs and instability of gait. Neurological examination showed loss of vision bilaterally and minor sensory disturbances of the legs with diminished tendon reflexes. As extensive further examination showed no specific abnormalities, the tentative diagnosis 'arteriitis temporalis' was made. Despite treatment with corticosteroids his condition deteriorated. Only after a repeat medical history had been taken did it become clear that in the past he had had homosexual contact with a number of partners. This increased the likelihood of a sexually transmitted disease in the differential diagnosis. In the meantime the results from serological tests became known: there were strongly elevated titres for syphilis in both serum and cerebral spinal fluid. Eventually the patient was diagnosed with neurosyphilis with ocular involvement and tabes dorsalis. He recovered almost completely in a few months after treatment with doxycycline.     

Clinical reasoning and decision-making in practice. A man with jaundice, renal function disorder, thrombocytopenia, muscular pain and confusion

A 51-year-old man became jaundiced, after experiencing flu-like symptoms for a week. On admission anaemia, thrombocytopenia, and hepatic and renal function disorders were present. One month previously he had had sinusitis. Thrombotic thrombocytopenic purpura (TTP)/haemolitic-uraemic syndrome (HUS) was suspected, but the thrombocyte count rose spontaneously. The leukocytosis indicated an infectious cause of the symptoms and the jaundice appeared to originate from intrahepatic cholestasis. All symptoms could be traced to leptospirosis icterohaemorrhagica (known as 'Weil's disease'). The source was possibly the patient's fishpond. He recovered spontaneously and was also treated with antibiotics. Leptospirosis, a multi-organ disease is seen ever more infrequently as an occupational disease in stock farmers and increasingly frequently in people whose hobbies involved animals.     

Clinical reasoning and decision-making in practice. A 31-year-old woman with transient monocular blindness and polycythaemia

A 31-year-old woman presented with recurrent transient monocular blindness. As transient ischaemic attacks were suspected further investigations were targeted at evaluation of premature atherosclerotic lesions in the internal carotid artery. Initially laboratory tests were not performed. After referral to a cardiovascular-disease prevention outpatient clinic, laboratory evaluation disclosed a marked isolated polycythaemia that turned out to be secondary to right-left shunting through multiple pulmonary arteriovenous malformations. The ultimate diagnosis was hereditary haemorrhagic telangiectasia. Later on, physical signs such as telangiectasia and central cyanosis were noticed. In the clinical decision-making process, laboratory tests associated with causes of transient monocular visual loss were not carried out and therefore clues important for the ultimate diagnosis were not obtained. In only a minority of young patients with transient monocular visual loss can this be ascribed to premature atherosclerosis. For these reasons, a proper physical examination and laboratory tests directed towards other causes must be part of the initial diagnostic work-up in young patients with visual disturbances and suspected transient ischaemic attacks.     

Clinical reasoning and decision-making in practice. A young boy with fever, pancytopenia and an enlarged spleen

A 5-year-old boy presented with fever and fatigue after a holiday in northern Italy. On physical examination a marked splenomegaly was found. Laboratory investigations showed a pancytopenia as well as several markers suggesting an autoimmune disease. The splenomegaly and pancytopenia continued to progress despite treatment with prednisolone and intravenous immunoglobulins. One and a half years after presentation, the spleen had grown to such an extent that it was causing mechanical problems. Splenectomy was performed for diagnostic and therapeutic purposes. Histological investigation of the spleen showed amastigotes of Leishmania. PCR confirmed the diagnosis visceral leishmaniasis. Leishmaniasis is too often considered to be a tropical disease only. In recent years it has frequently been seen in southern European countries around the Mediterranean Sea.     

Clinical reasoning and decision-making in practice. A 76 year old woman with gastric carcinoma and cardiac valve disease

A 76-year-old woman with combined aortic and mitral valve disease presented with anaemia due to a gastric carcinoma. Further staging revealed no evidence for metastatic disease. Approval for surgery for the carcinoma was obtained after a cardiologist and anaesthesiologist were consulted. On the day of surgery, however, the attending anaesthesiologist estimated the operative risk to be unacceptable. The patient reversed her decision and decided not to have the oncological operation, as she felt well at the moment and considered the limited additional survival time not worth the anxiety. The expert opinion of an anaesthesiologist, a cardiologist and an oncologist not primarily involved in this case show that they differ as to the question who is responsible for the decision-making process in patients such as this one. The evidence on estimating operative risk in patients with cardiac valve disease undergoing noncardiac surgery is not unequivocal. Furthermore, there is a shared responsibility when more physicians are involved in the process of decision-making. Agreement on operative risk between physicians is necessary in order to prevent transfer of conflicting information to the patient.