godfather of surgery

Chapter 1395 Training
Yang Ping nodded, walked to his desk, picked up the phone, and dialed the neurology department's number: "This is Yang Ping. The MRI results for the patient in bed 15 are in; it's hypertrophic pachymeningitis. I suggest checking ANCA, ACE, antinuclear antibodies, immunoglobulins, complement, HIV, syphilis, and tuberculosis T-SPOT. Do another lumbar puncture to check the cerebrospinal fluid ACE and IgG synthesis rates. If all of these are normal, do a whole-body PET-CT scan. The treatment plan is to start with methylprednisolone pulse therapy for three days, followed by oral prednisone."

After hanging up the phone, he turned around and looked at Zaxi: "From today onwards, you will be in charge of this patient. You will be responsible for following up on all the examinations and treatments. If you have any questions, come to me anytime."

Tashi nodded vigorously.

From then on, Zaxi devoted almost all his time to this patient. Every morning he would make rounds, ask about changes in the patient's condition, perform physical examinations, and record the patient's progress. He consulted a large amount of literature, learning about the etiology, pathology, diagnosis, treatment, and prognosis of hypertrophic pachymeningitis. He also learned how to interpret MRI scans of this disease and how to determine the extent and degree of dural enhancement.

The patient's test results came back one after another: ANCA negative, ACE normal, antinuclear antibody negative, rheumatoid factor negative, immunoglobulins normal, complement normal, HIV negative, syphilis negative, tuberculosis T-SPOT negative, cerebrospinal fluid ACE normal, and IgG synthesis rate normal. All secondary causes were ruled out. Finally, a whole-body PET-CT scan was performed, which also revealed no evidence of tumors or sarcoidosis.

Therefore, this is idiopathic hypertrophic pachymeningitis.

The treatment was very effective. After three days of methylprednisolone pulse therapy, the patient's headache was significantly reduced. A week later, her blurred vision improved. Two weeks later, the strength in her right limbs returned, and she could walk normally again. Upon discharge, the patient's fundus examination showed that the papilledema had subsided by more than half. She held Zaxi's hand and said, "Dr. Zaxi, thank you."

Zaxi shook his head: "It wasn't me, it was Professor Yang."

The patient smiled and said, "Professor Yang said it was you who made the diagnosis."

Zaxi paused for a moment, then smiled and said nothing.

The neurology department director and doctors nearby were also very envious of Zaxi, because he was a student personally taught by Professor Yang, and his future prospects in medicine were self-evident.

That evening, Zaxi sat in his dormitory, flipping through his notebook and looking at all the notes he had written over the past two weeks. From the bewilderment he felt upon first seeing the medical records, to the anxiety while researching literature, to the tension during the MRI scan, to the relief upon finally receiving the diagnosis. This process was like solving a crime: finding the crucial clue from a pile of seemingly unrelated leads, and then following it step by step until the truth came to light.

He finally understood why Professor Yang had asked him to participate in Professor Zhou's case. It wasn't to solve the case, but to teach him a way of thinking—a way of thinking that finds order in chaos. This way of thinking could be used in solving cases, and even more so in treating patients.

On Friday afternoon, Zaxi pushed open the door to Professor Yang's office with his notebook in hand. Inside, besides Yang Ping, sat another person: Director Liu of the Neurology Department, a man in his fifties with gray hair, one of the founders of the Neurology Department at Sanbo Hospital. Zaxi's heart skipped a beat. Director Liu's personal visit indicated something serious was amiss.

"Sit down!" Yang Ping pointed to the chair, and after Zaxi sat down, he continued, "Director Liu has a patient he'd like you to take a look at."

Zaxi was taken aback. Should he go and see?

Director Liu noticed his confusion, smiled, and took a thick medical record from his briefcase, placing it on the table. “Dr. Zaxi, your treatment of that hypertrophic pachymeningitis patient was very successful. Our entire department discussed it; your diagnostic approach was very clear, and your literature review was comprehensive. We’ve been working on this patient for half a month without understanding him, and we’d like to ask for your help.”

Zaxi's face flushed red. He was just a visiting doctor, while Director Liu was a doctoral supervisor and a leading figure in the field.

He opened his mouth as if to say something, but Yang Ping raised his hand to stop him.

"Don't be so modest." Yang Ping's tone was very calm. "Since Director Liu has given you a chance to practice, go and take a look. It would be best if you could diagnose it, but if you can't, consider it a learning experience."

"I've already spoken to the attending physician. You can contact him directly and assist him with patient management. You can also visit the neurology department to see the patient and review the medical records at any time." Director Liu was a shrewd person. Since Professor Yang valued Dr. Zaxi so much, he figured he might as well do him a favor. So, here's another patient who seems quite difficult to handle, let Dr. Zaxi get some experience.

Zaxi didn't stand on ceremony and took the medical record. The patient was a 35-year-old male, a local resident of Nandu, and a programmer. Chief complaint: recurrent fever, joint pain, and rash for six months, worsening with confusion for one week. He continued flipping through the records. The admission record stated that the patient began experiencing recurrent fevers six months ago, with temperatures between 38 and 39 degrees Celsius, without a clear pattern. He also experienced symmetrical joint pain in his limbs, mainly in his wrists, knees, and ankles, which worsened with activity. He also had recurring red rashes that weren't itchy, subsided after a few days, but reappeared after a few more. He had consulted several hospitals and had blood tests, liver and kidney function tests, rheumatoid factor tests, and autoantibodies, all of which showed no significant abnormalities. He was diagnosed with "undifferentiated arthritis" and had been treated with nonsteroidal anti-inflammatory drugs (NSAIDs), but the effect was poor. A week ago, the patient suddenly became confused, incoherent, and sometimes didn't recognize his family members, so his family took him to the neurology department of Sanbo Hospital.

This case is indeed difficult to handle. So far, he has no experience whatsoever. He only solved the previous case by consulting the literature.

Zaxi frowned. Fever, joint pain, and rash were symptoms of rheumatic immune disease. But the sudden onset of confusion indicated that the central nervous system was also affected. He continued flipping through the pages. The neurology examination records showed that the patient was confused, disoriented, had decreased calculation ability, and significantly impaired recent memory upon admission. Physical examination revealed no obvious neurological focal signs, no hemiplegia, no sensory disturbances, and no ataxia. The head CT scan was normal. Lumbar puncture showed normal intracranial pressure, normal cerebrospinal fluid routine examination, normal biochemistry, normal cytology, and negative pathogen tests. The electroencephalogram showed diffuse slow wave activity, suggesting diffuse brain dysfunction.

He flipped to the lab report. Complete blood count, liver and kidney function, electrolytes, and coagulation function were all normal. The three rheumatoid factors—rheumatoid factor, anti-CCP antibody, and antinuclear antibody—were all normal. Inflammatory markers—erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)—were slightly elevated. Tumor markers were normal. HIV, syphilis, Epstein-Barr virus (EBV), and cytomegalovirus (CMV) were all negative.

Zaxi closed the medical record. It was another patient with all routine tests normal. Fever, joint pain, rash, and confusion. Rheumatic immune disease affecting the central nervous system? But all autoantibodies were negative. Infectious encephalitis? But the cerebrospinal fluid was normal, and pathogen tests were negative. A tumor? But tumor markers were normal, and the head CT scan was normal. What was this disease?

He looked up at Director Liu, who said, "How about it? Want to give it a try?"

"With Director Liu backing you up, what are you afraid of? Go ahead and do it boldly according to your ideas. If you need to order any tests, especially invasive ones, be sure to communicate with the attending physician and report to Director Liu." Yang Ping instructed Zaxi, "Training is training, but safety comes first. In medical teaching, this is called 'letting go but not keeping an eye on things.'"

"Director Liu, I'm going to check on the patient," Zaxi said.

Director Liu nodded: "Okay, the patient is in bed 15 of our neurology department. You can go now. I still need to talk to Professor Yang. I've already spoken to Dr. Zeng, who is in charge of the patient there."

Zaxi stood up, walked to the door, and suddenly looked back at Yang Ping. Yang Ping waved his hand: "Go ahead, don't be afraid. You're a clinician, you'll see and do a lot, and you'll naturally become skilled."

"Hmm!" Zaxi took a deep breath, and the stage fright he felt the first time was clearly gone.

Zaxi arrived at the neurology department, where Dr. Zeng accompanied him to bed 15. The patient was a young-looking, thin, pale man lying in bed with his eyes open, but his gaze was somewhat unfocused. His wife was sitting by the bed and stood up when she saw Zaxi enter.

Dr. Zeng explained, "This is Dr. Zaxi, whom we invited for a consultation."

The patient's wife, seeing that the doctor was young and didn't seem very skilled, showed her displeasure, feeling that the hospital wasn't taking her husband's condition seriously. She stood by the bed, not giving Dr. Zaxi any space to examine her.

Tashi ignored her obvious unwelcome attitude and said, "I'm Professor Yang's student, I've come to visit."

Upon hearing that he was Professor Yang's student, the patient's wife immediately stepped aside, her expression instantly becoming respectful, and she even managed a fawning smile.

Tashi walked to the bedside and said softly, "Hello, my surname is Tashi, Dr., may I ask you a few questions?"

The patient turned his head and looked at him, his eyes somewhat confused. After a while, he said woodenly, "Who are you?" His voice was very soft, as if he were talking in his sleep.

Zaxi's heart sank; the patient's level of confusion was quite high. He sat down and began asking about the patient's medical history. Most of the questions were answered by the patient's wife. The patient had been ill for six months, initially with a fever, which he thought was a cold. He took cold medicine, the fever subsided, but returned a few days later. Later, he developed joint pain—his wrists, knees, and ankles ached, sometimes so badly he couldn't even type. The rash appeared later, patches of red spots all over his body, not itchy, disappearing in a few days, only to reappear in other places. He had seen several hospitals and undergone countless blood tests, but they all said they couldn't find anything wrong. A week ago, he suddenly started talking incoherently. At work, his colleagues noticed his speech was incoherent and thought he was joking. Later, when he returned home, he didn't even recognize his wife.

Zaxi asked, "Did he take any special medicine or get any vaccines before he fell ill?" His wife thought for a moment and shook her head, "No, he has always been in good health, he never gets sick, and he rarely even catches a cold."

Zaxi then asked, "Do you have any pets? Have you ever been to the wild?"

The wife said, "No, he's a programmer. He sits in front of the computer all day and doesn't go anywhere."

Zaxi noted down all the information and then performed a physical examination on the patient. His temperature was 37.8 degrees Celsius, a mild fever. There were indeed scattered red rashes on his skin, mainly distributed on the trunk and proximal extremities; they were indistinctly bordered erythematous patches that blanched upon pressure. His joints did not show obvious redness, swelling, heat, or pain, but there was significant resistance to movement. Neurological examination revealed confusion and disorientation. When asked his date, he said he didn't know. When asked where he was, he said he was in the hospital. When asked his name, he answered correctly after three attempts. His calculation ability was impaired. He thought for a long time about 100 minus 7, saying 93, but couldn't recall the answer to "subtract 7 again." His memory was also impaired; after five minutes, he couldn't remember any of the three words he was asked to memorize. There was no hemiplegia, sensory disturbance, or ataxia. Meningeal signs were negative.

After finishing his examination, Zaxi stood by the bedside for a while before leaving the ward, and the patient's wife immediately followed him out.

"Dr. Zaxi, I'm sorry. I mistook you for an ordinary young doctor. I didn't realize you were Professor Yang's student," the patient's wife explained embarrassedly.

Tashi smiled and said, "It's alright, don't worry, we will do our best."

"Okay, thank you, thank you!" The patient's wife was relieved.

Back in Yang Ping's office, Yang Ping was still reading documents; Director Liu had already left.

"How is the situation?" Yang Ping put down the documents.

Zaxi sat down and recounted the findings. Afterward, he looked at Yang Ping: "Professor Yang, this patient's symptoms are very complex. Fever, joint pain, and rash are symptoms of rheumatic immune diseases. But all autoantibodies are negative. The confusion indicates central nervous system involvement, but the cerebrospinal fluid is normal. I can't understand it."

Yang Ping looked at him but didn't answer. Instead, he asked, "With the experience of diagnosing a difficult case last time, you should have an easier time this time."

Zaxi thought for a moment and said, "I need to check the literature. This disease is too atypical. I need to see if there are any similar case reports."

Yang Ping nodded: "Okay, go check it out."

Zaxi returned to his study room, turned on his computer, and began searching on PubMed. He used several keyword combinations: fever, arthralgia, rash, encephalopathy, and negative autoantibodies. The search returned few articles. He read the titles and abstracts one by one, then translated them using translation software, and finally asked a available PhD student to proofread them. He now understood the importance of English.

He found a case report from the *New England Journal of Medicine*, titled "A Case of Adult-onset Still's Disease Presenting with Fever, Joint Pain, Rash, and Encephalopathy." Zaxi opened the full text and quickly skimmed through it. The patient described in the case report was almost identical to the one he had seen in the ward: a middle-aged man with fever, joint pain, and a rash, later developing confusion. All autoantibodies were negative, erythrocyte sedimentation rate and C-reactive protein were slightly elevated, cerebrospinal fluid was normal, and the final diagnosis was adult-onset Still's disease.

Zaxi's heart raced as he continued reading. The article stated that adult-onset Still's disease is a rare autoinflammatory disease of unknown cause, characterized primarily by fever, joint pain, and rash, and can affect multiple systems, including the central nervous system. Diagnosis lacks specific laboratory indicators and relies mainly on clinical criteria and exclusion of other diseases. The commonly used diagnostic criteria are the Yamaguchi criteria, with primary criteria: fever ≥39 degrees Celsius for more than one week, joint pain for more than two weeks, typical rash, and white blood cell count ≥10×10/L; secondary criteria: sore throat, lymphadenopathy, hepatosplenomegaly, abnormal liver function, and negative rheumatoid factor and antinuclear antibody. A diagnosis can be made if five or more criteria are met, with at least two of the primary criteria.

Zaxi copied down the criteria for diagnosis and compared them with the patient's clinical presentation. Fever—present, but only slightly over 38 degrees Celsius, not reaching 39 degrees Celsius, thus not meeting the primary criterion. Joint pain—present, lasting for six months, meeting the primary criterion. Typical rash—present, meeting the primary criterion. White blood cell count—normal, not meeting the primary criterion. Sore throat—not mentioned by the patient, not meeting the secondary criterion. Lymphadenopathy—not found in the patient, not meeting the secondary criterion. Hepatomegaly and splenomegaly—not present, not meeting the criteria. Abnormal liver function—the patient's liver function was normal, not meeting the criteria. Negative autoantibodies—meeting the secondary criterion. In total, only three criteria were met—joint pain, rash, and negative autoantibodies. Not enough for five criteria.

Zaxi frowned. If it didn't meet the Yamaguchi criteria, then it wasn't adult-onset Still's disease. He continued reading the literature. There was a review article discussing atypical presentations of adult-onset Still's disease. The article stated that some patients might have a fever below 39 degrees Celsius, and some might have normal white blood cell counts. For these atypical cases, diagnosis was more difficult, requiring the exclusion of all other possibilities.

He put down the first article and searched for several more related ones. One article from the *Annals of Rheumatology* discussed a spectrum of diseases called "autoinflammatory syndromes." The article stated that some patients present with recurrent fever, joint pain, and rashes, but do not meet the diagnostic criteria for any known autoinflammatory disease. These patients may belong to "unclassified autoinflammatory syndromes." Diagnosis relies on clinical presentation and exclusion of other diseases. Treatment may involve glucocorticoids and IL-1 antagonists.

Zaxi printed out the relevant literature and looked up some information about autoinflammatory diseases. He spent an entire afternoon reading through all the related literature. Then he drew a mind map in his notebook, listing all possible diagnoses centered on the patient's symptoms, and then eliminated them one by one using the process of elimination.

Infectious disease – The patient has had recurrent fever for six months, but no local signs of infection, pathogen tests are negative, and anti-infective treatment is ineffective; this is unlikely. Neoplastic disease – The patient has no evidence of a primary tumor, tumor markers are normal, and a PET-CT scan has not yet been performed, but this is also unlikely. Rheumatic immune disease – The patient has fever, joint pain, and rash, but all autoantibodies are negative, which does not conform to typical rheumatic immune diseases. Autoinflammatory disease – This is consistent, but the specific type is unknown. Drug reaction – The patient has no history of long-term medication use; this is ruled out. Hyperthyroidism – The patient's thyroid function is normal; this is ruled out…

Tashi now deeply understands that an excellent doctor must learn to consult English literature in order to learn from the experience of doctors all over the world.

He closed his notebook, glanced at his watch; it was already six in the afternoon. He stood up, picked up the documents, and headed towards Professor Yang's office, where Professor Yang hadn't yet left work.

He was looking at an MRI scan when he saw Zaxi come in. He put the scan down and asked, "Did you find it?"

Zaxi placed the literature on the table and began his report. He explained all the information he had found, his analysis process, and the reasons for ruling out other diagnoses. Finally, he stated his conclusion: "Professor Yang, I suspect this patient has an autoinflammatory disease, possibly an atypical presentation of adult-onset Still's disease, or perhaps an unclassified autoinflammatory syndrome. I recommend a whole-body PET-CT scan to rule out potential tumors and infections. Simultaneously, check serum ferritin and glycated ferritin—serum ferritin is usually significantly elevated in adult-onset Still's disease patients, while the proportion of glycated ferritin is decreased. If ferritin is significantly elevated, it would support the diagnosis."

After listening, Yang Ping didn't speak immediately. He opened the documents and read them page by page. After finishing, he dialed Director Liu's cell phone.

"Director Liu, hello. For the patient in bed 15, I suggest checking serum ferritin and glycated ferritin. Yes, and a whole-body PET-CT scan as well. Okay, thank you."

After hanging up the phone, he looked at Zaxi and said, "Just like last time, you'll analyze the results after they come out."

Tashi nodded.

Three days later, the test results came back. Serum ferritin was 1500 nanograms per milliliter, while the normal range is 20 to 200. Glycated ferritin was 20%, while the normal range is above 50%. Ferritin was significantly elevated, and the glycated ferritin percentage was significantly decreased. This is a typical presentation of adult-onset Still's disease. The PET-CT scan also came back, showing no evidence of tumors or infections. There were no abnormal metabolic foci throughout the body, only a slightly enlarged spleen.

Zaxi took the results to Yang Ping. Yang Ping looked at the results and nodded: "The diagnosis is clear, adult-onset Still's disease, affecting the central nervous system. It seems you've learned how to consult the literature. Now that the diagnosis is clear, let's get on with the treatment."

Zaxi explained his treatment plan: "Glucocorticoids, methylprednisolone pulse therapy, followed by oral prednisone with slow tapering. If the effect is not good, IL-1 antagonists, such as anakinin, or IL-6 antagonists, such as tocilizumab, can be added."

"Have you noticed that hormones can be used in many situations, and in some situations, it's the only way? Excellent doctors must master the use of hormones," Yang Ping reminded him.

This patient was still under Zaxi's care, as he was the attending physician assisting the neurology department.

Every morning he would make his rounds, just like before, asking about changes in the patient's condition, performing physical examinations, and recording the patient's medical history. He consulted a large amount of literature, learning about the etiology, pathology, diagnosis, treatment, and prognosis of adult-onset Still's disease. This time, he also learned how to interpret PET-CT scans of this disease and how to determine the size and metabolic activity of the spleen.

The treatment was very effective; after three days of methylprednisolone injections, the patient's temperature returned to normal. A week later, the joint pain significantly decreased, and the rash subsided. Two weeks later, he fully regained consciousness. When he was discharged, he held Zaxi's hand and said, "Dr. Zaxi, thank you. I finally know what disease I have."

Zaxi smiled and said, "You're welcome. It's our duty."