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General Public Hospital St. Pölten Department for Children and Youth - Head of Department: Prim. Univ. Doz. Dr. XXXXX Mrs. Dr. XXXXX Kurzentrum A-8344 Bad Gleichenberg
Discharge Report Dear colleagues, This is a brief summary concerning XXXXXX Xxx, born on 7/1/1992. Hospitalized on 9/23 - 9/25/1997, on 11/3 - 11/4/1997, on 12/1 - 12/2/1997, on 12/30 1997, on 1/20/1998, on 2/11/1998, on 3/9/1998, on 3/31/1998, and on 4/23/1998 in addition outpatient check-ups starting September 1997 till May 1998, last check-up on 5/4/1998. Diagnosis: Suspected X-chromosomal agammaglobulinaemia Bronchiectasis Chronic relapsing respiratory infections Anamnesis: This is a summary of the case history: After uneventful pregnancy, normal, vaginal, spontaneous delivery in the 41st week of gestation, BW 3600 g, Apgar 9/10/10. As a baby or a small child he showed no particular susceptibility to infections. The mother reported sporadic otitis without special complications. Xxx was hospitalized twice: from 8/9 till 8/16/93 due to scarlet fever, first febrile convulsion, air passage infection with purulent rhinitis, aphthous stomatitis, and injury with secondary dig. indic. man. dext infection. Course of illness and EEG-examination were normal. Chest X-ray showed an unobtrusive perihilar structural increase in the sense of peribronchitic changes. From 7/1 - 7/5/1994: Examination due to unexplained spasms - emotional spasms. EEG and chest X-ray at that point NAD (physician letters enclosed). In October 1996 and in November 1996, pneumonia in the left lower lobe region (three times) followed by relapsing bronchitis. Diagnosed and treated by the local pediatrician. Due to the chronic course and reoccurring pneumonia hospitalized in our department from 7/14 - 7/28/1997. Diagnosis of immunodeficiency as basic disease (physician letter). From 8/26 - 8/28/1997 briefly hospitalized in pediatric hospital Salzburg for bronchoscopic clarification. The bronchoscopy findings show larynx, trachea, and bronchi anatomically normal (see enclosed physician letter). Starting 9/23/1997 regular check-up and immunoglobulin substitution in our department. Family anamnesis: Mother suffers from Psoriasis. In 1991 one abort. A few close relatives suffer from cerebral spasms. No indication of immunodeficiency. Present status on 5/4/1998: 5 years 9 months old boy in relatively good general condition, obstructed nasal breathing, eardrum on both sides - as far as visible - undifferentiated, questionable tympanitis (tympanic discharge), on both sides small lymph nodes in mandibular angle, above the lung in left middle field and lower field constant medium bubbling rale auscultated, otherwise pediatric status normal . Weight on 9/23/97: 19 kg, on 5/4/98: 19.5 (25th percentile) Height on 9/23/97: 115 cm, on 5/4/98: 118 cm (50th - 75th percentile) Blood, serum, urine, and culture findings: BC on 9/23/1997: leuco 14,6 G/1, erythr 5,25 T/1, Hb 13,4 g/dl, Hk 40 %, MCV 76,2, MCH 25,6, MCHC 33,5, thrombo 431 G/1. Blood count on 4/23/1998: leuco 7,0 G/1, erythr 4,76 T/1, Hb 13,5 g/dl, Hk 39,8 %, MCV 83,6, MCH 28,3, MCHC 33,8, thrombo 395 G/1. CRP on 9/23/1997: 0,0 mg/dl, on 4/23/98: 0,5 mg/dl. Humoral immune status on 7/28/1997: Serious antibody deficiency in the sense of agammaglobulinaemia. Now showing inflammatory symptoms. The examined specific antibodies not detectable. Cellular immune status on 7/28/1997: Peripheral B-cells not detectable. This result agrees with the diagnoses of inherited X-chromosomal agammaglobulinaemia (Bruton's agammaglobulinaemia). Increased numbers of activated T-cells, T-NK cells, CD4- and CD8-subset in normal range or increased (T-cells), lymphocytes can be stimulated with mitogen, in normal range, Antigen (Tet Tox) low, but detectable. Recommend tetanus-booster inoculation, after 4 - 6 weeks check-up on cellular immune status. Humoral immune status on 11/5/1997: Serum Immunglobulin clearly reduced almost not detectable, known agammaglobulin- aemia. IVIG substitution therapy not sufficient. Humoral immune status on 3/31/1998: Known serious primary antibody deficiency in the sense of an inherited X-chromosomal agammaglobulinaemia. IVIG substitution therapy is adequate, recommend continuation of dosage as in the past. Titre in normal range (IgG antibodies using substitution therapy). Recommend immunological progress supervision in four months. (Medical report: Univ. Doz. Dr. XXXXX). Microbiological monitoring/sputum culture: Relatively constant proof of haemophilic influenza, according to the antibiotic spectrum sensitive to Augmentin/not sensitive to Clarithromycin, additional isolated proof of Xanthomonas maltofilia, greenish streptococcus and Haemophilus parainfluenzae occuring only once. Last findings: 4/16/1998: mouth germs 4/23/1998: haemophilic influenza Imaging Techniques: Lung CT on 11/4/1997: Unfortunately, the examination was considerably limited due to patient movement . The most noticeable finding is in the left apical lower lobe segment reaching to the posterior basal lower lobe segment; a sharply bordered structural concentration with partially positive aerobronchogramm. Bordering this are several clearly visible bronchiectasis. The interlobium is drawn somewhat to the left so that a dystelactic component may also be suspected. In inferior lingula portion, also very discrete signs of bronchiectasis. A pulmonary sequestration is very unlikely. Otherwise nothing noticable. Result: Bronchiectasis as well as residual infiltrate in the left lower lobe apex, as described. Chest X-ray pa/lateral on 2/12/1998: Somewhat augmented structures basal on both sides. No indication of an acute infiltrate. Konsiliar-Examinations: Lung functions on 5/4/1998: As far as one can judge not having optimal compliance: VC-max 0,93 - 59,4 % of set-point, FVC 0.93 -62.6 % of set-point, FEV-1 0.85 - 68.0 % of set-point, FEV-1 relating to %max. 91.9 - 107.3 % of set-point, MEF-50 1.22 - 61.6 % of set-point. Restricted curvature, large spirometry planed. Therapy and Progress: The initial IVIG substitution with Endobulin 400 mg/kg KG about every five weeks was not sufficient. Substitution cycle was changed to every three weeks so that IgG values always above 400 mg/dl, last on 3/31/1998: 447 mg/dl and on 4/27/1998: 433 mg/dl. Starting in august 1997 till 12/2/1997 oral antibiotic therapy using Klacid 250 mg 2 x 1/2 ML daily. Due to increased coughing and mucus formation change of antibotic therapy to Augmentin 312.5 mg/5 ml 3 x 1 ML. Using Augmentin marked improvement of symptoms, decreasing mucus formation and reduced coughing. Therapy with Augmentin was continued till 2/11/1998. Ten days after discontinuing the antibiotic therapy, on 2/22/1998, a middle ear inflammation occurred on the left side. Ambulatory start of another antibiosis with Augmentin for about three weeks. Middle ear inflammation healed without complications. In addition to the middle ear inflammation, symptoms of an air passage infection developed in the above described observation period. Last symptoms noticed on 4/23/1998. No fever and no increased CRP. General condition always good. According to Xxx's mother, he inhaled rather regularly 3 x daily 1 1/2 to 2 ml 2% salt solution using "Pari-Inhalierboy" as secretolytic agend. About five minutes before inhalation of salt solution 2 dosages of Bricanyl using the pre-chamber. Furthermore, Xxx does physiotherapy in our department about once a week (inhalation therapy, autogenetic drainage). According to Xxx's mother he integrated well into the kindergarten and is generally a bright boy. Present therapy: Nasivin 0.025 for about another week, inhalation therapy: 3 x 2 dosages Bricanyl using the pre-chamber, than hypertonic salt solution, 3 x 1 1/2 to 2 ml 2% NaCl. Regular physiotherapy. Next IVIG-therapy: 8 g Endobulin once a week starting on 5/11 to 5/15/1998. Note: The parents were informed that no FSME protection exists. Therefore a special immunglobulin dose is recommended if infested with ticks. The local paediatrician vaccinated for tetanus, flu, and pneumococcus. A continuous molecular/genetic clarification of the immune deficiency. Sincerely, Prim. Univ. Doz. Dr. XXXXX. Ass. Dr. XXXXX Kopies to: Herrn Dr. XXXXX Roseggerstr. 18
Frau Dr. XXXXX Mautstr. 17 A
State Hospital Salzburg Children's Hospital
KH St. Pölten Department for children and youth Hr. OA Dr. XXXXX Propst Führer-Str. 4
Patient: XXXXXX Xxx, born on 7/1/1992 Diagnosis: Suspected common variable immune deficiency with chronic relapsing respiratory infections
Dear Walter, In-patient report from 8/26 -8/28/1997. Anamnesis: You know the case history very well. To summarize: Xxx had since October 96 three pneumonia cases between repeated episodes of bacterial bronchitis. General delay and insufficient reaction to antibiotic therapies. Previously existed no special susceptibility to infections. This year, in July, Xxx had to be admitted into our hospital again to be treated for pneumonia. Common variable immune deficiency (CVID) was suspected. At this time, the immunology tests had not been completed. X-rays, submitted by Xxx, don't show strong pneumonic infiltration, on the same spot on the left lower lobe. Localization varies. Furthermore there are changes on the right lung. To judge anatomical conditions, Xxx will be admitted for bronchoscopy. Findings on Admission: Good general condition. Still heavy and productive cough with purulent secretion. Some intensified breathing sounds and left some basal dullness, normal ENT status, cor NAD, abdomen NAD, development and neurological normal. Findings: Bronchoscopy Report: Larynx, trachea, and bronchies anatomical normal. On both main bronchi sufficient yellowish purulent secretion. Left more than right. Slight chronic inflammation of mucous membrane. No stenosis, no FK Diagnosis: Bronchiectasis with CVID Bronchial Mucus: Enlarged streptococcus, haemophilus and branhamella catarrhalis,
BC: Leuco 19,000 Erythro 5.4 mio., Hb 13.7, Hkt 40%, thrombo 479,000 Diff: Eo 3, Seg 45, Ly 45, Mono 75. Electrolyte, urea, krea, CHE, protein, coagulation and CRP in normal range. Beurteilung: We substituted 400mg/kg immunoglobulin before the bronchoscopy because the last dose of immunoglobulin was already three weeks old. The bronchoscopy confirmed a suppurativ pulmonary disease on both lungs. Left stronger than right. No anatomical narrowness or FK. I think that the immune deficiency explains these changes. Since the left side is visibly worse than the right side, I would suggest a HR-CT in about one year. Sometimes this form of bronchiectasis localized strongly on one lobe. Surgical removal may make sense. CT images will help in making a decision. At the time, as mentioned, also clear changes on the right. The X-ray doesn't show the changes as well as the bronchoscopy. I would suggest to start an antibiotic therapy with Klacid oral for about ½ a year. This antibiotic is antibacterial and also reduces mucification. Recent studies show success when used to treat bronchiectasis. An additional physiotherapy with autogenic drainage or "flutter therapy" or something similar seems necessary to eliminate secretions (similar as done for mucoviscidosis). The immunoglobulin substitution will also be necessary. In case the condition worsens seriously, even though he is in antibiotics therapy, one has to expect the germs haemophilus and branhamella catarrhalis. Both germs are generally not very sensitive to the macrolid antibiotic. Nevertheless, I recommend a permanent therapy of this antibiosis. In case of an acute exacerbation, a therapy with amino penicillin or a second generation cephalosporin, like Curocef or Mandokef would seem appropriate. Thank you again for the referral and for the good cooperation. I'd like to apologize again for the delay of this letter. Sincerely yours,
Sepp Riedler P.S.: Dear Walter, please excuse the delay of this physician letter. But you certainly know the strange paths of such letters.
General Public Hospital St. Pölten Department for Children and Youth - Head of Department: Prim. Univ. Doz. Dr. XXXXX
Frau Dr. XXXXX Schillerstr. 7 3300 Amstetten
Record on Discharge
Dear colleagues, This is the hospitalization report for XXXXXX Xxx, born on 7/1/1992, from 7/1 - 7/5/1994, ambulatory check-up on 7/21/1994. Diagnosis: Thorough examination during unexplained convulsions, Condition after febrile convulsion in August 1993, Systolic murmur Anamnesis: Uneventful pregnancy and normal vaginal spontaneous delivery in the 41st week of gestation. BW 3600g, Apgar score 9/10/10. Normal psychomotor development. One hospital visit due to febrile convulsions during Scarlet fever in August 1993. Family anamnesis normal. Admission of the child because of unexplained convulsions. For example, during a fight with other children he would suddenly stop breathing, shake throughout the whole body and would for a view minutes lose consciousness. Sometimes the mother ended the attack by applying a towel soaked in cold water. For the first time, these convulsions appeared after the febrile convulsion in August 1993. Admission Status: Two year old boy in good general condition, a little restless, defensive, multiple mosquito bites in the face and extremities. Cor: 80/min, weak systolic murmur in the 2nd to the 3rd intercostal space. Pulmo: vesicular respiration. Abdomen soft, liver and spleen impalpable, normal peristalsis, femoral pulse palpable on both sides, genitals normal male testis scrotum, tympanic membrane on both sides only partially visible, bland, throat bland. Blood, Serum, Urine, and Culture Findings: BSG 2/6 mm/h Blood count: Leuko 10.1 G/I, Ery 4.33 T/l, Hb 12.3 g/dl, Hk 34.1 %, Thrombo 325 G/l Diff. blood count: in normal range for his age Biochemical profile (consisting of electrolytes of liver, kidney, phosphate, bilirubin, blood-sugar, albumin, CRP): LDH 287 U/l, bilirubin 122 mg/dl, other values in normal range. Urine findings: Specific weight 1005, pH 7,0, chemical and microscopical NAD. Weight: 12.2 kg (50. - 75. percentile). Height: 90 cm (50. - 75. percentile). Relative weight: 94 % BMI 15.06 Imaging Techniques: ECG: Sinus rhythm, normal type, negative T in V1 and V2. Chest X-ray: normal Cerebral MRI: normal EEG: Within the normal range. No pathological signs. No signs of increased cerebral irritability. Konsiliarius Findings: Fundus report: Fundi on both sides normal. Therapy and Progress: Under hospital supervision, the child was clinically normal. The findings, except for slightly elevated bilirubin levels, were normal. The sporadic systolic murmur may perhaps be accidental. ECG and chest X-ray were normal. We recommend an echocardiographic check-up in our ultrasound ambulance. Nutrition at discharge: Infant food. Therapy at discharge: None. Check-ups: Discussion of findings in our ambulance on 7/21/1994 at 8:00 o'clock. Echocardiographic check-up with chest X-ray on 9/1/1994 at 8:30 o'clock (physician referral requested). Ambulatory check-up on 7/21/1994 Anamnesis: At this time, the child has had no attacks. According to his mother, the attacks occur whenever the child can't have its way. Procedure: Considering findings and anamnesis we conclude that the attacks may likely be affective spasms. Therefore no therapy is necessary at this point. If the attacks reoccur, check-up at our ambulance with a child psychologist. In addition we recommend if no attacks occur a final EEG check-up in six months. Yours sincerely,
Prim. Univ. Doz. Dr. XXXXX Dr. XXXXX
Copy to: Herrn Dr. XXXXX Roseggerstr. 18 3385 Prinzersdorf
General Public Hospital St. Pölten Department for Children and Youth - Head of Department: Prim. Univ. Doz. Dr. XXXXX
Herrn Dr. XXXXX 3385 Prinzersdorf
Record on Discharge Dear colleagues, This is the in-patient report for XXXXXX Xxx, born on 7/1/1992, from 8/9 - 8/16/1993. Diagnosis: Scarlet fever and first febrile convulsions, respiratory tract infection with purulent rhinitis, aphthous stomatitis, secondary infection dig. indic. man. dext. Anamnesis: Admission of the child via emergency vehicle after a questionable first attack, with fall to the back, convulsions on all extremities. You could not talk to the child for about one minute. Subsequently very tired. Temperature slightly increased at admission 37.9 °C Status at Admission One year and one month old boy. At the time of admission already in good general condition. Palatoglossal arch slightly red, phimosis, otherwise internal normal, secondary ruptured crush injury on the distal joint of right index finger. Temperature at admission 37.9 °C. Serum, Blood, and Urine Findings: BSG at admission 4/13 mm/h, just before discharge 30/65 mm/h. Requested check-up with pediatrician in two weeks. Blood count before discharge: Ery 4.13 T/l, Leuko 5.1 G/1 Hb 11.2 g/dl, Hk 32.9 %, Thrombo 212 G/l. Diff. blood count before discharge: Baso 2 %, Mono 2 %, Lympho 87 %, 2 kinds of stimulation. CRP at admission 0, check-up 1.2 mg/dl. Electrolytes, kidney, and liver values normal Blood sugar 118 mg/dl at admission (child with full stomach). On empty stomach normal. Astrup before discharge normal. Blood culture: Aerob and anaerob cultures no growth. Urine: chemical and microscopic normal Weight at admision 9.95 kg, on discharge 9.8 kg. Length: 82 cm Imaging Techniques: Chest X-ray: discrete perihilar structural augmentation, in the sense of peribronchitical changes, otherwise normal. Therapy and Progress: During the first three days in the hospital the temperature of the child increased up to 40 °C, a purulent rhinitis and a productive cough developed. On the third day, near the incisors, an aphtous stomatitis developed. On the fourth day the temperature of the child went back to normal, typical Scarlet fever. Due to normal inflamatory, the child didn't receive any antibiotics during the hospitalization. We treated the secondary infection on the index finger with Betaisodon - bath and ointment. Continuation of this treatment with check-up at the emergency department was recommended. The respiratory tract infection was treated with Mucosolvan ointment. Vibrocil nosegel alternated with children's nose ointment was used. The child also inhaled NaCl and Berodualin. At the time of discharge, the child's temperature was down to normal, the exanthem defloresced. The aphtous stomatitis, which was treated with Xylocalin and brush applications with Pyralvex, was clearly reduced, lung clear, the wound on the finger improving. EEG stayed normal for his age, without signs of increased cerebral excitability, without signs of diffused or local cerebral dysfunction. Nutrition at Discharge: Milumil 2 and baby food
Therapy at Discharge: 2 x 2,5 ml Mucosolvan juice, Vibrocil nosegel alternated with children's nose ointment, Beta ointment right index finger, 3x/day. Inhaltion with 1,5 ml NaCl + 10 drops Berodualin with Pari inhalator for three months.
Dear Colleague, Please excuse the delay of this letter. The reason for this delay is the extreme shortage of administrative personnel.
Sincerely,
Prim. Univ. Doz. Dr. XXXXX Ass. Dr. XXXXX
General Public Hospital St. Pölten Neurological Department - Head of the Department: Prim. Dr. XXXXX XXXXXX Xxx, born on 7/1/1992 St. Pölten, Trautsonstr. 11
EEG Findings Basis for the Examination: 12 convexity electrodes, 16-channel-EEG-unit (Beckman), bipolar and common averaged reference. Conditions for EEC on Patient: Good Child sleeps spontaneously. In light spontaneous sleep general theta-delta-activity, with typical location of theta-delta-activity, with sleep spindles in typical location. During the whole procedure, the graph stayed the same. Summary: Sleep EEG normal for patient's age, in spontaneous sleep general theta-delta with beta spindles on typical location, typical vertex peaks. No signs of increased cerebral excitability, no signs of diffused cerebral dysfunction. No signs of local cerebral dysfunction.
OA Dr. XXXXX
General Public Hospital St. Pölten Department for Children and Youth - Head of Department: Prim. Univ. Doz. Dr. XXXXX
Kinderspital Salzburg z.H. Hr. Doz. Dr. XXXXX Müllner Hauptstr. 48 5020 Salzburg
Record on Discharge
Dear Josef, Dear colleagues, As agreed on the telephone, our patient XXXXXX Xxx, born on 7/1/1992 (hospitalized in our department from 7/14 - 7/28/1997) is referred to you for bronchoscopy and further pulmonary clarification. Diagnosis: Relapsing lower left lobe pneumonia. Patient is suspected of having bronchiectasis or fibrotic lungprocess. In this section, relapsing posterior upper lobe pneumonia. CVID (common variable immunodeficiency) is suspected. Anamnesis: Xxx was at our hospital from 7/14/1997 - 7/28/1997 because of the above mentioned basic disease. Pneumonia on the left lower lobe region has been recurring since November 1996 three times (October 1996, 2 x in November 1996). Since that time Xxx has had recurring bronchitis, large amounts of sputum was the dominating symptom. The external antibiotic treatment resulted in relatively quick improvement, but the disease took a rather chronic course. The existing susceptibility to infections is not unusual. The mother mentioned occasional otitis without special complications. Xxx has been in our hospital twice. A copy of the physician letter is enclosed. Periodically, Xxx has been admitted in our hospital because of respiratory tract symptoms with high temperatures, productive cough, and abdominal pain. The result of our clinical examinations showed clear signs of pneumonia. Family anamnesis: On mother's side and grandparents predisposed to Psoriasis, a allergy anamnesis is negative, also a external test on the patient himself showed nothing unusual. Social anamnesis: no siblings Xxx has been in Kindergarten for one year. Vaccination complications: none so far. Vaccinations: see certificate of vaccination Previous diseases: affect spasms, AT in November 1995 and the above mentioned relapsing otitis. Childhood diseases: until now none Stool, urine, and miction normal Findings on admission: Five year-old, delicate boy in reduced general condition, feverish, pale, and frequent coughing. Clinical signs: the right eardrum vascular injection, arched to the front, left questionable discharge, but bland. Pulmo: slight tachypnoea with frequency of about 30/min, in the middle on the left side distinctive, dry bronchial breathing. The expansion could be heard to the axillary line. Basal damping of 3 to 4 cm, hypersonorous percussion sound above the right lung, a few isolated rales, discrete obstructive component, otherwise normal. Weight at admission 17.5 kg (10th - 25th percentile, in respect to body length). Length 113 cm (just above the 50th percentile). Blood, serum, urine, and culture findings: The initial blood count showed with deviation to the left a leucocytosis of 29.9 G/l with almost normal red blood count. Blood count on discharge on 7/25/1997: Hb 11.3 g/dl with 4.4 T/l Ery, 6.0 G/l Leuko and 519 G/l thrombocytes, differentiated according to age. Electrolyte, kidney values, and liver values in normal range. Bilirubin with 1.21 mg/dl slightly increased. Protein 5.72 g/dl CRP 10.4 mg/dl Electrophoresis showed a distinct reduction of gamma-fraction with 1.4 % which is 0.8 g/l, alpha-1 4.1 %, Alpha-2 19.5 % beta 11.1 % albumin 63.9 %, albumin/globulin quotient 1.77. Repetition of electrophoresis resulted in almost the same values. Humoral immune status: see enclosed copy of findings. HIV-antibody negative Candida antigen negative Cellular immune status has not been finished. Probably finished next week. Direct questions to Mrs. Prof. Dr. XXXXX in Vienna. Urine chemically and sedimentation normal Sputum culture on 7/14/97: Branhamella catarrhalis and greenish streptococcus. Sweat test: 25 mmol/l NaCl. Mendel Mantoux test negative. Imaging Techniques: See enclosed images, please return originals. Chest ultrasound on 7/16/1997: On the left, basal is a small, partially chambered pleural effusion of insignificant size. Therapy and Progress: Due to the initial unawareness of the immunological situation, the patient was treated with Augmentin for five days. Based on insufficient response, we switched the antibiotic therapy to Curocef and Certomycin. Using this treatment, the inflammation reduced quickly. Xxx had no temperature and was complaint free. Noticeable was a massive production of grey-greenish sputum which reminded of expectoration of CF patients. After receipt of a second immunological evaluation, we administered, beginning on 7/25 until 7/27/1997, fractionated Endobulin, altogether 6.8 g, which is 400 mg/kg KG. Supporting provisions consisted of moistening of air: Bricanyl, salt solution, and easy physiotherapy. Discharge occurred on 7/28/1997 in good general condition but distinctive clinical residues in the left lower field. We continued ambulatory antibiosis with Biocef until beginning of August. Xxx continued to inhale at home. Ambulatory check-up on 8/18/1997: Xxx had no complaints, in left middle and lower field distinctive auscultatory medium, moist, bubbling rales. Hint of a otitis media sin. and relapsing conjunctivitis. Comment: I think, the pulmonary problem with persistent infiltrations in the left lower lobe, can be brought in connection with CVID (common variable immune deficiency). Bronchiectasis or fibrotic lung changes in this region are possible. A congenital immune deficiency seems unlikely since Xxx developed normally and showed an almost normal susceptibility to infections until 1996. An inborn lung abnormality would certainly have been noticed during the hospitalization in July 1994. At that time a chest X-ray was considered normal. Unfortunately, the cellular immune status has not been submitted yet, therefore an exact differentiation is not possible at this time. Based on the bronchoscopy, we hope to receive an overview of the anatomical situation and perhaps sensible therapy possibilities. Depending on symptoms, we plan to administer substitution-immunoglobulin. We also plan check-up on progress. I would appreciate additional diagnostic and therapeutic Tips.
Greetings to the Team in Salzburg,
OA Dr. XXXXX
Copies of the letters to:
Frau Dr. XXXXX Mautstr. 17A
Herrn Dr. XXXXX Roseggerstr. 18
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine
A.ö. Krankenhaus St. Pölten Ki-Abt. Ii Propst Führerstrasse 4
Prot. Nr.: 56762/ 7/23/97 Patient: XXXXXX Xxx 7/1/1992 AZ: 97-101011 Station: Kinder-II
Referral diagnosis: rez. Pneumonia Humoral Immune Status
Analysis Value Normal range ______________________________________________________________________________________
You will find data on German copies
Comments: Preliminary findings: Serumim immunoglobulin heavily reduced, in the sense of a agammaglobulinaemia, signs of inflammation. Massive antibody deficiency, urgent suspicion of primary antibody deficiency, check-up and evaluation of cellular immune status in the near future (sending of fresh heparin blood, after consultation) absolutely necessary. Live vaccine contra-indicated. IVIG substitution therapy is based on these findings absolutely necessary. Please consult.
Univ. Doz. Dr. XXXXX i.V. Prof. Dr. XXXXX
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine
A.ö. Krankenhaus St. Pölten Propst Führerstrasse 4 Prot. Nr.: 56762/ 7/23/97 Patient: XXXXXX Xxx 7/1/1992 AZ: 97-101011 Station: Kinder-II
Referral diagnosis: rez. Pneumonia Humoral Immune Status
Analysis Value Normal range ______________________________________________________________________________________
You will find data on German copies
Comments: Serum immunglobulin massively reduced in the sense of agammaglobulinaemia, signs of inflammation. Titration analysis showed low or almost no specific antibodies. Massive antibody deficiency, urgent suspicion of primary antibody deficiency, check-up and evaluation of cellular immune status in the near future (sending of fresh heparin blood, after consultation) absolutely necessary. Live vaccine contra-indicated. Based on these findings IVG substitution is absolutely necessary.
Univ. Doz. Dr. XXXXX i.V. Prof. Dr. XXXXX
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine Same as previous page A.ö. Krankenhaus St. Pölten Propst Führerstrasse 4 Prot. Nr.: 56762/ 7/23/97 Patient: XXXXXX Xxx 7/1/1992 AZ: 97-101011 Station: Kinder-II
Referral diagnosis: rez. Pneumonia Humoral Immune Status
Analysis Value Normal range ______________________________________________________________________________________
You will find data on German copies
Comments: Serum immunglobulin massively reduced in the sense of agammaglobulinaemia, signs of inflammation. Titration analysis showed low or almost no specific antibodies. Massive antibody deficiency, urgent suspicion of primary antibody deficiency, check-up and evaluation of cellular immune status in the near future (sending of fresh heparin blood, after consultation) absolutely necessary. Live vaccine contra-indicated. Based on these findings IVG substitution is absolutely necessary.
Univ. Doz. Dr. XXXXX i.V. Prof. Dr. XXXXX
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine
A.ö. Krankenhaus St. Pölten Propst Führerstrasse 4 Prot. Nr.: 56771/ 7/28/97 Patient: XXXXXX Xxx 7/1/1992 AZ: Station:
Referral diagnosis: CVID?
Humoral Immune Status
Analysis Value Normal range ______________________________________________________________________________________
You will find data on German copies
Comments: Serious antibody deficiency in the sense of agammaglobulinaemia. At the time signs of inflammation. Specific antibodies not detectable. These findings confirm the previous findings of 7/23/97. The findings show an absolute necessity for regular, inravenous IgG substitution therapy. Dosage, for example, Endobulin 400 mg/kg body weight, every 3 - 4 weeks. Live vaccine contra-indicated. To monitor IVIG substitution, we recommend the next immunologic check-up in two weeks. Univ. Doz. Dr. XXXXX i.V. Prof. Dr. XXXXX Vienna, 9/3/97
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine
A.ö. Krankenhaus St. Pölten Propst Führerstrasse 4 Prot. Nr.: 56771/ 7/28/97 Patient: XXXXXX Xxx 7/1/1992 AZ: Station:
Referral diagnosis: CVID?
Cellular Immune Status
Analysis Value Normal range ______________________________________________________________________________________
You will find data on German copies
Univ.-Prof. Dr. XXXXX Specialist for Internal Medicine Page 2
Prot. Nr.: 56771/ 7/28/97 Patient: XXXXXX Xxx 7/1/1992 AZ:
Analysis Value Normal range ______________________________________________________________________________________ Comments: Unable to proof B-cells in the periphery. These findings correspond with the diagnosis of inherited X-chromosomal agammaglobulinaemia (bruton's agammaglobulinaemia). Activated T-cells increased in number, T-, NK-cells, CD4- and CD8-subset in normal range or increased in number, (T-cells) excitability of lyphocytes with mitogen in normal range, with anitgen (tet tox) now but detectable. Recommend tetanus booster inoculation, 4 - 6 weeks later check-up on cellular immune status. Univ. Doz. Dr. XXXXX i.V. Prof. Dr. XXXXX
Vienna, 9/3/1997
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