Tumor-induced hypophosphatemic rickets in an adolescent boy--clinical presentation, diagnosis, and histological findings in growth plate and muscle tissue.

CONTEXT: The mechanism behind disabling muscle weakness in tumor-induced hypophosphatemic rickets is obscure. Histological investigation of growth plate tissue of patients with tumor-induced osteomalacia has so far not been reported. PATIENT: A mesenchymal tumor was detected in the left distal fibula by (68)Ga-DOTATOC in a 17-yr-old boy with adolescent onset of severe hypophosphatemic rickets. Disabling muscle weakness improved within days after surgery, and normal mobility was restored within months. METHODS AND RESULTS: The resected tissue included part of the growth plate allowing immunohistochemical investigation. Positive staining of FGF23 was found in the tumor cells and in hypertrophic chondrocytes, osteoblasts, and osteoclasts of the adjacent growth plate. This distribution matched that found in growth plate tissue of a healthy control. We found positive staining for the somatostatin receptor not only in the tumor but also within the growth plate and adjacent bony tissue in the patient and the healthy control. Muscle tissue provided evidence for a partial defect in respiratory chain complexes I-IV. Biochemical markers were nearly or completely restored to normal 12 months after surgery. CONCLUSIONS: Hypertrophic growth plate chondrocytes are a target or source of FGF23 in tumor-induced osteomalacia. Low serum phosphate, FGF23, or other factors produced by the tumor may interfere with mitochondrial function.

[Femoral head dysplasia and its relationship to femoral head necrosis (Perthes' disease)]. / Dysplasia Capitis Femoris und ihre Beziehung zur Hüftkopfnekrose (Morbus Perthes).

Fifty-five patients totaling 98 hips afflicted by Dysplasia Capitis Femoris (DFC), monitored between 1960 and 1983, were analyzed. Mean follow-up: 8 years and 4 months (minimum 2 years, maximum 22 years and 2 months). Forty-three (78%) patients were males; fourteen (25%) were females. Non displayed any alteration in other osteoarticular segments. In 24 of the 98 affected hips it was possible to determine the exact radiological age appearance of the Epiphyseal Nucleus. Thirty-seven of the 55 patients had moderate signs and symptoms which were of short evolution. According to presence or absence of necrotic signs of the femoral epiphysis, two types of lesions were classified: Type 1: Pure Dysplasia Capitis Femoris (88 hips). Type 2: Dysplasia Capitis Femoris with unequivocal signs of necrosis (10 hips). Upon review of the 98 diseased hips, 80 (81.63%) had been fully repaired, while 18 (18.37%) were in reparatory stage. From the 80 fully healed hips, 56 were normal; 18 exhibited a Spherical Coxa Magna and 6 had a non-spherical epiphysis. As treatment concerns, patients with D.C.F. Type 1-A or Type 1-B only require observation and periodical control in addition to moderate restrictions in physical activities upon presence of symptomatic pain that is transitional. Patients with D.C.F. Type 2 must be treated as a classic case of Perthes Disease. Finally, a hypothesis of their etiopathogenis and its relationship to Perthes Disease is brought forth.

[Long-term follow-up of 166 surgically treated patients with congenital hip dislocation]. / Langfristige Beobachtung von 166 operierten Patienten mit angeborener Hüftverrenkung.

UNLABELLED: 166 patients with congenital dislocation of the hip, ranging from 10 months to 5 years old, were operated between 1958 and 1971. 140 patients were women (84%) and 26 patients were men (16%). 96 patients had unilateral and 70 patients had bilateral dislocation; that makes a total of 236 dislocated hips. 61 patients with bilateral hip dislocation were operated simultaneously on both hips. All the patients were treated with adductor tenotomy and open reduction through Smith Petersen incision with section or elongation of the psoas tendon. The postoperative immobilization consisted in a period of one month pelvic toecast followed by 2 plaster casts with abduction rod during 3 to 5 months. The postoperatory follow-up ranged from 9 years to 26 years, 2 months; average 14 years, 5 months. The clinical evaluation comprises pain, hip mobility, gait and muscle power. RESULTS: excellent 138 (59%); good 66 (28%); fair 29 (12%); and poor 3 (1%). The radiological evaluation considered: Mose; acetabular femoral head index; Wiberg's CD-angle; cervico-diaphysiary angle and radial quotient. RESULTS: normal 5 (2%); excellent 44 (19%); good 78 (33%); subtotal 54%; fair 90 (38%), and poor 19 (8%). Complications (hips); infections 12 (5%); 3 of them deep ones (1%); residual subluxation 19 (8%); reluxation 1 (0.5%). Isquemic necrosis Grade II of Tönnis 31 (13%); Grade III 2 (1%) and Grade IV 1 (0.5%).